Inverse comorbidity in multiple sclerosis: Findings in a complete nationwide cohort.

PubMed

Thormann, Anja; Koch-Henriksen, Nils; Laursen, Bjarne; Sørensen, Per Soelberg; Magyari, Melinda

2016-11-01

Inverse comorbidity is disease occurring at lower rates than expected among persons with a given index disease. The objective was to identify inverse comorbidity in MS. We performed a combined case-control and cohort study in a total nationwide cohort of cases with clinical onset of MS 1980-2005. We randomly matched each MS-case with five population controls. Comorbidity data were obtained from multiple, independent nationwide registries. Cases and controls were followed from January 1977 to the index date, and from the index date through December 2012. We controlled for false discovery rate and investigated each of eight pre-specified comorbidity categories: psychiatric, cerebrovascular, cardiovascular, lung, and autoimmune comorbidities, diabetes, cancer, and Parkinson's disease. A total of 8947 MS-cases and 44,735 controls were eligible for inclusion. We found no inverse associations with MS before the index date. After the index date, we found a decreased occurrence of chronic lung disease (asthma and chronic obstructive pulmonary disease) (HR 0.80 (95% CI 0.75-0.86, p<0.00025)) and overall cancer (HR 0.88 (95% CI 0.81-0.95, p=0.0005)) among MS-cases. This study showed a decreased risk of cancers and pulmonary diseases after onset of MS. Identification of inverse comorbidity and of its underlying mechanisms may provide important new entry points into the understanding of MS. Copyright © 2016 Elsevier B.V. All rights reserved.

Oral cavity squamous cell carcinoma - characteristics and survival in aboriginal and non-aboriginal Western australians.

PubMed

Frydrych, A M; Slack-Smith, L M; Parsons, R; Threlfall, T

2014-01-01

Squamous cell carcinoma (SCC) is the most common type of malignancy affecting the oral cavity. While exposures to main risk factors for oral SCC such as smoking and alcohol use are higher amongst the Aboriginal people, little is known about oral cancer in this population. This study aimed to describe characteristics and survival of oral SCC in Aboriginal and non-Aboriginal Western Australians. All primary oral SCC cases reported to the Western Australian Cancer Registry (WACR) between 1990 and 1999 were analysed with respect to person characteristics including: date of birth, sex and indigenous status; and disease characteristics including: date of biopsy, disease stage and site as well as date of recurrence and date of death. Exclusion criteria included diagnosis not based on incisional or excisional biopsy, diagnosis other than oral SCC or a history of another malignant neoplasm. Aboriginal individuals were more likely to reside in rural areas. No statistically significant differences in oral SCC characteristics and survival were noted between Aboriginal and non-Aboriginal Western Australians. This study provides new information on person and disease characteristics of Aboriginal Western Australians diagnosed with oral SCC.

[Record linkage of a large clinical practice patient cohort with the Cancer Registry Schleswig-Holstein].

PubMed

Obi, N; Waldmann, A; Babaev, V; Katalinic, A

2011-07-01

A precondition for the evaluation of outcomes in cohort studies and screening programmes is the availability of follow-up data. In Germany, established cancer registries provide such data for incident primary cancer diseases and mortality. To utilise these cancer registry data a person's identifying code has to be correctly linked to study or programme records, a procedure which, up to date, has been only rarely used in Germany. Exemplarily, the feasibility and validity of record linkage of a cohort of 173 050 patients from the Quality-assured Mamma Diagnostic programme (QuaMaDi) to the cancer registry Schleswig-Holstein was assessed by the accuracy of the classified outcome. Name, date of birth and address of the QuaMaDi cohort members were coded in the confidential administration center of the registry. These codes were passed by the codes of 129 455 female cancer registry records. Datasets were synchronised for each match, so that QuaMaDi participants could be identified in the registry file. In a next step epidemiological registry records were linked to the QuaMaDi study records. The accuracy of classifying outcome was assessed by agreement measures, i. e., Cohen's kappa. In cases of disagreement, a questionnaire has been sent to QuaMaDi patients' gynaecologists to validate the final diagnosis. Synchronisation of both cohorts resulted in 18 689 one to one matches with any kind of malignant tumour, therein 8 449 breast cancers (ICD-10 C50, D05). Absolute agreement between files according to diagnosed or suspected breast cancer was 97.6% with a kappa value of 0.79. When suspicious BIRADS 4 cases from QuaMaDi were excluded, agreement and kappa rose to 99.5% and 0.948, respectively. After correction of the final diagnosis according to the physician's responses, agreement measures slightly improved in both groups of ascertained diagnosis including and excluding the suspected cases. Within QuaMaDi the diagnosed breast cancer cases were predominantly notified in the cancer registry. Discordant matches (false negatives and false positives) may have resulted due to various causes, thereof a very low percentage of record linkages from different persons. In conclusion, synchronisation of study cohort files to registry files using pseudonymous personal data is feasible and valid. The generated combined datasets can be used for comparative analysis of several objectives. One of them will be the evaluation of screening programmes in the near future. © Georg Thieme Verlag KG Stuttgart · New York.

Comprehensive description of clinical characteristics of a large systemic lupus erythematosus cohort from the Spanish Rheumatology Society Lupus Registry (RELESSER) with emphasis on complete versus incomplete lupus differences.

PubMed

Rúa-Figueroa, Íñigo; Richi, Patricia; López-Longo, Francisco Javier; Galindo, María; Calvo-Alén, Jaime; Olivé-Marqués, Alejandro; Loza-Santamaría, Estíbaliz; Vicente, Sabina Pérez; Erausquin, Celia; Tomero, Eva; Horcada, Loreto; Uriarte, Esther; Sánchez-Atrio, Ana; Rosas, José; Montilla, Carlos; Fernández-Nebro, Antonio; Rodríguez-Gómez, Manuel; Vela, Paloma; Blanco, Ricardo; Freire, Mercedes; Silva, Lucía; Díez-Álvarez, Elvira; Ibáñez-Barceló, Mónica; Zea, Antonio; Narváez, Javier; Martínez-Taboada, Víctor; Marenco, José Luis; de Castro, Mónica Fernández; Fernández-Berrizbeitia, Olaia; Hernández-Beriain, José Ángel; Gantes, Marian; Hernández-Cruz, Blanca; Pérez-Venegas, José J; Pecondón, Ángela; Marras, Carlos; Carreira, Patricia; Bonilla, Gema; Torrente, Vicente; Castellví, Iván; Alegre, Juan; Moreno, Mireia; Raya, Enrique; de la Peña, Paloma García; Vázquez, Tomás; Aguirre, Ángeles; Quevedo, Víctor; Pego-Reigosa, José M

2015-01-01

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multiple organ involvement and pronounced racial and ethnic heterogeneity. The aims of the present work were (1) to describe the cumulative clinical characteristics of those patients included in the Spanish Rheumatology Society SLE Registry (RELESSER), focusing on the differences between patients who fulfilled the 1997 ACR-SLE criteria versus those with less than 4 criteria (hereafter designated as incomplete SLE (iSLE)) and (2) to compare SLE patient characteristics with those documented in other multicentric SLE registries.RELESSER is a multicenter hospital-based registry, with a collection of data from a large, representative sample of adult patients with SLE (1997 ACR criteria) seen at Spanish rheumatology departments. The registry includes demographic data, comprehensive descriptions of clinical manifestations, as well as information about disease activity and severity, cumulative damage, comorbidities, treatments and mortality, using variables with highly standardized definitions.A total of 4.024 SLE patients (91% with ≥4 ACR criteria) were included. Ninety percent were women with a mean age at diagnosis of 35.4 years and a median duration of disease of 11.0 years. As expected, most SLE manifestations were more frequent in SLE patients than in iSLE ones and every one of the ACR criteria was also associated with SLE condition; this was particularly true of malar rash, oral ulcers and renal disorder. The analysis-adjusted by gender, age at diagnosis, and disease duration-revealed that higher disease activity, damage and SLE severity index are associated with SLE [OR: 1.14; 95% CI: 1.08-1.20 (P < 0.001); 1.29; 95% CI: 1.15-1.44 (P < 0.001); and 2.10; 95% CI: 1.83-2.42 (P < 0.001), respectively]. These results support the hypothesis that iSLE behaves as a relative stable and mild disease. SLE patients from the RELESSER register do not appear to differ substantially from other Caucasian populations and although activity [median SELENA-SLEDA: 2 (IQ: 0-4)], damage [median SLICC/ACR/DI: 1 (IQ: 0-2)], and severity [median KATZ index: 2 (IQ: 1-3)] scores were low, 1 of every 4 deaths was due to SLE activity.RELESSER represents the largest European SLE registry established to date, providing comprehensive, reliable and updated information on SLE in the southern European population.

The pooling of manpower and resources through the establishment of European reference networks and rare disease patient registries is a necessary area of collaboration for rare renal disorders.

PubMed

Parker, Samantha

2014-09-01

This review aims to provide guidance on emerging concepts and policy related to European reference networks (ERNs) for rare diseases (RDs) and the development and management of RD patient registries. A major problem facing many RDs including rare renal disorders is that patients do not have a specialist centre that they can attend where clinicians, working as a multidisciplinary team, are experts in the particular disease. Furthermore, for most RDs, no single centre, and in many cases no single country, has sufficient numbers of patients and resources to fully understand the natural history or to conduct clinical and translational research. Therefore, the pooling of manpower and resources through the establishment of ERN and RD patient registries is a common and necessary area of collaboration. The concept of European networks for RDs dates back to the early 2000s and the Commission launch of a call for European pilot reference networks for RDs. These networks of expert centres have been brought together through the desire for further knowledge and innovation in RD areas. Networks demand a holistic approach and long-term vision with close collaboration between clinicians, diagnostic laboratories, scientists, patients and their families. The development of legal measures for ERNs is in progress at the Commission and these networks will be a shared responsibility of the Commission and member states. In the context of ERNs, an essential activity is the patient registries. Patient registries are organized databases where patient information, including demographic, medical and family history, are collected, stored and available for retrieval via standardized and secure methods. Patient registries are increasingly recognized as crucial tools for RD research for which international collaboration is absolutely essential to understand the pathogenesis of rare genotypes, achieve a unified collection of phenotypic data, foster natural history studies providing the foundation for successful orphan drug development, facilitate studies to identify appropriate clinical endpoints or biomarkers, identify participants for research and clinical trials and support discussions with regulators including the safety and efficacy evaluation of potential therapies. Furthermore, patient registries are often used as part of regulatory decisions and post-marketing surveillance requirements. Data can be entered into a registry by patients, clinicians, researchers or directly imported from patient's health records. The major concern in maintaining the dynamic of these networks and registries is sustainability, as the infrastructures and coordination have a cost. © The Author 2014. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Standardization of Questions in Rare Disease Registries: The PRISM Library Project.

PubMed

Richesson, Rachel Lynn; Shereff, Denise; Andrews, James Everett

2012-10-10

Patient registries are often a helpful first step in estimating the impact and understanding the etiology of rare diseases - both requisites for the development of new diagnostics and therapeutics. The value and utility of patient registries rely on the use of both well-constructed structured research questions and relevant answer sets accompanying them. There are currently no clear standards or specifications for developing registry questions, and there are no banks of existing questions to support registry developers. This paper introduces the [Rare Disease] PRISM (Patient Registry Item Specifications and Metadata for Rare Disease) project, a library of standardized questions covering a broad spectrum of rare diseases that can be used to support the development of new registries, including Internet-based registries. A convenience sample of questions was identified from well-established (>5 years) natural history studies in various diseases and from several existing registries. Face validity of the questions was determined by review by many experts (both terminology experts at the College of American Pathologists (CAP) and research and informatics experts at the University of South Florida (USF)) for commonality, clarity, and organization. Questions were re-worded slightly, as needed, to make the full semantics of the question clear and to make the questions generalizable to multiple diseases where possible. Questions were indexed with metadata (structured and descriptive information) using a standard metadata framework to record such information as context, format, question asker and responder, and data standards information. At present, PRISM contains over 2,200 questions, with content of PRISM relevant to virtually all rare diseases. While the inclusion of disease-specific questions for thousands of rare disease organizations seeking to develop registries would present a challenge for traditional standards development organizations, the PRISM library could serve as a platform to liaison between rare disease communities and existing standardized controlled terminologies, item banks, and coding systems. If widely used, PRISM will enable the re-use of questions across registries, reduce variation in registry data collection, and facilitate a bottom-up standardization of patient registries. Although it was initially developed to fulfill an urgent need in the rare disease community for shared resources, the PRISM library of patient-directed registry questions can be a valuable resource for registries in any disease - whether common or rare. N/A.

Standardization of Questions in Rare Disease Registries: The PRISM Library Project

PubMed Central

Shereff, Denise; Andrews, James Everett

2012-01-01

Background Patient registries are often a helpful first step in estimating the impact and understanding the etiology of rare diseases - both requisites for the development of new diagnostics and therapeutics. The value and utility of patient registries rely on the use of both well-constructed structured research questions and relevant answer sets accompanying them. There are currently no clear standards or specifications for developing registry questions, and there are no banks of existing questions to support registry developers. Objective This paper introduces the [Rare Disease] PRISM (Patient Registry Item Specifications and Metadata for Rare Disease) project, a library of standardized questions covering a broad spectrum of rare diseases that can be used to support the development of new registries, including Internet-based registries. Methods A convenience sample of questions was identified from well-established (>5 years) natural history studies in various diseases and from several existing registries. Face validity of the questions was determined by review by many experts (both terminology experts at the College of American Pathologists (CAP) and research and informatics experts at the University of South Florida (USF)) for commonality, clarity, and organization. Questions were re-worded slightly, as needed, to make the full semantics of the question clear and to make the questions generalizable to multiple diseases where possible. Questions were indexed with metadata (structured and descriptive information) using a standard metadata framework to record such information as context, format, question asker and responder, and data standards information. Results At present, PRISM contains over 2,200 questions, with content of PRISM relevant to virtually all rare diseases. While the inclusion of disease-specific questions for thousands of rare disease organizations seeking to develop registries would present a challenge for traditional standards development organizations, the PRISM library could serve as a platform to liaison between rare disease communities and existing standardized controlled terminologies, item banks, and coding systems. Conclusions If widely used, PRISM will enable the re-use of questions across registries, reduce variation in registry data collection, and facilitate a bottom-up standardization of patient registries. Although it was initially developed to fulfill an urgent need in the rare disease community for shared resources, the PRISM library of patient-directed registry questions can be a valuable resource for registries in any disease – whether common or rare. Trial Registration N/A PMID:23611924

The Victorian Lung Cancer Registry pilot: improving the quality of lung cancer care through the use of a disease quality registry.

PubMed

Stirling, Rob G; Evans, S M; McLaughlin, P; Senthuren, M; Millar, J; Gooi, J; Irving, L; Mitchell, P; Haydon, A; Ruben, J; Conron, M; Leong, T; Watkins, N; McNeil, J J

2014-10-01

Lung cancer remains a major disease burden in Victoria (Australia) and requires a complex and multidisciplinary approach to ensure optimal care and outcomes. To date, no uniform mechanism is available to capture standardized population-based outcomes and thereby provide benchmarking. The establishment of such a data platform is, therefore, a primary requisite to enable description of process and outcome in lung cancer care and to drive improvement in the quality of care provided to individuals with lung cancer. A disease quality registry pilot has been established to capture prospective data on all adult patients with clinical or tissue diagnoses of small cell and non-small cell lung cancer. Steering and management committees provide clinical governance and supervise quality indicator selection. Quality indicators were selected following extensive literature review and evaluation of established clinical practice guidelines. A minimum dataset has been established and training and data capture by data collectors is facilitated using a web-based portal. Case ascertainment is established by regular institutional reporting of ICD-10 discharge coding. Recruitment is optimized by provision of opt-out consent. The collection of a standardized minimum data set optimizes capacity for harmonized population-based data capture. Data collection has commenced in a variety of settings reflecting metropolitan and rural, and public, and private health care institutions. The data set provides scope for the construction of a risk-adjusted model for outcomes. A data access policy and a mechanism for escalation policy for outcome outliers has been established. The Victorian Lung Cancer Registry provides a unique capacity to provide and confirm quality assessment in lung cancer and to drive improvement in quality of care across multidisciplinary stakeholders.

Designing exposure registries for improved tracking of occupational exposure and disease.

PubMed

Arrandale, Victoria H; Bornstein, Stephen; King, Andrew; Takaro, Timothy K; Demers, Paul A

2016-06-27

Registries are one strategy for collecting information on occupational exposure and disease in populations. Recently leaders in the Canadian occupational health and safety community have shown an interest in the use of occupational exposure registries. The primary goal of this study was to review a series of Canadian exposure registries to identify their strengths and weaknesses as a tool for tracking occupational exposure and disease in Canada. A secondary goal was to identify the features of an exposure registry needed to specifically contribute to prevention, including the identification of new exposure-disease relationships. A documentary review of five exposure registries from Canada was completed. Strengths and limitations of the registries were compared and key considerations for designing new registries were identified. The goals and structure of the exposure registries varied considerably. Most of the reviewed registries had voluntary registration, which presents challenges for the use of the data for either surveillance or epidemiology. It is recommended that eight key issues be addressed when planning new registries: clear registry goal(s), a definition of exposure, data to be collected (and how it will be used), whether enrolment will be mandatory, as well as ethical, privacy and logistical considerations. When well constructed, an exposure registry can be a valuable tool for surveillance, epidemiology and ultimately the prevention of occupational disease. However, exposure registries also have a number of actual and potential limitations that need to be considered.

CKD.QLD: chronic kidney disease surveillance and research in Queensland, Australia

PubMed Central

Venuthurupalli, Sree K.; Hoy, Wendy E.; Healy, Helen G.; Salisbury, Anne; Fassett, Robert G.

2012-01-01

Background Chronic kidney disease (CKD) is recognized as a major public health problem in Australia with significant mortality, morbidity and economic burden. However, there is no comprehensive surveillance programme to collect, collate and analyse data on CKD in a systematic way. Methods We describe an initiative called CKD Queensland (CKD.QLD), which was established in 2009 to address this deficiency, and outline the processes and progress made to date. The foundation is a CKD Registry of all CKD patients attending public health renal services in Queensland, and patient recruitment and data capture have started. Results We have established through early work of CKD.QLD that there are over 11 500 CKD patients attending public renal services in Queensland, and these are the target population for our registry. Progress so far includes conducting two CKD clinic site surveys, consenting over 3000 patients into the registry and initiation of baseline data analysis of the first 600 patients enrolled at the Royal Brisbane and Women's Hospital (RBWH) site. In addition, research studies in dietary intake and CKD outcomes and in models of care in CKD patient management are underway. Conclusions Through the CKD Registry, we will define the distribution of CKD patients referred to renal practices in the public system in Queensland by region, remoteness, age, gender, ethnicity and socioeconomic status. We will define the clinical characteristics of those patients, and the CKD associations, stages, co-morbidities and current management. We will follow the course and outcomes in individuals over time, as well as group trends over time. Through our activities and outcomes, we are aiming to provide a nidus for other states in Australia to join in a national CKD registry and network. PMID:23115138

Comparative study on the National Renal Disease Registry in America, England and Iran.

PubMed

Ajami, Sima; Askarianzadeh, Mahdi; Saghaeiannejad-Isfahani, Sakineh; Mortazavi, Mojgan; Ehteshami, Asghar

2014-01-01

A disease registry is a database that includes information about people diagnosed with specific types of diseases. The registry collects information that can be used for capturing, managing, and organizing specific information for patients. The aim of this study was to identify and compare the National Renal Disease Registry (NRDR) in selected countries including the United States, United Kingdom, and Iran. Retrieval of data of the NRDR performed through scholars responsible in related agencies, including the Ministry of Health and Medical Education, and Renal Disease charity, and data registries in the United States, United Kingdom, and Iran. This research was an applied and descriptive, comparative study. The study population consisted of the National Renal Disease Registry of the selected countries including the United States, United Kingdom, and Iran, from which data were collected using forms that were designed according to the study objectives. Sources of data were researchers, scholars responsible in related agencies, including the Ministry of Health and Medical Education, and Renal Disease charity, data registries, articles, books, journals, databases, websites, and related documents. Data were gathered through phone, e-mail, study, observation, and interview. The researchers collected data for each country based on the study objectives and then put them in comparative tables. Data were analyzed by descriptive, comparative, and theoretical methods. There is no NRDR in Iran to report the short- and long-term results of renal disease. Most of the renal transplant teams report their own results as single-center experiences. America and Britain have pre-eminent national registry of renal disease, compared to other countries. The Iranian Society of Nephrology should be actively involved to create a National Renal Registry in Iran. The registry should have representatives from the universities, government, armed forces, and private sectors. Researchers proposed to design the Iran National Renal Registry according to the UK Renal Disease Registry model because of its prominent healthcare system.

Use of Proton Pump Inhibitors and the Risk of Listeriosis: A Nationwide Registry-based Case-Control Study.

PubMed

Kvistholm Jensen, Anne; Simonsen, Jacob; Ethelberg, Steen

2017-04-01

Recent studies suggest that proton pump inhibitors (PPIs) may increase the risk for listeriosis. We investigated a potential association in cases of nonpregnancy-associated listeriosis using registry data. We conducted a population-based, case-control study using Danish health registries. Cases (n = 721) were defined as patients aged ≥45 years notified with listeriosis from July 1994 to December 2012. We selected 34800 controls using risk-set sampling. Controls were individually matched for age, sex, and municipality. Data on use of PPIs and other drugs and hospitalization diagnoses over a 5-year period were extracted from nationwide health registries. A comorbidity index (CMI) was constructed. We calculated the association between use of PPIs and related drugs within 30 days (current use) and other time windows before the index date. Using conditional logistic regression, matched odds ratios (ORs) adjusted for CMI and confounders were estimated. The adjusted OR for current use of PPIs and development of listeriosis was 2.81 (95% confidence interval [CI], 2.14-3.69). PPI usage up to 90 days before the index date remained statistically significant. Subgroup analyses revealed increasing ORs with decreasing age and level of comorbidity and an increased OR for concurrent glucocorticoid treatment (OR, 4.61; 95% CI, 3.01-7.06). No significant association was found for current use of histamine-2-receptor antagonists (adjusted OR, 1.82; 95% CI, 0.89-3.71). Prescribed PPIs were associated with increased risk of listeriosis. The risk waned with time since last prescription redemption. PPIs may have unwanted side effects in vulnerable populations. © The Author 2016. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail: journals.permissions@oup.com.

Assessing the feasibility of a web-based registry for multiple orphan lung diseases: the Australasian Registry Network for Orphan Lung Disease (ARNOLD) experience.

PubMed

Casamento, K; Laverty, A; Wilsher, M; Twiss, J; Gabbay, E; Glaspole, I; Jaffe, A

2016-04-18

We investigated the feasibility of using an online registry to provide prevalence data for multiple orphan lung diseases in Australia and New Zealand. A web-based registry, The Australasian Registry Network of Orphan Lung Diseases (ARNOLD) was developed based on the existing British Paediatric Orphan Lung Disease Registry. All adult and paediatric respiratory physicians who were members of the Thoracic Society of Australia and New Zealand in Australia and New Zealand were sent regular emails between July 2009 and June 2014 requesting information on patients they had seen with any of 30 rare lung diseases. Prevalence rates were calculated using population statistics. Emails were sent to 649 Australian respiratory physicians and 65 in New Zealand. 231 (32.4%) physicians responded to emails a total of 1554 times (average 7.6 responses per physician). Prevalence rates of 30 rare lung diseases are reported. A multi-disease rare lung disease registry was implemented in the Australian and New Zealand health care settings that provided prevalence data on orphan lung diseases in this region but was limited by under reporting.

Linked Registries: Connecting Rare Diseases Patient Registries through a Semantic Web Layer

PubMed Central

González-Castro, Lorena; Carta, Claudio; van der Horst, Eelke; Lopes, Pedro; Kaliyaperumal, Rajaram; Thompson, Mark; Thompson, Rachel; Queralt-Rosinach, Núria; Lopez, Estrella; Wood, Libby; Robertson, Agata; Lamanna, Claudia; Gilling, Mette; Orth, Michael; Merino-Martinez, Roxana; Taruscio, Domenica; Lochmüller, Hanns

2017-01-01

Patient registries are an essential tool to increase current knowledge regarding rare diseases. Understanding these data is a vital step to improve patient treatments and to create the most adequate tools for personalized medicine. However, the growing number of disease-specific patient registries brings also new technical challenges. Usually, these systems are developed as closed data silos, with independent formats and models, lacking comprehensive mechanisms to enable data sharing. To tackle these challenges, we developed a Semantic Web based solution that allows connecting distributed and heterogeneous registries, enabling the federation of knowledge between multiple independent environments. This semantic layer creates a holistic view over a set of anonymised registries, supporting semantic data representation, integrated access, and querying. The implemented system gave us the opportunity to answer challenging questions across disperse rare disease patient registries. The interconnection between those registries using Semantic Web technologies benefits our final solution in a way that we can query single or multiple instances according to our needs. The outcome is a unique semantic layer, connecting miscellaneous registries and delivering a lightweight holistic perspective over the wealth of knowledge stemming from linked rare disease patient registries. PMID:29214177

Linked Registries: Connecting Rare Diseases Patient Registries through a Semantic Web Layer.

PubMed

Sernadela, Pedro; González-Castro, Lorena; Carta, Claudio; van der Horst, Eelke; Lopes, Pedro; Kaliyaperumal, Rajaram; Thompson, Mark; Thompson, Rachel; Queralt-Rosinach, Núria; Lopez, Estrella; Wood, Libby; Robertson, Agata; Lamanna, Claudia; Gilling, Mette; Orth, Michael; Merino-Martinez, Roxana; Posada, Manuel; Taruscio, Domenica; Lochmüller, Hanns; Robinson, Peter; Roos, Marco; Oliveira, José Luís

2017-01-01

Patient registries are an essential tool to increase current knowledge regarding rare diseases. Understanding these data is a vital step to improve patient treatments and to create the most adequate tools for personalized medicine. However, the growing number of disease-specific patient registries brings also new technical challenges. Usually, these systems are developed as closed data silos, with independent formats and models, lacking comprehensive mechanisms to enable data sharing. To tackle these challenges, we developed a Semantic Web based solution that allows connecting distributed and heterogeneous registries, enabling the federation of knowledge between multiple independent environments. This semantic layer creates a holistic view over a set of anonymised registries, supporting semantic data representation, integrated access, and querying. The implemented system gave us the opportunity to answer challenging questions across disperse rare disease patient registries. The interconnection between those registries using Semantic Web technologies benefits our final solution in a way that we can query single or multiple instances according to our needs. The outcome is a unique semantic layer, connecting miscellaneous registries and delivering a lightweight holistic perspective over the wealth of knowledge stemming from linked rare disease patient registries.

Respiratory diseases registries in the national registry of rare diseases.

PubMed

Lara Gallego, Beatriz; Abaitua Borda, Ignacio; Galán Gil, Genaro; Castillo Villegas, Diego; Casanova Espinosa, Álvaro; Cano Jiménez, Esteban; Ojanguren Arranz, Iñigo; Posada de la Paz, Manuel

2014-09-01

This report describes the general characteristics, objectives and organizational aspects of the registries of rare respiratory diseases included in the National Registry of Rare Diseases of the Research Institute for Rare Diseases (ISCIII), in order to publicize their existence and encourage the participation of professionals. Information is collected on the following conditions: alpha-1 antitrypsin deficiency, idiopathic tracheal stenosis, adult pulmonary Langerhans' cell histiocytosis, lymphangioleiomyomatosis, alveolar proteinosis, and sarcoidosis. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

Creating a global rare disease patient registry linked to a rare diseases biorepository database: Rare Disease-HUB (RD-HUB).

PubMed

Rubinstein, Yaffa R; Groft, Stephen C; Bartek, Ronald; Brown, Kyle; Christensen, Ronald A; Collier, Elaine; Farber, Amy; Farmer, Jennifer; Ferguson, John H; Forrest, Christopher B; Lockhart, Nicole C; McCurdy, Kate R; Moore, Helen; Pollen, Geraldine B; Richesson, Rachel; Miller, Vanessa Rangel; Hull, Sara; Vaught, Jim

2010-09-01

A movement to create a global patient registry for as many as 7,000 rare diseases was launched at a workshop, "Advancing Rare Disease Research: The Intersection of Patient Registries, Biospecimen Repositories, and Clinical Data." http://rarediseases.info.nih.gov/PATIENT_REGISTRIES_WORKSHOP/. The workshop was sponsored by the Office of Rare Diseases Research (ORDR). The focus was the building of an infrastructure for an internet-based global registry linking to biorepositories. Such a registry would serve the patients, investigators, and drug companies. To aid researchers the participants suggested the creation of a centralized database of biorepositories for rare biospecimens (RD-HUB)http://biospecimens.ordr.info.nih.gov/ that could be linked to the registry. Over two days of presentations and breakout sessions, several hundred attendees discussed government rules and regulations concerning privacy and patients' rights and the nature and scope of data to be entered into a central registry as well as concerns about how to validate patient and clinician-entered data to ensure data accuracy. Mechanisms for aggregating data from existing registries were also discussed. The attendees identified registry best practices, model coding systems, international systems for recruiting patients into clinical trials and novel ways of using the internet directly to invite participation in research. They also speculated about who would bear ultimate responsibility for the informatics in the registry and who would have access to the information. Hurdles associated with biospecimen collection and how to overcome them were detailed. The development of the recommendations was, in itself, an indication of the commitment of the rare disease community as never before. Published by Elsevier Inc.

Data Quality in Rare Diseases Registries.

PubMed

Kodra, Yllka; Posada de la Paz, Manuel; Coi, Alessio; Santoro, Michele; Bianchi, Fabrizio; Ahmed, Faisal; Rubinstein, Yaffa R; Weinbach, Jérôme; Taruscio, Domenica

2017-01-01

In the field of rare diseases, registries are considered power tool to develop clinical research, to facilitate the planning of appropriate clinical trials, to improve patient care and healthcare planning. Therefore high quality data of rare diseases registries is considered to be one of the most important element in the establishment and maintenance of a registry. Data quality can be defined as the totality of features and characteristics of data set that bear on its ability to satisfy the needs that result from the intended use of the data. In the context of registries, the 'product' is data, and quality refers to data quality, meaning that the data coming into the registry have been validated, and ready for use for analysis and research. Determining the quality of data is possible through data assessment against a number of dimensions: completeness, validity; coherence and comparability; accessibility; usefulness; timeliness; prevention of duplicate records. Many others factors may influence the quality of a registry: development of standardized Case Report Form and security/safety controls of informatics infrastructure. With the growing number of rare diseases registries being established, there is a need to develop a quality validation process to evaluate the quality of each registry. A clear description of the registry is the first step when assessing data quality or the registry evaluation system. Here we report a template as a guide for helping registry owners to describe their registry.

Design of a framework for the deployment of collaborative independent rare disease-centric registries: Gaucher disease registry model.

PubMed

Bellgard, Matthew I; Napier, Kathryn R; Bittles, Alan H; Szer, Jeffrey; Fletcher, Sue; Zeps, Nikolajs; Hunter, Adam A; Goldblatt, Jack

2018-02-01

Orphan drug clinical trials often are adversely affected by a lack of high quality treatment efficacy data that can be reliably compared across large patient cohorts derived from multiple governmental and country jurisdictions. It is critical that these patient data be captured with limited corporate involvement. For some time, there have been calls to develop collaborative, non-proprietary, patient-centric registries for post-market surveillance of aspects related to orphan drug efficacy. There is an urgent need for the development and sustainable deployment of these 'independent' registries that can capture comprehensive clinical, genetic and therapeutic information on patients with rare diseases. We therefore extended an open-source registry platform, the Rare Disease Registry Framework (RDRF) to establish an Independent Rare Disease Registry (IRDR). We engaged with an established rare disease community for Gaucher disease to determine system requirements, methods of data capture, consent, and reporting. A non-proprietary IRDR model is presented that can serve as autonomous data repository, but more importantly ensures that the relevant data can be made available to appropriate stakeholders in a secure, timely and efficient manner to improve clinical decision-making and the lives of those with a rare disease. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Strengthening health data on a rare and heterogeneous disease: sarcoma incidence and histological subtypes in Germany.

PubMed

Ressing, Meike; Wardelmann, Eva; Hohenberger, Peter; Jakob, Jens; Kasper, Bernd; Emrich, Katharina; Eberle, Andrea; Blettner, Maria; Zeissig, Sylke Ruth

2018-02-12

The population-based incidence of sarcoma and its histological subtypes in Germany is unknown. Up-to-date information on a disease with an incidence comparable to other cancer entities is of high public health relevance. The aim of this study was to determine this incidence and to detect significant changes in incidence trends using data from German epidemiological cancer registries. Pooled data from the German Centre for Cancer Registry Data with a primary diagnosis occurring in 2013 were used. To date, this is the latest data on cancer incidence available for Germany. All German cancer registries with sufficient completeness were included (10 out of 11), covering a population of 70.0 million people, representing 87% of the German population. All malignant sarcomas according to the RARECARE Project and the WHO classification 2002 were considered for analysis and, above all, gastrointestinal stromal tumours (GIST) of uncertain behaviour. Sensitivity analysis was performed excluding certain histologies. The analysis included 3404 cases in men and 3442 cases in women diagnosed in 2013. The age adjusted sarcoma incidence (European standard) was 7.4 (men) and 6.6 (women) per 100,000 inhabitants. About 70% of sarcomas were soft tissue sarcomas, about 22% GIST, and about 9% bone sarcomas. The most common histological subtypes besides GIST were fibrosarcomas (14%) and liposarcomas (12%) in men and complex mixed and stromal neoplasms (22%), non-uterine leiomysarcomas (10%) and fibrosarcomas (9%) in women. Considering the trend for the years of diagnosis 2004 to 2013, there was a significant increase in incidence for GIST while the incidence of soft tissue sarcomas (only men) as well as of bone sarcoma stayed constant over time. As to soft tissue sarcoma in women, the incidence stayed constant up to the year 2009 and significantly decreased afterwards. This study is the first detailed analysis of a German-wide population-based sarcoma incidence showing results comparable to the incidence detected in the RARECARE Project.

Occupational Disease Registries-Characteristics and Experiences.

PubMed

Davoodi, Somayeh; Haghighi, Khosro Sadeghniat; Kalhori, Sharareh Rostam Niakan; Hosseini, Narges Shams; Mohammadzadeh, Zeinab; Safdari, Reza

2017-06-01

Due to growth of occupational diseases and also increase of public awareness about their consequences, attention to various aspects of diseases and improve occupational health and safety has found great importance. Therefore, there is the need for appropriate information management tools such as registries in order to recognitions of diseases patterns and then making decision about prevention, early detection and treatment of them. These registries have different characteristics in various countries according to their occupational health priorities. Aim of this study is evaluate dimensions of occupational diseases registries including objectives, data sources, responsible institutions, minimum data set, classification systems and process of registration in different countries. In this study, the papers were searched using the MEDLINE (PubMed) Google scholar, Scopus, ProQuest and Google. The search was done based on keyword in English for all motor engines including "occupational disease", "work related disease", "surveillance", "reporting", "registration system" and "registry" combined with name of the countries including all subheadings. After categorizing search findings in tables, results were compared with each other. Important aspects of the registries studied in ten countries including Finland, France, United Kingdom, Australia, Czech Republic, Malaysia, United States, Singapore, Russia and Turkey. The results show that surveyed countries have statistical, treatment and prevention objectives. Data sources in almost the rest of registries were physicians and employers. The minimum data sets in most of them consist of information about patient, disease, occupation and employer. Some of countries have special occupational related classification systems for themselves and some of them apply international classification systems such as ICD-10. Finally, the process of registration system was different in countries. Because occupational diseases are often preventable, but not curable, it is necessary to all countries, to consider prevention and early detection of occupational diseases as the objectives of their registry systems. Also it is recommended that all countries reach an agreement about global characteristics of occupational disease registries. This enables country to compare their data at international levels.

Is there a role for prophylactic colectomy in Lynch syndrome patients with inflammatory bowel disease?

PubMed

McNamara, Kate L; Aronson, Melyssa D; Cohen, Zane

2016-01-01

Lynch syndrome and chronic inflammatory bowel disease are two important risk factors for colorectal cancer. It is unclear whether Lynch syndrome patients with inflammatory bowel disease are at sufficiently increased risk for colorectal cancer to warrant prophylactic colectomy. This study aims to identify all cases of Lynch syndrome and concurrent inflammatory bowel disease in a large familial gastrointestinal cancer registry, define incidence of colorectal cancer, and characterize mismatch repair protein gene mutation status and inflammatory bowel disease-associated colorectal cancer risk factors. We retrospectively identified and collected clinical data for all cases with confirmed diagnoses of Lynch syndrome and inflammatory bowel disease in the Familial Gastrointestinal Cancer Registry at Mount Sinai Hospital in Toronto, Canada. Twelve cases of confirmed Lynch syndrome, and concurrent inflammatory bowel disease were identified. Four cases developed colorectal cancer. An additional five cases had colectomy; one was performed for severe colitis, and four were performed for low-grade dysplasia. None of these surgical specimens contained malignancy or high-grade dysplasia. The presentation of Lynch syndrome with inflammatory bowel disease is uncommon and not well described in the literature. This small but important series of twelve cases is the largest reported to date. In this series, patients with Lynch syndrome and concurrent inflammatory bowel disease do not appear to have sufficiently increased risk for colorectal cancer to recommend prophylactic surgery. Therefore, the decision to surgery should continue to be guided by surgical indications for each disease. Further evaluation of this important area will require multi-institutional input.

Review of U.S. registries for psoriasis.

PubMed

Amin, Mina; No, Daniel J; Wu, Jashin J

2017-12-01

Patient registries are databases comprised of standardized clinical data for a specific population of patients with a particular disease or medical condition. Information from patient registries allows clinicians to assess long-lasting outcomes in patients with a specific disease, such as psoriasis. Our primary objective was to identify available psoriasis registries in the United States (U.S.) and evaluate the application of patient registries compared to clinical trials. We searched Google, the Registry of Patient Registries, Orphanet and ClinicalTrials.gov to create a list of U.S. psoriasis registries. We also performed a literature review on the application of psoriasis registries using PubMed. We identified 6 psoriasis patient registries in the United States. Patient registries are frequently used for psoriasis in the U.S. and provide important information about the safety, efficacy and long-term effects of systemic therapies.

Implications for registry-based vaccine effectiveness studies from an evaluation of an immunization registry: a cross-sectional study.

PubMed

Mahon, Barbara E; Shea, Kimberly M; Dougherty, Nancy N; Loughlin, Anita M

2008-05-14

Population-based electronic immunization registries create the possibility of using registry data to conduct vaccine effectiveness studies which could have methodological advantages over traditional observational studies. For study validity, the base population would have to be clearly defined and the immunization status of members of the population accurately recorded in the registry. We evaluated a city-wide immunization registry, focusing on its potential as a tool to study pertussis vaccine effectiveness, especially in adolescents. We conducted two evaluations - one in sites that were active registry participants and one in sites that had implemented an electronic medical record with plans for future direct data transfer to the registry - of the ability to match patients' medical records to registry records and the accuracy of immunization records in the registry. For each site, records from current pediatric patients were chosen randomly. Data regarding pertussis-related immunizations, clinic usage, and demographic and identifying information were recorded; for 11-17-year-old subjects, information on MMR, hepatitis B, and varicella immunizations was also collected. Records were then matched, when possible, to registry records. For records with a registry match, immunization data were compared. Among 350 subjects from sites that were current registry users, 307 (87.7%) matched a registry record. Discrepancies in pertussis-related data were common for up-to-date status (22.6%), number of immunizations (34.7%), dates (10.2%), and formulation (34.4%). Among 442 subjects from sites that planned direct electronic transfer of immunization data to the registry, 393 (88.9%) would have matched a registry record; discrepancies occurred frequently in number of immunizations (11.9%), formulation (29.1%), manufacturer (94.4%), and lot number (95.1%.) Inability to match and immunization discrepancies were both more common in subjects who were older at their first visit to the provider site. For 11-17-year-old subjects, discrepancies were also common for MMR, hepatitis B, and varicella vaccination data. Provider records frequently could not be matched to registry records or had discrepancies in key immunization data. These issues were more common for older children and were present even with electronic data transfer. These results highlight general challenges that may face investigators wishing to use registry-based immunization data for vaccine effectiveness studies, especially in adolescents.

Implications for registry-based vaccine effectiveness studies from an evaluation of an immunization registry: A cross-sectional study

PubMed Central

Mahon, Barbara E; Shea, Kimberly M; Dougherty, Nancy N; Loughlin, Anita M

2008-01-01

Background Population-based electronic immunization registries create the possibility of using registry data to conduct vaccine effectiveness studies which could have methodological advantages over traditional observational studies. For study validity, the base population would have to be clearly defined and the immunization status of members of the population accurately recorded in the registry. We evaluated a city-wide immunization registry, focusing on its potential as a tool to study pertussis vaccine effectiveness, especially in adolescents. Methods We conducted two evaluations – one in sites that were active registry participants and one in sites that had implemented an electronic medical record with plans for future direct data transfer to the registry – of the ability to match patients' medical records to registry records and the accuracy of immunization records in the registry. For each site, records from current pediatric patients were chosen randomly. Data regarding pertussis-related immunizations, clinic usage, and demographic and identifying information were recorded; for 11–17-year-old subjects, information on MMR, hepatitis B, and varicella immunizations was also collected. Records were then matched, when possible, to registry records. For records with a registry match, immunization data were compared. Results Among 350 subjects from sites that were current registry users, 307 (87.7%) matched a registry record. Discrepancies in pertussis-related data were common for up-to-date status (22.6%), number of immunizations (34.7%), dates (10.2%), and formulation (34.4%). Among 442 subjects from sites that planned direct electronic transfer of immunization data to the registry, 393 (88.9%) would have matched a registry record; discrepancies occurred frequently in number of immunizations (11.9%), formulation (29.1%), manufacturer (94.4%), and lot number (95.1%.) Inability to match and immunization discrepancies were both more common in subjects who were older at their first visit to the provider site. For 11–17-year-old subjects, discrepancies were also common for MMR, hepatitis B, and varicella vaccination data. Conclusion Provider records frequently could not be matched to registry records or had discrepancies in key immunization data. These issues were more common for older children and were present even with electronic data transfer. These results highlight general challenges that may face investigators wishing to use registry-based immunization data for vaccine effectiveness studies, especially in adolescents. PMID:18479517

Considerations Before Establishing an Environmental Health Registry

PubMed Central

Muravov, Oleg I.; Sapp, James; Larson, Theodore C.; Pallos, L. Laszlo; Sanchez, Marchelle E.; Williamson, G. David; Horton, D. Kevin

2015-01-01

Public health registries can provide valuable information when health consequences of environmental exposures are uncertain or will likely take long to develop. They can also aid research on diseases that may have environmental causes that are not completely well defined. We discuss factors to consider when deciding whether to create an environmental health registry. Those factors include public health significance, purpose and outcomes, duration and scope of data collection and availability of alternative data sources, timeliness, availability of funding and administrative capabilities, and whether the establishment of a registry can adequately address specific health concerns. We also discuss difficulties, limitations, and benefits of exposure and disease registries, based on the experience of the Agency for Toxic Substances and Disease Registry. PMID:26066912

Genetics Home Reference: Pompe disease

MedlinePlus

... Genetic Testing (2 links) Genetic Testing Registry: Glycogen storage disease type II, infantile Genetic Testing Registry: Glycogen storage disease, type II Other Diagnosis and Management Resources ( ...

Testing Electronic Algorithms to Create Disease Registries in a Safety Net System

PubMed Central

Hanratty, Rebecca; Estacio, Raymond O.; Dickinson, L. Miriam; Chandramouli, Vijayalaxmi; Steiner, John F.; Havranek, Edward P.

2008-01-01

Electronic disease registries are a critical feature of the chronic disease management programs that are used to improve the care of individuals with chronic illnesses. These registries have been developed primarily in managed care settings; use in safety net institutions—organizations whose mission is to serve the uninsured and underserved—has not been described. We sought to assess the feasibility of developing disease registries from electronic data in a safety net institution, focusing on hypertension because of its importance in minority populations. We compared diagnoses obtained from algorithms utilizing electronic data, including laboratory and pharmacy records, against diagnoses derived from chart review. We found good concordance between diagnoses identified from electronic data and those identified by chart review, suggesting that registries of patients with chronic diseases can be developed outside the setting of closed panel managed care organizations. PMID:18469416

Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).

PubMed

Knoebl, P; Marco, P; Baudo, F; Collins, P; Huth-Kühne, A; Nemes, L; Pellegrini, F; Tengborn, L; Lévesque, H

2012-04-01

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Five hundred and one (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003 and 2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. Fifty-seven per cent of the non-pregnancy-related cases were male. Four hundred and seventy-four bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. Four hundred and seventy-seven patients underwent immunosuppression, and 72.6% achieved complete remission. Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA. © 2012 International Society on Thrombosis and Haemostasis.

Research Registries: A Tool to Advance Understanding of Rare Neuro-Ophthalmic Diseases

PubMed Central

Blankshain, Kimberly D; Moss, Heather E

2016-01-01

Background Medical research registries (MRR) are organized systems used to collect, store and analyze patient information. They are important tools for medical research with particular application to the study of rare diseases, including those seen in neuro-ophthalmic practice. Evidence Acquisition Evidence for this review was gathered from the writers’ experiences creating a comprehensive neuro-ophthalmology registry and review of the literature. Results MRR are typically observational and prospective databases of de-identified patient information. The structure is flexible and can accommodate a focus on specific diseases or treatments, surveillance of patient populations, physician quality improvement, or recruitment for future studies. They are particularly useful for the study of rare diseases. They can be integrated into the hierarchy of medical research at many levels provided their construction is well organized and they have several key characteristics including an easily manipulated database, comprehensive information on carefully selected patients and comply with human subjects regulations. MRR pertinent to neuro-ophthalmology include the UIC neuro-ophthalmology registry, Susac Syndrome Registry, Intracranial Hypertension Registry as well as larger scale patient outcome registries being developed by professional societies. Conclusion Medical research registries have a variety of forms and applications. With careful planning and clear goals, they are flexible and powerful research tools that can support multiple different study designs, and through this have the potential to advance understanding and care of neuro-ophthalmic diseases. PMID:27389624

A modular approach to disease registry design: successful adoption of an internet-based rare disease registry.

PubMed

Bellgard, Matthew I; Macgregor, Andrew; Janon, Fred; Harvey, Adam; O'Leary, Peter; Hunter, Adam; Dawkins, Hugh

2012-10-01

There is a need to develop Internet-based rare disease registries to support health care stakeholders to deliver improved quality patient outcomes. Such systems should be architected to enable multiple-level access by a range of user groups within a region or across regional/country borders in a secure and private way. However, this functionality is currently not available in many existing systems. A new approach to the design of an Internet-based architecture for disease registries has been developed for patients with clinical and genetic data in geographical disparate locations. The system addresses issues of multiple-level access by key stakeholders, security and privacy. The system has been successfully adopted for specific rare diseases in Australia and is open source. The results of this work demonstrate that it is feasible to design an open source Internet-based disease registry system in a scalable and customizable fashion and designed to facilitate interoperability with other systems. © 2012 Wiley Periodicals, Inc.

The European Cystic Fibrosis Society Patient Registry: valuable lessons learned on how to sustain a disease registry.

PubMed

Viviani, Laura; Zolin, Anna; Mehta, Anil; Olesen, Hanne Vebert

2014-06-07

Disease registries have the invaluable potential to provide an insight into the natural history of the disease under investigation, to provide useful information (e.g. through health indicators) for planning health care services and to identify suitable groups of patients for clinical trials enrolment. However, the establishment and maintenance of disease registries is a burdensome initiative from economical and organisational points of view and experience sharing on registries management is important to avoid waste of resources. The aim of this paper is to discuss the problems embedded in the institution and management of an international disease registry to warn against common mistakes that can derail the best of intentions: we share the experience of the European Cystic Fibrosis Society Patient Registry, which collects data on almost 30,000 patients from 23 countries. We discuss the major problems that researchers often encounter in the creation and management of disease registries: definition of the aims the registry has to reach, definition of the criteria for patients referral to the registry, definition of the information to record, set up of a data quality process, handling of missing data, maintenance of data confidentiality, regulation of data use and dissemination of research results. We give examples on how many crucial aspects were solved by the European Cystic Fibrosis Society Patient Registry regarding objectives, inclusion criteria and variables definition, data management, data quality controls, missing data handling, confidentiality maintenance, data use and results dissemination. We suggest an extensive literature research and discussions in working groups with different stake holders, including patient representatives, on the objectives, inclusion criteria and the information to record. We propose to pilot the recording of few variables and test the applicability of their definition first. The use of a shared electronic platform for data collection that automatically computes derived variables, and automatically performs basic data quality controls is a good data management practice, that also helps in reducing missing data. We found crucial for success the collaboration with existing national and international registries, cystic fibrosis organisations and patients' associations.

The European Cystic Fibrosis Society Patient Registry: valuable lessons learned on how to sustain a disease registry

PubMed Central

2014-01-01

Background Disease registries have the invaluable potential to provide an insight into the natural history of the disease under investigation, to provide useful information (e.g. through health indicators) for planning health care services and to identify suitable groups of patients for clinical trials enrolment. However, the establishment and maintenance of disease registries is a burdensome initiative from economical and organisational points of view and experience sharing on registries management is important to avoid waste of resources. The aim of this paper is to discuss the problems embedded in the institution and management of an international disease registry to warn against common mistakes that can derail the best of intentions: we share the experience of the European Cystic Fibrosis Society Patient Registry, which collects data on almost 30,000 patients from 23 countries. Methods We discuss the major problems that researchers often encounter in the creation and management of disease registries: definition of the aims the registry has to reach, definition of the criteria for patients referral to the registry, definition of the information to record, set up of a data quality process, handling of missing data, maintenance of data confidentiality, regulation of data use and dissemination of research results. Results We give examples on how many crucial aspects were solved by the European Cystic Fibrosis Society Patient Registry regarding objectives, inclusion criteria and variables definition, data management, data quality controls, missing data handling, confidentiality maintenance, data use and results dissemination. Conclusions We suggest an extensive literature research and discussions in working groups with different stake holders, including patient representatives, on the objectives, inclusion criteria and the information to record. We propose to pilot the recording of few variables and test the applicability of their definition first. The use of a shared electronic platform for data collection that automatically computes derived variables, and automatically performs basic data quality controls is a good data management practice, that also helps in reducing missing data. We found crucial for success the collaboration with existing national and international registries, cystic fibrosis organisations and patients’ associations. PMID:24908055

76 FR 20690 - International Consortium of Orthopedic Registries; Public Workshop

Federal Register 2010, 2011, 2012, 2013, 2014

2011-04-13

... orthopedic implant information and create a research network to advance the methodology and conduct of research related to orthopedic device performance. Date and Time: The public workshop will be held on May 9... discussion among FDA and international orthopedic registries and develop a research consortium (ICOR) that...

I RBH - First Brazilian Hypertension Registry.

PubMed

Jardim, Paulo César Brandão Veiga; Souza, Weimar Kunz Sebba Barroso de; Lopes, Renato Delascio; Brandão, Andréa Araújo; Malachias, Marcus V Bolívar; Gomes, Marco Mota; Moreno Júnior, Heitor; Barbosa, Eduardo Costa Duarte; Póvoa, Rui Manoel Dos Santos

2016-08-01

A registry assessing the care of hypertensive patients in daily clinical practice in public and private centers in various Brazilian regions has not been conducted to date. Such analysis is important to elucidate the effectiveness of this care. To document the current clinical practice for the treatment of hypertension with identification of the profile of requested tests, type of administered treatment, level of blood pressure (BP) control, and adherence to treatment. National, observational, prospective, and multicenter study that will include patients older than 18 years with hypertension for at least 4 weeks, following up in public and private centers and after signing a consent form. The study will exclude patients undergoing dialysis, hospitalized in the previous 30 days, with class III or IV heart failure, pregnant or nursing, with severe liver disease, stroke or acute myocardial infarction in the past 30 days, or with diseases with a survival prognosis < 1 year. Evaluations will be performed at baseline and after 1 year of follow-up. The parameters that will be evaluated include anthropometric data, lifestyle habits, BP levels, lipid profile, metabolic syndrome, and adherence to treatment. The primary outcomes will be hospitalization due to hypertensive crisis, cardiocirculatory events, and cardiovascular death, while secondary outcomes will be hospitalization for heart failure and requirement of dialysis. A subgroup analysis of 15% of the sample will include noninvasive central pressure evaluation at baseline and study end. The estimated sample size is 3,000 individuals for a prevalence of 5%, sample error of 2%, and 95% confidence interval. The results will be presented after the final evaluation, which will occur at the end of a 1-year follow-up. The analysis of this registry will improve the knowledge and optimize the treatment of hypertension in Brazil, as a way of modifying the prognosis of cardiovascular disease in the country.

48 CFR 22.805 - Procedures.

Code of Federal Regulations, 2010 CFR

2010-10-01

... National Preaward Registry via the Internet at http://www.dol-esa.gov/preaward/; (ii) The projected award date is within 24 months of the proposed contractor's Notice of Compliance completion date in the... the OFCCP regional office at least 30 days before the proposed award date. (7) Within 15 days of the...

Inflammatory bowel disease registries for collection of patient iron parameters in Europe

PubMed Central

Halfvarson, Jonas; Cummings, Fraser; Grip, Olof; Savoye, Guillaume

2018-01-01

Iron deficiency without anemia and iron deficiency anemia are common and frequently overlooked complications of inflammatory bowel disease. Despite the frequency and impact of iron deficiency in inflammatory bowel disease, there are gaps in our understanding about its incidence, prevalence and natural history and, consequently, patients may be undertreated. Medical registries have a key role in collecting data on the disease’s natural history, the safety and effectiveness of drugs in routine clinical practice, and the quality of care delivered by healthcare services. Even though iron deficiency impacts inflammatory bowel disease patients and healthcare systems substantially, none of the established European inflammatory bowel disease registries systematically collects information on iron parameters and related outcomes. Collection of robust iron parameter data from patient registries is one way to heighten awareness about the importance of iron deficiency in this disease and to generate data to improve the quality of patient care, patient outcomes, and thus quality of life. This objective could be achieved through collection of specific laboratory, clinical, and patient-reported measurements that could be incorporated into existing registries. This review describes the status of current European inflammatory bowel disease registries and the data they generate, in order to highlight their potential role in collecting iron data, to discuss how such information gathering could contribute to our understanding of iron deficiency anemia, and to provide practical information in regard to the incorporation of accumulated iron parameter data into registries. PMID:29563751

Reducing selection bias in case-control studies from rare disease registries.

PubMed

Cole, J Alexander; Taylor, John S; Hangartner, Thomas N; Weinreb, Neal J; Mistry, Pramod K; Khan, Aneal

2011-09-12

In clinical research of rare diseases, where small patient numbers and disease heterogeneity limit study design options, registries are a valuable resource for demographic and outcome information. However, in contrast to prospective, randomized clinical trials, the observational design of registries is prone to introduce selection bias and negatively impact the validity of data analyses. The objective of the study was to demonstrate the utility of case-control matching and the risk-set method in order to control bias in data from a rare disease registry. Data from the International Collaborative Gaucher Group (ICGG) Gaucher Registry were used as an example. A case-control matching analysis using the risk-set method was conducted to identify two groups of patients with type 1 Gaucher disease in the ICGG Gaucher Registry: patients with avascular osteonecrosis (AVN) and those without AVN. The frequency distributions of gender, decade of birth, treatment status, and splenectomy status were presented for cases and controls before and after matching. Odds ratios (and 95% confidence intervals) were calculated for each variable before and after matching. The application of case-control matching methodology results in cohorts of cases (i.e., patients with AVN) and controls (i.e., patients without AVN) who have comparable distributions for four common parameters used in subject selection: gender, year of birth (age), treatment status, and splenectomy status. Matching resulted in odds ratios of approximately 1.00, indicating no bias. We demonstrated bias in case-control selection in subjects from a prototype rare disease registry and used case-control matching to minimize this bias. Therefore, this approach appears useful to study cohorts of heterogeneous patients in rare disease registries.

Characteristics and temporal trends in patient registries: focus on the life sciences industry, 1981–2012

PubMed Central

Travers, Karin; Sallum, Rachel H; Burns, Meghan D; Barr, Charles E; Beattie, Mary S; Pashos, Chris L; Luce, Bryan R

2015-01-01

Purpose Patient registries are used to monitor safety, examine real-world effectiveness, and may potentially contribute to comparative effectiveness research. To our knowledge, life sciences industry (LSI)-sponsored registries have not been systematically categorized. This study represents a first step toward understanding such registries over time. Methods Studies described as registries were identified in the ClinicalTrials.gov database. Characteristics from these registry records were abstracted and analyzed. Results Of 1202 registries identified, approximately 47% reported LSI sponsorship. These 562 LSI registries varied in focus: medical devices (n = 193, 34%), specific drugs (n = 173, 31%), procedures (n = 29, 5%), or particular diseases (n = 139, 25%). Thirty-three registries (<6%) evaluated pregnancy outcomes. The most common therapeutic area was cardiovascular (n = 234, 42%); others included endocrinology, immunology, oncology, musculoskeletal disorders, and neurology. The two most often measured outcomes were clinical effectiveness and safety, each of which appeared in 363/562 (65%) of LSI registries. Other outcomes included real-world clinical practice patterns (n = 122, 22%), patient-reported outcomes (n = 106, 19%), disease epidemiology/natural history (n = 69, 12%), and economic outcomes (n = 30, 5%). The number of LSI registries and their geographic diversity has increased over time. Conclusions The LSI registries represent a substantial proportion of all patient registries documented in ClinicalTrials.gov. These prospective studies are growing in number and encompass diverse therapeutic areas and geographic regions. Most registries measure multiple outcomes and capture real-world data that may be unavailable through other study designs. This classification of LSI registries documents their use for studying heterogeneity of diseases, examining treatment patterns, measuring patient-reported outcomes, examining economic outcomes, and performing comparative effectiveness research. © 2014 The Authors. Pharmacoepidemiology and Drug Safety published by John Wiley & Sons, Ltd. PMID:25079108

Creating an effective clinical registry for rare diseases

PubMed Central

D’Agnolo, Hedwig MA; Kievit, Wietske; Andrade, Raul J; Karlsen, Tom Hemming; Wedemeyer, Heiner

2015-01-01

The exposure of clinicians to patients with rare gastrointestinal diseases is limited. This hurts clinical studies, which impedes accumulation of scientific knowledge on the natural disease course, treatment outcomes and prognosis in these patients. An excellent method to detect patterns on an aggregate level that would not be possible to discover in individual cases, is a registry study. This paper aims to describe a template to create a successful international registry for rare diseases. We focus mainly on rare hepatic diseases, but lessons from this paper serve other fields in medicine, as well. PMID:27403298

Validity of deterministic record linkage using multiple indirect personal identifiers: linking a large registry to claims data.

PubMed

Setoguchi, Soko; Zhu, Ying; Jalbert, Jessica J; Williams, Lauren A; Chen, Chih-Ying

2014-05-01

Linking patient registries with administrative databases can enhance the utility of the databases for epidemiological and comparative effectiveness research. However, registries often lack direct personal identifiers, and the validity of record linkage using multiple indirect personal identifiers is not well understood. Using a large contemporary national cardiovascular device registry and 100% Medicare inpatient data, we linked hospitalization-level records. The main outcomes were the validity measures of several deterministic linkage rules using multiple indirect personal identifiers compared with rules using both direct and indirect personal identifiers. Linkage rules using 2 or 3 indirect, patient-level identifiers (ie, date of birth, sex, admission date) and hospital ID produced linkages with sensitivity of 95% and specificity of 98% compared with a gold standard linkage rule using a combination of both direct and indirect identifiers. Ours is the first large-scale study to validate the performance of deterministic linkage rules without direct personal identifiers. When linking hospitalization-level records in the absence of direct personal identifiers, provider information is necessary for successful linkage. © 2014 American Heart Association, Inc.

Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data.

PubMed

Keogh, Ruth H; Szczesniak, Rhonda; Taylor-Robinson, David; Bilton, Diana

2018-03-01

Cystic fibrosis (CF) is the most common inherited disease in Caucasians, affecting around 10,000 individuals in the UK today. Prognosis has improved considerably over recent decades with ongoing improvements in treatment and care. Providing up-to-date survival predictions is important for patients, clinicians and health services planning. Flexible parametric survival modelling of UK CF Registry data from 2011 to 2015, capturing 602 deaths in 10,428 individuals. Survival curves were estimated from birth; conditional on reaching older ages; and projected under different assumptions concerning future mortality trends, using baseline characteristics of sex, CFTR genotype (zero, one, two copies of F508del) and age at diagnosis. Male sex was associated with better survival, as was older age at diagnosis, but only in F508del non-homozygotes. Survival did not differ by genotype among individuals diagnosed at birth. Median survival ages at birth in F508del homozygotes were 46years (males) and 41years (females), and similar in non-homozygotes diagnosed at birth. F508del heterozygotes diagnosed aged 5 had median survival ages of 57 (males) and 51 (females). Conditional on survival to 30, median survival age rises to 52 (males) and 49 (females) in homozygotes. Mortality rates decreased annually by 2% during 2006-2015. Future improvements at this rate suggest median survival ages for F508del homozygous babies of 65 (males) and 56 (females). Over half of babies born today, and of individuals aged 30 and above today, can expect to survive into at least their fifth decade. Evidence before this study We searched PubMed with terms "(cystic fibrosis survival) and (projection OR model OR registry OR United Kingdom OR UK)" to identify relevant studies on survival estimates for individuals with cystic fibrosis (CF). We also considered the most recent annual report from the UK Cystic Fibrosis Registry (Cystic Fibrosis Trust, 2016), a review by Buzzetti and colleagues (2009), the chapter on Epidemiology of Cystic Fibrosis by MacNeill (2016), the study of MacKenzie and colleagues (2014), and references therein. There have been many studies of factors associated with survival in CF; most have focused on identifying risk factors, and only a few have presented estimated survival curves, which are the focus of this work. The most recent study of survival in the UK is by Dodge and colleagues (2007), who used data obtained from CF clinics and the national death register, and gave an estimate of survival for babies born in 2003. We found no previous studies that have obtained detailed information on survival using UK Cystic Fibrosis Registry data. Jackson and colleagues obtained survival estimates for the US and Ireland using registry data (Jackson et al., 2011). MacKenzie and colleagues used US Cystic Fibrosis Foundation Patient Registry data from 2000 to 2010 to project survival for children born and diagnosed with CF in 2010, accounting for sex, genotype and age at diagnosis (MacKenzie et al., 2014). Previous studies on estimated survival in CF have become out of date or have not accounted for the full range of patient characteristics available at birth. Few have presented conditional survival estimates (Dodge et al., 2007). Added value of this study This is the first study to yield detailed survival statistics using the UK Cystic Fibrosis Registry, which is one of the largest national CF registries outside of the US and has almost complete coverage of the UK CF population. The primary goal was to leverage the long-term follow-up of the nearly complete UK CF population available in the Registry for the purposes of producing accurate, precise predictions in the modern era of CF care. Estimates are presented from birth and conditional on survival to older ages. These are the first conditional estimates in CF to also account for genotype, sex and age at diagnosis, which were each included in the modelling using a flexible approach. Projections are also provided under different scenarios based on downward trends in mortality rates. Our use of flexible parametric survival models is novel in this field, and our approach could be used to provide modern survival statistics for other chronic diseases and disorders. Implications of all the available evidence Our estimates of future survival in CF under a range of different scenarios are based on data on nearly all individuals living with the disease in the UK in recent times, reflective of a modern era of care, and are most appropriate for the families of babies being born in the present day with CF. Conditional estimates inform patients who have already reached an older age, and their clinicians. Over half of babies born today, and of individuals aged 30years and above alive today, can expect to survive into their fifth decade. Insights based on our survival projections can be used to inform future needs in CF health care provision. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

Myositis registries and biorepositories: powerful tools to advance clinical, epidemiologic and pathogenic research

PubMed Central

Rider, Lisa G.; Dankó, Katalin; Miller, Frederick W.

2016-01-01

Purpose of review Clinical registries and biorepositories have proven extremely useful in many studies of diseases, especially rare diseases. Given their rarity and diversity, the idiopathic inflammatory myopathies, or myositis syndromes, have benefited from individual researchers’ collections of cohorts of patients. Major efforts are being made to establish large registries and biorepositories that will allow many additional studies to be performed that were not possible before. Here we describe the registries developed by investigators and patient support groups that are currently available for collaborative research purposes. Recent findings We have identified 46 myositis research registries, including many with biorepositories, which have been developed for a wide variety of purposes and have resulted in great advances in understanding the range of phenotypes, clinical presentations, risk factors, pathogenic mechanisms, outcome assessment, therapeutic responses, and prognoses. These are now available for collaborative use to undertake additional studies. Two myositis patient registries have been developed for research, and myositis patient support groups maintain demographic registries with large numbers of patients available to be contacted for potential research participation. Summary Investigator-initiated myositis research registries and biorepositories have proven extremely useful in understanding many aspects of these rare and diverse autoimmune diseases. These registries and biorepositories, in addition to those developed by myositis patient support groups, deserve continued support to maintain the momentum in this field as they offer major opportunities to improve understanding of the pathogenesis and treatment of these diseases in cost-effective ways. PMID:25225838

Myositis registries and biorepositories: powerful tools to advance clinical, epidemiologic and pathogenic research.

PubMed

Rider, Lisa G; Dankó, Katalin; Miller, Frederick W

2014-11-01

Clinical registries and biorepositories have proven extremely useful in many studies of diseases, especially rare diseases. Given their rarity and diversity, the idiopathic inflammatory myopathies, or myositis syndromes, have benefited from individual researchers' collections of cohorts of patients. Major efforts are being made to establish large registries and biorepositories that will allow many additional studies to be performed that were not possible before. Here, we describe the registries developed by investigators and patient support groups that are currently available for collaborative research purposes. We have identified 46 myositis research registries, including many with biorepositories, which have been developed for a wide variety of purposes and have resulted in great advances in understanding the range of phenotypes, clinical presentations, risk factors, pathogenic mechanisms, outcome assessment, therapeutic responses, and prognoses. These are now available for collaborative use to undertake additional studies. Two myositis patient registries have been developed for research, and myositis patient support groups maintain demographic registries with large numbers of patients available to be contacted for potential research participation. Investigator-initiated myositis research registries and biorepositories have proven extremely useful in understanding many aspects of these rare and diverse autoimmune diseases. These registries and biorepositories, in addition to those developed by myositis patient support groups, deserve continued support to maintain the momentum in this field as they offer major opportunities to improve understanding of the pathogenesis and treatment of these diseases in cost-effective ways.

Clinical disease registries in acute myocardial infarction.

PubMed

Ashrafi, Reza; Hussain, Hussain; Brisk, Robert; Boardman, Leanne; Weston, Clive

2014-06-26

Disease registries, containing systematic records of cases, have for nearly 100 years been valuable in exploring and understanding various aspects of cardiology. This is particularly true for myocardial infarction, where such registries have provided both epidemiological and clinical information that was not readily available from randomised controlled trials in highly-selected populations. Registries, whether mandated or voluntary, prospective or retrospective in their analysis, have at their core a common study population and common data definitions. In this review we highlight how registries have diversified to offer information on epidemiology, risk modelling, quality assurance/improvement and original research-through data mining, transnational comparisons and the facilitation of enrolment in, and follow-up during registry-based randomised clinical trials.

Developing and testing a cost data collection instrument for noncommunicable disease registry planning.

PubMed

Subramanian, Sujha; Tangka, Florence; Edwards, Patrick; Hoover, Sonja; Cole-Beebe, Maggie

2016-12-01

This article reports on the methods and framework we have developed to guide economic evaluation of noncommunicable disease registries. We developed a cost data collection instrument, the Centers for Disease Control and Prevention's (CDC's) International Registry Costing Tool (IntRegCosting Tool), based on established economics methods We performed in-depth case studies, site visit interviews, and pilot testing in 11 registries from multiple countries including India, Kenya, Uganda, Colombia, and Barbados to assess the overall quality of the data collected from cancer and cardiovascular registries. Overall, the registries were able to use the IntRegCosting Tool to assign operating expenditures to specific activities. We verified that registries were able to provide accurate estimation of labor costs, which is the largest expenditure incurred by registries. We also identified several factors that can influence the cost of registry operations, including size of the geographic area served, data collection approach, local cost of living, presence of rural areas, volume of cases, extent of consolidation of records to cases, and continuity of funding. Internal and external registry factors reveal that a single estimate for the cost of registry operations is not feasible; costs will vary on the basis of factors that may be beyond the control of the registries. Some factors, such as data collection approach, can be modified to improve the efficiency of registry operations. These findings will inform both future economic data collection using a web-based tool and cost and cost-effectiveness analyses of registry operations in low- and middle-income countries (LMICs) and other locations with similar characteristics. Copyright © 2016 Elsevier Ltd. All rights reserved.

Cost of Operating Central Cancer Registries and Factors That Affect Cost: Findings From an Economic Evaluation of Centers for Disease Control and Prevention National Program of Cancer Registries

PubMed Central

Tangka, Florence K. L.; Subramanian, Sujha; Beebe, Maggie Cole; Weir, Hannah K.; Trebino, Diana; Babcock, Frances; Ewing, Jean

2016-01-01

Context The Centers for Disease Control and Prevention evaluated the economics of the National Program of Cancer Registries to provide the Centers for Disease Control and Prevention, the registries, and policy makers with the economic evidence-base to make optimal decisions about resource allocation. Cancer registry budgets are under increasing threat, and, therefore, systematic assessment of the cost will identify approaches to improve the efficiencies of this vital data collection operation and also justify the funding required to sustain registry operations. Objectives To estimate the cost of cancer registry operations and to assess the factors affecting the cost per case reported by National Program of Cancer Registries–funded central cancer registries. Methods We developed a Web-based cost assessment tool to collect 3 years of data (2009-2011) from each National Program of Cancer Registries–funded registry for all actual expenditures for registry activities (including those funded by other sources) and factors affecting registry operations. We used a random-effects regression model to estimate the impact of various factors on cost per cancer case reported. Results The cost of reporting a cancer case varied across the registries. Central cancer registries that receive high-quality data from reporting sources (as measured by the percentage of records passing automatic edits) and electronic data submissions, and those that collect and report on a large volume of cases had significantly lower cost per case. The volume of cases reported had a large effect, with low-volume registries experiencing much higher cost per case than medium- or high-volume registries. Conclusions Our results suggest that registries operate with substantial fixed or semivariable costs. Therefore, sharing fixed costs among low-volume contiguous state registries, whenever possible, and centralization of certain processes can result in economies of scale. Approaches to improve quality of data submitted and increasing electronic reporting can also reduce cost. PMID:26642226

Research Registries: A Tool to Advance Understanding of Rare Neuro-Ophthalmic Diseases.

PubMed

Blankshain, Kimberly D; Moss, Heather E

2016-09-01

Medical research registries (MRR) are organized systems used to collect, store, and analyze patient information. They are important tools for medical research with particular application to the study of rare diseases, including those seen in neuro-ophthalmic practice. Evidence for this review was gathered from the writers' experiences creating a comprehensive neuro-ophthalmology registry and review of the literature. MRR are typically observational and prospective databases of de-identified patient information. The structure is flexible and can accommodate a focus on specific diseases or treatments, surveillance of patient populations, physician quality improvement, or recruitment for future studies. They are particularly useful for the study of rare diseases. They can be integrated into the hierarchy of medical research at many levels provided their construction is well organized and they have several key characteristics including an easily manipulated database, comprehensive information on carefully selected patients, and comply with human subjects regulations. MRR pertinent to neuro-ophthalmology include the University of Illinois at Chicago neuro-ophthalmology registry, Susac Syndrome Registry, Intracranial Hypertension Registry, and larger-scale patient outcome registries being developed by professional societies. MRR have a variety of forms and applications. With careful planning and clear goals, they are flexible and powerful research tools that can support multiple different study designs, and this can provide the potential to advance understanding and care of neuro-ophthalmic diseases.

The development of the NZ-based international upper limb surgery registry.

PubMed

Sinnott, K A; Dunn, J A; Rothwell, A G; Hall, A S; Post, M W M

2014-08-01

Implementation study. To describe the development and potential value of the New Zealand (NZ) upper limb surgery registry and report the demographic and spinal cord injury characteristics of individuals with tetraplegia collated to date. Multi Center-coordinated from Burwood Spinal Unit, NZ. Following discussions with eight international units, clinical information and outcomes measures were agreed upon for use in this specific population. To implement this consensus, a web-based upper limb surgery registry was developed in NZ. Inclusion criteria included referral to a hand clinic for clinical assessment for suitability for tendon transfer surgery. Clinical data were collected regardless of acceptance of surgery thereby creating a self-selected control group. Twenty-eight years of retrospective NZ data was entered into the registry, as well as 3 years of prospective data collected in NZ. From 1982 to 2013, a total of 357 persons with tetraplegia were assessed as suitable for surgery. Of those, 223 individuals underwent surgery and 134 declined the intervention(s). The prospective group currently comprises 55 assessments with 23 surgery individuals and 32 who have declined surgery to date. Clinical information is now available within a web-based registry for all individuals reviewed in hand clinics from when upper limb surgery was first introduced. A broad range of outcomes of interest can easily be reported directly from the registry. The self-selected control group will allow comparative studies to be explicitly linked to the specific interventions of interest.

Patient care outcomes of a tobacco use registry in an academic family practice.

PubMed

Ripley-Moffitt, Carol; Neutze, Dana; Gwynne, Mark; Goldstein, Adam O

2015-01-01

While the potential benefit of a chronic disease registry for tobacco use is great, outcome reports have not been generated. We examined the effect of implementing a tobacco use registry, including a decision support tool, on treatment outcomes within an academic family medicine clinic. A chart review of 200 patients who smoked and attended the clinic before and after registry implementation assessed the number of patients with clinic notes documenting (1) counseling for tobacco use, (2) recommendations for cessation medication, (3) a set quit date, (4) referrals to the on-site Nicotine Dependence Program (NDP) and/or QuitlineNC, and (5) pneumococcal vaccine. Data from the NDP, QuitlineNC, and clinic billing records before and after implementation compared the number of clinic-generated QuitlineNC fax referrals, new scheduled appointments for the NDP, and visits coded for tobacco counseling reimbursement. Significant increases in documentation occurred across most chart review variables. Significant increases in the number of clinic-generated fax referrals to QuitlineNC (from 27 to 96), initial scheduled appointments for the NDP (from 84 to 148), and coding for tobacco counseling (from 101 to 287) also occurred when compared with total patient visits during the same time periods. Patient attendance at the NDP (52%) and acceptance of QuitlineNC services (31%) remained constant. The tobacco use registry's decision support tool increased evidenced-based tobacco use treatment (referrals, medications, and counseling) for patients at an academic family medicine clinic. This novel tool offers standardized care for all patients who use tobacco, ensuring improved access to effective tobacco use counseling and medication treatments. © Copyright 2015 by the American Board of Family Medicine.

Cohort profile: the Nordic Antireflux Surgery Cohort (NordASCo)

PubMed Central

Wahlin, Karl; Artama, Miia; Brusselaers, Nele; Färkkilä, Martti; Lynge, Elsebeth; Mattsson, Fredrik; Pukkala, Eero; Romundstad, Pål; Tryggvadóttir, Laufey; von Euler-Chelpin, My; Lagergren, Jesper

2017-01-01

Purpose To describe a newly created all-Nordic cohort of patients with gastro-oesophageal reflux disease (GORD), entitled the Nordic Antireflux Surgery Cohort (NordASCo), which will be used to compare participants having undergone antireflux surgery with those who have not regarding risk of cancers, other diseases and mortality. Participants Included were individuals with a GORD diagnosis recorded in any of the nationwide patient registries in the Nordic countries (Denmark, Finland, Iceland, Norway and Sweden) in 1964–2014 (with various start and end years in different countries). Data regarding cancer, other diseases and mortality were retrieved from the nationwide registries for cancer, patients and causes of death, respectively. Findings to date The NordASCo includes 945 153 individuals with a diagnosis of GORD. Of these, 48 433 (5.1%) have undergone primary antireflux surgery. Median age at primary antireflux surgery ranged from 47 to 52 years in the different countries. The coding practices of GORD seem to have differed between the Nordic countries. Future plans The NordASCo will initially be used to analyse the risk of developing known or potential GORD-related cancers, that is, tumours of the oesophagus, stomach, larynx, pharynx and lung, and to evaluate the mortality in the short-term and long-term perspectives. Additionally, the cohort will be used to evaluate the risk of non-malignant respiratory conditions that might be caused by aspiration of gastric contents. PMID:28600380

Novel Surgical Treatments for Gastroesophageal Reflux Disease: Systematic Review of Magnetic Sphincter Augmentation and Electric Stimulation Therapy

PubMed Central

Stanak, Michal; Erdos, Judit; Hawlik, Katharina; Birsan, Tudor

2018-01-01

Electric stimulation therapy (EST) and magnetic sphincter augmentation (MSA) represent novel methods for the surgical treatment of gastroesophageal reflux disease (GERD). The aim of this review was to assess the effectiveness and safety of EST and magnetic sphincter augmentation device (MSAD) comapred to laparoscopic fundoplication (LF) and proton pump inhibitor therapy (in case of EST). We performed a systematic literature search without restrictions on publication dates in five electronic databases (MEDLINE, EMBASE, the Cochrane library, PubMed, and Centre for Reviews and Dissemination), complemented by hand search, search in trial registries, and documentation provided by the manufacturers. No study passed inclusion criteria for analyzing EST effectiveness. Concerning safety, lead erosion through the esophagus and trocar perforation of the small bowel occurred in 2.4% of patients (in one study). Only the registry study fulfilled inclusion criteria for effectiveness analysis of MSAD. The crucial outcome of GERD-health-related quality of life (HRQL) score improved from 20 to 3 points in MSAD patients, and from 23 to 3.5 points in LF patients. However, the LF patients were in a more severe stage of the disease. The results yield indefinite conclusions about the use of both MSAD and EST. Clinical effectiveness and safety of both MSAD and EST are not sufficiently proven and are yet to be supported by high quality evidence from randomized controlled trials.

Rare Disease Roadmap: Navigating the challenges and barriers to deliver improved outcomes for patients living with a Rare Disease

USDA-ARS?s Scientific Manuscript database

Rare Disease registries have now been recognized as a global priority for progress in monitoring, documenting natural course, preventing and treating rare diseases. However, a disease registry is only one element of Rare Disease translational research. Here, we outline what we believe are ten key co...

My Retina Tracker™: An On-line International Registry for People Affected with Inherited Orphan Retinal Degenerative Diseases and their Genetic Relatives - A New Resource.

PubMed

Fisher, Joan K; Bromley, Russell L; Mansfield, Brian C

2016-01-01

My Retina Tracker™ is a new on-line registry for people affected with inherited orphan retinal degenerative diseases, and their unaffected, genetic relatives. Created and supported by the Foundation Fighting Blindness, it is an international resource designed to capture the disease from the perspective of the registry participant and their retinal health care providers. The registry operates under an Institutional Review Board (IRB)-approved protocol and allows sharing of de-identified data with participants, researchers and clinicians. All participants sign an informed consent that includes selecting which data they wish to share. There is no minimum age of participation. Guardians must sign on behalf of minors, and children between the ages of 12 to 17 also sign an informed assent. Participants may compare their disease to others in the registry using graphical interpretations of the aggregate registry data. Researchers and clinicians have two levels of access. The first provides an interface to interrogate all data fields registrants have agreed to share based on their answers in the IRB informed consent. The second provides a route to contact people in the registry who may be eligible for studies or trials, through the Foundation.

Support for immunization registries among parents of vaccinated and unvaccinated school-aged children: a case control study.

PubMed

Linkins, Robert W; Salmon, Daniel A; Omer, Saad B; Pan, William Ky; Stokley, Shannon; Halsey, Neal A

2006-09-22

Immunizations have reduced childhood vaccine preventable disease incidence by 98-100%. Continued vaccine preventable disease control depends on high immunization coverage. Immunization registries help ensure high coverage by recording childhood immunizations administered, generating reminders when immunizations are due, calculating immunization coverage and identifying pockets needing immunization services, and improving vaccine safety by reducing over-immunization and providing data for post-licensure vaccine safety studies. Despite substantial resources directed towards registry development in the U.S., only 48% of children were enrolled in a registry in 2004. Parental attitudes likely impact child participation. Consequently, the purpose of this study was to assess the attitudes of parents of vaccinated and unvaccinated school-aged children regarding: support for immunization registries; laws authorizing registries and mandating provider reporting; opt-in versus opt-out registry participation; and financial worth and responsibility of registry development and implementation. A case control study of parents of 815 children exempt from school vaccination requirements and 1630 fully vaccinated children was conducted. Children were recruited from 112 elementary schools in Colorado, Massachusetts, Missouri, and Washington. Surveys administered to the parents, asked about views on registries and perceived utility and safety of vaccines. Parental views were summarized and logistic regression models compared differences between parents of exempt and vaccinated children. Surveys were completed by 56.1% of respondents. Fewer than 10% of parents were aware of immunization registries in their communities. Among parents aware of registries, exempt children were more likely to be enrolled (65.0%) than vaccinated children (26.5%) (p value = 0.01). A substantial proportion of parents of exempt children support immunization registries, particularly if registries offer choice for participation. Few parents of vaccinated (6.8%) and exempt children (6.7%) were aware of laws authorizing immunization registries. Support for laws authorizing registries and requiring health care providers to report to registries was more common among parents of vaccinated than exempt children. Most parents believed that the government, vaccine companies or insurance companies should pay for registries. Parental support for registries was relatively high. Parental support for immunization registries may increase with greater parental awareness of the risks of vaccine preventable diseases and utility of vaccination.

[Huntington's Disease in Balearic Islands Population-Based Registry of Rare Diseases: Prevalence and Mortality during the Period 2010-2013. Spain].

PubMed

Cáffaro Rovira, Mercedes; Salom Castell, M Magdalena

2017-02-16

Huntington's disease is a hereditary disease with low prevalence. The low frequency of Huntington's disease leads to its inclusion as one of the pathologies in the Registry of Rare Diseases. The Balearic Islands Population-based Registry of Rare Diseases began in 2010. Previously, there had been no prevalence or mortality data for Huntington's disease in the Balearic Islands. The aim of this study was to determine the prevalence and mortality of Huntington's disease in the Balearic Islands between 2010 and 2013. The data sources were the Balearic Islands Population-based Registry of Rare Diseases, from which the diagnosed cases were obtained; the Balearic Islands Mortality Register, from which the deceased cases were obtained; the Balearic Islands Health Service, from which the number of Health Cards was obtained; and the National Institute for Statistics, from which population data were obtained. Prevalence and mortality rates were calculated. The Balearic Islands Population-based Registry of Rare Diseases registered 27 cases of Huntington's disease between 2010-2013. 63% of these were women. The period prevalence rate was 2.6 per 100,000 and the period mortality rate was 1.1 per 100,000. Menorca was the island with the highest rates, the prevalence rate was 5,9 per 100,000 and the mortality rate was 2,1 per 100,000. Prevalence and mortality of Huntington's disease in the Balearic Islands are low compared to similar areas.

Strengthening Renal Registries and ESRD Research in Africa.

PubMed

Davids, M Razeen; Caskey, Fergus J; Young, Taryn; Balbir Singh, Gillian K

2017-05-01

In Africa, the combination of noncommunicable diseases, infectious diseases, exposure to environmental toxins, and acute kidney injury related to trauma and childbirth are driving an epidemic of chronic kidney disease and end-stage renal disease (ESRD). Good registry data can inform the planning of renal services and can be used to argue for better resource allocation, audit the delivery and quality of care, and monitor the impact of interventions. Few African countries have established renal registries and most have failed owing to resource constraints. In this article we briefly review the burden of chronic kidney disease and ESRD in Africa, and then consider the research questions that could be addressed by renal registries. We describe examples of the impact of registry data and summarize the sparse primary literature on country-wide renal replacement therapy in African countries over the past 20 years. Finally, we highlight some initiatives and opportunities for strengthening research on ESRD and renal replacement therapy in Africa. These include the establishment of the African Renal Registry and the availability of new areas for research. We also discuss capacity building, collaboration, open-access publication, and the strengthening of local journals, all measures that may improve the quantity, visibility, and impact of African research outputs. Copyright © 2017 Elsevier Inc. All rights reserved.

United States Transuranium and Uranium Registries

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kathren, R.L.; Filipy, R.E.; Dietert, S.E.

1991-06-01

This report summarizes the primary scientific activities of the United States Transuranium and Uranium Registries for the period October 1, 1989 through September 30, 1990. The Registries are parallel human tissue research programs devoted to the study of the actinide elements in humans. To date there have been 261 autopsy or surgical specimen donations, which include 11 whole bodies. The emphasis of the Registry was directed towards quality improvement and the development of a fully computerized data base that would incorporate not only the results of postmortem radiochemical analysis, but also medical and monitoring information obtained during life. Human subjectsmore » reviews were also completed. A three compartment biokinetic model for plutonium distribution is proposed. 2 tabs.« less

The Genotype and Phenotype (GaP) registry: a living biobank for the analysis of quantitative traits.

PubMed

Gregersen, Peter K; Klein, Gila; Keogh, Mary; Kern, Marlena; DeFranco, Margaret; Simpfendorfer, Kim R; Kim, Sun Jung; Diamond, Betty

2015-12-01

We describe the development of the Genotype and Phenotype (GaP) Registry, a living biobank of normal volunteers who are genotyped for genetic markers related to human disease. Participants in the GaP can be recalled for hypothesis driven study of disease associated genetic variants. The GaP has facilitated functional studies of several autoimmune disease associated loci including Csk, Blk, PDRM1 (Blimp-1) and PTPN22. It is likely that expansion of such living biobank registries will play an important role in studying and understanding the function of disease associated alleles in complex disease.

Rare disease registries: a call to action.

PubMed

Lacaze, Paul; Millis, Nicole; Fookes, Megan; Zurynski, Yvonne; Jaffe, Adam; Bellgard, Matthew; Winship, Ingrid; McNeil, John; Bittles, Alan H

2017-09-01

When registries collect accurate clinical data over time, they can act as fundamental support structures for patients and their families and powerful cost-effective instruments to support clinical trials and translational research to improve quality of care, quality of life and survival. Registries are critical for rare diseases (RD) with low prevalence and propensity for variation in treatment and outcomes. Rare Voices Australia is leading a call for action to the research and clinical community to prioritise RD data collection and develop an integrated RD Registry strategy for Australia. Financial, operational and governance challenges exist for establishing and maintaining RD registries. As a multidisciplinary team whose interests converge on RD, we highlight the need for the establishment of an Australian RD Registry Alliance. This 'umbrella' organisation will: (i) bring together existing RD registries across Australia; (ii) establish National RD Registry Standards to support interoperability and cohesion across registries; (iii) develop strategies to attract sustainable funding from government and other sources to maximise the utility of existing RD registries and support the development of new RD registries. The most important role for the Alliance would be to use the RD registries for translational research to address current knowledge gaps about RD and to improve the care for the over 1.4 million Australians estimated to live with RD. © 2017 Royal Australasian College of Physicians.

Analysis and visualization of disease courses in a semantically-enabled cancer registry.

PubMed

Esteban-Gil, Angel; Fernández-Breis, Jesualdo Tomás; Boeker, Martin

2017-09-29

Regional and epidemiological cancer registries are important for cancer research and the quality management of cancer treatment. Many technological solutions are available to collect and analyse data for cancer registries nowadays. However, the lack of a well-defined common semantic model is a problem when user-defined analyses and data linking to external resources are required. The objectives of this study are: (1) design of a semantic model for local cancer registries; (2) development of a semantically-enabled cancer registry based on this model; and (3) semantic exploitation of the cancer registry for analysing and visualising disease courses. Our proposal is based on our previous results and experience working with semantic technologies. Data stored in a cancer registry database were transformed into RDF employing a process driven by OWL ontologies. The semantic representation of the data was then processed to extract semantic patient profiles, which were exploited by means of SPARQL queries to identify groups of similar patients and to analyse the disease timelines of patients. Based on the requirements analysis, we have produced a draft of an ontology that models the semantics of a local cancer registry in a pragmatic extensible way. We have implemented a Semantic Web platform that allows transforming and storing data from cancer registries in RDF. This platform also permits users to formulate incremental user-defined queries through a graphical user interface. The query results can be displayed in several customisable ways. The complex disease timelines of individual patients can be clearly represented. Different events, e.g. different therapies and disease courses, are presented according to their temporal and causal relations. The presented platform is an example of the parallel development of ontologies and applications that take advantage of semantic web technologies in the medical field. The semantic structure of the representation renders it easy to analyse key figures of the patients and their evolution at different granularity levels.

Sex bias in diagnostic delay in bronchiectasis: An analysis of the Spanish Historical Registry of Bronchiectasis

PubMed Central

de Gracia Roldán, Javier; Olveira, Casilda; Vendrell, Montserrat; Martínez-García, Miguel Ángel; de la Rosa, David; Máiz, Luis; Ancochea, Julio; Vázquez, Liliana; Borderías, Luis; Polverino, Eva; Martínez-Moragón, Eva; Rajas, Olga; Soriano, Joan B

2017-01-01

Diagnostic delay is common in most respiratory diseases, particularly in bronchiectasis. However, sex bias in diagnostic delay has not been studied to date. Objective: Assessment of diagnostic delay in bronchiectasis by sex. Methods: The Spanish Historical Registry of Bronchiectasis recruited adults diagnosed with bronchiectasis from 2002 to 2011 in 36 centres in Spain. From a total of 2113 patients registered we studied 2099, of whom 1125 (53.6%) were women. Results: No differences were found for sex or age (61.0 ± 20.6, p = 0.88) or for localization of bronchiectasis (p = 0.31). Bronchiectasis of unknown aetiology and secondary to asthma, childhood infections and tuberculosis was more common in women (all ps < 0.05). More men than women were chronic obstructive pulmonary disease-related bronchiectasis and colonized by Haemophilus influenzae (p < 0.001 for both). Onset of symptoms was earlier in women. The diagnostic delay for women with bronchiectasis was 2.1 years more than for men (p = 0.001). Discussion: We recorded a substantial delay in the diagnosis of bronchiectasis. This delay was significantly longer in women than in men (>2 years). Independent factors associated with this sex bias were age at onset of symptoms, smoking history, daily expectoration and reduced lung function. PMID:28393532

The CNDR: collaborating to translate new therapies for Canadians.

PubMed

Korngut, Lawrence; Campbell, Craig; Johnston, Megan; Benstead, Timothy; Genge, Angela; Mackenzie, Alex; McCormick, Anna; Biggar, Douglas; Bourque, Pierre; Briemberg, Hannah; O'Connell, Colleen; Dojeiji, Suzan; Dooley, Joseph; Grant, Ian; Hogan, Gillian; Johnston, Wendy; Kalra, Sanjay; Katzberg, Hans D; Mah, Jean K; McAdam, Laura; McMillan, Hugh J; Melanson, Michel; Selby, Kathryn; Shoesmith, Christen; Smith, Garth; Venance, Shannon L; Wee, Joy

2013-09-01

Patient registries represent an important method of organizing "real world" patient information for clinical and research purposes. Registries can facilitate clinical trial planning and recruitment and are particularly useful in this regard for uncommon and rare diseases. Neuromuscular diseases (NMDs) are individually rare but in aggregate have a significant prevalence. In Canada, information on NMDs is lacking. Barriers to performing Canadian multicentre NMD research exist which can be overcome by a comprehensive and collaborative NMD registry. We describe the objectives, design, feasibility and initial recruitment results for the Canadian Neuromuscular Disease Registry (CNDR). The CNDR is a clinic-based registry which launched nationally in June 2011, incorporates paediatric and adult neuromuscular clinics in British Columbia, Alberta, Ontario, Quebec, New Brunswick and Nova Scotia and, as of December 2012, has recruited 1161 patients from 12 provinces and territories. Complete medical datasets have been captured on 460 "index disease" patients. Another 618 "non-index" patients have been recruited with capture of physician-confirmed diagnosis and contact information. We have demonstrated the feasibility of blended clinic and central office-based recruitment. "Index disease" patients recruited at the time of writing include 253 with Duchenne and Becker muscular dystrophy, 161 with myotonic dystrophy, and 71 with ALS. The CNDR is a new nationwide registry of patients with NMDs that represents an important advance in Canadian neuromuscular disease research capacity. It provides an innovative platform for organizing patient information to facilitate clinical research and to expedite translation of recent laboratory findings into human studies.

TOSCA - first international registry to address knowledge gaps in the natural history and management of tuberous sclerosis complex.

PubMed

Kingswood, John C; Bruzzi, Paolo; Curatolo, Paolo; de Vries, Petrus J; Fladrowski, Carla; Hertzberg, Christoph; Jansen, Anna C; Jozwiak, Sergiusz; Nabbout, Rima; Sauter, Matthias; Touraine, Renaud; O'Callaghan, Finbar; Zonnenberg, Bernard; Crippa, Stefania; Comis, Silvia; d'Augères, Guillaume Beaure; Belousova, Elena; Carter, Tom; Cottin, Vincent; Dahlin, Maria; Ferreira, José Carlos; Macaya, Alfons; Benedik, Mirjana Perkovic; Sander, Valentin; Youroukos, Sotirios; Castellana, Ramon; Ulker, Bulent; Feucht, Martha

2014-11-26

Tuberous sclerosis complex (TSC) is a rare, multisystem, genetic disorder with an estimated prevalence between 1/6800 and 1/15000. Although recent years have seen huge progress in understanding the pathophysiology and in the management of TSC, several questions remain unanswered. A disease registry could be an effective tool to gain more insights into TSC and thus help in the development of improved management strategies. TuberOus SClerosis registry to increase disease Awareness (TOSCA) is a multicentre, international disease registry to assess manifestations, interventions, and outcomes in patients with TSC. Patients of any age diagnosed with TSC, having a documented visit for TSC within the preceding 12 months, or newly diagnosed individuals are eligible. Objectives include mapping the course of TSC manifestations and their effects on prognosis, identifying patients with rare symptoms and co-morbidities, recording interventions and their outcomes, contributing to creation of an evidence-base for disease assessment and therapy, informing further research on TSC, and evaluating the quality of life of patients with TSC. The registry includes a 'core' section and subsections or 'petals'. The 'core' section is designed to record general information on patients' background collected at baseline and updated annually. Subsections will be developed over time to record additional data related to specific disease manifestations and will be updated annually. The registry aimed to enrol approximately 2000 patients from about 250 sites in 31 countries. The initial enrolment period was of 24 months. A follow-up observation period of up to 5 years is planned. A pre-planned administrative analysis of 'core' data from the first 100 patients was performed to evaluate the feasibility of the registry. Results showed a high degree of accuracy of the data collection procedure. Annual interim analyses are scheduled. Results of first interim analysis will be presented subsequent to data availability in 2014. The results of TOSCA will assist in filling the gaps in understanding the natural history of TSC and help in planning better management and surveillance strategies. This large-scale international registry to study TSC could serve as a model to encourage planning of similar registries for other rare diseases.

Support for immunization registries among parents of vaccinated and unvaccinated school-aged children: a case control study

PubMed Central

Linkins, Robert W; Salmon, Daniel A; Omer, Saad B; Pan, William KY; Stokley, Shannon; Halsey, Neal A

2006-01-01

Background Immunizations have reduced childhood vaccine preventable disease incidence by 98–100%. Continued vaccine preventable disease control depends on high immunization coverage. Immunization registries help ensure high coverage by recording childhood immunizations administered, generating reminders when immunizations are due, calculating immunization coverage and identifying pockets needing immunization services, and improving vaccine safety by reducing over-immunization and providing data for post-licensure vaccine safety studies. Despite substantial resources directed towards registry development in the U.S., only 48% of children were enrolled in a registry in 2004. Parental attitudes likely impact child participation. Consequently, the purpose of this study was to assess the attitudes of parents of vaccinated and unvaccinated school-aged children regarding: support for immunization registries; laws authorizing registries and mandating provider reporting; opt-in versus opt-out registry participation; and financial worth and responsibility of registry development and implementation. Methods A case control study of parents of 815 children exempt from school vaccination requirements and 1630 fully vaccinated children was conducted. Children were recruited from 112 elementary schools in Colorado, Massachusetts, Missouri, and Washington. Surveys administered to the parents, asked about views on registries and perceived utility and safety of vaccines. Parental views were summarized and logistic regression models compared differences between parents of exempt and vaccinated children. Results Surveys were completed by 56.1% of respondents. Fewer than 10% of parents were aware of immunization registries in their communities. Among parents aware of registries, exempt children were more likely to be enrolled (65.0%) than vaccinated children (26.5%) (p value = 0.01). A substantial proportion of parents of exempt children support immunization registries, particularly if registries offer choice for participation. Few parents of vaccinated (6.8%) and exempt children (6.7%) were aware of laws authorizing immunization registries. Support for laws authorizing registries and requiring health care providers to report to registries was more common among parents of vaccinated than exempt children. Most parents believed that the government, vaccine companies or insurance companies should pay for registries. Conclusion Parental support for registries was relatively high. Parental support for immunization registries may increase with greater parental awareness of the risks of vaccine preventable diseases and utility of vaccination. PMID:16995946

Evaluating the completeness of the national ALS registry, United States.

PubMed

Kaye, Wendy E; Wagner, Laurie; Wu, Ruoming; Mehta, Paul

2018-02-01

Our objective was to evaluate the completeness of the United States National ALS Registry (Registry). We compared persons with ALS who were passively identified by the Registry with those actively identified in the State and Metropolitan Area ALS Surveillance project. Cases in the two projects were matched using a combination of identifiers, including, partial social security number, name, date of birth, and sex. The distributions of cases from the two projects that matched/did not match were compared and Chi-square tests conducted to determine statistical significance. There were 5883 ALS cases identified by the surveillance project. Of these, 1116 died before the Registry started, leaving 4767 cases. We matched 2720 cases from the surveillance project to those in the Registry. The cases identified by the surveillance project that did not match cases in the Registry were more likely to be non-white, Hispanic, less than 65 years of age, and from western states. The methods used by the Registry to identify ALS cases, i.e. national administrative data and self-registration, worked well but missed cases. These findings suggest that developing strategies to identify and promote the Registry to those who were more likely to be missing, e.g. non-white and Hispanic, could be beneficial to improving the completeness of the Registry.

Unequal care for dying patients in Sweden: a comparative registry study of deaths from heart disease and cancer.

PubMed

Brännström, Margareta; Hägglund, Lena; Fürst, Carl Johan; Boman, Kurt

2012-12-01

The Swedish Palliative Registry is a nationwide quality registry aimed at facilitating improvement in end-of-life care. The goal is for the registry to list and report quality indicators related to care during the last week of life in all cases expected death in Sweden. To examine the quality of care during the last week of life as reported to the registry for patients with heart disease compared to those with cancer. A retrospective registry study. Patients dying of heart disease compared to those dying from cancer had more shortness of breath, fewer drugs prescribed as needed against the usual symptoms and often died alone. Furthermore, they and their close relatives received less information about the imminence of death and bereavement follow-up was less common. The healthcare personnel were less aware of the heart disease patients' symptoms and less often knew about where they wished to die. Great differences were found in registered end-of-life care suggesting that the care given to patients with heart disease and cancer was unequal even after adjustment for age, sex and setting at the time of death. If our observational findings are confirmed in future studies there is obviously a need for new models for end-of-life management in order to facilitate the provision of equal care to dying patients regardless of diagnosis.

Agency for Toxic Substances and Disease Registry

MedlinePlus

... fact sheet. Hexachlorobenzene Learn important information about Hexachlorobenzene. ALS Registry 7th Anniversary Read how the Registry has been working to better understand ALS for 7 years now. Protect the Health of ...

Proportion of patients in the Uganda rheumatic heart disease registry with advanced disease requiring urgent surgical interventions.

PubMed

Zhang, WanZhu; Okello, Emmy; Nyakoojo, Wilson; Lwabi, Peter; Mondo, Charles K

2015-12-01

Since the establishment of the Uganda Rheumatic Heart Registry, over 900 patients have been enrolled. We sought to stratify the patients in the registry according to disease severity and optimal management strategy. We reviewed data of 618 patients who had enrolled in the Registry between March 2010 and February 2013. The 67 patients who had died were excluded leaving 551 patients who were recruited. The optimum management strategy was determined according to the 2012 European Society of Cardiology guidelines on the management of valvular heart disease. Out of the 551 patient's records evaluated, 398 (72.3%) required invasive intervention, with 332(60.3%) patients requiring surgery and 66 (12.0%) requiring percutaneous mitral commissurotomy (PMC). This leaves only 27.7% of patients who required only medical management. Currently, majority of the patients (498, 90.4%) in the registry are on medical treatment. Of the 60.3% requiring surgical intervention, only 8.0% (44 patients) underwent valvular surgery and 5(1.0%) patients of the 66 (12.0%) underwent PMC successfully. There is a high proportion of patients with severe disease that require surgical treatment yet they cannot access this therapy due to absence of local expertise.

Challenges in Identifying Patients with Type 2 Diabetes for Quality-Improvement Interventions in Primary Care Settings and the Importance of Valid Disease Registries.

PubMed

Wozniak, Lisa; Soprovich, Allison; Rees, Sandra; Johnson, Steven T; Majumdar, Sumit R; Johnson, Jeffrey A

2015-10-01

Patient registries are considered an important foundation of chronic disease management, and diabetes patient registries are associated with better processes and outcomes of care. The purpose of this article is to describe the development and use of registries in the Alberta's Caring for Diabetes (ABCD) project to identify and reach target populations for quality-improvement interventions in the primary care setting. We applied the reach, effectiveness, adoption, implementation and maintenance (RE-AIM) framework and expanded the definition of reach beyond the individual (i.e. patient) level to include the ability to identify target populations at an organizational level. To characterize reach and the implementation of registries, semistructured interviews were conducted with key informants, and a usual-care checklist was compiled for each participating Primary Care Network (PCN). Content analysis was used to analyze qualitative data. Using registries to identify and recruit participants for the ABCD interventions proved challenging. The quality of the registries depended on whether physicians granted PCN access to patient lists, the strategies used in development, the reliability of diagnostic information and the data elements collected. In addition, once a diabetes registry was developed, there was limited ability to update it. Proactive management of chronic diseases like diabetes requires the ability to reach targeted patients at the population level. We observed several challenges to the development and application of patient registries. Given the importance of valid registries, strong collaborations and novel strategies that involve policy-makers, PCNs and providers are needed to help find solutions to improve registry quality and resolve maintenance issues. Copyright © 2015 Canadian Diabetes Association. Published by Elsevier Inc. All rights reserved.

50 CFR 600.1410 - Registry process.

Code of Federal Regulations, 2010 CFR

2010-10-01

....nmfs.noaa.gov or by calling a toll-free telephone number available by contacting NMFS or at the NMFS website. (b) Individuals must submit their name; address; telephone number; date of birth; region(s) of... vessel owner or operator must submit vessel owner name, address, date of birth, and telephone number...

50 CFR 600.1410 - Registry process.

Code of Federal Regulations, 2014 CFR

2014-10-01

....nmfs.noaa.gov or by calling a toll-free telephone number available by contacting NMFS or at the NMFS website. (b) Individuals must submit their name; address; telephone number; date of birth; region(s) of... vessel owner or operator must submit vessel owner name, address, date of birth, and telephone number...

50 CFR 600.1410 - Registry process.

Code of Federal Regulations, 2013 CFR

2013-10-01

....nmfs.noaa.gov or by calling a toll-free telephone number available by contacting NMFS or at the NMFS website. (b) Individuals must submit their name; address; telephone number; date of birth; region(s) of... vessel owner or operator must submit vessel owner name, address, date of birth, and telephone number...

50 CFR 600.1410 - Registry process.

Code of Federal Regulations, 2011 CFR

2011-10-01

....nmfs.noaa.gov or by calling a toll-free telephone number available by contacting NMFS or at the NMFS website. (b) Individuals must submit their name; address; telephone number; date of birth; region(s) of... vessel owner or operator must submit vessel owner name, address, date of birth, and telephone number...

50 CFR 600.1410 - Registry process.

Code of Federal Regulations, 2012 CFR

2012-10-01

....nmfs.noaa.gov or by calling a toll-free telephone number available by contacting NMFS or at the NMFS website. (b) Individuals must submit their name; address; telephone number; date of birth; region(s) of... vessel owner or operator must submit vessel owner name, address, date of birth, and telephone number...

Occupational Disease Registries–Characteristics and Experiences

PubMed Central

Davoodi, Somayeh; Haghighi, Khosro Sadeghniat; Kalhori, Sharareh Rostam Niakan; Hosseini, Narges Shams; Mohammadzadeh, Zeinab; Safdari, Reza

2017-01-01

Introduction: Due to growth of occupational diseases and also increase of public awareness about their consequences, attention to various aspects of diseases and improve occupational health and safety has found great importance. Therefore, there is the need for appropriate information management tools such as registries in order to recognitions of diseases patterns and then making decision about prevention, early detection and treatment of them. These registries have different characteristics in various countries according to their occupational health priorities. Aim: Aim of this study is evaluate dimensions of occupational diseases registries including objectives, data sources, responsible institutions, minimum data set, classification systems and process of registration in different countries. Material and Methods: In this study, the papers were searched using the MEDLINE (PubMed) Google scholar, Scopus, ProQuest and Google. The search was done based on keyword in English for all motor engines including “occupational disease”, “work related disease”, “surveillance”, “reporting”, “registration system” and “registry” combined with name of the countries including all subheadings. After categorizing search findings in tables, results were compared with each other. Results: Important aspects of the registries studied in ten countries including Finland, France, United Kingdom, Australia, Czech Republic, Malaysia, United States, Singapore, Russia and Turkey. The results show that surveyed countries have statistical, treatment and prevention objectives. Data sources in almost the rest of registries were physicians and employers. The minimum data sets in most of them consist of information about patient, disease, occupation and employer. Some of countries have special occupational related classification systems for themselves and some of them apply international classification systems such as ICD-10. Finally, the process of registration system was different in countries. Conclusion: Because occupational diseases are often preventable, but not curable, it is necessary to all countries, to consider prevention and early detection of occupational diseases as the objectives of their registry systems. Also it is recommended that all countries reach an agreement about global characteristics of occupational disease registries. This enables country to compare their data at international levels. PMID:28883681

The Psoriatic Arthritis Registry of Turkey: results of a multicentre registry on 1081 patients.

PubMed

Kalyoncu, Umut; Bayindir, Özün; Ferhat Öksüz, Mustafa; Doğru, Atalay; Kimyon, Gezmiş; Tarhan, Emine Figen; Erden, Abdulsamet; Yavuz, Şule; Can, Meryem; Çetin, Gözde Yıldırım; Kılıç, Levent; Küçükşahin, Orhan; Omma, Ahmet; Ozisler, Cem; Solmaz, Dilek; Bozkirli, Emine Duygu Ersözlü; Akyol, Lütfi; Pehlevan, Seval Masatlıoğlu; Gunal, Esen Kasapoglu; Arslan, Fatos; Yılmazer, Barış; Atakan, Nilgun; Aydın, Sibel Zehra

2017-02-01

The aim was to assess the characteristics of PsA, find out how well the disease is controlled in real life, demonstrate the treatments and identify the unmet needs. The PsA registry of Turkey is a multicentre Web-based registry established in 2014 and including 32 rheumatology centres. Detailed data regarding demographics for skin and joint disease, disease activity assessments and treatment choices were collected. One thousand and eighty-one patients (64.7% women) with a mean (sd) PsA duration of 5.8 (6.7) years were enrolled. The most frequent type of PsA was polyarticular [437 (40.5%)], followed by oligoarticular [407 (37.7%)] and axial disease [372 (34.4%)]. The mean (sd) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8), respectively. Of these patients, 38.6% were on conventional synthetic DMARD monotherapy, 7.1% were on anti-TNF monotherapy, and 22.5% were using anti-TNF plus conventional synthetic DMARD combinations. According to DAS28, 86 (12.4%) patients had high and 105 (15.2%) had moderate disease activity. Low disease activity was achieved in 317 (45.7%) patients, and 185 (26.7%) were in remission. Minimal disease activity data could be calculated in 247 patients, 105 of whom (42.5%) had minimal disease activity. The major differences among sexes were that women were older and had less frequent axial disease, more fatigue, higher HAQ scores and less remission. The PsA registry of Turkey had similarities with previously published registries, supporting its external validity. The finding that women had more fatigue and worse functioning as well as the high percentage of active disease state highlight the unmet need in treatment of PsA. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Disease registries on the nationwide health information network.

PubMed

Russler, Daniel

2011-05-01

Donation by individuals of their protected health information (PHI) for evidence-based research potentially benefits all individuals with disease through improved understandings of disease patterns. In the future, a better understanding of how disease features combine into unique patterns of disease will generate new disease classifications, supporting greater specificity in health management techniques. However, without large numbers of people who donate their PHI to disease registries designed for research, it is difficult for researchers to discover the existence of complex patterns or to create more specific evidence-based management techniques. In order to identify new opportunities in disease registry design, an analysis of the current stage of maturity of the newly created U.S. Nationwide Health Information Network (NwHIN) related to large-scale consumer donation of PHI is presented. Utilizing a use-case analysis methodology, the consumer-centric designs of the policies and technologies created for the NwHIN were examined for the potential to support consumer donations of PHI to research. The NwHIN design has placed the enforcement point for the policy-based release of PHI over the Internet into a specialized gateway accessible to consumer authorization. However, current NwHIN policies leave the final decision regarding release of PHI for research to the health care providers rather than to the consumers themselves. Should disease registries designed for research be established on the NwHIN, consumers might then directly authorize the donation of their PHI to these disease registries. However, under current NwHIN policies, consumer authorization does not guarantee release of PHI by health providers. © 2011 Diabetes Technology Society.

Long-term Mortality of Patients With Tuberculous Meningitis in New York City: A Cohort Study.

PubMed

Vinnard, Christopher; King, Liza; Munsiff, Sonal; Crossa, Aldo; Iwata, Kentaro; Pasipanodya, Jotam; Proops, Douglas; Ahuja, Shama

2017-02-15

Tuberculous meningitis (TBM) is the most devastating clinical presentation of infection with Mycobacterium tuberculosis; delayed initiation of effective antituberculosis therapy is associated with poor treatment outcomes. Our objective was to determine the relationship between drug resistance and 10-year mortality among patients with TBM. We conducted a retrospective cohort study of 324 patients with culture-confirmed TBM, susceptibility results reported for isoniazid and rifampin, and initiation of at least 2 antituberculosis drugs, reported to the tuberculosis registry in New York City between 1 January 1992 and 31 December 2001. Date of death was ascertained by matching the tuberculosis registry with death certificate data for 1992-2012 from the New York Office of Vital Statistics. Human immunodeficiency virus (HIV) status was ascertained by medical records review, matching with the New York City HIV Surveillance registry, and review of cause of death. Among 257 TBM patients without rifampin-resistant isolates, isoniazid resistance was associated with mortality after the first 60 days of treatment when controlling for age and HIV infection (adjusted hazard ratio, 1.94 [95% confidence interval, 1.08-3.94]). Death occurred before completion of antituberculosis therapy in 63 of 67 TBM patients (94%) with rifampin-resistant disease. Among patients with culture-confirmed TBM, we observed rapid early mortality in patients with rifampin-resistant isolates, and an independent association between isoniazid-resistant isolates and death after 60 days of therapy. These findings support the continued evaluation of rapid diagnostic techniques and the empiric addition of second-line drugs for patients with clinically suspected drug-resistant TBM. © The Author 2017. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail: journals.permissions@oup.com.

Predicting trauma patient mortality: ICD [or ICD-10-AM] versus AIS based approaches.

PubMed

Willis, Cameron D; Gabbe, Belinda J; Jolley, Damien; Harrison, James E; Cameron, Peter A

2010-11-01

The International Classification of Diseases Injury Severity Score (ICISS) has been proposed as an International Classification of Diseases (ICD)-10-based alternative to mortality prediction tools that use Abbreviated Injury Scale (AIS) data, including the Trauma and Injury Severity Score (TRISS). To date, studies have not examined the performance of ICISS using Australian trauma registry data. This study aimed to compare the performance of ICISS with other mortality prediction tools in an Australian trauma registry. This was a retrospective review of prospectively collected data from the Victorian State Trauma Registry. A training dataset was created for model development and a validation dataset for evaluation. The multiplicative ICISS model was compared with a worst injury ICISS approach, Victorian TRISS (V-TRISS, using local coefficients), maximum AIS severity and a multivariable model including ICD-10-AM codes as predictors. Models were investigated for discrimination (C-statistic) and calibration (Hosmer-Lemeshow statistic). The multivariable approach had the highest level of discrimination (C-statistic 0.90) and calibration (H-L 7.65, P= 0.468). Worst injury ICISS, V-TRISS and maximum AIS had similar performance. The multiplicative ICISS produced the lowest level of discrimination (C-statistic 0.80) and poorest calibration (H-L 50.23, P < 0.001). The performance of ICISS may be affected by the data used to develop estimates, the ICD version employed, the methods for deriving estimates and the inclusion of covariates. In this analysis, a multivariable approach using ICD-10-AM codes was the best-performing method. A multivariable ICISS approach may therefore be a useful alternative to AIS-based methods and may have comparable predictive performance to locally derived TRISS models. © 2010 The Authors. ANZ Journal of Surgery © 2010 Royal Australasian College of Surgeons.

Registries in orthopaedics.

PubMed

Delaunay, C

2015-02-01

The first nationwide orthopaedic registry was created in Sweden in 1975 to collect data on total knee arthroplasty (TKA). Since then, several countries have established registries, with varying degrees of success. Managing a registry requires time and money. Factors that contribute to successful registry management include the use of a single identifier for each patient to ensure full traceability of all procedures related to a given implant; a long-term funding source; a contemporary, rapid, Internet-based data collection method; and the collection of exhaustive data, at least for innovative implants. The effects of registries on practice patterns should be evaluated. The high cost of registries raises issues of independence and content ownership. Scandinavian countries have been maintaining orthopaedic registries for nearly four decades (since 1975). The first English-language orthopaedic registry was not created until 1998 (in New Zealand), and both the US and many European countries are still struggling to establish orthopaedic registries. To date, there are 11 registered nationwide registries on total knee and total hip replacement. The data they contain are often consistent, although contradictions occur in some cases due to major variations in cultural and market factors. The future of registries will depend on the willingness of health authorities and healthcare professionals to support the creation and maintenance of these tools. Surgeons feel that registries should serve merely to compare implants. Health authorities, in contrast, have a strong interest in practice patterns and healthcare institution performances. Striking a balance between these objectives should allow advances in registry development in the near future. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Constructing a Local Potential Participant Registry to Improve Alzheimer's Disease Clinical Research Recruitment.

PubMed

Grill, Joshua D; Hoang, Dan; Gillen, Daniel L; Cox, Chelsea G; Gombosev, Adrijana; Klein, Kirsten; O'Leary, Steve; Witbracht, Megan; Pierce, Aimee

2018-01-01

Potential participant registries are tools to address the challenge of slow recruitment to clinical research. In particular, registries may aid recruitment to secondary prevention clinical trials for Alzheimer's disease (AD), which enroll cognitively normal older individuals meeting specific genetic or biomarker criteria. Evidence of registry effectiveness is sparse, as is guidance on optimal designs or methods of conduct. We report our experiences of developing a novel local potential participant registry that implemented online enrollment and data collection. In the first year of operation, 957 individuals submitted email addresses to the registry, of whom 592 self-reported demographic, family history, and medical data. In addition, registrants provided information related to their interest and willingness to be contacted about studies. Local earned media and community education were the most effective methods of recruitment into the registry. Seventy-six (26%) of 298 registrants contacted about studies in the first year enrolled in those studies. One hundred twenty-nine registrants were invited to enroll in a preclinical AD trial, of whom 25 (18%) screened and 6 were randomized. These results indicate that registries can aid recruitment and provide needed guidance for investigators initiating new local registries.

Electronic health records and disease registries to support integrated care in a health neighbourhood: an ontology-based methodology.

PubMed

Liaw, Siaw-Teng; Taggart, Jane; Yu, Hairong; Rahimi, Alireza

2014-01-01

Disease registries derived from Electronic Health Records (EHRs) are widely used for chronic disease management (CDM). However, unlike national registries which are specialised data collections, they are usually specific to an EHR or organization such as a medical home. We approached registries from the perspective of integrated care in a health neighbourhood, considering data quality issues such as semantic interoperability (consistency), accuracy, completeness and duplication. Our proposition is that a realist ontological approach is required to systematically and accurately identify patients in an EHR or data repository of EHRs, assess intrinsic data quality and fitness for use by members of the multidisciplinary integrated care team. We report on this approach as applied to routinely collected data in an electronic practice based research network in Australia.

An i2b2-based, generalizable, open source, self-scaling chronic disease registry

PubMed Central

Quan, Justin; Ortiz, David M; Bousvaros, Athos; Ilowite, Norman T; Inman, Christi J; Marsolo, Keith; McMurry, Andrew J; Sandborg, Christy I; Schanberg, Laura E; Wallace, Carol A; Warren, Robert W; Weber, Griffin M; Mandl, Kenneth D

2013-01-01

Objective Registries are a well-established mechanism for obtaining high quality, disease-specific data, but are often highly project-specific in their design, implementation, and policies for data use. In contrast to the conventional model of centralized data contribution, warehousing, and control, we design a self-scaling registry technology for collaborative data sharing, based upon the widely adopted Integrating Biology & the Bedside (i2b2) data warehousing framework and the Shared Health Research Information Network (SHRINE) peer-to-peer networking software. Materials and methods Focusing our design around creation of a scalable solution for collaboration within multi-site disease registries, we leverage the i2b2 and SHRINE open source software to create a modular, ontology-based, federated infrastructure that provides research investigators full ownership and access to their contributed data while supporting permissioned yet robust data sharing. We accomplish these objectives via web services supporting peer-group overlays, group-aware data aggregation, and administrative functions. Results The 56-site Childhood Arthritis & Rheumatology Research Alliance (CARRA) Registry and 3-site Harvard Inflammatory Bowel Diseases Longitudinal Data Repository now utilize i2b2 self-scaling registry technology (i2b2-SSR). This platform, extensible to federation of multiple projects within and between research networks, encompasses >6000 subjects at sites throughout the USA. Discussion We utilize the i2b2-SSR platform to minimize technical barriers to collaboration while enabling fine-grained control over data sharing. Conclusions The implementation of i2b2-SSR for the multi-site, multi-stakeholder CARRA Registry has established a digital infrastructure for community-driven research data sharing in pediatric rheumatology in the USA. We envision i2b2-SSR as a scalable, reusable solution facilitating interdisciplinary research across diseases. PMID:22733975

An i2b2-based, generalizable, open source, self-scaling chronic disease registry.

PubMed

Natter, Marc D; Quan, Justin; Ortiz, David M; Bousvaros, Athos; Ilowite, Norman T; Inman, Christi J; Marsolo, Keith; McMurry, Andrew J; Sandborg, Christy I; Schanberg, Laura E; Wallace, Carol A; Warren, Robert W; Weber, Griffin M; Mandl, Kenneth D

2013-01-01

Registries are a well-established mechanism for obtaining high quality, disease-specific data, but are often highly project-specific in their design, implementation, and policies for data use. In contrast to the conventional model of centralized data contribution, warehousing, and control, we design a self-scaling registry technology for collaborative data sharing, based upon the widely adopted Integrating Biology & the Bedside (i2b2) data warehousing framework and the Shared Health Research Information Network (SHRINE) peer-to-peer networking software. Focusing our design around creation of a scalable solution for collaboration within multi-site disease registries, we leverage the i2b2 and SHRINE open source software to create a modular, ontology-based, federated infrastructure that provides research investigators full ownership and access to their contributed data while supporting permissioned yet robust data sharing. We accomplish these objectives via web services supporting peer-group overlays, group-aware data aggregation, and administrative functions. The 56-site Childhood Arthritis & Rheumatology Research Alliance (CARRA) Registry and 3-site Harvard Inflammatory Bowel Diseases Longitudinal Data Repository now utilize i2b2 self-scaling registry technology (i2b2-SSR). This platform, extensible to federation of multiple projects within and between research networks, encompasses >6000 subjects at sites throughout the USA. We utilize the i2b2-SSR platform to minimize technical barriers to collaboration while enabling fine-grained control over data sharing. The implementation of i2b2-SSR for the multi-site, multi-stakeholder CARRA Registry has established a digital infrastructure for community-driven research data sharing in pediatric rheumatology in the USA. We envision i2b2-SSR as a scalable, reusable solution facilitating interdisciplinary research across diseases.

Methodological Challenges When Comparing Demographic and Clinical Characteristics of International Observational Registries.

PubMed

Verstappen, Suzanne M M; Askling, Johan; Berglind, Niklas; Franzen, Stefan; Frisell, Thomas; Garwood, Christopher; Greenberg, Jeffrey D; Holmqvist, Marie; Horne, Laura; Lampl, Kathy; Michaud, Kaleb; Nyberg, Fredrik; Pappas, Dimitrios A; Reed, George; Symmons, Deborah P M; Tanaka, Eiichi; Tran, Trung N; Yamanaka, Hisashi; Ho, Meilien

2015-12-01

Comparisons of data from different registries can be helpful in understanding variations in many aspects of rheumatoid arthritis (RA). The study aim was to assess and improve the comparability of demographic, clinical, and comorbidity data from 5 international RA registries. Using predefined definitions, 2 subsets of patients (main cohort and subcohort) from 5 international observational registries (Consortium of Rheumatology Researchers of North America Registry [CORRONA], the Swedish Rheumatology Quality of Care Register [SRR], the Norfolk Arthritis Register [NOAR], the Institute of Rheumatology Rheumatoid Arthritis cohort [IORRA], and CORRONA International) were evaluated and compared. Patients ages >18 years with RA, and present in or recruited to the registry from January 1, 2000, were included in the main cohort. Patients from the main cohort with positive rheumatoid factor and/or erosive RA who had received ≥1 synthetic disease-modifying antirheumatic drug (DMARD), and switched to or added another DMARD, were included in the subcohort at time of treatment switch. Age and sex distributions were fairly similar across the registries. The percentage of patients with a high Disease Activity Score in 28 joints score varied between main cohorts (17.5% IORRA, 18.9% CORRONA, 24.7% NOAR, 27.7% CORRONA International, and 36.8% SRR), with IORRA, CORRONA, and CORRONA International including more prevalent cases of RA; the differences were smaller for the subcohort. Prevalence of comorbidities varied across registries (e.g., coronary artery disease ranged from 1.5% in IORRA to 7.9% in SRR), partly due to the way comorbidity data were captured and general cultural differences; the pattern was similar for the subcohorts. Despite different inclusion criteria for the individual RA registries, it is possible to improve the comparability and interpretability of differences across RA registries by applying well-defined cohort definitions. © 2015, American College of Rheumatology.

Clinical patient registry recruitment and retention: a survey of patients in two chronic disease registries.

PubMed

Solomon, Daniel H; Shadick, Nancy A; Weinblatt, Michael E; Frits, Michelle; Iannaccone, Christine; Zak, Agnes; Korzenik, Joshua R

2017-04-17

The collection of routine clinical data in the setting of research registries can serve an important role in understanding real world care. However, relatively little is known about the patient experience in registries, motivating us to survey patients enrolled in two chronic disease registries. We conducted similar surveys in two disease-based registries based at one academic medical center in the US. One group of patients with rheumatoid arthritis (RA) had been enrolled in a registry, and we focused on retention factors. In a second group of patients with inflammatory bowel disease (IBD) recently enrolled or considering enrollment, we examined factors that would influence their enrollment and willingness to answer frequent questionnaires and give biospecimens. The surveys were analyzed using descriptive statistics and the two cohorts were compared using nonparametric and chi-square tests. We received 150 (50%) completed surveys from RA and 169 (63%) from IBD patients. Mean age of subjects was 62 years in RA and 43 in IBD with more women respondents with RA (83%) than IBD (62%). The two groups described very similar factors as the top three motivations for participation: desire to help others, desire to improve care of own disease, and ease of volunteering. Preferred methods of surveying included mail, e-mail, but telephone was not favored; age was an important correlate of this preference. Respondents preferred surveys either every 1-3 months (28.7% RA and 55.0% IBD) or every 4-6 months (50.7% RA and 29.0% IBD). They differed in the preference for payment for answering surveys with 68.0% with RA answering that no payment was necessary but only 36.1% with IBD felt similarly. Patients engaged in clinical registries demonstrate a high level of commitment to improve care and many report a willingness to answer questions relatively frequently.

Chronic myelogenous leukemia in eastern Pennsylvania: an assessment of registry reporting.

PubMed

Mertz, Kristen J; Buchanich, Jeanine M; Washington, Terri L; Irvin-Barnwell, Elizabeth A; Woytowitz, Donald V; Smith, Roy E

2015-01-01

Chronic myelogenous leukemia (CML) has been reportable to the Pennsylvania Cancer Registry (PCR) since the 1980s, but the completeness of reporting is unknown. This study assessed CML reporting in eastern Pennsylvania where a cluster of another myeloproliferative neoplasm was previously identified. Cases were identified from 2 sources: 1) PCR case reports for residents of Carbon, Luzerne, or Schuylkill County with International Classification of Diseases for Oncology, Third Edition (ICD-O-3) codes 9875 (CML, BCR-ABL+), 9863 (CML, NOS), and 9860 (myeloid leukemia) and date of diagnosis 2001-2009, and 2) review of billing records at hematology practices. Participants were interviewed and their medical records were reviewed by board-certified hematologists. PCR reports included 99 cases coded 9875 or 9863 and 9 cases coded 9860; 2 additional cases were identified by review of billing records. Of the 110 identified cases, 93 were mailed consent forms, 23 consented, and 12 medical records were reviewed. Hematologists confirmed 11 of 12 reviewed cases as CML cases; all 11 confirmed cases were BCR/ABL positive, but only 1 was coded as positive (code 9875). Very few unreported CML cases were identified, suggesting relatively complete reporting to the PCR. Cases reviewed were accurately diagnosed, but ICD-0-3 coding often did not reflect BCR-ABL-positive tests. Cancer registry abstracters should look for these test results and code accordingly.

Cost of Operating Central Cancer Registries and Factors That Affect Cost: Findings From an Economic Evaluation of Centers for Disease Control and Prevention National Program of Cancer Registries.

PubMed

Tangka, Florence K L; Subramanian, Sujha; Beebe, Maggie Cole; Weir, Hannah K; Trebino, Diana; Babcock, Frances; Ewing, Jean

2016-01-01

The Centers for Disease Control and Prevention (CDC) evaluated the economics of the National Program of Cancer Registries to provide the CDC, the registries, and policy makers with the economics evidence-base to make optimal decisions about resource allocation. Cancer registry budgets are under increasing threat, and, therefore, systematic assessment of the cost will identify approaches to improve the efficiencies of this vital data collection operation and also justify the funding required to sustain registry operations. To estimate the cost of cancer registry operations and to assess the factors affecting the cost per case reported by National Program of Cancer Registries-funded central cancer registries. We developed a Web-based cost assessment tool to collect 3 years of data (2009-2011) from each National Program of Cancer Registries-funded registry for all actual expenditures for registry activities (including those funded by other sources) and factors affecting registry operations. We used a random-effects regression model to estimate the impact of various factors on cost per cancer case reported. The cost of reporting a cancer case varied across the registries. Central cancer registries that receive high-quality data from reporting sources (as measured by the percentage of records passing automatic edits) and electronic data submissions, and those that collect and report on a large volume of cases had significantly lower cost per case. The volume of cases reported had a large effect, with low-volume registries experiencing much higher cost per case than medium- or high-volume registries. Our results suggest that registries operate with substantial fixed or semivariable costs. Therefore, sharing fixed costs among low-volume contiguous state registries, whenever possible, and centralization of certain processes can result in economies of scale. Approaches to improve quality of data submitted and increasing electronic reporting can also reduce cost.

78 FR 38982 - Availability of Draft Toxicological Profiles

Federal Register 2010, 2011, 2012, 2013, 2014

2013-06-28

... DEPARTMENT OF HEALTH AND HUMAN SERVICES Agency for Toxic Substances and Disease Registry [Docket... and Disease Registry (ATSDR), Department of Health and Human Services (DHHS). ACTION: Notice of... substances during the profile development process, this Federal Register notice solicits any relevant...

EHR-based disease registries to support integrated care in a health neighbourhood: an ontology-based methodology.

PubMed

Liaw, Siaw-Teng; Taggart, Jane; Yu, Hairong

2014-01-01

Disease registries derived from Electronic Health Records (EHRs) are widely used for chronic disease management. We approached registries from the perspective of integrated care in a health neighbourhood, considering data quality issues such as semantic interoperability (consistency), accuracy, completeness and duplication. Our proposition is that a realist ontological approach is required to accurately identify patients in an EHR or data repository, assess data quality and fitness for use by the multidisciplinary integrated care team. We report on this approach with routinely collected data in a practice based research network in Australia.

Population-based survey of the prevalence of type 1 and type 2 diabetes in school children in Philadelphia.

PubMed

Lipman, Terri H; Ratcliffe, Sarah J; Cooper, Rhona; Levitt Katz, Lorraine E

2013-12-01

Population-based (PB) registries of type 1 diabetes mellitus (T1DM) in children have been essential in determining the geographic, racial, and temporal patterns of the disease. There is a paucity of PB data on the prevalence of type 1 and type 2 diabetes (T2DM) in youth. The prevalence of diabetes in children was determined using a PB survey of the 628 schools in Philadelphia. Data obtained included type of diabetes, date of birth, race, gender, date of diagnosis, diabetes treatment, and most recent height and weight. The survey was completed by nurses at 510 schools (81% of schools) representing 252,896 children (70% of children in Philadelphia). Prevalence (per 1000) was computed. The survey identified 492 cases (355 T1DM, 88 T2DM, 49 type unknown). The overall prevalence of T1DM was 1.58 (0.73 White, 0.56 African American, 0.50 Hispanic); of T2DM was 0.35 (0.03 White, 0.28 African American, 0.05 Hispanic). Mean age at diagnosis was 8.6 and 11.9 years for T1DM and T2DM, respectively. The prevalence of T1DM was higher in boys--T2DM was higher in girls. Of children with T2DM, 25% were treated with insulin. BMI was ≥95th percentile in 20% of children weighed (10% of T1DM, 57% of T2DM). Although the Philadelphia Pediatric Diabetes Registry is the longest ongoing US registry of its kind, these are the first PB diabetes prevalence data of children in Philadelphia. PB studies in schools are able to capture children with diabetes who are diagnosed and treated in a variety of settings. © 2013 Wiley Publishing Asia Pty Ltd and Ruijin Hospital, Shanghai Jiaotong University School of Medicine.

A population-based registry as a source of health indicators for rare diseases: the ten-year experience of the Veneto Region’s rare diseases registry

PubMed Central

2014-01-01

Background Although rare diseases have become a major public health issue, there is a paucity of population-based data on rare diseases. The aim of this epidemiological study was to provide descriptive figures referring to a sizable group of unrelated rare diseases. Methods Data from the rare diseases registry established in the Veneto Region of north-east Italy (population 4,900,000), referring to the years from 2002 to 2012, were analyzed. The registry is based on a web-based system accessed by different users. Cases are enrolled by two different sources: clinicians working at Centers of expertise officially designated to diagnose and care patients with rare diseases and health professionals working in the local health districts. Deaths of patients are monitored by Death Registry. Results So far, 19,547 patients with rare diseases have been registered, and 23% of them are pediatric cases. The overall raw prevalence of the rare diseases monitored in the population under study is 33.09 per 10,000 inhabitants (95% CI 32.56-33.62), whilst the overall incidence is 3.85 per 10,000 inhabitants (95% CI 3.67-4.03). The most commonly-recorded diagnoses belong to the following nosological groups: congenital malformations (Prevalence: 5.45/10,000), hematological diseases (4.83/10,000), ocular disorders (4.47/10,000), diseases of the nervous system (3.51/10,000), and metabolic disorders (2,95/10,000). Most of the deaths in the study population occur among pediatric patients with congenital malformations, and among adult cases with neurological diseases. Rare diseases of the central nervous system carry the highest fatality rate (71.36/1,000). Rare diseases explain 4.2% of general population Years of Life Lost (YLLs), comparing to 1.2% attributable to infectious diseases and 2.6% to diabetes mellitus. Conclusions Our estimates of the burden of rare diseases at population level confirm that these conditions are a relevant public health issue. Our snapshot of their epidemiology is important for public health planning purposes, going to show that population-based registries are useful tools for generating health indicators relating to a considerable number of rare diseases, rather than to specific conditions. PMID:24646171

An innovative and collaborative partnership between patients with rare disease and industry-supported registries: the Global aHUS Registry.

PubMed

Woodward, Len; Johnson, Sally; Walle, Johan Vande; Beck, Joran; Gasteyger, Christoph; Licht, Christoph; Ariceta, Gema

2016-11-21

Patients are becoming increasingly involved in research which can promote innovation through novel ideas, support patient-centred actions, and facilitate drug development. For rare diseases, registries that collect data from patients can increase knowledge of the disease's natural history, evaluate clinical therapies, monitor drug safety, and measure quality of care. The active participation of patients is expected to optimise rare-disease management and improve patient outcomes. However, few reports address the type and frequency of interactions involving patients, and what research input patient groups have. Here, we describe a collaboration between an international group of patient organisations advocating for patients with atypical haemolytic uraemic syndrome (aHUS), the aHUS Alliance, and an international aHUS patient registry (ClinicalTrials.gov NCT01522183). The aHUS Registry Scientific Advisory Board (SAB) invited the aHUS Alliance to submit research ideas important to patients with aHUS. This resulted in 24 research suggestions from patients and patient organisations being presented to the SAB. The proposals were classified under seven categories, the most popular of which were understanding factors that cause disease manifestations and learning more about the clinical and psychological/social impact of living with the disease. Subsequently, aHUS Alliance members voted for up to five research priorities. The top priority was: "What are the outcomes of a transplant without eculizumab and what non-kidney damage is likely in patients with aHUS?". This led directly to the initiation of an ongoing analysis of the data collected in the Registry on patients with kidney transplants. This collaboration resulted in several topics proposed by the aHUS Alliance being selected as priority activities for the aHUS Registry, with one new analysis already underway. A clear pathway was established for engagement between a patient advocacy group and an international research network. This should ensure the development of a long-term partnership which clearly benefits both groups.

Improving diabetes population management efficiency with an informatics solution.

PubMed

Zai, Adrian; Grant, Richard; Andrews, Carl; Yee, Ronnie; Chueh, Henry

2007-10-11

Despite intensive resource use for diabetes management in the U.S., our care continues to fall short of evidence-based goals, partly due to system inefficiencies. Diabetes registries are increasingly being utilized as a critical tool for population level disease management by providing real-time data. Since the successful adoption of a diabetes registry depends on how well it integrates with disease management workflows, we optimized our current diabetes management workflow and designed our registry application around it.

A Comparison of Patient Characteristics and Outcomes in Selected European and U.S. Rheumatoid Arthritis Registries

PubMed Central

Curtis, Jeffrey R; Jain, Archana; Askling, Johan; Bridges, Lou; Carmona, Loreto; Dixon, William; Finckh, Axel; Hyrich, Kimme; Greenberg, Jeffrey; Kremer, Joel; Listing, Joachim; Michaud, Kaleb; Mikuls, Ted; Shadick, Nancy; Solomon, Daniel H; Wolfe, Fred; Zink, Angela

2010-01-01

Purpose To provide a qualitative comparison of selected US and European rheumatoid arthritis (RA) biologics registries and cohorts including ARTIS, BIOBADASER, BSRBR, BRASS, CLEAR, CORRONA, NDB, RABBIT, SCQM, and VARA. Randomized controlled trials (RCTs) have demonstrated the efficacy of biologic agents in treatment of rheumatic diseases. However, results from RCTs may not be generalizable to clinical practice because of their strict inclusion and exclusion criteria. Assessment of safety using RCT data also is limited by short duration of follow-up and relatively small sample sizes which generally preclude analysis of longer-term outcomes and rare adverse events. In rheumatology, various observational cohorts and registries have been created to complement information obtained from RCTs, some with the primary purpose of monitoring effectiveness and safety of biologic agents. Most registries are either drug based or disease based. These registries include patients with a variety of rheumatic diseases including RA. A careful comparison of these registries, as provided in this article, can provide a basis for understanding the many similarities and differences inherent in their design, as well as societal context and content, all of which can significantly impact their results and comparisons across registers. Summary The increasing use of biologic agents for treatment of rheumatic diseases has raised important questions about cost, safety and effectiveness of these agents. The unique and variable features of patient populations and registry designs in Europe and the U.S. provide valuable and complementary data on comparative effectiveness and safety of biologic agents to what can be derived from RCTs. PMID:20674669

The St. Thomas' UK Adult Twin Registry.

PubMed

Spector, Tim D; MacGregor, Alex J

2002-10-01

The Registry consists of nearly 10,000 monozygous and dizygous adult caucasian twins aged 18-80 from all over the UK and was started in 1993. This is a volunteer sample recruited by successive media campaigns without selecting for particular diseases or traits. All twins receive a series of disease questionnaires. In addition over half the twins have been assessed in detail clinically for several hundred phenotypes related to common diseases or intermediate traits. The focus has been primarily on cardiovascular, metabolic, musculoskeletal, dermatological, and opthalmological diseases. Over 3000 DZ twins have had a genome wide scan performed as well as many candidate genes allowing both linkage and association studies. The registry has led to many successful innovative research projects, particularly in common diseases previously thought to be predominantly environmental and helped positionally clone some novel genes for common diseases.

A methodological approach for assessing the uptake of core outcome sets using ClinicalTrials.gov: findings from a review of randomised controlled trials of rheumatoid arthritis.

PubMed

Kirkham, Jamie J; Clarke, Mike; Williamson, Paula R

2017-05-17

Objective  To assess the uptake of the rheumatoid arthritis core outcome set using a new assessment method of calculating uptake from data in clinical trial registry entries. Design  Review of randomised trials. Setting  ClinicalTrials.gov. Subjects  273 randomised trials of drug interventions for the treatment of rheumatoid arthritis and registered in ClinicalTrials.gov between 2002 and 2016. Full publications were identified for completed studies from information in the trial registry or from an internet search using Google and the citation database Web of Science. Main outcome measure  The percentage of trials reporting or planning to measure the rheumatoid arthritis core outcome set calculated from the information presented in the trial registry and compared with the percentage reporting the rheumatoid arthritis core outcome set in the resulting trial publications. Results  The full rheumatoid arthritis core outcome set was reported in 81% (116/143) of trials identified on the registry as completed (or terminated) for which results were found in either the published literature or the registry. For trials identified on the registry as completed (or terminated), using information only available in the registry gives an estimate for uptake of 77% (145/189). Conclusions  The uptake of the rheumatoid arthritis core outcome set in clinical trials has continued to increase over time. Using the information on outcomes listed for completed or terminated studies in a trial registry provides a reasonable estimate of the uptake of a core outcome set and is a more efficient and up-to-date approach than examining the outcomes in published trial reports. The method proposed may provide an efficient approach for an up-to-date assessment of the uptake of the 300 core outcome sets already published. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

California’s Parkinson’s Disease Registry Pilot Project - Coordination Center and Northern California Ascertainment

DTIC Science & Technology

2011-03-01

project effort is being directed to investigate possible associations between PD and toxicant exposure using state databases, to define disease ... prevalence and care patterns among registrants, and to assess the value of the registry to stakeholders.

California’s Parkinson’s Disease Registry Pilot Project - Coordination Center and Northern California Ascertainment

DTIC Science & Technology

2012-03-01

project effort is being directed to investigate possible associations between PD and toxicant exposure using state databases, to define disease ... prevalence and care patterns among registrants, and to assess the value of the registry to stakeholders.

SEER Bibliography

Cancer.gov

Search this database of articles and other publications produced by cancer registry staff and Surveillance Research Program staff. Search by author, title, date, and organization. Provides links to PubMed and abstracts.

Disease Prediction Models and Operational Readiness

DOE Office of Scientific and Technical Information (OSTI.GOV)

Corley, Courtney D.; Pullum, Laura L.; Hartley, David M.

2014-03-19

INTRODUCTION: The objective of this manuscript is to present a systematic review of biosurveillance models that operate on select agents and can forecast the occurrence of a disease event. One of the primary goals of this research was to characterize the viability of biosurveillance models to provide operationally relevant information for decision makers to identify areas for future research. Two critical characteristics differentiate this work from other infectious disease modeling reviews. First, we reviewed models that attempted to predict the disease event, not merely its transmission dynamics. Second, we considered models involving pathogens of concern as determined by the USmore » National Select Agent Registry (as of June 2011). Methods: We searched dozens of commercial and government databases and harvested Google search results for eligible models utilizing terms and phrases provided by public health analysts relating to biosurveillance, remote sensing, risk assessments, spatial epidemiology, and ecological niche-modeling, The publication date of search results returned are bound by the dates of coverage of each database and the date in which the search was performed, however all searching was completed by December 31, 2010. This returned 13,767 webpages and 12,152 citations. After de-duplication and removal of extraneous material, a core collection of 6,503 items was established and these publications along with their abstracts are presented in a semantic wiki at http://BioCat.pnnl.gov. Next, PNNL’s IN-SPIRE visual analytics software was used to cross-correlate these publications with the definition for a biosurveillance model resulting in the selection of 54 documents that matched the criteria resulting Ten of these documents, However, dealt purely with disease spread models, inactivation of bacteria, or the modeling of human immune system responses to pathogens rather than predicting disease events. As a result, we systematically reviewed 44 papers and the results are presented in this analysis.« less

Seventeen years' experience of peritoneal dialysis in Iran: first official report of the Iranian peritoneal dialysis registry.

PubMed

Najafi, Iraj; Alatab, Sudabeh; Atabak, Shahnaz; Majelan, Nader Nouri; Sanadgol, Houshang; Makhdoomi, Khadijeh; Ardalan, Mohammad Reza; Azmandian, Jalal; Shojaee, Abbas; Keshvari, Amir; Hosseini, Mostafa

2014-01-01

To facilitate planning, national renal registries provide reliable and up-to-date information on numbers of patients with end-stage renal disease (ESRD), developing trends, treatment modalities, and outcomes. To that end, the present publication represents the first official report from Iranian Peritoneal Dialysis Registry. The prevalence, demographics, and clinical characteristics of patients on peritoneal dialysis (PD) were collected from all PD centers throughout the country. By the end of 2009, the prevalence of ESRD was 507 per million population in Iran. The most common renal replacement modality was hemodialysis (51.2%), followed by kidney transplantation (44.7%), and then PD (4.1%). The mean age of PD patients was 46 years, and the most common causes of ESRD were diabetes (33.5%), hypertension (24.4%), and glomerulonephritis (8.2%). Overall patient mortality was 25%, with cardiac events (46%), cerebral stroke (10%), and infection (8%) being the main causes of death. The 1-, 3-, and 5-year survivals were 89%, 64%, and 49% respectively. The most common cause of dropout was peritonitis (17.6%). Staphylococcus (coagulase-negative and S. aureus) was the most prevalent causative organism in peritonitis episodes; however, in more than 50% of episodes, a sterile culture was reported. Mean baseline serum hemoglobin and albumin were 10.7 g/dL and 3.6 g/dL respectively. Our registry results, representing the second largest report of PD in the Middle East, is almost comparable to available regional data. We hope that, in future, we can improve our shortcomings and lessen the gap with developed countries. Copyright © 2014 International Society for Peritoneal Dialysis.

Methodological challenges in monitoring new treatments for rare diseases: lessons from the cryopyrin-associated periodic syndrome registry.

PubMed

Tilson, Hugh; Primatesta, Paola; Kim, Dennis; Rauer, Barbara; Hawkins, Philip N; Hoffman, Hal M; Kuemmerle-Deschner, Jasmin; van der Poll, Tom; Walker, Ulrich A

2013-09-10

The Cryopyrin-Associated Periodic Syndromes (CAPS) are a group of rare hereditary autoinflammatory diseases and encompass Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS), and Neonatal Onset Multisystem Inflammatory Disease (NOMID). Canakinumab is a monoclonal antibody directed against IL-1 beta and approved for CAPS patients but requires post-approval monitoring due to low and short exposures during the licensing process. Creative approaches to observational methodology are needed, harnessing novel registry strategies to ensure Health Care Provider reporting and patient monitoring. A web-based registry was set up to collect information on long-term safety and effectiveness of canakinumab for CAPS. Starting in November 2009, this registry enrolled 241 patients in 43 centers and 13 countries by December 31, 2012. One-third of the enrolled population was aged < 18; the overall population is evenly divided by gender. Enrolment is ongoing for children. Innovative therapies in orphan diseases require post-approval structures to enable in depth understanding of safety and natural history of disease. The rarity and distribution of such diseases and unpredictability of treatment require innovative methods for enrolment and follow-up. Broad international practice-based recruitment and web-based data collection are practical.

78 FR 5811 - Statement of Organization, Functions, and Delegations of Authority

Federal Register 2010, 2011, 2012, 2013, 2014

2013-01-28

... of Organization, Functions, and Delegations of Authority Part J (Agency for Toxic Substances and Disease Registry) of the Statement of Organization, Functions, and Delegations of Authority of the... Disease Registry. Section T-B, Organization and Functions, is hereby amended as follows: Delete in its...

75 FR 70276 - Statement of Organization, Functions, and Delegations of Authority

Federal Register 2010, 2011, 2012, 2013, 2014

2010-11-17

... of Organization, Functions, and Delegations of Authority Part J (Agency for Toxic Substances and Disease Registry) of the Statement of Organization, Functions, and Delegations of Authority of the... Toxic Substances and Disease Registry. Section J-B, Organization and Functions, is hereby amended as...

78 FR 4147 - Notice of Development of Set 26 Toxicological Profiles

Federal Register 2010, 2011, 2012, 2013, 2014

2013-01-18

... DEPARTMENT OF HEALTH AND HUMAN SERVICES Agency for Toxic Substances and Disease Registry [ATSDR-277] Notice of Development of Set 26 Toxicological Profiles AGENCY: Agency for Toxic Substances and Disease Registry (ATSDR), Department of Health and Human Services (HHS). ACTION: Notice. SUMMARY: This...

77 FR 6800 - Notice of Development of Set 25 Toxicological Profiles

Federal Register 2010, 2011, 2012, 2013, 2014

2012-02-09

... DEPARTMENT OF HEALTH AND HUMAN SERVICES Agency for Toxic Substances and Disease Registry [ATSDR-273] Notice of Development of Set 25 Toxicological Profiles AGENCY: Agency for Toxic Substances and Disease Registry (ATSDR), Department of Health and Human Services (DHHS). ACTION: Notice. SUMMARY: This...

The United Kingdom Primary Immune Deficiency (UKPID) registry 2012 to 2017.

PubMed

Shillitoe, B; Bangs, C; Guzman, D; Gennery, A R; Longhurst, H J; Slatter, M; Edgar, D M; Thomas, M; Worth, A; Huissoon, A; Arkwright, P D; Jolles, S; Bourne, H; Alachkar, H; Savic, S; Kumararatne, D S; Patel, S; Baxendale, H; Noorani, S; Yong, P F K; Waruiru, C; Pavaladurai, V; Kelleher, P; Herriot, R; Bernatonienne, J; Bhole, M; Steele, C; Hayman, G; Richter, A; Gompels, M; Chopra, C; Garcez, T; Buckland, M

2018-06-01

This is the second report of the United Kingdom Primary Immunodeficiency (UKPID) registry. The registry will be a decade old in 2018 and, as of August 2017, had recruited 4758 patients encompassing 97% of immunology centres within the United Kingdom. This represents a doubling of recruitment into the registry since we reported on 2229 patients included in our first report of 2013. Minimum PID prevalence in the United Kingdom is currently 5·90/100 000 and an average incidence of PID between 1980 and 2000 of 7·6 cases per 100 000 UK live births. Data are presented on the frequency of diseases recorded, disease prevalence, diagnostic delay and treatment modality, including haematopoietic stem cell transplantation (HSCT) and gene therapy. The registry provides valuable information to clinicians, researchers, service commissioners and industry alike on PID within the United Kingdom, which may not otherwise be available without the existence of a well-established registry. © 2018 British Society for Immunology.

Second generation registry framework.

PubMed

Bellgard, Matthew I; Render, Lee; Radochonski, Maciej; Hunter, Adam

2014-01-01

Information management systems are essential to capture data be it for public health and human disease, sustainable agriculture, or plant and animal biosecurity. In public health, the term patient registry is often used to describe information management systems that are used to record and track phenotypic data of patients. Appropriate design, implementation and deployment of patient registries enables rapid decision making and ongoing data mining ultimately leading to improved patient outcomes. A major bottleneck encountered is the static nature of these registries. That is, software developers are required to work with stakeholders to determine requirements, design the system, implement the required data fields and functionality for each patient registry. Additionally, software developer time is required for ongoing maintenance and customisation. It is desirable to deploy a sophisticated registry framework that can allow scientists and registry curators possessing standard computing skills to dynamically construct a complete patient registry from scratch and customise it for their specific needs with little or no need to engage a software developer at any stage. This paper introduces our second generation open source registry framework which builds on our previous rare disease registry framework (RDRF). This second generation RDRF is a new approach as it empowers registry administrators to construct one or more patient registries without software developer effort. New data elements for a diverse range of phenotypic and genotypic measurements can be defined at any time. Defined data elements can then be utilised in any of the created registries. Fine grained, multi-level user and workgroup access can be applied to each data element to ensure appropriate access and data privacy. We introduce the concept of derived data elements to assist the data element standards communities on how they might be best categorised. We introduce the second generation RDRF that enables the user-driven dynamic creation of patient registries. We believe this second generation RDRF is a novel approach to patient registry design, implementation and deployment and a significant advance on existing registry systems.

Second generation registry framework

PubMed Central

2014-01-01

Background Information management systems are essential to capture data be it for public health and human disease, sustainable agriculture, or plant and animal biosecurity. In public health, the term patient registry is often used to describe information management systems that are used to record and track phenotypic data of patients. Appropriate design, implementation and deployment of patient registries enables rapid decision making and ongoing data mining ultimately leading to improved patient outcomes. A major bottleneck encountered is the static nature of these registries. That is, software developers are required to work with stakeholders to determine requirements, design the system, implement the required data fields and functionality for each patient registry. Additionally, software developer time is required for ongoing maintenance and customisation. It is desirable to deploy a sophisticated registry framework that can allow scientists and registry curators possessing standard computing skills to dynamically construct a complete patient registry from scratch and customise it for their specific needs with little or no need to engage a software developer at any stage. Results This paper introduces our second generation open source registry framework which builds on our previous rare disease registry framework (RDRF). This second generation RDRF is a new approach as it empowers registry administrators to construct one or more patient registries without software developer effort. New data elements for a diverse range of phenotypic and genotypic measurements can be defined at any time. Defined data elements can then be utilised in any of the created registries. Fine grained, multi-level user and workgroup access can be applied to each data element to ensure appropriate access and data privacy. We introduce the concept of derived data elements to assist the data element standards communities on how they might be best categorised. Conclusions We introduce the second generation RDRF that enables the user-driven dynamic creation of patient registries. We believe this second generation RDRF is a novel approach to patient registry design, implementation and deployment and a significant advance on existing registry systems. PMID:24982690

The value of patient registries in advancing pediatric surgical care.

PubMed

Skarsgard, Erik D

2018-05-01

Pediatric surgeons treat a variety of conditions that are distinguished by their low occurrence rate, complexity, and need for integrated multidisciplinary care. Although randomized controlled trials (RCTs) are considered the gold standard for generating evidence to inform best practice, they are poorly suited to rare diseases based on the variability of illness severity, unpredictability in clinical course, and the impact limitations of studying a single intervention at a time. An alternative to RCTs for comparative effectiveness research for rare diseases in pediatric surgery is the patient registry, which collects detailed and condition-specific patient level data related to illness severity, treatment, and outcome, and allows a large, disease-specific database to be created for the dual purposes of collaborative research and quality improvement across participating sites. This review discusses the various functions of a patient registry in fulfilling its mandate of evidence-based practice and outcome improvement using examples from a variety of existing pediatric surgical registries. The value proposition of patient registries as sources of knowledge, facilitators of practice standardization, and enablers of continuous quality improvement is discussed. Copyright © 2018 Elsevier Inc. All rights reserved.

Gene therapy in rare diseases: the benefits and challenges of developing a patient-centric registry for Strimvelis in ADA-SCID.

PubMed

Stirnadel-Farrant, Heide; Kudari, Mahesh; Garman, Nadia; Imrie, Jessica; Chopra, Bikramjit; Giannelli, Stefania; Gabaldo, Michela; Corti, Ambra; Zancan, Stefano; Aiuti, Alessandro; Cicalese, Maria Pia; Batta, Rohit; Appleby, Jonathan; Davinelli, Mario; Ng, Pauline

2018-04-06

Strimvelis (autologous CD34+ cells transduced to express adenosine deaminase [ADA]) is the first ex vivo stem cell gene therapy approved by the European Medicines Agency (EMA), indicated as a single treatment for patients with ADA-severe combined immunodeficiency (ADA-SCID) who lack a suitable matched related bone marrow donor. Existing primary immunodeficiency registries are tailored to transplantation outcomes and do not capture the breadth of safety and efficacy endpoints required by the EMA for the long-term monitoring of gene therapies. Furthermore, for extended monitoring of Strimvelis, the young age of children treated, small patient numbers, and broad geographic distribution of patients all increase the risk of loss to follow-up before sufficient data have been collected. Establishing individual investigator sites would be impractical and uneconomical owing to the small number of patients from each location receiving Strimvelis. An observational registry has been established to monitor the safety and effectiveness of Strimvelis in up to 50 patients over a minimum of 15 years. To address the potential challenges highlighted above, data will be collected by a single investigator site at Ospedale San Raffaele (OSR), Milan, Italy, and entered into the registry via a central electronic platform. Patients/families and the patient's local physician will also be able to submit healthcare information directly to the registry using a uniquely designed electronic platform. Data entry will be monitored by a Gene Therapy Registry Centre (funded by GlaxoSmithKline) who will ensure that necessary information is collected and flows between OSR, the patient/family and the patient's local healthcare provider. The Strimvelis registry sets a precedent for the safety monitoring of future gene therapies. A unique, patient-focused design has been implemented to address the challenges of long-term follow-up of patients treated with gene therapy for a rare disease. Strategies to ensure data completeness and patient retention in the registry will help fulfil pharmacovigilance requirements. Collaboration with partners is being sought to expand from a treatment registry into a disease registry. Using practical and cost-efficient approaches, the Strimvelis registry is hoped to encourage further innovation in registry design within orphan drug development.

Rare Disease Biospecimens and Patient Registries: Interoperability for Research Promotion, a European Example: EuroBioBank and SpainRDR-BioNER.

PubMed

Rubinstein, Yaffa R; Posada de la Paz, Manuel; Mora, Marina

2017-01-01

Well-annotated and properly preserved specimens are crucial both for diagnostic purposes and for use in basic and pre-clinical research, and are especially important for rare disease (RD) studies. Several consortia have been established in the recent years in order to facilitate research and to maximise access to rare biological samples and data stored in rare disease biobanks and registries, among them the EuroBioBank network and the Spain National Rare Disease Registry (RDR) and Biobank (BioNER).EuroBioBank, established in 2001, was the first network of RD biobanks to operate in Europe as a service distributing human DNA, cells, and tissue to the scientific community conducting research on rare diseases.The Spanish RDR and BioNER were created for facilitating rare disease research and health-related matters. The coordination of these two bodies represents an example of great scientific value as biological samples donated by patients at BioNER are linked to clinical information collected in the RDR.Rare disease biobanks and registries will need for the future to increase their effort to improve interconnection so to enable investigators to better locate samples and associated data, while protecting security of the data and privacy of the participants and adhering to international ethical and legal requirements.

76 FR 53906 - Availability of Final Toxicological Profile for RDX

Federal Register 2010, 2011, 2012, 2013, 2014

2011-08-30

... DEPARTMENT OF HEALTH AND HUMAN SERVICES Agency for Toxic Substances and Disease Registry [ATSDR... Disease Registry (ATSDR), Department of Health and Human Services (HHS). ACTION: Notice of availability... 10 of the U.S. Code directs the Secretary of Defense to notify the Secretary of Health and Human...

Completeness and timeliness of tuberculosis notification in Taiwan

PubMed Central

2011-01-01

Tuberculosis (TB) is a notifiable disease by the Communicable Disease Control Law in Taiwan. Several measures have been undertaken to improve reporting of TB but the completeness and timeliness of TB notification in Taiwan has not yet been systemically evaluated. Methods To assess completeness and timeliness of TB notification, potential TB cases diagnosed by health care facilities in the year 2005-2007 were identified using the reimbursement database of national health insurance (NHI), which has 99% population coverage in Taiwan. Potential TB patients required notification were defined as those who have TB-related ICD-9 codes (010-018) in the NHI reimbursement database in 2005-2007, who were not diagnosed with TB in previous year, and who have been prescribed with 2 or more types of anti-TB drugs. Each potential TB case was matched to the national TB registry maintained at Taiwan Centers for Disease Control (CDC) by using national identity number or, if non-citizen, passport number to determine whether the patients had been notified to local public health authorities and Taiwan CDC. The difference in the number of days between date of anti-tuberculosis treatment and date of notification was calculated to determine the timeliness of TB reporting. Results Of the 57,405 TB patients who were prescribed with 2 or more anti-tuberculosis drugs, 55,291 (96.3%) were notified to National TB Registry and 2,114 (3.7%) were not. Of the 55,291 notified cases, 45,250 (81.8%) were notified within 7 days of anti-tuberculosis treatment (timely reporting) and 10,041(18.2%) after 7 days (delayed reporting). Factors significantly associated with failure of notification are younger age, previously notified cases, foreigner, those who visited clinics and those who visited health care facilities only once or twice in 6 months. Conclusion A small proportion of TB cases were not notified and a substantial proportion of notified TB cases had delayed reporting, findings with implication for strengthening surveillance of tuberculosis in Taiwan. Countries where the completeness and timeliness of TB notification has not yet been evaluated should take similar action to strengthen surveillance of TB. PMID:22151346

The establishment and utility of Sweha-Reg: a Swedish population-based registry to understand hereditary angioedema

PubMed Central

Mallbris, Lotus; Nordenfelt, Patrik; Björkander, Janne; Lindfors, Anders; Werner, Sonja; Wahlgren, Carl-Fredrik

2007-01-01

Background The importance of acquiring comprehensive epidemiological and clinical data on hereditary angioedema has increasingly caught the attention of physicians and scientists around the world. The development of networks and creation of comprehensive policies to improve care of people suffering from rare diseases, such as hereditary angioedema, is a stated top priority of the European Union. Hereditary angioedema is a rare disease, that it may be life-threatening. Although the exact prevalence is unknown, current estimates suggest that it is 1/10,000–1/150,000 individuals. The low prevalence requires combined efforts to gain accurate epidemiological data on the disease and so give us tools to reduce morbidity and mortality, and improve quality of life of sufferers. Methods Sweha-Reg is a population-based registry of hereditary angioedema in Sweden with the objectives of providing epidemiological data, and so creates a framework for the study of this disease. The registry contains individual-based data on diagnoses, treatments and outcomes. Conclusion The present manuscript seeks to raise awareness of the existence of Sweha-Reg to stimulate the international collaboration of registries. A synthesis of data from similar registries across several countries is required to approach an inclusive course understanding of HAE. PMID:18053127

Reported Numbers of Patients with Rare Diseases Based on Ten-Year Longitudinal National Disability Registries in Taiwan

ERIC Educational Resources Information Center

Lin, Jin-Ding; Lin, Lan-Ping; Hung, Wen-Jiu

2013-01-01

This paper aims to describe a general demographic picture of patients with rare diseases in Taiwan and particularly focuses on the prevalence of rare diseases over time, age and gender distributions. We analyzed data mainly from the national disability registry from 2002 to 2011 in Taiwan, Republic of China. The results showed that the number of…

Registry Assessment of Peripheral Interventional Devices (RAPID)　- Registry Assessment of Peripheral Interventional Devices Core Data Elements.

PubMed

Jones, W Schuyler; Krucoff, Mitchell W; Morales, Pablo; Wilgus, Rebecca W; Heath, Anne H; Williams, Mary F; Tcheng, James E; Marinac-Dabic, J Danica; Malone, Misti L; Reed, Terrie L; Fukaya, Rie; Lookstein, Robert; Handa, Nobuhiro; Aronow, Herbert D; Bertges, Daniel J; Jaff, Michael R; Tsai, Thomas T; Smale, Joshua A; Zaugg, Margo J; Thatcher, Robert J; Cronenwett, Jack L; Nc, Durham; Md, Silver Spring; Japan, Tokyo; Ny, New York; Ri, Providence; Vt, Burlington; Mass, Newton; Colo, Denver; Ariz, Tempe; Calif, Santa Clara; Minn, Minneapolis; Nh, Lebanon

2018-01-25

The current state of evaluating patients with peripheral artery disease and more specifically of evaluating medical devices used for peripheral vascular intervention (PVI) remains challenging because of the heterogeneity of the disease process, the multiple physician specialties that perform PVI, the multitude of devices available to treat peripheral artery disease, and the lack of consensus about the best treatment approaches. Because PVI core data elements are not standardized across clinical care, clinical trials, and registries, aggregation of data across different data sources and physician specialties is currently not feasible.Methods and Results:Under the auspices of the U.S. Food and Drug Administration's Medical Device Epidemiology Network initiative-and its PASSION (Predictable and Sustainable Implementation of the National Registries) program, in conjunction with other efforts to align clinical data standards-the Registry Assessment of Peripheral Interventional Devices (RAPID) workgroup was convened. RAPID is a collaborative, multidisciplinary effort to develop a consensus lexicon and to promote interoperability across clinical care, clinical trials, and national and international registries of PVI. The current manuscript presents the initial work from RAPID to standardize clinical data elements and definitions, to establish a framework within electronic health records and health information technology procedural reporting systems, and to implement an informatics-based approach to promote the conduct of pragmatic clinical trials and registry efforts in PVI. Ultimately, we hope this work will facilitate and improve device evaluation and surveillance for patients, clinicians, health outcomes researchers, industry, policymakers, and regulators.

78 FR 26780 - Agency Forms Undergoing Paperwork Reduction Act Review

Federal Register 2010, 2011, 2012, 2013, 2014

2013-05-08

... Sclerosis (ALS) Registry--Revision (0923-0041, Expiration 7/31/13)--Agency for Toxic Substances and Disease Registry (ATSDR). Background and Brief Description On October 10, 2008, President Bush signed S. 1382: ALS... Lateral Sclerosis (ALS) Registry. The activities described are part of the effort to create the National...

Lessons from implementing a combined workflow-informatics system for diabetes management.

PubMed

Zai, Adrian H; Grant, Richard W; Estey, Greg; Lester, William T; Andrews, Carl T; Yee, Ronnie; Mort, Elizabeth; Chueh, Henry C

2008-01-01

Shortcomings surrounding the care of patients with diabetes have been attributed largely to a fragmented, disorganized, and duplicative health care system that focuses more on acute conditions and complications than on managing chronic disease. To address these shortcomings, we developed a diabetes registry population management application to change the way our staff manages patients with diabetes. Use of this new application has helped us coordinate the responsibilities for intervening and monitoring patients in the registry among different users. Our experiences using this combined workflow-informatics intervention system suggest that integrating a chronic disease registry into clinical workflow for the treatment of chronic conditions creates a useful and efficient tool for managing disease.

Lessons from Implementing a Combined Workflow–Informatics System for Diabetes Management

PubMed Central

Zai, Adrian H.; Grant, Richard W.; Estey, Greg; Lester, William T.; Andrews, Carl T.; Yee, Ronnie; Mort, Elizabeth; Chueh, Henry C.

2008-01-01

Shortcomings surrounding the care of patients with diabetes have been attributed largely to a fragmented, disorganized, and duplicative health care system that focuses more on acute conditions and complications than on managing chronic disease. To address these shortcomings, we developed a diabetes registry population management application to change the way our staff manages patients with diabetes. Use of this new application has helped us coordinate the responsibilities for intervening and monitoring patients in the registry among different users. Our experiences using this combined workflow-informatics intervention system suggest that integrating a chronic disease registry into clinical workflow for the treatment of chronic conditions creates a useful and efficient tool for managing disease. PMID:18436907

Update of literature from cystic fibrosis registries 2012-2015. Part 6: Epidemiology, nutrition and complications.

PubMed

Salvatore, Donatello; Buzzetti, Roberto; Mastella, Gianni

2017-03-01

Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries. Some studies offer insights into pubertal growth and non-pulmonary complications, such as liver disease, nephropathy, and cancer. Pediatr Pulmonol. 2017;52:390-398. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Next-generation registries: fusion of data for care, and research.

PubMed

Mandl, Kenneth D; Edge, Stephen; Malone, Chad; Marsolo, Keith; Natter, Marc D

2013-01-01

Disease-based registries are a critical tool for electronic data capture of high-quality, gold standard data for clinical research as well as for population management in clinical care. Yet, a legacy of significant operational costs, resource requirements, and poor data liquidity have limited their use. Research registries have engendered more than $3 Billion in HHS investment over the past 17 years. Health delivery systems and Accountable Care Organizations are investing heavily in registries to track care quality and follow-up of patient panels. Despite the investment, regulatory and financial models have often enforced a "single purpose" limitation on each registry, restricting the use of data to a pre-defined set of protocols. The need for cost effective, multi-sourced, and widely shareable registry data sets has never been greater, and requires next-generation platforms to robustly support multi-center studies, comparative effectiveness research, post-marketing surveillance and disease management. This panel explores diverse registry efforts, both academic and commercial, that have been implemented in leading-edge clinical, research, and hybrid use cases. Panelists present their experience in these areas as well as lessons learned, challenges addressed, and near innovations and advances.

Post-approval Studies for Rare Disease Treatments and Orphan Drugs.

PubMed

Maier, William C; Christensen, Ronald A; Anderson, Patricia

2017-01-01

Drug development involves a multi-stage process of drug discovery, animal studies and human clinical trials to assess the safety and efficacy of new medications. Rare disease drug development involves a much smaller number of affected patients, a predominance of pediatric patients and more complicated disease presentation. Post-approval studies are designed to address several limitations associated with the rare disease clinical trials.National and international regulatory agencies in the US and Europe have adopted similar approaches to requirements post-approval data for rare diseases and orphan drug indications. The US FDA published guidance in 2011 and the European Medicines Agency in 2015.Post-approval studies for rare diseases include observational studies, pragmatic trials and randomized controlled studies. Observational studies include both original data collection studies and the use of secondary data (retrospective studies). Original data collection can address limitations of retrospective studies resulting from incomplete information in secondary data sources. Disease registries focus on detail about a broad range of patients with a rare disease while product-related registries focus on specific health care outcomes associated with a single product and may incorporate a comparator of an alternative therapy or therapies.Rare disease patients can be difficult to find and enroll in a registry using conventional physician based driven recruitment. The study process also needs to recognize changes in the patient's disease and lifestyle and adapt both the study design and methods over time. Many rare diseases have strong patient advocacy groups that can in aid the design and execution of rare disease registries.

Meeting The Joint Commission's Dose Incident Identification and External Benchmarking Requirements Using the ACR's Dose Index Registry.

PubMed

Bohl, Michael A; Goswami, Roopa; Strassner, Brett; Stanger, Paula

2016-08-01

The purpose of this investigation was to evaluate the potential of using the ACR's Dose Index Registry(®) to meet The Joint Commission's requirements to identify incidents in which the radiation dose index from diagnostic CT examinations exceeded the protocol's expected dose index range. In total, 10,970 records in the Dose Index Registry were statistically analyzed to establish both an upper and lower expected dose index for each protocol. All 2015 studies to date were then retrospectively reviewed to identify examinations whose total examination dose index exceeded the protocol's defined upper threshold. Each dose incident was then logged and reviewed per the new Joint Commission requirements. Facilities may leverage their participation in the ACR's Dose Index Registry to fully meet The Joint Commission's dose incident identification review and external benchmarking requirements. Copyright © 2016 American College of Radiology. Published by Elsevier Inc. All rights reserved.

Methodological challenges in monitoring new treatments for rare diseases: lessons from the cryopyrin-associated periodic syndrome registry

PubMed Central

2013-01-01

Background The Cryopyrin-Associated Periodic Syndromes (CAPS) are a group of rare hereditary autoinflammatory diseases and encompass Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS), and Neonatal Onset Multisystem Inflammatory Disease (NOMID). Canakinumab is a monoclonal antibody directed against IL-1 beta and approved for CAPS patients but requires post-approval monitoring due to low and short exposures during the licensing process. Creative approaches to observational methodology are needed, harnessing novel registry strategies to ensure Health Care Provider reporting and patient monitoring. Methods A web-based registry was set up to collect information on long-term safety and effectiveness of canakinumab for CAPS. Results Starting in November 2009, this registry enrolled 241 patients in 43 centers and 13 countries by December 31, 2012. One-third of the enrolled population was aged < 18; the overall population is evenly divided by gender. Enrolment is ongoing for children. Conclusions Innovative therapies in orphan diseases require post-approval structures to enable in depth understanding of safety and natural history of disease. The rarity and distribution of such diseases and unpredictability of treatment require innovative methods for enrolment and follow-up. Broad international practice-based recruitment and web-based data collection are practical. PMID:24016338

Unrelated haematopoietic stem cell transplantation in Taiwan and beyond.

PubMed

Yang, K L; Chang, C Y; Lin, S; Shyr, M H; Lin, P Y

2009-06-01

Since its inception in October 1993, the world-renowned Buddhist Tzu Chi Marrow Donor Registry has facilitated more than 1800 cases of stem cell donations for patients in 27 countries to date. Under the auspices of the Buddhist Tzu Chi Stem Cells Center (BTCSCC), the Registry (> 310,000 donors) offers, on average, one case of stem cell donation every day to national or international transplantation community. The accomplishment of the Registry stems from the philosophy and spirit of giving without reward that was inspired by its founder Dharma Master Cheng Yen, the Samaritan devotions of selfless voluntary stem cell donors and the efforts from a dedicated network of volunteer workers. Demographically speaking, slightly less than one third of the donations are provided to domestic patients and the rest to mainland China and countries in Asia, North America, Europe, Middle East, Oceania, and South Africa. While most of the patients belong to the Oriental ethnic group, a few of the patients are non-Oriental. In addition to the Registry, a non-profit umbilical cord blood (UCB) bank is operating since 2002 to provide a complimentary role for patients unable to identify appropriate bone marrow stem cell donors in the Registry in time. To date, with an inventory of over 12,000 units of UCB cryopreserved in the Tzu Chi Cord Blood Bank, 47 units have been employed in 37 cases of transplantation for both paediatric and adult patients domestically and internationally. The fact that Buddhist Tzu Chi Marrow Donor Registry and Cord Blood Bank are established and operating without governmental financial support is unique and special. To facilitate haematopoietic stem cells to its domestic patients experiencing financial burdens, the BTCSCC offers financial aids to the underprivileged for their medical relief. This humanitarian approach and compassion is definitely a role model for many countries in the world.

Current situation and challenge of registry in China.

PubMed

Zhang, Yang; Feng, Yuji; Qu, Zhi; Qi, Yali; Zhan, Siyan

2014-09-01

Increasing emphasis has been placed on registries for an organized system used in developing clinical research to improve health care. China has sufficient data that can be applied broadly, but the heterogeneity and irregularity of registries limit their applicability. This article aims to describe the status of registries in China and the related challenges. Patient registries for observational studies were retrieved from the International Clinical Trials Registry to quantitatively evaluate the number of comparatively high-quality registries in China. A literature search was also performed to provide support and updates. A total of 64 patient registries were retrieved from ClinicalTrials.gov using disease, product, and health service as criteria. The sample sizes ranged from 15 to 30,400, with only 12 registries marked as completed. This article describes and compares the detailed information in many aspects. The efficient use of registries has already made considerable progress in China; however, registries still require standardization, high-quality transition, and coordinated development.

Discrepancies between ClinicalTrials.gov recruitment status and actual trial status: a cross-sectional analysis.

PubMed

Jones, Christopher W; Safferman, Michelle R; Adams, Amanda C; Platts-Mills, Timothy F

2017-10-11

To determine the accuracy of the recruitment status listed on ClinicalTrials.gov as compared with the actual trial status. Cross-sectional analysis. Random sample of interventional phase 2-4 clinical trials registered between 2010 and 2012 on ClinicalTrials.gov. For each trial which was listed within ClinicalTrials.gov as ongoing, two investigators performed a comprehensive literature search for evidence that the trial had actually been completed. For each trial listed as completed or terminated early by ClinicalTrials.gov, we compared the date that the trial was actually concluded with the date the registry was updated to reflect the study's conclusion status. Among the 405 included trials, 92 had a registry status indicating that study activity was either ongoing or the recruitment status was unknown. Of these, published results were available for 34 (37%). Among the 313 concluded trials, the median delay between study completion and a registry update reflecting that the study had ended was 141 days (IQR 48-419), with delays of over 1 year present for 29%. In total, 125 trials (31%) either had a listed recruitment status which was incorrect or had a delay of more than 1 year between the time the study was concluded and the time the registry recruitment status was updated. At present, registry recruitment status information in ClinicalTrials.gov is often outdated or wrong. This inaccuracy has implications for the ability of researchers to identify completed trials and accurately characterise all available medical knowledge on a given subject. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Role of prospective registries in defining the value and effectiveness of spine care.

PubMed

McGirt, Matthew J; Parker, Scott L; Asher, Anthony L; Norvell, Dan; Sherry, Ned; Devin, Clinton J

2014-10-15

Literature review and case example. Describe methodological considerations of spine surgery registries. Review existing spine surgery registries. Describe the Vanderbilt Prospective Spine Registry (VPSR) as a case example and demonstrate its impact on comparative effectiveness research, value analysis, quality improvement, and practice-based learning. To bend the cost curve and ultimately achieve sustainability in health care, medical providers and surgical treatments of the highest quality and effectiveness must be preferentially used and purchased. As the current US health care environment continues to evolve, it will be essential for all spine clinicians to understand and be facile with the principles of evidence-based health care reform. We describe the methodological considerations of spine surgery registries, review the literature to describe existing spine surgery registries, and discuss the VPSR as a case example. We were able to obtain detailed information on 13 existing spine surgery registries through various internet-based resources. Of the 13, 2 registries had start dates before 2000, 3 between 2001 and 2005, 5 starting in 2006, and 3 were indeterminate. Follow-up rates were in the range from 22% to 79%, with longer follow-up times consistently producing lower follow-up rates. Prospective, longitudinal, patient-reported outcomes registries are powerful tools that allow measurement of cost, safety, effectiveness, and health care value across clinically meaningful episodes of care. Registries entirely based on claims or billing data, safety measures alone, process measures, or other proxies of outcome offer valuable insights, but do not provide comprehensive data to drive patient-centered value-based reform. As more spine-focused registries emerge and their integration into the US health care delivery evolve, the evidence to power value-based reform will be enabled.

Setting up of the Indian HIPEC Registry: A Registry for Indian Patients with Peritoneal Surface Malignancies.

PubMed

Bhatt, Aditi; Mehta, Sanket; Ramakrishnan As; Pande, Pankaj; Rajan, Firoz; Rangole, Ashvin; Saklani, Avanish; Sethna, Kayomarz; Singh, Shivendra; Zaveri, Shabber; Gopinath, K S

2017-12-01

There are various registries for patients with peritoneal metastases (PM) that aid pooling of data and generate evidence that dictates current clinical practice. This manuscript describes the setting up of the Indian HIPEC registry that was set up with a similar goal by a group of Indian surgeons. This is a registry for patients with PM treated with CRS and HIPEC in India. It also acts as a database for storing treatment-related information. Patients with PM from colorectal ovarian, gastric, appendiceal tumors, and other rare peritoneal tumors/metastases from rare tumors are enrolled in the registry. A coordinator updates the disease status of patients on a yearly basis. A private organization maintains the database. A non-disclosure agreement is signed between the company and each surgeon contributing to the registry to maintain confidentiality. For enrolling patients, securing institutional permission depends on the requirement of each institute; patient consent is mandatory. Data entry can be prospective or retrospective. To propose and conduct a study, the approval of a scientific committee linked to the registry is required. The Indian HIPEC registry is a practical database for Indian surgeons. There is no regulatory body that mandates collection and publication of scientific data in India. The onus is on each surgeon to capture valuable information pertaining to these common and rare diseases that could contribute to the existing scientific knowledge and guide the treatment of these patients in the future. The next challenge will be to enter data into the registry.

Participation in Clinical Research Registries: A Focus Group Study Examining Views From Patients With Arthritis and Other Chronic Illnesses.

PubMed

Lee, Sara B; Zak, Agnes; Iversen, Maura D; Polletta, Valerie L; Shadick, Nancy A; Solomon, Daniel H

2016-07-01

Patient registries have contributed substantially to progress in clinical research in rheumatic diseases. However, not much is known about how to optimize the patient experience in such registries. We assessed patient views, motivations, and potential barriers towards participation in registry research to better understand how registries can be improved to maximize patient engagement. Focus groups were held with 23 patients (mean ± SD age 59 ± 13 years) from the Boston area and led by a bilingual moderator trained in focus group methodology, using a semistructured moderator guide. Three separate focus groups were conducted to thematic saturation: patients with rheumatoid arthritis (RA) who had registry experience, patients with any chronic illness, and Spanish-speaking patients with RA or osteoarthritis. Patients in the latter 2 groups had no prior registry experience. Focus groups were audiotaped and transcribed. Four researchers independently analyzed transcripts using open data coding to identify themes. A normative group process was used to consolidate and refine themes. Seven major themes were identified, including personalization/convenience of data collection, trust and confidentiality, camaraderie, learning about yourself and your disease, altruism, material motivators, and capturing mental health and other elements of the lived experience. We observed distinct differences in the discussion content of the Spanish-speaking patients compared to the English-speaking patients. This study identified patient attitudes towards registry research among those with and without prior experience in a registry. The results provide insight into strategies for registry design to maximize patient engagement, which can lead to more robust registry data. © 2016, American College of Rheumatology.

Can rheumatoid arthritis (RA) registries provide contextual safety data for modern RA clinical trials? The case for mortality and cardiovascular disease.

PubMed

Michaud, Kaleb; Berglind, Niklas; Franzén, Stefan; Frisell, Thomas; Garwood, Christopher; Greenberg, Jeffrey D; Ho, Meilien; Holmqvist, Marie; Horne, Laura; Inoue, Eisuke; Nyberg, Fredrik; Pappas, Dimitrios A; Reed, George; Symmons, Deborah; Tanaka, Eiichi; Tran, Trung N; Verstappen, Suzanne M M; Wesby-van Swaay, Eveline; Yamanaka, Hisashi; Askling, Johan

2016-10-01

We implemented a novel method for providing contextual adverse event rates for a randomised controlled trial (RCT) programme through coordinated analyses of five RA registries, focusing here on cardiovascular disease (CVD) and mortality. Each participating registry (Consortium of Rheumatology Researchers of North America (CORRONA) (USA), Swedish Rheumatology Quality of Care Register (SRR) (Sweden), Norfolk Arthritis Register (NOAR) (UK), CORRONA International (East Europe, Latin America, India) and Institute of Rheumatology, Rheumatoid Arthritis (IORRA) (Japan)) defined a main cohort from January 2000 onwards. To address comparability and potential bias, we harmonised event definitions and defined several subcohorts for sensitivity analyses based on disease activity, treatment, calendar time, duration of follow-up and RCT exclusions. Rates were standardised for age, sex and, in one sensitivity analysis, also HAQ. The combined registry cohorts included 57 251 patients with RA (234 089 person-years)-24.5% men, mean (SD) baseline age 58.2 (13.8) and RA duration 8.2 (11.7) years. Standardised registry mortality rates (per 100 person-years) varied from 0.42 (CORRONA) to 0.80 (NOAR), with 0.60 for RCT patients. Myocardial infarction and major adverse cardiovascular events (MACE) rates ranged from 0.09 and 0.31 (IORRA) to 0.39 and 0.77 (SRR), with RCT rates intermediate (0.18 and 0.42), respectively. Additional subcohort analyses showed small and mostly consistent changes across registries, retaining reasonable consistency in rates across the Western registries. Additional standardisation for HAQ returned higher mortality and MACE registry rates. This coordinated approach to contextualising RA RCT safety data demonstrated reasonable differences and consistency in rates for mortality and CVD across registries, and comparable RCT rates, and may serve as a model method to supplement clinical trial analyses for drug development programmes. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Eye Care Professionals' Perspectives on Eye Donation and an Eye Donation Registry for Research: A Single-Institution, Cross-Sectional Study.

PubMed

Williams, Andrew M; Allingham, R Rand; Stamer, W Daniel; Muir, Kelly W

2016-06-01

A centralized eye donation registry for research could help to bridge the gap between patients interested in donating their eyes to science and scientists who conduct research on human eye tissue. Previous research has demonstrated patient and family support for such a registry. In this study, we assessed the views that eye care professionals have toward an eye donation registry for research. Surveys were distributed to all 46 clinical faculty members of the Duke University Eye Center. In addition to collecting demographic information, the surveys assessed clinicians' experience with discussing eye donation with patients, described the proposed eye donation registry for research and asked how the registry would affect the clinicians' practice. A total of 21 eye care professionals returned the survey. Thirty-three percent reported discussing eye donation with patients, and 43% reported that a patient has asked about donating their eyes for research on their disease. Eighty-six percent of eye care professionals reported that a centralized registry would improve the way they work with patients who express a desire to donate their eyes for research. The majority of eye care professionals at our academic institution indicated that an eye donation registry for research would improve how they work with patients who are interested in donating their eyes for research on their disease. Future research should examine how best to communicate this registry to ophthalmic patients.

Registry Assessment of Peripheral Interventional Devices (RAPID): Registry assessment of peripheral interventional devices core data elements.

PubMed

Jones, W Schuyler; Krucoff, Mitchell W; Morales, Pablo; Wilgus, Rebecca W; Heath, Anne H; Williams, Mary F; Tcheng, James E; Marinac-Dabic, J Danica; Malone, Misti L; Reed, Terrie L; Fukaya, Rie; Lookstein, Robert A; Handa, Nobuhiro; Aronow, Herbert D; Bertges, Daniel J; Jaff, Michael R; Tsai, Thomas T; Smale, Joshua A; Zaugg, Margo J; Thatcher, Robert J; Cronenwett, Jack L

2018-02-01

The current state of evaluating patients with peripheral artery disease and more specifically of evaluating medical devices used for peripheral vascular intervention (PVI) remains challenging because of the heterogeneity of the disease process, the multiple physician specialties that perform PVI, the multitude of devices available to treat peripheral artery disease, and the lack of consensus about the best treatment approaches. Because PVI core data elements are not standardized across clinical care, clinical trials, and registries, aggregation of data across different data sources and physician specialties is currently not feasible. Under the auspices of the U.S. Food and Drug Administration's Medical Device Epidemiology Network initiative-and its PASSION (Predictable and Sustainable Implementation of the National Registries) program, in conjunction with other efforts to align clinical data standards-the Registry Assessment of Peripheral Interventional Devices (RAPID) workgroup was convened. RAPID is a collaborative, multidisciplinary effort to develop a consensus lexicon and to promote interoperability across clinical care, clinical trials, and national and international registries of PVI. The current manuscript presents the initial work from RAPID to standardize clinical data elements and definitions, to establish a framework within electronic health records and health information technology procedural reporting systems, and to implement an informatics-based approach to promote the conduct of pragmatic clinical trials and registry efforts in PVI. Ultimately, we hope this work will facilitate and improve device evaluation and surveillance for patients, clinicians, health outcomes researchers, industry, policymakers, and regulators. Copyright © 2017 Society for Vascular Surgery. All rights reserved.

Development of a Web-Based Registry to Support Diabetes Care in Free Medical Clinics.

PubMed

McFadden, Norman; Daniel, Bryan; Hoyt, Robert; Snider, Dallas

2017-01-01

The United States has more than 1,000 free medical clinics. Because these clinics do not bill Medicare or Medicaid, they are not eligible for federal reimbursement for electronic health record (EHR) adoption. As a result, most do not have EHRs or electronic disease registries. A web-based diabetes registry was created with all open-source components for use in an urban free clinic to manage patients with type 2 diabetes and comorbidities. The registry was modeled after the Chronic Disease Electronic Management System and recommendations of the American Diabetes Association. The software was enhanced to include multiple other features, such as progress notes, so that it can function as a simple EHR. The configuration permits other free clinics to join securely, and the software can be shared.

Primary Healthcare-based Diabetes Registry in Puducherry: Design and Methods

PubMed Central

Lakshminarayanan, Subitha; Kar, Sitanshu Sekhar; Gupta, Rajeev; Xavier, Denis; Bhaskar Reddy, S. Vijaya

2017-01-01

Background: Diabetes registries monitor the population prevalence and incidence of diabetes, monitor diabetes control program, provide information of quality of care to health service providers, and provide a sampling frame for interventional studies. This study documents the process of establishing a prospective diabetes registry in a primary health-care setting in Puducherry. Methods: This is a facility-based prospective registry conducted in six randomly selected urban health centers in Puducherry, with enrollment of all known patients with diabetes attending chronic disease clinics. Administrative approvals were obtained from Government Health Services. Manuals for training of medical officers, health-care workers, and case report forms were developed. Diabetes registry was prepared using Epi Info software. Results: In the first phase, demographic characteristics, risk factors, complications, coexisting chronic conditions, lifestyle and medical management, and clinical outcomes were recorded. Around 2177 patients with diabetes have been registered in six Primary Health Centres out of a total of 2948 participants seeking care from chronic disease clinic. Registration coverage ranges from 61% to 105% in these centers. Conclusion: This study has documented methodological details, and learning experiences gained while developing a diabetes registry at the primary health care level and the scope for upscaling to a Management Information System for Diabetes and a State-wide Registry. Improvement in patient care through needs assessment and quality assurance in service delivery is an important theme envisioned by this registry. PMID:28553589

Measuring Adolescent Human Papillomavirus Vaccine Coverage: A Match of Sexually Transmitted Disease Clinic and Immunization Registry Data.

PubMed

Pathela, Preeti; Jamison, Kelly; Papadouka, Vikki; Kabir, Rezaul; Markowitz, Lauri E; Dunne, Eileen F; Schillinger, Julia A

2016-12-01

Human papillomavirus (HPV) vaccine is recommended for adolescents. By the end of 2013, 64% of female and 40% of male New York City residents aged 13-18 years had received ≥1 HPV vaccine dose. Adolescents attending sexually transmitted disease (STD) clinics are at high risk for HPV exposure and could benefit from vaccination. Our objective was to estimate HPV vaccination coverage for this population. We matched records of New York City's STD clinic patients aged 13-18 years during 2010-2013 with the Citywide Immunization Registry. We assessed HPV vaccine initiation (≥1 dose) and series completion (≥3 doses among those who initiated) as of clinic visit date and by patient demographics. We compared receipt of ≥1 dose for HPV, tetanus-diphtheria-acellular pertussis, and meningococcal conjugate vaccine. Eighty-two percent of clinic attendees (13,505/16,364) had records in the Citywide Immunization Registry. Receipt of ≥1 HPV dose increased during 2010-2013 (females: 57.6%-69.7%; males: 1.5%-36.3%). Among females, ≥1-dose coverage was lowest among whites (53.4%) and highest among Hispanics (73.3%); among males, ≥1-dose coverage was lowest among whites (6.9%) and highest among Asians (20.9%). Series completion averaged 57.7% (females) and 28.0% (males), with little variation by race/ethnicity or poverty level. Receipt of ≥1 dose was 59.7% for HPV, 82% for tetanus-diphtheria-acellular pertussis, and 76% for meningococcal conjugate vaccines. HPV vaccine initiation and completion were low among adolescent STD clinic patients; coverage was lower compared with other recommended vaccines. STD clinics may be good venues for delivering HPV vaccine, thereby enhancing efforts to improve HPV vaccination. Copyright © 2016 Society for Adolescent Health and Medicine. All rights reserved.

Optimization of Inflammatory Bowel Disease Cohort Studies in Asia.

PubMed

Leung, Wai K

2015-07-01

With the incidence of inflammatory bowel disease (IBD) increasing rapidly in many Asian countries, including Hong Kong, it is important that patient characteristics are better understood. For example, are the phenotypes, behaviors, complications, and even treatment responses found in Asian patients similar to those of their Western counterparts? To formally address these questions, a properly designed local cohort study is needed. Whilst IBD is still relatively uncommon in Asia, the establishment of a local IBD registry will significantly contribute to the answering of these questions. The Hong Kong IBD registry was established to fill the gap in the understanding of IBD patients, and to foster research into IBD in Hong Kong. The Hong Kong IBD registry is a territory-wide registry that includes all public hospitals in Hong Kong. We included all IBD patients who were currently receiving medical care at these hospitals. With the help of the central computer medical record system of the Hospital Authority of Hong Kong, all clinical events, medications usage, endoscopy records, and laboratory results of patients in the registry were captured. Apart from data collection, the registry is also establishing a bio-specimen bank of blood and stool samples of IBD patients for future research. The IBD registry is a very useful platform for population-based studies on IBD in Asia.

Registry of Hypogonadism in Men (RHYME): design of a multi-national longitudinal, observational registry of exogenous testosterone use in hypogonadal men.

PubMed

Rosen, Raymond C; Wu, Frederick C W; Behre, Hermann M; Roehrborn, Claus G; Schröder, Fritz H; Siami, Flora S; Martha, Julia F; Finn, Joseph D; Araujo, Andre B

2013-03-01

Despite the prevalence of hypogonadism (HG) and widespread use of testosterone therapy, little is known about the safety/effectiveness of long-term testosterone use. The Registry of Hypogonadism in Men (RHYME) is a multi-national patient registry assessing prostate health and other outcomes associated with testosterone treatment in men. Observational patient disease registry. RHYME is a non-interventional disease registry with longitudinal data collection on a large sample (N = 999) of well-characterized, hypogonadal men aged 18 years or older. The Registry will prospectively evaluate male patients diagnosed with HG, who have not previously been treated with testosterone therapy. Key design features include: (1) broad inclusion/exclusion criteria, (2) standardized central laboratory hormone assays, (3) independent adjudication of prostate biopsies and mortalities, (4) standard of care treatment, (5) comprehensive medical record and questionnaire data at six months and annually post-enrollment and (6) adequate statistical power for assessing prostate endpoints at 36 months. A total of 25 clinical sites in six European countries (Germany, Italy, the Netherlands, Spain, Sweden and the United Kingdom) have completed recruitment for the study. Recruitment was initiated in May 2009, and completed in December 2011. Data collection is ongoing with a minimum of two years of follow-up on all patients.

Design of the Familial Hypercholesterolaemia Australasia Network Registry: Creating Opportunities for Greater International Collaboration.

PubMed

Bellgard, Matthew I; Walker, Caroline E; Napier, Kathryn R; Lamont, Leanne; Hunter, Adam A; Render, Lee; Radochonski, Maciej; Pang, Jing; Pedrotti, Annette; Sullivan, David R; Kostner, Karam; Bishop, Warrick; George, Peter M; O'Brien, Richard C; Clifton, Peter M; Bockxmeer, Frank M Van; Nicholls, Stephen J; Hamilton-Craig, Ian; Dawkins, Hugh Js; Watts, Gerald F

2017-10-01

Familial Hypercholesterolemia (FH) is the most common and serious monogenic disorder of lipoprotein metabolism that leads to premature coronary heart disease. There are over 65,000 people estimated to have FH in Australia, but many remain undiagnosed. Patients with FH are often under-treated, but with early detection, cascade family testing and adequate treatment, patient outcomes can improve. Patient registries are key tools for providing new information on FH and enhancing care worldwide. The development and design of the FH Australasia Network Registry is a crucial component in the comprehensive model of care for FH, which aims to provide a standardized, high-quality and cost-effective system of care that is likely to have the highest impact on patient outcomes. Informed by stakeholder engagement, the FH Australasia Network Registry was collaboratively developed by government, patient and clinical networks and research groups. The open-source, web-based Rare Disease Registry Framework was the architecture chosen for this registry owing to its open-source standards, modular design, interoperability, scalability and security features; all these are key components required to meet the ever changing clinical demands across regions. This paper provides a high level blueprint for other countries and jurisdictions to help inform and map out the critical features of an FH registry to meet their particular health system needs.

Pulmonary Hypertension Care Center Network: Improving Care and Outcomes in Pulmonary Hypertension.

PubMed

Sahay, Sandeep; Melendres-Groves, Lana; Pawar, Leena; Cajigas, Hector R

2017-04-01

Pulmonary hypertension (PH) is a chronic, progressive, life-threatening disease that requires expert multidisciplinary care. To facilitate this level of care, the Pulmonary Hypertension Association established across the United States a network of pulmonary hypertension care centers (PHCCs) with special expertise in PH, particularly pulmonary arterial hypertension, to raise the overall quality of care and outcomes for patients with this life-threatening disease. Since the inception of PHCCs in September 2014, to date 35 centers have been accredited in the United States. This model of care brings together physicians and specialists from other disciplines to provide care, facilitate basic and clinical research, and educate the next generation of providers. PHCCs also offer additional opportunities for improvements in PH care. The patient registry offered through the PHCCs is an organized system by which data are collected to evaluate the outcomes of patients with PH. This registry helps in detecting variations in outcomes across centers, thus identifying opportunities for improvement. Multiple tactics were undertaken to implement the strategic plan, training, and tools throughout the PHCC network. In addition, strategies to foster collaboration between care center staff and individuals with PH and their families are the cornerstone of the PHCCs. The Pulmonary Vascular Network of the American College of Chest Physicians believes this to be a positive step that will improve the quality of care delivered in the United States to patients with PH. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Starting of biological disease modifying antirheumatic drugs may be postponed in rheumatoid arthritis patients with multimorbidity: Single center real life results.

PubMed

Armagan, Berkan; Sari, Alper; Erden, Abdulsamet; Kilic, Levent; Erdat, Efe Cem; Kilickap, Saadettin; Kiraz, Sedat; Bilgen, Sule Apras; Karadag, Omer; Akdogan, Ali; Ertenli, Ihsan; Kalyoncu, Umut

2018-03-01

The objective of this study was to assess the frequency of comorbidities and multimorbidities in rheumatoid arthritis (RA) patients under biologic therapy and their effects on biological disease modifying antirheumatic drugs (DMARDs) choice, timing, and response.Hacettepe University Biologic Registry (HUR-BIO) is single center biological DMARD registry. Cardiovascular, infectious, cancer, and other comorbidities were recorded with face to face interviews. Multimorbidity is defined as >1 comorbidity. Disease duration, initial date of biological DMARDs, initial and overall biological DMARD choice were recorded. Disease activity score-28 (DAS-28) responses were compared to comorbidity presence and multimorbidity.Total of 998 RA patients were enrolled into the study. The mean age was 53.1 (12.5) and mean disease duration (standard deviation [SD]) was 11.7 (7.5) years. At least 1 comorbidity was detected in 689 (69.1%) patients, 375 (37.9%) patients had multimorbidity. Patients had mean 1.36 ± 1.32 comorbidity. The median durations of first biological DMARDs prescription were 60 (3-552) months after RA diagnosis. For multimorbidity patients, the median first biological prescription duration was longer than the duration for patients without multimorbidity (72 [3-552] vs 60 [3-396] months, P < .001). The physicians prescribe tumor necrosis factor inhibitor (TNFi) biological drugs less frequently than other biological DMARDs in patients with at least 1 comorbidity (66.2% vs 74.5%, P = .007) or multimorbidity (34.6% vs 43.5%, P = .006). Patients with comorbidities and multimorbidity achieved DAS-28 remission less frequently than patients without comorbidity (31.6% vs 42.6%, P = .012 and 27.2% vs 39.7%, P = .001, respectively).In real life, physicians may postpone to prescribe biological DMARDs and less frequently choose TNFi biological drugs in patients with multimorbidity. Furthermore, comorbidity may have a negative effect on the treatment response.

Starting of biological disease modifying antirheumatic drugs may be postponed in rheumatoid arthritis patients with multimorbidity

PubMed Central

Armagan, Berkan; Sari, Alper; Erden, Abdulsamet; Kilic, Levent; Erdat, Efe Cem; Kilickap, Saadettin; Kiraz, Sedat; Bilgen, Sule Apras; Karadag, Omer; Akdogan, Ali; Ertenli, Ihsan; Kalyoncu, Umut

2018-01-01

Abstract The objective of this study was to assess the frequency of comorbidities and multimorbidities in rheumatoid arthritis (RA) patients under biologic therapy and their effects on biological disease modifying antirheumatic drugs (DMARDs) choice, timing, and response. Hacettepe University Biologic Registry (HUR-BIO) is single center biological DMARD registry. Cardiovascular, infectious, cancer, and other comorbidities were recorded with face to face interviews. Multimorbidity is defined as >1 comorbidity. Disease duration, initial date of biological DMARDs, initial and overall biological DMARD choice were recorded. Disease activity score-28 (DAS-28) responses were compared to comorbidity presence and multimorbidity. Total of 998 RA patients were enrolled into the study. The mean age was 53.1 (12.5) and mean disease duration (standard deviation [SD]) was 11.7 (7.5) years. At least 1 comorbidity was detected in 689 (69.1%) patients, 375 (37.9%) patients had multimorbidity. Patients had mean 1.36 ± 1.32 comorbidity. The median durations of first biological DMARDs prescription were 60 (3–552) months after RA diagnosis. For multimorbidity patients, the median first biological prescription duration was longer than the duration for patients without multimorbidity (72 [3–552] vs 60 [3–396] months, P < .001). The physicians prescribe tumor necrosis factor inhibitor (TNFi) biological drugs less frequently than other biological DMARDs in patients with at least 1 comorbidity (66.2% vs 74.5%, P = .007) or multimorbidity (34.6% vs 43.5%, P = .006). Patients with comorbidities and multimorbidity achieved DAS-28 remission less frequently than patients without comorbidity (31.6% vs 42.6%, P = .012 and 27.2% vs 39.7%, P = .001, respectively). In real life, physicians may postpone to prescribe biological DMARDs and less frequently choose TNFi biological drugs in patients with multimorbidity. Furthermore, comorbidity may have a negative effect on the treatment response. PMID:29595700

Using Registries to Identify Adverse Events in Rheumatic Diseases

PubMed Central

Lionetti, Geraldina; Kimura, Yukiko; Schanberg, Laura E.; Beukelman, Timothy; Wallace, Carol A.; Ilowite, Norman T.; Winsor, Jane; Fox, Kathleen; Natter, Marc; Sundy, John S.; Brodsky, Eric; Curtis, Jeffrey R.; Del Gaizo, Vincent; Iyasu, Solomon; Jahreis, Angelika; Meeker-O’Connell, Ann; Mittleman, Barbara B.; Murphy, Bernard M.; Peterson, Eric D.; Raymond, Sandra C.; Setoguchi, Soko; Siegel, Jeffrey N.; Sobel, Rachel E.; Solomon, Daniel; Southwood, Taunton R.; Vesely, Richard; White, Patience H.; Wulffraat, Nico M.; Sandborg, Christy I.

2013-01-01

The proven effectiveness of biologics and other immunomodulatory products in inflammatory rheumatic diseases has resulted in their widespread use as well as reports of potential short- and long-term complications such as infection and malignancy. These complications are especially worrisome in children who often have serial exposures to multiple immunomodulatory products. Post-marketing surveillance of immunomodulatory products in juvenile idiopathic arthritis (JIA) and pediatric systemic lupus erythematosus is currently based on product-specific registries and passive surveillance, which may not accurately reflect the safety risks for children owing to low numbers, poor long-term retention, and inadequate comparators. In collaboration with the US Food and Drug Administration (FDA), patient and family advocacy groups, biopharmaceutical industry representatives and other stakeholders, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) and the Duke Clinical Research Institute (DCRI) have developed a novel pharmacosurveillance model (CARRA Consolidated Safety Registry [CoRe]) based on a multicenter longitudinal pediatric rheumatic diseases registry with over 8000 participants. The existing CARRA infrastructure provides access to much larger numbers of subjects than is feasible in single-product registries. Enrollment regardless of medication exposure allows more accurate detection and evaluation of safety signals. Flexibility built into the model allows the addition of specific data elements and safety outcomes, and designation of appropriate disease comparator groups relevant to each product, fulfilling post-marketing requirements and commitments. The proposed model can be applied to other pediatric and adult diseases, potentially transforming the paradigm of pharmacosurveillance in response to the growing public mandate for rigorous post-marketing safety monitoring. PMID:24144710

Validation of diagnosis of aplastic anaemia in La Rioja (Spain) by International Classification of Diseases codes for case ascertainment for the Spanish National Rare Diseases Registry.

PubMed

Ruiz, Elena; Ramalle-Gómara, Enrique; Quiñones, Carmen; Rabasa, Pilar; Pisón, Carlos

2015-05-01

To analyse the validity of diagnosis of aplastic anaemia (AA) by International Classification of Diseases codes in hospital discharge data (MBDS) and the mortality registry (MR) of La Rioja to detect cases to be included in the Spanish National Rare Diseases Registry. International Classification of Diseases (ICD) codes were used to detect AA cases during the period 2007-2012 from two administrative databases: the MBDS and the MR of La Rioja (Spain). Medical records of population selected by merging both databases were used to confirm true AA cases. The annual mean incidence rate of AA was calculated using confirmed incident cases. By merging both databases, 62 hypothetical AA incident patients were detected during the period 2007-2012. The medical records of the 89% of them could be revised, and they confirmed that only the 15% of the patients actually suffered AA. The annual mean AA incidence in La Rioja was 4.17 per million inhabitants (6.23 per million, males; 2.10 per million, females). The MBDS and the MR are not in themselves sufficient to ascertain AA cases in La Rioja and medical records should be reviewed to confirm true AA cases to be included in the Spanish National Rare Diseases Registry. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Japan Diabetic Nephropathy Cohort Study: study design, methods, and implementation.

PubMed

Furuichi, Kengo; Shimizu, Miho; Toyama, Tadashi; Koya, Daisuke; Koshino, Yoshitaka; Abe, Hideharu; Mori, Kiyoshi; Satoh, Hiroaki; Imanishi, Masahito; Iwano, Masayuki; Yamauchi, Hiroyuki; Kusano, Eiji; Fujimoto, Shouichi; Suzuki, Yoshiki; Okuda, Seiya; Kitagawa, Kiyoki; Iwata, Yasunori; Kaneko, Shuichi; Nishi, Shinichi; Yokoyama, Hitoshi; Ueda, Yoshihiko; Haneda, Masakazu; Makino, Hirofumi; Wada, Takashi

2013-12-01

Diabetic nephropathy, leading to end-stage renal disease, has a considerable impact on public health and the social economy. However, there are few national registries of diabetic nephropathy in Japan. The aims of this prospective cohort study are to obtain clinical data and urine samples for revising the clinical staging of diabetic nephropathy, and developing new diagnostic markers for early diabetic nephropathy. The Japanese Society of Nephrology established a nationwide, web-based, and prospective registry system. On the system, there are two basic registries; the Japan Renal Biopsy Registry (JRBR), and the Japan Kidney Disease Registry (JKDR). In addition to the two basic registries, we established a new prospective registry to the system; the Japan Diabetic Nephropathy Cohort Study (JDNCS), which collected physical and laboratory data. We analyzed the data of 321 participants (106 female, 215 male; average age 65 years) in the JDNCS. Systolic and diastolic blood pressure was 130.1 and 72.3 mmHg, respectively. Median estimated glomerular filtration rate (eGFR) was 33.3 ml/min/1.73 m(2). Proteinuria was 1.8 g/gCr, and serum levels of albumin were 3.6 g/dl. The majority of the JDNCS patients presented with preserved eGFR and low albuminuria or low eGFR and advanced proteinuria. In the JRBR and JKDR registries, 484 and 125 participants, respectively, were enrolled as having diabetes mellitus. In comparison with the JRBR and JKDR registries, the JDNCS was characterized by diabetic patients presenting with low proteinuria with moderately preserved eGFR. There are few national registries of diabetic nephropathy to evaluate prognosis in Japan. Future analysis of the JDNCS will provide clinical insights into the epidemiology and renal and cardiovascular outcomes of type 2 diabetic patients in Japan.

The ASSURE ROT Registry: Bioresorbable Vascular Scaffold Following Rotablation for Complex Coronary Lesions

ClinicalTrials.gov

2015-02-05

Cardiovascular Diseases; Coronary Artery Disease; Myocardial Ischemia; Coronary Disease; Coronary Restenosis; Heart Diseases; Coronary Stenosis; Arteriosclerosis; Arterial Occlusive Diseases; Vascular Diseases

Characteristics of national registries for occupational diseases: international development and validation of an audit tool (ODIT).

PubMed

Spreeuwers, Dick; de Boer, Angela G E M; Verbeek, Jos H A M; van Dijk, Frank J H

2009-10-23

The aim of the study was to develop quality indicators that can be used for quality assessment of registries of occupational diseases in relation to preventive policy on a national level. The research questions were: 1. Which indicators determine the quality of national registries of occupational diseases with respect to their ability to provide appropriate information for preventive policy? 2. What are the criteria that can distinguish low quality from high quality? First, we performed a literature search to assess which output of registries can be considered appropriate for preventive policy and to develop a set of preliminary indicators and criteria. Second, final indicators and criteria were assessed and their content validity was tested in a Delphi study, for which experts from the 25 EU Member States were invited. The literature search revealed two different types of information output to be appropriate for preventive policy: monitor and alert information. For the evaluation of the quality of the monitor and alert function we developed ten indicators and criteria. Sixteen of the twenty-five experts responded in the first round of the Delphi study, and eleven in the second round. Based on their comments, we assessed the final nine indicators: the completeness of the notification form, coverage of registration, guidelines or criteria for notification, education and training of reporting physicians, completeness of registration, statistical methods used, investigation of special cases, presentation of monitor information, and presentation of alert information. Except for the indicator "coverage of registration" for the alert function, all the indicators met the preset requirements of content validity. We have developed quality indicators and criteria to evaluate registries for occupational diseases on the ability to provide appropriate information for preventive policy on a national level. Together, these indicators form a tool which can be used for quality improvement of registries of occupational diseases.

75 FR 52535 - Availability of Draft Toxicological Profile

Federal Register 2010, 2011, 2012, 2013, 2014

2010-08-26

... toxicological profile on unregulated hazardous substances that was prepared for the Department of Defense (DOD... DEPARTMENT OF HEALTH AND HUMAN SERVICES Agency for Toxic Substances and Disease Registry [ATSDR... Registry (ATSDR), [[Page 52536

Development of a Web-Based Registry to Support Diabetes Care in Free Medical Clinics

PubMed Central

McFadden, Norman; Daniel, Bryan; Hoyt, Robert; Snider, Dallas

2017-01-01

The United States has more than 1,000 free medical clinics. Because these clinics do not bill Medicare or Medicaid, they are not eligible for federal reimbursement for electronic health record (EHR) adoption. As a result, most do not have EHRs or electronic disease registries. A web-based diabetes registry was created with all open-source components for use in an urban free clinic to manage patients with type 2 diabetes and comorbidities. The registry was modeled after the Chronic Disease Electronic Management System and recommendations of the American Diabetes Association. The software was enhanced to include multiple other features, such as progress notes, so that it can function as a simple EHR. The configuration permits other free clinics to join securely, and the software can be shared. PMID:28566990

United Kingdom Carotid Artery Stent Registry: Short- and Long-Term Outcomes

DOE Office of Scientific and Technical Information (OSTI.GOV)

Goode, S. D., E-mail: s.goode@sheffield.ac.uk; Cleveland, T. J.; Gaines, P. A.

Background: Carotid artery stenting (CAS) has evolved to treat carotid artery disease with the intention of prevent stroke. The British Society of Interventional Radiologists developed a voluntary registry to monitor the practice of this novel procedure. We present the data from the United Kingdom (UK) CAS registry for short and long-term outcomes for symptomatic and asymptomatic carotid disease. Methods: The UK CAS registry collected data from 1998 to 2010 from 31 hospitals across the UK for 1,154 patients. All interventions were enrolled in the registry for both asymptomatic and symptomatic patients. Initial entry forms were completed for each patient enteredmore » with data including indications, demographic data, CAS data (including stents and protection device details) and 30-day outcomes. Complications were documented. Follow-up data were collected at yearly intervals. Results: Nine hundred fifty-three (83 %) symptomatic and 201 (17 %) asymptomatic patients were enrolled into the registry. The 30-day all stroke and death rates for symptomatic patients were 5.5 and 2.2 % for those with asymptomatic disease. The 30-day mortality rate was 1.7 % for symptomatic and 0.6 % for asymptomatic patients. For symptomatic patients undergoing CAS, the 7-year all-cause mortality rate was 22.2 % and for asymptomatic patients 18.1 %. The 7-year all-cause mortality and disabling stroke rates were 25.3 and 19.4 %, respectively. Conclusion: These data indicate that outside of the tight constraints of a randomised trial, CAS provides effective prophylaxis against stroke and death.« less

Evaluation of participant recruitment methods to a rare disease online registry.

PubMed

Johnson, Kimberly J; Mueller, Nancy L; Williams, Katherine; Gutmann, David H

2014-07-01

Internet communication advances provide new opportunities to assemble individuals with rare diseases to online patient registries from wide geographic areas for research. However, there is little published information on the efficacy of different recruitment methods. Here we describe recruitment patterns and the characteristics of individuals with the self-identified autosomal dominant genetic disorder neurofibromatosis type 1 (NF1) who participated in an online patient registry during the 1-year period from 1/1/2012 to 12/31/2012. We employed four main mechanisms to alert potential participants to the registry: (1) Facebook and Google advertising, (2) government and academic websites, (3) patient advocacy groups, and (4) healthcare providers. Participants reported how they first heard about the registry through an online questionnaire. During the 1-year period, 880 individuals participated in the registry from all 50 U.S. States, the District of Columbia, Puerto Rico, and 39 countries. Facebook and Google were reported as referral sources by the highest number of participants (n=550, 72% Facebook), followed by healthcare providers (n=74), and government and academic websites (n=71). The mean participant age was 29±18 years and most participants reported White race (73%) and female sex (62%) irrespective of reported referral source. Internet advertising, especially through Facebook, resulted in efficient enrollment of large numbers of individuals with NF1. Our study demonstrates the potential utility of this approach to assemble individuals with a rare disease from across the world for research studies. © 2014 Wiley Periodicals, Inc.

The need for a disease-specific prospective pregnancy registry for multiple sclerosis (MS).

PubMed

Alwan, Sura; Chambers, Christina D; Armenti, Vincent T; Sadovnick, A Dessa

2015-01-01

Multiple sclerosis (MS) is the most commonly acquired neurological disorder affecting young adults of reproductive age with an approximately 3:1 female to male ratio. Although pregnancy is not contraindicated in MS, data are limited regarding pregnancy outcome among MS patients, and the safety or risk to the fetus associated with most maternal MS treatments, such as disease modifying therapies (DMTs), during pregnancy is unknown. We review available epidemiological and registry data on MS and pregnancy and discuss the need to initiate a North American Multiple Sclerosis Pregnancy Registry that will prospectively identify pregnancies in women with MS, obtain information on the disease, and its treatment during gestation and lactation and follow the children to determine their health status. Copyright © 2014 Elsevier B.V. All rights reserved.

Integrated image data and medical record management for rare disease registries. A general framework and its instantiation to theGerman Calciphylaxis Registry.

PubMed

Deserno, Thomas M; Haak, Daniel; Brandenburg, Vincent; Deserno, Verena; Classen, Christoph; Specht, Paula

2014-12-01

Especially for investigator-initiated research at universities and academic institutions, Internet-based rare disease registries (RDR) are required that integrate electronic data capture (EDC) with automatic image analysis or manual image annotation. We propose a modular framework merging alpha-numerical and binary data capture. In concordance with the Office of Rare Diseases Research recommendations, a requirement analysis was performed based on several RDR databases currently hosted at Uniklinik RWTH Aachen, Germany. With respect to the study management tool that is already successfully operating at the Clinical Trial Center Aachen, the Google Web Toolkit was chosen with Hibernate and Gilead connecting a MySQL database management system. Image and signal data integration and processing is supported by Apache Commons FileUpload-Library and ImageJ-based Java code, respectively. As a proof of concept, the framework is instantiated to the German Calciphylaxis Registry. The framework is composed of five mandatory core modules: (1) Data Core, (2) EDC, (3) Access Control, (4) Audit Trail, and (5) Terminology as well as six optional modules: (6) Binary Large Object (BLOB), (7) BLOB Analysis, (8) Standard Operation Procedure, (9) Communication, (10) Pseudonymization, and (11) Biorepository. Modules 1-7 are implemented in the German Calciphylaxis Registry. The proposed RDR framework is easily instantiated and directly integrates image management and analysis. As open source software, it may assist improved data collection and analysis of rare diseases in near future.

Health risk communication at the Agency for Toxic Substances and Disease Registry

DOE Office of Scientific and Technical Information (OSTI.GOV)

Johnson, B.L.

The purpose of this paper is to describe efforts related to risk communication at the Agency for Toxic Substances and Disease Registry (ATSDR). The agency was created by Congress to fulfill health-related responsibilities specified in Superfund. Four areas are described for which ATSDR has responsibility under Superfund in the context of how each area relates to risk communication. These include health assessments, toxicological profiles, medical education and consultation, and exposure registries. The discussion is structured around four elements: a description of the activity, the message that is being communicated, the target audience, and how the risk is communicated to thismore » group.« less

Malignancy in Pediatric-onset Systemic Lupus Erythematosus.

PubMed

Bernatsky, Sasha; Clarke, Ann E; Zahedi Niaki, Omid; Labrecque, Jeremy; Schanberg, Laura E; Silverman, Earl D; Hayward, Kristen; Imundo, Lisa; Brunner, Hermine I; Haines, Kathleen A; Cron, Randy Q; Oen, Kiem; Wagner-Weiner, Linda; Rosenberg, Alan M; O'Neil, Kathleen M; Duffy, Ciarán M; von Scheven, Emily; Joseph, Lawrence; Lee, Jennifer L; Ramsey-Goldman, Rosalind

2017-10-01

To determine cancer incidence in a large pediatric-onset systemic lupus erythematosus (SLE) population. Data were examined from 12 pediatric SLE registries in North America. Patients were linked to their regional cancer registries to detect cancers observed after cohort entry, defined as date first seen in the clinic. The expected number of malignancies was obtained by multiplying the person-years in the cohort (defined from cohort entry to end of followup) by the geographically matched age-, sex-, and calendar year-specific cancer rates. The standardized incidence ratio (SIR; ratio of cancers observed to expected) was generated, with 95% CI. A total of 1168 patients were identified from the registries. The mean age at cohort entry was 13 years (SD 3.3), and 83.7% of the subjects were female. The mean duration of followup was 7.6 years, resulting in a total observation period of 8839 years spanning the calendar period 1974-2009. During followup, fourteen invasive cancers occurred (1.6 cancers per 1000 person-yrs, SIR 4.13, 95% CI 2.26-6.93). Three of these were hematologic (all lymphomas), resulting in an SIR for hematologic cancers of 4.68 (95% CI 0.96-13.67). SIR were increased for both male and female patients, and across age groups. Although cancer remains a relatively rare outcome in pediatric-onset SLE, our data do suggest an increase in cancer for patients followed an average of 7.6 years. About one-fifth of the cancers were hematologic. Longer followup, and study of drug effects and disease activity, is warranted.

THC:CBD spray and MS spasticity symptoms: data from latest studies.

PubMed

Rekand, Tiina

2014-01-01

New clinical experience with 9-delta-tetrahydocannabinol (THC) and cannabidiol (CBD) oromucosal spray (Sativex®) involving more than an additional 1,000 patients with MS spasticity (approximately 150 in clinical studies and 900 in post-marketing surveillance studies) have become available in 2013 and are reviewed. A randomized, placebo controlled long-term follow-up clinical trial with THC:CBD spray versus placebo demonstrated that it was not associated with cognitive decline, depression or significant mood changes after 12 months of treatment. Furthermore, in a prospective observational pilot study involving 33 patients (60% female) aged 33-68 years and a mean disease duration of 6.6 years, THC:CBD oromucosal spray did not adversely influence standard driving ability in patients with moderate to severe MS spasticity. Other new long term observational data about the use of THC:CBD oromucosal spray in clinical practice are available from patient registries in the UK, Germany and Spain. Findings to date reinforce the efficacy and safety observed in Phase III clinical trials. It is of interest that in practice average dosages used by patients tended to be lower than those reported in clinical studies (5-6.4 vs. >8 sprays/day), and effectiveness was maintained in the majority of patients. Importantly, no additional safety concerns were identified in the registry studies which included findings from patients who have been treated for prolonged periods (in the German/UK registry 45% of patients had >2 years exposure). Thus, these new data support a positive benefit-risk relationship for THC:CBD oromucosal spray during longer-term use. © 2014 S. Karger AG, Basel.

Dispelling myths about rare disease registry system development

PubMed Central

2013-01-01

Rare disease registries (RDRs) are an essential tool to improve knowledge and monitor interventions for rare diseases. If designed appropriately, patient and disease related information captured within them can become the cornerstone for effective diagnosis and new therapies. Surprisingly however, registries possess a diverse range of functionality, operate in different, often-times incompatible, software environments and serve various, and sometimes incongruous, purposes. Given the ambitious goals of the International Rare Diseases Research Consortium (IRDiRC) by 2020 and beyond, RDRs must be designed with the agility to evolve and efficiently interoperate in an ever changing rare disease landscape, as well as to cater for rapid changes in Information Communication Technologies. In this paper, we contend that RDR requirements will also evolve in response to a number of factors such as changing disease definitions and diagnostic criteria, the requirement to integrate patient/disease information from advances in either biotechnology and/or phenotypying approaches, as well as the need to adapt dynamically to security and privacy concerns. We dispel a number of myths in RDR development, outline key criteria for robust and sustainable RDR implementation and introduce the concept of a RDR Checklist to guide future RDR development. PMID:24131574

IL-6 blockade in systemic juvenile idiopathic arthritis - achievement of inactive disease and remission (data from the German AID-registry).

PubMed

Bielak, M; Husmann, E; Weyandt, N; Haas, J-P; Hügle, B; Horneff, G; Neudorf, U; Lutz, T; Lilienthal, E; Kallinich, T; Tenbrock, K; Berendes, R; Niehues, T; Wittkowski, H; Weißbarth-Riedel, E; Heubner, G; Oommen, P; Klotsche, J; Foell, Dirk; Lainka, E

2018-04-05

Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in the pathogenesis has been ascribed to proinflammatory cytokines like interleukin-6 (IL-6), and effective drugs inhibiting their signaling are being developed. This study evaluates sJIA patients treated with the IL-6 inhibitor tocilizumab (TCZ) concerning clinical response rate, disease course and adverse effects in a real-life clinical setting. In 2009 a clinical and research consortium was established, including an online registry for autoinflammatory diseases (AID) ( https://aid-register.de ). Data for this retrospective TCZ study were documented by 13 centers. From 7/2009 to 4/2014, 200 patients with sJIA were recorded in the AID-registry. Out of these, 46 (19 m, 27 f, age 1-18 years) received therapy with TCZ. Long term treatment (median 23 months) has been documented in 24/46 patients who were evaluated according to Wallace criteria (active disease 6/24, inactive disease 5/24, remission 13/24 cases). Under observation co-medication were used in 40/46 cases. Adverse events were reported in 11/46 patients. The clinical response rate (no clinical manifestation, no increased inflammation parameters) within the first 12 weeks of treatment was calculated to be 35%. Out of 200 sJIA children reported in the German AID-registry, 46 were treated with TCZ, showing a clinical response rate of 35% during the first 12 weeks, and inactive disease and/or remission under medication in 75% after one year. Adverse events were seen in 24% and severe adverse events in 4%. The AID-Registry is funded by the BMBF (01GM08104, 01GM1112D, 01GM1512D).

ABSORB: Postmarketing Surveillance Registry to Monitor the Everolimus-eluting Bioresorbable Vascular Scaffold in Patients With Coronary Artery Disease

ClinicalTrials.gov

2016-12-08

Cardiovascular Diseases; Coronary Artery Disease; Myocardial Ischemia; Coronary Disease; Coronary Restenosis; Heart Diseases; Coronary Stenosis; Arteriosclerosis; Arterial Occlusive Diseases; Vascular Diseases

48 CFR 752.247-70 - Preference for privately owned U.S.-flag commercial vessels.

Code of Federal Regulations, 2010 CFR

2010-10-01

... (computed separately for dry bulk carriers, dry cargo liners, and tankers) shall be transported in privately... registry. (iv) Date of loading. (v) Port of loading. (vi) Port of final discharge. (vii) Description of...

The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporary management of adult patients with cardiomyopathies.

PubMed

Charron, Philippe; Elliott, Perry M; Gimeno, Juan R; Caforio, Alida L P; Kaski, Juan Pablo; Tavazzi, Luigi; Tendera, Michal; Maupain, Carole; Laroche, Cécile; Rubis, Pawel; Jurcut, Ruxandra; Calò, Leonardo; Heliö, Tiina M; Sinagra, Gianfranco; Zdravkovic, Marija; Kavoliuniene, Aušra; Felix, Stephan B; Grzybowski, Jacek; Losi, Maria-Angela; Asselbergs, Folkert W; García-Pinilla, José Manuel; Salazar-Mendiguchia, Joel; Mizia-Stec, Katarzyna; Maggioni, Aldo P

2018-05-21

The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001). By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe.

Development and Implementation of Team-Based Panel Management Tools: Filling the Gap between Patient and Population Information Systems.

PubMed

Watts, Brook; Lawrence, Renée H; Drawz, Paul; Carter, Cameron; Shumaker, Amy Hirsch; Kern, Elizabeth F

2016-08-01

Effective team-based models of care, such as the Patient-Centered Medical Home, require electronic tools to support proactive population management strategies that emphasize care coordination and quality improvement. Despite the spread of electronic health records (EHRs) and vendors marketing population health tools, clinical practices still may lack the ability to have: (1) local control over types of data collected/reports generated, (2) timely data (eg, up-to-date data, not several months old), and accordingly (3) the ability to efficiently monitor and improve patient outcomes. This article describes a quality improvement project at the hospital system level to develop and implement a flexible panel management (PM) tool to improve care of subpopulations of patients (eg, panels of patients with diabetes) by clinical teams. An in-depth case analysis approach is used to explore barriers and facilitators in building a PM registry tool for team-based management needs using standard data elements (eg, laboratory values, pharmacy records) found in EHRs. Also described are factors that may contribute to sustainability; to date the tool has been adapted to 6 disease-focused subpopulations encompassing more than 200,000 patients. Two key lessons emerged from this initiative: (1) though challenging, team-based clinical end users and information technology needed to work together consistently to refine the product, and (2) locally developed population management tools can provide efficient data tracking for frontline clinical teams and leadership. The preliminary work identified critical gaps that were successfully addressed by building local PM registry tools from EHR-derived data and offers lessons learned for others engaged in similar work. (Population Health Management 2016;19:232-239).

Temporal changes in myocardial infarction incidence rates are associated with periods of perceived psychosocial stress: A SWEDEHEART national registry study.

PubMed

Wallert, John; Held, Claes; Madison, Guy; Olsson, Erik Mg

2017-09-01

Psychosocial stress might trigger myocardial infarction (MI). Increased MI incidence coincides with recurrent time periods during the year perceived as particularly stressful in the population. A stress-triggering hypothesis on the risk of MI onset was investigated with Swedish population data on MI hospital admission date and symptom onset date (N=156,690; 148,176) as registered from 2006 through 2013 in the national quality registry database Swedish Web-system for Enhancement and Development of Evidence-based care in Heart disease Evaluated According to Recommended Therapies (SWEDEHEART). Poisson regression was applied to analyze daily MI rates during days belonging to the Christmas and New Year holidays, turns of the month, Mondays, weekends, and summer vacation in July compared with remaining control days. Adjusted incidence rate ratios (IRRs) for MI rates were higher during Christmas and New Year holidays (IRR=1.07 [1.04-1.09], P<.001) and on Mondays (IRR=1.11 [1.09-1.13], P<.001) and lower in July (IRR=0.92 [0.90-0.94], P<.001) and over weekends (IRR=0.88 [0.87-0.89], P<.001), yet not during the turns of the month (IRR=1.01 [1.00-1.02], P=.891). These findings were also predominantly robust with symptom onset as alternative outcome, when adjusting for both established and some suggested-but-untested confounders, and in 8 subgroups. Fluctuations in daily MI incidence rates are systematically related to time periods of presumed psychosocial stress. Further research might clarify mechanisms that are amenable to clinical alteration. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Assessment of immunization registry databases as supplemental sources of data to improve ascertainment of vaccination coverage estimates in the national immunization survey.

PubMed

Khare, Meena; Piccinino, Linda; Barker, Lawrence E; Linkins, Robert W

2006-08-01

To evaluate the use of immunization registry data to supplement missing or incomplete vaccination data reported by immunization providers (referred to as "providers" hereafter) in the National Immunization Survey. Cross-sectional, random-digit-dialing, telephone survey to measure vaccination coverage among children aged 19 to 35 months in the United States. Four sites with mature (with >67% of provider participation in the area) immunization registries. Of the 639 children with complete household interviews, interviewers had consent from the respondents for 569 (89.0%) children to contact their providers and for 556 (87.0%) children to contact both providers and registries. Percentages of children up-to-date for vaccines based on data from providers, registries, and both sources combined. According to provider-reported data, weighted estimates of coverage for the recommended childhood vaccine series 4:3:1:3 at the 4 sites were 65.6%, 78.8%, 81.6%, and 77.0%. According to registry data, these coverage rates were consistently lower: 31.7% (P<.05), 65.4%, 71.9%, and 61.8%, respectively. When all unique vaccine doses were combined from both sources, the pooled 4:3:1:3 coverage rates increased to 72.0%, 92.0%, 88.7%, and 80.2%, respectively. The quality and completeness of vaccination histories from the registries were inconsistent and varied by sites. Vaccination coverage estimates were the lowest when only registry-reported data were used and were the highest when provider- and registry-reported histories were combined. Although registries enrolled and matched more children, vaccination histories were missing, incomplete, and inconsistent. The quality and completeness of the registry data must be improved and must be comparable across all states before further consideration may be given to supplement or replace the provider-reported National Immunization Survey data.

American College of Cardiology (ACC)'s PINNACLE India Quality Improvement Program (PIQIP)-Inception, progress and future direction: A report from the PIQIP Investigators.

PubMed

Kalra, Ankur; Glusenkamp, Nathan; Anderson, Karen; Kalra, Ram N; Kerkar, Prafulla G; Kumar, Ganesh; Maddox, Thomas M; Oetgen, William J; Virani, Salim S

2016-12-01

Cardiovascular diseases have surpassed infectious disorders to become the leading cause of morbidity and mortality in India. 1 A national-level registry comprehensively documenting the current-day prevalence of cardiovascular risk factors and disease burden among patients seeking care in the outpatient setting in India is currently non-existent. With a burgeoning urban population, the cardiovascular disease burden in India is set to skyrocket, with an estimated 18 million productive years of life lost by 2030. 2 While there are limited quality improvement registries in India, for example, the Kerala acute coronary syndrome and Trivandrum heart failure registries, their focus is on in-patient care quality improvement, while the vast majority of patients with cardiovascular diseases worldwide, including India, interact with the health care system in the outpatient setting. 3,4 Recognizing this unmet need, the American College of Cardiology partnered with local stakeholders in India to establish India's first outpatient cardiovascular disease performance measurement initiative in 2011, the PINNACLE (Practice Innovation and Clinical Excellence) India Quality Improvement Program (PIQIP). 5 This manuscript discusses the inception of the PIQIP registry, the progress it has made and challenges thus far, and its future direction and the promise it holds for cardiovascular care quality improvement in India. Copyright © 2016. Published by Elsevier B.V.

Norrie disease: extraocular clinical manifestations in 56 patients.

PubMed

Smith, Sharon E; Mullen, Thomas E; Graham, Dionne; Sims, Katherine B; Rehm, Heidi L

2012-08-01

Norrie disease (ND) is an X-linked recessive disorder characterized by congenital blindness, progressive sensorineural hearing loss and cognitive impairment. The ocular phenotype has been well described, while the extraocular manifestations of the disorder are not well understood. We present the data from the Norrie Disease Registry, which consists of 56 patients with detailed clinical histories and genotype data. This study represents the largest, detailed investigation into the phenotypic spectrum of ND to date and more importantly expands knowledge of the extraocular clinical manifestations. We identify several novel aspects of the syndrome that will improve the management of these patients. In particular, we expand our understanding of the neurologic manifestations in ND and identify a chronic seizure disorder in approximately 10% of all patients. In addition, details of the hearing phenotype are described including the median age of onset (12 years of age) and how genotype affects onset. Moreover, we find vascular disease to be a significant component of ND; and vascular health should be, in the future, a component of patient clinical care. In summary, the results expand our understanding of the phenotypic variability and genotypic heterogeneity in ND patients. Copyright © 2012 Wiley Periodicals, Inc.

Web resources for rare auto-inflammatory diseases: towards a common patient registry.

PubMed

Touitou, Isabelle; Hentgen, Véronique; Koné-Paut, Isabelle

2009-06-01

To review information resources on rare auto-inflammatory disorders (AIDs) for use by health care professionals, focusing particularly on patient registries. Using relevant key words, we surveyed the websites of several scientific societies of immunology, paediatrics and rheumatology, as well as Pubmed and specialized databases for AIDs. The Internet provides a wide variety of information related to AIDs. Moreover, several other initiatives have been undertaken to create new resources for professionals. We reviewed six patient registries for rare AIDs, taking a special interest in the submission questionnaire. We revealed a wide overlap between the items used in the questionnaires, whereas the currently available registries appeared inappropriate for AIDs patients with complex or undefined diagnosis. AIDs share common clinical features, pathophysiological pathways and therapeutic approaches. Although several resources are now available for rare AIDs, a unique and dedicated site gathering all aspects of these diseases as a whole is still lacking, i.e. covering research as well as the needs of AIDs patients and health care professionals. Our study thus advocates a merging of existing patient registries or the creation of a common database.

EDRN Longitudinal Serum Biorepository — EDRN Public Portal

Cancer.gov

Four percent of EDRN Registry mutation carriers developed cancer within a year after beginning follow-up in this registry; within 2 years, 10% had developed cancer. Within 2 years, 17% had developed either a cancer or a pre-malignant lesion (e.g. adenoma). Our goal is to recruit 265 mutation carriers from the EDRN High Risk Registry and Hereditary Cancer Center for the Longitudinal Serum Biorepository (LSB). Based on the cancer rates observed to date in the EDRN Registry, four years of follow-up in a group of this size would be expected to yield 53 invasive and in situ cancers and 37 benign premalignant lesions. All subjects in the serum biorepository (1)have signed consent (2) are counseled carriers (APC, BRCA1, BRCA2, CDH1, CDKN2A, MLH1, MSH2, MSH6) and (3) are enrolled in family studies through CU's Department of Preventive Medicine and/or are enrolled in the EDRN High Risk Registry. Post specimen medical history is collected annually. Serum and plasma are aliquoted for respository storage and stored in labeled cryovials in an ultra low temperature (-86C) freezer. Specimens in the LSB are available for use in EDRN biomarker development and validation studies.

Analysis of Existing Guidelines for the Systematic Planning Process of Clinical Registries.

PubMed

Löpprich, Martin; Knaup, Petra

2016-01-01

Clinical registries are a powerful method to observe the clinical practice and natural disease history. In contrast to clinical trials, where guidelines and standardized methods exist and are mandatory, only a few initiatives have published methodological guidelines for clinical registries. The objective of this paper was to review these guidelines and systematically assess their completeness, usability and feasibility according to a SWOT analysis. The results show that each guideline has its own strengths and weaknesses. While one supports the systematic planning process, the other discusses clinical registries in great detail. However, the feasibility was mostly limited and the special requirements of clinical registries, their flexible, expandable and adaptable technological structure was not addressed consistently.

DOE Office of Scientific and Technical Information (OSTI.GOV)

David, S.A.; Goodwin, G.M.; Gardner, K.

This document contains a listing of the written scientific information originating in the Materials Joining (formerly the Welding and Brazing Group), Metals and Ceramics Division, Oak Ridge National Laboratory during 1951 through June 1991. This registry of documents is as much as possible, in the order of issue date.

Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

PubMed

Giannakoulas, G; Vasiliadis, K; Frogoudaki, A; Ntellos, C; Tzifa, A; Brili, S; Manginas, A; Papaphylactou, M; Parcharidou, D; Kampouridis, N; Pitsis, A; Chamaidi, A; Kolios, M; Papadopoulos, G; Douras, A; Davlouros, P; Ntiloudi, D; Karvounis, H; Kalangos, A; Tsioufis, C; Rammos, S

2017-10-15

The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation. Copyright © 2017 Elsevier B.V. All rights reserved.

Discrimination between stages of Alzheimer's disease with subsets of Mini-Mental State Examination items. An analysis of Consortium to Establish a Registry for Alzheimer's Disease data.

PubMed

Fillenbaum, G G; Wilkinson, W E; Welsh, K A; Mohs, R C

1994-09-01

To identify minimal sets of Mini-Mental State Examination (MMSE) items that can distinguish normal control subjects from patients with mild Alzheimer's disease (AD), patients with mild from those with moderate AD, and those with moderate from those with severe AD. Two randomly selected equivalent half samples. Results of logistic regression analysis from data from the first half of the sample were confirmed by receiver operating characteristic curves on the second half. Memory disorders clinics at major medical centers in the United States affiliated with the Consortium to establish a Registry for Alzheimer's Disease (CERAD). White, normal control subjects (n = 412) and patients with AD (n = 621) who met CERAD criteria; nonwhite subjects (n = 165) and persons with missing data (n = 27) were excluded. Three four-item sets of MMSE items that discriminate, respectively, (1) normal controls from patients with mild AD, (2) patients with mild from those with moderate AD, and (3) patients with moderate from those with severe AD. The MMSE items discriminating normal controls from patients with mild AD were day, date, recall of apple, and recall of penny; those discriminating patients with mild from those with moderate AD were month, city, spelling world backward, and county, and those discriminating patients with moderate from those with severe AD were floor of building, repeating the word table, naming watch, and folding paper in half. Performance on the first two four-item sets was comparable with that of the full MMSE; the third set distinguished patients with moderate from those with severe AD better than chance. A minimum set of MMSE items can effectively discriminate normal controls from patients with mild AD and between successive levels of severity of AD. Data apply only to white patients with AD. Performance in minorities, more heterogeneous groups, or normal subjects with questionable cognitive status has not been assessed.

Predictable And SuStainable Implementation Of National Cardiovascular Registries Infrastructure: A Think Tank Report from MDEpiNet

PubMed Central

Zeitler, Emily P.; Al-Khatib, Sana M.; Drozda, Joseph P.; Kessler, Larry G.; Kirtane, Ajay J.; Kong, David F.; Laschinger, John; Marinac-Dabic, Danica; Morice, Marie-Claude; Reed, Terrie; Sedrakyan, Art; Stein, Kenneth M.; Tcheng, James; Krucoff, Mitchell W.

2015-01-01

The Medical Device Epidemiological Network Initiative (MDEpiNet) is a public-private partnership between the US Food and Drug Administration (FDA) Center for Devices and Radiological Health (CDRH) and participating partners. The Predictable and SuStainable Implementation of National Cardiovascular Registries (PASSION) program is an MDEpiNet-sponsored program which aims to demonstrate the goals of MDEpiNet by using cardiovascular medical device registries to bridge evidence gaps across the medical device total product life cycle (TPLC). To this end, a PASSION Think Tank meeting took place in October 2014 in Silver Spring, MD, to facilitate discussion between stakeholders about the successes, challenges, and future novel applications of medical device registries, with particular emphasis on identifying pilot projects. Participants spanned a broad range of groups including patients, device manufacturers, regulators, physicians/academicians, professional societies, providers, and payers. The meeting focus included four areas of cardiovascular medicine intended to cultivate interest in four MDEpiNet Disease Specific/Device Specific Working Groups: coronary intervention, electrophysiology, valvular disease, and peripheral vascular disease. In addition, more general issues applying to registry-based infrastructure and analytical methodologies for assessing device benefit/risk were considered to provide context for the Working Groups as PASSION programs going forward. This article summarizes the discussions at the meeting and the future directions of the PASSION program. PMID:26699602

The USC Adult Twin Cohorts: International Twin Study and California Twin Program.

PubMed

Cozen, Wendy; Hwang, Amie E; Cockburn, Myles G; Hamilton, Ann S; Zadnick, John; Mack, Thomas M

2013-02-01

The study of twin subjects permits the documentation of crude heritability and may promote the identification of specific causal alleles. We believe that at the current time, the chief research advantage of twins as subjects, especially monozygotic twins, is that the commonality of their genetic and cultural identity simplifies the interpretation of biological associations. In order to study genetic and environmental determinants of cancer and chronic diseases, we developed two twin registries, maintained at the University of Southern California: The International Twin Study (ITS) and the California Twin Program (CTP). The ITS is a volunteer registry of twins with cancer and chronic disease consisting of 17,245 twin pairs affected by cancer and chronic disease, respectively, ascertained by advertising in periodicals from 1980-1991. The CTP is a population-based registry of California-born twin pairs ascertained by linking the California birth records to the State Department of Motor Vehicles. Over 51,000 individual California twins representing 36,965 pairs completed and returned 16-page questionnaires. Cancer diagnoses in the California twins are updated by regular linkage to the California Cancer Registry. Over 5,000 cancer patients are represented in the CTP. Twins from both registries have participated extensively in studies of breast cancer, melanoma, lymphoma, multiple sclerosis, systemic lupus erythematosus, diabetes mellitus type 1, mammographic density, smoking, and other traits and conditions.

Methodology of clinical research in rare diseases: development of a research program in juvenile neuronal ceroid lipofuscinosis (JNCL) via creation of a patient registry and collaboration with patient advocates

PubMed Central

de Blieck, Elisabeth A.; Augustine, Erika F.; Marshall, Frederick J.; Adams, Heather; Cialone, Jennifer; Dure, Leon; Kwon, Jennifer M.; Newhouse, Nicole; Rose, Katherine; Rothberg, Paul G.; Vierhile, Amy; Mink, Jonathan W.

2013-01-01

Introduction Juvenile neuronal ceroid lipofuscinosis (JNCL; Batten disease) is a rare, inherited, fatal lysosomal storage childhood disorder. True for many rare diseases, there are no treatments that impact the course of JNCL. The University of Rochester Batten Center’s (URBC) mission is to find treatments to slow, halt, or prevent JNCL. Objectives Our initial objective was to develop clinical research infrastructure preparatory to clinical trials, establish a JNCL research cohort, construct a disease-specific clinical outcome measure, and validate a non-invasive diagnostic sampling method. The long-term objective is to design and implement JNCL clinical trials. Methods The Unified Batten Disease Rating Scale (UBDRS) was developed. The Batten Disease Support and Research Association (BDSRA) referred participants; annual BDSRA meetings provided a mobile research setting for registry enrollment and UBDRS piloting. Neuropsychological examinations were performed, enabling external validation of the UBDRS. Buccal epithelial cell collection for genotyping was introduced. Telemedicine for remote UBDRS assessment was piloted. Results The registry enrolled 198 families representing 237 children with NCL. The UBDRS was piloted, validated and has been used to collect natural history data from 120 subjects. Funding and regulatory approval were obtained for a recently launched phase II clinical trial. Several additional lines of inquiry were reported. Conclusion The registry and BDSRA collaboration have enabled development of a clinical rating scale, natural history and neuropsychological studies, and genetic studies for disease confirmation. This work highlights an approach for preparatory natural history research and infrastructure development needed to facilitate efficient implementation of clinical trials in rare diseases. PMID:23628560

Applying Registry Services to Spaceflight Technologies to Aid in the Assignment of Assigned Numbers to Disparate Systems and Their Technologies to Further Enable Interoperability

NASA Technical Reports Server (NTRS)

Bradford, Robert N.; Nichols, Kelvin F.

2006-01-01

To date very little effort has been made to provide interoperability between various space agency projects. To effectively get to the Moon and beyond systems must interoperate. To provide interoperability, standardization and registries of various technologies will be required. These registries will be created as they relate to space flight. With the new NASA Moon/Mars initiative a requirement to standardize and control the naming conventions of very disparate systems and technologies are emerging. The need to provide numbering to the many processes, schemas, vehicles, robots, space suits and technologies (e.g. versions), to name a few, in the highly complex Constellation Initiative is imperative. The number of corporations, developer personnel, system interfaces, people interfaces will require standardization and registries on a scale not currently envisioned. It would only take one exception (stove piped system development) to weaken, if not, destroy interoperability. To start, a standardized registry process must be defined that allows many differing engineers, organizations and operators the ability to easily access disparate registry information across numerous technological and scientific disciplines. Once registries are standardized the need to provide registry support in terms of setup and operations, resolution of conflicts between registries and other issues will need to be addressed. Registries should not be confused with repositories. No end user data is "stored" in a registry nor is it a configuration control system. Once a registry standard is created and approved, the technologies that should be registered must be identified and prioritized. In this paper, we will identify and define a registry process that is compatible with the Constellation Initiative and other non related space activities and organizations. We will then identify and define the various technologies that should use a registry to provide interoperability. The first set of technologies will be those that are currently in need of expansion namely the assignment of satellite designations and the process which controls assignments. Second, we will analyze the technologies currently standardized under the Consultative Committee for Space Data Systems (CCSDS) banner. Third, we will analyze the current CCSDS working group and birds of a feather activities to ascertain registry requirements. Lastly, we will identify technologies that are either currently under the auspices of another

The HOPE (Helping to Outline Paediatric Eating Disorders) Project: development and debut of a paediatric clinical eating disorder registry

PubMed Central

2013-01-01

Background The HOPE (Helping to Outline Paediatric Eating Disorders) Project is an ongoing registry study made up of a sequential cross-sectional sample prospectively recruited over 17 years, and is designed to answer empirical questions about paediatric eating disorders. This paper introduces the HOPE Project, describes the registry sample to-date, and discusses future directions and challenges and accomplishments. The project and clinical service were established in a tertiary academic hospital in Western Australia in 1996 with a service development grant. Research processes were inbuilt into the initial protocols and data collection was maintained in the following years. Recognisable progress with the research agenda accelerated only when dedicated research resources were obtained. The registry sample consists of consecutive children and adolescents assessed at the eating disorder program from 1996 onward. Standardised multidisciplinary data collected from family intake interview, parent and child clinical interviews, medical review, parent, child and teacher psychometric assessments, and inpatient admission records populate the HOPE Project database. Results The registry database to-date contains 941 assessments, of whom 685 met DSM-IV diagnostic criteria for an eating disorder at admission. The majority of the sample were females (91%) from metropolitan Perth (83%). The cases with eating disorders consist of eating disorders not otherwise specified (68%), anorexia nervosa (25%) and bulimia nervosa (7%). Among those with eating disorders, a history of weight loss since illness onset was almost universal (96%) with fear of weight gain (71%) common, and the median duration of illness was 8 months. Conclusions Over the next five years and more, we expect that the HOPE Project will make a strong scientific contribution to paediatric eating disorders research and will have important real-world applications to clinical practice and policy as the research unfolds. PMID:24999409

Delays in Initiation of Disease-Modifying Therapy in Rheumatoid Arthritis Patients: Data from a US-Based Registry.

PubMed

Pappas, Dimitrios A; Kent, Jeffrey D; Greenberg, Jeffrey D; Mason, Marc A; Kremer, Joel M; Holt, Robert J

2015-12-01

The goal of this study was to evaluate how frequently rheumatoid arthritis (RA) therapy is instituted promptly and to describe the characteristics of patients who are not treated early upon diagnosis. The percentage of patients who at the time of enrollment in the Corrona registry were not receiving any RA-directed therapy was evaluated and their characteristics were summarized. The time to subsequent initiation of any RA-directed therapy was also estimated. Among 35,485 patients enrolled in Corrona, 34,735 (97.9%) were on appropriate therapy for RA and 750 (2.1%) had no history of any RA-directed therapy at time of enrollment. Among patients without any history of RA-directed therapy, the overall disease duration was 5.5 ± 9.0 years, with only 50.7% of patients having early disease (duration ≤1 year). Patients with no history of directed RA therapy did not have lower disease activity at enrollment compared with those receiving therapy. Clinical Disease Activity Index (CDAI) was 18.3 ± 15.0; 34% of patients had high and 27.6% moderate disease activity by CDAI. Patients were followed for a median (95% CI) time of 29.5 months (24.6-33.8). During the follow-up period, 372 out of 750 (49.6%) patients initiated RA-directed therapy. The median time to initiation of any RA-directed therapy was 12.1 months (95% CI 9.3-14.8). In this registry analysis, approximately 98% of patients were on appropriate RA therapy for their RA. However, a minority of patients with RA did not have a history of receiving disease-modifying therapy within a mean of approximately 5 years of RA onset and approximately 50% of them did not initiate any therapy within 12 months of registry follow-up. This delay in therapy did not appear to be related to a better controlled, or lower, RA disease activity state at the time of enrollment in the registry. Corrona, LLC.

Demyelination during tumour necrosis factor antagonist therapy for psoriasis: a case report and review of the literature.

PubMed

Mahil, Satveer K; Andrews, Thomasin C; Brierley, Charlotte; Barker, Jonathan N; Smith, Catherine H

2013-02-01

Central nervous system (CNS) demyelination in a patient receiving tumour necrosis factor alpha (TNF-α) antagonist therapy in our practice prompted a search of the literature to assess the evidence for a causal relationship between TNF antagonist therapy and demyelination. We summarise clinical data extracted on 65 reported cases of CNS demyelination in patients receiving TNF antagonist therapy and show that the data are consistent with a drug-related aetiology given the temporal relationship between TNF antagonist initiation and symptoms, de-challenge-re-challenge phenomenon and the later age of disease onset compared with sporadic multiple sclerosis. Research on TNF signalling pathways also suggests a plausible causative role of TNF antagonist therapy in demyelination. However to date, controlled trial and pharmacovigilance data do not show an increased risk of demyelination in patients receiving TNF antagonist therapy. These data may be underpowered to exclude such a risk and pooled, collaborative data from multiple registries are warranted. Given the uncertainty in this area, clinicians should adhere to existing clinical guidance advising avoidance of TNF antagonist therapy in patients with a personal or family history of demyelination, and ensure all suitable patients are enrolled in long term safety registries in countries where these are established.

Review article: Use of renal registry data for research, health-care planning and quality improvement: what can we learn from registry data in the Asia-Pacific region?

PubMed

Lim, Teck-Onn; Goh, Adrian; Lim, Yam-Ngo; Morad, Zaki

2008-12-01

We review renal registry data from the Asia-Pacific region with an emphasis on their uses in health care and in dialysis care in particular. The review aims to demonstrate the information value of registry data. While renal registry provides a useful data resource for epidemiological research, there are severe methodological limitations in its application for analytical or therapeutic research. However, it is the use of renal registry data for public health and health-care management purposes that registry really comes into its own, and it is primarily for these that governments have invested in national patient and disease registries. We apply data from several renal registries in the Asia-Pacific region to illustrate its wide application for planning dialysis services, for evaluating dialysis practices and health outcomes, with a view to improving the quality of dialysis care. In the course of preparing the review, we have found that the quality and accessibility of renal registry data were highly variable across the region. Given the value of renal registry, every country in the Asia-Pacific region should establish one or should ensure that their current registries are better resourced and developed. Greater data sharing and collaboration among registries in the region could help advance the nephrology to serve our patients better.

The Impact of Coexisting Asthma, Chronic Obstructive Pulmonary Disease and Tuberculosis on Survival in Patients with Lung Squamous Cell Carcinoma.

PubMed

Huang, Jing-Yang; Jian, Zhi-Hong; Ndi Nfor, Oswald; Jhang, Kai-Ming; Ku, Wen-Yuan; Ko, Pei-Chieh; Jan, Shiou-Rung; Ho, Chien-Chang; Lung, Chia-Chi; Pan, Hui-Hsien; Liang, Yu-Chiu; Liaw, Yung-Po

2015-01-01

Pulmonary diseases [asthma, chronic obstructive pulmonary disease (COPD), and tuberculosis (TB)] are associated with lung cancer mortality. However, the relationship between coexisting pulmonary diseases and survival in patients with lung squamous cell carcinoma (SqCC) has not been well defined. Patients newly diagnosed with SqCC between 2003 and 2008 were identified by linking the National Health Insurance Research Database and Taiwan Cancer Registry Database. Cases with SqCC were followed up until death, loss to follow-up, or study end in 2010. Information on health status, date of death and the main causes of death was ascertained from the National Death Registry Database. Cox proportional hazard regression was used to calculate the hazard ratio (HR) of coexisting asthma, COPD and/or TB. During the study period, a total of 5406 cases with SqCC were enrolled. For all cause-mortality, HRs were 1.08 [95% confidence interval (CI), 0.99-1.18], 1.04 (95% CI, 0.97-1.12), and 1.14 (95% CI, 1.00-1.31) for individuals with asthma, COPD, and TB, respectively. Specifically, among men with coexisting pulmonary diseases, the HRs were 1.56 (95% CI, 1.23-1.97) and 1.11 (95% CI, 1.00-1.24) for individuals with asthma+COPD+TB and asthma+COPD, respectively. Among male patients with stage III SqCC, HRs were 3.41 (95%CI, 1.27-9.17) and 1.65 (95%CI, 1.10-2.47) for individuals with asthma+TB and asthma+COPD+TB, respectively. Among male patients with stage IV SqCC, HRs were 1.40 (95%CI, 1.00-1.97) and 1.25 (95%CI, 1.03-1.52) for individuals with asthma+ COPD+TB and asthma. Among female patients with stage I and II, HR was 0.19 (95%CI, 005-0.77) for individuals with asthma. Coexisting pulmonary diseases increased the risk of mortality from SqCC in male patients. For female patients with early stage SqCC, pre-existing asthma decreased mortality. These patients deserve greater attention while undergoing cancer treatment.

A method for using real world data in breast cancer modeling.

PubMed

Pobiruchin, Monika; Bochum, Sylvia; Martens, Uwe M; Kieser, Meinhard; Schramm, Wendelin

2016-04-01

Today, hospitals and other health care-related institutions are accumulating a growing bulk of real world clinical data. Such data offer new possibilities for the generation of disease models for the health economic evaluation. In this article, we propose a new approach to leverage cancer registry data for the development of Markov models. Records of breast cancer patients from a clinical cancer registry were used to construct a real world data driven disease model. We describe a model generation process which maps database structures to disease state definitions based on medical expert knowledge. Software was programmed in Java to automatically derive a model structure and transition probabilities. We illustrate our method with the reconstruction of a published breast cancer reference model derived primarily from clinical study data. In doing so, we exported longitudinal patient data from a clinical cancer registry covering eight years. The patient cohort (n=892) comprised HER2-positive and HER2-negative women treated with or without Trastuzumab. The models generated with this method for the respective patient cohorts were comparable to the reference model in their structure and treatment effects. However, our computed disease models reflect a more detailed picture of the transition probabilities, especially for disease free survival and recurrence. Our work presents an approach to extract Markov models semi-automatically using real world data from a clinical cancer registry. Health care decision makers may benefit from more realistic disease models to improve health care-related planning and actions based on their own data. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

Comparison of disease clusters in two elderly populations hospitalized in 2008 and 2010.

PubMed

Marengoni, A; Nobili, A; Pirali, C; Tettamanti, M; Pasina, L; Salerno, F; Corrao, S; Iorio, A; Marcucci, M; Franchi, C; Mannucci, P M

2013-01-01

As chronicity represents one of the major challenges in the healthcare of aging populations, the understanding of how chronic diseases distribute and co-occur in this part of the population is needed. The aims of this study were to evaluate and compare patterns of diseases identified with cluster analysis in two samples of hospitalized elderly. Data were obtained from the multicenter 'Registry Politerapie SIMI (REPOSI)' that included people aged 65 or older hospitalized in internal medicine and geriatric wards in Italy during 2008 and 2010. The study sample from the first wave included 1,411 subjects enrolled in 38 hospitals wards, whereas the second wave included 1,380 subjects in 66 wards located in different regions of Italy. To analyze patterns of multimorbidity, a cluster analysis was performed including the same diseases (19 chronic conditions with a prevalence >5%) collected at hospital discharge during the two waves of the registry. Eight clusters of diseases were identified in the first wave of the REPOSI registry and six in the second wave. Several diseases were included in similar clusters in the two waves, such as malignancy and liver cirrhosis; anemia, gastric and intestinal diseases; diabetes and coronary heart disease; chronic obstructive pulmonary disease and prostate hypertrophy. These findings strengthened the idea of an association other than by chance of diseases in the elderly population. Copyright © 2013 S. Karger AG, Basel.

76 FR 23600 - Availability of Draft Toxicological Profile

Federal Register 2010, 2011, 2012, 2013, 2014

2011-04-27

... DEPARTMENT OF HEALTH AND HUMAN SERVICES Agency for Toxic Substances and Disease Registry [CDC-2011... Registry (ATSDR), Department of Health and Human Services (DHHS). ACTION: Notice of availability. SUMMARY... of uranium. Although ATSDR considered key studies for uranium during the profile development process...

End stage renal disease in French Guiana (data from R.E.I.N registry): South American or French?

PubMed

Rochemont, Dévi Rita; Meddeb, Mohamed; Roura, Raoul; Couchoud, Cécile; Nacher, Mathieu; Basurko, Célia

2017-06-30

End-Stage renal disease (ESRD) causes considerable morbidity and mortality, and significantly alters patients' quality of life. There are very few published data on this problem in the French Overseas territories. The development of a registry on end stage renal disease in French Guiana in 2011 allowed to describe the magnitude of this problem in the region for the first time. Using data from the French Renal Epidemiology and Information Network registry (R.E.I.N). Descriptive statistics on quantitative and qualitative variables in the registry were performed on prevalent cases and incident cases in 2011, 2012 and 2013. French Guiana has one of the highest ESRD prevalence and incidence in France. The two main causes of ESRD were hypertensive and diabetic nephropathies. The French Guianese population had a different demographic profile (younger, more women, more migrants) than in mainland France. Most patients had at least one comorbidity, predominantly (95.3%) hypertension. In French Guiana dialysis was initiated in emergency for 71.3% of patients versus 33% in France (p < 0.001). These first results give important public health information: i) End stage renal disease has a very high prevalence relative to mainland France ii) Patients have a different demographic profile and enter care late in the course of their renal disease. These data are closer to what is observed in the Caribbean or in Latin America than in Mainland France.

ClinicalTrials.gov as a data source for semi-automated point-of-care trial eligibility screening.

PubMed

Pfiffner, Pascal B; Oh, JiWon; Miller, Timothy A; Mandl, Kenneth D

2014-01-01

Implementing semi-automated processes to efficiently match patients to clinical trials at the point of care requires both detailed patient data and authoritative information about open studies. To evaluate the utility of the ClinicalTrials.gov registry as a data source for semi-automated trial eligibility screening. Eligibility criteria and metadata for 437 trials open for recruitment in four different clinical domains were identified in ClinicalTrials.gov. Trials were evaluated for up to date recruitment status and eligibility criteria were evaluated for obstacles to automated interpretation. Finally, phone or email outreach to coordinators at a subset of the trials was made to assess the accuracy of contact details and recruitment status. 24% (104 of 437) of trials declaring on open recruitment status list a study completion date in the past, indicating out of date records. Substantial barriers to automated eligibility interpretation in free form text are present in 81% to up to 94% of all trials. We were unable to contact coordinators at 31% (45 of 146) of the trials in the subset, either by phone or by email. Only 53% (74 of 146) would confirm that they were still recruiting patients. Because ClinicalTrials.gov has entries on most US and many international trials, the registry could be repurposed as a comprehensive trial matching data source. Semi-automated point of care recruitment would be facilitated by matching the registry's eligibility criteria against clinical data from electronic health records. But the current entries fall short. Ultimately, improved techniques in natural language processing will facilitate semi-automated complex matching. As immediate next steps, we recommend augmenting ClinicalTrials.gov data entry forms to capture key eligibility criteria in a simple, structured format.

Iliac Arteries: How Registries Can Help Improve Outcomes

PubMed Central

Tapping, Charles Ross; Uberoi, Raman

2014-01-01

There are many publications reporting excellent short and long-term results with endovascular techniques. Patients included in trials are often highly selected and may not represent real world practice. Registries are important to interventional radiologists for several reasons; they reflect prevailing practice and can be used to establish real world standards of care and safety profiles. This information allows individuals and centers to evaluate their outcomes compared with national norms. The British Iliac Angioplasty and Stenting (BIAS) registry is an example of a mature registry that has been collecting data since 2000 and has been reporting outcomes since 2001. This article discusses the evidence to support both endovascular and surgical intervention for aortoiliac occlusive disease, the role of registries, and optimal techniques for aortoiliac intervention. PMID:25435659

Evidence of prostate cancer "reverse stage migration" toward more advanced disease at diagnosis: Data from the Pennsylvania Cancer Registry.

PubMed

Reese, Adam C; Wessel, Sean R; Fisher, Susan G; Mydlo, Jack H

2016-08-01

The widespread adoption of prostate-specific antigen-based prostate cancer screening caused a stage migration toward earlier stage disease at diagnosis. We investigated whether this stage migration has persisted in a contemporary analysis of a population-based statewide cancer registry. We analyzed the Pennsylvania Cancer Registry, a statewide registry of all newly diagnosed cancers. Data were collected on prostate cancers diagnosed between 1992 and 2012. We determined age-adjusted prostate cancer incidence and mortality rates, as well as the distribution of tumor stage (localized, regional, or metastatic) at diagnosis, and assessed for changes in these variables over time using joinpoint analysis. Between 1992 and 2012, 210,831 new cases of prostate cancer were diagnosed in Pennsylvania, and 33,948 men died of disease. Age-adjusted prostate cancer incidence rates, and specifically the incidence of localized disease, have decreased dramatically since 2007 to 2008. Due to the decreased diagnosis of localized disease, regional and metastatic tumors have made up a greater percentage of all prostate cancer diagnoses in recent years, despite a relatively stable incidence of these advanced stage tumors. Over the past 2 decades, age-adjusted prostate cancer incidence rates in Pennsylvania have decreased, primarily because of the decreased detection of early-stage disease. There has been a corresponding shift toward more advanced disease at diagnosis. These findings may be explained by the decreased use of prostate-specific antigen-based screening, among other factors. The 2012 United States Preventative Services Task Force recommendations against prostate cancer screening may exacerbate this concerning trend, potentially resulting in an increase in prostate cancer-specific mortality. Copyright © 2016 Elsevier Inc. All rights reserved.

Changes in the management and comorbidities of acromegaly over three decades: the French Acromegaly Registry.

PubMed

Maione, Luigi; Brue, Thierry; Beckers, Albert; Delemer, Brigitte; Petrossians, Patrick; Borson-Chazot, Françoise; Chabre, Olivier; François, Patrick; Bertherat, Jérôme; Cortet-Rudelli, Christine; Chanson, Philippe

2017-05-01

Acromegaly is a rare disease associated with chronic multisystem complications. National registries have been created in several countries. The French Registry contains data on acromegaly epidemiology, management and comorbidities recorded over more than three decades, retrospectively until 1999 and prospectively from 1999 to 2012. Data could be analyzed for 999 of the 1034 patients included in the registry (46% males). Disease control, defined as IGF-I normalization (adjusted for age and sex), was achieved in 75% of patients at the last follow-up visit. Half the patients with uncontrolled disease had IGF-I levels below 1.5 times the upper limit of normal (ULN). The proportion of patients with surgically cured disease did not change markedly over time, whereas the proportion of patients with uncontrolled disease fell and the proportion of patients with medically controlled disease rose. Cardiovascular, metabolic, respiratory and rheumatologic comorbidities and their outcomes were recorded for most patients, and no noteworthy overall deterioration was noted over time. Cancer occurred in 10% of patients, for a standardized incidence ratio of 1.34 (95% CI: 0.94-1.87) in men and 1.24 (0.77-1.73) in women. Forty-one patients died during follow-up, for a standardized mortality ratio of 1.05 (0.70-1.42). Most deaths were due to cancer. The majority of patients with acromegaly now have successful disease control thanks to the multistep management. The incidence of comorbidities following diagnosis of acromegaly is very low. Life expectancy is now close to that of the general population, probably owing to better management of the GH/IGF-I excess and comorbidities. © 2017 European Society of Endocrinology.

Pulmonary hypertension registry of Kerala, India (PRO-KERALA) - Clinical characteristics and practice patterns.

PubMed

Harikrishnan, S; Sanjay, G; Ashishkumar, M; Menon, Jaideep; Rajesh, Gopalan Nair; Kumar, Raman Krishna; George Koshy, A; Attacheril, Thankachan V; George, Raju; Punnoose, Eapen; Ashraf, S M; Arun, S R; Cholakkal, Mohammed; Jeemon, Panniyammakal

2018-08-15

Epidemiological data on pulmonary hypertension (PH) are scarce from developing countries including India. We established a multi-center registry of PH, the PRO-KERALA registry, in Kerala, India. Fifty hospitals enrolled consecutive adult (>18 years) patients for one year. Echocardiographic criteria (right ventricular systolic pressure - RVSP > 50 mmHg) or invasively obtained mean pulmonary artery pressure > 25 mmHg was the criteria for entry. There were 2003 patients (52% Women, mean age 56 ± 16.1 years) enrolled. The mean RVSP was 68.2 (SD = 17.9) mmHg. Majority of the study participants (59%) belonged to group 2 of the WHO Nice Classification 2013 (PH secondary to left heart disease). One-fifth (21.2%) belonged to group 1, while 13.3%, 3.8% and 2.4% of the study population belonged to groups 3, 4 and 5 respectively. More than a quarter (27%) reported PH due to left heart disease with valvular disease etiology; while 20.7% had coronary artery disease. The other common etiological factors were chronic obstructive pulmonary disease (10.6%), congenital heart disease (14.6%), idiopathic pulmonary hypertension (5.8%), and chronic thromboembolic pulmonary hypertension (3.8%). Only one of two patients with pulmonary artery hypertension was receiving PH specific therapies. The use of combination therapy was negligible and PH-specific therapies were prescribed off-label to a small proportion of patients too. PRO-KERALA is the first PH registry from South Asia and the second largest globally. Left heart diseases attribute to three fifths of patients with PH. Utilization rates of PH specific drug therapies are remarkably lower than the Western population. Copyright © 2018 Elsevier B.V. All rights reserved.

Why does primary angioplasty not work in registries? Quantifying the susceptibility of real-world comparative effectiveness data to allocation bias.

PubMed

Sen, Sayan; Davies, Justin E; Malik, Iqbal S; Foale, Rodney A; Mikhail, Ghada W; Hadjiloizou, Nearchos; Hughes, Alun; Mayet, Jamil; Francis, Darrel P

2012-11-01

Meta-analysis of registries (comparative effectiveness research) shows that primary angioplasty and fibrinolysis have equivalent real-world survival. Yet, randomized, controlled trials consistently find primary angioplasty superior. Can unequal allocation of higher-risk patients in registries have masked primary angioplasty benefit? First, we constructed a model to demonstrate the potential effect of allocation bias. We then analyzed published registries (55022 patients) for allocation of higher-risk patients (Killip class ≥1) to determine whether the choice of reperfusion therapy was affected by the risk level of the patient. Meta-regression was used to examine the relationship between differences in allocation of high-risk patient to primary angioplasty or fibrinolysis and mortality. Initial modeling suggested that registry outcomes are sensitive to allocation bias of high-risk patients. Across the registries, the therapy receiving excess high-risk patients had worse mortality. Unequal distribution of high-risk status accounted for most of the between-registry variance (adjusted R(2)(meta)=83.1%). Accounting for differential allocation of higher-risk patients, primary angioplasty gave 22% lower mortality (odds ratio, 0.78; 95% confidence interval, 0.64-0.97; P=0.029). We derive a formula, called the number needed to abolish, highlighting situations in which comparative effectiveness studies are particularly vulnerable to this bias. In ST-segment elevation myocardial infarction, clinicians' preference for management of a few high-risk patients can shift mortality substantially. Comparative effectiveness research in any disease is vulnerable to this, especially diseases with an immediately identifiable high-risk subgroup that clinicians prefer to allocate to 1 therapy. For this reason, preliminary indications from registry-based comparative effectiveness research should be definitively tested by randomized, controlled trials.

International Registry for Patients With Castleman Disease

ClinicalTrials.gov

2017-07-12

Castleman Disease; Castleman's Disease; Giant Lymph Node Hyperplasia; Angiofollicular Lymph Hyperplasia; Angiofollicular Lymph Node Hyperplasia; Angiofollicular Lymphoid Hyperplasia; GLNH; Hyperplasia, Giant Lymph Node; Lymph Node Hyperplasia, Giant

Analysis of the time and workers needed to conduct systematic reviews of medical interventions using data from the PROSPERO registry

PubMed Central

Borah, Rohit; Brown, Andrew W; Capers, Patrice L; Kaiser, Kathryn A

2017-01-01

Objectives To summarise logistical aspects of recently completed systematic reviews that were registered in the International Prospective Register of Systematic Reviews (PROSPERO) registry to quantify the time and resources required to complete such projects. Design Meta-analysis. Data sources and study selection All of the 195 registered and completed reviews (status from the PROSPERO registry) with associated publications at the time of our search (1 July 2014). Data extraction All authors extracted data using registry entries and publication information related to the data sources used, the number of initially retrieved citations, the final number of included studies, the time between registration date to publication date and number of authors involved for completion of each publication. Information related to funding and geographical location was also recorded when reported. Results The mean estimated time to complete the project and publish the review was 67.3 weeks (IQR=42). The number of studies found in the literature searches ranged from 27 to 92 020; the mean yield rate of included studies was 2.94% (IQR=2.5); and the mean number of authors per review was 5, SD=3. Funded reviews took significantly longer to complete and publish (mean=42 vs 26 weeks) and involved more authors and team members (mean=6.8 vs 4.8 people) than those that did not report funding (both p<0.001). Conclusions Systematic reviews presently take much time and require large amounts of human resources. In the light of the ever-increasing volume of published studies, application of existing computing and informatics technology should be applied to decrease this time and resource burden. We discuss recently published guidelines that provide a framework to make finding and accessing relevant literature less burdensome. PMID:28242767

The Brustkrebs-Studien.de website for breast cancer patients: User acceptance of a German internet portal offering information on the disease and treatment options, and a clinical trials matching service

PubMed Central

2010-01-01

Background The internet portal http://www.brustkrebs-studien.de (BKS) was launched in 2000 by the German Society of Senology (DGS) and the Baden-Württemberg Institute for Women's Health (IFG) to provide expert-written information on breast cancer online and to encourage and facilitate the participation of breast cancer patients in clinical trials. We describe the development of BKS and its applications, and report on website statistics and user acceptance. Methods Existing registries, including ClinicalTrials.gov, were analysed before we designed BKS, which combines a trial registry, a knowledge portal, and an online second opinion service. An advisory board guided the process. Log files and patient enquiries for trial participation and second opinions were analysed. A two-week user satisfaction survey was conducted online. Results During 10/2005-06/2010, the portal attracted 702,655 visitors, generating 15,507,454 page views. By 06/2010, the website's active scientific community consisted of 189 investigators and physicians, and the registry covered 163 clinical trial protocols. In 2009, 143 patients requested trial enrolment and 119 sought second opinions or individual treatment advice from the expert panel. During the two-week survey in 2008, 5,702 BKS visitors submitted 507 evaluable questionnaires. Portal acceptance was high. Respondents trusted information correctness (80%), welcomed self-matching to clinical trials (79%) and planned to use the portal in the future (76%) and recommend it to others (81%). Conclusions BKS is an established and trusted breast cancer information platform offering up-to-date resources and protocols to the growing physician and patient community to encourage participation in clinical trials. Further studies are needed to assess potential increases in trial enrolment by eligibility matching services. PMID:21126358

A medical birth registry at Kilimanjaro Christian Medical Centre.

PubMed

Bergsjø, Per; Mlay, Joseph; Lie, Rolv T; Lie-Nielsen, E; Shao, John F

2007-04-01

To establish a medical birth registry intended to serve clinical, administrative and research purposes. Starting in July 2000, every birth at Kilimanjaro Christian Medical Centre (KCMC) in Moshi, Tanzania has been recorded in a separate database. The information is obtained through personal interviews with each mother, conducted by specially trained midwives, and supplied with data from the medical records. A secretary enters the data into the electronic file. Data are collected about the mother and father: education, occupation and living conditions, mother's health before and during present pregnancy, expected date of delivery, smoking and drinking (alcohol) habits, use of drugs, plus HIV and syphilis status (if known). This is followed by particulars on the delivery: spontaneous or induced, and complications; the child or children: weight, height and Apgar score, malformations and other diagnoses. Mode of birth: spontaneous or operative intervention. If perinatal death: when? Transfer to intensive neonatal unit? The mother's reproductive history (births, miscarriages, ectopic pregnancies) is also recorded, with outcomes. We describe the process based on more than six years' experience, including obstacles and how they were overcome. The registry serves as a monitoring tool, with a set of key activities and events being issued monthly, indicating changes and trends in, e.g., bleeding complications, caesarean section rates and perinatal mortality, as early warning signs. Monthly reports on key issues are presented. Confidentiality and data protection are key issues. Day-to-day recording of births is vulnerable to personnel shortage, whether from disease or holidays. Validation and quality checks leave the overall impression that the database is largely accurate and credible. There are plenty of opportunities for research. Clinicians and epidemiologists will profit from using the database to test hypotheses and clarify problem issues, to the ultimate benefit of labouring women and their children.

Psychiatric and neurological symptoms in patients with Niemann-Pick disease type C (NP-C): Findings from the International NPC Registry.

PubMed

Bonnot, Olivier; Gama, Clarissa S; Mengel, Eugen; Pineda, Mercè; Vanier, Marie T; Watson, Louise; Watissée, Marie; Schwierin, Barbara; Patterson, Marc C

2017-10-09

Niemann-Pick disease type C (NP-C) is a rare inherited neurovisceral disease that should be recognised by psychiatrists as a possible underlying cause of psychiatric abnormalities. This study describes NP-C patients who had psychiatric manifestations at enrolment in the international NPC Registry, a unique multicentre, prospective, observational disease registry. Treating physicians' data entries describing psychiatric manifestations in NPC patients were coded and grouped by expert psychiatrists. Out of 386 NP-C patients included in the registry as of October 2015, psychiatric abnormalities were reported to be present in 34% (94/280) of those with available data. Forty-four patients were confirmed to have identifiable psychiatric manifestations, with text describing these psychiatric manifestations. In these 44 patients, the median (range) age at onset of psychiatric manifestations was 17.9 years (2.5-67.9; n = 15), while the median (range) age at NP-C diagnosis was 23.7 years (0.2-69.8; n = 34). Almost all patients (43/44; 98%) had an occurrence of ≥1 neurological manifestation at enrolment. These data show that substantial delays in diagnosis of NP-C are long among patients with psychiatric symptoms and, moreover, patients presenting with psychiatric features and at least one of cognitive impairment, neurological manifestations, and/or visceral symptoms should be screened for NP-C.

Consensus on the criteria needed for creating a rare-disease patient registry. A Delphi study.

PubMed

Cavero-Carbonell, Clara; Gras-Colomer, Elena; Guaita-Calatrava, Rosana; López-Briones, Carmen; Amorós, Rubén; Abaitua, Ignacio; Posada, Manuel; Zurriaga, Oscar

2016-06-01

Patient registries (PRs) are important tools for public-health surveillance and rare-disease research. The purpose of this study is to identify the most important criteria for the creation of a rare-disease PR that could be used by public-health authorities to develop health policies. A consensus-development Delphi study was used, with participants selected for their expertize in rare diseases and registries. Participants were asked to complete a questionnaire on the most important criteria for creating PRs. Three rounds were performed. Agreement was reached on half the questions in the first round and on 89% of questions in the final round, with a total expert participation rate of around 60% by the final stage. This study made it possible to reach a broader consensus starting from experts' initial assessment of the features that should be considered for the creation of a rare-disease PR. The consensus method used made it possible to define the characteristics of a PR based on expert opinion within a rare-disease framework. This study may serve as a guide for helping other researchers plan and build a rare-disease PR. © The Author 2015. Published by Oxford University Press on behalf of Faculty of Public Health. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

PLUTO first report.

PubMed

Otte, Jean-Bernard; Meyers, Rebecka

2010-11-01

The PLUTO is a registry developed by an international collaboration of the Liver Tumors Strategy Group (SIOPEL) of the SIOP. Although the number of patients collected in PLUTO to date is too small to add any analytic power to the existing literature, this new registry has great promise. It has been created to clarify issues regarding the role of liver transplantation in the treatment of children with unresectable liver tumors. By reviewing the results to date, we hope we can motivate more centers to participate, enroll patients, complete data entry, and boost the potential impact of the collaborative effort. To achieve this goal, a large number of patients are needed, which requires an intensified international collaboration. Pediatric oncologists, pediatric surgical oncologists, and pediatric liver transplant surgeons are all encouraged to participate and contribute. This is a preliminary glimpse of what we hope to be a series of interim reports over the next decade from the steering committee to help guide therapy in this very challenging group of children. © 2010 John Wiley & Sons A/S.

75 FR 71132 - Availability of Draft Toxicological Profiles

Federal Register 2010, 2011, 2012, 2013, 2014

2010-11-22

... DEPARTMENT OF HEALTH AND HUMAN SERVICES Agency for Toxic Substances and Disease Registry [ATSDR... Registry (ATSDR), Department of Health and Human Services (DHHS). ACTION: Notice of availability. SUMMARY... documents is also available at: http://www.atsdr.cdc.gov/toxprofiles/index.asp . Written comments and other...

Patient and Provider Assessment of Lipid Management Registry

ClinicalTrials.gov

2016-02-15

Hyperlipidemia; Hypercholesterolemia; Cardiovascular Disease; Diabetes; Chronic Kidney Disease (CKD); Hypertension; Smoking; Myocardial Infarction (MI); Unstable Angina; Angina; Coronary Artery Disease (CAD); Stroke; Transient Ischemic Attack (TIA); Carotid Stenosis; Peripheral Arterial Disease; Atherosclerosis; Claudication

Statistical Process Control: A Quality Tool for a Venous Thromboembolic Disease Registry.

PubMed

Posadas-Martinez, Maria Lourdes; Rojas, Liliana Paloma; Vazquez, Fernando Javier; De Quiros, Fernan Bernaldo; Waisman, Gabriel Dario; Giunta, Diego Hernan

2016-01-01

We aim to describe Statistical Control Process as a quality tool for the Institutional Registry of Venous Thromboembolic Disease (IRTD), a registry developed in a community-care tertiary hospital in Buenos Aires, Argentina. The IRTD is a prospective cohort. The process of data acquisition began with the creation of a computerized alert generated whenever physicians requested imaging or laboratory study to diagnose venous thromboembolism, which defined eligible patients. The process then followed a structured methodology for patient's inclusion, evaluation, and posterior data entry. To control this process, process performance indicators were designed to be measured monthly. These included the number of eligible patients, the number of included patients, median time to patient's evaluation, and percentage of patients lost to evaluation. Control charts were graphed for each indicator. The registry was evaluated in 93 months, where 25,757 patients were reported and 6,798 patients met inclusion criteria. The median time to evaluation was 20 hours (SD, 12) and 7.7% of the total was lost to evaluation. Each indicator presented trends over time, caused by structural changes and improvement cycles, and therefore the central limit suffered inflexions. Statistical process control through process performance indicators allowed us to control the performance of the registry over time to detect systematic problems. We postulate that this approach could be reproduced for other clinical registries.

The international dystrophic epidermolysis bullosa patient registry: an online database of dystrophic epidermolysis bullosa patients and their COL7A1 mutations.

PubMed

van den Akker, Peter C; Jonkman, Marcel F; Rengaw, Trebor; Bruckner-Tuderman, Leena; Has, Cristina; Bauer, Johann W; Klausegger, Alfred; Zambruno, Giovanna; Castiglia, Daniele; Mellerio, Jemima E; McGrath, John A; van Essen, Anthonie J; Hofstra, Robert M W; Swertz, Morris A

2011-10-01

Dystrophic epidermolysis bullosa (DEB) is a heritable blistering disorder that can be inherited autosomal dominantly (DDEB) or recessively (RDEB) and covers a group of several distinctive phenotypes. A large number of unique COL7A1 mutations have been shown to underlie DEB. Although general genotype-phenotype correlation rules have emerged, many exceptions to these rules exist, compromising disease diagnosing and genetic counseling. We therefore constructed the International DEB Patient Registry (http://www.deb-central.org), aimed at worldwide collection and sharing of phenotypic and genotypic information on DEB. As of May 2011, this MOLGENIS-based registry contains detailed information on 508 published and 71 unpublished patients and their 388 unique COL7A1 mutations, and includes all combinations of mutations. The current registry RDEB versus DDEB ratio of 4:1, if compared to prevalence figures, suggests underreporting of DDEB in the literature. Thirty-eight percent of mutations stored introduce a premature termination codon (PTC) and 43% an amino acid change. Submission wizards allow users to quickly and easily share novel information. This registry will be of great help in disease diagnosing and genetic counseling and will lead to novel insights, especially in the rare phenotypes of which there is often lack of understanding. Altogether, this registry will greatly benefit the DEB patients. © 2011 Wiley-Liss, Inc.

A web-based, patient driven registry for Angelman syndrome: the global Angelman syndrome registry.

PubMed

Napier, Kathryn R; Tones, Megan; Simons, Chloe; Heussler, Helen; Hunter, Adam A; Cross, Meagan; Bellgard, Matthew I

2017-08-01

Angelman syndrome (AS) is a rare neurodevelopmental disorder that is characterised by severe global developmental delays, ataxia, loss of speech, epilepsy, sleep disorders, and a happy disposition. There is currently no cure for AS, though several pharmaceutical companies are anticipating drug trials for new therapies to treat AS. The Foundation for Angelman Therapeutics (FAST) Australia therefore identified a need for a global AS patient registry to identify patients for recruitment for clinical trials.The Global AS Registry was deployed in September 2016 utilising the Rare Disease Registry Framework, an open-source tool that enables the efficient creation and management of patient registries. The Global AS Registry is web-based and allows parents and guardians worldwide to register, provide informed consent, and enter data on individuals with AS. 286 patients have registered in the first 8 months since deployment.We demonstrate the successful deployment of the first patient-driven global registry for AS. The data generated from the Global AS Registry will be crucial in identifying patients suitable for clinical trials and in informing research that will identify treatments for AS, and ultimately improve the lives of individuals and their families living with AS.

Medical Specialty Society Sponsored Data Registries – Opportunities in Plastic Surgery

PubMed Central

Hume, Keith M.; Crotty, Catherine A.; Simmons, Christopher J.; Neumeister, Michael W.; Chung, Kevin C.

2014-01-01

Clinical data registries are commonly used worldwide and are implemented for a variety of purposes ranging from physician or facility clinic logs for tracking patients, collecting outcomes data, to measuring quality improvement or safety of medical devices. In the United States, the Food and Drug Administration has used data collected through registries to facilitate the drug and device regulatory process, ongoing surveillance during the product life-cycle, and for disease appraisals. Furthermore, the Centers for Medicare and Medicaid Services, in certain instances, base registry participation and submitting data to registries as factors for reimbursement decisions. The purpose of this article is to discuss the use of clinical data registries, the role that medical specialty societies, in particular the American Society of Plastic Surgeons and The Plastic Surgery Foundation, can have in the development and management of registries, and the opportunities for registry use in Plastic Surgery. As outcomes data are becoming essential measures of quality healthcare delivery, participating in registry development and centralized data collection has become a critical effort for Plastic Surgery to engage in to proactively participate in the national quality and performance measurement agenda. PMID:23806935

Drug-Induced Liver Injury Network Causality Assessment: Criteria and Experience in the United States.

PubMed

Hayashi, Paul H

2016-02-04

Hepatotoxicity due to drugs, herbal or dietary supplements remains largely a clinical diagnosis based on meticulous history taking and exclusion of other causes of liver injury. In 2004, the U.S. Drug-Induced Liver Injury Network (DILIN) was created under the auspices of the U.S. National Institute of Diabetes and Digestive and Kidney Diseases with the aims of establishing a large registry of cases for clinical, epidemiological and mechanistic study. From inception, the DILIN has used an expert opinion process that incorporates consensus amongst three different DILIN hepatologists assigned to each case. It is the most well-established, well-described and vigorous expert opinion process for DILI to date, and yet it is an imperfect standard. This review will discuss the DILIN expert opinion process, its strengths and weaknesses, psychometric performance and future.

Traffic-Related Air Pollution and Parkinson’s Disease in Denmark: A Case–Control Study

PubMed Central

Ritz, Beate; Lee, Pei-Chen; Hansen, Johnni; Lassen, Christina Funch; Ketzel, Matthias; Sørensen, Mette; Raaschou-Nielsen, Ole

2015-01-01

Background Very little is currently known about air pollutants’ adverse effects on neurodegenerative diseases even though recent studies have linked particulate exposures to brain pathologies associated with Parkinson’s and Alzheimer’s disease. Objective In the present study, we investigated long-term exposure to traffic-related air pollution and Parkinson’s disease. Methods In a case–control study of 1,696 Parkinson’s disease (PD) patients identified from Danish hospital registries and diagnosed 1996–2009 and 1,800 population controls matched by sex and year of birth, we assessed long-term traffic-related air pollutant exposures (represented by nitrogen dioxide; NO2) from a dispersion model, using residential addresses from 1971 to the date of diagnosis or first cardinal symptom for cases and the corresponding index date for their matched controls. Odds ratios (ORs) and 95% confidence intervals (CIs) were estimated with logistic regression, adjusting for matching factors and potential confounders. Results We found ambient air pollution from traffic sources to be associated with risk of PD, with a 9% higher risk (95% CI: 3, 16.0%) per interquartile range increase (2.97 μg/m3) in modeled NO2. For participants living for ≥ 20 years in the capital city, ORs were larger (OR = 1.21; 95% CI: 1.11, 1.31) than in provincial towns (OR = 1.10; 95% CI: 0.97, 1.26), whereas there was no association among rural residents. Conclusions Our findings raise concerns about potential effects of air pollution from traffic and other sources on the risk of PD, particularly in populations with high or increasing exposures. Citation Ritz B, Lee PC, Hansen J, Funch Lassen C, Ketzel M, Sørensen M, Raaschou-Nielsen O. 2016. Traffic-related air pollution and Parkinson’s disease in Denmark: a case–control study. Environ Health Perspect 124:351–356; http://dx.doi.org/10.1289/ehp.1409313 PMID:26151951

Marrow donor registry and cord blood bank in Taiwan.

PubMed

Lee, Tsung Dao

2002-08-01

Unrelated Bone marrow transplant was initiated thirty years ago. Though there are over millions of donors registered with the bone marrow registries worldwide, Asian patients rarely find a match with all these donors. Tzu Chi Marrow Donor Registry was established to meet this need. It has become the largest Asian marrow donor registry in the world. With the introduction of high technology to test the HLA of the donors and recipients, the success rate of bone marrow transplant is greatly improved among Asian countries. 50% of blood disease Asian patients who cannot find a bone marrow matched donor will be complemented by the establishment of cord blood banks in Taiwan.

Probability of Finding Marrow Unrelated Donor (MUD) for an Indian patient in a Multi-national Human Leukocyte Antigen (HLA) Registry.

PubMed

Tiwari, Aseem K; Bhati-Kushwaha, Himakshi; Kukreja, Pooja; Mishra, Vikash C; Tyagi, Neetu; Sharma, Ashish; Raina, Vimarsh

2015-06-01

With an increase in the number of transplants happening globally, hematopoietic stem cells (HSC) transplantation from matched unrelated donor (MUD) has begun. The increasing trend of MUD transplants across countries has been largely facilitated with the conspicuous growth of volunteer HSC donor noted in the last decade i.e. 8 million HSC donors in 2002 to more than 22 million in 2013 registered in 71 member registries of the Bone Marrow Donor Worldwide (BMDW). Some populations of the world are still very poorly represented in these registries. Since, the chances of successful engraftment and disease free survival are directly proportional to the HLA compatibility between the recipient and the prospective donor, the diversity of the HLA system at the antigenic and allelic level and the heterogeneity of HLA data of the registered donors has a bearing on the probability of finding a volunteer unrelated HSC donor for patients from such populations. In the present study 126 patients were identified suffering from hematological diseases requiring MUD transplant. Their HLA typing was performed and search was done using BMDW database. The search results for these Indian patients in the multinational registry as well as in the Indian Registries were analyzed using mean, range, standard deviation and finally evaluated in terms of probability for finding matched donor (MUD). Total Asian population is only 11 % in the BMDW making it difficult to find a MUD for an Asian patient. The current study supports this, experimentally; revealing that the probability of finding an allele match for an Indian patient in the multinational Human Leukocyte Antigen (HLA) registries is 16 % and a dismal 0.008 % in the Indian registries (donors in Indian registries is just 33,678 as compared to 22.5 million in BMDW). This greatly, emphasizes on enhancing the number of Indian donors in Indian and multi-national registries.

Prognostic and therapeutic implications of statin and aspirin therapy in individuals with nonobstructive coronary artery disease: results from the CONFIRM (COronary CT Angiography EvaluatioN For Clinical Outcomes: An InteRnational Multicenter registry) registry.

PubMed

Chow, Benjamin J W; Small, Gary; Yam, Yeung; Chen, Li; McPherson, Ruth; Achenbach, Stephan; Al-Mallah, Mouaz; Berman, Daniel S; Budoff, Matthew J; Cademartiri, Filippo; Callister, Tracy Q; Chang, Hyuk-Jae; Cheng, Victor Y; Chinnaiyan, Kavitha; Cury, Ricardo; Delago, Augustin; Dunning, Allison; Feuchtner, Gundrun; Hadamitzky, Martin; Hausleiter, Jörg; Karlsberg, Ronald P; Kaufmann, Philipp A; Kim, Yong-Jin; Leipsic, Jonathon; LaBounty, Troy; Lin, Fay; Maffei, Erica; Raff, Gilbert L; Shaw, Leslee J; Villines, Todd C; Min, James K

2015-04-01

We sought to examine the risk of mortality associated with nonobstructive coronary artery disease (CAD) and to determine the impact of baseline statin and aspirin use on mortality. Coronary computed tomographic angiography permits direct visualization of nonobstructive CAD. To date, the prognostic implications of nonobstructive CAD and the potential benefit of directing therapy based on nonobstructive CAD have not been carefully examined. A total of 27 125 consecutive patients who underwent computed tomographic angiography (12 enrolling centers and 6 countries) were prospectively entered into the COronary CT Angiography EvaluatioN For Clinical Outcomes: An InteRnational Multicenter (CONFIRM) registry. Patients, without history of previous CAD or obstructive CAD, for whom baseline statin and aspirin use was available were analyzed. Each coronary segment was classified as normal or nonobstructive CAD (1%-49% stenosis). Patients were followed up for a median of 27.2 months for all-cause mortality. The study comprised 10 418 patients (5712 normal and 4706 with nonobstructive CAD). In multivariable analyses, patients with nonobstructive CAD had a 6% (95% confidence interval, 1%-12%) higher risk of mortality for each additional segment with nonobstructive plaque (P=0.021). Baseline statin use was associated with a reduced risk of mortality (hazard ratio, 0.44; 95% confidence interval, 0.28-0.68; P=0.0003), a benefit that was present for individuals with nonobstructive CAD (hazard ratio, 0.32; 95% confidence interval, 0.19-0.55; P<0.001) but not for those without plaque (hazard ratio, 0.66; 95% confidence interval, 0.30-1.43; P=0.287). When stratified by National Cholesterol Education Program/Adult Treatment Program III, no mortality benefit was observed in individuals without plaque. Aspirin use was not associated with mortality benefit, irrespective of the status of plaque. The presence and extent of nonobstructive CAD predicted mortality. Baseline statin therapy was associated with a significant reduction in mortality for individuals with nonobstructive CAD but not for individuals without CAD. URL: http://clinicaltrials.gov/. Unique identifier NCT01443637. © 2015 American Heart Association, Inc.

The design and conduct of a community-based registry and biorepository: a focus on cardiometabolic health in Latinos.

PubMed

Shaibi, Gabriel Q; Coletta, Dawn K; Vital, Veronica; Mandarino, Lawrence J

2013-12-01

Latinos are disproportionately impacted by obesity and type 2 diabetes but remain underrepresented in biomedical research. Therefore, the purpose of this project was to develop a research registry and biorepository to examine cardiometabolic disease risk in the Latino community of Phoenix, Arizona. The overarching goal was to establish the research infrastructure that would encourage transdisciplinary research regarding the biocultural mechanisms of obesity-related health disparities and facilitate access to this research for the Latino community. Prior to recruitment, key stakeholders from the local Latino community were engaged to develop a broad rapport within the community and seek advice regarding recruitment, enrollment, and follow-up. Self-identified community-dwelling Latinos underwent a comprehensive cardiometabolic health assessment that included anthropometrics, a fasting laboratory panel, and a 2-hour oral glucose tolerance test with measures of insulin and glucose to estimate insulin action and secretion. Separate consent was requested for future contact and banking of serum, DNA, and RNA. Research collaborations were sought out based on the cultural and metabolic profile of participants, faculty research agendas, and the potential for generating hypotheses. A total of 667 participants (20.4% children, and 79.6% adults) were enrolled with 97% consenting to the registry and 94% to banking of samples. The prevalence of overweight/obesity was 50% in children and 81% in adults. Nearly 20% of children and more than 45% of the adults exhibited some degree of hyperglycemia. To date, more than 15 research projects have been supported through this infrastructure and have included projects on the molecular biology of insulin resistance to the sociocultural determinants of health behaviors and outcomes. The high prevalence of obesity and cardiometabolic disease risk factors coupled with the overwhelming majority of participants consenting to be re-contacted, highlights the importance of supporting research infrastructure to generate hypotheses about obesity-related health in Latinos. Future studies that stem from the initial project will likely advance the limited understanding regarding the biocultural determinants of health disparities in the Latino community. © 2013 Wiley Periodicals, Inc.

The Design and Conduct of a Community‐Based Registry and Biorepository: A Focus on Cardiometabolic Health in Latinos

PubMed Central

Coletta, Dawn K.; Vital, Veronica; Mandarino, Lawrence J.

2013-01-01

Abstract Background Latinos are disproportionately impacted by obesity and type 2 diabetes but remain underrepresented in biomedical research. Therefore, the purpose of this project was to develop a research registry and biorepository to examine cardiometabolic disease risk in the Latino community of Phoenix, Arizona. The overarching goal was to establish the research infrastructure that would encourage transdisciplinary research regarding the biocultural mechanisms of obesity‐related health disparities and facilitate access to this research for the Latino community. Methods Prior to recruitment, key stakeholders from the local Latino community were engaged to develop a broad rapport within the community and seek advice regarding recruitment, enrollment, and follow‐up. Self‐identified community‐dwelling Latinos underwent a comprehensive cardiometabolic health assessment that included anthropometrics, a fasting laboratory panel, and a 2‐hour oral glucose tolerance test with measures of insulin and glucose to estimate insulin action and secretion. Separate consent was requested for future contact and banking of serum, DNA, and RNA. Research collaborations were sought out based on the cultural and metabolic profile of participants, faculty research agendas, and the potential for generating hypotheses. Results A total of 667 participants (20.4% children, and 79.6% adults) were enrolled with 97% consenting to the registry and 94% to banking of samples. The prevalence of overweight/obesity was 50% in children and 81% in adults. Nearly 20% of children and more than 45% of the adults exhibited some degree of hyperglycemia. To date, more than 15 research projects have been supported through this infrastructure and have included projects on the molecular biology of insulin resistance to the sociocultural determinants of health behaviors and outcomes. Conclusions The high prevalence of obesity and cardiometabolic disease risk factors coupled with the overwhelming majority of participants consenting to be re‐contacted, highlights the importance of supporting research infrastructure to generate hypotheses about obesity‐related health in Latinos. Future studies that stem from the initial project will likely advance the limited understanding regarding the biocultural determinants of health disparities in the Latino community. PMID:24119012

76 FR 4092 - National Saltwater Angler Registry Program

Federal Register 2010, 2011, 2012, 2013, 2014

2011-01-24

..., Florida and Louisiana as exempted States for anglers, spear fishers and for-hire fishing vessels. NMFS has... fishers. DATES: The designation of the States as exempted States is effective on January 24, 2011. [[Page... exempted States only for anglers and spear fishers: Massachusetts, Maryland, and Virginia. Massachusetts...

Economic evaluation of Mumbai and its satellite cancer registries: Implications for expansion of data collection☆

PubMed Central

Koyande, Shravani; Subramanian, Sujha; Edwards, Patrick; Hoover, Sonja; Deshmane, Vinay; Tankga, Florence; Dikshit, Rajesh; Saraiya, Mona

2018-01-01

Background The Mumbai Cancer Registry is a population-based cancer registry that has been in operation for more than five decades and has successfully initiated and integrated three satellite registries in Pune, Nagpur, and Aurangabad, each covering specific urban populations of the Indian state Maharashtra. Data collectors at the satellites perform data abstraction, but Mumbai carries out all other core registration activities such as data analysis and quality assurance. Each of the three satellite registries follows the same data collection methodology as the main Mumbai Cancer Registry. This study examines the cost of operating the Mumbai and its satellite cancer registries. Methods We modified and used the Centers for Disease Control and Prevention’s (CDC’s) International Registry Costing Tool (IntRegCosting Tool) to collect cost and resource use data for the Mumbai Cancer Registry and three satellites. Results Almost 60% of the registration expenditure was borne by the Indian Cancer Society, which hosts the Mumbai Cancer Registry, and more than half of the registry expenditure was related to data collection activities. Across the combined registries, 93% of the expenditure was spent on labor. Overall, registration activities had a low cost per case of 226.10 Indian rupees (or a little less than 4.00 US dollars in 2014 [used average exchange rate in 2014: 1 US $ = 60 Indian rupees]). Conclusion The centralization of fixed-cost activities in Mumbai likely resulted in economies of scale in operating the Mumbai and satellite registries, which, together, report on almost 20,000 cancer cases annually. In middle-income countries like India, where financial resources are limited, the operational framework provided by the Mumbai and satellite registries can serve as a model for other registries looking to expand data collection. PMID:27726981

Economic evaluation of Mumbai and its satellite cancer registries: Implications for expansion of data collection.

PubMed

Koyande, Shravani; Subramanian, Sujha; Edwards, Patrick; Hoover, Sonja; Deshmane, Vinay; Tankga, Florence; Dikshit, Rajesh; Saraiya, Mona

2016-12-01

The Mumbai Cancer Registry is a population-based cancer registry that has been in operation for more than five decades and has successfully initiated and integrated three satellite registries in Pune, Nagpur, and Aurangabad, each covering specific urban populations of the Indian state Maharashtra. Data collectors at the satellites perform data abstraction, but Mumbai carries out all other core registration activities such as data analysis and quality assurance. Each of the three satellite registries follows the same data collection methodology as the main Mumbai Cancer Registry. This study examines the cost of operating the Mumbai and its satellite cancer registries. We modified and used the Centers for Disease Control and Prevention's (CDC's) International Registry Costing Tool (IntRegCosting Tool) to collect cost and resource use data for the Mumbai Cancer Registry and three satellites. Almost 60% of the registration expenditure was borne by the Indian Cancer Society, which hosts the Mumbai Cancer Registry, and more than half of the registry expenditure was related to data collection activities. Across the combined registries, 93% of the expenditure was spent on labor. Overall, registration activities had a low cost per case of 226.10 Indian rupees (or a little less than 4.00 US dollars in 2014 [used average exchange rate in 2014: 1 US $=60 Indian rupees]). The centralization of fixed-cost activities in Mumbai likely resulted in economies of scale in operating the Mumbai and satellite registries, which, together, report on almost 20,000 cancer cases annually. In middle-income countries like India, where financial resources are limited, the operational framework provided by the Mumbai and satellite registries can serve as a model for other registries looking to expand data collection. Copyright © 2016 Elsevier Ltd. All rights reserved.

Delay in Recognition of Pulmonary Arterial Hypertension

PubMed Central

Brown, Lynette M.; Chen, Hubert; Halpern, Scott; Taichman, Darren; McGoon, Michael D.; Farber, Harrison W.; Frost, Adaani E.; Liou, Theodore G.; Turner, Michelle; Feldkircher, Kathy; Miller, Dave P.

2011-01-01

Background: Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder. Despite the emergence of effective therapy, PAH is commonly at an advanced stage when recognized. Factors associated with a prolonged symptomatic period before the recognition of PAH have not been fully evaluated. Methods: The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) enrolled 2,967 US adult patients with PAH from March 2006 to September 2007. Patients were considered to have delayed disease recognition if > 2 years elapsed between symptom onset and the patient receiving a PAH diagnosis, starting on PAH-specific therapy, or receiving a diagnosis by right-sided heart catheterization. Results: In 21.1% of patients, symptoms were experienced for > 2 years before PAH was recognized. Patients with onset of PAH symptoms before age 36 years showed the highest likelihood of delayed disease recognition (OR, 3.07; 95% CI, 2.03-4.66). History of obstructive airways disease (OR, 1.93; 95% CI, 1.5-2.47) and sleep apnea (OR, 1.72; 95% CI, 1.33-2.22) were independently associated with delayed PAH recognition. Six-minute walk distance < 250 m (OR, 1.91; 95% CI, 1.16-3.13), right atrial pressure < 10 mm Hg (OR, 1.77; 95% CI, 1.26-2.48), and pulmonary vascular resistance < 10 Wood units (OR, 1.28; 95% CI, 1.02-1.60) were also associated with delayed disease recognition, but sex, race/ethnicity, and geographic region showed no association. Conclusions: One in five patients in the REVEAL Registry who were diagnosed with PAH reported symptoms for > 2 years before their disease was recognized. Younger individuals and patients with histories of common respiratory disorders were most likely to experience delayed PAH recognition. Trial registry: ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov PMID:21393391

Chinese SLE Treatment and Research group (CSTAR) registry: II. Prevalence and risk factors of pulmonary arterial hypertension in Chinese patients with systemic lupus erythematosus.

PubMed

Li, M; Wang, Q; Zhao, J; Li, Z; Ye, Z; Li, C; Li, X; Zhu, P; Wang, Z; Zheng, Y; Li, X; Zhang, M; Tian, Z; Liu, Y; He, J; Zhang, F; Zhao, Y; Zeng, X

2014-09-01

To estimate the prevalence of pulmonary arterial hypertension (PAH) and risk factors for PAH in patients registered in the Chinese SLE Treatment and Research group (CSTAR) database, the first online registry of Chinese patients with systemic lupus erythematosus (SLE). A prospective cross-sectional study of patients with SLE was conducted using the CSTAR registry. Resting transthoracic echocardiography was used to estimate pulmonary artery pressure (PAP); PAH was defined as systolic PAP (PASP)≥40 mmHg. Patients with interstitial lung disease, valvular disease or cardiomyopathy were excluded because of disease influence on PAP. We explored potential risk factors for PAH including patient characteristics, organ involvement, laboratory findings and SLE disease activity. Of 1934 patients with SLE, 74 had PASP with 54.2±17.1 (40,106) mmHg and were diagnosed with probable PAH. The incidences of lupus nephritis, pleuritis, pericarditis, hypocomplementemia, anti-SSA, and anti-ribonucleoprotein (RNP) were significantly higher in patients with PAH than in those without (p<0.05). SLE disease activity was significantly higher in patients with PAH than in unaffected patients (p<0.05). Multivariate analysis indicated that pericarditis (odds ratio (OR)=4.248), pleuritis (OR=3.061) and anti-RNP (OR=2.559) were independent risk factors for PAH in patients with SLE (p<0.05). The possible prevalence of PAH was 3.8% in Chinese patients with SLE in the CSTAR registry. The significant association of pericarditis, pleuritis and anti-RNP positivity with PAH suggests that higher disease activity and vasculopathy may both contribute to the development of PAH in SLE, which need be treated aggressively to improve prognosis. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

Cancer Reporting: Timeliness Analysis and Process Reengineering

ERIC Educational Resources Information Center

Jabour, Abdulrahman M.

2016-01-01

Introduction: Cancer registries collect tumor-related data to monitor incident rates and support population-based research. A common concern with using population-based registry data for research is reporting timeliness. Data timeliness have been recognized as an important data characteristic by both the Centers for Disease Control and Prevention…

Rapid Development of Specialty Population Registries and Quality Measures from Electronic Health Record Data*. An Agile Framework.

PubMed

Kannan, Vaishnavi; Fish, Jason S; Mutz, Jacqueline M; Carrington, Angela R; Lai, Ki; Davis, Lisa S; Youngblood, Josh E; Rauschuber, Mark R; Flores, Kathryn A; Sara, Evan J; Bhat, Deepa G; Willett, DuWayne L

2017-06-14

Creation of a new electronic health record (EHR)-based registry often can be a "one-off" complex endeavor: first developing new EHR data collection and clinical decision support tools, followed by developing registry-specific data extractions from the EHR for analysis. Each development phase typically has its own long development and testing time, leading to a prolonged overall cycle time for delivering one functioning registry with companion reporting into production. The next registry request then starts from scratch. Such an approach will not scale to meet the emerging demand for specialty registries to support population health and value-based care. To determine if the creation of EHR-based specialty registries could be markedly accelerated by employing (a) a finite core set of EHR data collection principles and methods, (b) concurrent engineering of data extraction and data warehouse design using a common dimensional data model for all registries, and (c) agile development methods commonly employed in new product development. We adopted as guiding principles to (a) capture data as a byproduct of care of the patient, (b) reinforce optimal EHR use by clinicians, (c) employ a finite but robust set of EHR data capture tool types, and (d) leverage our existing technology toolkit. Registries were defined by a shared condition (recorded on the Problem List) or a shared exposure to a procedure (recorded on the Surgical History) or to a medication (recorded on the Medication List). Any EHR fields needed - either to determine registry membership or to calculate a registry-associated clinical quality measure (CQM) - were included in the enterprise data warehouse (EDW) shared dimensional data model. Extract-transform-load (ETL) code was written to pull data at defined "grains" from the EHR into the EDW model. All calculated CQM values were stored in a single Fact table in the EDW crossing all registries. Registry-specific dashboards were created in the EHR to display both (a) real-time patient lists of registry patients and (b) EDW-generated CQM data. Agile project management methods were employed, including co-development, lightweight requirements documentation with User Stories and acceptance criteria, and time-boxed iterative development of EHR features in 2-week "sprints" for rapid-cycle feedback and refinement. Using this approach, in calendar year 2015 we developed a total of 43 specialty chronic disease registries, with 111 new EHR data collection and clinical decision support tools, 163 new clinical quality measures, and 30 clinic-specific dashboards reporting on both real-time patient care gaps and summarized and vetted CQM measure performance trends. This study suggests concurrent design of EHR data collection tools and reporting can quickly yield useful EHR structured data for chronic disease registries, and bodes well for efforts to migrate away from manual abstraction. This work also supports the view that in new EHR-based registry development, as in new product development, adopting agile principles and practices can help deliver valued, high-quality features early and often.

Rapid Development of Specialty Population Registries and Quality Measures from Electronic Health Record Data.

PubMed

Kannan, Vaishnavi; Fish, Jason S; Mutz, Jacqueline M; Carrington, Angela R; Lai, Ki; Davis, Lisa S; Youngblood, Josh E; Rauschuber, Mark R; Flores, Kathryn A; Sara, Evan J; Bhat, Deepa G; Willett, DuWayne L

2017-01-01

Creation of a new electronic health record (EHR)-based registry often can be a "one-off" complex endeavor: first developing new EHR data collection and clinical decision support tools, followed by developing registry-specific data extractions from the EHR for analysis. Each development phase typically has its own long development and testing time, leading to a prolonged overall cycle time for delivering one functioning registry with companion reporting into production. The next registry request then starts from scratch. Such an approach will not scale to meet the emerging demand for specialty registries to support population health and value-based care. To determine if the creation of EHR-based specialty registries could be markedly accelerated by employing (a) a finite core set of EHR data collection principles and methods, (b) concurrent engineering of data extraction and data warehouse design using a common dimensional data model for all registries, and (c) agile development methods commonly employed in new product development. We adopted as guiding principles to (a) capture data as a byproduct of care of the patient, (b) reinforce optimal EHR use by clinicians, (c) employ a finite but robust set of EHR data capture tool types, and (d) leverage our existing technology toolkit. Registries were defined by a shared condition (recorded on the Problem List) or a shared exposure to a procedure (recorded on the Surgical History) or to a medication (recorded on the Medication List). Any EHR fields needed - either to determine registry membership or to calculate a registry-associated clinical quality measure (CQM) - were included in the enterprise data warehouse (EDW) shared dimensional data model. Extract-transform-load (ETL) code was written to pull data at defined "grains" from the EHR into the EDW model. All calculated CQM values were stored in a single Fact table in the EDW crossing all registries. Registry-specific dashboards were created in the EHR to display both (a) real-time patient lists of registry patients and (b) EDW-gener-ated CQM data. Agile project management methods were employed, including co-development, lightweight requirements documentation with User Stories and acceptance criteria, and time-boxed iterative development of EHR features in 2-week "sprints" for rapid-cycle feedback and refinement. Using this approach, in calendar year 2015 we developed a total of 43 specialty chronic disease registries, with 111 new EHR data collection and clinical decision support tools, 163 new clinical quality measures, and 30 clinic-specific dashboards reporting on both real-time patient care gaps and summarized and vetted CQM measure performance trends. This study suggests concurrent design of EHR data collection tools and reporting can quickly yield useful EHR structured data for chronic disease registries, and bodes well for efforts to migrate away from manual abstraction. This work also supports the view that in new EHR-based registry development, as in new product development, adopting agile principles and practices can help deliver valued, high-quality features early and often. Schattauer GmbH.

Feasibility of creating a National ALS Registry using administrative data in the United States

PubMed Central

KAYE, WENDY E.; SANCHEZ, MARCHELLE; WU, JENNIFER

2015-01-01

Uncertainty about the incidence and prevalence of amyotrophic lateral sclerosis (ALS), as well as the role of the environment in the etiology of ALS, supports the need for a surveillance system/registry for this disease. Our aim was to evaluate the feasibility of using existing administrative data to identify cases of ALS. The Agency for Toxic Substances and Disease Registry (ATSDR) funded four pilot projects at tertiary care facilities for ALS, HMOs, and state based organizations. Data from Medicare, Medicaid, the Veterans Health Administration, and Veterans Benefits Administration were matched to data available from site-specific administrative and clinical databases for a five-year time-period (1 January 2001–31 December 2005). Review of information in the medical records by a neurologist was considered the gold standard for determining an ALS case. We developed an algorithm using variables from the administrative data that identified true cases of ALS (verified by a neurologist). Individuals could be categorized into ALS, possible ALS, and not ALS. The best algorithm had sensitivity of 87% and specificity of 85%. We concluded that administrative data can be used to develop a surveillance system/ registry for ALS. These methods can be explored for creating surveillance systems for other neurodegenerative diseases. PMID:24597459

An international registry for primary ciliary dyskinesia.

PubMed

Werner, Claudius; Lablans, Martin; Ataian, Maximilian; Raidt, Johanna; Wallmeier, Julia; Große-Onnebrink, Jörg; Kuehni, Claudia E; Haarman, Eric G; Leigh, Margaret W; Quittner, Alexandra L; Lucas, Jane S; Hogg, Claire; Witt, Michal; Priftis, Kostas N; Yiallouros, Panayiotis; Nielsen, Kim G; Santamaria, Francesca; Ückert, Frank; Omran, Heymut

2016-03-01

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course.To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59% (95% CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care. Copyright ©ERS 2016.

Predictable and SuStainable Implementation of National Cardiovascular Registries (PASSION) infrastructure: A think tank report from Medical Device Epidemiological Network Initiative (MDEpiNet).

PubMed

Zeitler, Emily P; Al-Khatib, Sana M; Drozda, Joseph P; Kessler, Larry G; Kirtane, Ajay J; Kong, David F; Laschinger, John; Marinac-Dabic, Danica; Morice, Marie-Claude; Reed, Terrie; Sedrakyan, Art; Stein, Kenneth M; Tcheng, James; Krucoff, Mitchell W

2016-01-01

The MDEpiNet is a public-private partnership between the US Food and Drug Administration's Center for Devices and Radiological Health and participating partners. The PASSION program is an MDEpiNet-sponsored program that aims to demonstrate the goals of MDEpiNet by using cardiovascular medical device registries to bridge evidence gaps across the medical device total product life cycle. To this end, a PASSION Think Tank meeting took place in October 2014 in Silver Spring, MD, to facilitate discussion between stakeholders about the successes, challenges, and future novel applications of medical device registries, with particular emphasis on identifying pilot projects. Participants spanned a broad range of groups including patients, device manufacturers, regulators, physicians/academicians, professional societies, providers, and payers. The meeting focus included 4 areas of cardiovascular medicine intended to cultivate interest in 4 MDEpiNet disease-specific/device-specific working groups: coronary intervention, electrophysiology, valvular disease, and peripheral vascular disease. In addition, more general issues applying to registry-based infrastructure and analytical methodologies for assessing device benefit/risk were considered to provide context for the working groups as PASSION programs going forward. This article summarizes the discussions at the meeting and the future directions of the PASSION program. Copyright © 2015 Elsevier Inc. All rights reserved.

The Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans: Toward Comparative Effectiveness in the Pediatric Rheumatic Diseases.

PubMed

Ringold, Sarah; Nigrovic, Peter A; Feldman, Brian M; Tomlinson, George A; von Scheven, Emily; Wallace, Carol A; Huber, Adam M; Schanberg, Laura E; Li, Suzanne C; Weiss, Pamela F; Fuhlbrigge, Robert C; Morgan, Esi M; Kimura, Yukiko

2018-05-01

The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research methods. Innovative comparative effectiveness approaches are needed to efficiently study treatment strategies and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes. To date, CTPs have been published for 8 different diseases and disease manifestations. The approach has been successfully piloted for juvenile localized scleroderma, systemic juvenile idiopathic arthritis (JIA), polyarticular JIA, dermatomyositis, and lupus nephritis. Large-scale studies are underway for systemic JIA and polyarticular JIA, with the CARRA patient registry serving as the data collection platform. These studies have been designed with stakeholder involvement, including active input from CARRA providers, patients, and parents, with the goal of increasing feasibility and ensuring the relevance of the outcomes. These studies include ancillary biologic specimen collection intended to support additional translational and mechanistic studies. Data from these ongoing CTP studies will provide more information on the ability of this approach to identify effective treatment strategies and improve outcomes in the pediatric rheumatic diseases. © 2018, American College of Rheumatology.

Towards data integration automation for the French rare disease registry.

PubMed

Maaroufi, Meriem; Choquet, Rémy; Landais, Paul; Jaulent, Marie-Christine

2015-01-01

Building a medical registry upon an existing infrastructure and rooted practices is not an easy task. It is the case for the BNDMR project, the French rare disease registry, that aims to collect administrative and medical data of rare disease patients seen in different hospitals. To avoid duplicating data entry for health professionals, the project plans to deploy connectors with the existing systems to automatically retrieve data. Given the data heterogeneity and the large number of source systems, the automation of connectors creation is required. In this context, we propose a methodology that optimizes the use of existing alignment approaches in the data integration processes. The generated mappings are formalized in exploitable mapping expressions. Following this methodology, a process has been experimented on specific data types of a source system: Boolean and predefined lists. As a result, effectiveness of the used alignment approach has been enhanced and more good mappings have been detected. Nonetheless, further improvements could be done to deal with the semantic issue and process other data types.

Towards data integration automation for the French rare disease registry

PubMed Central

Maaroufi, Meriem; Choquet, Rémy; Landais, Paul; Jaulent, Marie-Christine

2015-01-01

Building a medical registry upon an existing infrastructure and rooted practices is not an easy task. It is the case for the BNDMR project, the French rare disease registry, that aims to collect administrative and medical data of rare disease patients seen in different hospitals. To avoid duplicating data entry for health professionals, the project plans to deploy connectors with the existing systems to automatically retrieve data. Given the data heterogeneity and the large number of source systems, the automation of connectors creation is required. In this context, we propose a methodology that optimizes the use of existing alignment approaches in the data integration processes. The generated mappings are formalized in exploitable mapping expressions. Following this methodology, a process has been experimented on specific data types of a source system: Boolean and predefined lists. As a result, effectiveness of the used alignment approach has been enhanced and more good mappings have been detected. Nonetheless, further improvements could be done to deal with the semantic issue and process other data types. PMID:26958224

Ubiquitous Nature of Distal Athero/Thromboembolic Events during Lower Extremity Atherectomy Procedures Involving the Superficial Femoral Artery

PubMed Central

Wasty, Najam; Khakwani, M. Z.; Kotev, Spas; Boiangiu, Catalin; Hasan, Omar; Anna, Manjusha; Tayal, Rajiv; Kaid, Khalil; Baker, Gail; Cohen, Marc

2016-01-01

This study aims to evaluate occurrence, size, composition, and clinical significance of embolized debris during superficial femoral artery atherectomy using all commercially available atherectomy devices. Distal athero/thromboembolic events (DATE) are a universal phenomenon in lower extremity atherectomy procedures (LEAPs) due to the sheer volume of atheroma and the thrombus burden in peripheral arterial disease. Some of these events can be clinically significant. We prospectively gathered clinical and histopathological data on all commercially available atherectomy devices by using embolic protection devices (EPD) in every case. After intervention, the contents of EPD were examined both microscopically and macroscopically. Data from 59 consecutive patients undergoing LEAP were analyzed. DATE occurred 100% of the time. The composition of particulate debris varied with the device used. Grossly visible agglomerated debris was captured by the filter in the majority of patients 54/59 (91.5%). Clinically significant debris, defined by the Preventing Lower Extremity Distal Embolization Using Embolic Filter Protection registry as particle diameter > 0.2 cm, was found in 33/59 (56%) patients. The size of captured debris particles ranged from 0.1 to 2.4 mm. While DATE occurred in all patients, clinically significant DATE occurred in 56% patients undergoing LEAP regardless of the atherectomy device. In spite of a large fraction of the clinically significant debris occurring on our routine dual antiplatelet regimen, no patient suffered an amputation. Although DATE was prevented by the use of EPD in all 59 cases, more data are needed to determine whether the use of EPD translates into a long-term clinical benefit. Use of EPD and optimal thromboprophylaxis should be considered in patients, especially in the setting of compromised distal runoff. PMID:27867291

Sunset Dates of Chemicals Subject to Final TSCA Section 4 and Related 12(b) Actions

EPA Pesticide Factsheets

This Table lists, in ascending chemical Abstract Service (CAS) Registry number order, all chemical substances and mixtures that are and/or have been the subject of final TSCA Section 4 test rules and/or TSCA Section 4 enforceable consent agreements/orders.

Impact of Inclusion of Industry Trial Results Registries as an Information Source for Systematic Reviews

PubMed Central

Potthast, Regine; Vervölgyi, Volker; McGauran, Natalie; Kerekes, Michaela F.; Wieseler, Beate; Kaiser, Thomas

2014-01-01

Background Clinical trial results registries may contain relevant unpublished information. Our main aim was to investigate the potential impact of the inclusion of reports from industry results registries on systematic reviews (SRs). Methods We identified a sample of 150 eligible SRs in PubMed via backward selection. Eligible SRs investigated randomized controlled trials of drugs and included at least 2 bibliographic databases (original search date: 11/2009). We checked whether results registries of manufacturers and/or industry associations had also been searched. If not, we searched these registries for additional trials not considered in the SRs, as well as for additional data on trials already considered. We reanalysed the primary outcome and harm outcomes reported in the SRs and determined whether results had changed. A “change” was defined as either a new relevant result or a change in the statistical significance of an existing result. We performed a search update in 8/2013 and identified a sample of 20 eligible SRs to determine whether mandatory results registration from 9/2008 onwards in the public trial and results registry ClinicalTrials.gov had led to its inclusion as a standard information source in SRs, and whether the inclusion rate of industry results registries had changed. Results 133 of the 150 SRs (89%) in the original analysis did not search industry results registries. For 23 (17%) of these SRs we found 25 additional trials and additional data on 31 trials already included in the SRs. This additional information was found for more than twice as many SRs of drugs approved from 2000 as approved beforehand. The inclusion of the additional trials and data yielded changes in existing results or the addition of new results for 6 of the 23 SRs. Of the 20 SRs retrieved in the search update, 8 considered ClinicalTrials.gov or a meta-registry linking to ClinicalTrials.gov, and 1 considered an industry results registry. Conclusion The inclusion of industry and public results registries as an information source in SRs is still insufficient and may result in publication and outcome reporting bias. In addition to an essential search in ClinicalTrials.gov, authors of SRs should consider searching industry results registries. PMID:24743113

Registries in European post-marketing surveillance: a retrospective analysis of centrally approved products, 2005-2013.

PubMed

Bouvy, Jacoline C; Blake, Kevin; Slattery, Jim; De Bruin, Marie L; Arlett, Peter; Kurz, Xavier

2017-12-01

Regulatory agencies and other stakeholders increasingly rely on data collected through registries to support their decision-making. Data from registries are a cornerstone of post-marketing surveillance for monitoring the use of medicines in clinical practice. This study was aimed at gaining further insight into the European Medicines Agency's (EMA) requests for new registries and registry studies using existing registries and to review the experience gained in their conduct. European Public Assessment Reports were consulted to identify products for which a request for a registry was made as a condition of the marketing authorisation. All centrally authorised products that received a positive opinion of the EMA Committee for Medicinal Products for Human Use between 1 January 2005 and 31 December 2013 were included. Data regarding registry design and experiences were collected from EMA electronic record keeping systems. Of 392 products that received a positive Committee for Medicinal Products for Human Use opinion during 2005-2013, 31 registries were requested for 30 products in total. Sixty-five percent were product registries whereas 35% were disease registries and 71% of the registries had a primary safety objective. Most commonly reported issues with registries were delayed time to start and low patient accrual rates. The delays found in getting new registries up and running support the need to improve the timeliness of data collection in the post-marketing setting. Methodological challenges met in conducting this study highlighted the need for a clarification of definitions and epidemiological concepts around patient registries. The results will inform the EMA Patient Registry initiative to support use of existing patient registries for the post-authorisation benefit-risk monitoring of medicinal products. © 2017 Commonwealth of Australia. Pharmacoepidemiology & Drug Safety © 2017 John Wiley & Sons, Ltd. © 2017 Commonwealth of Australia. Pharmacoepidemiology & Drug Safety © 2017 John Wiley & Sons, Ltd.

Decision counseling and participation in a pancreas cancer registry.

PubMed

Myers, Ronald; Lavu, Harish; Keith, Scott W; Kelly, Heidi; O'Rourke, Nadine; Cocroft, James; Quinn, Anna; Potluri, Vishnu; Yeo, Charles J

2014-01-01

Cancer registries play a vital role in research, as they provide important data that can be used to assess disease etiology and risk. Specialty registries can help to address the need for information on defined cancer types. However, achieving high rates of participation in such registries is problematic.We studied the impact of decision support on patient participation in a hospital-based pancreas cancer registry, the Jefferson Pancreas Tumor Registry (JPTR). In this study, we assembled a nonrandomized cohort of 40 patients, of whom 20 were exposed to the intervention and 20 were exposed to routine recruiting methods. Patients in the control group were invited to join the JPTR; while those in the intervention group were also invited to join the JPTR, and received decision support related to participation. Registry participation was assessed at 90 days. At baseline, patient gender, race, and stage of pancreatic cancer did not vary significantly between study groups. Overall, participation in the intervention group was significantly higher (P = 0.01) than in the control group (55% and 10%, respectively). In the intervention group, altruism was the major factor motivating patient participation, while patient concerns related to treatment recovery, registration time and complexity, and the confidentiality of registry data discouraged participation.

Nordic Cancer Registries - an overview of their procedures and data comparability.

PubMed

Pukkala, Eero; Engholm, Gerda; Højsgaard Schmidt, Lise Kristine; Storm, Hans; Khan, Staffan; Lambe, Mats; Pettersson, David; Ólafsdóttir, Elínborg; Tryggvadóttir, Laufey; Hakanen, Tiina; Malila, Nea; Virtanen, Anni; Johannesen, Tom Børge; Larønningen, Siri; Ursin, Giske

2018-04-01

The Nordic Cancer Registries are among the oldest population-based registries in the world, with more than 60 years of complete coverage of what is now a combined population of 26 million. However, despite being the source of a substantial number of studies, there is no published paper comparing the different registries. Therefore, we did a systematic review to identify similarities and dissimilarities of the Nordic Cancer Registries, which could possibly explain some of the differences in cancer incidence rates across these countries. We describe and compare here the core characteristics of each of the Nordic Cancer Registries: (i) data sources; (ii) registered disease entities and deviations from IARC multiple cancer coding rules; (iii) variables and related coding systems. Major changes over time are described and discussed. All Nordic Cancer Registries represent a high quality standard in terms of completeness and accuracy of the registered data. Even though the information in the Nordic Cancer Registries in general can be considered more similar than any other collection of data from five different countries, there are numerous differences in registration routines, classification systems and inclusion of some tumors. These differences are important to be aware of when comparing time trends in the Nordic countries.

High Mortality without ESCAPE: The Registry of Heart Failure Patients Receiving Pulmonary Artery Catheters without Randomization

PubMed Central

Allen, Larry A.; Rogers, Joseph G.; Warnica, J. Wayne; DiSalvo, Thomas G.; Tasissa, Gudaye; Binanay, Cynthia; O’Connor, Christopher M.; Califf, Robert M.; Leier, Carl V.; Shah, Monica R.; Stevenson, Lynne W.

2008-01-01

Background In ESCAPE, there was no difference in days alive and out of the hospital for patients with decompensated heart failure (HF) randomly assigned to therapy guided by pulmonary artery catheter (PAC) plus clinical assessment versus clinical assessment alone. The external validity of these findings is debated. Methods and Results ESCAPE sites enrolled 439 patients receiving PAC without randomization in a prospective registry. Baseline characteristics, pertinent trial exclusion criteria, reasons for PAC use, hemodynamics, and complications were collected. Survival was determined from the National Death Index and the Alberta Registry. On average, registry patients had lower blood pressure, worse renal function, less neurohormonal antagonist therapy, and higher use of intravenous inotropes as compared with trial patients. Although clinical assessment anticipated less volume overload and greater hypoperfusion among the registry population, measured filling pressures were similarly elevated in the registry and trial, while measured perfusion was slightly higher among registry patients. Registry patients had longer hospitalization (13 vs. 6 days, p <0.001) and higher 6-month mortality (34% vs. 20%, p < 0.001) than trial patients. Conclusions The decision to use PAC without randomization identified a population with higher disease severity and risk of mortality. This prospective registry highlights the complex context of patient selection for randomized trials. PMID:18926438

Reuma.pt contribution to the knowledge of immune-mediated systemic rheumatic diseases.

PubMed

Santos, Maria José; Canhão, Helena; Mourão, Ana Filipa; Oliveira Ramos, Filipa; Ponte, Cristina; Duarte, Cátia; Barcelos, Anabela; Martins, Fernando; Melo Gomes, José António

2017-01-01

Patient registries are key instruments aimed at a better understanding of the natural history of diseases, at assessing the effectiveness of therapeutic interventions, as well as identifying rare events or outcomes that are not captured in clinical trials. However, the potential of registries goes far beyond these aspects. For example, registries promote the standardization of clinical practice, can also provide information on domains that are not routinely collected in clinical practice and can support decision-making. Being aware of the importance of registries, the Portuguese Society of Rheumatology developed the Rheumatic Diseases Portuguese Register- Reuma.pt - which proved to be an innovative instrument essential to a better understanding of systemic immune-mediated rheumatic diseases. To describe the contribution of Reuma.pt to the knowledge of systemic immune-mediated rheumatic diseases. Reuma.pt is widely implemented, with 77 centres actively contributing to the recruitment and follow-up of patients. Reuma.pt follows in a standardized way patients with the following systemic inflammatory rheumatic diseases: rheumatoid arthritis (n=6218), psoriatic arthritis (n=1498), spondyloarthritis (n=2529), juvenile idiopathic arthritis (n =1561), autoinflammatory syndromes (n=122), systemic lupus erythematosus (n =1718), systemic sclerosis (n=180) and vasculitis (n=221). This platform is intended for use as an electronic medical record, provides standardized assessment of patients and support to the clinical decision, thereby contributing to a better quality of care of rheumatic patients. The research based on Reuma.pt identified genetic determinants of susceptibility and response to therapy, characterized in detail systemic rheumatic diseases and their long-term impact, critically appraised the performance of instruments for monitoring the disease activity, established the effectiveness and safety of biologic therapies and identified predictors of response, and proactively engaged patients in the management of their disease. Reuma.pt is an innovative tool, widely established in the country that contributes to a clinical practice of excellence and simultaneously to increase the knowledge of systemic immune-mediated rheumatic diseases. Additionally, Reuma.pt fosters patients' participation in the management of the disease.

A Global Cancer Surveillance Framework Within Noncommunicable Disease Surveillance: Making the Case for Population-Based Cancer Registries.

PubMed

Piñeros, Marion; Znaor, Ariana; Mery, Les; Bray, Freddie

2017-01-01

The growing burden of cancer among several major noncommunicable diseases (NCDs) requires national implementation of tailored public health surveillance. For many emerging economies where emphasis has traditionally been placed on the surveillance of communicable diseases, it is critical to understand the specificities of NCD surveillance and, within it, of cancer surveillance. We propose a general framework for cancer surveillance that permits monitoring the core components of cancer control. We examine communalities in approaches to the surveillance of other major NCDs as well as communicable diseases, illustrating key differences in the function, coverage, and reporting in each system. Although risk factor surveys and vital statistics registration are the foundation of surveillance of NCDs, population-based cancer registries play a unique fundamental role specific to cancer surveillance, providing indicators of population-based incidence and survival. With an onus now placed on governments to collect these data as part of the monitoring of NCD targets, the integration of cancer registries into existing and future NCD surveillance strategies is a vital requirement in all countries worldwide. The Global Initiative for Cancer Registry Development, endorsed by the World Health Organization, provides a means to enhance cancer surveillance capacity in low- and middle-income countries. © The Author 2017. Published by Oxford University Press on behalf of the Johns Hopkins Bloomberg School of Public Health. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry.

PubMed

Denton, Christopher P; Krieg, Thomas; Guillevin, Loic; Schwierin, Barbara; Rosenberg, Daniel; Silkey, Mariabeth; Zultak, Maurice; Matucci-Cerinic, Marco

2012-05-01

The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). The DUO Registry is a European, prospective, multicentre, observational, registry of SSc patients with ongoing digital ulcer disease, irrespective of treatment regimen. Data collected included demographics, SSc duration, SSc subset, internal organ manifestations, autoantibodies, previous and ongoing interventions and complications related to digital ulcers. Up to 19 November 2010 a total of 2439 patients had enrolled into the registry. Most were classified as either limited cutaneous SSc (lcSSc; 52.2%) or diffuse cutaneous SSc (dcSSc; 36.9%). Digital ulcers developed earlier in patients with dcSSc compared with lcSSc. Almost all patients (95.7%) tested positive for antinuclear antibodies, 45.2% for anti-scleroderma-70 and 43.6% for anticentromere antibodies (ACA). The first digital ulcer in the anti-scleroderma-70-positive patient cohort occurred approximately 5 years earlier than the ACA-positive patient group. This study provides data from a large cohort of SSc patients with a history of digital ulcers. The early occurrence and high frequency of digital ulcer complications are especially seen in patients with dcSSc and/or anti-scleroderma-70 antibodies.

Adoption and implementation of mandated diabetes registries by community health centers.

PubMed

Helfrich, Christian D; Savitz, Lucy A; Swiger, Kathleen D; Weiner, Bryan J

2007-07-01

Innovations adopted by healthcare organizations are often externally mandated. However, few studies examine how mandated innovations progress from adoption to sustained effective use. This study uses Rogers's model of organizational innovation to explore community health centers' (CHCs') mandated adoption and implementation of disease registries in the federal Health Disparities Collaborative (HDC). Case studies were conducted on six CHCs in North Carolina participating in the HDC on type 2 diabetes mellitus. Data were collected from semistructured interviews with key staff, and from site-level and individual-level surveys. Although disease registry adoption and implementation were mandated, CHCs exercised prerogative in the timing of registry adoption and the functions emphasized. Executive and medical director involvement, often directly on the HDC teams, was the single most salient influence on adoption and implementation. Staff members' personal experience with diabetes also provided context and gave registries added significance. Participants lauded HDC's technique of small-scale, rapid-cycle change, but valued even more shared problem solving and peer learning among HDC teams. However, lack of cross-training, inadequate resources, and staff turnover posed serious threats to sustainability of the registries. The present study illustrates the usefulness of Rogers's model for studying mandated innovation and highlights several key factors, including direct, personal involvement of organizational leadership, and shared problem solving and peer learning facilitated by the HDC. However, these six CHCs elected to participate early in the HDC, and may not be typical of North Carolina's remaining CHCs. Furthermore, most face important long-term challenges that threaten routinization.

The Mataró Stroke Registry: a 10-year registry in a community hospital.

PubMed

Palomeras Soler, E; Fossas Felip, P; Casado Ruiz, V; Cano Orgaz, A; Sanz Cartagena, P; Muriana Batiste, D

2015-06-01

A prospective stroke registry leads to improved knowledge of the disease. We present data on the Mataró Hospital Registry. In February-2002 a prospective stroke registry was initiated in our hospital. It includes sociodemographic data, previous diseases, clinical, topographic, etiological and prognostic data. We have analyzed the results of the first 10 years. A total of 2,165 patients have been included, 54.1% male, mean age 73 years. The most frequent vascular risk factor was hypertension (65.4%). Median NIHSS on admission: 3 (interquartile range, 1-8). Stroke subtype: 79.7% ischemic strokes, 10.9% hemorrhagic, and 9.4% TIA. Among ischemic strokes, the etiology was cardioembolic in 26.5%, large-vessel disease in 23.7%, and small-vessel in 22.9%. The most frequent topography of hemorrhages was lobar (47.4%), and 54.8% were attributed to hypertension. The median hospital stay was 8 days. At discharge, 60.7% of patients were able to return directly to their own home, and 52.7% were independent for their daily life activities. After 3 months these percentages were 76.9% and 62.9%, respectively. Hospital mortality was 6.5%, and after 3 months 10.9%. Our patient's profile is similar to those of other series, although the severity of strokes was slightly lower. Length of hospital stay, short-term and medium term disability, and mortality rates are good, if we compare them with other series. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.

Objectives and methodology of BIOBADASER phase iii.

PubMed

Sanchez-Piedra, Carlos; Hernández Miguel, M Victoria; Manero, Javier; Roselló, Rosa; Sánchez-Costa, Jesús Tomás; Rodríguez-Lozano, Carlos; Campos, Cristina; Cuende, Eduardo; Fernández-Lopez, Jesús Carlos; Bustabad, Sagrario; Martín Domenech, Raquel; Pérez-Pampín, Eva; Del Pino-Montes, Javier; Millan-Arcineas, Ana Milena; Díaz-González, Federico; Gómez-Reino, Juan Jesús

2017-09-18

Describe the objectives, methods and results of the first year of the new version of the Spanish registry of adverse events involving biological therapies and synthetic drugs with an identifiable target in rheumatic diseases (BIOBADASER III). Multicenter prospective registry of patients with rheumatic inflammatory diseases being treated with biological drugs or synthetic drugs with an identifiable target in rheumatology departments in Spain. The main objective of BIOBADASER Phase III is the registry and analysis of adverse events; moreover, a secondary objective was added consisting of assessing the effectiveness by means of the registry of activity indexes. Patients in the registry are evaluated at least once every year and whenever they experience an adverse event or a change in treatment. The collection of data for phase iii began on 17 December 2015. During the first year, 35 centers participated. The number of patients included in this new phase in December 2016 was 2,664. The mean age was 53.7 years and the median duration of treatment was 8.1 years. In all, 40.4% of the patients were diagnosed with rheumatoid arthritis. The most frequent adverse events were infections and infestations. BIOBADASER Phase III has been launched to adapt to a changing pharmacological environment, with the introduction of biosimilars and small molecules in the treatment of rheumatic diseases. This new stage is adapted to the changes in the reporting of adverse events and now includes information related to activity scores. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

Patient registries: useful tools for clinical research in myasthenia gravis.

PubMed

Baggi, Fulvio; Mantegazza, Renato; Antozzi, Carlo; Sanders, Donald

2012-12-01

Clinical registries may facilitate research on myasthenia gravis (MG) in several ways: as a source of demographic, clinical, biological, and immunological data on large numbers of patients with this rare disease; as a source of referrals for clinical trials; and by allowing rapid identification of MG patients with specific features. Physician-derived registries have the added advantage of incorporating diagnostic and treatment data that may allow comparison of outcomes from different therapeutic approaches, which can be supplemented with patient self-reported data. We report the demographic analysis of MG patients in two large physician-derived registries, the Duke MG Patient Registry, at the Duke University Medical Center, and the INNCB MG Registry, at the Istituto Neurologico Carlo Besta, as a preliminary study to assess the consistency of the two data sets. These registries share a common structure, with an inner core of common data elements (CDE) that facilitate data analysis. The CDEs are concordant with the MG-specific CDEs developed under the National Institute of Neurological Disorders and Stroke Common Data Elements Project. © 2012 New York Academy of Sciences.

Effects of the length of central cancer registry operations on identification of subsequent cancers and on survival estimates.

PubMed

Qiao, Baozhen; Schymura, Maria J; Kahn, Amy R

2016-10-01

Population-based cancer survival analyses have traditionally been based on the first primary cancer. Recent studies have brought this practice into question, arguing that varying registry reference dates affect the ability to identify earlier cancers, resulting in selection bias. We used a theoretical approach to evaluate the extent to which the length of registry operations affects the classification of first versus subsequent cancers and consequently survival estimates. Sequence number central was used to classify tumors from the New York State Cancer Registry, diagnosed 2001-2010, as either first primaries (value=0 or 1) or subsequent primaries (≥2). A set of three sequence numbers, each based on an assumed reference year (1976, 1986 or 1996), was assigned to each tumor. Percent of subsequent cancers was evaluated by reference year, cancer site and age. 5-year relative survival estimates were compared under four different selection scenarios. The percent of cancer cases classified as subsequent primaries was 15.3%, 14.3% and 11.2% for reference years 1976, 1986 and 1996, respectively; and varied by cancer site and age. When only the first primary was included, shorter registry operation time was associated with slightly lower 5-year survival estimates. When all primary cancers were included, survival estimates decreased, with the largest decreases seen for the earliest reference year. Registry operation length affected the identification of subsequent cancers, but the overall effect of this misclassification on survival estimates was small. Survival estimates based on all primary cancers were slightly lower, but might be more comparable across registries. Copyright © 2016 Elsevier Ltd. All rights reserved.

Resource Disambiguator for the Web: Extracting Biomedical Resources and Their Citations from the Scientific Literature.

PubMed

Ozyurt, Ibrahim Burak; Grethe, Jeffrey S; Martone, Maryann E; Bandrowski, Anita E

2016-01-01

The NIF Registry developed and maintained by the Neuroscience Information Framework is a cooperative project aimed at cataloging research resources, e.g., software tools, databases and tissue banks, funded largely by governments and available as tools to research scientists. Although originally conceived for neuroscience, the NIF Registry has over the years broadened in the scope to include research resources of general relevance to biomedical research. The current number of research resources listed by the Registry numbers over 13K. The broadening in scope to biomedical science led us to re-christen the NIF Registry platform as SciCrunch. The NIF/SciCrunch Registry has been cataloging the resource landscape since 2006; as such, it serves as a valuable dataset for tracking the breadth, fate and utilization of these resources. Our experience shows research resources like databases are dynamic objects, that can change location and scope over time. Although each record is entered manually and human-curated, the current size of the registry requires tools that can aid in curation efforts to keep content up to date, including when and where such resources are used. To address this challenge, we have developed an open source tool suite, collectively termed RDW: Resource Disambiguator for the (Web). RDW is designed to help in the upkeep and curation of the registry as well as in enhancing the content of the registry by automated extraction of resource candidates from the literature. The RDW toolkit includes a URL extractor from papers, resource candidate screen, resource URL change tracker, resource content change tracker. Curators access these tools via a web based user interface. Several strategies are used to optimize these tools, including supervised and unsupervised learning algorithms as well as statistical text analysis. The complete tool suite is used to enhance and maintain the resource registry as well as track the usage of individual resources through an innovative literature citation index honed for research resources. Here we present an overview of the Registry and show how the RDW tools are used in curation and usage tracking.

Resource Disambiguator for the Web: Extracting Biomedical Resources and Their Citations from the Scientific Literature

PubMed Central

Ozyurt, Ibrahim Burak; Grethe, Jeffrey S.; Martone, Maryann E.; Bandrowski, Anita E.

2016-01-01

The NIF Registry developed and maintained by the Neuroscience Information Framework is a cooperative project aimed at cataloging research resources, e.g., software tools, databases and tissue banks, funded largely by governments and available as tools to research scientists. Although originally conceived for neuroscience, the NIF Registry has over the years broadened in the scope to include research resources of general relevance to biomedical research. The current number of research resources listed by the Registry numbers over 13K. The broadening in scope to biomedical science led us to re-christen the NIF Registry platform as SciCrunch. The NIF/SciCrunch Registry has been cataloging the resource landscape since 2006; as such, it serves as a valuable dataset for tracking the breadth, fate and utilization of these resources. Our experience shows research resources like databases are dynamic objects, that can change location and scope over time. Although each record is entered manually and human-curated, the current size of the registry requires tools that can aid in curation efforts to keep content up to date, including when and where such resources are used. To address this challenge, we have developed an open source tool suite, collectively termed RDW: Resource Disambiguator for the (Web). RDW is designed to help in the upkeep and curation of the registry as well as in enhancing the content of the registry by automated extraction of resource candidates from the literature. The RDW toolkit includes a URL extractor from papers, resource candidate screen, resource URL change tracker, resource content change tracker. Curators access these tools via a web based user interface. Several strategies are used to optimize these tools, including supervised and unsupervised learning algorithms as well as statistical text analysis. The complete tool suite is used to enhance and maintain the resource registry as well as track the usage of individual resources through an innovative literature citation index honed for research resources. Here we present an overview of the Registry and show how the RDW tools are used in curation and usage tracking. PMID:26730820

Education predicts cervical cancer survival: a Lithuanian cohort study.

PubMed

Vincerževskiene, Ieva; Jasilionis, Domantas; Austys, Donatas; Stukas, Rimantas; Kaceniene, Auguste; Smailyte, Giedre

2017-06-01

: We examined inequalities in cervical cancer survival in Lithuania by education and place of residence. : The study is based on the linked dataset that includes all records of the 2001 population Census, all records from Lithuanian Cancer Registry (cancer incidence) and all death and emigration records from Statistics Lithuania for the period between 6 April 2001 and 31 December 2009. The study group includes cervical cancers registered in the Cancer Registry from 1 January 2002 to 31 December 2006. Analysis was restricted to women who were 25-64 years old at the Census date (in total 1 866 cases). : During the study period there were 671 deaths corresponding to an overall 5-year survival proportion 64.13% (95% CI 61.86-66.31). Place of residence and education of cervical cancer patients had strong impact on survival; 5-year survival was higher in women living in urban areas than in rural (68.61 and 55.93%) and survival decreased with decreasing education: from 79.77% in highest education group to 64.85 and 50.48% in groups with secondary and lower than secondary education. The effect of place of residence declined when stage of disease was included in the model and became not significant in final model with education adjustment. The effect of education declined after inclusion of stage and other variables, however, remained significant. : We found that women with higher education experienced higher survival following a cervical cancer diagnosis, and stage of disease at the time of diagnosis explains only the part of observed differences. © The Author 2017. Published by Oxford University Press on behalf of the European Public Health Association. All rights reserved.

Description of Patients Treated with Biologic Drugs as First-Line Systemic Therapy in the BIOBADADERM Registry Between 2008 and 2016.

PubMed

Carretero Hernández, G; Ferrándiz, C; Rivera Díaz, R; Daudén Tello, E; de la Cueva-Dobao, P; Gómez-García, F J; Herrera-Ceballos, E; Belinchón Romero, I; López-Estebaranz, J L; Alsina Gibert, M; Sánchez-Carazo, J L; Ferrán Farrés, M; González Quesada, A; Carrascosa Carrillo, J M; Llamas-Velasco, M; Mendiola Fernández, M V; Ruiz Genao, D; Muñoz Santos, C; García-Doval, I; Descalzo, M A

2018-06-07

Biologic drugs are usually prescribed as second-line treatment for psoriasis, that is, after the patient has first been treated with a conventional psoriasis drug. There are, however, cases where, depending on the characteristics of the patient or the judgement of the physician, biologics may be chosen as first-line therapy. No studies to date have analyzed the demographics or clinical characteristics of patients in this setting or the safety profile of the agents used. The main aim of this study was to characterize these aspects of first-line biologic therapy and compare them to those observed for patients receiving biologics as second-line therapy. We conducted an observational study of 181 patients treated in various centers with a systemic biologic drug as first-line treatment for moderate to severe psoriasis between January 2008 and November 2016. All the patients were registered in the Spanish Registry of Adverse Events Associated with Biologic Drugs in Dermatology. The characteristics of the first- and second-line groups were very similar, although the patients receiving a biologic as first-line treatment for their psoriasis were older. No differences were observed for disease severity (assessed using the PASI) or time to diagnosis. Hypertension, diabetes, and liver disease were all more common in the first-line group. There were no differences between the groups in terms of reasons for drug withdrawal or occurrence of adverse effects. No major differences were found between patients with psoriasis receiving biologic drugs as first- or second-line therapy, a finding that provides further evidence of the safety of biologic therapy in patients with psoriasis. Publicado por Elsevier España, S.L.U.

Development of the Andalusian Registry of Patients Receiving Community Case Management, for the follow-up of people with complex chronic diseases.

PubMed

Morales-Asencio, Jose M; Kaknani-Uttumchandani, Shakira; Cuevas-Fernández-Gallego, Magdalena; Palacios-Gómez, Leopoldo; Gutiérrez-Sequera, José L; Silvano-Arranz, Agustina; Batres-Sicilia, Juan Pedro; Delgado-Romero, Ascensión; Cejudo-Lopez, Ángela; Trabado-Herrera, Manuel; García-Lara, Esteban L; Martin-Santos, Francisco J; Morilla-Herrera, Juan C

2015-10-01

Complex chronic diseases are a challenge for the current configuration of health services. Case management is a service frequently provided for people with chronic conditions, and despite its effectiveness in many outcomes, such as mortality or readmissions, uncertainty remains about the most effective form of team organization, structures and the nature of the interventions. Many processes and outcomes of case management for people with complex chronic conditions cannot be addressed with the information provided by electronic clinical records. Registries are frequently used to deal with this weakness. The aim of this study was to generate a registry-based information system of patients receiving case management to identify their clinical characteristics, their context of care, events identified during their follow-up, interventions developed by case managers and services used. The study was divided into three phases, covering the detection of information needs, the design and its implementation in the health care system, using literature review and expert consensus methods to select variables that would be included in the registry. A total of 102 variables representing structure, processes and outcomes of case management were selected for their inclusion in the registry after the consensus phase. A web-based registry with modular and layered architecture was designed. The framework follows a pattern based on the model-view-controller approach. In its first 6 months after the implementation, 102 case managers have introduced an average number of 6.49 patients each one. The registry permits a complete and in-depth analysis of the characteristics of the patients who receive case management, the interventions delivered and some major outcomes as mortality, readmissions or adverse events. © 2015 John Wiley & Sons, Ltd.

The CARRA Registry

ClinicalTrials.gov

2015-11-16

Juvenile Idiopathic Arthritis; Systemic Lupus Erythematosus; Mixed Connective Tissue Disease; Juvenile Ankylosing Spondylitis; Juvenile Dermatomyositis; Localized Scleroderma; Systemic Sclerosis; Vasculitis; Sarcoid; Fibromyalgia, Primary; Auto-inflammatory Disease; Idiopathic Uveitis Idiopathic

[Pain registries and similar data collections : A systematic review].

PubMed

Freytag, A; Scriba, B; Kaiser, U; Meißner, W

2016-12-01

Registries and similar data collections are a valuable addition to prospective studies as they provide data from real life treatment. In pain medicine only few such data collections exist so far. Aim of the study was to identify German-language registries or similar data collections that record patient-reported and pain-associated outcomes together with other data. A systematic search was carried out, which included the following sources: the data bases PubMed/MEDLINE and Embase, the German Registry for Clinical Trials (DRKS), ClinicalTrials.gov and registry portals known to us. Furthermore, an extended internet search was carried out via Google Scholar. References from personal scientific contacts and from operators of registries were also included. Questionnaires regarding registry items were sent to registry operators. Out of 381 search hits, 37 potentially relevant projects received a questionnaire and 35 answered. From the 35 responders 23 registries or similar data collections fulfilling inclusion criteria could be identified: 5 primarily pain-associated, 3 therapy-associated, 2 population-associated and 13 disease-associated (rheumatism/arthritis 5, joints/spine 4, hernias 1 and cancer 3). The reader obtains contact information on relevant data collections associated with pain, the contents, objectives and the pain assessment instruments applied. This review could give an important impulse for increased networking in health services research on pain. A limitation of the study was that identification of registries was made difficult due to an inconsistent definition and application of the term "registry", incomplete or insufficiently updated registry portals, missing scientific publications as well as two non-responders.

Australia and New Zealand Dialysis and Transplant Registry.

PubMed

McDonald, Stephen P

2015-06-01

The ANZDATA Registry includes all patients treated with renal replacement therapy (RRT) throughout Australia and New Zealand. Funding is predominantly from government sources, together with the non-government organization Kidney Health Australia. Registry operations are overseen by an Executive committee, and a Steering Committee with wide representation. Data is collected from renal units throughout Australia and New Zealand on a regular basis, and forwarded to the Registry. Areas covered include demographic details, primary renal disease, type of renal replacement therapy, process measures, and a variety of outcomes. From this data collection a number of themes of work are produced. These include production of Registry reports with an extensive range of national and regional data, a suite of quality assurance reports, key process indicator (KPI) reports, and data sets for a variety of audit and research purposes. The various types of information from the ANZDATA Registry are used in a wide variety of areas, including health services planning, safety and quality programs, and clinical research projects.

Silicosis in Turkey: Is it an Endless Nightmare or is There Still Hope?

PubMed Central

Akgün, Metin

2018-01-01

Silicosis is an ancient but still life-threatening occupational lung disease because of its incurable nature. Although its risks are known in many occupational settings and effective control strategies are well established, new cases, even epidemics, continue to occur in different sectors of Turkey. Before taking action, defining the magnitude of the problem is essential. In this concise review, we aimed to present the current situation of silicosis in Turkey. According to the data available to date, silicosis continues to be a major health problem in different sectors. Sandblasting seems to have the highest risk for the development of silicosis. Disease onset at early age and history of short exposure duration may indicate intense silica exposure. After denim sandblasters, dental technicians seem to be the new and recently recognized high-risk occupation group as per the increasing reports. Because of the lack of a definite treatment of silicosis, prevention of the disease should be the main target. Better occupational disease registry systems would be useful to assess the magnitude of the problem. In addition to implementing the necessary regulations, a close inspection of the workplaces for potential risks is essential. Other social and economic factors related with the occurrence of disease, such as unregistered employment rate and unlicensed and uninsured work, should also be considered. Finally, optimal healthcare and better living conditions for patients with silicosis should be ensured. PMID:29755813

Heart Failure in Sub-Saharan Africa

PubMed Central

Bloomfield, Gerald S; Barasa, Felix A; Doll, Jacob A; Velazquez, Eric J

2013-01-01

The heart failure syndrome has been recognized as a significant contributor to cardiovascular disease burden in sub-Saharan African for many decades. Seminal knowledge regarding heart failure in the region came from case reports and case series of the early 20th century which identified infectious, nutritional and idiopathic causes as the most common. With increasing urbanization, changes in lifestyle habits, and ageing of the population, the spectrum of causes of HF has also expanded resulting in a significant burden of both communicable and non-communicable etiologies. Heart failure in sub-Saharan Africa is notable for the range of etiologies that concurrently exist as well as the healthcare environment marked by limited resources, weak national healthcare systems and a paucity of national level data on disease trends. With the recent publication of the first and largest multinational prospective registry of acute heart failure in sub-Saharan Africa, it is timely to review the state of knowledge to date and describe the myriad forms of heart failure in the region. This review discusses several forms of heart failure that are common in sub-Saharan Africa (e.g., rheumatic heart disease, hypertensive heart disease, pericardial disease, various dilated cardiomyopathies, HIV cardiomyopathy, hypertrophic cardiomyopathy, endomyocardial fibrosis, ischemic heart disease, cor pulmonale) and presents each form with regard to epidemiology, natural history, clinical characteristics, diagnostic considerations and therapies. Areas and approaches to fill the remaining gaps in knowledge are also offered herein highlighting the need for research that is driven by regional disease burden and needs. PMID:23597299

Validity of Administrative Data in Identifying Cancer-related Events in Adolescents and Young Adults: A Population-based Study Using the IMPACT Cohort.

PubMed

Gupta, Sumit; Nathan, Paul C; Baxter, Nancy N; Lau, Cindy; Daly, Corinne; Pole, Jason D

2018-06-01

Despite the importance of estimating population level cancer outcomes, most registries do not collect critical events such as relapse. Attempts to use health administrative data to identify these events have focused on older adults and have been mostly unsuccessful. We developed and tested administrative data-based algorithms in a population-based cohort of adolescents and young adults with cancer. We identified all Ontario adolescents and young adults 15-21 years old diagnosed with leukemia, lymphoma, sarcoma, or testicular cancer between 1992-2012. Chart abstraction determined the end of initial treatment (EOIT) date and subsequent cancer-related events (progression, relapse, second cancer). Linkage to population-based administrative databases identified fee and procedure codes indicating cancer treatment or palliative care. Algorithms determining EOIT based on a time interval free of treatment-associated codes, and new cancer-related events based on billing codes, were compared with chart-abstracted data. The cohort comprised 1404 patients. Time periods free of treatment-associated codes did not validly identify EOIT dates; using subsequent codes to identify new cancer events was thus associated with low sensitivity (56.2%). However, using administrative data codes that occurred after the EOIT date based on chart abstraction, the first cancer-related event was identified with excellent validity (sensitivity, 87.0%; specificity, 93.3%; positive predictive value, 81.5%; negative predictive value, 95.5%). Although administrative data alone did not validly identify cancer-related events, administrative data in combination with chart collected EOIT dates was associated with excellent validity. The collection of EOIT dates by cancer registries would significantly expand the potential of administrative data linkage to assess cancer outcomes.

40 CFR 374.6 - Addresses.

Code of Federal Regulations, 2011 CFR

2011-07-01

... Agency, 1200 Sixth Avenue, Seattle, WA 98101. Administrator, Agency for Toxic Substances and Disease Registry, Center for Disease Control, 200 Independence Avenue, SW., Washington, DC 20201. Attorney General...

40 CFR 374.6 - Addresses.

Code of Federal Regulations, 2010 CFR

2010-07-01

... Agency, 1200 Sixth Avenue, Seattle, WA 98101. Administrator, Agency for Toxic Substances and Disease Registry, Center for Disease Control, 200 Independence Avenue, SW., Washington, DC 20201. Attorney General...

40 CFR 374.6 - Addresses.

Code of Federal Regulations, 2012 CFR

2012-07-01

... Agency, 1200 Sixth Avenue, Seattle, WA 98101. Administrator, Agency for Toxic Substances and Disease Registry, Center for Disease Control, 200 Independence Avenue, SW., Washington, DC 20201. Attorney General...

40 CFR 374.6 - Addresses.

Code of Federal Regulations, 2014 CFR

2014-07-01

... Agency, 1200 Sixth Avenue, Seattle, WA 98101. Administrator, Agency for Toxic Substances and Disease Registry, Center for Disease Control, 200 Independence Avenue, SW., Washington, DC 20201. Attorney General...

40 CFR 374.6 - Addresses.

Code of Federal Regulations, 2013 CFR

2013-07-01

... Agency, 1200 Sixth Avenue, Seattle, WA 98101. Administrator, Agency for Toxic Substances and Disease Registry, Center for Disease Control, 200 Independence Avenue, SW., Washington, DC 20201. Attorney General...

Mayo AVC Registry and BioBank

ClinicalTrials.gov

2018-03-05

Arrhythmogenic Right Ventricular Cardiomyopathy; Cardiomyopathies; Heart Diseases; Cardiovascular Diseases; Sudden Cardiac Arrest; Sudden Cardiac Death; Arrhythmogenic Right Ventricular Dysplasia; Arrhythmogenic Ventricular Cardiomyopathy; Familial Dilated Cardiomyopathy; Cardiovascular Abnormalities

The Italian Registry of Antiphospholipid Antibodies.

PubMed

Finazzi, G

1997-01-01

The clinical importance of antiphospholipid antibodies (APA) derives from their association with a syndrome of venous and arterial thrombosis, recurrent fetal loss and thrombocytopenia known as the antiphospholipid syndrome (APS). The Italian Registry of Antiphospholipid Antibodies was set up in 1989 for the purpose of collecting a large number of patients with lupus anticoagulant (LA) or anticardiolipin antibodies (ACA) for clinical studies in order to obtain more information on the clinical features of APS. The Italian Registry has completed two clinical studies and proposed an international trial on the treatment of APS patients. These activities of the Registry are reviewed herein. Additional information has been obtained from pertinent articles and abstracts published in journals covered by the Science Citation Index and Medline. The first study of the Registry was a retrospective analysis of enrolled patients which showed that: a) the prevalence of thrombosis and thrombocytopenia was similar in cases with idiopathic APA or APA secondary to systemic lupus erythematosus, and b) the rate of thrombosis was significantly reduced in patients with severe thrombocytopenia but not in those with only a mild reduction of the platelet count. The second study was a prospective survey of the natural history of the disease, showing that a) previous thrombosis and ACA titer > 40 units were independent predictors of subsequent vascular complications; b) a history of miscarriage or thrombosis is significantly associated with adverse pregnancy outcome; c) hematological malignancies can develop during follow-up and patients with APA should be considered at increased risk of developing NHL. Thus the possibility of a hematologic neoplastic disease should be borne in mind in the initial evaluation and during the follow-up of these patients. The latest initiative of the Registry was the proposal of an international, randomized clinical trial (WAPS study) aimed at assessing the efficacy and safety of high-dose warfarin in preventing recurrent thrombosis in patients with APA and vascular disease. The study is scheduled to start in March 1997.

Analysis of the Spanish national registry for pediatric home enteral nutrition (NEPAD): implementation rates and observed trends during the past 8 years.

PubMed

Pedrón-Giner, C; Navas-López, V M; Martínez-Zazo, A B; Martínez-Costa, C; Sánchez-Valverde, F; Blasco-Alonso, J; Moreno-Villares, J M; Redecillas-Ferreiro, S; Canals-Badía, M J; Rosell-Camps, A; Gil-Ortega, D; Gómez-López, L; García-Romero, R; Gutierrez-Junquera, C; Balmaseda-Serrano, E M; Bousoño-García, C; Marugán-Miguelsanz, J M; Peña-Quintana, L; González-Santana, D; López-Ruzafa, E; Chicano-Marín, F J; Cabrera-Rodriguez, R; Murray-Hurtado, M; Pérez-Moneo, B

2013-04-01

The home enteral nutrition (HEN) provides nutritional support to children with chronic diseases who are nutritionally compromised and allows them to be discharged more quickly from hospitals. In 2003, a web-based registry (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition -NEPAD-) was created with the objective of gathering information about pediatric HEN practices in Spain. The aim of this study was to report the implementation of the NEPAD (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition) registry of pediatric HEN in Spain and to analyze data evolution trends from 2003 to 2010. The data from the Spanish NEPAD registry were analyzed according to the following variables: demographic data, diagnosis, indication for HEN, nutritional support regime and administration route. Over the study period, 952 patients (1048 episodes) from 20 Spanish hospitals were included in the NEPAD registry. The most frequent indication for HEN was decreased oral intake (64%), and neurological disease was the most prevalent illness. HEN was delivered via a nasogastric tube in 573 episodes (54.7%), by gastrostomy in 375 episodes (35.8%), oral feeding in 77 episodes (7.3%) and by jejunal access in 23 episodes (2.2%). Significant differences in the mode of administration were observed based on the pathology of the child (χ(2), P<0.0001). The cyclic feeding was the most widely used technique for the administration of HEN. Most of the patients used a pump and a polymeric formula. Transition to oral feeding was the primary reason for discontinuation of this type of support. Since the NEPAD registry was established in Spain, the number of documented patients has increased more than 25-fold. Many children with chronic illness benefit from HEN, mainly those suffering from neurological diseases.

Correlation of psoriasis activity with socioeconomic status: cross-sectional analysis of patients enrolled in the Psoriasis Longitudinal Assessment and Registry (PSOLAR).

PubMed

Kimball, A B; Augustin, M; Gordon, K B; Krueger, G G; Pariser, D; Fakharzadeh, S; Goyal, K; Calabro, S; Lee, S; Lin, R; Li, N; Srivastava, B; Guenther, L

2018-05-10

The interdependence between socioeconomic status and disease control in patients with severe psoriasis is not well understood. To assess whether worse disease control among patients with historically severe psoriasis correlated with negative socioeconomic status, we conducted a cross-sectional analysis from Psoriasis Longitudinal Assessment and Registry (PSOLAR), a large, observational study of psoriasis patients receiving, or eligible to receive, conventional systemic or biologic therapies. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Childhood fish oil supplementation modifies associations between traffic related air pollution and allergic sensitisation.

PubMed

Hansell, Anna L; Bakolis, Ioannis; Cowie, Christine T; Belousova, Elena G; Ng, Kitty; Weber-Chrysochoou, Christina; Britton, Warwick J; Leeder, Stephen R; Tovey, Euan R; Webb, Karen L; Toelle, Brett G; Marks, Guy B

2018-03-27

Studies of potential adverse effects of traffic related air pollution (TRAP) on allergic disease have had mixed findings. Nutritional studies to examine whether fish oil supplementation may protect against development of allergic disease through their anti-inflammatory actions have also had mixed findings. Extremely few studies to date have considered whether air pollution and dietary factors such as fish oil intake may interact, which was the rationale for this study. We conducted a secondary analysis of the Childhood Asthma Prevention Study (CAPS) birth cohort, where children were randomised to fish oil supplementation or placebo from early life to age 5 years. We examined interactions between supplementation and TRAP (using weighted road density at place of residence as our measure of traffic related air pollution exposure) with allergic disease and lung function outcomes at age 5 and 8 years. Outcome information was available on approximately 400 children (~ 70% of the original birth cohort). Statistically significant interactions between fish oil supplementation and TRAP were seen for house dust mite (HDM), inhalant and all-allergen skin prick tests (SPTs) and for HDM-specific interleukin-5 response at age 5. Adjusting for relevant confounders, relative risks (RRs) for positive HDM SPT were RR 1.74 (95% CI 1.22-2.48) per 100 m local road or 33.3 m of motorway within 50 m of the home for those randomised to the control group and 1.03 (0.76-1.41) for those randomised to receive the fish oil supplement. The risk differential was highest in an analysis restricted to those who did not change address between ages 5 and 8 years. In this sub-group, supplementation also protected against the effect of traffic exposure on pre-bronchodilator FEV 1 /FVC ratio. Results suggest that fish oil supplementation may protect against pro-allergic sensitisation effects of TRAP exposure. Strengths of this analysis are that supplementation was randomised and independent of TRAP exposure, however, findings need to be confirmed in a larger experimental study with the interaction investigated as a primary hypothesis, potentially also exploring epigenetic mechanisms. More generally, studies of adverse health effects of air pollution may benefit from considering potential effect modification by diet and other factors. Australia New Zealand Clinical Trial Registry. www.anzctr.org.au Registration: ACTRN12605000042640 , Date: 26th July 2005. Retrospectively registered, trial commenced prior to registry availability.

Applying Registry Services to Spaceflight Technologies to Aid in the Assignment of Assigned Numbers to Disparate Systems and their Technologies to Further Enable Interoperability

NASA Technical Reports Server (NTRS)

Bradford, Robert N.; Nichols, Kelvin F.; Witherspoon, Keith R.

2006-01-01

To date very little effort has been made to provide interoperability between various space agency projects. To effectively get to the Moon and beyond systems must interoperate. To provide interoperability, standardization and registries of various technologies will be required. These registries will be created as they relate to space flight. With the new NASA Moon/Mars initiative, a requirement to standardize and control the naming conventions of very disparate systems and technologies is emerging. The need to provide numbering to the many processes, schemas, vehicles, robots, space suits and technologies (e.g. versions), to name a few, in the highly complex Constellation initiative is imperative. The number of corporations, developer personnel, system interfaces, people interfaces will require standardization and registries on a scale not currently envisioned. It would only take one exception (stove piped system development) to weaken, if not, destroy interoperability. To start, a standardized registry process must be defined that allows many differing engineers, organizations and operators the ability to easily access disparate registry information across numerous technological and scientific disciplines. Once registries are standardized the need to provide registry support in terms of setup and operations, resolution of conflicts between registries and other issues will need to be addressed. Registries should not be confused with repositories. No end user data is "stored" in a registry nor is it a configuration control system. Once a registry standard is created and approved, the technologies that should be registered must be identified and prioritized. In this paper, we will identify and define a registry process that is compatible with the Constellation initiative and other non related space activities and organizations. We will then identify and define the various technologies that should use a registry to provide interoperability. The first set of technologies will be those that are currently in need of expansion namely the assignment of satellite designations and the process which controls assignments. Second, we will analyze the technologies currently standardized under the Consultative Committee for Space Data Systems (CCSDS) banner. Third, we will analyze the current CCSDS working group and Birds of a Feather (BoF) activities to ascertain registry requirements. Lastly, we will identify technologies that are either currently under the auspices of another standards body or technologies that are currently not standardized. For activities one through three, we will provide the analysis by either discipline or technology with rationale, identification and brief description of requirements and precedence. For activity four, we will provide a list of current standards bodies e.g. IETF and a list of potential candidates.

ISHKS joint registry: A preliminary report.

PubMed

Pachore, Jawahir A; Vaidya, Shrinand V; Thakkar, Chandrasekhar J; Bhalodia, Haresh Kumar P; Wakankar, Hemant M

2013-09-01

Total knee arthroplasty (TKA) and total hip arthroplasty (THA) are the most widely practiced surgical options for arthritis all over the world and its application is rising in India. Indian Society of Hip and Knee Surgeons (ISHKS) has established a joints registry and has been collecting data for last 6 years. All members of ISHKS are encouraged to actively participate in the registry. A simple two page knee and hip form can be downloaded from the website www.ishks.com. The information collected includes patient demographics, indication for surgery, implant details and in case of revision arthroplasty: the details of implants removed and the cause of failure of primary arthroplasty. These forms are mailed to the central registry office and the data is fed in computerized registry. Data collection started in October 2006. Joint registry is a very important initiative of ISHKS and till date, have data of 34,478 TKAs and 3604 THAs, contributed by 42 surgeons across India. Some important observations have emerged. Data of 34,478 TKAs was assessed: These included 8612 males (25%) and 25,866 females (75%). Average age was 64.4 years (Osteoarthritis range: 45 to 88 years; Rheumatoid arthritis range: 22 to 74 years). Average body mass index was 29.1 (Range: 18.1 to 42.9). The indication for TKA was osteoarthritis in 33,444 (97%) and rheumatoid arthritis in 759 (2.2%). Total of 3604 THA procedures were recorded. These included 2162 (60%) male patients and 1442 (40%) female patients. Average age was 52 years (Range 17 to 85 years) and average BMI was 25.8 (Range: 17.3 to 38.5). The indications for THA was AVN in 49%. The registry will become more meaningful in years to come. Active participation of all arthroplasty surgeons across India is vital for the success of the joints registry.

Prevalence of autosomal dominant polycystic kidney disease in the European Union.

PubMed

Willey, Cynthia J; Blais, Jaime D; Hall, Anthony K; Krasa, Holly B; Makin, Andrew J; Czerwiec, Frank S

2017-08-01

Autosomal dominant polycystic kidney disease (ADPKD) is a leading cause of end-stage renal disease, but estimates of its prevalence vary by >10-fold. The objective of this study was to examine the public health impact of ADPKD in the European Union (EU) by estimating minimum prevalence (point prevalence of known cases) and screening prevalence (minimum prevalence plus cases expected after population-based screening). A review of the epidemiology literature from January 1980 to February 2015 identified population-based studies that met criteria for methodological quality. These examined large German and British populations, providing direct estimates of minimum prevalence and screening prevalence. In a second approach, patients from the 2012 European Renal Association‒European Dialysis and Transplant Association (ERA-EDTA) Registry and literature-based inflation factors that adjust for disease severity and screening yield were used to estimate prevalence across 19 EU countries (N = 407 million). Population-based studies yielded minimum prevalences of 2.41 and 3.89/10 000, respectively, and corresponding estimates of screening prevalences of 3.3 and 4.6/10 000. A close correspondence existed between estimates in countries where both direct and registry-derived methods were compared, which supports the validity of the registry-based approach. Using the registry-derived method, the minimum prevalence was 3.29/10 000 (95% confidence interval 3.27-3.30), and if ADPKD screening was implemented in all countries, the expected prevalence was 3.96/10 000 (3.94-3.98). ERA-EDTA-based prevalence estimates and application of a uniform definition of prevalence to population-based studies consistently indicate that the ADPKD point prevalence is <5/10 000, the threshold for rare disease in the EU. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA.

Diet and Rheumatoid Arthritis Symptoms: Survey Results From a Rheumatoid Arthritis Registry.

PubMed

Tedeschi, Sara K; Frits, Michelle; Cui, Jing; Zhang, Zhi Zack; Mahmoud, Taysir; Iannaccone, Christine; Lin, Tzu-Chieh; Yoshida, Kazuki; Weinblatt, Michael E; Shadick, Nancy A; Solomon, Daniel H

2017-12-01

Patients with rheumatoid arthritis (RA) often ask whether specific foods, popularized as inflammatory or antiinflammatory, can improve or worsen their RA. Patients with RA took a survey on diet and RA symptoms, and the survey data were collected and analyzed. A dietary survey was mailed to 300 subjects in a single-center RA registry at a large academic center. Subjects were asked about their consumption of 20 foods and whether these foods make their RA symptoms better, worse, or unchanged. Semiannual registry data include demographics, medications, comorbidities, and disease activity scores. Fisher's exact test and Wilcoxon's rank sum tests evaluated associations between subject characteristics from the most recent registry assessment and changes in RA symptoms from specific foods. Of the 217 subjects (72% response rate), 83% were female; the median RA duration was 17 years (interquartile range 9-27 years), and 58% were taking a biologic disease-modifying antirheumatic drug. Twenty-four percent of subjects reported that foods affect their RA symptoms, with 15% reporting improvement and 19% reporting worsening. Blueberries and spinach were the foods most often reported to improve RA symptoms, while soda with sugar and desserts were those most often reported to worsen RA symptoms. Younger age and noting that sleep, warm room temperature, and vitamin/mineral supplements improve RA were each associated with reporting that foods affect RA symptoms. Medication use, sex, body mass index, smoking, disease duration, disease activity scores, and self-reported RA flares were not associated with reporting that foods affect RA. Nearly one-quarter of RA subjects with longstanding disease reported that diet had an effect on their RA symptoms. © 2017, American College of Rheumatology.

Cardiovascular ischemic event rates in outpatients with symptomatic atherothrombosis or risk factors in the united states: insights from the REACH Registry.

PubMed

Eagle, Kim A; Hirsch, Alan T; Califf, Robert M; Alberts, Mark J; Steg, P Gabriel; Cannon, Christopher P; Brennan, Danielle M; Bhatt, Deepak L

2009-06-01

Atherothrombosis, defined as coronary artery, cerebrovascular, and peripheral arterial disease, is the leading cause of death in the United States. Limited data are available from outpatient populations to describe contemporary cardiovascular ischemic event rates and associated use of risk reduction treatments in patients with clinically manifest, or at risk for, atherothrombosis. The REduction of Atherothrombosis for Continued Health (REACH) Registry is an international, prospective, observational study of patients with either documented atherothrombotic syndromes or 3 or more risk factors designed to fill this knowledge gap. Baseline demographics and 1-year outcomes were evaluated for US patients enrolled in the REACH Registry. Multivariate analytic models were constructed using baseline characteristics to determine independent predictors of 1-year event rates. In the United States, 25,686 patients were enrolled into the registry. Among symptomatic patients (n = 19,069), 19% had disease in >or=1 arterial bed. As of July 2006, 1-year outcomes were available for 93.4% (n = 23,985) of patients. The composite cardiovascular death, nonfatal myocardial infarction, and nonfatal stroke event rate was 4.3% for the overall population and highest in patients with triple bed disease (9.9%). There was a relatively high use of risk reduction medications among symptomatic patients. However, opportunity for improvement remains. Approximately 9% of symptomatic patients were not using any antithrombotic, 7% were not using any antihypertensive agents, and 17% were not taking a lipid-lowering agent, whereas >80% of patients suffered from hypertension or dyslipidemia. US patients with established atherothrombotic disease continue to experience high cardiovascular ischemic event rates; these rates increase in close association with polyvascular disease. Despite the use of risk reduction interventions, ideal secondary prevention of ischemic events has not been achieved.

On the Creation, Utility and Sustaining of Rare Diseases Research Networks: Lessons learned from the Urea Cycle Disorders Consortium, the Japanese Urea Cycle Disorders Consortium and the European Registry and Network for Intoxication Type Metabolic Diseases.

PubMed

Summar, Marshall L; Endo, Fumio; Kölker, Stefan

2014-01-01

The past two decades has seen a rapid expansion in the scientific and public interest in rare diseases and their treatment. One consequence of this has been the formation of registries/longitudinal natural history studies for these disorders. Given the expense and effort needed to develop and maintain such programs, we describe our experience with three linked registries on the same disease group, urea cycle disorders. The Urea Cycle Disorders Consortium (UCDC) was formed in the U.S. in 2003 in response to a request for application from the National Institutes of Health (NIH); the European Registry and Network for Intoxication Type Metabolic Diseases (E-IMD) was formed in 2011 in response to a request for applications from the Directorate-General for Health and Consumers (DG SANCO) of the EU; and the Japanese Urea Cycle Disorders Consortium (JUCDC) was founded in 2012 as a sister organization to the UCDC and E-IMD. The functions of these groups are to collect natural history data, educate the professional and lay population, develop and test new treatments, and establish networks of excellence for the care for these disorders. The UCDC and JUCDC focus exclusively on urea cycle disorders while the E-IMD includes patients with urea cycle disorders and organic acidurias. More than 1400 patients have been enrolled in the three consortia, and numerous projects have been developed and joint meetings held including an international UCDC/E-IMD/JUCDC Urea Cycle meeting in Barcelona in 2013. This article summarizes some of the experiences from the three groups regarding formation, funding, and models for sustainability. Copyright © 2014 Elsevier Inc. All rights reserved.

On the Creation, Utility and Sustaining of Rare Diseases Research Networks: Lessons learned from the Urea Cycle Disorders Consortium, the Japanese Urea Cycle Disorders Consortium and the European Registry and Network for Intoxication Type Metabolic Diseases

PubMed Central

Summar, Marshall L.; Endo, Fumio; Kölker, Stefan

2016-01-01

The past two decades has seen a rapid expansion in the scientific and public interest in rare diseases and their treatment. One consequence of this has been the formation of registries/longitudinal natural history studies for these disorders. Given the expense and effort needed to develop and maintain such programs, we describe our experience with three linked registries on the same disease group, urea cycle disorders. The Urea Cycle Disorders Consortium (UCDC) was formed in the U.S. in 2003 in response to a request for application from the National Institutes of Health (NIH); the European Registry and Network for Intoxication Type Metabolic Diseases (E-IMD) was formed in 2011 in response to a request for applications from the Directorate-General for Health and Consumers (DG SANCO) of the EU; and the Japanese Urea Cycle Disorders Consortium (JUCDC) was founded in 2012 as a sister organization to the UCDC and E-IMD. The functions of these groups are to collect natural history data, educate the professional and lay population, develop and test new treatments, and establish networks of excellence for the care for these disorders. The UCDC and JUCDC focus exclusively on urea cycle disorders while the E-IMD includes patients with urea cycle disorders and organic acidurias. More than 1400 patients have been enrolled in the three consortia, and numerous projects have been developed and joint meetings held including an international UCDC/E-IMD/JUCDC Urea Cycle meeting in Barcelona in 2013. This article summarizes some of the experiences from the three groups regarding formation, funding, and models for sustainability. PMID:25261246

Preliminary Results of National Amyotrophic Lateral Sclerosis (ALS) Registry Risk Factor Survey Data

PubMed Central

2016-01-01

Background The National ALS Registry is made up of two components to capture amyotrophic lateral sclerosis (ALS) cases: national administrative databases (Medicare, Medicaid, Veterans Health Administration and Veterans Benefits Administration) and self-identified cases captured by the Registry’s web portal. This study describes self-reported characteristics of U.S. adults with ALS using the data collected by the National ALS Registry web portal risk factor surveys only from October 19, 2010 through December 31, 2013. Objective To describe findings from the National ALS Registry’s web portal risk factor surveys. Measurements The prevalence of select risk factors among adults with ALS was determined by calculating the frequencies of select risk factors—smoking and alcohol (non, current and former) histories, military service and occupational history, and family history of neurodegenerative diseases such as ALS, Alzheimer’s and/or Parkinson’s. Results Nearly half of survey respondents were ever smokers compared with nearly 41% of adults nationally. Most respondents were ever drinkers which is comparable to national estimates. The majority were light drinkers. Nearly one-quarter of survey respondents were veterans compared with roughly 9% of US adults nationally. Most respondents were retired or disabled. The industries in which respondents were employed for the longest time were Professional and Scientific and Technical Services. When family history of neurodegenerative diseases in first degree relatives was evaluated against our comparison group, the rates of ALS were similar, but were higher for Parkinson’s disease, Alzheimer’s disease and any neurodegenerative diseases. Conclusions The National ALS Registry web portal, to our knowledge, is the largest, most geographically diverse collection of risk factor data about adults living with ALS. Various characteristics were consistent with other published studies on ALS risk factors and will allow researchers to generate hypotheses for future research. PMID:27124833

Rapid Development of Specialty Population Registries and Quality Measures from Electronic Health Record Data: An Agile Framework

PubMed Central

Kannan, V; Fish, JS; Mutz, JM; Carrington, AR; Lai, K; Davis, LS; Youngblood, JE; Rauschuber, MR; Flores, KA; Sara, EJ; Bhat, DG; Willett, DL

2017-01-01

Summary Background Creation of a new electronic health record (EHR)-based registry often can be a "one-off" complex endeavor: first developing new EHR data collection and clinical decision support tools, followed by developing registry-specific data extractions from the EHR for analysis. Each development phase typically has its own long development and testing time, leading to a prolonged overall cycle time for delivering one functioning registry with companion reporting into production. The next registry request then starts from scratch. Such an approach will not scale to meet the emerging demand for specialty registries to support population health and value-based care. Objective To determine if the creation of EHR-based specialty registries could be markedly accelerated by employing (a) a finite core set of EHR data collection principles and methods, (b) concurrent engineering of data extraction and data warehouse design using a common dimensional data model for all registries, and (c) agile development methods commonly employed in new product development. Methods We adopted as guiding principles to (a) capture data as a by product of care of the patient, (b) reinforce optimal EHR use by clinicians, (c) employ a finite but robust set of EHR data capture tool types, and (d) leverage our existing technology toolkit. Registries were defined by a shared condition (recorded on the Problem List) or a shared exposure to a procedure (recorded on the Surgical History) or to a medication (recorded on the Medication List). Any EHR fields needed—either to determine registry membership or to calculate a registry-associated clinical quality measure (CQM)—were included in the enterprise data warehouse (EDW) shared dimensional data model. Extract-transform-load (ETL) code was written to pull data at defined “grains” from the EHR into the EDW model. All calculated CQM values were stored in a single Fact table in the EDW crossing all registries. Registry-specific dashboards were created in the EHR to display both (a) real-time patient lists of registry patients and (b) EDW-generated CQM data. Agile project management methods were employed, including co-development, lightweight requirements documentation with User Stories and acceptance criteria, and time-boxed iterative development of EHR features in 2-week “sprints” for rapid-cycle feedback and refinement. Results Using this approach, in calendar year 2015 we developed a total of 43 specialty chronic disease registries, with 111 new EHR data collection and clinical decision support tools, 163 new clinical quality measures, and 30 clinic-specific dashboards reporting on both real-time patient care gaps and summarized and vetted CQM measure performance trends. Conclusions This study suggests concurrent design of EHR data collection tools and reporting can quickly yield useful EHR structured data for chronic disease registries, and bodes well for efforts to migrate away from manual abstraction. This work also supports the view that in new EHR-based registry development, as in new product development, adopting agile principles and practices can help deliver valued, high-quality features early and often. PMID:28930362

Developing the Safety of Atrial Fibrillation Ablation Registry Initiative (SAFARI) as a collaborative pan-stakeholder critical path registry model: a Cardiac Safety Research Consortium "Incubator" Think Tank.

PubMed

Al-Khatib, Sana M; Calkins, Hugh; Eloff, Benjamin C; Kowey, Peter; Hammill, Stephen C; Ellenbogen, Kenneth A; Marinac-Dabic, Danica; Waldo, Albert L; Brindis, Ralph G; Wilbur, David J; Jackman, Warren M; Yaross, Marcia S; Russo, Andrea M; Prystowsky, Eric; Varosy, Paul D; Gross, Thomas; Pinnow, Ellen; Turakhia, Mintu P; Krucoff, Mitchell W

2010-10-01

Although several randomized clinical trials have demonstrated the safety and efficacy of catheter ablation of atrial fibrillation (AF) in experienced centers, the outcomes of this procedure in routine clinical practice and in patients with persistent and long-standing persistent AF remain uncertain. Brisk adoption of this therapy by physicians with diverse training and experience highlights potential concerns regarding the safety and effectiveness of this procedure. Some of these concerns could be addressed by a national registry of AF ablation procedures such as the Safety of Atrial Fibrillation Ablation Registry Initiative that was initially proposed at a Cardiac Safety Research Consortium Think Tank meeting in April 2009. In January 2010, the Cardiac Safety Research Consortium, in collaboration with the Duke Clinical Research Institute, the US Food and Drug Administration, the American College of Cardiology, and the Heart Rhythm Society, held a follow-up meeting of experts in the field to review the construct and progress to date. Other participants included the National Heart, Lung, and Blood Institute; the Centers for Medicare and Medicaid Services; the Agency for Healthcare Research and Quality; the AdvaMed AF working group; and additional industry representatives. This article summarizes the discussions that occurred at the meeting of the state of the Safety of Atrial Fibrillation Ablation Registry Initiative, the identification of a clear pathway for its implementation, and the exploration of solutions to potential issues in the execution of this registry. Copyright © 2010 Mosby, Inc. All rights reserved.

Creation of an international registry to support discovery in schwannomatosis.

PubMed

Ostrow, K L; Bergner, A L; Blakeley, J; Evans, D G; Ferner, R; Friedman, J M; Harris, G J; Jordan, J T; Korf, B; Langmead, S; Leschziner, G; Mautner, V; Merker, V L; Papi, L; Plotkin, S R; Slopis, J M; Smith, M J; Stemmer-Rachamimov, A; Yohay, K; Belzberg, A J

2017-02-01

Schwannomatosis is a tumor suppressor syndrome that causes multiple tumors along peripheral nerves. Formal diagnostic criteria were first published in 2005. Variability in clinical presentation and a relative lack of awareness of the syndrome have contributed to difficulty recognizing affected individuals and accurately describing the natural history of the disorder. Many critical questions such as the mutations underlying schwannomatosis, genotype-phenotype correlations, inheritance patterns, pathologic diagnosis of schwannomatosis-associated schwannomas, tumor burden in schwannomatosis, the incidence of malignancy, and the effectiveness of current, or new treatments remain unanswered. A well-curated registry of schwannomatosis patients is needed to facilitate research in field. An international consortium of clinicians and scientists across multiple disciplines with expertise in schwannomatosis was established and charged with the task of designing and populating a schwannomatosis patient registry. The International Schwannomatosis Registry (ISR) was built around key data points that allow confirmation of the diagnosis and identification of potential research subjects to advance research to further the knowledge base for schwannomatosis. A registry with 389 participants enrolled to date has been established. Twenty-three additional subjects are pending review. A formal process has been established for scientific investigators to propose research projects, identify eligible subjects, and seek collaborators from ISR sites. Research collaborations have been created using the information collected by the registry and are currently being conducted. The ISR is a platform from which multiple research endeavors can be launched, facilitating connections between affected individuals interested in participating in research and researchers actively investigating a variety of aspects of schwannomatosis. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Lyme disease: the promise of Big Data, companion diagnostics and precision medicine

PubMed Central

Stricker, Raphael B; Johnson, Lorraine

2016-01-01

Lyme disease caused by the spirochete Borrelia burgdorferi has become a major worldwide epidemic. Recent studies based on Big Data registries show that >300,000 people are diagnosed with Lyme disease each year in the USA, and up to two-thirds of individuals infected with B. burgdorferi will fail conventional 30-year-old antibiotic therapy for Lyme disease. In addition, animal and human evidence suggests that sexual transmission of the Lyme spirochete may occur. Improved companion diagnostic tests for Lyme disease need to be implemented, and novel treatment approaches are urgently needed to combat the epidemic. In particular, therapies based on the principles of precision medicine could be modeled on successful “designer drug” treatment for HIV/AIDS and hepatitis C virus infection featuring targeted protease inhibitors. The use of Big Data registries, companion diagnostics and precision medicine will revolutionize the diagnosis and treatment of Lyme disease. PMID:27672336

Effects of the intradiscal implantation of stromal vascular fraction plus platelet rich plasma in patients with degenerative disc disease.

PubMed

Comella, Kristin; Silbert, Robert; Parlo, Michelle

2017-01-13

Stromal vascular fraction (SVF) can easily be obtained from a mini-lipoaspirate procedure of fat tissue and platelet rich plasma (PRP) can be obtained from peripheral blood. The SVF contains a mixture of cells including ADSCs and growth factors and has been depleted of the adipocyte (fat cell) population. We evaluated the safety and efficacy of administering SVF and PRP intra-discally into patients with degenerative disc disease. A total of 15 patients underwent a local tumescent liposuction procedure to remove approximately 60 ml of fat tissue. The fat was separated to isolate the SVF and the cells were delivered into the disc nucleus of patients with degenerative disc disease. The subjects were then monitored for adverse events, range of motion, visual analog scale (VAS), present pain intensity (PPI), Oswestry Disability Index (ODI), Beck Depression Inventory (BDI), Dallas Pain Questionnaire and Short Form (SF)-12 scores over a period of 6 months. Safety events were followed for 12 months. No severe adverse events (SAEs) were reported during a 12 month follow up period with no incidences of infection. Patients demonstrated statistically significant improvements in several parameters including flexion, pain ratings, VAS, PPI, and short form questionnaires. In addition, both ODI and BDI data was trending positive and a majority of patients reported improvements in their Dallas Pain Questionnaire scores. Overall, patients were pleased with the treatment results. More importantly, the procedure demonstrated a strong safety profile with no severe adverse events or complications linked to the therapy. Trial registration NCT02097862. Name of registry: www.clinicaltrials.gov . https://clinicaltrials.gov/ct2/show/NCT02097862?term=bioheart&rank=6 . Date of registration: March 25, 2014; Date of enrollment: March 2014.

[Spanish patients with central hypoventilation syndrome included in the European Registry. The 2015 data].

PubMed

García Teresa, María Angeles; Porto Abal, Raquel; Rodríguez Torres, Silvia; García Urabayen, Diego; García Martínez, Silvia; Trang, Ha; Campos Barros, Angel; Llorente de la Fuente, Ana; Hernández González, Arturo; Bustinza Arriortua, Amaya; de la Cruz Moreno, Jesús; Pons Odena, Martí; Ventura Faci, Purificación; Rubio Ortega, Laura; Pérez Ruiz, Estela; Aguilar Fernández, Antonio; Pérez Ocón, Amaya; Osona, Borja; Delgado Pecellin, Isabel; Arroyo Carrera, Ignacio; Sayas Catalán, Javier; González Salas, Elvira; de Vicente, Carlos Martin

2017-05-01

Congenital Central Hypoventilation Syndrome (CCHS) is a very rare genetic disease. In 2012 the European Central Hypoventilation Syndrome (EuCHS) Consortium created an online patient registry in order to improve care. To determine the characteristics and outcomes of Spanish patients with CCHS, and detect clinical areas for improvement. An assessment was made on the data from Spanish patients in the European Registry, updated on December 2015. The Registry contained 38 patients, born between 1987 and 2013, in 18 hospitals. Thirteen (34.2%) were older than 18 years. Three patients had died. Genetic analysis identified PHOX2B mutations in 32 (86.5%) out of 37 patients assessed. The 20/25, 20/26 and 20/27 polyalanine repeat mutations (PARMs) represented 84.3% of all mutations. Longer PARMs had more, as well as more severe, autonomic dysfunctions. Eye diseases were present in 47%, with 16% having Hirschsprung disease, 13% with hypoglycaemia, and 5% with tumours. Thirty patients (79%) required ventilation from the neonatal period onwards, and 8 (21%) later on in life (late onset/presentation). Eight children (21%) were using mask ventilation at the first home discharge. Five of them were infants with neonatal onset, two of them, both having a severe mutation, were switched to tracheostomy after cardiorespiratory arrest at home. Approximately one-third (34.3%) of patients were de-cannulated and switched to mask ventilation at a mean age of 13.7 years. Educational reinforcement was required in 29.4% of children attending school. The implementation of the EuCHS Registry in Spain has identified some relevant issues for optimising healthcare, such as the importance of genetic study for diagnosis and assessment of severity, the high frequency of eye disease and educational reinforcement, as well as some limitations in ventilatory techniques. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

A comparative study of the risk profile of hemodialysis patients in a for profit network and in two regional registries of the Italian Society of Nephrology.

PubMed

Postorino, Maurizio; Amato, Claudia; Mancini, Elena; Carioni, Paola; D'Arrigo, Graziella; Di Benedetto, Attilio; Cerino, Fabrizio; Marino, Carmela; Vilasi, Antonio; Tripepi, Giovanni; Stuard, Stefano; Capasso, Giovanbattista; Santoro, Antonio; Zoccali, Carmine

2018-02-01

In 2013, the Italian Society of Nephrology joined forces with Nephrocare-Italy to create a clinical research cohort of patients on file in the data-rich clinical management system (EUCLID) of this organization for the performance of observational studies in the hemodialysis (HD) population. To see whether patients in EUCLID are representative of the HD population in Italy, we set out to compare the whole EUCLID population with patients included in the regional HD registries in Emilia-Romagna (Northern Italy) and in Calabria (Southern Italy), the sole regions in Italy which have systematically collected an enlarged clinical data set allowing comparison with the data-rich EUCLID system. An analysis of prevalent and incident patients in 2010 and 2011 showed that EUCLID patients had a lower prevalence of coronary heart disease, peripheral vascular disease, heart failure, valvular heart disease, liver disease, peptic ulcer and other comorbidities and risk factors and a higher fractional urea clearance (Kt/V) than those in the Emilia Romagna and Calabria registries. Accordingly, survival analysis showed a lower mortality risk in the EUCLID 2010 and 2011 cohorts than in the combined two regional registries in the corresponding years: for 2010, hazard ratio (HR) EUCLID vs. Regional registries: 0.80 [95% confidence interval: 0.71-0.90]; for 2011, HR: 0.76 [0.65-0.90]. However, this difference was nullified by statistical adjustment for the difference in comorbidities and risk factors, indicating that the longer survival in the EUCLID database was attributable to the lower risk profile of patients included in that database. This preliminary analysis sets the stage for future observational studies and indicates that appropriate adjustment for difference in comorbidities and risk factors is needed to generalize to the Italian HD population analyses based on the data-rich EUCLID database.

78 FR 66254 - Airworthiness Directives; The Boeing Company Airplanes

Federal Register 2010, 2011, 2012, 2013, 2014

2013-11-05

... AD. Costs of Compliance We estimate that this AD affects 84 airplanes of U.S. registry. We estimate... inspection cycle. We have received no definitive data that would enable us to provide a cost estimate for the... 2 of paragraph 1.E., ``Compliance,'' of Boeing Service Bulletin 747-53A2688, Revision 1, dated...

77 FR 22632 - Privacy Act of 1974; System of Records

Federal Register 2010, 2011, 2012, 2013, 2014

2012-04-16

...--Repository (SCIDO-R)-VA'' (108VA11S) as set forth in the Federal Register 74 FR 11185-11186 dated March 16.... SUPPLEMENTARY INFORMATION: The Spinal Cord Injury and Disorders Outcomes--Repository (SCIDO-R) provides a registry of veterans with spinal cord injury and disorders (SCI&D). This repository contains pertinent...

International Rare Histiocytic Disorders Registry (IRHDR)

ClinicalTrials.gov

2018-04-18

Rare Histiocytic Disorders (RHDs); Juvenile Xanthogranuloma (JXG); Reticulohistiocytoma (Epithelioid Histiocytoma); Xanthoma Disseminatum (XD); Multicentric Reticulohistiocytosis (MRH); Systemic Juvenile Xanthogranuloma; Erdheim-Chester Disease (ECD); Multi-system Rosai-Dorfman Disease (RDD)

Intergenerational effects of endocrine-disrupting compounds: a review of the Michigan polybrominated biphenyl registry.

PubMed

Curtis, Sarah W; Conneely, Karen N; Marder, Mary E; Terrell, Metrecia L; Marcus, Michele; Smith, Alicia K

2018-06-11

Endocrine-disrupting compounds (EDCs) are a broad class of chemicals present in many residential products that can disrupt hormone signaling and cause health problems in humans. Multigenerational cohorts, like the Michigan polybrominated biphenyl registry, are ideal for studying the effects of intergenerational exposure. Registry participants report hormone-related health problems, particularly in those exposed before puberty or those in the second generation exposed through placental transfer or breastfeeding. However, more research is needed to determine how EDCs cause health problems and the mechanisms underlying intergenerational exposure. Utilizing existing data in this registry, along with genetic and epigenetic approaches, could provide insight to how EDCs cause human disease and help to determine the risk to exposed populations and future generations.

Revascularization Trends in Patients With Diabetes Mellitus and Multivessel Coronary Artery Disease Presenting With Non-ST Elevation Myocardial Infarction: Insights From the National Cardiovascular Data Registry Acute Coronary Treatment and Intervention Outcomes Network Registry-Get with the Guidelines (NCDR ACTION Registry-GWTG).

PubMed

Pandey, Ambarish; McGuire, Darren K; de Lemos, James A; Das, Sandeep R; Berry, Jarett D; Brilakis, Emmanouil S; Banerjee, Subhash; Marso, Steven P; Barsness, Gregory W; Simon, DaJuanicia N; Roe, Matthew; Goyal, Abhinav; Kosiborod, Mikhail; Amsterdam, Ezra A; Kumbhani, Dharam J

2016-05-01

Current guidelines recommend surgical revascularization (coronary artery bypass graft [CABG]) over percutaneous coronary intervention (PCI) in patients with diabetes mellitus and multivessel coronary artery disease. Few data are available describing revascularization patterns among these patients in the setting of non-ST-segment-elevation myocardial infarction. Using Acute Coronary Treatment and Intervention Outcomes Network Registry-Get with the Guidelines (ACTION Registry-GWTG), we compared the in-hospital use of different revascularization strategies (PCI versus CABG versus no revascularization) in diabetes mellitus patients with non-ST-segment-elevation myocardial infarction who had angiography, demonstrating multivessel coronary artery disease between July 2008 and December 2014. Factors associated with use of CABG versus PCI were identified using logistic multivariable regression analyses. A total of 29 769 patients from 539 hospitals were included in the study, of which 10 852 (36.4%) were treated with CABG, 13 760 (46.2%) were treated with PCI, and 5157 (17.3%) were treated without revascularization. The overall use of revascularization increased over the study period with an increase in the proportion undergoing PCI (45% to 48.9%; Ptrend=0.0002) and no change in the proportion undergoing CABG (36.1% to 34.7%; ptrend=0.88). There was significant variability between participating hospitals in the use of PCI and CABG (range: 22%-100%; 0%-78%, respectively; P value <0.0001 for both). Patient-level, but not hospital-level, characteristics were statistically associated with the use of PCI versus CABG, including anatomic severity of the disease, early treatment of adenosine diphosphate receptor antagonists at presentation, older age, female sex, and history of heart failure. Among patients with diabetes mellitus and multivessel coronary artery disease presenting with non-ST-segment-elevation myocardial infarction, only one third undergo CABG during the index admission. Furthermore, the use of PCI, but not CABG, increased modestly over the past 6 years. © 2016 American Heart Association, Inc.

Long-term health outcomes in patients with Prader-Willi Syndrome: a nationwide cohort study in Denmark.

PubMed

Hedgeman, E; Ulrichsen, S P; Carter, S; Kreher, N C; Malobisky, K P; Braun, M M; Fryzek, J; Olsen, M S

2017-10-01

Prader-Willi syndrome (PWS) is a rare congenital disease that affects growth, sexual development, cognitive function and behavior. Individuals exhibit food preoccupation and hyperphagia, which may lead to obesity with premature morbidity and mortality. The aim of this work was to evaluate the risk of venous thromboembolisms (VTEs), myocardial infarction, pulmonary hypertension, sleep apnea, depression, anxiety and all-cause mortality among persons with PWS as compared with an age- and sex-matched general-population cohort. All persons diagnosed with PWS (n=155) were identified in the Danish Health Registries; an age- and sex-matched comparison group was selected from the general population of Denmark (n=15 500); diseases of interest were identified through the health registry and cause of death register. Follow-up began on date of birth or first medical record availability through to first occurrence of an outcome of interest; follow-up ceased at emigration from Denmark or end of study. Incidence rates (IRs) were calculated and Cox's proportional hazards models were used to understand the relative risk (RR) of disease. The IRs for VTE among patients with PWS was 144 (60-347) per 100 000 person-years. Risks for VTE events and all-cause mortality were 9.4 times (95% confidence interval (CI): 3.7-23.5) and 11.0 times (95% CI: 5.7-21.1) higher, respectively, for patients with PWS versus the general population. Increased risks were also found individually for deep venous thromboses (DVTs) (RR: 9.1; 95% CI: 3.2-25.2), pulmonary embolisms (RR: 11.0; 95% CI: 1.4-86.9), myocardial infarction (RR: 7.2; 95% CI: 1.7-30.2) and anxiety (RR: 2.8; 95% CI: 1.0-7.5). No cases of pulmonary hypertension, sleep apnea or depressive disorders were identified within this PWS cohort. Multiple cardiovascular and behavioral illnesses are more likely to occur among patients with PWS than within the general population. These increased risks may provide an impetus for enhanced disease prevention, screening, diagnosis and treatment.

The rationale and design of the national familial hypercholesterolemia registries in Turkey: A-HIT1 and A-HIT2 studies.

PubMed

Kayıkçıoğlu, Meral; Tokgözoğlu, Lale

2017-04-01

Familial hypercholesterolemia (FH) is a genetic disease characterized by extremely high levels of cholesterol, leading to premature atherosclerosis. Although many countries have already addressed the burden of FH by means of national registries, Turkey has no national FH registry or national screening program to detect FH. Creation of a series of FH registries is planned as part of Turkish FH Initiative endorsed by the Turkish Society of Cardiology to meet this need. This article provides detailed information on the rationale and design of the first 2 FH registries (A-HIT1 and A-HIT2). A-HIT1 is a nationwide survey of adult homozygous FH (HoFH) patients undergoing low-density lipoprotein (LDL) apheresis (LA) in Turkey. A-HIT1 will provide insight into the clinical status of HoFH patients undergoing LA. Primary objective of this cross-sectional study is to identify how HoFH patients on LA are managed. Inclusion criteria are age >12 years, diagnosis of HoFH, and regular LA treatment. All available apheresis centers were electronically invited to participate in the study. The principal physicians of each center will respond to a questionnaire regarding their attitude toward LA. For each patient, another questionnaire will be used to collect data on clinical status, medication use, and disease data. In addition, patients will be asked to complete self-report questionnaires that provide information on quality of life, disease-related anxiety, and depression. A-HIT2 is a registry of adult FH patients presenting at outpatient clinics. At least 1000 FH patients will be recruited from 30 outpatient clinics representing the 12 statistical regions in Turkey based on the EU NUTS classification. Sites specializing in cardiology, internal medicine, and endocrinology were invited to participate. The primary objective of this cross-sectional study is to determine clinical status and management of patients in Turkey diagnosed with FH. Eligibility for screening was defined as having LDL-cholesterol level >160 mg/dL. Inclusion criteria are age >18 years and diagnosis as possible FH (total score of >2 according to Dutch Lipid Clinic Network criteria). In addition to measuring clinical status of patients, a short survey to assess patient level of disease awareness will also be administered. A-HIT1 and A-HIT2 are the first nationwide FH registries in Turkey and will provide important information on the management of Turkish FH patients. In addition, it is planned that they will guide establishment of a national policy for the diagnosis and treatment of FH in Turkey.

USB Storage Device Forensics for Windows 10.

PubMed

Arshad, Ayesha; Iqbal, Waseem; Abbas, Haider

2018-05-01

Significantly increased use of USB devices due to their user-friendliness and large storage capacities poses various threats for many users/companies in terms of data theft that becomes easier due to their efficient mobility. Investigations for such data theft activities would require gathering critical digital information capable of recovering digital forensics artifacts like date, time, and device information. This research gathers three sets of registry and logs data: first, before insertion; second, during insertion; and the third, after removal of a USB device. These sets are analyzed to gather evidentiary information from Registry and Windows Event log that helps in tracking a USB device. This research furthers the prior research on earlier versions of Microsoft Windows and compares it with latest Windows 10 system. Comparison of Windows 8 and Windows 10 does not show much difference except for new subkey under USB Key in registry. However, comparison of Windows 7 with latest version indicates significant variances. © 2017 American Academy of Forensic Sciences.

Long-term risk of renal and urinary tract diseases in childhood cancer survivors: A population-based cohort study.

PubMed

Bonnesen, Trine Gade; Winther, Jeanette F; Asdahl, Peter H; de Fine Licht, Sofie; Gudmundsdottir, Thorgerdur; Sällfors Holmqvist, Anna; Madanat-Harjuoja, Laura-Maria; Tryggvadottir, Laufey; Wesenberg, Finn; Birn, Henrik; Olsen, Jørgen H; Hasle, Henrik

2016-09-01

Childhood cancer has been associated with long-term risk of urinary tract diseases, but risk patterns remain to be comprehensively investigated. We analysed the lifetime risk of urinary tract diseases in survivors of childhood cancer in the Nordic countries. We identified 32,519 one-year survivors of childhood cancer diagnosed since the 1940s and 1950s in the five Nordic cancer registries and selected 211,156 population comparisons of a corresponding age, sex, and country of residence from the national population registries. To obtain information on all first-time hospitalizations for a urinary tract disease, we linked all study subjects to the national hospital registry of each country. Relative risks (RRs) and absolute excess risks (AERs) and associated 95% confidence intervals (CIs) for urinary tract diseases among cancer survivors were calculated with the appropriate morbidity rates among comparisons as reference. We observed 1645 childhood cancer survivors ever hospitalized for urinary tract disease yielding an RR of 2.5 (95% CI 2.4-2.7) and an AER of 229 (95% CI 210-248) per 100,000 person-years. The cumulative risk at age 60 was 22% in cancer survivors and 10% in comparisons. Infections of the urinary system and chronic kidney disease showed the highest excess risks, whereas survivors of neuroblastoma, hepatic and renal tumours experienced the highest RRs. Survivors of childhood cancer had an excess risk of urinary tract diseases and for most diseases the risk remained elevated throughout life. The highest risks occurred following therapy of childhood abdominal tumours. Copyright © 2016 Elsevier Ltd. All rights reserved.

DBMap: a TreeMap-based framework for data navigation and visualization of brain research registry

NASA Astrophysics Data System (ADS)

Zhang, Ming; Zhang, Hong; Tjandra, Donny; Wong, Stephen T. C.

2003-05-01

The purpose of this study is to investigate and apply a new, intuitive and space-conscious visualization framework to facilitate efficient data presentation and exploration of large-scale data warehouses. We have implemented the DBMap framework for the UCSF Brain Research Registry. Such a novel utility would facilitate medical specialists and clinical researchers in better exploring and evaluating a number of attributes organized in the brain research registry. The current UCSF Brain Research Registry consists of a federation of disease-oriented database modules, including Epilepsy, Brain Tumor, Intracerebral Hemorrphage, and CJD (Creuzfeld-Jacob disease). These database modules organize large volumes of imaging and non-imaging data to support Web-based clinical research. While the data warehouse supports general information retrieval and analysis, there lacks an effective way to visualize and present the voluminous and complex data stored. This study investigates whether the TreeMap algorithm can be adapted to display and navigate categorical biomedical data warehouse or registry. TreeMap is a space constrained graphical representation of large hierarchical data sets, mapped to a matrix of rectangles, whose size and color represent interested database fields. It allows the display of a large amount of numerical and categorical information in limited real estate of computer screen with an intuitive user interface. The paper will describe, DBMap, the proposed new data visualization framework for large biomedical databases. Built upon XML, Java and JDBC technologies, the prototype system includes a set of software modules that reside in the application server tier and provide interface to backend database tier and front-end Web tier of the brain registry.

The RD-Connect Registry & Biobank Finder: a tool for sharing aggregated data and metadata among rare disease researchers.

PubMed

Gainotti, Sabina; Torreri, Paola; Wang, Chiuhui Mary; Reihs, Robert; Mueller, Heimo; Heslop, Emma; Roos, Marco; Badowska, Dorota Mazena; de Paulis, Federico; Kodra, Yllka; Carta, Claudio; Martìn, Estrella Lopez; Miller, Vanessa Rangel; Filocamo, Mirella; Mora, Marina; Thompson, Mark; Rubinstein, Yaffa; Posada de la Paz, Manuel; Monaco, Lucia; Lochmüller, Hanns; Taruscio, Domenica

2018-05-01

In rare disease (RD) research, there is a huge need to systematically collect biomaterials, phenotypic, and genomic data in a standardized way and to make them findable, accessible, interoperable and reusable (FAIR). RD-Connect is a 6 years global infrastructure project initiated in November 2012 that links genomic data with patient registries, biobanks, and clinical bioinformatics tools to create a central research resource for RDs. Here, we present RD-Connect Registry & Biobank Finder, a tool that helps RD researchers to find RD biobanks and registries and provide information on the availability and accessibility of content in each database. The finder concentrates information that is currently sparse on different repositories (inventories, websites, scientific journals, technical reports, etc.), including aggregated data and metadata from participating databases. Aggregated data provided by the finder, if appropriately checked, can be used by researchers who are trying to estimate the prevalence of a RD, to organize a clinical trial on a RD, or to estimate the volume of patients seen by different clinical centers. The finder is also a portal to other RD-Connect tools, providing a link to the RD-Connect Sample Catalogue, a large inventory of RD biological samples available in participating biobanks for RD research. There are several kinds of users and potential uses for the RD-Connect Registry & Biobank Finder, including researchers collaborating with academia and the industry, dealing with the questions of basic, translational, and/or clinical research. As of November 2017, the finder is populated with aggregated data for 222 registries and 21 biobanks.

The Mexican Acromegaly Registry: Clinical and Biochemical Characteristics at Diagnosis and Therapeutic Outcomes.

PubMed

Portocarrero-Ortiz, Lesly A; Vergara-Lopez, Alma; Vidrio-Velazquez, Maricela; Uribe-Diaz, Ana María; García-Dominguez, Avril; Reza-Albarrán, Alfredo Adolfo; Cuevas-Ramos, Daniel; Melgar, Virgilio; Talavera, Juan; Rivera-Hernandez, Aleida de Jesus; Valencia-Méndez, Carla Valentina; Mercado, Moisés

2016-11-01

Acromegaly is a systemic disorder caused by a GH-secreting pituitary adenoma. As with other rare diseases, acromegaly registries developed in various European countries have provided us with important information. The objective of the study was to analyze the epidemiological, clinical, biochemical, and therapeutic data from the Mexican Acromegaly Registry (MAR). The setting of the study was a nationwide patient registry. The MAR was created in 2009. It gathers data from 24 participating centers belonging to three different institutions using a specifically designed on-line platform. Only patients diagnosed after 1990 were included in the program. A total of 2057 patients (51% female, mean age at diagnosis 41.1 ± 24.5 y) have been registered for an estimated prevalence of 18 cases per 1 million inhabitants. Hypertension, glucose intolerance, diabetes, and dyslipidemia were present in 27%, 18.4%, 30%, and 24% of the patients, respectively. The IGF-1 level at diagnosis and the concomitant presence of hypertension were significantly associated with the development of diabetes. Transsphenoidal surgery was the primary treatment in 72% of the patients. Pharmacological treatment, mostly with somatostatin analogs, was administered primarily and adjunctively in 26% and 54% of the patients, respectively. Treatment choice varied among the three participating institutions, with the predominance of pharmacological therapy in two of them and of radiation therapy in the third. Therapeutic outcomes were similar to those reported in the European registries. The MAR is the largest and first non-European registry of the disease. Our findings highlight important within-country differences in treatment choice due to variations in the availability of resources.

Initial Report of the Korean Organ Transplant Registry (KOTRY): Heart Transplantation.

PubMed

Lee, Hae Young; Jeon, Eun Seok; Kang, Seok Min; Kim, Jae Joong

2017-11-01

The Korean Organ Transplant Registry (KOTRY), which was the first national transplant registry in Korea, was founded by the Korean Society for Transplantation and the Korean Center for Disease Control in 2014. Here, we present the initial report of the Korean Heart Transplant Registry. A total of 183 heart transplantation (HTPL) patients performed at 4 nationally representative hospitals were collected from April 2014 to December 2015. We analyzed donor and recipient characteristics, treatment patterns, and immediate post-transplantation outcomes. One hundred and eighty-three patients were enrolled. The mean age of the patients was 50.5±13.5 years. The mean age of the male recipients was 4 years greater than that of the female recipients (51.7±13.3 years vs. 47.9±13.7 years, p<0.050). The mean age of donors was more than 12 years younger than that of heart recipients (37.6±10.1 years). Dilated cardiomyopathy was the predominant cause (69%) of heart failure in recipients, followed by ischemic heart diseases (14%) and valvular heart disease (4%). Rejection episodes were most frequent in the 1-6-month period after transplantation (48%), and rarely required intensive treatment. Infection episodes were most frequent <1 month after transplantation (66%) and bacterial and viral infections were equally reported. The 1-year survival rate was 91.6% and most mortality cases occurred during the perioperative period within 1 month after transplantation. With the establishment of the KOTRY in 2014, it is now possible to present nationwide epidemiological data for HTPL in Korea for the first time. The KOTRY is the first national HTPL registry in Korea, and will continue until 2023. Copyright © 2017. The Korean Society of Cardiology

End stage renal disease in Brunei Darussalam - report from the first Brunei Dialysis Transplant Registry (BDTR).

PubMed

Tan, Jackson

2013-09-01

The Brunei Dialysis and Transplant Registry (BDTR) was established in 2011 to collect data from patients undergoing renal replacement therapy (RRT) in Brunei Darussalam. The chief aims of the registry are to obtain general demographic data for RRT patients and to determine disease burden attributable to End Stage Renal Disease (ESRD). The registry population comprises of all ESRD patients treated in Brunei Darussalam. Data domains include general demographic data, medical history, ESRD etiological causes, laboratory investigations, dialysis treatment and outcomes. There were 545 prevalent RRT patients in Brunei at the end of 2011. The incidence and prevalence of ESRD were 265 and 1250 per million population. Hemodialysis (HD), Peritoneal Dialysis (PD) and Transplant comprised of 83%, 11% and 6% of the RRT population, respectively. Diabetes mellitus accounted for 57% of all new incident cases. The mean serum hemoglobin, phosphate, calcium and iPTH were 11.0 ± 1.6 g/dL, 1.9 ± 0.5 mmol/L, 2.3 ± 0.2 mmol/L and 202.5 ± 323.4 ng/mL. Dialysis adequacy for HD and PD were 65.1 (urea reduction ratio) and 2.0 ± 0.3 (Kt/v). 71 % of all prevalent HD had functioning AV fistulae and the peritonitis incidence was one in 24.5 patient-month/episode. The first BDTR has identified some deficiencies in the renal services in Brunei. However, it signals an important milestone for the establishment of benchmarked renal practice in the country. We hoped to maintain and improve our registry for years to come and will strive to align our standards to acceptable international practice.

[Freiburg keratoconus registry : Example of application of smart data for clinical research and inititial results].

PubMed

Lang, S J; Böhringer, D; Reinhard, T

2016-06-01

Keratoconus is a progressive corneal disease with thinning and scarring of the cornea. Diagnostic and treatment options are usually evaluated in large prospective or retrospective trials. Big data and smart data provide the possibility to analyze routine data for clinical research. In this article we report the generation of a monocentric keratoconus registry by means of computerized data analysis of routine data. This demonstrates the potential of clinical research by means of routine data. A "clinical data warehouse" was created from all available routine electronic data. At the time of first presentation, each eye was classified into one out of four categories: suspected, early disease, late disease and status postkeratoplasty. Through integration of multiple data sources the clinical course for each patient was documented in the registry. A total of 3681 eyes from 1841 patients were included. The median follow-up time was 0.54 years. Patient age was higher in the groups with more severe stages of keratoconus, the proportion of female patients was higher in the group of suspected keratoconus and patient age and male to female ratios showed statistically significant differences between the groups (p < 0.001). We were able to create a "clinical data warehouse" by linking multiple data sources and normalizing the data. With this tool we established a novel, monocentric keratoconus registry. Only the grading of disease severity and the exclusion of false positive results were carried out manually. In our opinion establishing a structured clinical data warehouse has a huge potential for clinical and retrospective studies and proves the value of the Smart Data concept.

The EPIRARE proposal of a set of indicators and common data elements for the European platform for rare disease registration.

PubMed

Taruscio, Domenica; Mollo, Emanuela; Gainotti, Sabina; Posada de la Paz, Manuel; Bianchi, Fabrizio; Vittozzi, Luciano

2014-01-01

The European Union acknowledges the relevance of registries as key instruments for developing rare disease (RD) clinical research, improving patient care and health service (HS) planning and funded the EPIRARE project to improve standardization and data comparability among patient registries and to support new registries and data collections. A reference list of patient registry-based indicators has been prepared building on the work of previous EU projects and on the platform stakeholders' information needs resulting from the EPIRARE surveys and consultations. The variables necessary to compute these indicators have been analysed for their scope and use and then organized in data domains. The reference indicators span from disease surveillance, to socio-economic burden, HS monitoring, research and product development, policy equity and effectiveness. The variables necessary to compute these reference indicators have been selected and, with the exception of more sophisticated indicators for research and clinical care quality, they can be collected as data elements common (CDE) to all rare diseases. They have been organized in data domains characterized by their contents and main goal and a limited set of mandatory data elements has been defined, which allows case notification independently of the physician or the health service. The definition of a set of CDE for the European platform for RD patient registration is the first step in the promotion of the use of common tools for the collection of comparable data. The proposed organization of the CDE contributes to the completeness of case ascertainment, with the possible involvement of patients and patient associations in the registration process.

High intensity focused ultrasound ablation for atrial fibrillation: results from the National Spanish Registry.

PubMed

Reyes, Guillermo; Ruyra, Xavier; Valderrama, Francisco; Jimenez, Antonio; Duran, Dario; Perez, Enrique; Daroca, Tomas; Moya, Javier; Ramirez, Ulises; Aldamiz, Gonzalo

2016-10-01

A National Spanish Registry to compile all patients treated with high intensity focused ultrasound (HIFU) energy for atrial fibrillation (AF) was created to evaluate the safety and efficacy of AF surgical ablation. A national Spanish registry was created, and ten hospitals using HIFU to ablate AF joined it. A total of 412 patients undergoing cardiac surgery between 2006 and February 2013 were included. AF was divided between paroxysmal AF (33%) and persistent AF (67%) with a mean AF duration of 29.3±108.2 months. Mean left atrial diameter was 51.2±6.5 mm. Mean underlying heart disease were aortic valve disease (49.3%), ischemic disease (25.2%) and mitral disease (33.2%) Clinical follow-up of patients and a 6 months postoperative echocardiogram were performed in all patients. A pacemaker implantation was needed in 4.9% of patients with a perioperative stroke in 2.5%. Rhythm at discharge from hospital was sinus rhythm in 58%, AF in 35.9% and atrial flutter in 0.8% of patients. Sinus rhythm restoration at 6, 12, 24 and 36 months follow-up was achieved in 66.1%, 63.8%, 63.9% and 45.9% of patients respectively. Multivariate analysis showed paroxysmal AF and sinus rhythm restoration in the operating theatre as factors related to sinus rhythm long term restoration. The Spanish national registry showed an efficacy of AF ablation with the HIFU Epicor system of 66.1%, 63.8%, 63.9% and 45.9% at 6, 12, 24 and 36 months follow-up. There were no device-related complications.

Lymphatic Anomalies Registry

ClinicalTrials.gov

2018-01-23

Lymphatic Malformation; Generalized Lymphatic Anomaly (GLA); Central Conducting Lymphatic Anomaly; CLOVES Syndrome; Gorham-Stout Disease ("Disappearing Bone Disease"); Blue Rubber Bleb Nevus Syndrome; Kaposiform Lymphangiomatosis; Kaposiform Hemangioendothelioma/Tufted Angioma; Klippel-Trenaunay Syndrome; Lymphangiomatosis

Semi-Automated Annotation of Biobank Data Using Standard Medical Terminologies in a Graph Database.

PubMed

Hofer, Philipp; Neururer, Sabrina; Goebel, Georg

2016-01-01

Data describing biobank resources frequently contains unstructured free-text information or insufficient coding standards. (Bio-) medical ontologies like Orphanet Rare Diseases Ontology (ORDO) or the Human Disease Ontology (DOID) provide a high number of concepts, synonyms and entity relationship properties. Such standard terminologies increase quality and granularity of input data by adding comprehensive semantic background knowledge from validated entity relationships. Moreover, cross-references between terminology concepts facilitate data integration across databases using different coding standards. In order to encourage the use of standard terminologies, our aim is to identify and link relevant concepts with free-text diagnosis inputs within a biobank registry. Relevant concepts are selected automatically by lexical matching and SPARQL queries against a RDF triplestore. To ensure correctness of annotations, proposed concepts have to be confirmed by medical data administration experts before they are entered into the registry database. Relevant (bio-) medical terminologies describing diseases and phenotypes were identified and stored in a graph database which was tied to a local biobank registry. Concept recommendations during data input trigger a structured description of medical data and facilitate data linkage between heterogeneous systems.

The results of interconnection of the evidence of professional exposure to genotoxic factors (regex) and cancer registry in the Czech Republic.

PubMed

Lehocká, Hana; Závacká, Ivona; Vavrošová, Jana; Janout, Vladimír

2017-03-01

The aim of this study is to analyze the genotoxic risks in the Moravian-Silesian Region in the Czech Republic and assess the significance of genotoxic factors in the etiology of cancer by bringing together the Registry of Occupational Exposure to Genotoxic Factors and the Cancer Registry and compare the rate of detected cancer in persons exposed to genotoxic factors via their work in the Moravian-Silesian Region with the occurrence of cancer in the population of the Czech Republic. The results show: (a) For the monitored group (748 person) for the period 1996-2008, according to gender, was no statistically significant difference in the incidence of oncological diseases compared to the population of the Czech Republic. (b) But statistically significant difference was found in the cases of oncological diseases in groups according to % AB.C. using the Cytogenetic analysis of human peripheral lymphocytes (CAPL). The highest incidence was in the group with a higher incidence of % AB.C. High values of % AB.C. may predict the development of oncological diseases.

Gaps along the HIV care continuum: findings among a population seeking sexual health care services in New York City.

PubMed

Pathela, Preeti; Jamison, Kelly; Braunstein, Sarah L; Schillinger, Julia A; Tymejczyk, Olga; Nash, Denis

2018-03-02

Linkage/re-linkage to HIV care for virally unsuppressed persons with new sexually transmitted infections is critical for ending the HIV epidemic. We quantified HIV care continuum gaps, and viral suppression, among HIV-positive patients attending New York City (NYC) sexual health clinics (SHC). 1,649 HIV-positive patients and a 10% sample of 11,954 patients with unknown HIV status on clinic visit date (DOV) were matched against the NYC HIV registry. Using registry diagnosis dates, we categorized matched HIV-positive patients as "new-positives" (newly diagnosed on DOV), "recent-positives (diagnosed ≤90 days before DOV), "prevalent-positives" (diagnosed >90 days before DOV), and "unknown-positives" (previously diagnosed, but status unknown to clinic on DOV). We assessed HIV care continuum outcomes before and after DOV for new-positives, prevalent-positives, and unknown-positives using registry laboratory data. In addition to 1,626 known HIV-positive patients, 5% of the unknown sample (63/1,196) matched to the registry, signifying that about 630 additional HIV-positive patients attended SHCs. Of new-positives, 65% were linked to care after DOV. Of prevalent-positives, 66% were in care on DOV; 43% of the out-of-care were re-linked after DOV. Of unknown-positives, 40% were in care on DOV; 21% of the out-of-care re-linked after DOV. Viral suppression was achieved by: 88% of in-care unknown-positives, 76% in-care prevalent-positives, 50% new-positives, 42% out-of-care prevalent-positives, and 16% out-of-care unknown-positives. Many HIV-positive persons, including those with uncontrolled HIV infection, attend SHCs and potentially contribute to HIV spread. However, HIV status often is not known to staff, resulting in missed linkage/re-linkage to care opportunities. Better outcomes could be facilitated by real-time ascertainment of HIV status and HIV care status.

Analysis of the time and workers needed to conduct systematic reviews of medical interventions using data from the PROSPERO registry.

PubMed

Borah, Rohit; Brown, Andrew W; Capers, Patrice L; Kaiser, Kathryn A

2017-02-27

To summarise logistical aspects of recently completed systematic reviews that were registered in the International Prospective Register of Systematic Reviews (PROSPERO) registry to quantify the time and resources required to complete such projects. Meta-analysis. All of the 195 registered and completed reviews (status from the PROSPERO registry) with associated publications at the time of our search (1 July 2014). All authors extracted data using registry entries and publication information related to the data sources used, the number of initially retrieved citations, the final number of included studies, the time between registration date to publication date and number of authors involved for completion of each publication. Information related to funding and geographical location was also recorded when reported. The mean estimated time to complete the project and publish the review was 67.3 weeks (IQR=42). The number of studies found in the literature searches ranged from 27 to 92 020; the mean yield rate of included studies was 2.94% (IQR=2.5); and the mean number of authors per review was 5, SD=3. Funded reviews took significantly longer to complete and publish (mean=42 vs 26 weeks) and involved more authors and team members (mean=6.8 vs 4.8 people) than those that did not report funding (both p<0.001). Systematic reviews presently take much time and require large amounts of human resources. In the light of the ever-increasing volume of published studies, application of existing computing and informatics technology should be applied to decrease this time and resource burden. We discuss recently published guidelines that provide a framework to make finding and accessing relevant literature less burdensome. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Clinical and Patient-reported Outcomes in Patients with Psoriatic Arthritis (PsA) by Body Surface Area Affected by Psoriasis: Results from the Corrona PsA/Spondyloarthritis Registry.

PubMed

Mease, Philip J; Karki, Chitra; Palmer, Jacqueline B; Etzel, Carol J; Kavanaugh, Arthur; Ritchlin, Christopher T; Malley, Wendi; Herrera, Vivian; Tran, Melody; Greenberg, Jeffrey D

2017-08-01

Psoriatic arthritis (PsA) is commonly comorbid with psoriasis; the extent of skin lesions is a major contributor to psoriatic disease severity/burden. We evaluated whether extent of skin involvement with psoriasis [body surface area (BSA) > 3% vs ≤ 3%] affects overall clinical and patient-reported outcomes (PRO) in patients with PsA. Using the Corrona PsA/Spondyloarthritis Registry, patient characteristics, disease activity, and PRO at registry enrollment were assessed for patients with PsA aged ≥ 18 years with BSA > 3% versus ≤ 3%. Regression models were used to evaluate associations of BSA level with outcome [modified minimal disease activity (MDA), Health Assessment Questionnaire (HAQ) score, patient-reported pain and fatigue, and the Work Productivity and Activity Impairment questionnaire score]. Adjustments were made for age, sex, race, body mass index, disease duration, and history of biologics, disease-modifying antirheumatic drug, and prednisone use. This analysis included 1240 patients with PsA with known BSA level (n = 451, BSA > 3%; n = 789, BSA ≤ 3%). After adjusting for potential confounding variables, patients with BSA > 3% versus ≤ 3% had greater patient-reported pain and fatigue and higher HAQ scores (p = 2.33 × 10 -8 , p = 0.002, and p = 1.21 × 10 -7 , respectively), were 1.7× more likely not to be in modified MDA (95% CI 1.21-2.41, p = 0.002), and were 2.1× more likely to have overall work impairment (1.37-3.21, p = 0.0001). These Corrona Registry data show that substantial skin involvement (BSA > 3%) is associated with greater PsA disease burden, underscoring the importance of assessing and effectively managing psoriasis in patients with PsA because this may be a contributing factor in PsA severity.

Childhood Vesicoureteral Reflux Studies: Registries and Repositories Sources and Nosology

PubMed Central

Chesney, Russell W.; Patters, Andrea B.

2012-01-01

Despite several recent studies, the advisability of antimicrobial prophylaxis and certain imaging studies for urinary tract infections (UTIs) remains controversial. The role of vesicoureteral reflux (VUR) on the severity and re-infection rates for UTIs is also difficult to assess. Registries and repositories of data and biomaterials from clinical studies in children with VUR are valuable. Disease registries are collections of secondary data related to patients with a specific diagnosis, condition or procedure. Registries differ from indices in that they contain more extensive data. A research repository is an entity that receives, stores, processes and/or disseminates specimens (or other materials) as needed. It encompasses the physical location as well as the full range of activities associated with its operation. It may also be referred to as a biorepository. This report provides information about some current registries and repositories that include data and samples from children with VUR. It also describes the heterogeneous nature of the subjects, as some registries and repositories include only data or samples from patients with primary reflux while others also include those from patients with syndromic or secondary reflux. PMID:23044377

Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study.

PubMed

Hensman Moss, Davina J; Pardiñas, Antonio F; Langbehn, Douglas; Lo, Kitty; Leavitt, Blair R; Roos, Raymund; Durr, Alexandra; Mead, Simon; Holmans, Peter; Jones, Lesley; Tabrizi, Sarah J

2017-09-01

Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure. We generated a progression score on the basis of principal component analysis of prospectively acquired longitudinal changes in motor, cognitive, and imaging measures in the 218 indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008-11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers (data collected 2003-13). We did a genome-wide association analyses in terms of progression for 216 TRACK-HD participants and 1773 REGISTRY participants, then a meta-analysis of these results was undertaken. Longitudinal motor, cognitive, and imaging scores were correlated with each other in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression in TRACK-HD and REGISTRY gave a genome-wide significant signal (p=1·12 × 10 -10 ) on chromosome 5 spanning three genes: MSH3, DHFR, and MTRNR2L2. The genes in this locus were associated with progression in TRACK-HD (MSH3 p=2·94 × 10 -8 DHFR p=8·37 × 10 -7 MTRNR2L2 p=2·15 × 10 -9 ) and to a lesser extent in REGISTRY (MSH3 p=9·36 × 10 -4 DHFR p=8·45 × 10 -4 MTRNR2L2 p=1·20 × 10 -3 ). The lead single nucleotide polymorphism (SNP) in TRACK-HD (rs557874766) was genome-wide significant in the meta-analysis (p=1·58 × 10 -8 ), and encodes an aminoacid change (Pro67Ala) in MSH3. In TRACK-HD, each copy of the minor allele at this SNP was associated with a 0·4 units per year (95% CI 0·16-0·66) reduction in the rate of change of the Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score, and a reduction of 0·12 units per year (95% CI 0·06-0·18) in the rate of change of UHDRS Total Functional Capacity score. These associations remained significant after adjusting for age of onset. The multidomain progression measure in TRACK-HD was associated with a functional variant that was genome-wide significant in our meta-analysis. The association in only 216 participants implies that the progression measure is a sensitive reflection of disease burden, that the effect size at this locus is large, or both. Knockout of Msh3 reduces somatic expansion in Huntington's disease mouse models, suggesting this mechanism as an area for future therapeutic investigation. The European Commission FP7 NeurOmics project; CHDI Foundation; the Medical Research Council UK; the Brain Research Trust; and the Guarantors of Brain. Copyright © 2017 Elsevier Ltd. All rights reserved.

Quality improvement tools for chronic disease care--more effective processes are less likely to be implemented in developing countries.

PubMed

Hashim, Muhammad Jawad; Prinsloo, Adrianna; Mirza, Deen M

2013-01-01

Chronic disease services may be improved if care management processes (CMPs), such as disease-specific flowsheets and chronic disease registries, are used. The newly industrialized Gulf state health service has underdeveloped primary care but higher diabetes prevalence. This paper's aim is to investigate care management processes in United Arab Emirates (UAE) primary care clinics to explore these issues. A cross-sectional survey using self-administered questionnaires given to family physicians and nurses attending a UAE University workshop was used to collect data. All 38 participants completed the questionnaire: 68 per cent were women and 81 per cent physicians. Care management processes in use included: medical records, 76 per cent; clinical guidelines, 74 per cent; chronic disease care rooms, 74 per cent; disease-specific flowsheets, 61 per cent; medical record audits, 57 per cent; chronic disease nurse-educators, 58 per cent; electronic medical records (EMR), 34 per cent; and incentive plans based on clinical performance, 21 per cent. Only 62 per cent and 48 per cent reported that flowsheets and problem lists, respectively, were completed by physicians. Responses to the open-ended question included using traditional quality improvement (QI) approaches such as continuing education and staff meetings, but not proactive systems such as disease registries and self-management. The study used a small, non-random sample and the survey instrument's psychometric properties were not collected. Chronic disease care CMPs are present in UAE clinics but use is limited. Quality improvement should include disease registries, reminder-tracking systems, patient self-management support and quality incentives. This report highlights the lag regarding adopting more effective CMPs in developing countries.

The role of registries in rare genetic lipid disorders: Review and introduction of the first global registry in lipoprotein lipase deficiency.

PubMed

Steinhagen-Thiessen, Elisabeth; Stroes, Erik; Soran, Handrean; Johnson, Colin; Moulin, Philippe; Iotti, Giorgio; Zibellini, Marco; Ossenkoppele, Bas; Dippel, Michaela; Averna, Maurizio R

2017-07-01

A good understanding of the natural history of rare genetic lipid disorders is a pre-requisite for successful patient management. Disease registries have been helpful in this regard. Lipoprotein Lipase Deficiency (LPLD) is a rare, autosomal-recessive lipid disorder characterized by severe hypertriglyceridemia and a very high risk for recurrent acute pancreatitis, however, only limited data are available on its natural course. Alipogene tiparvovec (Glybera ® ) is the first gene therapy to receive Marketing Authorization in the European Union; GENIALL (GENetherapy In the MAnagement of Lipoprotein Lipase Deficiency), a 15-year registry focusing on LPLD was launched in 2014 as part of its Risk Management Plan. The aim of this publication is to introduce the GENIALL Registry within a structured literature review of registries in rare genetic lipid disorders. A total of 11 relevant initiatives/registries were identified (homozygous Familial Hypercholesterolemia (hoFH) [n = 5]; LPLD [n = 1]; Lysosomal Acid Lipase Deficiency [LALD, n = 1], detection of mutations in genetic lipid disorders [n = 4]). Besides one product registry in hoFH and the LALD registry, all other initiatives are local or country-specific. GENIALL is the first global prospective registry in LPLD that will collect physician and patient generated data on the natural course of LPLD, as well as long-term outcomes of gene therapy. There is a limited number of international initiatives focusing on the natural course of specific rare genetic lipid disorders. The GENIALL LPLD Registry could be the first step towards a future broader global initiative that collects data related to familial chylomicronemia syndrome and their underlying genetic causes. Copyright © 2016. Published by Elsevier B.V.

Increased risks of coronary heart disease and stroke among spousal caregivers of cancer patients.

PubMed

Ji, Jianguang; Zöller, Bengt; Sundquist, Kristina; Sundquist, Jan

2012-04-10

Spousal caregivers of cancer patients suffer psychological and physical burdens that may affect their risk of subsequently developing coronary heart disease and stroke. Cancer patients were identified in the Swedish Cancer Registry, and information on their spouses was retrieved from the Swedish Multi-Generation Register. Follow-up of caregivers was performed from the date of the first diagnosis of cancer in their spouses through 2008. Standardized incidence ratios were calculated for spousal caregivers of cancer patients compared with those without an affected spouse. After the cancer diagnosis in wives, the risks of coronary heart disease, ischemic stroke, and hemorrhagic stroke in husbands were 1.13 (95% confidence interval [CI], 1.10-1.16), 1.24 (95% CI, 1.21-1.27), and 1.25 (95% CI, 1.18-1.32), respectively. The corresponding risks in wives with an affected husband were 1.13 (95% CI, 1.10-1.16), 1.29 (95% CI, 1.26-1.32), and 1.27 (95% CI, 1.19-1.34). The increases were consistent over time and were more pronounced if the spouse was affected by a cancer with a high mortality rate, such as pancreatic and lung cancers. Spousal caregivers of cancer patients have increased risks of coronary heart disease and stroke that persist over time. Clinical attention should be paid to spousal caregivers, especially those caring for cancer patients with high mortality rates.

77 FR 45350 - Information Collection Being Submitted for Review and Approval to the Office of Management and...

Federal Register 2010, 2011, 2012, 2013, 2014

2012-07-31

... to the Office of Management and Budget AGENCY: Federal Communications Commission. ACTION: Notice and... of Management and Budget (OMB) control number. DATES: Written comments should be submitted on or... the do-not-call registry in 2009. See 74 FR 17863, April 17, 2009. Privacy Impact Assessment: Yes. The...

DOE Office of Scientific and Technical Information (OSTI.GOV)

David, S.A.; Goodwin, G.M.; Gardner, K.

This document contains a listing of the written scientific information originating in the Materials Joining (formerly the Welding and Brazing Group), Metals and Ceramics Division, Oak Ridge National Laboratory during 1951 through June 1989. This registry of documents is as much as possible, in the order of issue date. A complete cross-referenced listing of the technical literature of the Metals and Ceramics Division is also available.

76 FR 53301 - Airworthiness Directives; Saab AB, Saab Aerosystems Model SAAB 2000 Airplanes

Federal Register 2010, 2011, 2012, 2013, 2014

2011-08-26

....S. registry. We also estimate that it will take about 12 work-hours per product to comply with the basic requirements of this AD. The average labor rate is $85 per work-hour. Based on these figures, we... 01, dated September 3, 2009. (j) Within 30 days after accomplishing the inspection required by...

Developing a caries risk registry to support caries risk assessment and management for children: A quality improvement initiative.

PubMed

Ruff, Jesley C; Herndon, Jill Boylston; Horton, Roger A; Lynch, Julie; Mathwig, Dawn C; Leonard, Audra; Aravamudhan, Krishna

2017-10-27

Health registries are commonly used in medicine to support public health activities and are increasingly used in quality improvement (QI) initiatives. Illustrations of dental registries and their QI applications are lacking. Within dentistry, caries risk assessment implementation and documentation are vital to optimal patient care. The purpose of this article is to describe the processes used to develop a caries risk assessment registry as a QI initiative to support clinical caries risk assessment, caries prevention, and disease management for children. Developmental steps reflected Agency for Healthcare Research and Quality recommendations for planning QI registries and included engaging "champions," defining the project, identifying registry features, defining performance dashboard indicators, and pilot testing with participant feedback. We followed Standards for Quality Improvement Reporting Excellence guidelines. Registry eligibility is patients aged 0-17 years. QI tools include prompts to register eligible patients; decision support tools grounded in evidence-based guidelines; and performance dashboard reports delivered at the provider and aggregated levels at regular intervals. The registry was successfully piloted in two practices with documented caries risk assessment increasing from 57 percent to 92 percent and positive feedback regarding the potential to improve dental practice patient centeredness, patient engagement and education, and quality of care. The caries risk assessment registry demonstrates how dental registries may be used in QI efforts to promote joint patient and provider engagement, foster shared decision making, and systematically collect patient information to generate timely and actionable data to improve care quality and patient outcomes at the individual and population levels. © 2017 American Association of Public Health Dentistry.

Czech Registry of Monoclonal Gammopathies - Technical Solution, Data Collection and Visualisation.

PubMed

Brozova, L; Schwarz, D; Snabl, I; Kalina, J; Pavlickova, B; Komenda, M; Jarkovský, J; Němec, P; Horinek, D; Stefanikova, Z; Pour, L; Hájek, R; Maisnar, V

2017-01-01

The Registry of Monoclonal Gammopathies (RMG) was established by the Czech Myeloma Group in 2007. RMG is a registry designed for the collection of clinical data concerning diagnosis, treatment, treatment results and survival of patients with monoclonal gammopathies. Data on patients with monoclonal gammopathy of undetermined significance (MGUS), Waldenström macroglobulinaemia (WM), multiple myeloma (MM) or primary AL ("amyloid light-chain") amyloidosis are collected in the registry. Nineteen Czech centres and four Slovak centres currently contribute to the registry. The registry currently contains records on more than 5,000 patients with MM, almost 3,000 patients with MGUS, 170 patients with WM and 26 patients with primary AL amyloidosis, i.e. more than 8,000 records on patients with monoclonal gammopathies altogether. This paper describes technology employed for the collection, storage and subsequent online visualisation of data. The CLADE-IS platform is introduced as a new system for the collection and storage of data from the registry. The form structure and functions of the new system are described for all diagnoses in general; these functions facilitate data entry to the registry and minimise the error rate in data. Publicly available online visualisations of data on patients with MGUS, WM, MM or primary AL amyloidosis from all Czech or Slovak centres are introduced, together with authenticated visualisations of data on patients with MM from selected centres. The RMG represents a data basis that makes it possible to monitor the disease course in patients with monoclonal gammopathies on the population level.Key words: Registry of Monoclonal Gammopathies - RMG - registries - monoclonal gammopathies - CLADE-IS - data visualisation - database.

The Kola Birth Registry and perinatal mortality in Moncegorsk, Russia.

PubMed

Vaktskjold, Arild; Talykova, Ljudmila; Chashchin, Valerij; Nieboer, Evert; Odland, Jon Øyvind

2004-01-01

A population-based birth registry has been set up for the Arctic town of Moncegorsk in north-western Russia. In this investigation, the quality and the content of the registry are assessed and the perinatal mortality (PM) rates in the period 1973-97 estimated. Enrollment in the Kola Birth Registry (KBR) involved the retrospective inclusion of all births with at least 28 weeks of gestation in Moncegorsk in the period 1973-97. The data in the registry were assessed for data entry errors, completeness of data and population coverage. The annual PM rates were estimated for live- and stillborns with at least 28 weeks of gestation. The KBR contains detailed information about the newborn, delivery, pregnancy and mother for 21 214 births by women from Moncegorsk, covering at least 96% of all the births by the population in the period studied. No records were missing data for gender and birth date of the newborn, and more than 99.9% of the records contained data about gestational age and birthweight. Data concerning the mothers' employment were missing in 0.4% of the records. The annual PM rate fell from more than 20 to less than 10 deaths per 1000 births during this period. The KBR provides an extensive data source useful for case-control and register-based prospective studies, and constitutes the first such compilation in Russia. The homogeneity of the population in Moncegorsk makes it advantageous for epidemiological investigations. The PM rate in Moncegorsk was lower than the overall rate in Russia.

Proving Value in Radiology: Experience Developing and Implementing a Shareable Open Source Registry Platform Driven by Radiology Workflow.

PubMed

Gichoya, Judy Wawira; Kohli, Marc D; Haste, Paul; Abigail, Elizabeth Mills; Johnson, Matthew S

2017-10-01

Numerous initiatives are in place to support value based care in radiology including decision support using appropriateness criteria, quality metrics like radiation dose monitoring, and efforts to improve the quality of the radiology report for consumption by referring providers. These initiatives are largely data driven. Organizations can choose to purchase proprietary registry systems, pay for software as a service solution, or deploy/build their own registry systems. Traditionally, registries are created for a single purpose like radiation dosage or specific disease tracking like diabetes registry. This results in a fragmented view of the patient, and increases overhead to maintain such single purpose registry system by requiring an alternative data entry workflow and additional infrastructure to host and maintain multiple registries for different clinical needs. This complexity is magnified in the health care enterprise whereby radiology systems usually are run parallel to other clinical systems due to the different clinical workflow for radiologists. In the new era of value based care where data needs are increasing with demand for a shorter turnaround time to provide data that can be used for information and decision making, there is a critical gap to develop registries that are more adapt to the radiology workflow with minimal overhead on resources for maintenance and setup. We share our experience of developing and implementing an open source registry system for quality improvement and research in our academic institution that is driven by our radiology workflow.

Potential coeliac disease markers and autoimmunity in olmesartan induced enteropathy: A population-based study.

PubMed

Esteve, Maria; Temiño, Rocío; Carrasco, Anna; Batista, Lissette; Del Val, Adolfo; Blé, Michel; Santaolaria, Santos; Molina-Infante, Javier; Soriano, Germán; Agudo, Sandra; Zabana, Yamile; Andújar, Xavier; Aceituno, Montserrat; Ribes, Josepa; Madridejos, Rosa; Fernández-Bañares, Fernando

2016-02-01

(1) Assess the population-based incidence of severe olmesartan-associated enteropathy. (2) To describe patients of the Spanish registry. (3) Evaluate markers of potential coeliac disease and associated autoimmunity. Crude incidence rates in the area of Terrassa (Catalonia) were calculated. Clinical characteristics of patients in the Spanish registry were collected. Duodenal lymphocyte subpopulations and anti-TG2 IgA deposits were assessed in a subset of patients. Annual incidence rates (2011-2014) ranged from 0 to 22 cases per 10(4) treated patients. Twenty patients were included in the Spanish registry. Nineteen (95%) exhibited villous atrophy and 16 (80%) had severe enteropathy. Lupus-like disease occurred during olmesartan treatment in 3 patients. HLA-DQ2/DQ8 was positive in 64%. Markers of potential coeliac disease were present in 4 out of 8 patients (positive anti-TG2 deposits and/or increased CD3+gammadelta+ intraepithelial lymphocytes and reduced CD3-). Histopathological changes and clinical manifestations including autoimmune disorders improved after olmesartan discontinuation but not after gluten-free diet, irrespective of the presence or absence of coeliac markers. Incidence of severe olmesartan-associated enteropathy was low. Autoimmune phenomena were present in a subset of cases and reversed after olmesartan removal. A genetic coeliac disease background and the presence of potential coeliac markers might uncover predisposing factors. Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Vision for a Global Registry of Anticipated Public Health Studies

PubMed Central

Choi, Bernard C.K.; Frank, John; Mindell, Jennifer S.; Orlova, Anna; Lin, Vivian; Vaillancourt, Alain D.M.G.; Puska, Pekka; Pang, Tikki; Skinner, Harvey A.; Marsh, Marsha; Mokdad, Ali H.; Yu, Shun-Zhang; Lindner, M. Cristina; Sherman, Gregory; Barreto, Sandhi M.; Green, Lawrence W.; Svenson, Lawrence W.; Sainsbury, Peter; Yan, Yongping; Zhang, Zuo-Feng; Zevallos, Juan C.; Ho, Suzanne C.; de Salazar, Ligia M.

2007-01-01

In public health, the generation, management, and transfer of knowledge all need major improvement. Problems in generating knowledge include an imbalance in research funding, publication bias, unnecessary studies, adherence to fashion, and undue interest in novel and immediate issues. Impaired generation of knowledge, combined with a dated and inadequate process for managing knowledge and an inefficient system for transferring knowledge, mean a distorted body of evidence available for decisionmaking in public health. This article hopes to stimulate discussion by proposing a Global Registry of Anticipated Public Health Studies. This prospective, comprehensive system for tracking research in public health could help enhance collaboration and improve efficiency. Practical problems must be discussed before such a vision can be further developed. PMID:17413073

42 CFR 50.402 - To what program do these regulations apply?

Code of Federal Regulations, 2010 CFR

2010-10-01

... block grants, which are administered by the National Institutes of Health; The Centers for Disease Control and Prevention; the Agency for Toxic Substances and Disease Registry; the Food and Drug...

42 CFR 50.402 - To what program do these regulations apply?

Code of Federal Regulations, 2014 CFR

2014-10-01

... block grants, which are administered by the National Institutes of Health; The Centers for Disease Control and Prevention; the Agency for Toxic Substances and Disease Registry; the Food and Drug...

42 CFR 50.402 - To what program do these regulations apply?

Code of Federal Regulations, 2013 CFR

2013-10-01

... block grants, which are administered by the National Institutes of Health; The Centers for Disease Control and Prevention; the Agency for Toxic Substances and Disease Registry; the Food and Drug...

42 CFR 50.402 - To what program do these regulations apply?

Code of Federal Regulations, 2012 CFR

2012-10-01

... block grants, which are administered by the National Institutes of Health; The Centers for Disease Control and Prevention; the Agency for Toxic Substances and Disease Registry; the Food and Drug...

42 CFR 50.402 - To what program do these regulations apply?

Code of Federal Regulations, 2011 CFR

2011-10-01

... block grants, which are administered by the National Institutes of Health; The Centers for Disease Control and Prevention; the Agency for Toxic Substances and Disease Registry; the Food and Drug...

Genetics Home Reference: Paget disease of bone

MedlinePlus

... genetic cause of classic Paget disease of bone , accounting for 10 to 50 percent of cases that ... be inherited? More about Inheriting Genetic Conditions Diagnosis & Management Resources Genetic Testing (6 links) Genetic Testing Registry: ...

National Niemann-Pick Disease Foundation

MedlinePlus

... Disease Registry News & Media NNPDF Newsletters Foundation NewsLine Print Resources Video Resources NNPDF Webinars Vision of Hope ... nor does it host or receive funding from advertising or from the display of commercial content. This ...

Military Exposures

MedlinePlus

... burn pit registry, research findings, joint VA and Department of Defense programs, and more. Learn more » 4 Ways to Find Exposures Related Health Concerns Agent Orange Related Diseases Gulf War Veterans' Illnesses Radiation Related Diseases Vaccinations & Medications More ...

Quebec Trophoblastic Disease Registry: how to make an easy-to-use dynamic database.

PubMed

Sauthier, Philippe; Breguet, Magali; Rozenholc, Alexandre; Sauthier, Michaël

2015-05-01

To create an easy-to-use dynamic database designed specifically for the Quebec Trophoblastic Disease Registry (RMTQ). It is now well established that much of the success in managing trophoblastic diseases comes from the development of national and regional reference centers. Computerized databases allow the optimal use of data stored in these centers. We have created an electronic data registration system by producing a database using FileMaker Pro 12. It uses 11 external tables associated with a unique identification number for each patient. Each table allows specific data to be recorded, incorporating demographics, diagnosis, automated staging, laboratory values, pathological diagnosis, and imaging parameters. From January 1, 2009, to December 31, 2013, we used our database to register 311 patients with 380 diseases and have seen a 39.2% increase in registrations each year between 2009 and 2012. This database allows the automatic generation of semilogarithmic curves, which take into account β-hCG values as a function of time, complete with graphic markers for applied treatments (chemotherapy, radiotherapy, or surgery). It generates a summary sheet for a synthetic vision in real time. We have created, at a low cost, an easy-to-use database specific to trophoblastic diseases that dynamically integrates staging and monitoring. We propose a 10-step procedure for a successful trophoblastic database. It improves patient care, research, and education on trophoblastic diseases in Quebec and leads to an opportunity for collaboration on a national Canadian registry.

Enhancing Cancer Registry Data for Comparative Effectiveness Research (CER) Project: Overview and Methodology

PubMed Central

Chen, Vivien W.; Eheman, Christie R.; Johnson, Christopher J.; Hernandez, Monique N.; Rousseau, David; Styles, Timothy S.; West, Dee W.; Hsieh, Meichin; Hakenewerth, Anne M.; Celaya, Maria O.; Rycroft, Randi K.; Wike, Jennifer M.; Pearson, Melissa; Brockhouse, Judy; Mulvihill, Linda G.; Zhang, Kevin B.

2015-01-01

Following the Institute of Medicine's 2009 report on the national priorities for comparative effectiveness research (CER), funding for support of CER became available in 2009 through the American Recovery and Reinvestment Act. The Centers for Disease Control and Prevention (CDC) received funding to enhance the infrastructure of population-based cancer registries and to expand registry data collection to support CER. The CDC established 10 specialized registries within the National Program of Cancer Registries (NPCR) to enhance data collection for all cancers and to address targeted CER questions, including the clinical use and prognostic value of specific biomarkers. The project also included a special focus on detailed first course of treatment for cancers of the breast, colon, and rectum, as well as chronic myeloid leukemia (CML) diagnosed in 2011. This paper describes the methodology and the work conducted by the CDC and the NPCR specialized registries in collecting data for the 4 special focused cancers, including the selection of additional data variables, development of data collection tools and software modifications, institutional review board approvals, training, collection of detailed first course of treatment, and quality assurance. It also presents the characteristics of the study population and discusses the strengths and limitations of using population-based cancer registries to support CER as well as the potential future role of population-based cancer registries in assessing the quality of patient care and cancer control. PMID:25419602

Enhancing cancer registry data for comparative effectiveness research (CER) project: overview and methodology.

PubMed

Chen, Vivien W; Eheman, Christie R; Johnson, Christopher J; Hernandez, Monique N; Rousseau, David; Styles, Timothy S; West, Dee W; Hsieh, Meichin; Hakenewerth, Anne M; Celaya, Maria O; Rycroft, Randi K; Wike, Jennifer M; Pearson, Melissa; Brockhouse, Judy; Mulvihill, Linda G; Zhang, Kevin B

2014-01-01

Following the Institute of Medicine's 2009 report on the national priorities for comparative effectiveness research (CER), funding for support of CER became available in 2009 through the American Recovery and Re-investment Act. The Centers for Disease Control and Prevention (CDC) received funding to enhance the infrastructure of population-based cancer registries and to expand registry data collection to support CER. The CDC established 10 specialized registries within the National Program of Cancer Registries (NPCR) to enhance data collection for all cancers and to address targeted CER questions, including the clinical use and prognostic value of specific biomarkers. The project also included a special focus on detailed first course of treatment for cancers of the breast, colon, and rectum, as well as chronic myeloid leukemia (CML) diagnosed in 2011. This paper describes the methodology and the work conducted by the CDC and the NPCR specialized registries in collecting data for the 4 special focused cancers, including the selection of additional data variables, development of data collection tools and software modifications, institutional review board approvals, training, collection of detailed first course of treatment, and quality assurance. It also presents the characteristics of the study population and discusses the strengths and limitations of using population-based cancer registries to support CER as well as the potential future role of population-based cancer registries in assessing the quality of patient care and cancer control.

The Wisconsin immunization registry experience: comparing real-time and batched file submissions from health care providers.

PubMed

Schauer, Stephanie L; Maerz, Thomas R; Verdon, Matthew J; Hopfensperger, Daniel J; Davis, Jeffrey P

2014-06-01

The Wisconsin Immunization Registry is a confidential, web-based system used since 1999 as a centralized repository of immunization information for Wisconsin residents. Provide evidence based on Registry experiences with electronic data exchange, comparing the benefits and drawbacks of using the Health Level 7 standard, including the option for real time data exchange vs the flat file method. For data regarding vaccinations received by children aged 4 months through 6 years with Wisconsin addresses that were submitted to the Registry during 2010 and 2011, data timeliness (days from vaccine administration to date information was received) and completeness (percentage of records received that include core data elements for electronic storage) were compared by file submission method. Data submitted using Health Level 7 were substantially more timely than data submitted using the flat file method. Additionally, data submitted using Health Level 7 were substantially more complete for each of the core elements compared to flat file submission. Health care organizations that submit electronic data to immunization information systems should be aware that the technical decision to use the Health Level 7 format, particularly if real-time data exchange is employed, can result in more timely and accurate data. This will assist clinicians in adhering to the Advisory Committee on Immunization Practices schedule and reducing over-immunization.

Rationale and Design of the Registry for Stones of the Kidney and Ureter (ReSKU): A Prospective Observational Registry to Study the Natural History of Urolithiasis Patients.

PubMed

Chang, Helena C; Tzou, David T; Usawachintachit, Manint; Duty, Brian D; Hsi, Ryan S; Harper, Jonathan D; Sorensen, Mathew D; Stoller, Marshall L; Sur, Roger L; Chi, Thomas

2016-12-01

Registry-based clinical research in nephrolithiasis is critical to advancing quality in urinary stone disease management and ultimately reducing stone recurrence. A need exists to develop Health Insurance Portability and Accountability Act (HIPAA)-compliant registries that comprise integrated electronic health record (EHR) data using prospectively defined variables. An EHR-based standardized patient database-the Registry for Stones of the Kidney and Ureter (ReSKU™)-was developed, and herein we describe our implementation outcomes. Interviews with academic and community endourologists in the United States, Canada, China, and Japan identified demographic, intraoperative, and perioperative variables to populate our registry. Variables were incorporated into a HIPAA-compliant Research Electronic Data Capture database linked to text prompts and registration data within the Epic EHR platform. Specific data collection instruments supporting New patient, Surgery, Postoperative, and Follow-up clinical encounters were created within Epic to facilitate automated data extraction into ReSKU. The number of variables within each instrument includes the following: New patient-60, Surgery-80, Postoperative-64, and Follow-up-64. With manual data entry, the mean times to complete each of the clinic-based instruments were (minutes) as follows: New patient-12.06 ± 2.30, Postoperative-7.18 ± 1.02, and Follow-up-8.10 ± 0.58. These times were significantly reduced with the use of ReSKU structured clinic note templates to the following: New patient-4.09 ± 1.73, Postoperative-1.41 ± 0.41, and Follow-up-0.79 ± 0.38. With automated data extraction from Epic, manual entry is obviated. ReSKU is a longitudinal prospective nephrolithiasis registry that integrates EHR data, lowering the barriers to performing high quality clinical research and quality outcome assessments in urinary stone disease.

Rationale and Design of the Registry for Stones of the Kidney and Ureter (ReSKU): A Prospective Observational Registry to Study the Natural History of Urolithiasis Patients

PubMed Central

Chang, Helena C.; Tzou, David T.; Usawachintachit, Manint; Duty, Brian D.; Hsi, Ryan S.; Harper, Jonathan D.; Sorensen, Mathew D.; Stoller, Marshall L.; Sur, Roger L.

2016-01-01

Abstract Objectives: Registry-based clinical research in nephrolithiasis is critical to advancing quality in urinary stone disease management and ultimately reducing stone recurrence. A need exists to develop Health Insurance Portability and Accountability Act (HIPAA)-compliant registries that comprise integrated electronic health record (EHR) data using prospectively defined variables. An EHR-based standardized patient database—the Registry for Stones of the Kidney and Ureter (ReSKU™)—was developed, and herein we describe our implementation outcomes. Materials and Methods: Interviews with academic and community endourologists in the United States, Canada, China, and Japan identified demographic, intraoperative, and perioperative variables to populate our registry. Variables were incorporated into a HIPAA-compliant Research Electronic Data Capture database linked to text prompts and registration data within the Epic EHR platform. Specific data collection instruments supporting New patient, Surgery, Postoperative, and Follow-up clinical encounters were created within Epic to facilitate automated data extraction into ReSKU. Results: The number of variables within each instrument includes the following: New patient—60, Surgery—80, Postoperative—64, and Follow-up—64. With manual data entry, the mean times to complete each of the clinic-based instruments were (minutes) as follows: New patient—12.06 ± 2.30, Postoperative—7.18 ± 1.02, and Follow-up—8.10 ± 0.58. These times were significantly reduced with the use of ReSKU structured clinic note templates to the following: New patient—4.09 ± 1.73, Postoperative—1.41 ± 0.41, and Follow-up—0.79 ± 0.38. With automated data extraction from Epic, manual entry is obviated. Conclusions: ReSKU is a longitudinal prospective nephrolithiasis registry that integrates EHR data, lowering the barriers to performing high quality clinical research and quality outcome assessments in urinary stone disease. PMID:27758162

Pooled analysis of the CONFIRM registries: impact of gender on procedure and angiographic outcomes in patients undergoing orbital atherectomy for peripheral artery disease.

PubMed

Lee, Michael S; Canan, Timothy; Rha, Seung-Woon; Mustapha, Jihad; Adams, George L

2015-02-01

To compare the acute procedure and angiographic outcomes of peripheral artery disease (PAD) patients treated with orbital atherectomy stratified by gender. The CONFIRM I, II, and III registries are US multicenter, nonrandomized, all-comers registries of PAD patients who were treated with orbital atherectomy. All patients with gender specified in the registry database were included in the current analysis, which compared the final residual stenosis achieved after atherectomy and the rate of acute complications in female and male patients. The 3 registries included 3131 patients with 4761 lesions: 1261 women (mean age 73.2 ± 10.7 years) with 1874 lesions and 1870 men (mean age 70.4 ± 10.2) with 2887 lesions. The women were older (p < 0.001) and had a higher but nonsignificant prevalence of critical limb ischemia (p = 0.075). After treatment, the final residual stenosis in women vs. men was 9% ± 11% vs. 11% ± 11%, respectively (p < 0.001). Women had a higher rate of all types of dissection (13.3% vs. 9.9%, p<0.001). However, both genders had similar rates of flow-limiting dissections (1.6% vs. 1.4%, p = 0.61), perforation, slow flow, vessel closure, spasm, embolism, and thrombus formation. The gender analysis of the CONFIRM registries revealed that there was successful lesion modification with orbital atherectomy in both men and women; however, women had a higher rate of dissection (all types). This difference is likely because of the older age and higher percentage of critical limb ischemia in women in this cohort. These results, however, suggest that additional studies should be completed to further understand the increased risks for women vs. men during endovascular procedures. © The Author(s) 2015.

Cost effectiveness of drug-eluting stents as compared with bare metal stents in patients with coronary artery disease.

PubMed

Wisløff, Torbjørn; Atar, Dan; Sønbø Kristiansen, Ivar

2013-01-01

The aim of this study was to estimate the incremental cost effectiveness of replacing bare metal stents (BMS) by drug-eluting stents (DES) when using trial data and registry data. We developed a Markov model (model of cost effectiveness of coronary artery disease) in which 60-year-old patients started by undergoing percutaneous coronary intervention for acute or subacute coronary artery disease. The patients are followed until death or 100 years of age. Data on the occurrence of events (revascularization, acute myocardial infarction, and death) were based on Scandinavian registry data. Separate analyses were conducted with data on effectiveness based on randomized controlled trials and patient registries. On using trial data, it was found that sirolimus-eluting stents (SES) yield 0.003 greater life expectancy and $3300 lower costs than do BMS (dominant strategy). Paclitaxel-eluting stents (PES) yield 0.148 more life years than do SES at additional lifetime costs of $2800 ($21,400 per life year gained). On using registry data, the cost per life year gained was found to be $4900 when replacing BMS with DES. Probabilistic sensitivity analyses, on the other hand, indicate that PES only has a 50%-75% probability of being cost effective, regardless of the type of effectiveness data. DESs are cost effective with current willingness to pay for life year gains. Whether PES or SES is the most effective DES remains uncertain.

Using linked administrative and disease-specific databases to study end-of-life care on a population level.

PubMed

Maetens, Arno; De Schreye, Robrecht; Faes, Kristof; Houttekier, Dirk; Deliens, Luc; Gielen, Birgit; De Gendt, Cindy; Lusyne, Patrick; Annemans, Lieven; Cohen, Joachim

2016-10-18

The use of full-population databases is under-explored to study the use, quality and costs of end-of-life care. Using the case of Belgium, we explored: (1) which full-population databases provide valid information about end-of-life care, (2) what procedures are there to use these databases, and (3) what is needed to integrate separate databases. Technical and privacy-related aspects of linking and accessing Belgian administrative databases and disease registries were assessed in cooperation with the database administrators and privacy commission bodies. For all relevant databases, we followed procedures in cooperation with database administrators to link the databases and to access the data. We identified several databases as fitting for end-of-life care research in Belgium: the InterMutualistic Agency's national registry of health care claims data, the Belgian Cancer Registry including data on incidence of cancer, and databases administrated by Statistics Belgium including data from the death certificate database, the socio-economic survey and fiscal data. To obtain access to the data, approval was required from all database administrators, supervisory bodies and two separate national privacy bodies. Two Trusted Third Parties linked the databases via a deterministic matching procedure using multiple encrypted social security numbers. In this article we describe how various routinely collected population-level databases and disease registries can be accessed and linked to study patterns in the use, quality and costs of end-of-life care in the full population and in specific diagnostic groups.

Participation in research improves overall patient management: insights from the Global Rheumatic Heart Disease registry (REMEDY).

PubMed

Prendergast, E A; Perkins, S; Engel, M E; Cupido, B; Francis, V; Joachim, A; Al Kebsi, M; Bode-Thomas, F; Damasceno, A; Abul Fadl, A; El Sayed, A; Gitura, B; Kennedy, N; Ibrahim, A; Mucumbitsi, J; Adeoye, A M; Musuku, J; Okello, E; Olunuga, T; Sheta, S; Mayosi, B M; Zühlke, L J

Rheumatic heart disease (RHD) is a major public health problem in low- and middle-income countries (LIMCs), with a paucity of high-quality trial data to improve patient outcomes. Investigators felt that involvement in a recent large, observational RHD study impacted positively on their practice, but this was poorly defined. The purpose of this study was to document the experience of investigators and research team members from LMICs who participated in a prospective, multi-centre study, the global Rheumatic Heart Disease Registry (REMEDY), conducted in 25 centres in 14 countries from 2010 to 2012. We conducted an online survey of site personnel to identify and quantify their experiences. Telephone interviews were conducted with a subset of respondents to gather additional qualitative data. We asked about their experiences, positive and negative, and about any changes in RHD management practices resulting from their participation in REMEDY as a registry site. The majority of respondents in both the survey and telephone interviews indicated that participation as a registry site improved their management of RHD patients. Administrative changes included increased attention to follow-up appointments and details in patient records. Clinical changes included increased use of penicillin prophylaxis, and more frequent INR monitoring and contraceptive counselling. Our study demonstrates that participation in clinical research on RHD can have a positive impact on patient management. Furthermore, REMEDY has led to increased patient awareness and improved healthcare workers' knowledge and efficiency in caring for RHD patients.

Evaluation of immunization data completeness within a large community health care system exchanging data with a state immunization information system.

PubMed

Hendrickson, Bryan K; Panchanathan, Sarada S; Petitti, Diana

2015-01-01

Information systems are used by most states to maintain registries of immunization data both for monitoring population-level adherence and for use in clinical practice and research. Direct data exchange between such systems and electronic health record systems presents an opportunity to improve the completeness and quality of information available. Our goals were to describe and compare the completeness of the Arizona State Immunization System, the electronic health record at a large community health provider in Arizona exchanging electronic data with the Arizona system, and personal immunization records in an effort to contribute to the discussion on the completeness of state-run immunization registries and data exchange with these registries. Immunization histories from these sources were collected and reviewed sequentially. Unique dates of vaccination administrations were counted for each patient and tagged on the basis of comparisons across sources. We quantified completeness by combining information from all 3 sources and comparing each source with the complete set. We determined that the state registry was 71.8% complete, the hospital electronic health record was 81.9% complete, and personal records were 87.8% complete. Of the 2017 unique vaccination administrations, 65% were present in all 3 sources, 24.6% in 2 of the 3 sources, and 10.4% in only 1 source. Only 11% of patients had records in complete agreement across the 3 sources. This study highlights issues related to data completeness, exchange, and reporting of immunization information to state registries and suggests that there is some degree of deficiency in completeness of immunization registries and other sources. This study indicates that there is a need to strengthen links between electronic data sources with immunization information and describes potential improvements in completeness that such efforts could provide, enabling providers to better rely on state immunization registries and to improve research utilization of immunization information systems.

The Savant Syndrome Registry: A Preliminary Report.

PubMed

Treffert, Darold A; Rebedew, David L

2015-08-01

A registry has been established to document certain characteristics on a sizeable worldwide sample of individuals with savant syndrome, a rare but remarkable condition in which persons with developmental disabilities, brain injury, or brain disease have some spectacular "islands" of skill or ability that stand in jarring, marked contrast to overall handicap. Of the 319 savants included in the registry, 90% are congenital savants, while 10% are acquired savants. The registry includes individuals from 33 countries, with 70% from the United States or Canada. Sex distribution was 79% male vs. 21% female (4:1). This report summarizes the findings in the congenital savant syndrome category of the registry. Among the individuals with congenital savant syndrome, the most common underlying disability was Autistic Spectrum Disorder (75%); various other central nervous system (CNS) disorders were present in the other 25%. Fifty-five percent possessed a single special skill, while 45% had multiple skills. Music was the most frequent principal skill followed by art, memory, mathematics, calendar calculating, language, visual-spatial/mechanical, athletic, computer, extrasensory perception, and other skills.

Leflunomide is equally efficacious and safe compared to low dose rituximab in refractory rheumatoid arthritis given in combination with methotrexate: results from a randomized double blind controlled clinical trial.

PubMed

Wijesinghe, Harindu; Galappatthy, Priyadharshini; de Silva, Rajiva; Seneviratne, Suranjith L; Saravanamuttu, Ushagowry; Udagama, Preethi; Hart, Melanie; Kelleher, Peter; Senerath, Upul; Fernandopulle, Rohini; Weerasekera, Lilani P; Wijayaratne, Lalith S

2017-07-19

The standard dose of rituximab used in rheumatoid arthritis (RA) is 1000 mg but recent studies have shown that low dose (500 mg) is also effective. Efficacy of low dose rituximab in rheumatoid arthritis (RA) refractory to first-line non-biologic Disease Modifying Anti Rheumatic Drugs (DMARDs), compared to leflunomide is unknown. In a tertiary care referral setting, we conducted a randomized, double blind controlled clinical trial comparing the efficacy and safety of low-dose rituximab-methotrexate combination with leflunomide-methotrexate combination. Patients on methotrexate (10-20 mg/week) with a Disease Activity Score (DAS) > 3.2 were randomly assigned to rituximab (500 mg on days 1 and 15) or leflunomide (10-20 mg/day). The primary end-point was ACR20 at 24 weeks. Sample of 40 had 70% power to detect a 30% difference. ACR50, ACR70, DAS, EULAR good response, CD3 + (T cell), CD19 + (B cell) and CD19 + CD27+ (memory B cell) counts, tetanus and pneumococcal antibody levels were secondary end points. Baseline characteristics were comparable in the two groups. At week 24, ACR20 was 85% vs 84% (p = 0.93), ACR50 was 60% vs. 64% (p = 0.79) and ACR70 was 35% vs 32% (P = 0.84), in rituximab and in leflunomide groups respectively. Serious adverse events were similar. With rituximab there was significant reduction in B cells (p < 0.001), memory B cells (p < 0.001) and pneumococcal antibody levels (P < 0.05) without significant changes in T cells (p = 0.835) and tetanus antibody levels (p = 0.424) at 24 weeks. With leflunomide, significant reduction in memory B cells (p < 0.01) and pneumococcal antibody levels (p < 0.01) occurred without significant changes in B cells (P > 0.05), T cells (P > 0.05) or tetanus antibody levels (P > 0.05). Leflunomide-methotrexate combination is as efficacious as low-dose rituximab-methotrexate combination at 24 weeks, in RA patient's refractory to initial DMARDs. The high responses seen in both groups have favorable cost implications for patients in developing countries. Changes in immune parameters with leflunomide are novel and need further characterization. The trial was registered with the Sri Lanka Clinical Trials Registry (SLCTR), a publicly accessible primary registry linked to the registry network of the International Clinical Trials Registry Platform of the WHO (WHO-ICTRP) (registration number: SLCTR/2008/008 dated 16th May 2008).

The History and Use of Cancer Registry Data by Public Health Cancer Control Programs in the United States

PubMed Central

White, Mary C.; Babcock, Frances; Hayes, Nikki S.; Mariotto, Angela B.; Wong, Faye L.; Kohler, Betsy A.; Weir, Hannah K.

2018-01-01

Because cancer registry data provide a census of cancer cases, registry data can be used to: 1) define and monitor cancer incidence at the local, state, and national levels; 2) investigate patterns of cancer treatment; and 3) evaluate the effectiveness of public health efforts to prevent cancer cases and improve cancer survival. The purpose of this article is to provide a broad overview of the history of cancer surveillance programs in the United States, and illustrate the expanding ways in which cancer surveillance data are being made available and contributing to cancer control programs. The article describes the building of the cancer registry infrastructure and the successful coordination of efforts among the 2 federal agencies that support cancer registry programs, the Centers for Disease Control and Prevention and the National Cancer Institute, and the North American Association of Central Cancer Registries. The major US cancer control programs also are described, including the National Comprehensive Cancer Control Program, the National Breast and Cervical Cancer Early Detection Program, and the Colorectal Cancer Control Program. This overview illustrates how cancer registry data can inform public health actions to reduce disparities in cancer outcomes and may be instructional for a variety of cancer control professionals in the United States and in other countries. PMID:29205307

Oral cancer in Libya and development of regional oral cancer registries: A review.

PubMed

BenNasir, E; El Mistiri, M; McGowan, R; Katz, R V

2015-10-01

The aims of this paper are three-fold: (1) to summarize the current epidemiological data on oral cancer in Libya as reported in the published literature and as compared to other national oral cancer rates in the region; (2) to present both the history of the early development, and future goals, of population-based oral cancer tumor registries in Libya as they partner with the more established regional and international population-based cancer tumor registries; and, (3) to offer recommendations that will likely be required in the near future if these nascent, population-based Libyan oral cancer registries are to establish themselves as on-going registries for describing the oral cancer disease patterns and risk factors in Libya as well as for prevention and treatment. This comprehensive literature review revealed that the current baseline incidence of oral cancer in Libya is similar to those of other North Africa countries and China, but is relatively low compared to the United Kingdom, the United States, and India. The recently established Libyan National Cancer Registry Program, initiated in 2007, while envisioning five cooperating regional cancer registries, continues to operate at a relatively suboptimal level. Lack of adequate levels of national funding continue to plague its development…and the accompanying quality of service that could be provided to the Libyan people.

Escalating burden of breast cancer in southern Thailand: analysis of 1990-2010 incidence and prediction of future trends.

PubMed

Virani, Shama; Sriplung, Hutcha; Rozek, Laura S; Meza, Rafael

2014-06-01

Thailand is undergoing an epidemiologic transition, with decreasing incidence of infectious diseases and increasing rates of chronic conditions, including cancer. Breast cancer has the highest incidence rates among females both in the southern region Thailand and throughout Thailand. However, there is a lack of research on the epidemiology of this and other cancers. Here we use cancer incidence data from the Songkhla Cancer Registry to characterize and analyze the incidence of breast cancer in Southern Thailand. We use joinpoint analysis, age-period-cohort models and nordpred analysis to investigate the incidence of breast cancer in Southern Thailand from 1990 to 2010 and project future trends from 2010 to 2029. We found that age-adjusted breast cancer incidence rates in Southern Thailand increased by almost 300% from 1990 to 2010 going from 10.0 to 27.8 cases per 100,000 person-years. Both period and cohort effects played a role in shaping the increase in incidence. Three distinct incidence projection methods consistently suggested that incidence rates will continue to increase in the future with incidence for women age 50 and above increasing at a higher rate than for women below 50. To date, this is the first study to examine Thai breast cancer incidence from a regional registry. This study provides a basis for future planning strategies in breast cancer prevention and to guide hypotheses for population-based epidemiologic research in Thailand. Copyright © 2014 Elsevier Ltd. All rights reserved.

Triggering of acute myocardial infarction by different means of transportation.

PubMed

Peters, Annette; von Klot, Stephanie; Mittleman, Murray A; Meisinger, Christine; Hörmann, Allmut; Kuch, Bernhard; Wichmann, H Erich

2013-10-01

Prior studies have reported an association between traffic-related air pollution in urban areas and exacerbation of cardiovascular disease. We assess here whether time spent in different modes of transportation can trigger the onset of acute myocardial infarction (AMI). We performed a case-crossover study. We interviewed consecutive cases of AMI in the KORA Myocardial Infarction Registry in Augsburg, Southern Germany between February 1999 and December 2003 eliciting data on potential triggers in the four days preceding myocardial infarction onset. A total of 1459 cases with known date and time of AMI symptom onset, who had survived 24 hours after the onset, completed the registry's standard interview on potential triggers of AMI. An association between exposure to traffic and AMI onset 1 hour later was observed (odds ratio: 3.2; 95% confidence interval [CI]: 2.7-3.9, p < 0.001). Using a car was the most common source of traffic exposure; nevertheless, times spent in public transport or on a bicycle were similarly associated with AMI onset 1 hour later. While the highest risk for AMI onset was within 1 hour of exposure to traffic, the elevated risk persisted for up to 6 hours. Women, patients aged 65 years or older, patients not part of the workforce, and those with a history of angina or diabetes exhibited the largest associations between times spent in traffic and AMI onset 1 hour later. The data suggest that transient exposure to traffic regardless of the means of transportation may increase the risk of AMI transiently.

Derivation of novel genetically diverse human embryonic stem cell lines.

PubMed

Stefanova, Valentina T; Grifo, James A; Hansis, Christoph

2012-06-10

Human embryonic stem cells (hESCs) have the potential to revolutionize many biomedical fields ranging from basic research to disease modeling, regenerative medicine, drug discovery, and toxicity testing. A multitude of hESC lines have been derived worldwide since the first 5 lines by Thomson et al. 13 years ago, but many of these are poorly characterized, unavailable, or do not represent desired traits, thus making them unsuitable for application purposes. In order to provide the scientific community with better options, we have derived 12 new hESC lines at New York University from discarded genetically normal and abnormal embryos using the latest techniques. We examined the genetic status of the NYUES lines in detail as well as their molecular and cellular features and DNA fingerprinting profile. Furthermore, we differentiated our hESCs into the tissues most affected by a specific condition or into clinically desired cell types. To our knowledge, a number of characteristics of our hESCs have not been previously reported, for example, mutation for alpha thalassemia X-linked mental retardation syndrome, linkage to conditions with a genetic component such as asthma or poor sperm morphology, and novel combinations of ethnic backgrounds. Importantly, all of our undifferentiated euploid female lines tested to date did not show X chromosome inactivation, believed to result in superior potency. We continue to derive new hESC lines and add them to the NIH registry and other registries. This should facilitate the use of our hESCs and lead to advancements for patient-benefitting applications.

Visual impairment in children and adolescents in Norway.

PubMed

Haugen, Olav H; Bredrup, Cecilie; Rødahl, Eyvind

2016-06-01

BACKGROUND Due to failures in reporting and poor data security, the Norwegian Registry of Blindness was closed down in 1995. Since that time, no registration of visual impairment has taken place in Norway. All the other Nordic countries have registries for children and adolescents with visual impairment. The purpose of this study was to survey visual impairments and their causes in children and adolescents, and to assess the need for an ophthalmic registry.MATERIAL AND METHOD Data were collected via the county teaching centres for the visually impaired in the period from 2005 - 2010 on children and adolescents aged less than 20 years with impaired vision (n = 628). This was conducted as a point prevalence study as of 1 January 2004. Visual function, ophthalmological diagnosis, systemic diagnosis and additional functional impairments were recorded.RESULTS Approximately two-thirds of children and adolescents with visual impairment had reduced vision, while one-third were blind. The three largest diagnostic groups were neuro-ophthalmic diseases (37 %), retinal diseases (19 %) and conditions affecting the eyeball in general (14 %). The prevalence of additional functional impairments was high, at 53 %, most often in the form of motor problems or cognitive impairments.INTERPRETATION The results of the study correspond well with similar investigations in the other Nordic countries. Our study shows that the registries associated with teaching for the visually impaired are inadequate in terms of medical data, and this underlines the need for an ophthalmic registry of children and adolescents with visual impairment.

The German Aortic Valve Registry (GARY): in-hospital outcome

PubMed Central

Hamm, Christian W.; Möllmann, Helge; Holzhey, David; Beckmann, Andreas; Veit, Christof; Figulla, Hans-Reiner; Cremer, J.; Kuck, Karl-Heinz; Lange, Rüdiger; Zahn, Ralf; Sack, Stefan; Schuler, Gerhard; Walther, Thomas; Beyersdorf, Friedhelm; Böhm, Michael; Heusch, Gerd; Funkat, Anne-Kathrin; Meinertz, Thomas; Neumann, Till; Papoutsis, Konstantinos; Schneider, Steffen; Welz, Armin; Mohr, Friedrich W.

2014-01-01

Background Aortic stenosis is a frequent valvular disease especially in elderly patients. Catheter-based valve implantation has emerged as a valuable treatment approach for these patients being either at very high risk for conventional surgery or even deemed inoperable. The German Aortic Valve Registry (GARY) provides data on conventional and catheter-based aortic procedures on an all-comers basis. Methods and results A total of 13 860 consecutive patients undergoing repair for aortic valve disease [conventional surgery and transvascular (TV) or transapical (TA) catheter-based techniques] have been enrolled in this registry during 2011 and baseline, procedural, and outcome data have been acquired. The registry summarizes the results of 6523 conventional aortic valve replacements without (AVR) and 3464 with concomitant coronary bypass surgery (AVR + CABG) as well as 2695 TV AVI and 1181 TA interventions (TA AVI). Patients undergoing catheter-based techniques were significantly older and had higher risk profiles. The stroke rate was low in all groups with 1.3% (AVR), 1.9% (AVR + CABG), 1.7% (TV AVI), and 2.3% (TA AVI). The in-hospital mortality was 2.1% (AVR) and 4.5% (AVR + CABG) for patients undergoing conventional surgery, and 5.1% (TV AVI) and AVI 7.7% (TA AVI). Conclusion The in-hospital outcome results of this registry show that conventional surgery yields excellent results in all risk groups and that catheter-based aortic valve replacements is an alternative to conventional surgery in high risk and elderly patients. PMID:24022003

Cohort profile: the TrueNTH Global Registry - an international registry to monitor and improve localised prostate cancer health outcomes.

PubMed

Evans, Sue M; Millar, Jeremy L; Moore, Caroline M; Lewis, John D; Huland, Hartwig; Sampurno, Fanny; Connor, Sarah E; Villanti, Paul; Litwin, Mark S

2017-11-28

Globally, prostate cancer treatment and outcomes for men vary according to where they live, their race and the care they receive. The TrueNTH Global Registry project was established as an international registry monitoring care provided to men with localised prostate cancer (CaP). Sites with existing CaP databases in Movember fundraising countries were invited to participate in the international registry. In total, 25 Local Data Centres (LDCs) representing 113 participating sites across 13 countries have nominated to contribute to the project. It will collect a dataset based on the International Consortium for Health Outcome Measures (ICHOM) standardised dataset for localised CaP. A governance strategy has been developed to oversee registry operation, including transmission of reversibly anonymised data. LDCs are represented on the Project Steering Committee, reporting to an Executive Committee. A Project Coordination Centre and Data Coordination Centre (DCC) have been established. A project was undertaken to compare existing datasets, understand capacity at project commencement (baseline) to collect the ICHOM dataset and assist in determining the final data dictionary. 21/25 LDCs provided data dictionaries for review. Some ICHOM data fields were well collected (diagnosis, treatment start dates) and others poorly collected (complications, comorbidities). 17/94 (18%) ICHOM data fields were relegated to non-mandatory fields due to poor capture by most existing registries. Participating sites will transmit data through a web interface biannually to the DCC. Recruitment to the TrueNTH Global Registry-PCOR project will commence in late 2017 with sites progressively contributing reversibly anonymised data following ethical review in local regions. Researchers will have capacity to source deidentified data after the establishment phase. Quality indicators are to be established through a modified Delphi approach in later 2017, and it is anticipated that reports on performance against quality indicators will be provided to LDCs. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Promising practices in the prevention of intimate partner violence among adolescents.

PubMed

De Grace, Alyssa; Clarke, Angela

2012-01-01

To inform practitioners and researchers interested in the prevention of intimate partner violence (IPV) among adolescents, 9 principles of effective prevention programs (Nation et al., 2003) were described and examples of how these principles have been incorporated into existing teen dating violence prevention programs were provided. An investigation of current prevention practices for adolescent IPV resulted in one noteworthy program that has successfully incorporated all 9 principles of effective prevention programming-Safe Dates (Substance Abuse and Mental Health Services Administration, National Registry of Evidence-based Programs and Practices [SAMHSA-NREPP], 2006). Although Safe Dates serves as a model teen dating violence prevention program, it may not be equally effective across contexts and diverse groups. Therefore, as researchers and practitioners continue to develop and refine programs to reduce adolescent IPV, the principles of effective prevention programs should serve as a guiding framework.

Worldwide comparison of survival from childhood leukaemia for 1995-2009, by subtype, age, and sex (CONCORD-2): a population-based study of individual data for 89 828 children from 198 registries in 53 countries.

PubMed

Bonaventure, Audrey; Harewood, Rhea; Stiller, Charles A; Gatta, Gemma; Clavel, Jacqueline; Stefan, Daniela C; Carreira, Helena; Spika, Devon; Marcos-Gragera, Rafael; Peris-Bonet, Rafael; Piñeros, Marion; Sant, Milena; Kuehni, Claudia E; Murphy, Michael F G; Coleman, Michel P; Allemani, Claudia

2017-05-01

Global inequalities in access to health care are reflected in differences in cancer survival. The CONCORD programme was designed to assess worldwide differences and trends in population-based cancer survival. In this population-based study, we aimed to estimate survival inequalities globally for several subtypes of childhood leukaemia. Cancer registries participating in CONCORD were asked to submit tumour registrations for all children aged 0-14 years who were diagnosed with leukaemia between Jan 1, 1995, and Dec 31, 2009, and followed up until Dec 31, 2009. Haematological malignancies were defined by morphology codes in the International Classification of Diseases for Oncology, third revision. We excluded data from registries from which the data were judged to be less reliable, or included only lymphomas, and data from countries in which data for fewer than ten children were available for analysis. We also excluded records because of a missing date of birth, diagnosis, or last known vital status. We estimated 5-year net survival (ie, the probability of surviving at least 5 years after diagnosis, after controlling for deaths from other causes [background mortality]) for children by calendar period of diagnosis (1995-99, 2000-04, and 2005-09), sex, and age at diagnosis (<1, 1-4, 5-9, and 10-14 years, inclusive) using appropriate life tables. We estimated age-standardised net survival for international comparison of survival trends for precursor-cell acute lymphoblastic leukaemia (ALL) and acute myeloid leukaemia (AML). We analysed data from 89 828 children from 198 registries in 53 countries. During 1995-99, 5-year age-standardised net survival for all lymphoid leukaemias combined ranged from 10·6% (95% CI 3·1-18·2) in the Chinese registries to 86·8% (81·6-92·0) in Austria. International differences in 5-year survival for childhood leukaemia were still large as recently as 2005-09, when age-standardised survival for lymphoid leukaemias ranged from 52·4% (95% CI 42·8-61·9) in Cali, Colombia, to 91·6% (89·5-93·6) in the German registries, and for AML ranged from 33·3% (18·9-47·7) in Bulgaria to 78·2% (72·0-84·3) in German registries. Survival from precursor-cell ALL was very close to that of all lymphoid leukaemias combined, with similar variation. In most countries, survival from AML improved more than survival from ALL between 2000-04 and 2005-09. Survival for each type of leukaemia varied markedly with age: survival was highest for children aged 1-4 and 5-9 years, and lowest for infants (younger than 1 year). There was no systematic difference in survival between boys and girls. Global inequalities in survival from childhood leukaemia have narrowed with time but remain very wide for both ALL and AML. These results provide useful information for health policy makers on the effectiveness of health-care systems and for cancer policy makers to reduce inequalities in childhood cancer survival. Canadian Partnership Against Cancer, Cancer Focus Northern Ireland, Cancer Institute New South Wales, Cancer Research UK, US Centers for Disease Control and Prevention, Swiss Re, Swiss Cancer Research foundation, Swiss Cancer League, and the University of Kentucky. Copyright © 2017 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY license. Published by Elsevier Ltd.. All rights reserved.

Resource requirements for cancer registration in areas with limited resources: Analysis of cost data from four low- and middle-income countries☆

PubMed Central

Tangka, Florence K.L.; Subramanian, Sujha; Edwards, Patrick; Cole-Beebe, Maggie; Parkin, D. Maxwell; Bray, Freddie; Joseph, Rachael; Mery, Les; Saraiya, Mona

2018-01-01

Background The key aims of this study were to identify sources of support for cancer registry activities, to quantify resource use and estimate costs to operate registries in low- and middle-income countries (LMIC) at different stages of development across three continents. Methods Using the Centers for Disease Control and Prevention’s (CDC’s) International Registry Costing Tool (IntRegCosting Tool), cost and resource use data were collected from eight population-based cancer registries, including one in a low-income country (Uganda [Kampala)]), two in lower to middle-income countries (Kenya [Nairobi] and India [Mumbai]), and five in an upper to middle-income country (Colombia [Pasto, Barranquilla, Bucaramanga, Manizales and Cali cancer registries]). Results Host institution contributions accounted for 30%–70% of total investment in cancer registry activities. Cancer registration involves substantial fixed cost and labor. Labor accounts for more than 50% of all expenditures across all registries. The cost per cancer case registered in low-income and lower-middle-income countries ranged from US $3.77 to US $15.62 (United States dollars). In Colombia, an upper to middle-income country, the cost per case registered ranged from US $41.28 to US $113.39. Registries serving large populations (over 15 million inhabitants) had a lower cost per inhabitant (less than US $0.01 in Mumbai, India) than registries serving small populations (under 500,000 inhabitants) [US $0.22] in Pasto, Colombia. Conclusion This study estimates the total cost and resources used for cancer registration across several countries in the limited-resource setting, and provides cancer registration stakeholders and registries-with opportunities to identify cost savings and efficiency improvements. Our results suggest that cancer registration involve substantial fixed costs and labor, and that partnership with other institutions is critical for the operation and sustainability of cancer registries in limited resource settings. Although we included registries from a variety of limited-resource areas, information from eight registries in four countries may not be large enough to capture all the potential differences among the registries in limited-resource settings. PMID:27793574

Resource requirements for cancer registration in areas with limited resources: Analysis of cost data from four low- and middle-income countries.

PubMed

Tangka, Florence K L; Subramanian, Sujha; Edwards, Patrick; Cole-Beebe, Maggie; Parkin, D Maxwell; Bray, Freddie; Joseph, Rachael; Mery, Les; Saraiya, Mona

2016-12-01

The key aims of this study were to identify sources of support for cancer registry activities, to quantify resource use and estimate costs to operate registries in low- and middle-income countries (LMIC) at different stages of development across three continents. Using the Centers for Disease Control and Prevention's (CDC's) International Registry Costing Tool (IntRegCosting Tool), cost and resource use data were collected from eight population-based cancer registries, including one in a low-income country (Uganda [Kampala)]), two in lower to middle-income countries (Kenya [Nairobi] and India [Mumbai]), and five in an upper to middle-income country (Colombia [Pasto, Barranquilla, Bucaramanga, Manizales and Cali cancer registries]). Host institution contributions accounted for 30%-70% of total investment in cancer registry activities. Cancer registration involves substantial fixed cost and labor. Labor accounts for more than 50% of all expenditures across all registries. The cost per cancer case registered in low-income and lower-middle-income countries ranged from US $3.77 to US $15.62 (United States dollars). In Colombia, an upper to middle-income country, the cost per case registered ranged from US $41.28 to US $113.39. Registries serving large populations (over 15 million inhabitants) had a lower cost per inhabitant (less than US $0.01 in Mumbai, India) than registries serving small populations (under 500,000 inhabitants) [US $0.22] in Pasto, Colombia. This study estimates the total cost and resources used for cancer registration across several countries in the limited-resource setting, and provides cancer registration stakeholders and registries with opportunities to identify cost savings and efficiency improvements. Our results suggest that cancer registration involve substantial fixed costs and labor, and that partnership with other institutions is critical for the operation and sustainability of cancer registries in limited resource settings. Although we included registries from a variety of limited-resource areas, information from eight registries in four countries may not be large enough to capture all the potential differences among the registries in limited-resource settings. Copyright © 2016 Elsevier Ltd. All rights reserved.

Establishing of National Birth Defects Registry in Thailand.

PubMed

Pangkanon, Suthipong; Sawasdivorn, Siraporn; Kuptanon, Chulaluck; Chotigeat, Uraiwan; Vandepitte, Warunee

2014-06-01

Deaths attributed to birth defects are a major cause of infant and under-five mortality as well as lifetime disabilities among those who survive. In Thailand, birth defects contribute to 21% of neonatal deaths. There is currently no systematic registry for congenital anomalies in Thailand. Queen Sirikit National Institute of Child Health has initiated a Thailand Birth Defects Registry to capture birth defects among newborn infants. To establish the national birth defects registry in order to determine the burden of birth defects in Thailand. The birth defects data come from four main sources: National Birth Registry Database; National Health Security Office's reimbursement database; Online Birth Defect Registry Database designed to capture new cases that were detected later; and birth defects data from 20 participated hospitals. All data are linked by unique 13-digit national identification number and International Classification of Diseases (ICD)-10 codes. This registry includes 19 common structural birth defects conditions and pilots in 20 hospitals. The registry is hospital-based, hybrid reporting system, including only live births whose information was collected up to 1 year of age. 3,696 infants out of 67,813 live births (8.28% of total live births in Thailand) were diagnosed with congenital anomalies. The prevalence rate of major anomalies was 26.12 per 1,000 live births. The five most common birth defects were congenital heart defects, limb anomalies, cleft lip/cleft palate, Down syndrome, and congenital hydrocephalus respectively. The present study established the Birth Defects Registry by collecting data from four databases in Thailand. Information obtained from this registry and surveillance is essential in the planning for effective intervention programs for birth defects. The authors suggest that this program should be integrated in the existing public health system to ensure sustainability.

Infection rates in patients from five rheumatoid arthritis (RA) registries: contextualising an RA clinical trial programme

PubMed Central

Yamanaka, Hisashi; Askling, Johan; Berglind, Niklas; Franzen, Stefan; Frisell, Thomas; Garwood, Christopher; Greenberg, Jeffrey D; Ho, Meilien; Holmqvist, Marie; Novelli Horne, Laura; Inoue, Eisuke; Michaud, Kaleb; Pappas, Dimitrios A; Reed, George; Symmons, Deborah; Tanaka, Eiichi; Tran, Trung N; Verstappen, Suzanne M M; Wesby-van Swaay, Eveline; Nyberg, Fredrik

2017-01-01

Objective Patients with rheumatoid arthritis (RA) have an increased risk of serious infections. Comparing infection rates across RA populations is complicated by differences in background infection risk, population composition and study methodology. We measured infection rates from five RA registries globally, with the aim to contextualise infection rates from an RA clinical trials population. Methods We used data from Consortium of Rheumatology Research of North America (CORRONA) (USA), Swedish Rheumatology Quality of Care Register (Sweden), Norfolk Arthritis Register (UK), CORRONA International (multiple countries) and Institute of Rheumatology Rheumatoid Arthritis (Japan) and an RA clinical trial programme (fostamatinib). Within each registry, we analysed a main cohort of all patients with RA from January 2000 to last available data. Infection definitions were harmonised across registries. Sensitivity analyses to address potential confounding explored subcohorts defined by disease activity, treatment change and/or prior comorbidities and restriction by calendar time or follow-up. Rates of infections were estimated and standardised to the trial population for age/sex and, in one sensitivity analysis also, for Health Assessment Questionnaire (HAQ) score. Results Overall, age/sex-standardised rates of hospitalised infection were quite consistent across registries (range 1.14–1.62 per 100 patient-years). Higher and more consistent rates across registries and with the trial programme overall were seen when adding standardisation for HAQ score (registry range 1.86–2.18, trials rate 2.92) or restricting to a treatment initiation subcohort followed for 18 months (registry range 0.99–2.84, trials rate 2.74). Conclusion This prospective, coordinated analysis of RA registries provided incidence rate estimates for infection events to contextualise infection rates from an RA clinical trial programme and demonstrated relative comparability of hospitalised infection rates across registries. PMID:29081988

Risk of Serious Infection in Juvenile Idiopathic Arthritis Patients Associated With Tumor Necrosis Factor Inhibitors and Disease Activity in the German Biologics in Pediatric Rheumatology Registry.

PubMed

Becker, Ingrid; Horneff, Gerd

2017-04-01

To examine the effects of tumor necrosis factor inhibitors on the risk for serious infections and other influencing factors in a registry. Patients exposed for the first time to etanercept, adalimumab, or methotrexate and serious infections were identified in the German Biologic Registry for Pediatric Rheumatology (BIKER) registry. Serious infection rates per 1,000 observation-years and relative risks were calculated. Cox regression identified risk factors and provided hazard ratios (HRs) for occurrence of infections. A total of 3,350 patients with 5,919 observation-years fulfilled the inclusion criteria for the study. The first biologic agents were etanercept (1,720 cases) and adalimumab (177 cases). A total of 1,453 patients were treated with methotrexate and no biologic agent. In total, 28 serious infections were reported in 26 patients (4.7 per 1,000 patient-years), 5 with methotrexate (1.6 per 1,000 patient-years), 21 with etanercept (8.1 per 1,000 patient-years), and 2 with adalimumab (9.7 per 1,000 patient-years). Significant univariate risk factors for infection were therapy with biologic agents, disease duration before therapy start, corticosteroid medication, nonbiologic premedications, higher clinical Juvenile Arthritis Disease Activity Score including maximal 10 joints (cJADAS10) at therapy start, and higher mean cJADAS10 during therapy. In multivariate Cox regression, only biologic therapy and cJADAS10 at therapy start remained significant. Risk for infection was increased by etanercept (univariate HR 6.0 [95% confidence interval (95% CI) 2.0-17.5]) or adalimumab (HR 7.3 [95% CI 1.3-40.0]) compared to methotrexate as well as by an elevated cJADAS10 at therapy start (HR 1.1 [95% CI 1.0-1.2] per unit increase). The total rate of serious infections reported in the BIKER registry seems low. Treatment with etanercept or adalimumab increases the risk for serious infection slightly, compared to methotrexate. Disease activity expressed by cJADAS10 appears to be an independent risk factor. © 2016, American College of Rheumatology.

Self-reported quality of life in multiple sclerosis patients: preliminary results based on the Polish MS Registry.

PubMed

Brola, Waldemar; Sobolewski, Piotr; Fudala, Małgorzata; Flaga, Stanisław; Jantarski, Konrad; Ryglewicz, Danuta; Potemkowski, Andrzej

2016-01-01

The aim of the study was to analyze selected clinical and sociodemographic factors and their effects on the quality of life (QoL) of multiple sclerosis (MS) patients registered in the Polish MS Registry. This was a cross-sectional observational study performed in Poland. Data on personal and disease-specific factors were collected between January 1, 2011, and December 31, 2015, via the web portal of the Polish MS Registry. All patients were assessed by a physician and asked to complete the Polish language versions of the following self-evaluation questionnaires: EuroQol 5-Dimensions, EuroQoL Visual Analog Scale, and Multiple Sclerosis Impact Scale. Univariate analysis and logistic regression were performed to determine the factors associated with QoL. The study included 2,385 patients (female/male ratio 2.3:1) with clinically confirmed MS (mean age 37.8±9.2 years). Average EuroQol 5-Dimensions index was 0.72±0.24, and the mean EuroQoL Visual Analog Scale score was 64.2±22.8. The average Multiple Sclerosis Impact Scale score was 84.6±11.2 (62.2±18.4 for physical condition and 23.8±7.2 for mental condition). Lower QoL scores were significantly associated with higher level of disability (odds ratio [OR], 0.932; 95% confidence interval [CI], 0.876-0.984; P=0.001), age >40 years (OR, 1.042; 95% CI, 0.924-1.158; P=0.012), longer disease duration (OR, 0.482; 95% CI, 0.224-0.998; P=0.042), and lack of disease modifying therapies (OR, 0.024; 95% CI, 0.160-0.835; P=0.024). No significant associations were found between QoL, sex, type of MS course, patient's education, and marital status. The Polish MS Registry is the first national registry for long-term observation that allows for self-evaluation of the QoL. QoL of Polish patients with MS is significantly lower compared with the rest of the population. The parameter is mainly affected by the level of disability, duration of the disease, and limited access to immunomodulatory therapy.

Improving quality of care using a diabetes registry and disease management services in an integrated delivery network.

PubMed

Campion, Francis X; Tully, George L; Barrett, Jo-Ann; Andre, Paulo; Sweeney, Ann

2005-08-01

Disease management for chronic conditions is a call for collaboration among all parties of the health care system. The Caritas Christi Health Care System established a unified American Diabetes Association (ADA) recognized outpatient diabetes self-management education program (DSME) in each of its six hospital communities and has established an Internet data portal with managed care organizations to improve preventive care for thousands of patients with diabetes. This article describes the stepwise process of building the successful Caritas Diabetes Care Program and the central role of the Caritas Diabetes Registry over a 5-year period.

Cancer registries in four provinces in Turkey: a case study

PubMed Central

2012-01-01

Background The burden of cancer affects all countries; while high-income countries have the capacity and resources to establish comprehensive cancer control programs, low and middle-income countries have limited resources to develop such programs. This paper examines factors associated with the development of cancer registries in four provinces in Turkey. It looks at the progress made by these registries, the challenges they faced, and the lessons learned. Other countries with similar resources can benefit from the lessons identified in this case study. Methods A mix of qualitative case study methods including key informant interviews, document review and questionnaires was used. Results This case study showed that surveillance systems that accurately report current cancer-related data are essential components of a country’s comprehensive cancer control program. At the initial stages, Turkey established one cancer registry with international support, which was used as a model for other registries. The Ministry of Health recognized the value of the registry data and its contribution to the country’s cancer control program and is supporting sustainability of these registries as a result. Conclusions This study demonstrates how Turkey was able to use resources from multiple sources to enhance its population based cancer registry system in four provinces. With renewed international interest in non-communicable diseases and cancer following the 2011 UN high-level meeting on NCDs, low- and middle- income countries can benefit from Turkey’s experience. Other countries can utilize lessons learned from Turkey as they address cancer burden and establish their own registries. PMID:23110989

Childhood vesicoureteral reflux studies: registries and repositories sources and nosology.

PubMed

Chesney, Russell W; Patters, Andrea B

2013-12-01

Despite several recent studies, the advisability of antimicrobial prophylaxis and certain imaging studies for urinary tract infections (UTIs) remains controversial. The role of vesicoureteral reflux (VUR) on the severity and re-infection rates for UTIs is also difficult to assess. Registries and repositories of data and biomaterials from clinical studies in children with VUR are valuable. Disease registries are collections of secondary data related to patients with a specific diagnosis, condition or procedure. Registries differ from indices in that they contain more extensive data. A research repository is an entity that receives, stores, processes and/or disseminates specimens (or other materials) as needed. It encompasses the physical location as well as the full range of activities associated with its operation. It may also be referred to as a biorepository. This report provides information about some current registries and repositories that include data and samples from children with VUR. It also describes the heterogeneous nature of the subjects, as some registries and repositories include only data or samples from patients with primary reflux while others also include those from patients with syndromic or secondary reflux. Copyright © 2012 Journal of Pediatric Urology Company. All rights reserved.

A protocol for bladder cancer screening and medical surveillance among high-risk groups: The Drake Health Registry experience

DOE Office of Scientific and Technical Information (OSTI.GOV)

Marsh, G.M.; Callahan, C.; Pavlock, D.

In 1986, the Drake Health Registry Study initiated bladder cancer screening for 366 persons at high risk because of occupational exposure to beta-naphthylamine. The Drake Health Registry Study screening protocol consists of urinalysis, Papanicolaou cytology, and quantitative fluorescence image analysis. A positive screening test qualifies participants for a full diagnostic evaluation. The screening protocol has been modified during the first 3 years of the program's existence to address unexpected patterns of test results and to incorporate advances in screening technology. The current protocol, which has a two-tiered screening schedule, has been utilized successfully for 15 months. Of the 26 positivemore » results to date most have been based on abnormal Papanicolaou cytology and/or quantitative fluorescence image analysis. Bladder abnormalities were cited among most of the 18 study members who underwent diagnostic evaluation, including chronic cystitis, inflammation, hyperplasia, and dysplasia. We conclude that the screening program is detecting very early changes in a relatively young cohort and that these persons must be monitored over a number of years to ensure adequate medical surveillance.« less

Mutational analysis and genotype-phenotype relation in familial hypercholesterolemia: The SAFEHEART registry.

PubMed

Bourbon, Mafalda; Alves, Ana Catarina; Alonso, Rodrigo; Mata, Nelva; Aguiar, Pedro; Padró, Teresa; Mata, Pedro

2017-07-01

Familial hypercholesterolemia (FH) is an autosomal dominant disease of cholesterol metabolism that confers an increased risk of premature atherosclerotic cardiovascular disease (ASCVD). Therefore, early identification and treatment of these patients can improve prognosis and reduce the burden of cardiovascular mortality. The aim of this work was to perform the mutational analysis of the SAFEHEART (Spanish Familial Hypercholesterolaemia Cohort Study) registry. The study recruited 2938 individuals with genetic diagnosis of FH belonging to 775 families. Statistical analysis was performed using SPSS v23. A total of 194 variants have been detected in this study, 24 of them were never described before. About 88% of the patients have a pathogenic or likely pathogenic variant. Patients with null variants have a more severe phenotype than patients with defective variants, presenting with significantly higher levels of atherogenic particles (total cholesterol, LDL-cholesterol and apolipoprotein B). This study shows the molecular characteristics of the FH patients included in the SAFEHEART registry and the relationship with the phenotypic expression. The majority of the genetic variants are considered to be pathogenic or likely pathogenic, which confers a high level of confidence to the entry and follow-up data analysis performed with this registry concerning FH patients' prognosis, treatment and survival. Copyright © 2017 Elsevier B.V. All rights reserved.

The EMBARC European Bronchiectasis Registry: protocol for an international observational study

PubMed Central

Aliberti, Stefano; Polverino, Eva; Vendrell, Montserrat; Crichton, Megan; Loebinger, Michael; Dimakou, Katerina; Clifton, Ian; van der Eerden, Menno; Rohde, Gernot; Murris-Espin, Marlene; Masefield, Sarah; Gerada, Eleanor; Shteinberg, Michal; Ringshausen, Felix; Haworth, Charles; Boersma, Wim; Rademacher, Jessica; Hill, Adam T.; Aksamit, Timothy; O'Donnell, Anne; Morgan, Lucy; Milenkovic, Branislava; Tramma, Leandro; Neves, Joao; Menendez, Rosario; Paggiaro, Perluigi; Botnaru, Victor; Skrgat, Sabina; Wilson, Robert; Goeminne, Pieter; De Soyza, Anthony; Welte, Tobias; Torres, Antoni; Elborn, J. Stuart; Blasi, Francesco

2016-01-01

Bronchiectasis is one of the most neglected diseases in respiratory medicine. There are no approved therapies and few large-scale, representative epidemiological studies. The EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) registry is a prospective, pan-European observational study of patients with bronchiectasis. The inclusion criterion is a primary clinical diagnosis of bronchiectasis consisting of: 1) a clinical history consistent with bronchiectasis; and 2) computed tomography demonstrating bronchiectasis. Core exclusion criteria are: 1) bronchiectasis due to known cystic fibrosis; 2) age <18 years; and 3) patients who are unable or unwilling to provide informed consent. The study aims to enrol 1000 patients by April 2016 across at least 20 European countries, and 10 000 patients by March 2020. Patients will undergo a comprehensive baseline assessment and will be followed up annually for up to 5 years with the goal of providing high-quality longitudinal data on outcomes, treatment patterns and quality of life. Data from the registry will be available in the form of annual reports. and will be disseminated in conference presentations and peer-reviewed publications. The European Bronchiectasis Registry aims to make a major contribution to understanding the natural history of the disease, as well as guiding evidence-based decision making and facilitating large randomised controlled trials. PMID:27730179

The Rare Diseases Clinical Research Network's organization and approach to observational research and health outcomes research.

PubMed

Krischer, Jeffrey P; Gopal-Srivastava, Rashmi; Groft, Stephen C; Eckstein, David J

2014-08-01

Established in 2003 by the Office of Rare Diseases Research (ORDR), in collaboration with several National Institutes of Health (NIH) Institutes/Centers, the Rare Diseases Clinical Research Network (RDCRN) consists of multiple clinical consortia conducting research in more than 200 rare diseases. The RDCRN supports longitudinal or natural history, pilot, Phase I, II, and III, case-control, cross-sectional, chart review, physician survey, bio-repository, and RDCRN Contact Registry (CR) studies. To date, there have been 24,684 participants enrolled on 120 studies from 446 sites worldwide. An additional 11,533 individuals participate in the CR. Through a central data management and coordinating center (DMCC), the RDCRN's platform for the conduct of observational research encompasses electronic case report forms, federated databases, and an online CR for epidemiological and survey research. An ORDR-governed data repository (through dbGaP, a database for genotype and phenotype information from the National Library of Medicine) has been created. DMCC coordinates with ORDR to register and upload study data to dbGaP for data sharing with the scientific community. The platform provided by the RDCRN DMCC has supported 128 studies, six of which were successfully conducted through the online CR, with 2,352 individuals accrued and a median enrollment time of just 2 months. The RDCRN has built a powerful suite of web-based tools that provide for integration of federated and online database support that can accommodate a large number of rare diseases on a global scale. RDCRN studies have made important advances in the diagnosis and treatment of rare diseases.

Clinical presentation and management of stable coronary artery disease in Austria.

PubMed

Pichlhöfer, Otto; Maier, Manfred; Badr-Eslam, Roza; Ristl, Robin; Zebrowska, Magdalena; Lang, Irene M

2017-01-01

Cardiovascular disease is the main cause of death in Austria. However, no systematic information exists regarding characteristics and treatments of contemporary patients with stable coronary artery disease (CAD) in Austria. We assembled two retrospective physicians' databases to describe demographics, clinical profiles, and therapeutic strategies in patients with stable CAD. In addition, we compared patient profiles of secondary care internists and hospital-based cardiologists with those of general practitioners in a primary care setting outside of hospital. The study population was identified from retrospective chart review of 1020 patients from 106 primary care physicians in Austria (ProCor II registry), and was merged with a previous similar database of 1280 patients under secondary care (ProCor I registry) to yield a total patient number of 2300. Female patients with stable CAD were older, had more angina and/or heart failure symptoms, and more depression than males. Female gender, type 2 diabetes mellitus, higher CCS class and asthma/COPD were predictors of elevated heart rate, while previous coronary events/revascularization predicted a lower heart rate in multivariate analysis. There were no significant differences with regard to characteristics and management of patients of general practitioners in the primary care setting versus internists in secondary care. Characteristics and treatments of unselected patients with stable ischemic heart disease in Austria resemble the pattern of large international registries of stable ischemic heart disease, with the exception that diabetes and systemic hypertension were more prevalent.

76 FR 39879 - Request for Nominations of Candidates To Serve on the Board of Scientific Counselors, National...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-07-07

... Environmental Health/Agency for Toxic Substances and Disease Registry (BSC, NCEH/ATSDR), Centers for Disease... experience in preventing human diseases and disabilities caused by environmental conditions. Experts in the disciplines of toxicology, epidemiology, environmental or occupational medicine, behavioral science, risk...

Clinical characteristics during diagnosis of a prospective cohort of patients with systemic lupus erythematosus treated in Spanish Departments of Internal Medicine: The RELES study.

PubMed

Canora, J; García, M; Mitjavila, F; Espinosa, G; Suárez, S; González-León, R; Sopeña, B; Boldova, R; Castro, A; Ruiz-Irastorza, G

Patient registries are useful tools for assessing rare diseases. Our objective is to present the Spanish registry of patients with systemic lupus erythematosus (Registro español de pacientes con lupus eritematoso sistémico, RELES). RELES was started in 2008 as an observational, prospective, multicentre cohort registry that included patients from the time they were diagnosed. The registry's objective is to analyse the incidence and noninflammatory complications of systemic lupus erythematosus (SLE). The departments of internal medicine of 38 Spanish hospitals participate in this registry. A total of 298 patients with a mean age of 40.8±15.7 years were included, 88.9% of whom were women and 85.6% of whom were white. In the first visit, there was a predominance of joint manifestations (74.5%). One hundred and seventy-seven patients (59.4%) were positive for anti-native DNA. In these patients, there was a higher rate of lupus nephritis (26.7% vs. 14%, p=.009; relative risk [RR], 1.33), haemolytic anaemia (13.6% vs. 4.1%, p=.07; RR, 1.46) and lymphopenia (55.4% vs. 43.8%, p=.05; RR, 1.21). The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K) score was 9.64 points (interquartile range, 4-13). The patients treated with antimalarial drugs before the diagnosis of SLE had a median SLEDAI score in the first visit of 5, compared with 8 for those who were not treated with these drugs (p=.02). RELES constitutes the first Spanish patient cohort with SLE recorded from the time of the diagnosis. The presence of anti-DNA has been related to severe manifestations such as nephritis and haemolytic anaemia. Treatment with antimalarial drugs before the diagnosis was associated with less active disease at the initial presentation. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

Inherited Retinal Degenerative Disease Registry

ClinicalTrials.gov

2017-09-13

Eye Diseases Hereditary; Retinal Disease; Achromatopsia; Bardet-Biedl Syndrome; Bassen-Kornzweig Syndrome; Batten Disease; Best Disease; Choroidal Dystrophy; Choroideremia; Cone Dystrophy; Cone-Rod Dystrophy; Congenital Stationary Night Blindness; Enhanced S-Cone Syndrome; Fundus Albipunctatus; Goldmann-Favre Syndrome; Gyrate Atrophy; Juvenile Macular Degeneration; Kearns-Sayre Syndrome; Leber Congenital Amaurosis; Refsum Syndrome; Retinitis Pigmentosa; Retinitis Punctata Albescens; Retinoschisis; Rod-Cone Dystrophy; Rod Dystrophy; Rod Monochromacy; Stargardt Disease; Usher Syndrome

Feasibility of Linking Population-Based Cancer Registries and Cancer Center Biorepositories

PubMed Central

McCusker, Margaret E.; Allen, Mark; Fernandez-Ami, Allyn; Gandour-Edwards, Regina

2012-01-01

Purpose: Biospecimen-based research offers tremendous promise as a way to increase understanding of the molecular epidemiology of cancers. Population-based cancer registries can augment this research by providing more clinical detail and long-term follow-up information than is typically available from biospecimen annotations. In order to demonstrate the feasibility of this concept, we performed a pilot linkage between the California Cancer Registry (CCR) and the University of California, Davis Cancer Center Biorepository (UCD CCB) databases to determine if we could identify patients with records in both databases. Methods: We performed a probabilistic data linkage between 2180 UCD CCB biospecimen records collected during the years 2005–2009 and all CCR records for cancers diagnosed from 1988–2009 based on standard data linkage procedures. Results: The 1040 UCD records with a unique medical record number, tissue site, and pathology date were linked to 3.3 million CCR records. Of these, 844 (81.2%) were identified in both databases. Overall, record matches were highest (100%) for cancers of the cervix and testis/other male genital system organs. For the most common cancers, matches were highest for cancers of the lung and respiratory system (93%), breast (91.7%), and colon and rectum (89.5%), and lower for prostate (72.9%). Conclusions: This pilot linkage demonstrated that information on existing biospecimens from a cancer center biorepository can be linked successfully to cancer registry data. Linkages between existing biorepositories and cancer registries can foster productive collaborations and provide a foundation for virtual biorepository networks to support population-based biospecimen research. PMID:24845042

The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC): experiences from a successful ERS Clinical Research Collaboration.

PubMed

Chalmers, James D; Crichton, Megan; Goeminne, Pieter C; Loebinger, Michael R; Haworth, Charles; Almagro, Marta; Vendrell, Montse; De Soyza, Anthony; Dhar, Raja; Morgan, Lucy; Blasi, Francesco; Aliberti, Stefano; Boyd, Jeanette; Polverino, Eva

2017-09-01

In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare diseases such as cystic fibrosis, there has been little research and few clinical trials in bronchiectasis. Guidelines are primarily based on expert opinion and treatment is challenging because of the heterogeneous nature of the disease. In an effort to address decades of underinvestment in bronchiectasis research, education and clinical care, the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) was established in 2012 as a collaborative pan-European network to bring together bronchiectasis researchers. The European Respiratory Society officially funded EMBARC in 2013 as a Clinical Research Collaboration, providing support and infrastructure to allow the project to grow. EMBARC has now established an international bronchiectasis registry that is active in more than 30 countries both within and outside Europe. Beyond the registry, the network participates in designing and facilitating clinical trials, has set international research priorities, promotes education and has participated in producing the first international bronchiectasis guidelines. This manuscript article the development, structure and achievements of EMBARC from 2012 to 2017. To understand the role of Clinical Research Collaborations as the major way in which the European Respiratory Society can stimulate clinical research in different disease areasTo understand some of the key features of successful disease registriesTo review key epidemiological, clinical and translational studies of bronchiectasis contributed by the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) project in the past 5 yearsTo understand the key research priorities identified by EMBARC for the next 5 years.

The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC): experiences from a successful ERS Clinical Research Collaboration

PubMed Central

Crichton, Megan; Goeminne, Pieter C.; Loebinger, Michael R.; Haworth, Charles; Almagro, Marta; Vendrell, Montse; De Soyza, Anthony; Dhar, Raja ; Morgan, Lucy; Blasi, Francesco; Aliberti, Stefano; Boyd, Jeanette; Polverino, Eva

2017-01-01

In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare diseases such as cystic fibrosis, there has been little research and few clinical trials in bronchiectasis. Guidelines are primarily based on expert opinion and treatment is challenging because of the heterogeneous nature of the disease. In an effort to address decades of underinvestment in bronchiectasis research, education and clinical care, the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) was established in 2012 as a collaborative pan-European network to bring together bronchiectasis researchers. The European Respiratory Society officially funded EMBARC in 2013 as a Clinical Research Collaboration, providing support and infrastructure to allow the project to grow. EMBARC has now established an international bronchiectasis registry that is active in more than 30 countries both within and outside Europe. Beyond the registry, the network participates in designing and facilitating clinical trials, has set international research priorities, promotes education and has participated in producing the first international bronchiectasis guidelines. This manuscript article the development, structure and achievements of EMBARC from 2012 to 2017. Educational aims To understand the role of Clinical Research Collaborations as the major way in which the European Respiratory Society can stimulate clinical research in different disease areas To understand some of the key features of successful disease registries To review key epidemiological, clinical and translational studies of bronchiectasis contributed by the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) project in the past 5 years To understand the key research priorities identified by EMBARC for the next 5 years PMID:28894479

Patient reported outcomes in hip arthroplasty registries.

PubMed

Paulsen, Aksel

2014-05-01

PROs are used increasingly in orthopedics and in joint registries, but still many aspects of use in this area have not been examined in depth. To be able to introduce PROs in the DHR in a scientific fashion, my studies were warranted; the feasibility of four often used PROs (OHS, HOOS, EQ-5D and SF-12) was examined in a registry context. Having the PROs in the target language is an absolute necessity, so I translated, cross-culturally adapted and validated a Danish language version of an often used PRO (OHS), since this PRO had no properly developed Danish language version. To minimize data loss and to maximize the data quality I validated our data capture procedure, an up to date AFP system, by comparing scannable, paper-based PROs, with manual single-key- and double-key entered data. To help further registry-PRO studies, I calculated the number of patients needed to discriminate between subgroups of age, sex, diagnosis, and prosthesis type for each of four often used PROs (OHS, HOOS, EQ-5D and SF-12), and to simplify the clinical interpretation of PRO scores and PRO change scores in PRO studies, I estimated MCII and PASS for two often used PROs (EQ-5D and HOOS). The feasibility study included 5,747 THA patients registered in the DHR, and I found only minor differences between the disease-specific and the generic PROs regarding ceiling and floor effects as well as discarded items. The HOOS, the OHS, the SF-12, and the EQ-5D are all appropriate PROs for administration in a hip registry. I found that group sizes from 51 to 1,566 were needed for subgroup analysis, depending on descriptive factors and choice of PRO. The AFP study included 200 THA patients (398 PROs, 4,875 items and 21,887 data fields), and gave excellent results provided use of highly structured questionnaires. OMR performed equally as well as manual double-key entering, and better than single-key entering. The PRO translation and validation study included 2,278 patients (and 212 patients for the test-retest). I found that the translated PRO had good feasibility, an excellent response rate, no floor effect, but a high ceiling effect (as was expected with our postoperative patients) and few patients missed too many items to calculate a sum score. The translated PRO had high test-retest reliability and very high internal consistency, and appears to be a valid and reliable tool for outcome studies on THA patients in a hip registry setting. The MCII and PASS study included 1,335 patients, and I estimated that one year after THA, an improvement of 38-55% from mean baseline PRO score and absolute follow-up scores of 57-91% of the maximum score correspond to a minimal important improvement and acceptable symptom state, respectively.

Myotonic Dystrophy Family Registry

ClinicalTrials.gov

2016-03-28

Myotonic Dystrophy; Congenital Myotonic Dystrophy; Myotonic Dystrophy 1; Myotonic Dystrophy 2; Dystrophia Myotonica; Dystrophia Myotonica 1; Dystrophia Myotonica 2; Myotonia Dystrophica; Myotonic Dystrophy, Congenital; Myotonic Myopathy, Proximal; PROMM (Proximal Myotonic Myopathy); Proximal Myotonic Myopathy; Steinert Disease; Steinert Myotonic Dystrophy; Steinert's Disease; Myotonia Atrophica

Utility of an Australasian registry for children undergoing radiation treatment.

PubMed

Ahern, Verity

2014-12-01

The aim of this study was to evaluate the utility of an Australasian registry ('the Registry') for children undergoing radiation treatment (RT). Children under the age of 16 years who received a course of radiation between January 1997 and December 2010 and were enrolled on the Registry form the subjects of this study. A total of 2232 courses of RT were delivered, predominantly with radical intent (87%). Registrations fluctuated over time, but around one-half of children diagnosed with cancer undergo a course of RT. The most prevalent age range at time of RT was 10-15 years, and the most common diagnoses were central nervous system tumours (34%) and acute lymphoblastic leukaemia (20%). The Registry provides a reflection of the patterns of care of children undergoing RT in Australia and a mechanism for determining the resources necessary to manage children by RT (human, facilities and emerging technologies, such as proton therapy). It lacks the detail to provide information on radiotherapy quality and disease outcomes which should be the subject of separate audit studies. The utility of the Registry has been hampered by its voluntary nature and varying needs for consent. Completion of registry forms is a logical requirement for inclusion in the definition of a subspecialist in paediatric radiation oncology. © 2014 The Royal Australian and New Zealand College of Radiologists.

Electronic health record - public health (EHR-PH) system prototype for interoperability in 21st century healthcare systems.

PubMed

Orlova, Anna O; Dunnagan, Mark; Finitzo, Terese; Higgins, Michael; Watkins, Todd; Tien, Allen; Beales, Steven

2005-01-01

Information exchange, enabled by computable interoperability, is the key to many of the initiatives underway including the development of Regional Health Information Exchanges, Regional Health Information Organizations, and the National Health Information Network. These initiatives must include public health as a full partner in the emerging transformation of our nation's healthcare system through the adoption and use of information technology. An electronic health record - public health (EHR-PH)system prototype was developed to demonstrate the feasibility of electronic data transfer from a health care provider, i.e. hospital or ambulatory care settings, to multiple customized public health systems which include a Newborn Metabolic Screening Registry, a Newborn Hearing Screening Registry, an Immunization Registry and a Communicable Disease Registry, using HL7 messaging standards. Our EHR-PH system prototype can be considered a distributed EHR-based RHIE/RHIO model - a principal element for a potential technical architecture for a NHIN.

CREATION OF A MODEL TO PREDICT SURVIVAL IN PATIENTS WITH REFRACTORY COELIAC DISEASE USING A MULTINATIONAL REGISTRY

PubMed Central

Rubio-Tapia, Alberto; Malamut, Georgia; Verbeek, Wieke H.M.; van Wanrooij, Roy L.J.; Leffler, Daniel A.; Niveloni, Sonia I.; Arguelles-Grande, Carolina; Lahr, Brian D.; Zinsmeister, Alan R.; Murray, Joseph A.; Kelly, Ciaran P.; Bai, Julio C.; Green, Peter H.; Daum, Severin; Mulder, Chris J.J.; Cellier, Christophe

2016-01-01

Background Refractory coeliac disease is a severe complication of coeliac disease with heterogeneous outcome. Aim To create a prognostic model to estimate survival of patients with refractory coeliac disease. Methods We evaluated predictors of 5-year mortality using Cox proportional hazards regression on subjects from a multinational registry. Bootstrap re-sampling was used to internally validate the individual factors and overall model performance. The mean of the estimated regression coefficients from 400 bootstrap models was used to derive a risk score for 5-year mortality. Results The multinational cohort was composed of 232 patients diagnosed with refractory coeliac disease across 7 centers (range of 11–63 cases per center). The median age was 53 years and 150 (64%) were women. A total of 51 subjects died during 5-year follow-up (cumulative 5-year all-cause mortality = 30%). From a multiple variable Cox proportional hazards model, the following variables were significantly associated with 5-year mortality: age at refractory coeliac disease diagnosis (per 20 year increase, hazard ratio = 2.21; 95% confidence interval: 1.38, 3.55), abnormal intraepithelial lymphocytes (hazard ratio = 2.85; 95% confidence interval: 1.22, 6.62), and albumin (per 0.5 unit increase, hazard ratio = 0.72; 95% confidence interval: 0.61, 0.85). A simple weighted 3-factor risk score was created to estimate 5-year survival. Conclusions Using data from a multinational registry and previously-reported risk factors, we create a prognostic model to predict 5-year mortality among patients with refractory coeliac disease. This new model may help clinicians to guide treatment and follow-up. PMID:27485029

Angiographic Validation of the American College of Cardiology Foundation–The Society of Thoracic Surgeons Collaboration on the Comparative Effectiveness of Revascularization Strategies Study

PubMed Central

Chakrabarti, Anjan K.; Grau-Sepulveda, Maria V.; O’Brien, Sean; Abueg, Cassandra; Ponirakis, Angelo; Delong, Elizabeth; Peterson, Eric; Klein, Lloyd W.; Garratt, Kirk N.; Weintraub, William S.; Gibson, C. Michael

2017-01-01

Background The goal of this study was to compare angiographic interpretation of coronary arteriograms by sites in community practice versus those made by a centralized angiographic core laboratory. Methods and Results The study population consisted of 2013 American College of Cardiology–National Cardiovascular Data Registry (ACC–NCDR) records with 2- and 3- vessel coronary disease from 54 sites in 2004 to 2007. The primary analysis compared Registry (NCDR)-defined 2- and 3-vessel disease versus those from an angiographic core laboratory analysis. Vessel-level kappa coefficients suggested moderate agreement between NCDR and core laboratory analysis, ranging from kappa=0.39 (95% confidence intervals, 0.32–0.45) for the left anterior descending artery to kappa=0.59 (95% confidence intervals, 0.55–0.64) for the right coronary artery. Overall, 6.3% (n=127 out of 2013) of those patients identified with multivessel disease at NCDR sites had had 0- or 1-vessel disease by core laboratory reading. There was no directional bias with regard to overcall, that is, 12.3% of cases read as 3-vessel disease by the sites were read as <3-vessel disease by the core laboratory, and 13.9% of core laboratory 3-vessel cases were read as <3-vessel by the sites. For a subset of patients with left main coronary disease, registry overcall was not linked to increased rates of mortality or myocardial infarction. Conclusions There was only modest agreement between angiographic readings in clinical practice and those from an independent core laboratory. Further study will be needed because the implications for patient management are uncertain. PMID:24496239

Angiographic validation of the American College of Cardiology Foundation-the Society of Thoracic Surgeons Collaboration on the Comparative Effectiveness of Revascularization Strategies study.

PubMed

Chakrabarti, Anjan K; Grau-Sepulveda, Maria V; O'Brien, Sean; Abueg, Cassandra; Ponirakis, Angelo; Delong, Elizabeth; Peterson, Eric; Klein, Lloyd W; Garratt, Kirk N; Weintraub, William S; Gibson, C Michael

2014-02-01

The goal of this study was to compare angiographic interpretation of coronary arteriograms by sites in community practice versus those made by a centralized angiographic core laboratory. The study population consisted of 2013 American College of Cardiology-National Cardiovascular Data Registry (ACC-NCDR) records with 2- and 3- vessel coronary disease from 54 sites in 2004 to 2007. The primary analysis compared Registry (NCDR)-defined 2- and 3-vessel disease versus those from an angiographic core laboratory analysis. Vessel-level kappa coefficients suggested moderate agreement between NCDR and core laboratory analysis, ranging from kappa=0.39 (95% confidence intervals, 0.32-0.45) for the left anterior descending artery to kappa=0.59 (95% confidence intervals, 0.55-0.64) for the right coronary artery. Overall, 6.3% (n=127 out of 2013) of those patients identified with multivessel disease at NCDR sites had had 0- or 1-vessel disease by core laboratory reading. There was no directional bias with regard to overcall, that is, 12.3% of cases read as 3-vessel disease by the sites were read as <3-vessel disease by the core laboratory, and 13.9% of core laboratory 3-vessel cases were read as <3-vessel by the sites. For a subset of patients with left main coronary disease, registry overcall was not linked to increased rates of mortality or myocardial infarction. There was only modest agreement between angiographic readings in clinical practice and those from an independent core laboratory. Further study will be needed because the implications for patient management are uncertain.

Creation of a model to predict survival in patients with refractory coeliac disease using a multinational registry.

PubMed

Rubio-Tapia, A; Malamut, G; Verbeek, W H M; van Wanrooij, R L J; Leffler, D A; Niveloni, S I; Arguelles-Grande, C; Lahr, B D; Zinsmeister, A R; Murray, J A; Kelly, C P; Bai, J C; Green, P H; Daum, S; Mulder, C J J; Cellier, C

2016-10-01

Refractory coeliac disease is a severe complication of coeliac disease with heterogeneous outcome. To create a prognostic model to estimate survival of patients with refractory coeliac disease. We evaluated predictors of 5-year mortality using Cox proportional hazards regression on subjects from a multinational registry. Bootstrap resampling was used to internally validate the individual factors and overall model performance. The mean of the estimated regression coefficients from 400 bootstrap models was used to derive a risk score for 5-year mortality. The multinational cohort was composed of 232 patients diagnosed with refractory coeliac disease across seven centres (range of 11-63 cases per centre). The median age was 53 years and 150 (64%) were women. A total of 51 subjects died during a 5-year follow-up (cumulative 5-year all-cause mortality = 30%). From a multiple variable Cox proportional hazards model, the following variables were significantly associated with 5-year mortality: age at refractory coeliac disease diagnosis (per 20 year increase, hazard ratio = 2.21; 95% confidence interval, CI: 1.38-3.55), abnormal intraepithelial lymphocytes (hazard ratio = 2.85; 95% CI: 1.22-6.62), and albumin (per 0.5 unit increase, hazard ratio = 0.72; 95% CI: 0.61-0.85). A simple weighted three-factor risk score was created to estimate 5-year survival. Using data from a multinational registry and previously reported risk factors, we create a prognostic model to predict 5-year mortality among patients with refractory coeliac disease. This new model may help clinicians to guide treatment and follow-up. © 2016 John Wiley & Sons Ltd.

Reducing Racial Disparities in Breast Cancer Care: The Role of 'Big Data'.

PubMed

Reeder-Hayes, Katherine E; Troester, Melissa A; Meyer, Anne-Marie

2017-10-15

Advances in a wide array of scientific technologies have brought data of unprecedented volume and complexity into the oncology research space. These novel big data resources are applied across a variety of contexts-from health services research using data from insurance claims, cancer registries, and electronic health records, to deeper and broader genomic characterizations of disease. Several forms of big data show promise for improving our understanding of racial disparities in breast cancer, and for powering more intelligent and far-reaching interventions to close the racial gap in breast cancer survival. In this article we introduce several major types of big data used in breast cancer disparities research, highlight important findings to date, and discuss how big data may transform breast cancer disparities research in ways that lead to meaningful, lifesaving changes in breast cancer screening and treatment. We also discuss key challenges that may hinder progress in using big data for cancer disparities research and quality improvement.

A Pediatrician’s Practical Guide to Diagnosing and Treating Hereditary Spherocytosis in Neonates

PubMed Central

Yaish, Hassan M.; Gallagher, Patrick G.

2015-01-01

Newborn infants who have hereditary spherocytosis (HS) can develop anemia and hyperbilirubinemia. Bilirubin-induced neurologic dysfunction is less likely in these neonates if the diagnosis of HS is recognized and appropriate treatment provided. Among neonates listed in the USA Kernicterus Registry, HS was the third most common underlying hemolytic condition after glucose-6-phosphate dehydrogenase deficiency and ABO hemolytic disease. HS is the leading cause of direct antiglobulin test (direct Coombs) negative hemolytic anemia requiring erythrocyte transfusion in the first months of life. We anticipate that as physicians become more familiar with diagnosing HS in the newborn period, fewer neonates with HS will develop hazardous hyperbilirubinemia or present to emergency departments with unanticipated symptomatic anemia. We predict that early suspicion, prompt diagnosis and treatment, and anticipatory guidance will prevent adverse outcomes in neonates with HS. The purpose of this article was to review the neonatal presentation of HS and to provide practical and up-to-date means of diagnosing and treating HS in neonates. PMID:26009624

An analysis of the sequence of the BAD gene among patients with maturity-onset diabetes of the young (MODY).

PubMed

Antosik, Karolina; Gnyś, Piotr; Jarosz-Chobot, Przemysława; Myśliwiec, Małgorzata; Szadkowska, Agnieszka; Małecki, Maciej; Młynarski, Wojciech; Borowiec, Maciej

2017-01-01

Monogenic diabetes is a rare disease caused by single gene mutations. Maturity onset diabetes of the young (MODY) is one of the major forms of monogenic diabetes recognised in the paediatric population. To date, 13 genes have been related to MODY development. The aim of the study was to analyse the sequence of the BCL2-associated agonist of cell death (BAD) gene in patients with clinical suspicion of GCK-MODY, but who were negative for glucokinase (GCK) gene mutations. A group of 122 diabetic patients were recruited from the "Polish Registry for Paediatric and Adolescent Diabetes - nationwide genetic screening for monogenic diabetes" project. The molecular testing was performed by Sanger sequencing. A total of 10 sequence variants of the BAD gene were identified in 122 analysed diabetic patients. Among the analysed patients suspected of MODY, one possible pathogenic variant was identified in one patient; however, further confirmation is required for a certain identification.

Inflammatory bowel disease: A descriptive study of 716 local Chilean patients

PubMed Central

Simian, Daniela; Fluxá, Daniela; Flores, Lilian; Lubascher, Jaime; Ibáñez, Patricio; Figueroa, Carolina; Kronberg, Udo; Acuña, Raúl; Moreno, Mauricio; Quera, Rodrigo

2016-01-01

AIM: To demographically and clinically characterize inflammatory bowel disease (IBD) from the local registry and update data previously published by our group. METHODS: A descriptive study of a cohort based on a registry of patients aged 15 years or older who were diagnosed with IBD and attended the IBD program at Clínica Las Condes in Santiago, Chile. The registry was created in April 2012 and includes patients registered up to October 2015. The information was anonymously downloaded in a monthly report, and the information on patients with more than one visit was updated. The registry includes demographic, clinical and disease characteristics, including the Montreal Classification, medical treatment, surgeries and hospitalizations for crisis. Data regarding infection with Clostridium difficile (C. difficile) were incorporated in the registry in 2014. Data for patients who received consultations as second opinions and continued treatment at this institution were also analyzed. RESULTS: The study included 716 patients with IBD: 508 patients (71%) were diagnosed with ulcerative colitis (UC), 196 patients (27%) were diagnosed with Crohn’s disease (CD) and 12 patients (2%) were diagnosed with unclassifiable IBD. The UC/CD ratio was 2.6/1. The median age was 36 years (range 16-88), and 58% of the patients were female, with a median age at diagnosis of 29 years (range 5-76). In the past 15 years, a sustained increase in the number of patients diagnosed with IBD was observed, where 87% of the patients were diagnosed between the years 2001 and 2015. In the cohort examined in the present study, extensive colitis (50%) and colonic involvement (44%) predominated in the patients with UC and CD, respectively. In CD patients, non-stricturing/non-penetrating behavior was more frequent (80%), and perianal disease was observed in 28% of the patients. There were significant differences in treatment between UC and CD, with a higher use of corticosteroids, and immunosuppressive and biological therapies was observed in the patients with CD (P < 0.05 and P < 0.01). Significant surgical differences were also observed: 5% of the UC patients underwent surgery, whereas 38% of the CD patients required at least one surgery (P < 0.01). The patients with CD were hospitalized more often during their disease course than the patients with UC (55% and 35% of the patients, respectively; P < 0.01). C. difficile infection was acquired by 5% of the patients in each group at some point during the disease course. Nearly half of the patients consulted at the institution for a second opinion, and 32% of these individuals continued treatment at the institution. CONCLUSION: IBD has continued to increase in the study cohort, slowly approaching the level reported in developed countries. PMID:27298570

Long-term safety and efficacy of sapropterin: the PKUDOS registry experience.

PubMed

Longo, Nicola; Arnold, Georgianne L; Pridjian, Gabriella; Enns, Gregory M; Ficicioglu, Can; Parker, Susan; Cohen-Pfeffer, Jessica L

2015-04-01

The Phenylketonuria (PKU) Demographics, Outcomes and Safety (PKUDOS) registry is designed to provide longitudinal safety and efficacy data on subjects with PKU who are (or have been) treated with sapropterin dihydrochloride. The PKUDOS population consists of 1189 subjects with PKU: N = 504 who were continuously exposed to sapropterin from date of registry enrollment, N = 211 who had intermittent exposure to the drug, and N = 474 with some other duration of exposure. Subjects continuously exposed to sapropterin showed an average 34% decrease in blood phenylalanine (Phe)--from 591 ± 382 μmol/L at baseline to 392 ± 239 μmol/L (p = 0.0009) after 5 years. This drop in blood Phe was associated with an increase in dietary Phe tolerance [from 1000 ± 959 mg/day (pre-sapropterin baseline) to 1539 ± 840 mg/day after 6 years]. Drug-related adverse events (AEs) were reported in 6% of subjects, were mostly considered non-serious, and were identified in the gastrointestinal, respiratory, and nervous systems. Serious drug-related AEs were reported in ≤ 1% of subjects. Similar safety and efficacy data were observed for children<4 years. Long-term data from the PKUDOS registry suggest that sapropterin has a tolerable safety profile and that continuous use is associated with a significant and persistent decrease in blood Phe and improvements in dietary Phe tolerance. Copyright © 2015. Published by Elsevier Inc.

[Orange Platform].

PubMed

Toba, Kenji

2017-07-01

The Organized Registration for the Assessment of dementia on Nationwide General consortium toward Effective treatment in Japan (ORANGE platform) is a recently established nationwide clinical registry for dementia. This platform consists of multiple registries of patients with dementia stratified by the following clinical stages: preclinical, mild cognitive impairment, early-stage, and advanced-stage dementia. Patients will be examined in a super-longitudinal fashion, and their lifestyle, social background, genetic risk factors, and required care process will be assessed. This project is also notable because the care registry includes information on the successful, comprehensive management of patients with dementia. Therefore, this multicenter prospective cohort study will contribute participants to all clinical trials for Alzheimer's disease as well as improve the understanding of individuals with dementia.

Validity of cancer diagnosis in the National Health Insurance database compared with the linked National Cancer Registry in Taiwan.

PubMed

Kao, Wei-Heng; Hong, Ji-Hong; See, Lai-Chu; Yu, Huang-Ping; Hsu, Jun-Te; Chou, I-Jun; Chou, Wen-Chi; Chiou, Meng-Jiun; Wang, Chun-Chieh; Kuo, Chang-Fu

2017-08-16

We aimed to evaluate the validity of cancer diagnosis in the National Health Insurance (NHI) database, which has routinely collected the health information of almost the entire Taiwanese population since 1995, compared with the Taiwan National Cancer Registry (NCR). There were 26,542,445 active participants registered in the NHI database between 2001 and 2012. National Cancer Registry and NHI database records were compared for cancer diagnosis; date of cancer diagnosis; and 1, 2, and 5 year survival. In addition, the 10 leading causes of cancer deaths in Taiwan were analyzed. There were 908,986 cancer diagnoses in NCR and NHI database and 782,775 (86.1%) in both, with 53,192 (5.9%) in the NHI database only and 73,019 (8.0%) in the NCR only. The positive predictive value of the NHI database cancer diagnoses was 94% for all cancers; the positive predictive value of the 10 specific cancers ranged from 95% (lung cancer) to 82% (cervical cancer). The date of diagnosis in the NHI database was generally delayed by a median of 15 days (interquartile range 8-18) compared with the NCR. The 1, 2, and 5 year survival rates were 71.21%, 60.85%, and 47.44% using the NHI database and were 71.18%, 60.17%, and 46.09% using NCR data. Recording of cancer diagnoses and survival estimates based on these diagnosis codes in the NHI database are generally consistent with the NCR. Studies using NHI database data must pay careful attention to eligibility and record linkage; use of both sources is recommended. Copyright © 2017 John Wiley & Sons, Ltd.

Marked improvements in outcomes of contemporary percutaneous coronary intervention in patients with diabetes mellitus.

PubMed

Freeman, Andrew M; Abbott, J Dawn; Jacobs, Alice K; Vlachos, Helen A; Selzer, Faith; Laskey, Warren K; Detre, Katherine M; Williams, David O

2006-12-01

We sought to determine if advances in percutaneous coronary intervention (PCI) are associated with better outcomes among patients with diabetes mellitus (DM). Patients with DM enrolled in the National Heart, Lung, and Blood Institute (NHLBI) early PTCA Registry (1985-1986) were compared to those in the subsequent contemporary Dynamic Registry (1999-2002) for in-hospital and one-year cardiovascular outcomes. The study population included 945 adults with DM, 325 from the PTCA Registry and 620 from the Dynamic Registry. Multivariable Cox regression models were built to estimate the risk of clinical events. Dynamic Registry patients were older, had more noncardiac comorbidities, and a lower mean ejection fraction (50.5% vs 57.8%, P < or = 0.001) compared to the PTCA Registry patients. The incidence of in-hospital mortality (1.9% vs 4.3%, P < or = 0.05), myocardial infarction (MI) (1.0% vs 7.4%, P

Renal replacement therapy in Europe: a summary of the 2011 ERA-EDTA Registry Annual Report.

PubMed

Noordzij, Marlies; Kramer, Anneke; Abad Diez, José M; Alonso de la Torre, Ramón; Arcos Fuster, Emma; Bikbov, Boris T; Bonthuis, Marjolein; Bouzas Caamaño, Encarnación; Čala, Svetlana; Caskey, Fergus J; Castro de la Nuez, Pablo; Cernevskis, Harijs; Collart, Frederic; Díaz Tejeiro, Rafael; Djukanovic, Ljubica; Ferrer-Alamar, Manuel; Finne, Patrik; García Bazaga, María de Los Angelos; Garneata, Liliana; Golan, Eliezer; Gonzalez Fernández, Raquel; Heaf, James G; Hoitsma, Andries; Ioannidis, George A; Kolesnyk, Mykola; Kramar, Reinhard; Lasalle, Mathilde; Leivestad, Torbjørn; Lopot, Frantisek; van de Luijtgaarden, Moniek W M; Macário, Fernando; Magaz, Ángela; Martín Escobar, Eduardo; de Meester, Johan; Metcalfe, Wendy; Ots-Rosenberg, Mai; Palsson, Runolfur; Piñera, Celestino; Pippias, Maria; Prütz, Karl G; Ratkovic, Marina; Resić, Halima; Rodríguez Hernández, Aurelio; Rutkowski, Boleslaw; Spustová, Viera; Stel, Vianda S; Stojceva-Taneva, Olivera; Süleymanlar, Gültekin; Wanner, Christoph; Jager, Kitty J

2014-04-01

This article provides a summary of the 2011 ERA-EDTA Registry Annual Report (available at www.era-edta-reg.org). Data on renal replacement therapy (RRT) for end-stage renal disease (ESRD) from national and regional renal registries in 30 countries in Europe and bordering the Mediterranean Sea were used. From 27 registries, individual patient data were received, whereas 17 registries contributed data in aggregated form. We present the incidence and prevalence of RRT, and renal transplant rates in 2011. In addition, survival probabilities and expected remaining lifetimes were calculated for those registries providing individual patient data. The overall unadjusted incidence rate of RRT in 2011 among all registries reporting to the ERA-EDTA Registry was 117 per million population (pmp) (n = 71.631). Incidence rates varied from 24 pmp in Ukraine to 238 pmp in Turkey. The overall unadjusted prevalence of RRT for ESRD on 31 December 2011 was 692 pmp (n = 425 824). The highest prevalence was reported by Portugal (1662 pmp) and the lowest by Ukraine (131 pmp). Among all registries, a total of 22 814 renal transplantations were performed (37 pmp). The highest overall transplant rate was reported from Spain, Cantabria (81 pmp), whereas the highest rate of living donor transplants was reported from Turkey (39 pmp). For patients who started RRT between 2002 and 2006, the unadjusted 5-year patient survival on RRT was 46.8% [95% confidence interval (CI) 46.6-47.0], and on dialysis 39.3% (95% CI 39.2-39.4). The unadjusted 5-year patient survival after the first renal transplantation performed between 2002 and 2006 was 86.7% (95% CI 86.2-87.2) for kidneys from deceased donors and 94.3% (95% CI 93.6-95.0) for kidneys from living donors.

Impact of chronic kidney disease on use of evidence-based therapy in stable coronary artery disease: a prospective analysis of 22,272 patients.

PubMed

Kalra, Paul R; García-Moll, Xavier; Zamorano, José; Kalra, Philip A; Fox, Kim M; Ford, Ian; Ferrari, Roberto; Tardif, Jean-Claude; Tendera, Michal; Greenlaw, Nicola; Steg, Ph Gabriel

2014-01-01

To assess the frequency of chronic kidney disease (CKD), define the associated demographics, and evaluate its association with use of evidence-based drug therapy in a contemporary global study of patients with stable coronary artery disease. 22,272 patients from the ProspeCtive observational LongitudinAl RegIstry oF patients with stable coronary arterY disease (CLARIFY) were included. Baseline estimated glomerular filtration rate (eGFR) was calculated (CKD-Epidemiology Collaboration formula) and patients categorised according to CKD stage: >89, 60-89, 45-59 and <45 mL/min/1.73 m2. Mean (SD) age was 63.9±10.4 years, 77.3% were male, 61.8% had a history of myocardial infarction, 71.9% hypertension, 30.4% diabetes and 75.4% dyslipidaemia. Chronic kidney disease (eGFR<60 mL/min/1.73 m2) was seen in 22.1% of the cohort (6.9% with eGFR<45 mL/min/1.73 m2); lower eGFR was associated with increasing age, female sex, cardiovascular risk factors, overt vascular disease, other comorbidities and higher systolic but lower diastolic blood pressure. High use of secondary prevention was seen across all CKD stages (overall 93.4% lipid-lowering drugs, 95.3% antiplatelets, 75.9% beta-blockers). The proportion of patients taking statins was lower in patients with CKD. Antiplatelet use was significantly lower in patients with CKD whereas oral anticoagulant use was higher. Angiotensin-converting enzyme inhibitor use was lower (52.0% overall) and inversely related to declining eGFR, whereas angiotensin-receptor blockers were more frequently prescribed in patients with reduced eGFR. Chronic kidney disease is common in patients with stable coronary artery disease and is associated with comorbidities. Whilst use of individual evidence-based medications for secondary prevention was high across all CKD categories, there remains an opportunity to improve the proportion who take all three classes of preventive therapies. Angiotensin-converting enzyme inhibitors were used less frequently in lower eGRF categories. Surprisingly the reverse was seen for angiotensin-receptor blockers. Further evaluation is required to fully understand these associations. The CLARIFY (ProspeCtive observational LongitudinAl RegIstry oF patients with stable coronary arterY disease) Registry is registered in the ISRCTN registry of clinical trials with the number ISRCTN43070564. http://www.controlled-trials.com/ISRCTN43070564.

Rationale and design of three observational, prospective cohort studies including biobanking to evaluate and improve diagnostics, management strategies and risk stratification in venous thromboembolism: the VTEval Project

PubMed Central

Frank, Bernd; Ariza, Liana; Lamparter, Heidrun; Grossmann, Vera; Prochaska, Jürgen H; Ullmann, Alexander; Kindler, Florentina; Weisser, Gerhard; Walter, Ulrich; Lackner, Karl J; Espinola-Klein, Christine; Münzel, Thomas; Konstantinides, Stavros V; Wild, Philipp S

2015-01-01

Introduction Venous thromboembolism (VTE) with its two manifestations deep vein thrombosis (DVT) and pulmonary embolism (PE) is a major public health problem. The VTEval Project aims to investigate numerous research questions on diagnosis, clinical management, treatment and prognosis of VTE, which have remained uncertain to date. Methods and analysis The VTEval Project consists of three observational, prospective cohort studies on VTE comprising cohorts of individuals with a clinical suspicion of acute PE (with or without DVT), with a clinical suspicion of acute DVT (without symptomatic PE) and with an incidental diagnosis of VTE (PE or DVT). The VTEval Project expects to enrol a total of approximately 2000 individuals with subsequent active and passive follow-up investigations over a time period of 5 years per participant. Time points for active follow-up investigations are at months 3, 6, 12, 24 and 36 after diagnosis (depending on the disease cohort); passive follow-up investigations via registry offices and the cancer registry are performed 48 and 60 months after diagnosis for all participants. Primary short-term outcome is defined by overall mortality (PE-related death and all other causes of death), primary long-term outcome by symptomatic VTE (PE-related death, recurrence of non-fatal PE or DVT). The VTEval Project includes three ‘all-comer’ studies and involves the standardised acquisition of high-quality data, covering the systematic assessment of VTE including symptoms, risk profile, psychosocial, environmental and lifestyle factors as well as clinical and subclinical disease, and it builds up a large state-of-the-art biorepository containing various materials from serial blood samplings. Ethics and dissemination The VTEval Project has been approved by the local data safety commissioner and the responsible ethics committee (reference no. 837.320.12 (8421-F)). Trial results will be published in peer-reviewed journals and presented at national and international scientific meetings. Trial registration number NCT02156401. PMID:26133379

40 CFR 300.4 - Abbreviations.

Code of Federal Regulations, 2013 CFR

2013-07-01

... Substances and Disease Registry CDC—Centers for Disease Control DOC—Department of Commerce DOD—Department of... Response Advisory Team DRG—District Response Group ERT—Environmental Response Team ESF—Emergency Support... Action RCP—Regional Contingency Plan RD—Remedial Design RERT—Radiological Emergency Response Team RI...

Autoimmune Inner Ear Disease

MedlinePlus

... Programs Professional Development Home AcademyU Home Study Course Maintenance of Certification Conferences & Events Practice Management Home Resources Quality Clinical Data Registry Research Reimbursement ...

Superfund: Funding for the Agency for Toxic Substances and Disease Registry

DOE Office of Scientific and Technical Information (OSTI.GOV)

Not Available

Neither the Environmental Protection Agency nor the Office of Management and Budget is violating the law by reviewing or revising the Agency for Toxic Substances and Disease Registry's budget request before the President submits it to Congress. The review also does not violate section III (n) of the superfund Amendments and Reauthorization Act of 1986 which secures minimum funding for ATSDR that can only be changed by Congress. However, GAO believes that this provision prohibits EPA from specifying how ATSDR should spend superfund money. It is therefore recommending that EPA not place any restrictions on the use of funds earmarkedmore » for ATSDR under section III(n) of SARA.« less

The German Aortic Valve Registry (GARY): a nationwide registry for patients undergoing invasive therapy for severe aortic valve stenosis.

PubMed

Beckmann, A; Hamm, C; Figulla, H R; Cremer, J; Kuck, K H; Lange, R; Zahn, R; Sack, S; Schuler, G C; Walther, T; Beyersdorf, F; Böhm, M; Heusch, G; Funkat, A K; Meinertz, T; Neumann, T; Papoutsis, K; Schneider, S; Welz, A; Mohr, F W

2012-07-01

Background The increasing prevalence of severe aortic valve defects correlates with the increase of life expectancy. For decades, surgical aortic valve replacement (AVR), under the use of extracorporeal circulation, has been the gold standard for treatment of severe aortic valve diseases. In Germany ~12,000 patients receive isolated aortic valve surgery per year. For some time, percutaneous balloon valvuloplasty has been used as a palliative therapeutic option for very few patients. Currently, alternatives for the established surgical procedures such as transcatheter aortic valve implantation (TAVI) have become available, but there are only limited data from randomized studies or low-volume registries concerning long-time outcome. In Germany, the implementation of this new technology into hospital care increased rapidly in the past few years. Therefore, the German Aortic Valve Registry (GARY) was founded in July 2010 including all available therapeutic options and providing data from a large quantity of patients.Methods The GARY is assembled as a complete survey for all invasive therapies in patients with relevant aortic valve diseases. It evaluates the new therapeutic options and compares them to surgical AVR. The model for data acquisition is based on three data sources: source I, the mandatory German database for external performance measurement; source II, a specific registry dataset; and source III, a follow-up data sheet (generated by phone interview). Various procedures will be compared concerning observed complications, mortality, and quality of life up to 5 years after the initial procedure. Furthermore, the registry will enable a compilation of evidence-based indication criteria and, in addition, also a comparison of all approved operative procedures, such as Ross or David procedures, and the use of different mechanical or biological aortic valve prostheses.Results Since the launch of data acquisition in July 2010, almost all institutions performing aortic valve procedures in Germany joined the registry. By now, 91 sites which perform TAVI in Germany participate and more than 15,000 datasets are already in the registry.Conclusion The implementation of new or innovative medical therapies needs supervision under the conditions of a well-structured scientific project. Up to now relevant data for implementation of TAVI and long-term results are missing. In contrast to randomized controlled trials, GARY is a prospective, controlled, 5-year observational multicenter registry, and a real world investigation with only one exclusion criterion, the absence of patients' written consent. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Renal replacement therapy in Europe: a summary of the 2013 ERA-EDTA Registry Annual Report with a focus on diabetes mellitus.

PubMed

Kramer, Anneke; Pippias, Maria; Stel, Vianda S; Bonthuis, Marjolein; Abad Diez, José Maria; Afentakis, Nikolaos; Alonso de la Torre, Ramón; Ambuhl, Patrice; Bikbov, Boris; Bouzas Caamaño, Encarnación; Bubic, Ivan; Buturovic-Ponikvar, Jadranka; Caskey, Fergus J; Castro de la Nuez, Pablo; Cernevskis, Harijs; Collart, Frederic; Comas Farnés, Jordi; Garcia Bazaga, Maria de Los Ángeles; De Meester, Johan; Ferrer Alamar, Manuel; Finne, Patrik; Garneata, Liliana; Golan, Eliezer; G Heaf, James; Hemmelder, Marc; Ioannou, Kyriakos; Kantaria, Nino; Kolesnyk, Mykola; Kramar, Reinhard; Lassalle, Mathilde; Lezaic, Visnja; Lopot, Frantisek; Macário, Fernando; Magaz, Angela; Martín-Escobar, Eduardo; Metcalfe, Wendy; Ots-Rosenberg, Mai; Palsson, Runolfur; Piñera Celestino, Celestino; Resić, Halima; Rutkowski, Boleslaw; Santiuste de Pablos, Carmen; Spustová, Viera; Stendahl, Maria; Strakosha, Ariana; Süleymanlar, Gültekin; Torres Guinea, Marta; Varberg Reisæter, Anna; Vazelov, Evgueniy; Ziginskiene, Edita; Massy, Ziad A; Wanner, Christoph; Jager, Kitty J; Noordzij, Marlies

2016-06-01

This article provides a summary of the 2013 European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry Annual Report (available at http://www.era-edta-reg.org), with a focus on patients with diabetes mellitus (DM) as the cause of end-stage renal disease (ESRD). In 2015, the ERA-EDTA Registry received data on renal replacement therapy (RRT) for ESRD from 49 national or regional renal registries in 34 countries in Europe and bordering the Mediterranean Sea. Individual patient data were provided by 31 registries, while 18 registries provided aggregated data. The total population covered by the participating registries comprised 650 million people. In total, 72 933 patients started RRT for ESRD within the countries and regions reporting to the ERA-EDTA Registry, resulting in an overall incidence of 112 per million population (pmp). The overall prevalence on 31 December 2013 was 738 pmp (n = 478 990). Patients with DM as the cause of ESRD comprised 24% of the incident RRT patients (26 pmp) and 17% of the prevalent RRT patients (122 pmp). When compared with the USA, the incidence of patients starting RRT pmp secondary to DM in Europe was five times lower and the incidence of RRT due to other causes of ESRD was two times lower. Overall, 19 426 kidney transplants were performed (30 pmp). The 5-year adjusted survival for all RRT patients was 60.9% [95% confidence interval (CI) 60.5-61.3] and 50.6% (95% CI 49.9-51.2) for patients with DM as the cause of ESRD.

Demographics of US pediatric contact dermatitis registry providers.

PubMed

Goldenberg, Alina; Jacob, Sharon E

2015-01-01

Children are as likely as adults to be sensitized and reactive to contact allergens. However, the prevailing data on pediatric allergic contact dermatitis are quantitatively and qualitatively limited because of a narrow geographic localization of data-reporting providers. The aim of the study was to present the first quarter results from the Loma Linda Pediatric Contact Dermatitis Registry focused on registered providers who self-identified as providing care for pediatric allergic contact dermatitis (ACD) within the United States. The US providers were invited to join the registry via completion of an online, secure, 11-question registration survey addressing demographics and clinical practice essentials. The presented results reflect data gathered within the first quarter of registry recruitment; registration is ongoing. Of 169 responders from 48 states, the majority of providers were female (60.4%), academic (55.6%), and dermatologists (76.3%). Based on individual provider averages, the minimum cumulative number of pediatric patch-test evaluations performed each year ranged between 1372 and 3468 children. The Pediatric Contact Dermatitis Registry provides a description of the current leaders in the realm of pediatric ACD and gaps, which are in need of attention. The registry allows for a collaborative effort to exchange information, educate providers, and foster investigative research with the hope of legislation that can reduce the disease burden of ACD in US children.

Comprehensive computerized diabetes registry. Serving the Cree of Eeyou Istchee (eastern James Bay).

PubMed Central

Dannenbaum, D.; Verronneau, M.; Torrie, J.; Smeja, H.; Robinson, E.; Dumont, C.; Kovitch, I.; Webster, T.

1999-01-01

PROBLEM BEING ADDRESSED: Diabetes is rapidly evolving as a major health concern in the Cree population of eastern James Bay (Eeyou Istchee). The Cree Board of Health and Social Services of James Bay (CBHSSJB) diabetes registry was the initial phase in the development of a comprehensive program for diabetes in this region. OBJECTIVE OF PROGRAM: The CBHSSJB diabetes registry was developed to provide a framework to track the prevalence of diabetes and the progression of diabetic complications. The database will also identify patients not receiving appropriate clinical and laboratory screening for diabetic complications, and will provide standardized clinical flow sheets for routine patient management. MAIN COMPONENTS OF PROGRAM: The CBHSSJB diabetes registry uses a system of paper registration forms and clinical flow sheets kept in the nine community clinics. Information from these sheets is entered into a computer database annually. The flow sheets serve as a guideline for appropriate management of patients with diabetes, and provide a one-page summary of relevant clinical and laboratory information. CONCLUSIONS: A diabetes registry is vital to follow the progression of diabetes and diabetic complications in the region served by the CBHSSJB. The registry system incorporates both a means for regional epidemiologic monitoring of diabetes mellitus and clinical tools for managing patients with the disease. PMID:10065310

User experience analysis of e-TB Manager, a nationwide electronic tuberculosis recording and reporting system in Ukraine.

PubMed

Konduri, Niranjan; Sawyer, Kelly; Nizova, Nataliya

2017-04-01

Ukraine has successfully implemented e-TB Manager nationwide as its mandatory national tuberculosis registry after first introducing it in 2009. Our objective was to perform an end-of-programme evaluation after formal handover of the registry administration to Ukraine's Centre for Disease Control in 2015. We conducted a nationwide, cross-sectional, anonymous, 18-point user experience survey, and stratified the registry's transaction statistics to demonstrate usability. Contrary to initial implementation experience, older users (aged >50 years), often with limited or no computer proficiency prior to using the registry, had significantly better user experience scores for at least six of the 12 measures compared to younger users (aged 18-29 years). Using the registry for >3 years was associated with significantly higher scores for having capacity, adequacy of training received and satisfaction with the registry. Of the 5.9 million transactions over a 4-year period, nine out of 24 oblasts (regions) and Kiev city accounted for 62.5% of all transactions, and corresponded to 59% of Ukraine's tuberculosis burden. There were 437 unique active users in 486 rayons (districts) of Ukraine, demonstrating extensive reach. Our key findings complement the World Health Organization and European Respiratory Society's agenda for action on digital health to help implement the End TB Strategy.

Clinical presentation of intraocular retinoblastoma; 5-year hospital-based registry in Egypt.

PubMed

El Zomor, Hossam; Nour, Radwa; Alieldin, Adel; Taha, Hala; Montasr, Mohamed M; Moussa, Emad; El Nadi, Enas; Ezzat, Sameera; Alfaar, Ahmad S

2015-12-01

To study the presenting signs of Retinoblastoma in Egypt at Egypt's main pediatric oncology referral center. This is a prospective descriptive study (hospital-based registry) conducted at Children's Cancer Hospital Egypt between July 2007 and December 2012. Out of 262 patients diagnosed with retinoblastoma, 244 were suffering from intra-ocular disease at presentation. One hundred thirty-nine (57%) patients presented with unilateral disease, while 105 (43%) suffered bilateral disease. The mean age at presentation was 20.6 ± 17 months, averaging 18.87 ± 11.76 months for bilateral and 25.72 ± 18.78 months for unilateral disease. The most common clinical presentation was leukocoria in 180 (73.8%) patients, strabismus in 32 (13.1%) patients and decreased visual acuity in 12 (4.9%) patients. Group D and E disease represented 62% of all affected eyes. Patients with advanced disease (Group C-E) had longer duration of symptoms. In Egypt, retinoblastoma patients present more frequently with advanced disease. There is an ever-increasing need to develop a national team dedicated to studying disease significance and formulating a national awareness program. Copyright © 2015 The Authors. Production and hosting by Elsevier B.V. All rights reserved.

Validation of a case definition to define chronic dialysis using outpatient administrative data.

PubMed

Clement, Fiona M; James, Matthew T; Chin, Rick; Klarenbach, Scott W; Manns, Braden J; Quinn, Robert R; Ravani, Pietro; Tonelli, Marcello; Hemmelgarn, Brenda R

2011-03-01

Administrative health care databases offer an efficient and accessible, though as-yet unvalidated, approach to studying outcomes of patients with chronic kidney disease and end-stage renal disease (ESRD). The objective of this study is to determine the validity of outpatient physician billing derived algorithms for defining chronic dialysis compared to a reference standard ESRD registry. A cohort of incident dialysis patients (Jan. 1-Dec. 31, 2008) and prevalent chronic dialysis patients (Jan 1, 2008) was selected from a geographically inclusive ESRD registry and administrative database. Four administrative data definitions were considered: at least 1 outpatient claim, at least 2 outpatient claims, at least 2 outpatient claims at least 90 days apart, and continuous outpatient claims at least 90 days apart with no gap in claims greater than 21 days. Measures of agreement of the four administrative data definitions were compared to a reference standard (ESRD registry). Basic patient characteristics are compared between all 5 patient groups. 1,118,097 individuals formed the overall population and 2,227 chronic dialysis patients were included in the ESRD registry. The three definitions requiring at least 2 outpatient claims resulted in kappa statistics between 0.60-0.80 indicating "substantial" agreement. "At least 1 outpatient claim" resulted in "excellent" agreement with a kappa statistic of 0.81. Of the four definitions, the simplest (at least 1 outpatient claim) performed comparatively to other definitions. The limitations of this work are the billing codes used are developed in Canada, however, other countries use similar billing practices and thus the codes could easily be mapped to other systems. Our reference standard ESRD registry may not capture all dialysis patients resulting in some misclassification. The registry is linked to on-going care so this is likely to be minimal. The definition utilized will vary with the research objective.

How comparable are rates of malignancies in patients with rheumatoid arthritis across the world? A comparison of cancer rates, and means to optimise their comparability, in five RA registries.

PubMed

Askling, Johan; Berglind, Niklas; Franzen, Stefan; Frisell, Thomas; Garwood, Christopher; Greenberg, Jeffrey D; Ho, Meilien; Holmqvist, Marie; Horne, Laura; Inoue, Eisuke; Michaud, Kaleb; Nyberg, Fredrik; Pappas, Dimitrios A; Reed, George; Tanaka, Eiichi; Tran, Trung N; Verstappen, Suzanne M M; Yamanaka, Hisashi; Wesby-van Swaay, Eveline; Symmons, Deborah

2016-10-01

The overall incidence of cancer in patients with rheumatoid arthritis (RA) is modestly elevated. The extent to which cancer rates in RA vary across clinical cohorts and patient subsets, as defined by disease activity or treatment is less known but critical for understanding the safety of existing and new antirheumatic therapies. We investigated comparability of, and means to harmonise, malignancy rates in five RA registries from four continents. Participating RA registries were Consortium of Rheumatology Researchers of North America (CORRONA) (USA), Swedish Rheumatology Quality of Care Register (SRR) (Sweden), Norfolk Arthritis Register (NOAR) (UK), CORRONA International (several countries) and Institute of Rheumatology, Rheumatoid Arthritis (IORRA) (Japan). Within each registry, we analysed a main cohort of all patients with RA from January 2000 to last available data, and sensitivity analyses of sub-cohorts defined by disease activity, treatment change, prior comorbidities and restricted by calendar time or follow-up, respectively. Malignancy rates with 95% CIs were estimated, and standardised for age and sex, based on the distributions from a typical RA clinical trial programme population (fostamatinib). There was a high consistency in rates for overall malignancy excluding non-melanoma skin cancer (NMSC), for malignant lymphomas, but not for all skin cancers, across registries, in particular following age/sex standardisation. Standardised rates of overall malignancy excluding NMSC varied from 0.56 to 0.87 per 100 person-years. Within each registry, rates were generally consistent across sensitivity analyses, which differed little from the main analysis. In real-world RA populations, rates of both overall malignancy and of lymphomas are consistent. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Haemopoietic stem cell transplantation in Australia and New Zealand, 1992-2001: progress report from the Australasian Bone Marrow Transplant Recipient Registry.

PubMed

Nivison-Smith, I; Bradstock, K F; Dodds, A J; Hawkins, P A; Szer, J

2005-01-01

Bone marrow and blood stem cell transplantation is now used as curative therapy for a range of haematological malignancies and other conditions. The Australasian Bone Marrow Transplant Recipient Registry (ABMTRR) has recorded transplant activity in Australia since 1992; transplant centres in New Zealand have corresponded with the Registry since 1998. To describe allogeneic and autologous bone marrow and blood stem cell transplantation activity and outcomes in Australia and New Zealand from 1992 to 2001. Each haemopoietic stem cell transplant centre in Australia and New Zealand contributes information to the Registry via a single information form compiled when a transplant is performed. An annual follow-up request is then sent from the Registry to the contributing centre at the anniversary of each individual transplant. Haemopoietic stem cell transplants in Australia have increased in number from 478 in 1992 to 937 in 2001, whereas in New Zealand the number has grown from 91 in 1998 to 105 in 2001, mainly as a result of an increase in autologous blood stem cell transplants. The number of hospitals contributing to the ABMTRR has grown from 20 in 1992 to 37 in 2001. The most common indication for autologous transplantation in 2001 was non-Hodgkin's lymphoma, whereas for allogeneic transplants it was acute myeloid leukaemia. The 9-year actuarial disease-free survival probability for patients aged 16 and above between 1992 and 2000 was 37% for autologous, 39% for allogeneic related donor and 30% for allogeneic unrelated donor transplants. Recurrence of the underlying disease was the main cause of death post-transplant after both allogeneic (26.3% of deaths in the first year and 68.0% of deaths in the second year) and autologous transplants (59.0% and 86.2%). Treatment-related mortality was 16.9% after allogeneic transplantation and 2.1% after autologous transplantation in 2000. The ABMTRR provides a comprehensive source of information on the use of bone marrow transplant, and allows for continuing analysis of changes in the application of this high-cost technology and the outcome of patients undergoing these procedures. Registry data provide a means for directing future clinical research into perceived areas of priority for improvement of outcome, such as the reduction in the risk of disease recurrence post-transplant.

An overview and online registry of microvillus inclusion disease patients and their MYO5B mutations.

PubMed

van der Velde, K Joeri; Dhekne, Herschel S; Swertz, Morris A; Sirigu, Serena; Ropars, Virginie; Vinke, Petra C; Rengaw, Trebor; van den Akker, Peter C; Rings, Edmond H H M; Houdusse, Anne; van Ijzendoorn, Sven C D

2013-12-01

Microvillus inclusion disease (MVID) is one of the most severe congenital intestinal disorders and is characterized by neonatal secretory diarrhea and the inability to absorb nutrients from the intestinal lumen. MVID is associated with patient-, family-, and ancestry-unique mutations in the MYO5B gene, encoding the actin-based motor protein myosin Vb. Here, we review the MYO5B gene and all currently known MYO5B mutations and for the first time methodologically categorize these with regard to functional protein domains and recurrence in MYO7A associated with Usher syndrome and other myosins. We also review animal models for MVID and the latest data on functional studies related to the myosin Vb protein. To congregate existing and future information on MVID geno-/phenotypes and facilitate its quick and easy sharing among clinicians and researchers, we have constructed an online MOLGENIS-based international patient registry (www.MVID-central.org). This easily accessible database currently contains detailed information of 137 MVID patients together with reported clinical/phenotypic details and 41 unique MYO5B mutations, of which several unpublished. The future expansion and prospective nature of this registry is expected to improve disease diagnosis, prognosis, and genetic counseling. © 2013 WILEY PERIODICALS, INC.

Essential Hypotension and Allostasis Registry

ClinicalTrials.gov

2018-03-30

Blood Pressure; Depression; Panic Attack; Fibromyalgia; POTS; Inappropriate Sinus Tachycardia; Coronary Heart Disease; Acute Coronary Syndrome (ACS); Acute Myocardial Infarction (AMI); Cerebrovascular Disease (CVD); Transient Ischemic Attack (TIA); Atrial Fibrillation; Diabetes Mellitus; Cancer; Systolic Heart Failure; Diastolic Heart Failure; Chronic Fatigue Syndrome; Syncope; Vasovagal Syncope

Patients with inflammatory bowel disease have increased risk of autoimmune and inflammatory diseases

PubMed Central

Halling, Morten L; Kjeldsen, Jens; Knudsen, Torben; Nielsen, Jan; Hansen, Lars Koch

2017-01-01

AIM To investigate whether immune mediated diseases (IMD) are more frequent in patients with inflammatory bowel disease (IBD). METHODS In this population based registry study, a total of 47325 patients with IBD were alive and registered in the Danish National Patient Registry on December 16, 2013. Controls were randomly selected from the Danish Civil Registration System (CRS) and matched for sex, age, and municipality. We used ICD 10 codes to identify the diagnoses of the included patients. The IBD population was divided into three subgroups: Ulcerative colitis (UC), Crohn’s disease (CD) and Both the latter referring to those registered with both diagnoses. Subsequently, odds-ratios (OR) and 95%CI were obtained separately for each group and their respective controls. The use of Bonferoni post-test correction adjusted the significance level to P < 0.00125. P-values were estimated using Fisher’s exact test. RESULTS There were significantly more women than men in the registry, and a greater percentage of comorbidity in the IBD groups (P < 0.05). Twenty different IMDs were all significantly more frequent in the IBD group. Sixteen were associated with UC versus twelve with CD. In both UC and CD ORs were significantly increased (P < 0.00125) for primary sclerosing cholangitis (PSC), celiac disease, type 1 diabetes (T1D), sarcoidosis, asthma, iridocyclitis, psoriasis, pyoderma gangrenosum, rheumatoid arthritis, and ankylosing spondylitis. Restricted to UC (P < 0.00125) were autoimmune hepatitis, primary biliary cholangitis, Grave’s disease, polymyalgia rheumatica, temporal arteritis , and atrophic gastritis. Restricted to CD (P < 0.00125) were psoriatic arthritis and episcleritis. Restricted to women with UC (P < 0.00125) were atrophic gastritis, rheumatoid arthritis, temporal arteritis, and polymyalgia rheumatica. Restricted to women with CD were episcleritis, rheumatoid arthritis, and psoriatic arthritis. The only disease restricted to men (P < 0.00125) was sarcoidosis. CONCLUSION Immune mediated diseases were significantly more frequent in patients with IBD. Our results strengthen the hypothesis that some IMDs and IBD may have overlapping pathogenic pathways. PMID:28970729

Patients with inflammatory bowel disease have increased risk of autoimmune and inflammatory diseases.

PubMed

Halling, Morten L; Kjeldsen, Jens; Knudsen, Torben; Nielsen, Jan; Hansen, Lars Koch

2017-09-07

To investigate whether immune mediated diseases (IMD) are more frequent in patients with inflammatory bowel disease (IBD). In this population based registry study, a total of 47325 patients with IBD were alive and registered in the Danish National Patient Registry on December 16, 2013. Controls were randomly selected from the Danish Civil Registration System (CRS) and matched for sex, age, and municipality. We used ICD 10 codes to identify the diagnoses of the included patients. The IBD population was divided into three subgroups: Ulcerative colitis (UC), Crohn's disease (CD) and Both the latter referring to those registered with both diagnoses. Subsequently, odds-ratios (OR) and 95%CI were obtained separately for each group and their respective controls. The use of Bonferoni post-test correction adjusted the significance level to P < 0.00125. P -values were estimated using Fisher's exact test. There were significantly more women than men in the registry, and a greater percentage of comorbidity in the IBD groups ( P < 0.05). Twenty different IMDs were all significantly more frequent in the IBD group. Sixteen were associated with UC versus twelve with CD. In both UC and CD ORs were significantly increased ( P < 0.00125) for primary sclerosing cholangitis (PSC), celiac disease, type 1 diabetes (T1D), sarcoidosis, asthma, iridocyclitis, psoriasis, pyoderma gangrenosum, rheumatoid arthritis, and ankylosing spondylitis. Restricted to UC ( P < 0.00125) were autoimmune hepatitis, primary biliary cholangitis, Grave's disease, polymyalgia rheumatica, temporal arteritis , and atrophic gastritis. Restricted to CD ( P < 0.00125) were psoriatic arthritis and episcleritis. Restricted to women with UC ( P < 0.00125) were atrophic gastritis, rheumatoid arthritis, temporal arteritis, and polymyalgia rheumatica. Restricted to women with CD were episcleritis, rheumatoid arthritis, and psoriatic arthritis. The only disease restricted to men ( P < 0.00125) was sarcoidosis. Immune mediated diseases were significantly more frequent in patients with IBD. Our results strengthen the hypothesis that some IMDs and IBD may have overlapping pathogenic pathways.

Mathematical models used to inform study design or surveillance systems in infectious diseases: a systematic review.

PubMed

Herzog, Sereina A; Blaizot, Stéphanie; Hens, Niel

2017-12-18

Mathematical models offer the possibility to investigate the infectious disease dynamics over time and may help in informing design of studies. A systematic review was performed in order to determine to what extent mathematical models have been incorporated into the process of planning studies and hence inform study design for infectious diseases transmitted between humans and/or animals. We searched Ovid Medline and two trial registry platforms (Cochrane, WHO) using search terms related to infection, mathematical model, and study design from the earliest dates to October 2016. Eligible publications and registered trials included mathematical models (compartmental, individual-based, or Markov) which were described and used to inform the design of infectious disease studies. We extracted information about the investigated infection, population, model characteristics, and study design. We identified 28 unique publications but no registered trials. Focusing on compartmental and individual-based models we found 12 observational/surveillance studies and 11 clinical trials. Infections studied were equally animal and human infectious diseases for the observational/surveillance studies, while all but one between humans for clinical trials. The mathematical models were used to inform, amongst other things, the required sample size (n = 16), the statistical power (n = 9), the frequency at which samples should be taken (n = 6), and from whom (n = 6). Despite the fact that mathematical models have been advocated to be used at the planning stage of studies or surveillance systems, they are used scarcely. With only one exception, the publications described theoretical studies, hence, not being utilised in real studies.

Missing data in trauma registries: A systematic review.

PubMed

Shivasabesan, Gowri; Mitra, Biswadev; O'Reilly, Gerard M

2018-03-30

Trauma registries play an integral role in trauma systems but their valid use hinges on data quality. The aim of this study was to determine, among contemporary publications using trauma registry data, the level of reporting of data completeness and the methods used to deal with missing data. A systematic review was conducted of all trauma registry-based manuscripts published from 01 January 2015 to current date (17 March 2017). Studies were identified by searching MEDLINE, EMBASE, and CINAHL using relevant subject headings and keywords. Included manuscripts were evaluated based on previously published recommendations regarding the reporting and discussion of missing data. Manuscripts were graded on their degree of characterization of such observations. In addition, the methods used to manage missing data were examined. There were 539 manuscripts that met inclusion criteria. Among these, 208 (38.6%) manuscripts did not mention data completeness and 88 (16.3%) mentioned missing data but did not quantify the extent. Only a handful (n = 26; 4.8%) quantified the 'missingness' of all variables. Most articles (n = 477; 88.5%) contained no details such as a comparison between patient characteristics in cohorts with and without missing data. Of the 331 articles which made at least some mention of data completeness, the method of managing missing data was unknown in 34 (10.3%). When method(s) to handle missing data were identified, 234 (78.8%) manuscripts used complete case analysis only, 18 (6.1%) used multiple imputation only and 34 (11.4%) used a combination of these. Most manuscripts using trauma registry data did not quantify the extent of missing data for any variables and contained minimal discussion regarding missingness. Out of the studies which identified a method of managing missing data, most used complete case analysis, a method that may bias results. The lack of standardization in the reporting and management of missing data questions the validity of conclusions from research based on trauma registry data. Copyright © 2018 Elsevier Ltd. All rights reserved.

Clinical outcomes with the STENTYS self-apposing coronary stent in patients presenting with ST-segment elevation myocardial infarction: two-year insights from the APPOSITION III (A Post-Market registry to assess the STENTYS self-exPanding COronary Stent In AcuTe MyocardIal InfarctiON) registry.

PubMed

Lu, Huangling; Grundeken, Maik J; Vos, Nicola S; IJsselmuiden, Alexander J J; van Geuns, Robert-Jan; Wessely, Rainer; Dengler, Thomas; La Manna, Alessio; Silvain, Johanne; Montalescot, Gilles; Spaargaren, René; Tijssen, Jan G P; Amoroso, Giovanni; de Winter, Robbert J; Koch, Karel T

2017-08-04

The APPOSITION III registry evaluated the feasibility and performance of the STENTYS self-apposing stent in an ST-segment elevation myocardial infarction (STEMI) population. This novel self-apposing stent device lowers stent strut malapposition rates and therefore carries the potential to prevent stent undersizing during primary percutaneous coronary intervention (PCI) in STEMI patients. To date, no long-term data are available using this device in the setting of STEMI. We aimed to evaluate the long-term clinical outcomes of the APPOSITION III registry. This was an international, prospective, multicentre post-marketing registry. The study population consisted of 965 STEMI patients. The primary endpoint, major adverse cardiac events (MACE), was defined as the composite of cardiac death, recurrent target vessel myocardial infarction (TV-MI), and clinically driven target lesion revascularisation (CD-TLR). At two years, MACE occurred in 11.2%, cardiac death occurred in 2.3%, TV-MI occurred in 2.3% and CD-TLR in 9.2% of patients. The two-year definite stent thrombosis (ST) rate was 3.3%. Incremental event rates between one- and two-year follow-up were 1.0% for TV-MI, 1.8% for CD-TLR, and 0.5% for definite ST. Post-dilation resulted in significantly reduced CD-TLR and ST rates at 30-day landmark analyses. Results were equivalent between the BMS and PES STENTYS subgroups. This registry revealed low rates of adverse events at two-year follow-up, with an incremental ST rate as low as 0.5% in the second year, demonstrating that the self-apposing technique is feasible in STEMI patients on long-term follow-up while using post-dilatation.

The Importance of Registries in the Postmarketing Surveillance of Surgical Meshes.

PubMed

Köckerling, Ferdinand; Simon, Thomas; Hukauf, Martin; Hellinger, Achim; Fortelny, Rene; Reinpold, Wolfgang; Bittner, Reinhard

2017-06-07

To assess the role of registries in the postmarketing surveillance of surgical meshes. To date, surgical meshes are classified as group II medical devices. Class II devices do not require premarket clearance by clinical studies. Ethicon initiated a voluntary market withdrawal of Physiomesh for laparoscopic use after an analysis of unpublished data from the 2 large independent hernia registries-Herniamed German Registry and Danish Hernia Database. This paper now presents the relevant data from the Herniamed Registry. The present analysis compares the prospective perioperative and 1-year follow-up data collected for all patients with incisional hernia who had undergone elective laparoscopic intraperitoneal onlay mesh repair either with Physiomesh (n = 1380) or with other meshes recommended in the guidelines (n = 3834). Patients with Physiomesh repair had a markedly higher recurrence rate compared with the other recommended meshes (12.0% vs 5.0%; P < 0.001). In the multivariable analysis, the recurrence rate was highly significantly influenced by the mesh type used (P < 0.001). If Physiomesh was used, that led to a highly significant increase in the recurrence rate on 1-year follow-up (odds ratio 2.570, 95% CI 2.057, 3.210). The mesh type used also had a significant influence on chronic pain rates. The importance of real-world data for postmarketing surveillance of surgical meshes has been demonstrated in this registry-based study. Randomized controlled trials are needed for premarket approval of new devices. The role of sponsorship of device studies by the manufacturing company must be taken into account.This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0.

The CERTAIN Registry: a novel, web-based registry and research platform for pediatric renal transplantation in Europe.

PubMed

Plotnicki, L; Kohl, C D; Höcker, B; Krupka, K; Rahmel, A; Pape, L; Hoyer, P; Marks, S D; Webb, N J A; Söylemezoglu, O; Topaloglu, R; Szabo, A J; Seeman, T; Marlies Cornelissen, E A; Knops, N; Grenda, R; Tönshoff, B

2013-05-01

The results of pediatric renal transplantation have improved markedly in the last decade. However, a number of relevant clinical problems remain, such as organ damage caused by chronic rejection, long-term toxicity of immunosuppressive therapy, difficulty in developing tolerance-inducing protocols, secondary cardiovascular comorbidity, post-transplantation lymphoproliferative disease, suboptimal longitudinal growth, quality of life, adherence to immunosuppressive medication, and structured transition programs to adult care. These unmet clinical needs require intense collaborative and interdisciplinary clinical research. We recently founded the Cooperative European Paediatric Renal TransplAnt INitiative (CERTAIN; www.certain-registry.eu) as a research network and platform built on a novel, web-based registry. The registry's dataset provides essential information on generic kidney transplantation-related topics and also captures pediatric-specific topics, such as growth, physical and psychosocial development, and adherence. Due to its flexibility the system can be used as follows: (1) as a registry capturing a minimal or an extended dataset; (2) as a center and/or country-specific transplantation database; or (3) as a patient-specific electronic transplantation chart. The data can be exported directly from the CERTAIN web application into statistical software packages for scientific analyses. The rights regarding data ownership, evaluation, and publications are regulated in the registry's rules of procedure. Data quality is ensured by automatic software validation and a manual data review process. To avoid redundant data entry, CERTAIN has established interfaces for data change with Eurotransplant, the Collaborative Transplant Study (CTS), and the registry of the European Society of Pediatric Nephrology (ESPN) and European Renal Association - European Dialysis and Transplant Association (ERA-EDTA) (ESPN/ERA-EDTA registry). CERTAIN fulfils all regulatory and ethical requirements of the European Union and Germany, in particular, regarding patients' data privacy and security. Using modern information technology, the recently established multinational CERTAIN Registry fills a gap in Europe for collaborative 5 research and quality assurance in the field of pediatric renal transplantation. Copyright © 2013 Elsevier Inc. All rights reserved.

A global patient outcomes registry: Cochlear paediatric implanted recipient observational study (Cochlear(™) P-IROS).

PubMed

Sanderson, Georgina; Ariyaratne, Thathya V; Wyss, Josephine; Looi, Valerie

2014-01-01

Currently, there is a paucity of data concerning the long-term outcomes, educational placement and quality of life of children implanted with hearing devices from large and representative samples of the population. To address this concern, a large, prospective, multicentre, multinational patient-outcomes registry for paediatric recipients of implantable hearing devices was developed. The benefits of this registry, its approach and methodology are described. The Cochlear(™) Paediatric Implanted Recipient Observational Study (Cochlear P-IROS) is a prospective international patient-outcomes registry for children who are implanted in routine clinical practice with one or more hearing devices. The study aims to collect data on patient comorbidities, device use, auditory performance, quality of life and health-related utilities, across different types of implantable hearing devices from a range of manufacturers. Patients will be evaluated with a set of standardised and non-standardised questionnaires prior to initial device activation (baseline) and at six-monthly follow-up intervals up to 24 months and annually thereafter. The Cochlear P-IROS utilises a secure web interface to administer electronic case report forms to clinicians and families of implanted children. The web interface is currently available in five languages: English, Japanese, Korean, Mandarin and Russian. The interface also provides printable versions of the case report forms translated into 22 local languages for collection of data prior to entry online; additional languages may be added, as required. Participation in the Cochlear P-IROS registry is investigator-driven and voluntary. To date, the Cochlear P-IROS has recruited implant clinics across Australia, China, India, Indonesia, Turkey and Vietnam. The registry also aims to recruit multiple clinics in Cuba, Israel, Japan, Malaysia, Singapore, South Africa, South Korea and Russia. The use of a registry such as the Cochlear P-IROS will generate valuable data to support research interests of academics and clinicians around the globe. The data generated will be relevant for a wide range of stakeholders including regulators, payers, providers, policy makers, patients and their families, each with a different perspective for the acceptance and adoption of implantable hearing devices for the treatment of hearing loss.

A global patient outcomes registry: Cochlear paediatric implanted recipient observational study (Cochlear™ P-IROS)

PubMed Central

2014-01-01

Background Currently, there is a paucity of data concerning the long-term outcomes, educational placement and quality of life of children implanted with hearing devices from large and representative samples of the population. To address this concern, a large, prospective, multicentre, multinational patient-outcomes registry for paediatric recipients of implantable hearing devices was developed. The benefits of this registry, its approach and methodology are described. Methods/Design The Cochlear™ Paediatric Implanted Recipient Observational Study (Cochlear P-IROS) is a prospective international patient-outcomes registry for children who are implanted in routine clinical practice with one or more hearing devices. The study aims to collect data on patient comorbidities, device use, auditory performance, quality of life and health-related utilities, across different types of implantable hearing devices from a range of manufacturers. Patients will be evaluated with a set of standardised and non-standardised questionnaires prior to initial device activation (baseline) and at six-monthly follow-up intervals up to 24 months and annually thereafter. The Cochlear P-IROS utilises a secure web interface to administer electronic case report forms to clinicians and families of implanted children. The web interface is currently available in five languages: English, Japanese, Korean, Mandarin and Russian. The interface also provides printable versions of the case report forms translated into 22 local languages for collection of data prior to entry online; additional languages may be added, as required. Participation in the Cochlear P-IROS registry is investigator-driven and voluntary. To date, the Cochlear P-IROS has recruited implant clinics across Australia, China, India, Indonesia, Turkey and Vietnam. The registry also aims to recruit multiple clinics in Cuba, Israel, Japan, Malaysia, Singapore, South Africa, South Korea and Russia. Discussion The use of a registry such as the Cochlear P-IROS will generate valuable data to support research interests of academics and clinicians around the globe. The data generated will be relevant for a wide range of stakeholders including regulators, payers, providers, policy makers, patients and their families, each with a different perspective for the acceptance and adoption of implantable hearing devices for the treatment of hearing loss. PMID:25317075

Pediatric GERD (Gastro-Esophageal Reflux Disease)

MedlinePlus

... Programs Professional Development Home AcademyU Home Study Course Maintenance of Certification Conferences & Events Practice Management Home Resources Quality Clinical Data Registry Research Reimbursement ...

Stroke and Cerebrovascular Diseases Registry

ClinicalTrials.gov

2017-09-11

Stroke; Acute Stroke; Acute Brain Injury; Ischemic Stroke; Hemorrhagic Stroke; Transient Ischemic Attack; Subarachnoid Hemorrhage; Cerebral Ischemia; Cerebral Infarction; Cerebral Stroke; Venous Sinus Thrombosis, Cranial

Management of concomitant coronary artery disease in patients undergoing transcatheter aortic valve implantation: the United Kingdom TAVI Registry.

PubMed

Snow, Thomas M; Ludman, Peter; Banya, Winston; DeBelder, Mark; MacCarthy, Philip M; Davies, Simon W; Di Mario, Carlo; Moat, Neil E

2015-11-15

The management and impact of concomitant coronary artery disease in patients referred for TAVI remains contentious. We describe the prevalence, clinical impact and management of coronary artery disease (CAD) in patients in the United Kingdom TAVI Registry. All-inclusive study of patients undergoing TAVI in the United Kingdom (excluding Northern Ireland) from January 2007 to December 2011. Coronary artery disease at the time of TAVI was demonstrated on invasive angiography. 2588 consecutive patients were entered in the U.K. TAVI Registry. CAD was reported in 1171 pts with left main stem involvement in 12.4% of this cohort (n=145). Most patients were free of chest pain, but limited by dyspnoea (NYHA Class III & IV 81.9%). Angina was however more prevalent in those patients with CAD (p<0.0001). Hybrid PCI was uncommon, performed in only 14.7% of the CAD cohort (n=172). Survival at 30days, 1year, and 4years was 93.7%, 81.4% and 72.0% respectively. Adjusting for confounders in a multivariate model the presence and extent of CAD was not associated with early (30-days, p=0.36) or late (4years, p=0.10) survival. This contemporary study of coronary artery disease management in an "all-comers" patient population undergoing TAVI demonstrates that whilst often an indicator of significant underlying comorbidity coronary artery disease is not associated with decreased short or long-term survival. The majority of patients with aortic stenosis and concomitant CAD can be managed effectively by TAVI alone. However, the importance of the Heart Team in making decisions on individual patients must not be underestimated. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

The impact of smoke exposure on the clinical phenotype of alpha-1 antitrypsin deficiency in Ireland: exploiting a national registry to understand a rare disease.

PubMed

O'Brien, M Emmet; Pennycooke, Kevin; Carroll, Tomás P; Shum, Jonathan; Fee, Laura T; O'Connor, Catherine; Logan, P Mark; Reeves, Emer P; McElvaney, Noel G

2015-05-01

Individuals with Alpha-1 antitrypsin deficiency (AATD) have mutations in the SERPINA1 gene causing genetic susceptibility to early onset lung and liver disease that may result in premature death. Environmental interactions have a significant impact in determining the disease phenotype and outcome in AATD. The aim of this study was to assess the impact of smoke exposure on the clinical phenotype of AATD in Ireland. Clinical demographics and available thoracic computerised tomography (CT) imaging were detected from 139 PiZZ individuals identified from the Irish National AATD Registry. Clinical information was collected by questionnaire. Data was analysed to assess AATD disease severity and evaluate predictors of clinical phenotype. Questionnaires were collected from 107/139 (77%) and thoracic CT evaluation was available in 72/107 (67.2%). 74% of respondents had severe Chronic Obstructive Pulmonary Disease (COPD) (GOLD stage C or D). Cigarette smoking was the greatest predictor of impairment in FEV1 and DLCO (%predicted) and the extent of emphysema correlated most significantly with DLCO. Interestingly the rate of FEV1 decline was similar in ex-smokers when compared to never-smokers. Passive smoke exposure in childhood resulted in a greater total pack-year smoking history. Radiological evidence of bronchiectasis was a common finding and associated with increasing age. The Irish National AATD Registry facilitates clinical and basic science research of this condition in Ireland. This study illustrates the detrimental effect of smoke exposure on the clinical phenotype of AATD in Ireland and the benefit of immediate smoking cessation at any stage of lung disease.

Sex differences in the diagnosis, treatment, and outcome of patients with pulmonary arterial hypertension enrolled in the registry to evaluate early and long-term pulmonary arterial hypertension disease management.

PubMed

Shapiro, Shelley; Traiger, Glenna L; Turner, Michelle; McGoon, Michael D; Wason, Prieya; Barst, Robyn J

2012-02-01

Pulmonary arterial hypertension (PAH) is a life-threatening disease that affects more women than men. The reasons for the female preponderance are unclear, and there are limited data available for men with PAH. Data from the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) were analyzed to explore sex differences among patients with PAH with regard to 2-year survival from enrollment and 5-year survival from time of diagnosis. The data set included 2,318 women and 651 men. More women had PAH associated with connective tissue disease (P < .001), and more men had portopulmonary hypertension (P < .001) and HIV-associated PAH (P < .001). More women had congenital heart disease-associated PAH (P = .017), thyroid disease (P < .001), and depression reported (P ≤ .001). At diagnosis, men had higher mean pulmonary artery pressure (53 ± 14 vs 51 ± 14.3 mm Hg; P = .013) and mean right atrial pressure (10 ± 6 vs 9 ± 6 mm Hg; P = .031). Women had better survival estimates for 2 years from enrollment and for 5 years from diagnosis. Stratifying by age showed that survival from enrollment was similar between men and women aged < 60 years at enrollment, whereas men aged ≥ 60 years have lower survival rates compared with women aged ≥ 60 years. Our findings highlight similarities and differences between men and women with PAH, raising questions for future exploration regarding the role of hormones and sex in causation and survival in PAH. ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.

Study design and rationale of the 'Balloon-Expandable Cobalt Chromium SCUBA Stent versus Self-Expandable COMPLETE-SE Nitinol Stent for the Atherosclerotic ILIAC Arterial Disease (SENS-ILIAC Trial) Trial': study protocol for a randomized controlled trial.

PubMed

Choi, Woong Gil; Rha, Seung Woon; Choi, Cheol Ung; Kim, Eung Ju; Oh, Dong Joo; Cho, Yoon Hyung; Park, Sang Ho; Lee, Seung Jin; Hur, Ae Yong; Ko, Young Guk; Park, Sang Min; Kim, Ki Chang; Kim, Joo Han; Kim, Min Woong; Kim, Sang Min; Bae, Jang Ho; Bong, Jung Min; Kang, Won Yu; Seo, Jae Bin; Jung, Woo Yong; Cho, Jang Hyun; Kim, Do Hoi; Ahn, Ji Hoon; Kim, Soo Hyun; Jang, Ji Yong

2016-06-25

The self-expandable COMPLETE™ stent (Medtronic) has greater elasticity, allowing it to regain its shape after the compression force reduces, and has higher trackability, thus is easier to maneuver through tortuous vessels, whereas the balloon-expandable SCUBA™ stent (Medtronic) has higher radial stiffness and can afford more accurate placement without geographic miss, which is important in aortoiliac bifurcation lesions. To date, there have been no randomized control trials comparing efficacy and safety between the self-expanding stent and balloon-expandable stent in advanced atherosclerotic iliac artery disease. The purpose of our study is to examine primary patency (efficacy) and incidence of stent fracture and geographic miss (safety) between two different major representative stents, the self-expanding nitinol stent (COMPLETE-SE™) and the balloon-expanding cobalt-chromium stent (SCUBA™), in stenotic or occlusive iliac arterial lesions. This trial is designed as a prospective, randomized, multicenter trial to demonstrate a noninferiority of SCUBA™ stent to COMPLETE-SE™ stent following balloon angioplasty in iliac arterial lesions, and a total of 280 patients will be enrolled. The primary end point of this study is the rate of primary patency in the treated segment at 12 months after intervention as determined by catheter angiography, computed tomography angiography, or duplex ultrasound. The SENS-ILIAC trial will give powerful insight into whether the stent choice according to deployment mechanics would impact stent patency, geographic miss, or stent fracture in patients undergoing stent implantation in iliac artery lesions. National Institutes of Health Clinical Trials Registry (ClinicalTrials.gov identifier: NCT01834495 ), registration date: May 8, 2012.

Establishment of the Fox Chase Network Breast Cancer Risk Registry

DTIC Science & Technology

1996-10-01

Neurofibromatosis, type I h) Non-insulin dependent diabetes i) Turner Syndrome j) Tay Sachs Disease k) Marfan Syndrome 1) Cancer (tricky!) 2. Human chromosomes are...gastrointestinal and genitourinary cancers (4). Altogether, over 12 genetic-cancer syndromes have been localized to a specific gene (5). Some families suffer...component of the rare help test the best ways to Risk Registry._5, cases of breast cancer tions are likely to result Li-Fraumeni syndrome , only and up to

World Health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology.

PubMed

Prins, Kurt W; Thenappan, Thenappan

2016-08-01

Pulmonary arterial hypertension (PAH) is a debilitating disease characterized by pathologic remodeling of the resistance pulmonary arteries, ultimately leading to right ventricular (RV) failure and death. In this article we discuss the definition of PAH, the initial epidemiology based on the National Institutes of Health Registry, and the updated epidemiology gleaned from contemporary registries, pathogenesis of pulmonary vascular dysfunction and proliferation, and RV failure in PAH. Copyright © 2016 Elsevier Inc. All rights reserved.

California’s Parkinson’s Disease Registry Pilot Project - Coordination Center and Northern California Ascertainment

DTIC Science & Technology

2014-03-01

www.capdregistry.org) and email box were created and launched in March, 2008. Requests for information about the registry from patients, colleagues and...d. Review and determination of study diagnosis. For cases with multiple parkinsonism codes (i.e. diagnosed with more than one of 332.0, 333.0...individuals (10.7%) had ICD-9 codes for other forms of neurodegenerative parkinsonism . The remaining 2.1% were primarily drug- induced parkinsonism

Survival of gynecological cancers in Turkey: where are we at?

PubMed Central

Dundar, Selin; Kucukyildiz, Irem; Karaca, Mujdegul Zayifoglu; Boztas, Guledal; Turan, Semra Hatice; Hacikamiloglu, Ezgi; Keskinkilic, Bekir

2017-01-01

Objective To investigate the 5-year relative survival rates in gynecological cancers diagnosed and treated in Turkey by year 2009 and to compare the results with developed countries. Methods Data of patients diagnosed for ovarian, corpus uteri or cervix uteri cancer at year 2009 are collected from 9 national cancer registry centers. Date of deaths are retracted from governmental Identity Information Sharing System (KPS). In order to calculate relative survival rates, national general population mortality tables are obtained from Turkish Statistical Institute (TurkStat). Hakulinen method is used for computing curves by R program. Data for European, Asian and some developed countries were obtained from official web pages. Results A total of 1,553 patients are evaluated. Among these, 713 (45.9%) are corpus uteri cancers, while remaining 489 (31.5%) are ovarian and 351 (22.6%) are cervix uteri. Five-year overall relative survival rates are 85%, 50%, and 62% for corpus uteri, ovarian, and cervix uteri, respectively. These figures are between 73%–87% for corpus uteri, 31%–62% for ovarian and 61%–80% for cervix uteri in developed countries. Stage is the most important factor for survival in all cancers. Five-year relative survival rates in corpus uteri cancers are 92%, 66%, and 38% for localized, regional, and distant metastatic disease, respectively. These figures are 77%, 57%, and 29% for ovarian; 80%, 50%, and 22% for cervix uteri. Conclusion This is the first report from Turkey giving national overall relative survival for gynecological cancers from a population based cancer registry system. PMID:29027403

Survival of gynecological cancers in Turkey: where are we at?

PubMed

Gultekin, Murat; Dundar, Selin; Kucukyildiz, Irem; Karaca, Mujdegul Zayifoglu; Boztas, Guledal; Turan, Semra Hatice; Hacikamiloglu, Ezgi; Keskinkilic, Bekir

2017-11-01

To investigate the 5-year relative survival rates in gynecological cancers diagnosed and treated in Turkey by year 2009 and to compare the results with developed countries. Data of patients diagnosed for ovarian, corpus uteri or cervix uteri cancer at year 2009 are collected from 9 national cancer registry centers. Date of deaths are retracted from governmental Identity Information Sharing System (KPS). In order to calculate relative survival rates, national general population mortality tables are obtained from Turkish Statistical Institute (TurkStat). Hakulinen method is used for computing curves by R program. Data for European, Asian and some developed countries were obtained from official web pages. A total of 1,553 patients are evaluated. Among these, 713 (45.9%) are corpus uteri cancers, while remaining 489 (31.5%) are ovarian and 351 (22.6%) are cervix uteri. Five-year overall relative survival rates are 85%, 50%, and 62% for corpus uteri, ovarian, and cervix uteri, respectively. These figures are between 73%-87% for corpus uteri, 31%-62% for ovarian and 61%-80% for cervix uteri in developed countries. Stage is the most important factor for survival in all cancers. Five-year relative survival rates in corpus uteri cancers are 92%, 66%, and 38% for localized, regional, and distant metastatic disease, respectively. These figures are 77%, 57%, and 29% for ovarian; 80%, 50%, and 22% for cervix uteri. This is the first report from Turkey giving national overall relative survival for gynecological cancers from a population based cancer registry system. Copyright © 2017. Asian Society of Gynecologic Oncology, Korean Society of Gynecologic Oncology

Trauma registry record linkage: methodological approach to benefit from complementary data using the example of the German Pelvic Injury Register and the TraumaRegister DGU®

PubMed Central

2013-01-01

Background In Germany, hospitals can deliver data from patients with pelvic fractures selectively or twofold to two different trauma registries, i.e. the German Pelvic Injury Register (PIR) and the TraumaRegister DGU® (TR). Both registers are anonymous and differ in composition and content. We describe the methodological approach of linking these registries and reidentifying twofold documented patients. The aim of the approach is to create an intersection set that benefit from complementary data of each registry, respectively. Furthermore, the concordance of data entry of some clinical variables entered in both registries was evaluated. Methods PIR (4,323 patients) and TR (34,134 patients) data from 2004-2009 were linked together by using a specific match code including code of the trauma department, dates of admission and discharge, patient’s age, and sex. Data entry concordance was evaluated using haemoglobin and blood pressure levels at emergency department arrival, Injury Severity Score (ISS), and mortality. Results Altogether, 420 patients were identified as documented in both data sets. Linkage rates for the intersection set were 15.7% for PIR and 44.4% for TR. Initial fluid management for different Tile/OTA types of pelvic ring fractures and the patient’s posttraumatic course, including intensive care unit data, were now available for the PIR population. TR is benefiting from clinical use of the Tile/OTA classification and from correlation with the distinct entity “complex pelvic injury.” Data entry verification showed high concordance for the ISS and mortality, whereas initial haemoglobin and blood pressure data showed significant differences, reflecting inconsistency at the data entry level. Conclusions Individually, the PIR and the TR reflect a valid source for documenting injured patients, although the data reflect the emphasis of the particular registry. Linking the two registries enabled new insights into care of multiple-trauma patients with pelvic fractures even when linkage rates were poor. Future considerations and development of the registries should be done in close bilateral consultation with the aim of benefiting from complementary data and improving data concordance. It is also conceivable to integrate individual modules, e.g. a pelvic fracture module, into the TR likewise a modular system in the future. PMID:23496832

Connecting communities to health research: development of the Project CONNECT minority research registry.

PubMed

Green, Melissa A; Kim, Mimi M; Barber, Sharrelle; Odulana, Abedowale A; Godley, Paul A; Howard, Daniel L; Corbie-Smith, Giselle M

2013-05-01

Prevention and treatment standards are based on evidence obtained in behavioral and clinical research. However, racial and ethnic minorities remain relatively absent from the science that develops these standards. While investigators have successfully recruited participants for individual studies using tailored recruitment methods, these strategies require considerable time and resources. Research registries, typically developed around a disease or condition, serve as a promising model for a targeted recruitment method to increase minority participation in health research. This study assessed the tailored recruitment methods used to populate a health research registry targeting African-American community members. We describe six recruitment methods applied between September 2004 and October 2008 to recruit members into a health research registry. Recruitment included direct (existing studies, public databases, community outreach) and indirect methods (radio, internet, and email) targeting the general population, local universities, and African American communities. We conducted retrospective analysis of the recruitment by method using descriptive statistics, frequencies, and chi-square statistics. During the recruitment period, 608 individuals enrolled in the research registry. The majority of enrollees were African American, female, and in good health. Direct and indirect methods were identified as successful strategies for subgroups. Findings suggest significant associations between recruitment methods and age, presence of existing health condition, prior research participation, and motivation to join the registry. A health research registry can be a successful tool to increase minority awareness of research opportunities. Multi-pronged recruitment approaches are needed to reach diverse subpopulations. Copyright © 2013. Published by Elsevier Inc.

Connecting Communities to Health Research: Development of the Project CONNECT Minority Research Registry

PubMed Central

Green, Melissa A.; Kim, Mimi M.; Barber, Sharrelle; Odulana, Abedowale A.; Godley, Paul A.; Howard, Daniel L.; Corbie-Smith, Giselle M.

2013-01-01

Introduction Prevention and treatment standards are based on evidence obtained in behavioral and clinical research. However, racial and ethnic minorities remain relatively absent from the science that develops these standards. While investigators have successfully recruited participants for individual studies using tailored recruitment methods, these strategies require considerable time and resources. Research registries, typically developed around a disease or condition, serve as a promising model for a targeted recruitment method to increase minority participation in health research. This study assessed the tailored recruitment methods used to populate a health research registry targeting African-American community members. Methods We describe six recruitment methods applied between September 2004 and October 2008 to recruit members into a health research registry. Recruitment included direct (existing studies, public databases, community outreach) and indirect methods (radio, internet, and email) targeting the general population, local universities, and African American communities. We conducted retrospective analysis of the recruitment by method using descriptive statistics, frequencies, and chi-square statistics. Results During the recruitment period, 608 individuals enrolled in the research registry. The majority of enrollees were African American, female, and in good health. Direct and indirect methods were identified as successful strategies for subgroups. Findings suggest significant associations between recruitment methods and age, presence of existing health condition, prior research participation, and motivation to join the registry. Conclusions A health research registry can be a successful tool to increase minority awareness of research opportunities. Multi-pronged recruitment approaches are needed to reach diverse subpopulations. PMID:23340183

Patient-reported outcome measures in arthroplasty registries

PubMed Central

Eresian Chenok, Kate; Bohm, Eric; Lübbeke, Anne; Denissen, Geke; Dunn, Jennifer; Lyman, Stephen; Franklin, Patricia; Dunbar, Michael; Overgaard, Søren; Garellick, Göran; Dawson, Jill

2016-01-01

The International Society of Arthroplasty Registries (ISAR) Steering Committee established the Patient-Reported Outcome Measures (PROMs) Working Group to convene, evaluate, and advise on best practices in the selection, administration, and interpretation of PROMs and to support the adoption and use of PROMs for hip and knee arthroplasty in registries worldwide. The 2 main types of PROMs include generic (general health) PROMs, which provide a measure of general health for any health state, and specific PROMs, which focus on specific symptoms, diseases, organs, body regions, or body functions. The establishment of a PROM instrument requires the fulfillment of methodological standards and rigorous testing to ensure that it is valid, reliable, responsive, and acceptable to the intended population. A survey of the 41 ISAR member registries showed that 8 registries administered a PROMs program that covered all elective hip or knee arthroplasty patients and 6 registries collected PROMs for sample populations; 1 other registry had planned but had not started collection of PROMs. The most common generic instruments used were the EuroQol 5 dimension health outcome survey (EQ-5D) and the Short Form 12 health survey (SF-12) or the similar Veterans RAND 12-item health survey (VR-12). The most common specific PROMs were the Hip disability and Osteoarthritis Outcome Score (HOOS), the Knee injury and Osteoarthritis Outcome Score (KOOS), the Oxford Hip Score (OHS), the Oxford Knee Score (OKS), the Western Ontario and McMaster Universities Arthritis Index (WOMAC), and the University of California at Los Angeles Activity Score (UCLA). PMID:27168175

Discrepancy in term calculation from second trimester ultrasound scan versus last menstrual period in women with type 1 diabetes.

PubMed

Eidem, Ingvild; Vangen, Siri; Henriksen, Tore; Vollset, Stein E; Hanssen, Kristian F; Joner, Geir; Stene, Lars C

2014-08-01

To study differences in ultrasound-based compared to menstrual-based term estimation in women with type 1 diabetes. Nationwide register study. Norway. Deliveries in Norway 1999-2004 by women registered in the Norwegian Childhood Diabetes Registry (n = 342) and the background population (n = 307 248), with data on both ultrasound-based and menstrual-based gestational age notified in the Birth Registry of Norway. Births with major malformations were excluded. Linkage of two nationwide registries, the Medical Birth Registry of Norway and the Norwegian Childhood Diabetes Registry. Estimated gestational age at delivery based on routine second trimester ultrasound measurements and last menstrual period. In women with type 1 diabetes, the distribution of gestational age at delivery was shifted considerably towards a lower gestational age when using second trimester ultrasound data for estimation, compared with last menstrual period data. The difference between the two estimation methods was larger among women with type 1 diabetes, although also evident in the general population. One in four women with diabetes and a certain last menstrual period date had their ultrasound-calculated term postponed 1 week or more, while one in 10 had it postponed 2 weeks or more. Corresponding numbers in the background population were one in five and one in 20. We found a systematic postponement of ultrasound-based compared with menstrual-based term estimation in women with type 1 diabetes. Relying solely on routine ultrasound-based term calculation for delivery decision may imply a risk of going beyond an optimal pregnancy length. © 2014 Nordic Federation of Societies of Obstetrics and Gynecology.

Upper Airway Stimulation for Obstructive Sleep Apnea: Results from the ADHERE Registry.

PubMed

Boon, Maurits; Huntley, Colin; Steffen, Armin; Maurer, Joachim T; Sommer, J Ulrich; Schwab, Richard; Thaler, Erica; Soose, Ryan; Chou, Courtney; Strollo, Patrick; Kezirian, Eric J; Chia, Stanley; Withrow, Kirk; Weidenbecher, Mark; Strohl, Kingman; Doghramji, Karl; Hofauer, Benedikt; Heiser, Clemens

2018-03-01

Objective Upper airway stimulation (UAS) is an alternative treatment option for patients unable to tolerate continuous positive airway pressure (CPAP) for the treatment of obstructive sleep apnea (OSA). Studies support the safety and efficacy of this therapy. The aim of this registry is to collect retrospective and prospective objective and subjective outcome measures across multiple institutions in the United States and Germany. To date, it represents the largest cohort of patients studied with this therapy. Study Design Retrospective and prospective registry study. Setting Ten tertiary care hospitals in the United States and Germany. Subjects and Methods Patients were included who had moderate to severe OSA, were intolerant to CPAP, and were undergoing UAS implantation. Baseline demographic and sleep study data were collected. Objective and subjective treatment outcomes, adverse events, and patient and physician satisfaction were reviewed. Results The registry enrolled 301 patients between October 2016 and September 2017. Mean ± SD AHI decreased from 35.6 ± 15.3 to 10.2 ± 12.9 events per hour ( P < .0001), and Epworth Sleepiness Scale scores decreased from 11.9 ± 5.5 to 7.5 ± 4.7 ( P < .0001) from baseline to the posttitration visit. Patients utilized therapy for 6.5 hours per night. There were low rates of procedure- and device-related complications. Clinical global impression scores demonstrated that the majority of physicians (94%) saw improvement in their patients' symptoms with therapy. The majority of patients (90%) were more satisfied with UAS than CPAP. Conclusions Across a multi-institutional registry, UAS therapy demonstrates significant improvement in subjective and objective OSA outcomes, good therapy adherence, and high patient satisfaction.

Clinical characteristics of patients with multiple sclerosis enrolled in a new registry in Egypt.

PubMed

Zakaria, Magd; Zamzam, Dina A; Abdel Hafeez, Mohamed A; Swelam, Mahmoud S; Khater, Shaimaa S; Fahmy, Mai F; Abdel Hady, Ayman; Fouad, Mohamed M; Abdel Nasser, Azza; Aref, Hany; Gadallah, Mohsen

2016-11-01

Epidemiological studies of multiple sclerosis (MS) are lacking in Egypt. To study the characteristics of Egyptian patients with multiple sclerosis in a new registry in a major tertiary referral centre in Cairo, Egypt. Patients were from the project MS database of the Multiple Sclerosis Unit at Ain Shams University Hospitals (N=950). We conducted a detailed medical history and examination including the Expanded Disability Status Scale (EDSS). Females represented 72% of subjects (female: male ratio 2.57:1). The mean age of disease onset was 26.1±7.6 years. Relapsing-remitting MS (RRMS) was the most common presentation (74.6%). Visual or sensory symptoms were the most common at presentation with RRMS, while motor symptoms were the most common presentation in other types of MS. Time to diagnosis was delayed up to 2 years in 27.8% of patients. The mean EDSS score was 3.6±2.1; 55% had EDSS≤3. About half (49%) received a disease-modifying drug. Progressive MS and motor presentation were associated with higher disability. This is the first documented MS registry from Egypt. The clinical characteristics of MS in Egypt was similar to other Arab countries and western countries. MS is more common among females in Egypt, with RRMS being the most common presentation. Visual symptoms and motor symptoms were the most common presentations in RRMS and progressive MS, respectively. Our findings also highlight the value of establishing registries in Egypt in order to be able to study, prospectively, the clinical course of the disease, the response to various DMD's and the epidemiology of MS in Egypt. Copyright © 2016 Elsevier B.V. All rights reserved.

The use of a national transplant registry to benchmark transplant outcome for patients undergoing autologous and allogeneic stem cell transplantation in the United Kingdom and Ireland.

PubMed

Russell, N H; Szydlo, R; McCann, S; Potter, M N; Craddock, C; Towlson, K; Apperley, J F

2004-02-01

As part of its clinical governance programme the British Society for Blood and Marrow Transplantation (BSBMT) undertook an analysis of transplant outcome for adults undergoing human leucocyte antigen - identical sibling allogeneic transplantation for chronic myeloid leukaemia (CML) in first chronic phase (CP1) or autologous transplantation for Hodgkin's disease (HD). The study aimed to compare transplant-related mortality (TRM) and survival for patients reported to the BSBMT with patients transplanted in the rest of Europe, reported to the European Group for Blood and Marrow Transplantation (EBMT). The outcomes for 104 allogeneic transplants for CML in 24 UK/Irish centres were compared with 775 allografts in 145 other European centres. For HD, 241 autografts from 38 UK/Irish centres were compared with 1145 transplants in 239 other European centres. For both diseases, the cohorts were broadly matched with the exception of CML, where 85% of patients were transplanted <1 year from diagnosis in the UK/Ireland compared with 68% in the EBMT (P = 0.001). Cox regression analysis was undertaken using known delineated variables affecting transplant outcome in addition to the registry of origin. The adjusted survival curves for CML showed no significant differences between the two groups, with 3-year survival probabilities of 70.2% and 67.1% for the EBMT and BSBMT cohorts respectively. Likewise, the analysis for HD showed overlapping survival curves, with 3-year survival probabilities of 71.8% (EBMT) and 70.8% (BSBMT). TRM was not statistically different in either disease. This study demonstrates the potential for using national registries to benchmark transplant outcome against the EBMT registry.

Epidemiology of severe trauma in Spain. Registry of trauma in the ICU (RETRAUCI). Pilot phase.

PubMed

Chico-Fernández, M; Llompart-Pou, J A; Guerrero-López, F; Sánchez-Casado, M; García-Sáez, I; Mayor-García, M D; Egea-Guerrero, J; Fernández-Ortega, J F; Bueno-González, A; González-Robledo, J; Servià-Goixart, L; Roldán-Ramírez, J; Ballesteros-Sanz, M Á; Tejerina-Alvarez, E; García-Fuentes, C; Alberdi-Odriozola, F

2016-01-01

To describe the characteristics and management of severe trauma disease in Spanish Intensive Care Units (ICUs). Registry of trauma in the ICU (RETRAUCI). Pilot phase. A prospective, multicenter registry. Thirteen Spanish ICUs. Patients with trauma disease admitted to the ICU. None. Epidemiology, out-of-hospital attention, registry of injuries, resources utilization, complications and outcome were evaluated. Patients, n=2242. Mean age 47.1±19.02 years. Males 79%. Blunt trauma 93.9%. Injury Severity Score 22.2±12.1, Revised Trauma Score 6.7±1.6. Non-intentional in 84.4% of the cases. The most common causes of trauma were traffic accidents followed by pedestrian and high-energy falls. Up to 12.4% were taking antiplatelet medication or anticoagulants. Almost 28% had a suspected or confirmed toxic influence in trauma. Up to 31.5% required an out-of-hospital artificial airway. The time from trauma to ICU admission was 4.7±5.3hours. At ICU admission, 68.5% were hemodynamically stable. Brain and chest injuries predominated. A large number of complications were documented. Mechanical ventilation was used in 69.5% of the patients (mean 8.2±9.9 days), of which 24.9% finally required a tracheostomy. The median duration of stay in the ICU and in hospital was 5 (range 3-13) and 9 (5-19) days, respectively. The ICU mortality rate was 12.3%, while the in-hospital mortality rate was 16.0%. The pilot phase of the RETRAUCI offers a first impression of the epidemiology and management of trauma disease in Spanish ICUs. Copyright © 2015 Elsevier España, S.L.U. y SEMICYUC. All rights reserved.

Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland.

PubMed

Ling, Yi; Johnson, Martin K; Kiely, David G; Condliffe, Robin; Elliot, Charlie A; Gibbs, J Simon R; Howard, Luke S; Pepke-Zaba, Joanna; Sheares, Karen K K; Corris, Paul A; Fisher, Andrew J; Lordan, James L; Gaine, Sean; Coghlan, J Gerry; Wort, S John; Gatzoulis, Michael A; Peacock, Andrew J

2012-10-15

Incident pulmonary arterial hypertension was underrepresented in most pulmonary hypertension registries and may have a different disease profile to prevalent disease. To determine the characteristics and outcome of a purely incident, treatment-naive cohort of idiopathic, heritable, and anorexigen-associated pulmonary arterial hypertension and to determine the changes in presentations and survival over the past decade in the United Kingdom and Ireland. All consecutive newly diagnosed patients from 2001 to 2009 were identified prospectively. A total of 482 patients (93% idiopathic, 5% heritable, and 2% anorexigen-associated pulmonary arterial hypertension) were diagnosed, giving rise to an estimated incidence of 1.1 cases per million per year and prevalence of 6.6 cases per million in 2009. Younger patients (age ≤ 50 yrs) had shorter duration of symptoms, fewer comorbidities, better functional and exercise capacity, higher percent diffusing capacity of carbon monoxide, more severe hemodynamic impairment, but better survival compared with older patients. In comparison with the earlier cohorts, patients diagnosed in 2007-2009 were older, more obese, had lower percent diffusing capacity of carbon monoxide,(,) and more comorbidities, but better survival. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) equation, REVEAL risk score, and Pulmonary Hypertension Connection Registry survival equation accurately predicted survival of our incident cohort at 1 year. This study highlights the influence of age on phenotypes of incident pulmonary arterial hypertension and has shown the changes in demographics and epidemiology over the past decade in a national setting. The results suggest that there may be two subtypes of patients: the younger subtype with more severe hemodynamic impairment but better survival, compared with the older subtype who has more comorbidities.

Pilot study for the registry of complications in rheumatic diseases from the German Society of Surgery (DGORh): evaluation of methods and data from the first 1000 patients

PubMed Central

Kostuj, Tanja; Rehart, Stefan; Matta-Hurtado, Ronald; Biehl, Christoph; Willburger, Roland E; Schmidt, Klaus

2017-01-01

Objective Most patients suffering with rheumatic diseases who undergo surgical treatment are receiving immune-modulating therapy. To determine whether these medications affect their outcomes a national registry was established in Germany by the German Society of Surgery (DGORh). Data from the first 1000 patients were used in a pilot study to identify relevant corisk factors and to determine whether such a registry is suitable for developing accurate and relevant recommendations. Design and participants Data were collected from patients undergoing surgical treatments with their written consent. A second consent form was used, if complications occurred. During this pilot study, in order to obtain a quicker overview, risk factors were considered only in patients with complications. Only descriptive statistical analysis was employed in this pilot study due to limited number of observed complications and inhomogeneous data regarding the surgery and the medications the patients received. Analytical statistics will be performed to confirm the results in a future outcome study. Results Complications occurred in 26 patients and were distributed equally among the different types of surgeries. Twenty one of these patients were receiving immune-modulating therapy at the time, while five were not. Infections were observed in 2.3% of patients receiving and in 5.1% not receiving immunosuppression. Conclusions Due to the low number of cases, inhomogeneity in the diseases and the treatments received by the patients in this pilot study, it is not possible to develop standardised best-practice recommendations to optimise their care. Based on this observation we conclude that in order to be suitable to develop accurate and relevant recommendations a national registry must include the most important and relevant variables that impact the care and outcomes of these patients. PMID:29018066

Inflammatory bowel disease in pediatric patients: Characteristics of newly diagnosed patients from the CEDATA-GPGE Registry.

PubMed

Buderus, Stephan; Scholz, Dietmar; Behrens, Rolf; Classen, Martin; De Laffolie, Jan; Keller, Klaus-Michael; Zimmer, Klaus-Peter; Koletzko, Sibylle

2015-02-20

Inflammatory bowel disease (IBD) can arise at any age, with peak incidence in adolescence and young adulthood. A registry of pediatric cases of IBD offers the opportunity to document their diagnosis and treatment, with the ultimate aim of improving diagnosis and treatment in the future. In the German-language CEDATA-GPGE registry, 3991 cases of IBD in patients less than 18 years of age were documented from 2004 to 2014. The 1257 patients who were prospectively included in the registry upon diagnosis and whose further course was documented for at least three months were analyzed in two separate groups--under 10 years old, and 10 years and above--with respect to the type and duration of their symptoms until diagnosis, the completeness of the diagnostic evaluation, the disease phenotype, and the initial treatment. Of the 958 patients for whom full documentation was available, 616 (64.3%) had Crohn's disease (CD), 278 (29%) had ulcerative colitis (UC), 64 (6.7%) had an unclassified IBD, and 23.2% were under 10 years old. The latency to diagnosis was longer for CD than for UC (0.5 versus 0.3 years), regardless of age. 62.5% of the CD patients had ileocolonic involvement, and more than half had involvement of the upper gastrointestinal tract. 71% of the patients with UC had subtotal colitis or pancolitis. Continuous improvement was seen in diagnostic assessment according to published guidelines. For example, in 2004/2005, 69% of patients were evaluated endoscopically with ileocolonoscopy and esophagogastroduodenoscopy; this fraction had risen to nearly 100% by 2013/2014. Similarly, the percentage of patients who underwent a diagnostic evaluation of the small intestine, as recommended, rose from 41.2% to 60.9% over the same period. The most common initial treatments were 5- amino - salicylates (86.8% CD, 100% UC) and glucocorticoids (60.6% CD, 65.6% UC). 32% of the patients with CD received exclusive enteral nutrition therapy. Most of these pediatric patients with IBD, whether in the younger or the older age group, had extensive bowel involvement at the time of diagnosis. The registry data imply that improvement in clinical course may be achieved by shortening the time to diagnosis and by closer adherence to the diagnostic and therapeutic guidelines.

A Complex Contraception Registry

ClinicalTrials.gov

2018-03-13

Diabetes; Cardiovascular Disease; Epilepsy; Migraine; Neurological Disorders; Cancer; Bariatric Surgery Candidate; Organ or Tissue Transplant; Complications; Lupus Erythematosus, Systemic; Other Hematologic Conditions; Other Venous Embolism and Thrombosis

European Home Mechanical Ventilation Registry

ClinicalTrials.gov

2016-10-25

Pulmonary Disease, Chronic Obstructive; Amyotrophic Lateral Sclerosis; Spinal Cord Injury; Muscular Dystrophies; Obesity Hypoventilation Syndrome; Kyphoscoliosis; Congenital Central Hypoventilation Syndrome; Duchenne Muscular Dystrophy; Myopathies; Myotonic Dystrophy

The PTSD Practitioner Registry: An Innovative Tracking, Dissemination, and Support Tool for Providers in Military and Nonmilitary Settings

DTIC Science & Technology

2016-10-01

Watertown, MA 02472 REPORT DATE: October 2016 TYPE OF REPORT: Annual PREPARED FOR: U.S. Army Medical Research and Materiel Command Fort Detrick...Rebekah Zincavage Project Role: Qualitative Researcher Nearest person month worked: 1 Contribution to Project: Ms. Zincavage developed the...Military and Nonmilitary Settings PRINCIPAL INVESTIGATOR: Raymond C. Rosen, PhD CONTRACTING ORGANIZATION: New Englad Research Instituites, Inc

Utility of routine data sources for feedback on the quality of cancer care: an assessment based on clinical practice guidelines.

PubMed

Coory, Michael; Thompson, Bridie; Baade, Peter; Fritschi, Lin

2009-05-27

Not all cancer patients receive state-of-the-art care and providing regular feedback to clinicians might reduce this problem. The purpose of this study was to assess the utility of various data sources in providing feedback on the quality of cancer care. Published clinical practice guidelines were used to obtain a list of processes-of-care of interest to clinicians. These were assigned to one of four data categories according to their availability and the marginal cost of using them for feedback. Only 8 (3%) of 243 processes-of-care could be measured using population-based registry or administrative inpatient data (lowest cost). A further 119 (49%) could be measured using a core clinical registry, which contains information on important prognostic factors (e.g., clinical stage, physiological reserve, hormone-receptor status). Another 88 (36%) required an expanded clinical registry or medical record review; mainly because they concerned long-term management of disease progression (recurrences and metastases) and 28 (11.5%) required patient interview or audio-taping of consultations because they involved information sharing between clinician and patient. The advantages of population-based cancer registries and administrative inpatient data are wide coverage and low cost. The disadvantage is that they currently contain information on only a few processes-of-care. In most jurisdictions, clinical cancer registries, which can be used to report on many more processes-of-care, do not cover smaller hospitals. If we are to provide feedback about all patients, not just those in larger academic hospitals with the most developed data systems, then we need to develop sustainable population-based data systems that capture information on prognostic factors at the time of initial diagnosis and information on management of disease progression.

A Population-Based Study on Myelodysplastic Syndromes in the Lazio Region (Italy), Medical Miscoding and 11-Year Mortality Follow-Up: the Gruppo Romano-Laziale Mielodisplasie Experience of Retrospective Multicentric Registry.

PubMed

Mayer, Flavia; Faglioni, Laura; Agabiti, Nera; Fenu, Susanna; Buccisano, Francesco; Latagliata, Roberto; Ricci, Roberto; Spiriti, Maria Antonietta Aloe; Tatarelli, Caterina; Breccia, Massimo; Cimino, Giuseppe; Fianchi, Luana; Criscuolo, Marianna; Gumenyuk, Svitlana; Mancini, Stefano; Maurillo, Luca; Nobile, Carolina; Niscola, Pasquale; Piccioni, Anna Lina; Tafuri, Agostino; Trapè, Giulio; Andriani, Alessandro; De Fabritiis, Paolo; Voso, Maria Teresa; Davoli, Marina; Zini, Gina

2017-01-01

Data on Myelodysplastic Syndromes (MDS) are difficult to collect by cancer registries because of the lack of reporting and the use of different classifications of the disease. In the Lazio Region, data from patients with a confirmed diagnosis of MDS, treated by a hematology center, have been collected since 2002 by the Gruppo Romano-Laziale Mielodisplasie (GROM-L) registry, the second MDS registry existing in Italy. This study aimed at evaluating MDS medical miscoding during hospitalizations, and patients' survival. For these purposes, we selected 644 MDS patients enrolled in the GROM-L registry. This cohort was linked with two regional health information systems: the Hospital Information System (HIS) and the Mortality Information System (MIS) in the 2002-2012 period. Of the 442 patients who were hospitalized at least once during the study period, 92% had up to 12 hospitalizations. 28.5% of patients had no hospitalization episodes scored like MDS, code 238.7 of the International Classification of Disease, Ninth Revision, Clinical Modification (ICD-9-CM). The rate of death during a median follow-up of 46 months (range 0.9-130) was 45.5%. Acute myeloid leukemia (AML) was the first cause of mortality, interestingly a relevant portion of deaths is due to cerebro-cardiovascular events and second tumors. This study highlights that MDS diagnosis and treatment, which require considerable healthcare resources, tend to be under-documented in the HIS archive. Thus we need to improve the HIS to better identify information on MDS hospitalizations and outcome. Moreover, we underline the importance of comorbidity in MDS patients' survival.

Implant and clinical characteristics for pediatric and congenital heart patients in the national cardiovascular data registry implantable cardioverter defibrillator registry.

PubMed

Jordan, Christopher P; Freedenberg, Vicki; Wang, Yongfei; Curtis, Jeptha P; Gleva, Marye J; Berul, Charles I

2014-12-01

In 2010, the National Cardiovascular Data Registry enhanced pediatric, nonatherosclerotic structural heart disease and congenital heart disease (CHD) data collection. This report characterizes CHD and pediatric patients undergoing implantable cardioverter defibrillator implantation. In this article, we report implantable cardioverter defibrillator procedures (April 2010 to December 2012) in the registry for 2 cohorts: (1) all patients with CHD (atrial septal defect, ventricular septal defect, tetralogy of Fallot, Ebstein anomaly, transposition of the great vessels, and common ventricle) and (2) patients <21 years. We evaluated indications and characteristics to include transvenous and nontransvenous lead implants, CHD type, and New York Heart Association class. There were 3139 CHD procedures, 1601 for patients <21 years and 126 for CHD <21 years. Implantable cardioverter defibrillator indications for patients with CHD were primary prevention in 1943 (61.9%) and secondary prevention in 1107 (35.2%). Pediatric patients had 935 (58.4%) primary prevention and 588 (36.7%) secondary prevention devices. Primary prevention had higher New York Heart Association class. Nontransvenous age (35.9 ± 23.2 versus 40.1 ± 24.6 years; P=0.05) and nontransvenous height (167.1 ± 18.9 cm; range, 53-193 cm versus 170.4 ± 13.1 cm; range, 61-203 cm; P<0.01) were lower than for transvenous patients. CHD and pediatrics had similar rates of transvenous (97%) and nontransvenous (3%) leads and did not differ from the overall registry. Transposition of the great vessels and common ventricle had higher rates of nontransvenous leads. Primary prevention exceeds secondary prevention for CHD and pediatrics. Nontransvenous lead patients were younger, with higher rates of transposition of the great vessels and common ventricle patients compared with transvenous lead patients. © 2014 American Heart Association, Inc.

Persian Registry Of cardioVascular diseasE (PROVE): Design and methodology.

PubMed

Givi, Mahshid; Sarrafzadegan, Nizal; Garakyaraghi, Mohammad; Yadegarfar, Ghasem; Sadeghi, Masoumeh; Khosravi, Alireza; Azhari, Amir Hossein; Samienasab, Mohammad Reza; Shafie, Davood; Saadatnia, Mohammad; Roohafza, Hamidreza; Paydari, Navid; Soleimani, Azam; Hosseinzadeh, Mohsen; Ahmadi, Seyed Abdulah; Dehghani, Leila; Najafian, Jamshid; Andalib, Elham; Shahabi, Javad; Sabri, Mohammad Reza

2017-09-01

Our aim was to create and establish a database called "Persian Registry Of cardioVascular diseasE (PROVE)" in order to be used for future research and in addition, as a tool to develop national guidelines for diagnosis, treatment, and prevention of cardiovascular disease (CVD). In this paper, the design and methodology of the PROVE pilot study will be discussed, launched in Isfahan, Iran, in 2015-2016. Through establishing PROVE, patients' data were collected from hospitals and outpatient clinics prospectively or retrospectively and followed up for a maximum of three years based on the type of CVDs. The inclusion criteria were as patients with acute coronary syndrome (ACS), ST elevation myocardial infarction (STEMI), stroke, atrial fibrillation (AF), heart failure (HF), congenital heart disease (CHD), percutaneous coronary intervention (PCI), and chronic ischemic cardiovascular disease (CICD). Specific protocols, questionnaires, and glossaries were developed for each registry. In order to ensure the validation of the protocols, questionnaires, data collection, management, and analysis, a well-established quality control (QC) protocol was developed and implemented. Data confidentiality was considered. In order to register patients with ACS, STEMI, stroke, HF, PCI, and CICD, the hospital recorded data were used, whereas, in case of AF and CHD registries, the data were collected from hospitals and outpatient clinics. During the pilot phase of the study in Isfahan, from March 2015 to September 2016, 9427 patients were registered as ACS including 809 as STEMI, 1195 patients with HF, 363 with AF, 761 with stroke, 1136 with CHD, 1200 with PCI, and 9 with CICD. Data collection and management were performed under the supervision of the QC group. PROVE was developed and implemented in Isfahan as a pilot study, in order to be implemented at national level in future. It provides a valuable source of valid data that could be used for future research, re-evaluation of current CVD management and more specifically, gap analysis and as a tool for assessment of the type of CVDs, prevention, treatment, and control by health care decision makers.

[Hansen's disease in the northern region of Brazil--1986].

PubMed

Britto, R da S

1989-12-01

Some general facts about Hansen's Disease are presented: in the world, in the Americas, in 1986, with the purpose to focalize on the Northern Region of Brazil (Amazon Valley) where an attempt is made to specify, State, the clinical forms in the active registry, the respective coefficients of Prevalence, about the cases detected during the year, by clinical forms and the correspondent Coefficient of Incidence, the age range of less than 15 years, and above 15 years; and to analyze according to the township in the State of Amazonas in a series of 8 years, from 1979 to 1986, the registered cases under control and also without control, the new cases, by clinical form and Coefficient of Incidence, the positive cases among students in the capital and also in the country, and with more details, still by township and by Public Health Registry, in 1986, with the population, new cases, and Coefficient of Incidence, cases of the active registry, cases under control, Coefficient of Incidence, the relationship between patients/inhabitants, cases according to the decreasing intensity as far as the Prevalence and Health Department Regions: all this to give an idea, in detail, inclusive statistics, of the real position of Hansen's Disease in the Northern Region (Amazon Valley), with the highest figures of Prevalence and even of Incidence, in comparison with other Regions of the country, and to stress the grave situation of the endemic proportions of the disease, in Brazil in general and in that Regions, in particular.

The FOP Connection Registry: Design of an international patient-sponsored registry for Fibrodysplasia Ossificans Progressiva.

PubMed

Mantick, Neal; Bachman, Eric; Baujat, Genevieve; Brown, Matt; Collins, Oliver; De Cunto, Carmen; Delai, Patricia; Eekhoff, Marelise; Zum Felde, Roger; Grogan, Donna Roy; Haga, Nobuhiko; Hsiao, Edward; Kantanie, Sharon; Kaplan, Frederick; Keen, Richard; Milosevic, Jelena; Morhart, Rolf; Pignolo, Robert; Qian, Xiaobing; di Rocco, Maja; Scott, Christiaan; Sherman, Adam; Wallace, Marin; Williams, Nicky; Zhang, Keqin; Bogard, Betsy

2018-04-01

The Fibrodysplasia Ossificans Progressiva (FOP) Connection Registry is an international, voluntary, observational study that directly captures demographic and disease information initially from patients with FOP (the patient portal) and in the near future from treating physicians (the physician portal) via a secure web-based tool. It was launched by the International FOP Association (IFOPA) with a guiding vision to develop and manage one unified, global, and coordinated Registry allowing the assembly of the most comprehensive data on FOP. This will ultimately facilitate greater access and sharing of patient data and enable better and faster development of therapies and tracking their long-term treatment effectiveness and safety. This report outlines the FOP Connection Registry's design and procedures for data collection and reporting, as well as the long-term sustainability of Registry. Patient-reported, aggregate data are summarized for the first 196 enrolled patients, representing participation from 42 countries and approximately 25% of the world's known FOP population. Fifty-seven percent of the current Registry participants are female with a mean age of 23.8years (median=21years, range=1, 76years). Among the Registry participants who provided their FOP type, 51% reported FOP Classic (R206H), 41% reported FOP Type Unknown, and 8% reported FOP Variant. Patients reported 5.4years (median=3.0years, range=0, 45.8years) as the mean age at which they noticed their first FOP symptoms and a mean age at final FOP diagnosis of 7.5years (median=5.0years, range=0.1, 48.4years). Information on the patients' diagnostic journeys in arriving at a correct diagnosis of FOP is also presented. These early patient-reported data suggest that the IFOPA's vision of one, unified, global, and coordinated approach to the FOP Connection Registry is well underway to being realized. In addition, the positive response from the FOP patient community to the initial launch of the Registry's patient portal has created a solid foundation upon which to build the largest international registry for monitoring the clinical progression of FOP among patients. Copyright © 2017 Elsevier Inc. All rights reserved.

H.R. 2572: A Bill to amend the Comprehensive Environmental Response, Compensation, and Liability Act of 1980 to establish citizens advisory boards for Department of Energy defense nuclear facilities and to require the Administrator of the Agency for Toxic Substances and Disease Registry to conduct public health and exposure assessments of such facilities. Introduced in the House of Representatives, One Hundred Third Congress, First Session, June 30, 1993

DOE Office of Scientific and Technical Information (OSTI.GOV)

NONE

The report H.R. 2572 is a bill to amend the Comprehensive Environmental Response, Compensation, and Liabilities Act of 1980 to establish citizen advisory boards for Department of Energy defense nuclear facilities and to require the Administrator of the Agency for Toxic Substances and Disease Registry to conduct public health and exposure assessments of such facilities. The proposed legislative text is included.

The association of sociodemographic and objectively-assessed disease variables with fatigue in systemic sclerosis: an analysis of 785 Canadian Scleroderma Research Group Registry patients.

PubMed

Levis, Brooke; Kwakkenbos, Linda; Hudson, Marie; Baron, Murray; Thombs, Brett D

2017-02-01

Fatigue is prevalent among patients with systemic sclerosis (SSc). To date, studies investigating fatigue in SSc have been hampered by the instruments used to measure fatigue in SSc and have included patient-reported rather than objectively-rated measures of disease. The Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F) scale is a validated measure for assessing fatigue in SSc that, compared to other instruments, provides good coverage of the full range of the fatigue spectrum. The objective of this study was to assess sociodemographic and objectively-rated disease-related associates of fatigue, as measured by the FACIT-F, in a large sample of patients with SSc. Fatigue was assessed using the FACIT-F scale. Disease severity was assessed using Medsger's severity scale. Multivariable linear regression was performed to assess the independent associations between sociodemographic and medical variables and fatigue. Among 785 patients, the mean FACIT-F score was 32.2 (SD = 12.1). Being age 40-49 (reference = 60+; standardized regression coefficient (β) = -0.11), less than post-secondary education (β = 0.07), having more medical comorbidities (β = -0.11) and more severe muscle (β = -0.10), gastrointestinal (β = -0.15), lung (β = -0.13), and general system disease severity (β = -0.13) were independently associated with more fatigue (p < 0.05). Fatigue in SSc was independently associated with more severe disease. These data contribute to a better understanding of fatigue in SSc and help inform patient-centered research in SSc.

Genomics of Fibromuscular Dysplasia.

PubMed

Di Monaco, Silvia; Georges, Adrien; Lengelé, Jean-Philippe; Vikkula, Miikka; Persu, Alexandre

2018-05-21

Fibromuscular Dysplasia (FMD) is “an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries” (Persu, et al; 2014). FMD can lead to hypertension, arterial dissections, subarachnoid haemorrhage, stroke or mesenteric ischemia. The pathophysiology of the disease remains elusive. While familial cases are rare (<5%) in contemporary FMD registries, there is evidence in favour of the existence of multiple genetic factors involved in this vascular disease. Recent collaborative efforts allowed the identification of a first genetic locus associated with FMD. This intronic variant located in the phosphatase and actin regulator 1 gene ( PHACTR1 ) may influence the transcription activity of the endothelin-1 gene ( EDN1 ) located nearby on chromosome 6. Interestingly, the PHACTR1 locus has also been involved in vascular hypertrophy in normal subjects, carotid dissection, migraine and coronary artery disease. National and international registries of FMD patients, with deep and harmonised phenotypic and genetic characterisation, are expected to be instrumental to improve our understanding of the genetic basis and pathophysiology of this intriguing vascular disease.

TREatment of ATopic eczema (TREAT) Registry Taskforce: protocol for an international Delphi exercise to identify a core set of domains and domain items for national atopic eczema registries.

PubMed

Gerbens, Louise A A; Boyce, Aaron E; Wall, Dmitri; Barbarot, Sebastien; de Booij, Richard J; Deleuran, Mette; Middelkamp-Hup, Maritza A; Roberts, Amanda; Vestergaard, Christian; Weidinger, Stephan; Apfelbacher, Christian J; Irvine, Alan D; Schmitt, Jochen; Williamson, Paula R; Spuls, Phyllis I; Flohr, Carsten

2017-02-27

Patients with moderate-to-severe atopic eczema (AE) often require photo- or systemic immunomodulatory therapies to induce disease remission and maintain long-term control. The current evidence to guide clinical management is small, despite the frequent and often off-label use of these treatments. Registries of patients on photo- and systemic immunomodulatory therapies could fill this gap, and the collection of a core set concerning these therapies in AE will allow direct comparisons across registries as well as data sharing and pooling. Using an eDelphi approach, the international TREatment of ATopic eczema (TREAT) Registry Taskforce aims to seek consensus between key stakeholders internationally on a core set of domains and domain items for AE patient registries with a research focus that collect data of children and adults on photo- and systemic immunomodulatory therapies. Participants from six stakeholder groups will be invited: doctors, nurses, non-clinical researchers, patients, as well as industry and regulatory body representatives. The eDelphi will comprise three sequential online rounds, requesting participants to rate the importance of each proposed domain and domain items. Participants will be able to add domains and domain items to the proposed list in round 1. A final consensus meeting will be held with representatives of each stakeholder group. Identifying a uniform core set of domains and domain items to be captured by AE patient registries will increase the utility of individual registries, and provide greater insight into the effectiveness, safety and cost-effectiveness of photo- and systemic immunomodulatory therapies to guide clinical management across dermatology centres and country borders. Not applicable. This eDelphi study was registered in the Core Outcome Measures for Effectiveness Trials (COMET) database.

Renal replacement therapy in Europe: a summary of the 2013 ERA-EDTA Registry Annual Report with a focus on diabetes mellitus

PubMed Central

Kramer, Anneke; Pippias, Maria; Stel, Vianda S.; Bonthuis, Marjolein; Abad Diez, José Maria; Afentakis, Nikolaos; Alonso de la Torre, Ramón; Ambuhl, Patrice; Bikbov, Boris; Bouzas Caamaño, Encarnación; Bubic, Ivan; Buturovic-Ponikvar, Jadranka; Caskey, Fergus J.; Castro de la Nuez, Pablo; Cernevskis, Harijs; Collart, Frederic; Comas Farnés, Jordi; Garcia Bazaga, Maria de los Ángeles; De Meester, Johan; Ferrer Alamar, Manuel; Finne, Patrik; Garneata, Liliana; Golan, Eliezer; G. Heaf, James; Hemmelder, Marc; Ioannou, Kyriakos; Kantaria, Nino; Kolesnyk, Mykola; Kramar, Reinhard; Lassalle, Mathilde; Lezaic, Visnja; Lopot, Frantisek; Macário, Fernando; Magaz, Angela; Martín-Escobar, Eduardo; Metcalfe, Wendy; Ots-Rosenberg, Mai; Palsson, Runolfur; Piñera Celestino, Celestino; Resić, Halima; Rutkowski, Boleslaw; Santiuste de Pablos, Carmen; Spustová, Viera; Stendahl, Maria; Strakosha, Ariana; Süleymanlar, Gültekin; Torres Guinea, Marta; Varberg Reisæter, Anna; Vazelov, Evgueniy; Ziginskiene, Edita; Massy, Ziad A.; Wanner, Christoph; Jager, Kitty J.; Noordzij, Marlies

2016-01-01

Background This article provides a summary of the 2013 European Renal Association–European Dialysis and Transplant Association (ERA-EDTA) Registry Annual Report (available at http://www.era-edta-reg.org), with a focus on patients with diabetes mellitus (DM) as the cause of end-stage renal disease (ESRD). Methods In 2015, the ERA-EDTA Registry received data on renal replacement therapy (RRT) for ESRD from 49 national or regional renal registries in 34 countries in Europe and bordering the Mediterranean Sea. Individual patient data were provided by 31 registries, while 18 registries provided aggregated data. The total population covered by the participating registries comprised 650 million people. Results In total, 72 933 patients started RRT for ESRD within the countries and regions reporting to the ERA-EDTA Registry, resulting in an overall incidence of 112 per million population (pmp). The overall prevalence on 31 December 2013 was 738 pmp (n = 478 990). Patients with DM as the cause of ESRD comprised 24% of the incident RRT patients (26 pmp) and 17% of the prevalent RRT patients (122 pmp). When compared with the USA, the incidence of patients starting RRT pmp secondary to DM in Europe was five times lower and the incidence of RRT due to other causes of ESRD was two times lower. Overall, 19 426 kidney transplants were performed (30 pmp). The 5-year adjusted survival for all RRT patients was 60.9% [95% confidence interval (CI) 60.5–61.3] and 50.6% (95% CI 49.9–51.2) for patients with DM as the cause of ESRD. PMID:27274834

Rationale and design of a large registry on renal denervation: the Global SYMPLICITY registry.

PubMed

Böhm, Michael; Mahfoud, Felix; Ukena, Christian; Bauer, Axel; Fleck, Eckart; Hoppe, Uta C; Kintscher, Ulrich; Narkiewicz, Krzysztof; Negoita, Manuela; Ruilope, Luis; Rump, L Christian; Schlaich, Markus; Schmieder, Roland; Sievert, Horst; Weil, Joachim; Williams, Bryan; Zeymer, Uwe; Mancia, Giuseppe

2013-08-22

Hypertension is a global healthcare concern associated with a wide range of comorbidities. The recognition that elevated sympathetic drive plays an important role in the pathogenesis of hypertension led to the use of renal artery denervation to interrupt the efferent and afferent sympathetic nerves between the brain and kidneys to lower blood pressure. Clinical trials of the Symplicity™ renal denervation system have demonstrated that radiofrequency ablation of renal artery nerves is safe and significantly lowers blood pressure in patients with severe resistant (systolic BP >160 mmHg) hypertension. Smaller ancillary studies in hypertensive patients suggest a benefit from renal denervation in a variety of conditions such as chronic kidney disease, glucose intolerance, sleep apnoea and heart failure. The Global SYMPLICITY registry, which incorporates the GREAT SYMPLICITY registry initiated in Germany, is being conducted worldwide to evaluate the safety and efficacy of treatment with the Symplicity renal denervation system in real-world uncontrolled hypertensive patients, looking first at subjects with severe resistant hypertension to confirm the results of prior clinical trials, but then also subjects with a wider range of baseline blood pressure and coexisting comorbidities. The rationale, design and first baseline data from the Global SYMPLICITY registry are presented.

Registry of Youth Onset Diabetes in India (YDR): Rationale, Recruitment, and Current Status.

PubMed

Praveen, Pradeep A; Madhu, Sri Venkata; Mohan, Viswanathan; Das, Siddhartha; Kakati, Sanjeeb; Shah, Nalini; Chaddha, Manoj; Bhadada, Sanjay Kumar; Das, Ashok Kumar; Shukla, Deepak Kumar; Kaur, Tanvir; Tandon, Nikhil

2016-09-01

With the aim of addressing the relative scarcity of information on youth-onset diabetes in India, the Indian Council of Medical Research (ICMR) decided to establish the Registry of People with Diabetes with Young Age at Onset (YDR) in 2006. The major objectives of YDR are to generate information on disease pattern or types of youth-onset diabetes including their geographical variations within India and to estimate the burden of diabetes complications. YDR is an observational multicenter clinic based registry enlisting physician diagnosed diabetes in individuals below 25 years of age. Diabetes was classified using symptom based clinical criteria. YDR data collection is coordinated through regional collaborating centers and their interacting reporting centers across India. A baseline and an annual follow-up proformas are used to obtain information on sociodemographic details, clinical profile, and anthropometric and laboratory measurements of the patients. In phase 1, the registry has enrolled 5546 patients, in which type 1 diabetes mellitus (T1DM) was the most prevalent (63.9%), followed by youth-onset type 2 diabetes mellitus (T2DM) (25.3%). This registry provides a unique opportunity to study the natural history of youth-onset diabetes in India. © 2016 Diabetes Technology Society.

75 FR 53705 - Request for Nominations of Candidates To Serve on the Board of Scientific Counselors (BSC...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-09-01

... for Environmental Health/ Agency for Toxic Substances and Disease Registry (NCEH/ATSDR), Centers for... human diseases and disabilities caused by environmental conditions. Experts in the disciplines of toxicology, epidemiology, environmental or occupational medicine, behavioral science, risk assessment...

Usefulness of a centralized system of data collection for the development of an international multicentre registry of spondyloarthritis

PubMed Central

Schiotis, Ruxandra; Font, Pilar; Zarco, Pedro; Almodovar, Raquel; Gratacós, Jordi; Mulero, Juan; Juanola, Xavier; Montilla, Carlos; Moreno, Estefanía; Ariza Ariza, Rafael; Collantes-Estevez, Eduardo

2011-01-01

Objective. To present the usefulness of a centralized system of data collection for the development of an international multicentre registry of SpA. Method. The originality of this registry consists in the creation of a virtual network of researchers in a computerized Internet database. From its conception, the registry was meant to be a dynamic acquiring system. Results. REGISPONSER has two developing phases (Conception and Universalization) and gathers several evolving secondary projects (REGISPONSER-EARLY, REGISPONSER-AS, ESPERANZA and RESPONDIA). Each sub-project answered the necessity of having more specific and complete data of the patients even from the onset of the disease so, in the end, obtaining a well-defined picture of SpAs spectrum in the Spanish population. Conclusion. REGISPONSER is the first dynamic SpA database composed of cohorts with a significant number of patients distributed by specific diagnosis, which provides basic specific information of the sub-cohorts useful for patients’ evaluation in rheumatology ambulatory consulting. PMID:20823095

The Brain Health Registry: An internet-based platform for recruitment, assessment, and longitudinal monitoring of participants for neuroscience studies.

PubMed

Weiner, Michael W; Nosheny, Rachel; Camacho, Monica; Truran-Sacrey, Diana; Mackin, R Scott; Flenniken, Derek; Ulbricht, Aaron; Insel, Philip; Finley, Shannon; Fockler, Juliet; Veitch, Dallas

2018-05-08

Recruitment, assessment, and longitudinal monitoring of participants for neuroscience studies and clinical trials limit the development of new treatments. Widespread Internet use allows data capture from participants in an unsupervised setting. The Brain Health Registry, a website and online registry, collects data from participants and their study partners. The Brain Health Registry obtains self and study partner report questionnaires and neuropsychological data, including the Cogstate Brief Battery, Lumos Labs Neurocognitive Performance Test, and MemTrax Memory Test. Participants provide informed consent before participation. Baseline and longitudinal data were obtained from nearly 57,000 and 28,000 participants, respectively. Over 18,800 participants were referred to, and nearly 1800 were enrolled in, clinical Alzheimer's disease and aging studies, including five observational studies and seven intervention trials. Online assessments of participants and study partners provide useful information at relatively low cost for neuroscience studies and clinical trials and may ultimately be used in routine clinical practice. Copyright © 2018 the Alzheimer's Association. All rights reserved.

An Electronic Health Record - Public Health (EHR-PH) System Prototype for Interoperability in 21st Century Healthcare Systems

PubMed Central

Orlova, Anna O.; Dunnagan, Mark; Finitzo, Terese; Higgins, Michael; Watkins, Todd; Tien, Allen; Beales, Steven

2005-01-01

Information exchange, enabled by computable interoperability, is the key to many of the initiatives underway including the development of Regional Health Information Exchanges, Regional Health Information Organizations, and the National Health Information Network. These initiatives must include public health as a full partner in the emerging transformation of our nation’s healthcare system through the adoption and use of information technology. An electronic health record - public health (EHR-PH) system prototype was developed to demonstrate the feasibility of electronic data transfer from a health care provider, i.e. hospital or ambulatory care settings, to multiple customized public health systems which include a Newborn Metabolic Screening Registry, a Newborn Hearing Screening Registry, an Immunization Registry and a Communicable Disease Registry, using HL7 messaging standards. Our EHR-PH system prototype can be considered a distributed EHR-based RHIE/RHIO model - a principal element for a potential technical architecture for a NHIN. PMID:16779105

[The contribution of the Italian association of cancer registries (AIRTUM)].

PubMed

Crocetti, Emanuele; Buzzoni, Carlotta

2016-01-01

The study of cluster requires the ability to identify, with accuracy and completeness, the health events of interest and their geographical location and time of occurrence. For rare and complex diseases, such as childhood cancers, it is possible to observe a significant health migration from the place of residence, which makes the detection even more complex. The best tool to identify these rare diseases is represented by cancer registries (CRs). In fact, CRs collect, through many sources, information related to tumours that arise in the population resident in their areas of activity. The number of the sources of information has increased thanks to the computerization of health services. The availability of multiple sources of information increases the completeness of data collection overcoming the limits of a single source, and makes it possible to describe the diagnostic-therapeutic course and the outcome of the cases. Among all data sources, for childhood cancers the model 1.01, which summarize the clinical information of the cases treated in one of the Italian Association of paediatric haematology and oncology (AIEOP) centres, is relevant. Moreover, CRs produce reliable and comparable data due to the use of international rules and classifications for the definition of the topography and morphology of cancer, for the date of diagnosis, and for quality checks. In Italy, the Italian association of cancer registries (AIRTUM) coordinates the activities of 45 population CRs, both general and specialized (by age or tumour type). AIRTUM involves a population of over 6.7 million citizens under the age of 20 years, approximately 60% of the total resident population. AIRTUM plays a role of coordination, support, and harmonization for Italian CRs through training, accreditation, and a shared database, it promotes and participates in national and international collaboration involving scientific societies (AIEOP, Italian Association of medical oncology - AIOM, Italian Federation of volunteer-based cancer organisations - FAVO) and institutions (Italian national Institute of health, Italian national cancer institute of Milan) and performs analysis on key epidemiological indicators (incidence, mortality, survival, and prevalence). The AIRTUM database contains 19,650 cancer cases in child/ adolescent patients diagnosed from 1967 to 2011. The epidemiology of childhood cancers has been the subject of two AIRTUM monographs published in 2008 and in 2013 in collaboration with AIEOP; the latter includes specific contributions on polluted sites, on the psychological side, and on the experience of the parents of young cancer patients. The collaboration between different professionals, needs, and knowledge is the policy followed by AIRTUM to build up a complete picture of cancer epidemiology, even of childhood cancer, in Italy.

Hazardous substances releases associated with Hurricanes Katrina and Rita in industrial settings, Louisiana and Texas.

PubMed

Ruckart, Perri Zeitz; Orr, Maureen F; Lanier, Kenneth; Koehler, Allison

2008-11-15

The scientific literature concerning the public health response to the unprecedented hurricanes striking the Gulf Coast in August and September 2005 has focused mainly on assessing health-related needs and surveillance of injuries, infectious diseases, and other illnesses. However, the hurricanes also resulted in unintended hazardous substances releases in the affected states. Data from two states (Louisiana and Texas) participating in the Hazardous Substances Emergency Events Surveillance (HSEES) system were analyzed to describe the characteristics of hazardous substances releases in industrial settings associated with Hurricanes Katrina and Rita. HSEES is an active multi-state Web-based surveillance system maintained by the Agency for Toxic Substances and Disease Registry (ATSDR). In 2005, 166 hurricane-related hazardous substances events in industrial settings in Louisiana and Texas were reported. Most (72.3%) releases were due to emergency shut downs in preparation for the hurricanes and start-ups after the hurricanes. Emphasis is given to the contributing causal factors, hazardous substances released, and event scenarios. Recommendations are made to prevent or minimize acute releases of hazardous substances during future hurricanes, including installing backup power generation, securing equipment and piping to withstand high winds, establishing procedures to shutdown process operations safely, following established and up-to-date start-up procedures and checklists, and carefully performing pre-start-up safety reviews.

Racial disparities in paediatric kidney transplantation.

PubMed

Grace, Blair S; Kennedy, Sean E; Clayton, Philip A; McDonald, Stephen P

2014-01-01

Transplantation is the preferred treatment for children with end-stage kidney disease (ESKD). Pre-emptive transplants, those from live donors and with few human leukocyte antigen (HLA) mismatches provide the best outcomes. Studies into disparities in paediatric transplantation to date have not adequately disentangled different transplant types. We studied a retrospective cohort of 823 patients aged <18 years who started renal replacement therapy (RRT) in Australia 1990-2011, using the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA). The primary outcomes were time to first kidney transplant and kidney donor type (deceased or living), analysed using competing risk regression. Caucasian patients were most likely to receive any transplant, due largely to disparities in live donor transplantation. No Indigenous patients received a pre-emptive transplant. Indigenous patients were least likely to receive a transplant from a live donor (sub-hazard ratio 0.41, 95 % confidence interval 0.20-0.82, compared to Caucasians). Caucasian recipients had fewer HLA mismatches, were less sensitised and were more likely to have kidney diseases that could be diagnosed early or progress slowly. Caucasian paediatric patients are more likely to receive optimum treatment--a transplant from a living donor and fewer HLA mismatches. Further work is required to identify and address barriers to live donor transplantation among minority racial groups.

Protecting Confidentiality in Cancer Registry Data With Geographic Identifiers.

PubMed

Yu, Mandi; Reiter, Jerome Phillip; Zhu, Li; Liu, Benmei; Cronin, Kathleen A; Feuer, Eric J Rocky

2017-07-01

The National Cancer Institute's Surveillance, Epidemiology, and End Results Program releases research files of cancer registry data. These files include geographic information at the county level, but no finer. Access to finer geography, such as census tract identifiers, would enable richer analyses-for example, examination of health disparities across neighborhoods. To date, tract identifiers have been left off the research files because they could compromise the confidentiality of patients' identities. We present an approach to inclusion of tract identifiers based on multiply imputed, synthetic data. The idea is to build a predictive model of tract locations, given patient and tumor characteristics, and randomly simulate the tract of each patient by sampling from this model. For the predictive model, we use multivariate regression trees fitted to the latitude and longitude of the population centroid of each tract. We implement the approach in the registry data from California. The method results in synthetic data that reproduce a wide range (but not all) of analyses of census tract socioeconomic cancer disparities and have relatively low disclosure risks, which we assess by comparing individual patients' actual and synthetic tract locations. We conclude with a discussion of how synthetic data sets can be used by researchers with cancer registry data. Published by Oxford University Press on behalf of the Johns Hopkins Bloomberg School of Public Health 2017. This work is written by (a) US Government employee(s) and is in the public domain in the US.

Epidemiology of atlas fractures--a national registry-based cohort study of 1,537 cases.

PubMed

Matthiessen, Christian; Robinson, Yohan

2015-11-01

The epidemiology of fractures of the first cervical vertebra-the atlas-has not been well documented. Previous studies concerning atlas fractures focus on treatment and form a weak platform for epidemiologic study. This study aims to provide reliable epidemiologic data on atlas fractures. This was a national registry-based cohort study. A total of 1,537 cases of atlas fractures between 1997 and 2011 from the Swedish National Patient Registry (NPR). The outcome measures were annual incidence and mortality. Data from the NPR and the Swedish Cause of Death Registry were extracted, including age, gender, diagnosis, comorbidity, treatment codes, and date of death. The Charlson Comorbidity Index was calculated and a survival analysis performed. A total of 869 (56.5%) cases were men, and 668 (43.5%) were women. The mean age of the entire population was 64 years. The proportion of atlas fractures of all registered cervical fractures was 10.6%. In 19% of all cases, there was an additional fracture of the axis, and 7% of all cases had additional subaxial cervical fractures. Patients with fractures of the axis were older than patients with isolated atlas fractures. The annual incidence almost doubled during the study period, and in 2011, it was 17 per million inhabitants. The greatest increase in incidence occurred in the elderly population. Atlas fractures occurred predominantly in the elderly population. Further study is needed to determine the cause of the increasing incidence. Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.

Analyses of data of patients with Thrombotic Microangiopathy in the WAA registry.

PubMed

Mörtzell, M; Berlin, G; Nilsson, T; Axelsson, C G; Efvergren, M; Audzijoni, J; Griskevicius, A; Ptak, J; Blaha, M; Tomsova, H; Liumbruno, G M; Centoni, P; Newman, E; Eloot, S; Dhondt, A; Tomaz, J; Witt, V; Rock, G; Stegmayr, B

2011-10-01

Thrombotic Microangiopathy (TMA) is a histopathological feature of various diseases including thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. The aim of this study was to investigate the outcome and prognostic variables of TMA-patients. Data were consecutively retrieved from the WAA-apheresis registry (www.waa-registry.org) during 2003-2009. Included were all 120 patients (1237 procedures) who suffered from various forms of TMA, as registered by the ICD-10 code M31.1. Besides registry data, more extensive information was retrieved from the latest 64 patients. Adverse events of the TMA patients were compared to those of the other patients in the registry. The mean age was 46 years (range 11-85 years, 57% women). In 72% therapeutic apheresis was due to an acute indication while a long-term indication was present in 28%. Plasma exchange was performed by centrifugation and filtration technique (95% and 4%, respectively), and immunoadsorption in 1% of the patients. Only fresh frozen plasma was used as replacement fluid in 69% of procedures. Adverse events were more frequent than in the general apheresis population (10% versus 5%, RR 1.9, CI 1.6-2.3). No death occurred due to apheresis treatment. Three percent of the procedures were interrupted. Bronchospasm and/or anaphylactic shock were present in two patients and one patient suffered from TRALI. At admission 26% were bedridden and needed to be fed. The risk of dying during the treatment period was significantly higher if the patient also suffered from a compromising disease, such as cancer. There was an inverse correlation between the ADAMTS13 level and the antibody titer (r=-0.47, p=0.034). Patients with TMA have an increased risk for moderate and severe AE compared to the general apheresis population. Many patients were severely ill at admission. The prognosis is worse if the patient also has a severe chronic disease. Even slightly increased ADAMTS13-antibody titers seem to have a negative impact on the ADAMTS13 levels. Copyright © 2011 Elsevier Ltd. All rights reserved.

2-year results of the AUTAX (Austrian Multivessel TAXUS-Stent) registry beyond the SYNTAX (synergy between percutaneous coronary intervention with TAXUS and cardiac surgery) study.

PubMed

Gyöngyösi, Mariann; Christ, Günter; Lang, Irene; Kreiner, Gerhard; Sochor, Heinz; Probst, Peter; Neunteufl, Thomas; Badr-Eslam, Rosa; Winkler, Susanne; Nyolczas, Noemi; Posa, Aniko; Leisch, Franz; Karnik, Ronald; Siostrzonek, Peter; Harb, Stefan; Heigert, Matthias; Zenker, Gerald; Benzer, Werner; Bonner, Gerhard; Kaider, Alexandra; Glogar, Dietmar

2009-08-01

The multicenter AUTAX (Austrian Multivessel TAXUS-Stent) registry investigated the 2-year clinical/angiographic outcomes of patients with multivessel coronary artery disease after implantation of TAXUS Express stents (Boston Scientific, Natick, Massachusetts), in a "real-world" setting. The AUTAX registry included patients with 2- or 3-vessel disease, with/without previous percutaneous coronary intervention (PCI) and concomitant surgery. Patients (n = 441, 64 +/- 12 years, 78% men) (n = 1,080 lesions) with possible complete revascularization by PCI were prospectively included. Median clinical follow-up was 753 (quartiles 728 to 775) days after PCI in 95.7%, with control angiography of 78% at 6 months. The primary end point was the composite of major adverse cardiac (nonfatal acute myocardial infarction [AMI], all-cause mortality, target lesion revascularization [TLR]) and cerebrovascular events (MACCE). Potential risk factor effects on 2-year MACCE were evaluated using Cox regression. Complete revascularization was successful in 90.5%, with left main PCI of 6.8%. Rates of acute, subacute, and late stent thrombosis were 0.7%, 0.5%, and 0.5%. Two-year follow-up identified AMI (1.4%), death (3.6%), stroke (0.2%), and TLR (13.1%), for a composite MACCE of 18.3%. The binary restenosis rate was 10.8%. The median of cumulative SYNTAX score was 23.0 (range 12.0 to 56.5). The SYNTAX score did not predict TLR or MACCE, due to lack of scoring of restenotic or bypass stenoses (29.8%). Age (hazard ratio [HR]: 1.03, p = 0.019) and acute coronary syndrome (HR: 2.1, p = 0.001) were significant predictors of 2-year MACCE. Incomplete revascularization predicted death or AMI (HR: 3.84, p = 0.002). With the aim of complete revascularization, TAXUS stent implantations can be safe for patients with multivessel disease. The AUTAX registry including patients with post-PCI lesions provides additional information to the SYNTAX (Synergy Between Percutaneous Coronary Intervention With TAXUS and Cardiac Surgery) study. (Austrian Multivessel TAXUS-Stent Registry; NCT00738686).

Co-Care: A Registry for Individuals at Increased Risk for Colorectal Cancer.

PubMed

Sperling, Dylan; Jandorf, Lina; Sriphanlop, Pathu; Martinez, Clarissa; Brown, Karen L; Soper, Emily R; Hiraki, Susan; Itzkowitz, Steven H

2017-01-01

INTRODUCTION: Colorectal cancer (CRC) is one of the leading causes of cancer death for both men and women in the United States. Several factors can increase one’s risk of CRC, including a personal or family history of CRC, a diagnosis or family history of a hereditary colon cancer syndrome, or a diagnosis of chronic inflammatory bowel disease. The purpose of this project was to create a colorectal cancer registry (Co-Care) for individuals with a personal or family history of CRC, and those with disorders of the colon or rectum that are associated with an increased risk for developing CRC. Methods: To be eligible for the registry, patients either had a personal or family history of CRC, a diagnosis or family history of Lynch syndrome, familial adenomatous polyposis, or a diagnosis of Crohn’s colitis or ulcerative colitis with dysplasia. Participants were recruited after seeing their gastroenterologist or genetic counselor, or after undergoing a full or partial colectomy at Mount Sinai Hospital in New York City. Eligible patients who agreed to participate were interviewed by a member of the research staff and asked a wide range of questions pertaining to CRC risk. RESULTS: A total of 224 patients were enrolled in the registry. Participants are mostly white, born in the United States, and married, with a bachelor’s or graduate degree, reporting an annual household income of $100,000 or more. The largest portion have a family history of CRC (27.2%), and almost half of participants are of Jewish descent (46.2%) and have undergone full or partial colectomy (48.2%). More than half of participants have neither received genetic counseling (54.5%) nor undergone genetic testing (59.7%). Only 3.6% report that they currently smoke cigarettes, and 41.1% consume alcohol at least once per week. Lastly, 18.3%, 10.3%, and 27.7% of participants report that they currently take aspirin, folic acid/folate pills or tablets, or calcium pills/tablets, respectively. CONCLUSIONS: This registry can improve our understanding of CRC and related diseases, and be used to design future interventions related to disease risk, prognosis, and prevention of CRC.

Project VALOR: design and methods of a longitudinal registry of post-traumatic stress disorder (PTSD) in combat-exposed veterans in the Afghanistan and Iraqi military theaters of operations.

PubMed

Rosen, Raymond C; Marx, Brian P; Maserejian, Nancy N; Holowka, Darren W; Gates, Margaret A; Sleeper, Lynn A; Vasterling, Jennifer J; Kang, Han K; Keane, Terence M

2012-03-01

Few studies have investigated the natural history of post-traumatic stress disorder (PTSD). Project VALOR (Veterans' After-discharge Longitudinal Registry) was designed as a longitudinal patient registry assessing the course of combat-related PTSD among 1600 male and female Veterans who served in Operation Enduring Freedom (OEF) in Afghanistan or Operation Iraqi Freedom (OIF). Aims of the study include investigating patterns and predictors of progression or remission of PTSD and treatment utilization. The study design was based on recommendations from the Agency for Healthcare Quality and Research for longitudinal disease registries and used a pre-specified theoretical model to select the measurement domains for data collection and interpretation of forthcoming results. The registry will include 1200 male and female Veterans with a recent diagnosis of PTSD in the Department of Veteran Affairs (VA) electronic medical record and a comparison group of 400 Veterans without a medical record-based PTSD diagnosis, to also allow for case-control analyses. Data are collected from administrative databases, electronic medical records, a self-administered questionnaire, and a semi-structured diagnostic telephone interview. Project VALOR is a unique and timely registry study that will evaluate the clinical course of PTSD, psychosocial correlates, and health outcomes in a carefully selected cohort of returning OEF/OIF Veterans. Copyright © 2011 John Wiley & Sons, Ltd.

User experience analysis of e-TB Manager, a nationwide electronic tuberculosis recording and reporting system in Ukraine

PubMed Central

Sawyer, Kelly; Nizova, Nataliya

2017-01-01

Ukraine has successfully implemented e-TB Manager nationwide as its mandatory national tuberculosis registry after first introducing it in 2009. Our objective was to perform an end-of-programme evaluation after formal handover of the registry administration to Ukraine's Centre for Disease Control in 2015. We conducted a nationwide, cross-sectional, anonymous, 18-point user experience survey, and stratified the registry's transaction statistics to demonstrate usability. Contrary to initial implementation experience, older users (aged >50 years), often with limited or no computer proficiency prior to using the registry, had significantly better user experience scores for at least six of the 12 measures compared to younger users (aged 18–29 years). Using the registry for >3 years was associated with significantly higher scores for having capacity, adequacy of training received and satisfaction with the registry. Of the 5.9 million transactions over a 4-year period, nine out of 24 oblasts (regions) and Kiev city accounted for 62.5% of all transactions, and corresponded to 59% of Ukraine's tuberculosis burden. There were 437 unique active users in 486 rayons (districts) of Ukraine, demonstrating extensive reach. Our key findings complement the World Health Organization and European Respiratory Society's agenda for action on digital health to help implement the End TB Strategy. PMID:28512634

Evaluation of a chronic disease management system for the treatment and management of diabetes in primary health care practices in Ontario: an observational study.

PubMed

O'Reilly, D J; Bowen, J M; Sebaldt, R J; Petrie, A; Hopkins, R B; Assasi, N; MacDougald, C; Nunes, E; Goeree, R

2014-01-01

Computerized chronic disease management systems (CDMSs), when aligned with clinical practice guidelines, have the potential to effectively impact diabetes care. The objective was to measure the difference between optimal diabetes care and actual diabetes care before and after the introduction of a computerized CDMS. This 1-year, prospective, observational, pre/post study evaluated the use of a CDMS with a diabetes patient registry and tracker in family practices using patient enrolment models. Aggregate practice-level data from all rostered diabetes patients were analyzed. The primary outcome measure was the change in proportion of patients with up-to-date "ABC" monitoring frequency (i.e., hemoglobin A1c, blood pressure, and cholesterol). Changes in the frequency of other practice care and treatment elements (e.g., retinopathy screening) were also determined. Usability and satisfaction with the CDMS were measured. Nine sites, 38 health care providers, and 2,320 diabetes patients were included. The proportion of patients with up-to-date ABC (12%), hemoglobin A1c (45%), and cholesterol (38%) monitoring did not change over the duration of the study. The proportion of patients with up-to-date blood pressure monitoring improved, from 16% to 20%. Data on foot examinations, retinopathy screening, use of angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers, and documentation of self-management goals were not available or not up to date at baseline for 98% of patients. By the end of the study, attitudes of health care providers were more negative on the Training, Usefulness, Daily Practice, and Support from the Service Provider domains of the CDMS, but more positive on the Learning, Using, Practice Planning, CDMS, and Satisfaction domains. Few practitioners used the CDMS, so it was difficult to draw conclusions about its efficacy. Simply giving health care providers a potentially useful technology will not ensure its use. This real-world evaluation of a web-based CDMS for diabetes failed to impact physician practice due to limited use of the system. Patients and health care providers need timely access to information to ensure proper diabetes care. This study looked at whether a computer-based system at the doctor's office could improve diabetes management. However, few clinics and health care providers used the system, so no improvement in diabetes care was seen.

Myotonic Dystrophy and Facioscapulohumeral Muscular Dystrophy Registry

ClinicalTrials.gov

2017-08-11

Myotonic Dystrophy; Facioscapulohumeral Muscular Dystrophy; Muscular Dystrophy; Myotonic Dystrophy Type 1; Myotonic Dystrophy Type 2; Congenital Myotonic Dystrophy; PROMM (Proximal Myotonic Myopathy); Steinert's Disease; Myotonic Muscular Dystrophy

Renal replacement therapy in Europe: a summary of the 2011 ERA–EDTA Registry Annual Report

PubMed Central

Noordzij, Marlies; Kramer, Anneke; Abad Diez, José M.; Alonso de la Torre, Ramón; Arcos Fuster, Emma; Bikbov, Boris T.; Bonthuis, Marjolein; Bouzas Caamaño, Encarnación; Čala, Svetlana; Caskey, Fergus J.; Castro de la Nuez, Pablo; Cernevskis, Harijs; Collart, Frederic; Díaz Tejeiro, Rafael; Djukanovic, Ljubica; Ferrer-Alamar, Manuel; Finne, Patrik; García Bazaga, María de los Angelos; Garneata, Liliana; Golan, Eliezer; Gonzalez Fernández, Raquel; Heaf, James G.; Hoitsma, Andries; Ioannidis, George A.; Kolesnyk, Mykola; Kramar, Reinhard; Lasalle, Mathilde; Leivestad, Torbjørn; Lopot, Frantisek; van de Luijtgaarden, Moniek W.M.; Macário, Fernando; Magaz, Ángela; Martín Escobar, Eduardo; de Meester, Johan; Metcalfe, Wendy; Ots-Rosenberg, Mai; Palsson, Runolfur; Piñera, Celestino; Pippias, Maria; Prütz, Karl G.; Ratkovic, Marina; Resić, Halima; Rodríguez Hernández, Aurelio; Rutkowski, Boleslaw; Spustová, Viera; Stel, Vianda S.; Stojceva-Taneva, Olivera; Süleymanlar, Gültekin; Wanner, Christoph; Jager, Kitty J.

2014-01-01

Background This article provides a summary of the 2011 ERA–EDTA Registry Annual Report (available at www.era-edta-reg.org). Methods Data on renal replacement therapy (RRT) for end-stage renal disease (ESRD) from national and regional renal registries in 30 countries in Europe and bordering the Mediterranean Sea were used. From 27 registries, individual patient data were received, whereas 17 registries contributed data in aggregated form. We present the incidence and prevalence of RRT, and renal transplant rates in 2011. In addition, survival probabilities and expected remaining lifetimes were calculated for those registries providing individual patient data. Results The overall unadjusted incidence rate of RRT in 2011 among all registries reporting to the ERA–EDTA Registry was 117 per million population (pmp) (n = 71.631). Incidence rates varied from 24 pmp in Ukraine to 238 pmp in Turkey. The overall unadjusted prevalence of RRT for ESRD on 31 December 2011 was 692 pmp (n = 425 824). The highest prevalence was reported by Portugal (1662 pmp) and the lowest by Ukraine (131 pmp). Among all registries, a total of 22 814 renal transplantations were performed (37 pmp). The highest overall transplant rate was reported from Spain, Cantabria (81 pmp), whereas the highest rate of living donor transplants was reported from Turkey (39 pmp). For patients who started RRT between 2002 and 2006, the unadjusted 5-year patient survival on RRT was 46.8% [95% confidence interval (CI) 46.6–47.0], and on dialysis 39.3% (95% CI 39.2–39.4). The unadjusted 5-year patient survival after the first renal transplantation performed between 2002 and 2006 was 86.7% (95% CI 86.2–87.2) for kidneys from deceased donors and 94.3% (95% CI 93.6–95.0) for kidneys from living donors. PMID:25852881

Completeness and accuracy of the wisconsin immunization registry: an evaluation coinciding with the beginning of meaningful use.

PubMed

Koepke, Ruth; Petit, Ashley B; Ayele, Roman A; Eickhoff, Jens C; Schauer, Stephanie L; Verdon, Matthew J; Hopfensperger, Daniel J; Conway, James H; Davis, Jeffrey P

2015-01-01

Vaccination coverage rates can be improved through the application of complete and accurate immunization information systems (IISs). Evaluate the completeness and accuracy of Wisconsin's IIS, the Wisconsin Immunization Registry (WIR). Cross-sectional evaluation, comparing vaccination medical records (MRs) from provider clinics with WIR records. Medical records of patients born during 2009 were randomly selected from 251 Wisconsin clinics associated with the Vaccines for Children Program. Completeness: percentage of patients with client records in the WIR, percentage of patients up-to-date (%UTD) with the 4:3:1:3:3:1:4 vaccination series, and percentage of patients' MR vaccinations matched by administration date (±10 days) and type to vaccinations documented in the WIR. Accuracy: percentages of matched vaccinations with the same administration date, same trade name (TN), and same lot number. Of the 1863 selected patient MRs, 98% (n = 1833) had WIR client records and 97% of their 30 899 vaccinations were documented in the WIR. The %UTD was 49.3% using the MR only, 76.5% using the WIR only, and 75.2% as estimated by the National Immunization Survey. Among matched vaccinations, 99% had the same administration date, 96% had the same TN, and 95% had the same lot number. Compared with patients from clinics that entered data into the WIR using data exchange from electronic health records, patients from clinics that entered data using the Web-based user interface were less likely to have client records in the WIR (odds ratio: 0.3; 95% confidence interval: 0.1-0.9) and less likely to have accurate TNs (odds ratio: 0.3; 95% confidence interval: 0.1-0.5). The WIR was complete and accurate among this sample of children born during 2009 and provided a vaccination coverage assessment similar to the National Immunization Survey. Our results provide support for the expectation that meaningful use and other initiatives that increase data exchange from electronic health records to IISs will improve IIS data quality.

The history and use of cancer registry data by public health cancer control programs in the United States.

PubMed

White, Mary C; Babcock, Frances; Hayes, Nikki S; Mariotto, Angela B; Wong, Faye L; Kohler, Betsy A; Weir, Hannah K

2017-12-15

Because cancer registry data provide a census of cancer cases, registry data can be used to: 1) define and monitor cancer incidence at the local, state, and national levels; 2) investigate patterns of cancer treatment; and 3) evaluate the effectiveness of public health efforts to prevent cancer cases and improve cancer survival. The purpose of this article is to provide a broad overview of the history of cancer surveillance programs in the United States, and illustrate the expanding ways in which cancer surveillance data are being made available and contributing to cancer control programs. The article describes the building of the cancer registry infrastructure and the successful coordination of efforts among the 2 federal agencies that support cancer registry programs, the Centers for Disease Control and Prevention and the National Cancer Institute, and the North American Association of Central Cancer Registries. The major US cancer control programs also are described, including the National Comprehensive Cancer Control Program, the National Breast and Cervical Cancer Early Detection Program, and the Colorectal Cancer Control Program. This overview illustrates how cancer registry data can inform public health actions to reduce disparities in cancer outcomes and may be instructional for a variety of cancer control professionals in the United States and in other countries. Cancer 2017;123:4969-76. Published 2017. This article is a U.S. Government work and is in the public domain in the USA. Published 2017. This article is a U.S. Government work and is in the public domain in the USA.

Etiology of Bronchiectasis in a Cohort of 2047 Patients. An Analysis of the Spanish Historical Bronchiectasis Registry.

PubMed

Olveira, Casilda; Padilla, Alicia; Martínez-García, Miguel-Ángel; de la Rosa, David; Girón, Rosa-María; Vendrell, Montserrat; Máiz, Luis; Borderías, Luis; Polverino, Eva; Martínez-Moragón, Eva; Rajas, Olga; Casas, Francisco; Cordovilla, Rosa; de Gracia, Javier

2017-07-01

Bronchiectasis is caused by many diseases. Establishing its etiology is important for clinical and prognostic reasons. The aim of this study was to evaluate the etiology of bronchiectasis in a large patient sample and its possible relationship with demographic, clinical or severity factors, and to analyze differences between idiopathic disease, post-infectious disease, and disease caused by other factors. Multicenter, cross-sectional study of the SEPAR Spanish Historical Registry (RHEBQ-SEPAR). Adult patients with bronchiectasis followed by pulmonologists were included prospectively. Etiological studies were based on guidelines and standardized diagnostic tests included in the register, which were later included in the SEPAR guidelines on bronchiectasis. A total of 2,047 patients from 36 Spanish hospitals were analyzed. Mean age was 64.9years and 54.9% were women. Etiology was identified in 75.8% of cases (post-Infection: 30%; cystic fibrosis: 12.5%; immunodeficiencies: 9.4%; COPD: 7.8%; asthma: 5.4%; ciliary dyskinesia: 2.9%, and systemic diseases: 1.4%). The different etiologies presented different demographic, clinical, and microbiological factors. Post-infectious bronchiectasis and bronchiectasis caused by COPD and asthma were associated with an increased risk of poorer lung function. Patients with post-infectious bronchiectasis were older and were diagnosed later. Idiopathic bronchiectasis was more common in female non-smokers and was associated with better lung function, a higher body mass index, and a lower rate of Pseudomonas aeruginosa than bronchiectasis of known etiology. The etiology of bronchiectasis was identified in a large proportion of patients included in the RHEBQ-SEPAR registry. Different phenotypes associated with different causes could be identified. Copyright © 2016 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

Effectiveness and response predictors of omalizumab in a severe allergic asthma population with a high prevalence of comorbidities: the Australian Xolair Registry.

PubMed

Gibson, P G; Reddel, H; McDonald, V M; Marks, G; Jenkins, C; Gillman, A; Upham, J; Sutherland, M; Rimmer, J; Thien, F; Katsoulotos, G P; Cook, M; Yang, I; Katelaris, C; Bowler, S; Langton, D; Robinson, P; Wright, C; Yozghatlian, V; Burgess, S; Sivakumaran, P; Jaffe, A; Bowden, J; Wark, P A B; Yan, K Y; Kritikos, V; Peters, M; Hew, M; Aminazad, A; Bint, M; Guo, M

2016-09-01

Severe asthma is a high impact disease. Omalizumab targets the allergic inflammatory pathway; however, effectiveness data in a population with significant comorbidities are limited. To describe severe allergic asthma, omalizumab treatment outcomes and predictors of response among the Australian Xolair Registry participants. A web-based post-marketing surveillance registry was established to characterise the use, effectiveness and adverse effects of omalizumab (Xolair) for severe allergic asthma. Participants (n = 192) (mean age 51 years, 118 female) with severe allergic asthma from 21 clinics in Australia were assessed, and 180 received omalizumab therapy. They had poor asthma control (Asthma Control Questionnaire, ACQ-5, mean score 3.56) and significant quality of life impairment (Asthma-related Quality of Life Questionnaire score 3.57), and 52% were using daily oral corticosteroid (OCS). Overall, 95% had one or more comorbidities (rhinitis 48%, obesity 45%, cardiovascular disease 23%). The omalizumab responder rate, assessed by an improvement of at least 0.5 in ACQ-5, was high at 83%. OCS use was significantly reduced. The response in participants with comorbid obesity and cardiovascular disease was similar to those without these conditions. Baseline ACQ-5 ≥ 2.0 (P = 0.002) and older age (P = 0.05) predicted the magnitude of change in ACQ-5 in response to omalizumab. Drug-related adverse events included anaphylactoid reactions (n = 4), headache (n = 2) and chest pains (n = 1). Australian patients with severe allergic asthma report a high disease burden and have extensive comorbidity. Symptomatic response to omalizumab was high despite significant comorbid disease. Omalizumab is an effective targeted therapy for severe allergic asthma with comorbidity in a real-life setting. © 2016 Royal Australasian College of Physicians.

Changes in co-morbidity pattern in patients starting renal replacement therapy in Europe-data from the ERA-EDTA Registry.

PubMed

Ceretta, Maria L; Noordzij, Marlies; Luxardo, Rosario; De Meester, Johan; Abad Diez, Jose M; Finne, Patrik; Heaf, James G; Couchoud, Cécile; Kramar, Reinhard; Collart, Frederic; Cases, Aleix; Palsson, Runolfur; Reisæter, Anna V; Rydell, Helena; Massy, Ziad A; Jager, Kitty J; Kramer, Anneke

2018-01-18

Patients starting renal replacement therapy (RRT) for end-stage renal disease often present with one or more co-morbidities. This study explored the prevalence of co-morbidities in patients who started RRT in Europe during the period from 2005 to 2014. Using data from patients aged 20 years or older from all 11 national or regional registries providing co-morbidity data to the European Renal Association - European Dialysis and Transplant Association Registry, we examined the prevalence of the following co-morbidities: diabetes mellitus (DM) (primary renal disease and/or co-morbidity), ischaemic heart disease (IHD), congestive heart failure (CHF), peripheral vascular disease (PVD), cerebrovascular disease (CVD) and malignancy. Overall, 70% of 7578 patients who initiated RRT in 2014 presented with at least one co-morbidity: 39.0% presented with DM, 25.0% with IHD, 22.3% with CHF, 17.7% with PVD, 16.4% with malignancy and 15.5% with CVD. These percentages differed substantially between countries. Co-morbidities were more common in men than in women, in older patients than in younger patients, and in patients on haemodialysis at Day 91 when compared with patients on peritoneal dialysis. Between 2005 and 2014 the prevalence of DM and malignancy increased over time, whereas the prevalence of IHD and PVD declined. More than two-thirds of patients initiating RRT in Europe have at least one co-morbidity. With the rising age at the start of RRT over the last decade, there have been changes in the co-morbidity pattern: the prevalence of cardiovascular co-morbidities decreased, while the prevalence of DM and malignancy increased. © The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Is Survival for Patients with Resectable Lung Metastatic Colorectal Cancer Comparable to Those with Resectable Liver Disease? Results from the South Australian Metastatic Colorectal Registry.

PubMed

Patel, Dainik; Townsend, Amanda R; Karapetis, Christos; Beeke, Carol; Padbury, Rob; Roy, Amitesh; Maddern, Guy; Roder, David; Price, Timothy J

2016-10-01

Hepatic resection for colorectal (CRC) metastasis is considered a standard of care. Resection of metastasis isolated to lung also is considered potentially curable, although there is still some variation in recommendations. We explore outcomes for patients undergoing lung resection for mCRC, with the liver resection group as the comparator. South Australian (SA) metastatic CRC registry data were analysed to assess patient characteristics and survival outcomes for patients suitable for lung or liver resection. A total of 3241 patients are registered on the database to December 2014. One hundred two (3.1 %) patients were able to undergo a lung resection compared with 420 (12.9 %) who had a liver resection. Of the lung resection patients, 62 (61 %) presented with lung disease only, 21 % initially presented with liver disease only, 11 % had both lung and liver, and 7 % had brain or pelvic disease resection. Of these patients, 79 % went straight to surgery without any neoadjuvant treatment and 34 % had lung resection as the only intervention. Chemotherapy for metastatic disease was given more often to liver resection patients: 76.9 versus 53.9 %, p = 0.17. Median overall survival is 5.6 years for liver resection and has not been reached for lung resection (hazard ratio 0.82, 95 % confidence interval 0.54-1.24, p = 0.33). Lung resection was undertaken in 3.1 % of patients with mCRC in our registry. These data provide further support for long-term survival after lung resection in mCRC, survival that is at least comparable to those who undergo resection for liver metastasis in mCRC.

Recurrent invasive pneumococcal disease in children--host factors and vaccination response.

PubMed

Ingels, Helene Andrea Sinclair

2015-07-01

Streptococcus pneumoniae is still a leading cause of septicaemia, pneumonia and meningitis in young children world-wide with over half a million children dying annually from pneumococcal disease.  Some children are prone to repeated episodes of invasive pneumococcal disease (IPD) because of an underlying predisposing disease. Recurrent IPD (rIPD) is a rarity and published reports on rIPD are limited by having few children included, selected groups of patients or short follow-up periods. Deficiencies in the innate or adaptive immune system have been described in children with rIPD, but the frequency of immunodeficiency among such patients is unknown. The aim of this PhD thesis was to examine paediatric cases of laboratory-confirmed rIPD, over a 33-year period in Denmark, to determine risk factors and study aspects of the immunological background for this problem in children. In October 2007, a seven-valent pneumococcal conjugate vaccine (PCV7) was implemented in the Danish infant immunization programme. An additional aim of the thesis was to examine the impact of vaccination on a population level, following the first three years of general PCV7 vaccination in Denmark. The thesis consists of three papers, which are all directly or indirectly based on data retrieved from the National Streptococcus Pneumoniae Registry. This registry is nationwide and dates back to 1938. The registry contains data from all laboratory-confirmed cases of IPD in Denmark and is continually updated for national surveillance. In Paper 1, we conducted a 33-year retrospective nationwide study of paediatric rIPD. By using data from the National Streptococcus Pneumoniae Registry combined with clinical data from hospital records, we could describe one of the largest known cohorts of children (n:59) with rIPD . We covered epidemiological, microbiological, and clinical features of this clinical entity. Of all children experiencing rIPD, 47% had a known predisposing underlying disease at the time of the rIPD. Most common was immune deficiency due to transplantation. In 19% the episode of rIPD was the clinical manifestation that subsequently led to a diagnosis of an underlying disease. Finally, in 31% of the children no underlying disease was detected. Paper 2 covers data from a follow-up of the cohort of children described in Paper 1. Of this unselected cohort of rIPD, all children without an obvious underlying disease predisposing to pneumococcal disease (such as malignancy, HIV or cerebrospinal-fluid leakage) were invited to participate in the study by undergoing a thorough immunological evaluation. Basic immunological parameters including activity of complement-pathways and T-, B-, NK-cell count were examined in the children and their families. Furthermore, B-cell function including antibody response to polysaccharide-based pneumococcal vaccination and somatic hypermutation was evaluated. Toll like receptor (TLR) signalling function was evaluated in a functional assay. When children with classical risk factors for IPD were excluded, 15 individuals were eligible. Of whom, sex (40%) children with complement C2 deficiency were identified. Moreover, impaired vaccination response was found in six children: three with concurrent C2 deficiency and three with no other immune abnormality. One patient with a severe TLR signalling dysfunction was diagnosed. In Paper 3, we aimed to assess the impact of PCV7 in Denmark following the first three years of infant immunization. By comparing age-specific disease incidences of IPD in the pre-PCV7 (years 2000-2007) and the PCV7 periods (years 2008-2010) we sought to assess direct and indirect effects on incidence of IPD. In addition, changes in pneumococcal serotype distribution and IPD-related mortality were assessed. We documented a marked decline in the incidence of IPD in both vaccinated and non-vaccinated age groups. The overall incidence of IPD among children aged 0-5 years declined from 26.7 to 16.3 cases per 100,000 (IRR 0.58; 95% confidence interval (CI) [0.48-0.69]). A minor but statistically significant increase in the incidence of IPD due to non-vaccine type IPD was observed in both vaccinated and non-vaccinated groups, but with predominance of serotypes covered by the higher valence pneumococcal conjugate vaccines. This thesis confirms the existing knowledge on underlying diseases predisposing children to IPD, such as cerebrospinal fluid leakage, congenital heart disease and malignant diseases. Our findings support the notion that rIPD in a child should prompt a thorough search for an underlying disease. Moreover, our results underline that rIPD in a child without a known predisposing disease is a major pointer towards primary immune deficiency, such as complement deficiency and B cell dysfunction. This is in line with the guidelines put forward by various specialist committees. Finally, we reported data from the first three years of PCV7 vaccination in the Danish immunization programme, suggesting that the vaccine is effective against all serotypes included in the vaccine when administered in a 2+1 schedule.

The natural history of Leydig cell testicular tumours: an analysis of the National Cancer Registry.

PubMed

Nason, G J; Redmond, E J; Considine, S W; Omer, S I; Power, D; Sweeney, P

2018-05-01

Leydig cell tumour (LCT) of the testis is a rare histological subtype of stromal tumours, accounting for 1 to 3% of testicular neoplasms. The natural history of LCT is poorly understood. The aim of this study was to assess the incidence and natural history of Leydig cell tumours (LCT) of the testes. A search of the National Cancer Registry of Ireland database was performed regarding Leydig cell testicular tumours. Recurrence free survival (RFS) and disease-specific survival (DSS) were analysed. Between 1994 and 2013, 2755 new cases of testicular cancer were diagnosed in Ireland. Of these, 22 (0.79%) were Leydig cell tumours. Nineteen were invasive (stage T1) and three were in situ (stage Tis). One patient developed a local recurrence following an organ preserving procedure and underwent a completion orchidectomy 107 days after initial diagnosis. No further treatment was required. There have been no disease-specific deaths. The 1-, 3- and 5-year overall survival (OS) rates were 95.5, 88.2 and 73.3%, respectively. The 5-year disease-specific survival (DSS) was 100% and the 5-year recurrence free survival (RFS) was 93.3%. From the National Cancer Registry, LCT has been shown to be a rare subtype of testicular tumour. Due to the relatively favourable natural history, it may be possible to tailor less aggressive surveillance regimens in these patients.

The value of local registry data for describing cervical cancer management and outcomes over three decades in Australia.

PubMed

Roder, D; Davy, M; Selva-Nayagam, S; Gowda, R; Paramasivam, S; Adams, J; Keefe, D; Eckert, M; Powell, K; Fusco, K; Buranyi-Trevarton, D; Oehler, M K

2018-01-01

Registry data on invasive cervical cancers (n = 1,274) from four major hospitals (1984-2012) were analysed to determine their value for informing local service delivery in Australia. The methodology comprised disease-specific survival analyses using Kaplan-Meier product-limit estimates and Cox proportional hazards models and treatment analyses using logistic regression. Five- and 10-year survivals were 72% and 68%, respectively, equating with relative survival estimates for Australia and the USA. Most common treatments were surgery and radiotherapy. Systemic therapies increased in recent years, generally with radiotherapy, but were less common for residents from less accessible areas. Surgery was more common for younger women and early-stage disease, and radiotherapy for older women and regional and more advanced disease. The proportion of glandular cancers increased in-step with national trends. Little evidence of variation in risk-adjusted survival presented over time or by Local Health District. The study illustrates the value of local registry data for describing local treatment and outcomes. They show the lower use of systemic therapies among residents of less accessible areas which warrants further investigation. Risk-adjusted treatment and outcomes did not vary by socio-economic status, suggesting equity in service delivery. These data are important for local evaluation and were not available from other sources. © 2017 John Wiley & Sons Ltd.

The Quality of Rare Disease Registries: Evaluation and Characterization.

PubMed

Coi, Alessio; Santoro, Michele; Villaverde-Hueso, Ana; Lipucci Di Paola, Michele; Gainotti, Sabina; Taruscio, Domenica; Posada de la Paz, Manuel; Bianchi, Fabrizio

2016-01-01

The focus on the quality of the procedures for data collection, storing, and analysis in the definition and implementation of a rare disease registry (RDR) is the basis for developing a valid and long-term sustainable tool. The aim of this study was to provide useful information for characterizing a quality profile for RDRs using an analytical approach applied to RDRs participating in the European Platform for Rare Disease Registries 2011-2014 (EPIRARE) survey. An indicator of quality was defined by choosing a small set of quality-related variables derived from the survey. The random forest method was used to identify the variables best defining a quality profile for RDRs. Fisher's exact test was employed to assess the association with the indicator of quality, and the Cochran-Armitage test was used to check the presence of a linear trend along different levels of quality. The set of variables found to characterize high-quality RDRs focused on ethical and legal issues, governance, communication of activities and results, established procedures to regulate access to data and security, and established plans to ensure long-term sustainability. The quality of RDRs is usually associated with a good oversight and governance mechanism and with durable funding. The results suggest that RDRs would benefit from support in management, information technology, epidemiology, and statistics. © 2016 S. Karger AG, Basel.

Global cardiac risk assessment in the Registry Of Pregnancy And Cardiac disease: results of a registry from the European Society of Cardiology.

PubMed

van Hagen, Iris M; Boersma, Eric; Johnson, Mark R; Thorne, Sara A; Parsonage, William A; Escribano Subías, Pilar; Leśniak-Sobelga, Agata; Irtyuga, Olga; Sorour, Khaled A; Taha, Nasser; Maggioni, Aldo P; Hall, Roger; Roos-Hesselink, Jolien W

2016-05-01

To validate the modified World Health Organization (mWHO) risk classification in advanced and emerging countries, and to identify additional risk factors for cardiac events during pregnancy. The ongoing prospective worldwide Registry Of Pregnancy And Cardiac disease (ROPAC) included 2742 pregnant women (mean age ± standard deviation, 29.2 ± 5.5 years) with established cardiac disease: 1827 from advanced countries and 915 from emerging countries. In patients from advanced countries, congenital heart disease was the most prevalent diagnosis (70%) while in emerging countries valvular heart disease was more common (55%). A cardiac event occurred in 566 patients (20.6%) during pregnancy: 234 (12.8%) in advanced countries and 332 (36.3%) in emerging countries. The mWHO classification had a moderate performance to discriminate between women with and without cardiac events (c-statistic 0.711 and 95% confidence interval (CI) 0.686-0.735). However, its performance in advanced countries (0.726) was better than in emerging countries (0.633). The best performance was found in patients with acquired heart disease from developed countries (0.712). Pre-pregnancy signs of heart failure and, in advanced countries, atrial fibrillation and no previous cardiac intervention added prognostic value to the mWHO classification, with a c-statistic of 0.751 (95% CI 0.715-0.786) in advanced countries and of 0.724 (95% CI 0.691-0.758) in emerging countries. The mWHO risk classification is a useful tool for predicting cardiac events during pregnancy in women with established cardiac disease in advanced countries, but seems less effective in emerging countries. Data on pre-pregnancy cardiac condition including signs of heart failure and atrial fibrillation, may help to improve preconception counselling in advanced and emerging countries. © 2016 The Authors. European Journal of Heart Failure © 2016 European Society of Cardiology.

Maternal factors associated with offspring polycystic ovarian syndrome.

PubMed

Steiner, Anne Z

2018-04-19

In this Swedish national registry-based cohort study Valgeirsdottir et al. sought to determine the association between 1) in-utero exposures and 2) birth indices and the subsequent development of polycystic ovarian syndrome (PCOS) after age 15. To do this, they selected 681,123 girls, whose birth was recorded in the Swedish birth registry between 1982 and 1995. They followed the girls in the Swedish Patient Registry until 2010 for a diagnosis of PCOS using International Classification of Diseases (ICD) codes. The authors found that in-utero exposure to maternal obesity and smoking, but not maternal pre-eclampsia or gestational diabetes, increased the risk of PCOS as an adolescent or young adult. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Myeloproliferative Neoplasms (MPNs) Patient Registry

ClinicalTrials.gov

2017-10-27

Primary Myelofibrosis; Polycythemia Vera; Essential Thrombocythemia; Mastocytosis; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative; Leukemia, Myelomonocytic, Juvenile; Chronic Eosinophilic Leukemia-not Otherwise Specified; Myelodysplastic-Myeloproliferative Diseases; Neoplasms; Leukemia, Myelomonocytic, Chronic

Joint registries as continuous surveillance systems: the experience of the Catalan Arthroplasty Register (RACat).

PubMed

Allepuz, Alejandro; Martínez, Olga; Tebé, Cristian; Nardi, Joan; Portabella, Frederic; Espallargues, Mireia

2014-03-01

The aim was to present results on prosthesis performance in Catalonia for the period 2005-2010. All publicly funded hospitals submit in an electronic format data on hip and knee arthroplasties: patients' insurance identification number, hospital, joint (hip/knee), type of arthroplasty (primary/revision), side (right/left), date of surgery and prosthesis (manufacturer name and catalogue number). A standard survival analysis based on Kaplan-Meier estimation was carried out. Fifty-two hospitals have sent information to the RACat which has data on 36,951 knee and 26,477 hip arthroplasties. Cumulative prostheses revision risks at 3 years were 3.3% (95% CI: 3.1-3.6) for knee, 2.9% (95% CI: 2.5-3.3) for total hip and 2.5% (95% CI: 2.0-3.1) for partial hip. When compared to other registries a higher risk of revision was observed. © 2013.

Treatment of dental complications in sickle cell disease.

PubMed

Mulimani, Priti; Ballas, Samir K; Abas, Adinegara B L; Karanth, Laxminarayan

2016-04-22

Sickle cell disease is the most common single gene disorder and the commonest haemoglobinopathy found with high prevalence in many populations across the world. Management of dental complications in people with sickle cell disease requires special consideration for three main reasons. Firstly, dental and oral tissues are affected by the blood disorder resulting in several oro-facial abnormalities. Secondly, living with a haemoglobinopathy and coping with its associated serious consequences may result in individuals neglecting their oral health care. Finally, the treatment of these oral complications must be adapted to the systemic condition and special needs of these individuals, in order not to exacerbate or deteriorate their general health.Guidelines for the treatment of dental complications in this population who require special care are unclear and even unavailable in many aspects. Hence this review was undertaken to provide a basis for clinical care by investigating and analysing the existing evidence in the literature for the treatment of dental complications in people with sickle cell disease. To assess methods of treating dental complications in people with sickle cell disease. We searched the Cochrane Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books.Date of last search: 11 April 2016.Additionally, we searched nine online databases (PubMed, Google Scholar, ClinicalTrials.gov, WHO International Clinical Trials Registry Platform, Literature in the Health Sciences in Latin America and the Caribbean database, African Index Medicus, Index Medicus for South East Asia Region, Index Medicus for the Eastern Mediterranean Region, Indexing of Indian Medical Journals). We also searched the reference lists of relevant articles and reviews and contacted haematologists, experts in fields of dentistry, organizations, pharmaceutical companies and researchers working in this field.Date of last search: 03 March 2016. We searched for published or unpublished randomised controlled studies of treatments for dental complications in people with sickle cell disease. Two review authors intended to independently extract data and assess the risk of bias of the included studies using standard Cochrane methodologies; however, no studies were identified for inclusion in the review. No randomised controlled studies were identified. This Cochrane review did not identify any randomised controlled studies assessing interventions for the treatment of dental complications in people with sickle cell disease. There is an important need for randomised controlled studies in this area, so as to identify the most effective and safe method for treating dental complications in people with sickle cell disease.