Science.gov

Sample records for national disease registries

  1. 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.

  2. Establishment of an Australian National Genetic Heart Disease Registry.

    PubMed

    Ingles, Jodie; McGaughran, Julie; Vohra, Jitendra; Weintraub, Robert G; Davis, Andrew; Atherton, John; Semsarian, Christopher

    2008-12-01

    A National Genetic Heart Disease Registry has recently been established, with the aim to enroll every family in Australia with a genetically determined cardiomyopathy or primary arrhythmic disorder. The Registry seeks to further our understanding of the impact and burden of disease in this population; increase awareness and provide education to health professionals and families; and establish a large cardiac genetic cohort as a resource for approved research studies. The Registry is currently recruiting families with inherited cardiomyopathies (e.g. hypertrophic cardiomyopathy) and primary arrhythmogenic disorders (e.g. long QT syndrome), with scope to expand this in the future. Affected individuals, as well as their first-degree (at-risk) family members are eligible to enroll. Participants are currently being recruited from cardiac genetics clinics in approved recruitment sites and hope to expand to other Australian centres including general cardiology practice in the future. A significant focus of the Registry is to improve understanding and create awareness of inherited heart diseases, which includes ensuring families are aware of genetic testing options and current clinical screening recommendations for at-risk family members. A Registry Advisory Committee has been established under the NHMRC Guidelines, and includes a representative from each major recruitment centre. This committee approves all decisions relating to the Registry including approval of research studies. A National Genetic Heart Disease Registry will provide a valuable resource to further our knowledge of the clinical and genetic aspects of these diseases. Since most of the current data about the prevalence, natural history and outcomes of genetic heart diseases has emanated from the United States and Europe, characterising these Australian populations will be of significant benefit, allowing for more informed and specific health care planning and resource provision.

  3. National Registry of Designated Intractable Diseases in Japan: Present Status and Future Prospects

    PubMed Central

    KANATANI, Yasuhiro; TOMITA, Naoko; SATO, Yoko; ETO, Akiko; OMOE, Hiroe; MIZUSHIMA, Hiroshi

    2017-01-01

    Japan promotes research related to intractable diseases and financially supports patients with these diseases. Intractable diseases are designated as those that fulfill the following criteria: (1) rarity (affecting less than 0.1% of the population in Japan), (2) unknown etiology, (3) lack of effective treatment, (4) necessity of long-term treatment, and (5) existence of objective diagnostic criteria and not necessarily equal to rare diseases in other countries. The construction of a national database is required to promote research to clarify the pathogenesis of these diseases and to develop pharmaceutical products and medical devices. The Ministry of Health, Labor, and Welfare launched an online registration system in 2001, but many problems associated with gathering and utilizing information on patients with intractable diseases remain. In this paper, we describe the present status of the national registry of designated intractable diseases in Japan and discuss future prospects. PMID:27666154

  4. Record linkage between hospital discharges and mortality registries for motor neuron disease case ascertainment for the Spanish National Rare Diseases Registry.

    PubMed

    Ruiz, Elena; Ramalle-Gómara, Enrique; Quiñones, Carmen

    2014-06-01

    Our objective was to analyse the coverage of hospital discharge data and the mortality registry (MR) of La Rioja to ascertain motor neuron disease (MND) cases to be included in the Spanish National Rare Diseases Registry. MND cases that occurred in La Rioja during the period 1996-2011 were selected from hospital discharge data and the MR by means of the International Classification of Diseases. Review of the medical histories was carried out to confirm the causes of death reported. Characteristics of the population with MND were analysed. A total of 133 patients with MND were detected in La Rioja during the period 1996-2011; 30.1% were only recorded in the hospital discharges data, 12.0% only in the MR, and 57.9% were recorded by both databases. Medical records revealed a miscoding of patients who had been diagnosed with progressive supranuclear palsy but were recorded in the MR with an MND code. In conclusion, the hospital discharges data and the MR appear to be complementary and are valuable databases for the Spanish National Rare Diseases Registry when MNDs are properly codified. Nevertheless, it would be advisable to corroborate the validity of the MR as data source since the miscoding of progressive supranuclear palsy has been corrected.

  5. The Danish National Registry for Biological Therapy in Inflammatory Bowel Disease

    PubMed Central

    Larsen, Lone; Jensen, Michael Dam; Larsen, Michael Due; Nielsen, Rasmus Gaardskær; Thorsgaard, Niels; Vind, Ida; Wildt, Signe; Kjeldsen, Jens

    2016-01-01

    Aim The aims of The Danish National Registry for Biological Therapy in Inflammatory Bowel Disease are to ensure that biological therapy and the clinical management of patients with inflammatory bowel disease (IBD) receiving biological treatment are in accordance with the national clinical guidelines and, second, the database allows register-based clinical epidemiological research. Study population The study population comprises all Danish patients with IBD (both children and adults) with ulcerative colitis, Crohn’s disease, and IBD unclassified who receive biological therapy. Patients will be enrolled consecutively when biological treatment is initiated. Main variables The variables in the database are: diagnosis, time of diagnosis, disease manifestation, indication for biological therapy, previous biological and nonbiological therapy, date of visit, clinical indices, physician’s global assessment, pregnancy and breastfeeding (women), height (children), weight, dosage (current biological agent), adverse events, surgery, endoscopic procedures, and radiology. Descriptive data Eleven clinical indicators have been selected to monitor the quality of biological treatment. For each indicator, a standard has been defined based on the available evidence. National results will be published in an annual report and local results on a quarterly basis. The indicators will be reported as department-specific proportions with 95% confidence intervals, and the national average will be provided for comparison. An estimated 1,200–1,300 new biological therapies are initiated each year in Danish patients with IBD. Conclusion The database will be available for research during 2016. Data will be made available by The Danish Clinical Registries (www.rkkp.dk). PMID:27822107

  6. 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…

  7. Validation of diagnostic codes for Charcot–Marie–Tooth disease in the Danish National Patient Registry

    PubMed Central

    Vaeth, Signe; Jensen, Uffe Birk; Christensen, Rikke; Andersen, Henning

    2016-01-01

    Purpose To validate the diagnostic codes for Charcot–Marie–Tooth disease (CMT) in the Danish National Patient Registry (DNPR) using positive predictive value (PPV) as a measure of validity. Patients and methods We used the DNPR to identify all patients diagnosed with at least one primary CMT diagnosis at a specialized department in the Central Denmark Region during the period 1977–2012. From this population, we randomly selected 123 patients for the validation study. Medical files were reviewed and used as reference standard. We estimated the PPV of the CMT diagnoses and stratified the analysis according to age at diagnosis, gender, and calendar time. Results In the DNPR, 275 patients were identified. We were able to retrieve 96 medical files from the random sample of 123 patients, and 85 CMT diagnoses were confirmed. The average age at diagnosis was 42.5 years, and 34% were female. The PPV was 88.5% (95% confidence interval: 80.4–94.1). Conclusion The CMT diagnoses in the DNPR have high validity. The DNPR can be used as a data source for epidemiologic research on CMT. PMID:27920579

  8. If you build a rare disease registry, will they enroll and will they use it? Methods and data from the National Registry of Myotonic Dystrophy (DM) and Facioscapulohumeral Muscular Dystrophy (FSHD)

    PubMed Central

    Hilbert, James E.; Kissel, John T.; Luebbe, Elizabeth A.; Martens, William B.; McDermott, Michael P.; Sanders, Donald B.; Tawil, Rabi; Thornton, Charles A.; Moxley, Richard T.

    2011-01-01

    Introduction Registries are becoming increasingly important for rare diseases as experimental therapies develop. This report describes the methodology behind the National Registry of Myotonic Dystrophy (DM) and Facioscapulohumeral Muscular Dystrophy (FSHD) Patients and Family Members to facilitate the development of other rare disease registries. We also highlight data about the pathophysiology and select burdens of DM and FSHD reported at baseline and longitudinally. Methods The Registry consists of de-identified, patient reported information collected at baseline and annually and information from review of medical records. Investigators can use the Registry to analyze de-identified data and to facilitate recruitment into clinical studies. Results To date, the Registry has enrolled 1611 members, facilitated 24 studies, and collected data annually for up to 8 years. Genetic test results were obtained in 56.2% of enrollees. Approximately one-third of members used assistive devices and another one-third reported psychological problems at baseline. Wheelchair use was reported for both short and long distances by 7.0% of DM and 18.1% of FSHD members. Approximately 60% of members reported their employment was affected by their disease. Conclusions Strengths of the Registry include large sample sizes, stringent review of clinical and molecular data, annually updated information, and regular interactions between patients and investigators. Registry data provide new insights into the burdens of DM and FSHD, such as, psychological problems and reduced employment. Opportunities abound for investigators to utilize Registry resources to assess the impact of these and other burdens on health care costs, progression of symptoms, and quality of life. PMID:22155025

  9. Iranian Joint Registry (Iranian National Hip and Knee Arthroplasty Registry)

    PubMed Central

    Aslani, Hamidreza; Nourbakhsh, Seyed Taghi; Lahiji, Farivar A.; Heydarian, Keykavoos; Jabalameli, Mahmood; Ghazavi, Mohammad Taghi; Tahmasebi, Mohammad Naghi; Fayyaz, Mahmoud Reza; Sazegari, Mohammad Ali; Mohaddes, Maziar; Rajabpour, Mojtaba; Emami, Mohammad; Jazayeri, Seyyed Mohammad; Madadi, Firooz; Farahini, Hossein; Mirzatoloee, Fardin; Gharahdaghi, Mohammad; Ebrahimzadeh, Mohammad Hossein; Ebrahimian, Mohammadreza; Mirvakili, Hossein; Bashti, Kaveh; Almasizadeh, Mohtasham; Abolghasemian, Mansour; Taheriazam, Afshin; Motififard, Mehdi; Yazdi, Hamidreza; Mobarakeh, Mahmood Karimi; Shayestehazar, Masoud; Moghtadae, Mehdi; Siavashi, Babak; Sajjadi, Mohammadreza M.; Rasi, Alireza Manafi; Chabok, Seyyed Kazem; Zafarani, Zohreh; Salehi, Shahin; Ahmadi, Monireh; Mohammadi, Amin; Shahsavand, Mohammad Ebrahim

    2016-01-01

    Periodic evaluation and monitoring the health and economic outcome of joint replacement surgery is a common and popular process under the territory of joint registries in many countries. In this article we introduce the methodology used for the foundation of the National Iranian Joint Registry (IJR) with a joint collaboration of the Social Security Organization (SSO) and academic research departments considering the requirements of the Iran’s Ministry of Health and Education. PMID:27200403

  10. Iranian Joint Registry (Iranian National Hip and Knee Arthroplasty Registry).

    PubMed

    Aslani, Hamidreza; Nourbakhsh, Seyed Taghi; Lahiji, Farivar A; Heydarian, Keykavoos; Jabalameli, Mahmood; Ghazavi, Mohammad Taghi; Tahmasebi, Mohammad Naghi; Fayyaz, Mahmoud Reza; Sazegari, Mohammad Ali; Mohaddes, Maziar; Rajabpour, Mojtaba; Emami, Mohammad; Jazayeri, Seyyed Mohammad; Madadi, Firooz; Farahini, Hossein; Mirzatoloee, Fardin; Gharahdaghi, Mohammad; Ebrahimzadeh, Mohammad Hossein; Ebrahimian, Mohammadreza; Mirvakili, Hossein; Bashti, Kaveh; Almasizadeh, Mohtasham; Abolghasemian, Mansour; Taheriazam, Afshin; Motififard, Mehdi; Yazdi, Hamidreza; Mobarakeh, Mahmood Karimi; Shayestehazar, Masoud; Moghtadae, Mehdi; Siavashi, Babak; Sajjadi, Mohammadreza M; Rasi, Alireza Manafi; Chabok, Seyyed Kazem; Zafarani, Zohreh; Salehi, Shahin; Ahmadi, Monireh; Mohammadi, Amin; Shahsavand, Mohammad Ebrahim

    2016-04-01

    Periodic evaluation and monitoring the health and economic outcome of joint replacement surgery is a common and popular process under the territory of joint registries in many countries. In this article we introduce the methodology used for the foundation of the National Iranian Joint Registry (IJR) with a joint collaboration of the Social Security Organization (SSO) and academic research departments considering the requirements of the Iran's Ministry of Health and Education.

  11. The Corrona US registry of rheumatic and autoimmune diseases.

    PubMed

    Kremer, Joel M

    2016-01-01

    The Corrona US national registry collects data concerning patient status from both the rheumatologist and patient at routine clinical encounters. Corrona has functioning disease registries in rheumatoid arthritis, psoriatic arthritis, spondyloarthropathies, psoriasis and inflammatory bowel disease. Corrona merges data concerning long-term effectiveness and safety, as well as comparative and cost effectiveness of agents to treat these autoimmune diseases.

  12. Innovative measures to combat rare diseases in China: The national rare diseases registry system, larger-scale clinical cohort studies, and studies in combination with precision medicine research

    PubMed Central

    Song, Peipei; He, Jiangjiang; Li, Fen; Jin, Chunlin

    2017-01-01

    Summary China is facing the great challenge of treating the world's largest rare disease population, an estimated 16 million patients with rare diseases. One effort offering promise has been a pilot national project that was launched in 2013 and that focused on 20 representative rare diseases. Another government-supported special research program on rare diseases – the “Rare Diseases Clinical Cohort Study” – was launched in December 2016. According to the plan for this research project, the unified National Rare Diseases Registry System of China will be established as of 2020, and a large-scale cohort study will be conducted from 2016 to 2020. The project plans to develop 109 technical standards, to establish and improve 2 national databases of rare diseases – a multi-center clinical database and a biological sample library, and to conduct studies on more than 50,000 registered cases of 50 different rare diseases. More importantly, this study will be combined with the concept of precision medicine. Chinese population-specific basic information on rare diseases, clinical information, and genomic information will be integrated to create a comprehensive predictive model with a follow-up database system and a model to evaluate prognosis. This will provide the evidence for accurate classification, diagnosis, treatment, and estimation of prognosis for rare diseases in China. Numerous challenges including data standardization, protecting patient privacy, big data processing, and interpretation of genetic information still need to be overcome, but research prospects offer great promise. PMID:28357175

  13. The Danish National Multiple Myeloma Registry

    PubMed Central

    Gimsing, Peter; Holmström, Morten O; Klausen, Tobias Wirenfelt; Andersen, Niels Frost; Gregersen, Henrik; Pedersen, Robert Schou; Plesner, Torben; Pedersen, Per Trøllund; Frederiksen, Mikael; Frølund, Ulf; Helleberg, Carsten; Vangsted, Annette; de Nully Brown, Peter; Abildgaard, Niels

    2016-01-01

    Aim The Danish National Multiple Myeloma Registry (DMMR) is a population-based clinical quality database established in January 2005. The primary aim of the database is to ensure that diagnosis and treatment of plasma cell dyscrasia are of uniform quality throughout the country. Another aim is to support research. Patients are registered with their unique Danish personal identification number, and the combined use of DMMR, other Danish National registries, and the Danish National Cancer Biobank offers a unique platform for population-based translational research. Study population All newly diagnosed patients with multiple myeloma (MM), smoldering MM, solitary plasmacytomas, and plasma cell leukemia in Denmark are registered annually; ~350 patients. Amyloid light-chain amyloidosis, POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome), monoclonal gammopathy of undetermined significance and monoclonal gammopathy of undetermined significance with polyneuropathy have been registered since 2014. Main variables The main registered variables at diagnosis are patient demographics, baseline disease characteristics, myeloma-defining events, clinical complications, prognostics, first- and second-line treatments, treatment responses, progression free, and overall survival. Descriptive data Up to June 2015, 2,907 newly diagnosed patients with MM, 485 patients with smoldering MM, 64 patients with plasma cell leukemia, and 191 patients with solitary plasmacytomas were registered. Registration completeness of new patients is ~100%. A data validation study performed in 2013–2014 by the Danish Myeloma Study Group showed >95% data correctness. Conclusion The DMMR is a population-based data validated database eligible for clinical, epidemiological, and translational research. PMID:27822103

  14. 76 FR 4092 - National Saltwater Angler Registry Program

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-01-24

    ... National Oceanic and Atmospheric Administration RIN 0648-XA131 National Saltwater Angler Registry Program... INFORMATION: The final rule implementing the National Saltwater Angler Registry Program, 50 CFR part 600... registered under a qualifying State license and/or registry program, or to provide catch and effort data...

  15. 78 FR 49478 - National Saltwater Angler Registry Program

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-08-14

    ... National Oceanic and Atmospheric Administration RIN 0648-XC403 National Saltwater Angler Registry Program... Angler Registry Program. DATES: The registration fee will be required effective August 1, 2013. ADDRESSES... final rule implementing the National Saltwater Angler Registry Program, 50 CFR part 600, subpart P,...

  16. 76 FR 22082 - National Saltwater Angler Registry Program

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-20

    ... National Oceanic and Atmospheric Administration RIN 0648-XA368 National Saltwater Angler Registry Program... INFORMATION: The final rule implementing the National Saltwater Angler Registry Program, 50 CFR part 600... registered under a qualifying state license and/or registry program, or to provide catch and effort data...

  17. National Marrow Donor Program and Be The Match Registry

    MedlinePlus

    ... National Marrow Donor Program and Be The Match Registry Past Issues / Summer 2011 Table of Contents Creating ... Donor Program (NMDP) and its Be The Match Registry are nonprofit organizations dedicated to creating an opportunity ...

  18. 76 FR 38619 - Proposed Information Collection; Comment Request; National Saltwater Angler Registry and State...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-07-01

    ... Saltwater Angler Registry and State Exemption Program AGENCY: National Oceanic and Atmospheric... currently approved collection. The National Saltwater Angler Registry Program (Registry Program) was... fisheries surveys, including establishing a national saltwater angler and for-hire vessel registry,...

  19. National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis.

    PubMed

    Constantin, T; Ponyi, A; Orbán, I; Molnár, K; Dérfalvi, B; Dicso, F; Kálovics, T; Müller, J; Garami, M; Sallai, A; Balogh, Z; Szalai, Z; Fekete, G; Dankó, K

    2006-05-01

    Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease

  20. Inherited Retinal Degenerative Disease Registry

    ClinicalTrials.gov

    2016-03-21

    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

  1. Alaska Native Parkinson’s Disease Registry

    DTIC Science & Technology

    2010-11-01

    1-0001 Brian A Trimble, MD Alaska Native Parkinson’s Disease Registry Principal Investigator A. Introduction Parkinsonism (PS) is a syndrome...characterized by tremor , rigidity, slowness of movement, and problems with walking and balance. Parkinson’s disease is the most common form of PS... parkinsonism cases will be the Indian Health Service (IHS) provider database, called the Resource and Patient Management System (RPMS), but the protocol will

  2. Alaska Native Parkinson’s Disease Registry

    DTIC Science & Technology

    2009-11-01

    W81XWH-07-1-0001 Brian A Trimble, MD Alaska Native Parkinson’s Disease Registry Principal Investigator A. Introduction Parkinsonism (PS) is a...syndrome characterized by tremor , rigidity, slowness of movement, and problems with walking and balance. Parkinson’s disease is the most common form...protocol. The primary source of parkinsonism cases will be the Indian Health Service (IHS) provider database, called the Resource and Patient Management

  3. 76 FR 9578 - National Center for Environmental Health/Agency for Toxic Substances and Disease Registry (NCEH...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-18

    ... Health and Chemical Exposures Leadership Council. The National Conversation on Public Health and Chemical... an action agenda for strengthening the nation's approach to protecting the public's health from... National Conversation on Public Health and Chemical Exposures, visit this Web site:...

  4. The National Film Registry: Acquiring Our Film Heritage.

    ERIC Educational Resources Information Center

    Ziegler, Roy A.

    The National Film Registry, which is primarily a designated list of films to be preserved by the Library of Congress, is also a valuable tool for selecting "films that are culturally, historically, and aesthetically significant." Following a brief discussion of the history and selection process of the National Film Registry, Southeast…

  5. 77 FR 24103 - National Registry of Certified Medical Examiners

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-04-20

    ...FMCSA establishes a National Registry of Certified Medical Examiners (National Registry) with requirements that all medical examiners who conduct physical examinations for interstate commercial motor vehicle (CMV) drivers meet the following criteria: Complete certain training concerning FMCSA's physical qualification standards, pass a test to verify an understanding of those standards, and......

  6. Alaska Native Parkinson’s Disease Registry

    DTIC Science & Technology

    2013-07-01

    07-1-0001 TITLE: Alaska Native Parkinson’s Disease Registry PRINCIPAL INVESTIGATOR: Caroline M. Tanner, M.D...The views, opinions and/or findings contained in this report are those of the author( s ) and should not be construed as an official Department...GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR( S ) 5d. PROJECT NUMBER 5e. TASK NUMBER E-Mail: 5f. WORK UNIT NUMBER 7. PERFORMING

  7. Policies and procedures for establishing a national registry of persons exposed to hazardous substances (National Exposure Registry)

    SciTech Connect

    Not Available

    1988-01-01

    In the document, the Agency for Toxic Substances and Disease Registry (ATSDR) procedures for meeting the statutory mandate for a registry of persons exposed to hazardous substances are described. The rationale for and purpose of the Exposure Registry is to address health issues at hazardous waste sites and emergency chemical spills. The registry activity specifically addresses long-term health issues related to the exposures of hazardous substances and/or mixtures of hazardous substances. The creation of the registry is meant to provide--through the creation of large, valid data files--information needed by researchers to elucidate the presence or absence of the long-term health effects.

  8. Cancer registries in Japan: National Clinical Database and site-specific cancer registries.

    PubMed

    Anazawa, Takayuki; Miyata, Hiroaki; Gotoh, Mitsukazu

    2015-02-01

    The cancer registry is an essential part of any rational program of evidence-based cancer control. The cancer control program is required to strategize in a systematic and impartial manner and efficiently utilize limited resources. In Japan, the National Clinical Database (NCD) was launched in 2010. It is a nationwide prospective registry linked to various types of board certification systems regarding surgery. The NCD is a nationally validated database using web-based data collection software; it is risk adjusted and outcome based to improve the quality of surgical care. The NCD generalizes site-specific cancer registries by taking advantage of their excellent organizing ability. Some site-specific cancer registries, including pancreatic, breast, and liver cancer registries have already been combined with the NCD. Cooperation between the NCD and site-specific cancer registries can establish a valuable platform to develop a cancer care plan in Japan. Furthermore, the prognosis information of cancer patients arranged using population-based and hospital-based cancer registries can help in efficient data accumulation on the NCD. International collaboration between Japan and the USA has recently started and is expected to provide global benchmarking and to allow a valuable comparison of cancer treatment practices between countries using nationwide cancer registries in the future. Clinical research and evidence-based policy recommendation based on accurate data from the nationwide database may positively impact the public.

  9. 75 FR 16488 - National Center for Environmental Health/Agency for Toxic Substances and Disease Registry (NCEH...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-01

    ... National Conversation on Public Health and Chemical Exposures Leadership Council, which is convened by RESOLVE, a non-profit independent facilitator. The National Conversation on Public Health and Chemical... public's health from harmful chemical exposures. The Leadership Council provides overall guidance to...

  10. 75 FR 59727 - National Center for Environmental Health/Agency for Toxic Substances and Disease Registry (NCEH...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-28

    ... sixth meeting of the National Conversation on Public ] Health and Chemical Exposures Leadership Council... approach to protecting the public's health from harmful chemical exposures. The Leadership Council provides... agenda. For additional information on the National Conversation on Public Health and Chemical...

  11. 75 FR 75474 - National Center for Environmental Health/Agency for Toxic Substances and Disease Registry (NCEH...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-12-03

    ... seventh meeting of the National Conversation on Public Health and Chemical Exposures Leadership Council... approach to protecting the public's health from harmful chemical exposures. The Leadership Council provides... agenda. For additional information on the National Conversation on Public Health and Chemical...

  12. Portuguese National Registry on Cardiac Electrophysiology, 2013 and 2014.

    PubMed

    Cavaco, Diogo; Morgado, Francisco; Bonhorst, Daniel

    2016-01-01

    The authors present the results of the national registry of electrophysiology of the Portuguese Association for Arrhythmology, Pacing and Electrophysiology (APAPE) for 2013 and 2014. The registry is annual and voluntary, and data are collected retrospectively. Data for electrophysiological studies, ablations and cardioverter-defibrillator implantations for 2013 and 2014 are presented. Developments over the years and their implications are analyzed and discussed.

  13. 75 FR 41505 - National Center for Environmental Health/Agency for Toxic Substances and Disease Registry (NCEH...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-07-16

    ... on Public Health and Chemical Exposures Leadership Council Meeting Time and Date: 1 p.m.-5 p.m. EDT... National Conversation on Public Health and Chemical Exposures Leadership Council, which is convened by... protecting the public's health from harmful chemical exposures. The Leadership Council provides...

  14. [Legislation of cancer registries in Japan- an outline of the national cancer registry].

    PubMed

    Nishino, Yoshikazu

    2015-04-01

    The national cancer registry in Japan will commence operations in January 2016 under the Cancer Registry Promotion Act, which was established in December 2013. Although data on cancer incidence and survival rates in Japan have been available for limited regions for a long time, accurate nationwide data obtained from the national cancer registry database will contribute to the planning and evaluation of cancer control in Japan. It is expected that this database will be utilized in evaluating the quality of medical care for cancer patients, in assessing the accuracy of cancer screening, and in follow-up surveys in nationwide cohort studies. Furthermore, under the Cancer Registry Promotion Act, hospitals will be permitted to obtain vital patient information from data registered in the national cancer registry database, which will promote the publication of survival rates for cancer patients and accelerate research at hospitals. The founding of the Japanese national cancer registry is a landmark development in the promotion of cancer control and cancer research in Japan and it is essential that the Japanese population benefits from the information obtained from this database.

  15. The National Anesthesia Clinical Outcomes Registry.

    PubMed

    Liau, Adrian; Havidich, Jeana E; Onega, Tracy; Dutton, Richard P

    2015-12-01

    The Anesthesia Quality Institute (AQI) was chartered in 2008 by the American Society of Anesthesiologists to develop the National Anesthesia Clinical Outcomes Registry (NACOR). In this Technical Communication, we will describe how data enter NACOR, how they are authenticated, and how they are analyzed and reported. NACOR accepts case-level administrative, clinical, and quality capture data from voluntarily participating anesthesia practices and health care facilities in the United States. All data are transmitted to the AQI in summary electronic files generated by billing, quality capture, and electronic health care record software, typically on a monthly basis. All data elements are mapped to fields in the NACOR schema in accordance with a publicly available data dictionary. Incoming data are loaded into NACOR by AQI technologists and are subject to both manual and automated review to identify systematically missing elements, miscoding, and inadvertent corruption. Data are deidentified in compliance with Health Insurance Portability and Accountability Act regulations. The database server of AQI, which houses the NACOR database, is protected by 2 firewalls within the American Society of Anesthesiologists' network infrastructure; this system has not been breached. The NACOR Participant User File, a deidentified case-level dataset of information from NACOR, is available to researchers at participating institutions. NACOR architecture and the nature of the Participant User File include both strengths and weaknesses.

  16. 75 FR 65629 - Modification of the Annual National Registry Fee

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-26

    ... From the Federal Register Online via the Government Publishing Office FEDERAL FINANCIAL... Subcommittee (ASC) of the Federal Financial Institutions Examination Council. ACTION: Notice of modification of... appraisals for federally related transactions. Through the National Registry, lenders and consumers...

  17. Positive predictive values of International Classification of Diseases, 10th revision codes for dermatologic events and hypersensitivity leading to hospitalization or emergency room visit among women with postmenopausal osteoporosis in the Danish and Swedish national patient registries

    PubMed Central

    Adelborg, Kasper; Christensen, Lotte Brix; Munch, Troels; Kahlert, Johnny; Trolle Lagerros, Ylva; Tell, Grethe S; Apalset, Ellen M; Xue, Fei; Ehrenstein, Vera

    2017-01-01

    Background Clinical epidemiology research studies, including pharmacoepidemiology and pharmacovigilance studies, use routinely collected health data, such as diagnoses recorded in national health and administrative registries, to assess clinical effectiveness and safety of treatments. We estimated positive predictive values (PPVs) of International Classification of Diseases, 10th revision (ICD-10) codes for primary diagnoses of dermatologic events and hypersensitivity recorded at hospitalization or emergency room visit in the national patient registries of Denmark and Sweden among women with postmenopausal osteoporosis (PMO). Methods This validation study included women with PMO identified from the Danish and Swedish national patient registries (2005–2014). Medical charts of the potential cases served as the gold standard for the diagnosis confirmation and were reviewed and adjudicated by physicians. Results We obtained and reviewed 189 of 221 sampled medical records (86%). The overall PPV was 92.4% (95% confidence interval [CI], 85.1%–96.3%) for dermatologic events, while the PPVs for bullous events and erythematous dermatologic events were 52.5% (95% CI, 37.5%–67.1%) and 12.5% (95% CI, 2.2%–47.1%), respectively. The PPV was 59.0% (95% CI, 48.3%–69.0%) for hypersensitivity; however, the PPV of hypersensitivity increased to 100.0% (95% CI, 67.6%–100.0%) when restricting to diagnostic codes for anaphylaxis. The overall results did not vary by country. Conclusion Among women with PMO, the PPV for any dermatologic event recorded as the primary diagnosis at hospitalization or at an emergency room visit was high and acceptable for epidemiologic research in the Danish and Swedish national patient registries. The PPV was substantially lower for hypersensitivity leading to hospitalization or emergency room visit.

  18. National exposure registry: Tichloroethylene (TCE) subregistry (on CD-ROM). Data file

    SciTech Connect

    Not Available

    1995-01-01

    The 1994 National Exposure Registry: Trichloroethylene (TCE) Subregistry' contains data on approximately 4,000 persons and is the first CD-ROM product released in the National Exposure Registry Series. This CD-ROM is composed of three files: Demographics and Health, Mortality, and Environmental. The CD-ROM includes access software, the Statistical Export and Tabulation System (SETS). The TCE subregistry is one of three (dioxin and benzene subregistries are the other two). The National Exposure Registry was created in response to a mandate given in the Comprehensive Environmental Response, Compensation and Liability Act (CERCLA) of 1980 to the Agency for Toxic Substances and Disease Registry (ATSDR) to create a registry of persons exposed to hazardous substances. This mandate was reiterated in the Superfund Amendments and Reauthorization Act (SARA) of 1986. The National Exposure Registry is a database composed of names of persons, along with additional information on these persons, with documented exposure to specific chemicals. The purpose of the Registry is to aid in assessing the long-term health consequences of low-level, long-term exposure to environmental contaminants. One of the goals is to establish a database that will furnish the information needed to generate appropriate and valid hypotheses for future activities such as epidemiological studies. When supplemented with additional data, the combined file can then be used to carry out hypothesis-testing epidemiological investigations. The data collected for each member of the Registry include environmental levels, demographic information, smoking and occupational history, and self-reported responses to 25 general health status questions. The files for each chemical-specific subregistry are established at the time baseline data are collected and are updated and maintained by ATSDR on an ongoing basis (annually the first year, biennially thereafter).

  19. National Adult Cardiac Surgery Registry: past, present and future.

    PubMed

    Uva, Miguel Sousa; Mota, João Carlos

    2003-10-01

    A task force commission was created with the support of the Portuguese Society for Cardiothoracic and Vascular Surgery with the aim of organizing a National Adult Cardiac Surgery Registry, collecting clinical data and types of cardiac surgical procedure performed in Portugal. Selected variables include risk factors, cardiac status, preoperative hemodynamics, surgical procedure, hospital stay and mortality. Information is collected into a database in each institution and sent via the internet to a central database responsible for grouping and data analysis. It is hoped that this National Registry, through standardized data collection, will provide information on cardiac surgery activity in Portugal and its risk adjusted results.

  20. 76 FR 14366 - National Registry of Certified Medical Examiners

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-16

    ... Certified Medical Examiners AGENCY: Federal Motor Carrier Safety Administration, Transportation. ACTION... National Registry of Certified Medical Examiners (NRCME) published on December 1, 2008. In the comments on the NPRM, a commenter inquired as to what a motor carrier had to do to verify that a medical...

  1. The NAS-NRC Twin Registry of WWII military veteran twins. National Academy of Sciences-National Research Council.

    PubMed

    Page, William F

    2002-10-01

    The NAS-NRC Twin Registry is one of the oldest, national population based-twin registries in the United States. It consists of 15,924 white male twin pairs born in the years 1917-1927 (inclusive) both of whom served in the armed forces. The registry, which has been in operation more than 30 years, has collected data from a variety of sources. Records-based, computerized data have come largely from the Department of Veterans Affairs, and there have been three major epidemiologic questionnaires, undertaken roughly every 15 years. Classic twin studies on a variety of medical conditions were the early focus of the registry, which now has a strong focus on chronic disease epidemiology. Work on a DNA specimen bank has been proceeding slowly, but is now a top priority, due to the increasing force of mortality in this twin cohort.

  2. 49 CFR 391.42 - Schedule for use of medical examiners listed on the National Registry of Certified Medical...

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... the National Registry of Certified Medical Examiners. 391.42 Section 391.42 Transportation Other... examiners listed on the National Registry of Certified Medical Examiners. On and after May 21, 2014, each... the National Registry of Certified Medical Examiners....

  3. 49 CFR 391.42 - Schedule for use of medical examiners listed on the National Registry of Certified Medical...

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... the National Registry of Certified Medical Examiners. 391.42 Section 391.42 Transportation Other... examiners listed on the National Registry of Certified Medical Examiners. On and after May 21, 2014, each... the National Registry of Certified Medical Examiners....

  4. 49 CFR 391.42 - Schedule for use of medical examiners listed on the National Registry of Certified Medical...

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... the National Registry of Certified Medical Examiners. 391.42 Section 391.42 Transportation Other... examiners listed on the National Registry of Certified Medical Examiners. On and after May 21, 2014, each... the National Registry of Certified Medical Examiners....

  5. 76 FR 57742 - National Registry of Evidence-Based Programs and Practices

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-09-16

    ... HUMAN SERVICES National Registry of Evidence-Based Programs and Practices AGENCY: Substance Abuse and Mental Health Services Administration, HHS. ACTION: Notice Regarding Substance Abuse and Mental Health Services Administration's National Registry of Evidence-based Programs and Practices (NREPP):...

  6. [Primary Spinal Tumor Registry at the National Centre for Spinal Disorders].

    PubMed

    Szövérfi, Zsolt; Lazáry, Aron; Varga, Péter Pál

    2014-05-11

    Primary spinal tumors are rare diseases. Primary spinal tumor registry would be useful to help decision making in this complex field of spine surgery. In this article the authors present the latest findings from the Primary Spinal Tumor Registry at the National Centre for Spinal Disorders, Hungary. The registry is based on a novel database management software, the REDCap electronic data capture system. It contains data of 323 patients treated surgically during an 18-year period. Among the 126 malignant tumors, the most frequent was chordoma (61 cases). In the case of benign tumors schwannoma showed the largest prevalence (45 cases). The authors conclude that due to the rarity of the disease and the complexity of the management, multicenter, prospective registries are required to provide high level of evidence. The structure of the Primary Spinal Tumor Registry in the National Centre for Spinal Disorders in Hungary is optimal for user-friendly, fast and secure data collection providing a prospective database for scientific researches and clinical follow-up.

  7. Alaska Native Parkinson’s Disease Registry

    DTIC Science & Technology

    2011-06-01

    Investigator Parkinsonism (PS) is a syndrome characterized by tremor , rigidity, slowness of movement, and problems with walking and balance...2. Developing an identification protocol. The primary source of parkinsonism cases will be the Indian Health Service (IHS) provider database, called...of parkinsonism among Alaska Natives. Status: Complete 3. Developing a secure Alaska Native parkinsonism registry database. Status: The database

  8. 49 CFR 390.115 - Procedure for removal from the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 49 Transportation 5 2012-10-01 2012-10-01 false Procedure for removal from the National Registry... MOTOR CARRIER SAFETY REGULATIONS FEDERAL MOTOR CARRIER SAFETY REGULATIONS; GENERAL National Registry of Certified Medical Examiners § 390.115 Procedure for removal from the National Registry of Certified...

  9. 49 CFR 390.115 - Procedure for removal from the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 49 Transportation 5 2014-10-01 2014-10-01 false Procedure for removal from the National Registry... MOTOR CARRIER SAFETY REGULATIONS FEDERAL MOTOR CARRIER SAFETY REGULATIONS; GENERAL National Registry of Certified Medical Examiners § 390.115 Procedure for removal from the National Registry of Certified...

  10. 49 CFR 390.115 - Procedure for removal from the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 5 2013-10-01 2013-10-01 false Procedure for removal from the National Registry... MOTOR CARRIER SAFETY REGULATIONS FEDERAL MOTOR CARRIER SAFETY REGULATIONS; GENERAL National Registry of Certified Medical Examiners § 390.115 Procedure for removal from the National Registry of Certified...

  11. 49 CFR 390.113 - Reasons for removal from the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 5 2013-10-01 2013-10-01 false Reasons for removal from the National Registry of... MOTOR CARRIER SAFETY REGULATIONS FEDERAL MOTOR CARRIER SAFETY REGULATIONS; GENERAL National Registry of Certified Medical Examiners § 390.113 Reasons for removal from the National Registry of Certified...

  12. 49 CFR 390.113 - Reasons for removal from the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 49 Transportation 5 2014-10-01 2014-10-01 false Reasons for removal from the National Registry of... MOTOR CARRIER SAFETY REGULATIONS FEDERAL MOTOR CARRIER SAFETY REGULATIONS; GENERAL National Registry of Certified Medical Examiners § 390.113 Reasons for removal from the National Registry of Certified...

  13. 75 FR 68604 - National Saltwater Angler Registry Program Designation of Exempted States

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-08

    ... National Oceanic and Atmospheric Administration RIN 0648-XZ91 National Saltwater Angler Registry Program... INFORMATION: The final rule implementing the National Saltwater Angler Registry Program, 50 CFR Subpart P, was... under a qualifying state license and/or registry program, or to provide catch and effort data from...

  14. 49 CFR 390.113 - Reasons for removal from the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 49 Transportation 5 2012-10-01 2012-10-01 false Reasons for removal from the National Registry of... MOTOR CARRIER SAFETY REGULATIONS FEDERAL MOTOR CARRIER SAFETY REGULATIONS; GENERAL National Registry of Certified Medical Examiners § 390.113 Reasons for removal from the National Registry of Certified...

  15. Cohort Profile: The National Academy of Sciences-National Research Council Twin Registry (NAS-NRC Twin Registry).

    PubMed

    Gatz, Margaret; Harris, Jennifer R; Kaprio, Jaakko; McGue, Matt; Smith, Nicholas L; Snieder, Harold; Spiro, Avron; Butler, David A

    2015-06-01

    The National Academy of Sciences-National Research Council Twin Registry (NAS-NRC Twin Registry) is a comprehensive registry of White male twin pairs born in the USA between 1917 and 1927, both of the twins having served in the military. The purpose was medical research and ultimately improved clinical care. The cohort was assembled in the early 1960s with identification of approximately 16,000 twin pairs, review of service records, a brief mailed questionnaire assessing zygosity, and a health survey largely comparable to questionnaires used at that time with Scandinavian twin registries. Subsequent large-scale data collection occurred in 1974, 1985 and 1998, repeating the health survey and including information on education, employment history and earnings. Self-reported data have been supplemented with mortality, disability and medical data through record linkage. Potential collaborators should access the study website [http://www.iom.edu/Activities/Veterans/TwinsStudy.aspx] or e-mail the Medical Follow-up Agency at [Twins@nas.edu]. Questionnaire data are being prepared for future archiving with the National Archive of Computerized Data on Aging (NACDA) at the Inter-University Consortium for Political and Social Research (ICPSR), University of Michigan, MI.

  16. Inception of a national multidisciplinary registry for stereotactic radiosurgery.

    PubMed

    Sheehan, Jason P; Kavanagh, Brian D; Asher, Anthony; Harbaugh, Robert E

    2016-01-01

    Stereotactic radiosurgery (SRS) represents a multidisciplinary approach to the delivery of ionizing high-dose radiation to treat a wide variety of disorders. Much of the radiosurgical literature is based upon retrospective single-center studies along with a few randomized controlled clinical trials. More timely and effective evidence is needed to enhance the consistency and quality of and clinical outcomes achieved with SRS. The authors summarize the creation and implementation of a national SRS registry. The American Association of Neurological Surgeons (AANS) through NeuroPoint Alliance, Inc., started a successful registry effort with its lumbar spine initiative. Following a similar approach, the AANS and NeuroPoint Alliance collaborated with corporate partners and the American Society for Radiation Oncology to devise a data dictionary for an SRS registry. Through administrative and financial support from professional societies and corporate partners, a framework for implementation of the registry was created. Initial plans were devised for a 3-year effort encompassing 30 high-volume SRS centers across the country. Device-specific web-based data-extraction platforms were built by the corporate partners. Data uploaders were then used to port the data to a common repository managed by Quintiles, a national and international health care trials company. Audits of the data for completeness and veracity will be undertaken by Quintiles to ensure data fidelity. Data governance and analysis are overseen by an SRS board comprising equal numbers of representatives from the AANS and NeuroPoint Alliance. Over time, quality outcome assessments and post hoc research can be performed to advance the field of SRS. Stereotactic radiosurgery offers a high-technology approach to treating complex intracranial disorders. Improvements in the consistency and quality of care delivered to patients who undergo SRS should be afforded by the national registry effort that is underway.

  17. 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

  18. Alaska Native Parkinson’s Disease Registry

    DTIC Science & Technology

    2012-07-01

    Investigator 4 A. Introduction Parkinsonism (PS) is a syndrome characterized by tremor , rigidity, slowness of movement, and problems with walking...2011. The aims of this project are: Specific Aim 1: Identify cases of parkinsonism among Alaska Native people and populate a secure electronic...registry database. Specific Aim 2: Provide education on parkinsonism and its treatment to primary care physicians and other health care providers

  19. Alaska Native Parkinson’s Disease Registry

    DTIC Science & Technology

    2007-11-01

    Questionable 0 DK f. seborrheic dermatitis 0 Yes 0 No 0 Questionable 0 DK Exclusion criteria O Prominent postural instability in the first 3...4 A. Introduction Parkinsonism (PS) is a syndrome characterized by tremor, rigidity, slowness of movement, and problems with walking and balance...the Alaska Native Medical Center. B. Body The intent of this proposal is to establish a registry of parkinsonism cases among Alaska native

  20. 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

    2017-01-27

    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.

  1. 75 FR 53953 - The National Saltwater Angler Registry Program; Designation of Exempted States for Anglers, Spear...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-02

    ... National Oceanic and Atmospheric Administration RIN 0648-XX66 The National Saltwater Angler Registry... Registry Program, 50 CFR subpart P, was published in the Federal Register on December 30, 2008. The final... numbers of the persons licensed or registered under a qualifying state license and/or registry program,...

  2. 77 FR 16471 - Pipeline Safety: Implementation of the National Registry of Pipeline and Liquefied Natural Gas...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-03-21

    ...: Implementation of the National Registry of Pipeline and Liquefied Natural Gas Operators AGENCY: Pipeline and... registry of pipeline and liquefied natural gas operators. This notice provides updates to the information... and liquefied natural gas (LNG) operators. New operators use the national registry to obtain...

  3. 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.

  4. Developing National Cancer Registration in Developing Countries – Case Study of the Nigerian National System of Cancer Registries

    PubMed Central

    Jedy-Agba, Elima E.; Oga, Emmanuel A.; Odutola, Michael; Abdullahi, Yusuf M.; Popoola, Abiodun; Achara, Peter; Afolayan, Enoch; Banjo, Adekunbiola Aina Fehintola; Ekanem, Ima-Obong; Erinomo, Olagoke; Ezeome, Emmanuel; Igbinoba, Festus; Obiorah, Christopher; Ogunbiyi, Olufemi; Omonisi, Abidemi; Osime, Clement; Ukah, Cornelius; Osinubi, Patience; Hassan, Ramatu; Blattner, William; Dakum, Patrick; Adebamowo, Clement A.

    2015-01-01

    The epidemiological transition in sub-Saharan Africa (SSA) has given rise to a concomitant increase in the incidence of non-communicable diseases including cancers. Worldwide, cancer registries have been shown to be critical for the determination of cancer burden, conduct of research, and in the planning and implementation of cancer control measures. Cancer registration though vital is often neglected in SSA owing to competing demands for resources for healthcare. We report the implementation of a system for representative nation-wide cancer registration in Nigeria – the Nigerian National System of Cancer Registries (NSCR). The NSCR coordinates the activities of cancer registries in Nigeria, strengthens existing registries, establishes new registries, complies and analyses data, and makes these freely available to researchers and policy makers. We highlight the key challenges encountered in implementing this strategy and how they were overcome. This report serves as a guide for other low- and middle-income countries (LMIC) wishing to expand cancer registration coverage in their countries and highlights the training, mentoring, scientific and logistic support, and advocacy that are crucial to sustaining cancer registration programs in LMIC. PMID:26284233

  5. The national alpha-1 antitrypsin deficiency registry in Poland.

    PubMed

    Chorostowska-Wynimko, Joanna; Struniawski, Radoslaw; Sliwinski, Paweł; Wajda, Beata; Czajkowska-Malinowska, Małgorzata

    2015-05-01

    The alpha-1 antitrypsin deficiency (AATD) targeted screening program, together with the National Registry, were established in Poland in 2010 soon after the AATD diagnostics became available. Between 2010 and 2014 a total of 2525 samples were collected from respiratory patients countrywide; 55 patients with severe AAT deficiency or rare mutations were identified and registered, including 36 PiZZ subjects (65%). The majority of AATD patients were diagnosed with COPD (40%) or emphysema (7%), but also with bronchial asthma (16%) and bronchiectasis (13%). Therefore, the registry has proved instrumental in setting-up the AATD-dedicated network of respiratory medical centres in Poland. Since augmentation therapy is not reimbursed in our country, the smoking cessation guidance, optimal pharmacotherapy of respiratory symptoms as well the early detection, and effective treatment of exacerbations is absolutely essential.

  6. 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

  7. International Registry for Patients With Castleman Disease

    ClinicalTrials.gov

    2016-10-21

    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

  8. 16 CFR 310.8 - Fee for access to the National Do Not Call Registry.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... Registry. 310.8 Section 310.8 Commercial Practices FEDERAL TRADE COMMISSION REGULATIONS UNDER SPECIFIC ACTS... Call Registry. (a) It is a violation of this Rule for any seller to initiate, or cause any telemarketer... National Do Not Call Registry maintained by the Commission under § 310.4(b)(1)(iii)(B); provided,...

  9. 16 CFR 310.8 - Fee for access to the National Do Not Call Registry.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... Registry. 310.8 Section 310.8 Commercial Practices FEDERAL TRADE COMMISSION REGULATIONS UNDER SPECIFIC ACTS... Call Registry. (a) It is a violation of this Rule for any seller to initiate, or cause any telemarketer... National Do Not Call Registry maintained by the Commission under § 310.4(b)(1)(iii)(B); provided,...

  10. 16 CFR 310.8 - Fee for access to the National Do Not Call Registry.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... Registry. 310.8 Section 310.8 Commercial Practices FEDERAL TRADE COMMISSION REGULATIONS UNDER SPECIFIC ACTS... Call Registry. (a) It is a violation of this Rule for any seller to initiate, or cause any telemarketer... National Do Not Call Registry maintained by the Commission under § 310.4(b)(1)(iii)(B); provided,...

  11. 16 CFR 310.8 - Fee for access to the National Do Not Call Registry.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... Registry. 310.8 Section 310.8 Commercial Practices FEDERAL TRADE COMMISSION REGULATIONS UNDER SPECIFIC ACTS... Call Registry. (a) It is a violation of this Rule for any seller to initiate, or cause any telemarketer... National Do Not Call Registry maintained by the Commission under § 310.4(b)(1)(iii)(B); provided,...

  12. 49 CFR 390.111 - Requirements for continued listing on the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... Registry of Certified Medical Examiners. 390.111 Section 390.111 Transportation Other Regulations Relating... National Registry of Certified Medical Examiners § 390.111 Requirements for continued listing on the National Registry of Certified Medical Examiners. (a) To continue to be listed on the National Registry...

  13. 49 CFR 390.111 - Requirements for continued listing on the National Registry of Certified Medical Examiners.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... Registry of Certified Medical Examiners. 390.111 Section 390.111 Transportation Other Regulations Relating... National Registry of Certified Medical Examiners § 390.111 Requirements for continued listing on the National Registry of Certified Medical Examiners. (a) To continue to be listed on the National Registry...

  14. The Italian Twin Project: from the personal identification number to a national twin registry.

    PubMed

    Stazi, Maria Antonietta; Cotichini, Rodolfo; Patriarca, Valeria; Brescianini, Sonia; Fagnani, Corrado; D'Ippolito, Cristina; Cannoni, Stefania; Ristori, Giovanni; Salvetti, Marco

    2002-10-01

    The unique opportunity given by the "fiscal code", an alphanumeric identification with demographic information on any single person residing in Italy, introduced in 1976 by the Ministry of Finance, allowed a database of all potential Italian twins to be created. This database contains up to now name, surname, date and place of birth and home address of about 1,300,000 "possible twins". Even though we estimated an excess of 40% of pseudo-twins, this still is the world's largest twin population ever collected. The database of possible twins is currently used in population-based studies on multiple sclerosis, Alzheimer's disease, celiac disease, and type 1 diabetes. A system is currently being developed for linking the database with data from mortality and cancer registries. In 2001, the Italian Government, through the Ministry of Health, financed a broad national research program on twin studies, including the establishment of a national twin registry. Among all the possible twins, a sample of 500,000 individuals are going to be contacted and we expect to enrol around 120,000 real twin pairs in a formal Twin Registry. According to available financial resources, a sub sample of the enrolled population will be asked to donate DNA. A biological bank from twins will be then implemented, guaranteeing information on future etiological questions regarding genetic and modifiable factors for physical impairment and disability, cancers, cardiovascular diseases and other age related chronic illnesses.

  15. Factors facilitating a national quality registry to aid clinical quality improvement: findings of a national survey

    PubMed Central

    Eldh, Ann Catrine; Wallin, Lars; Fredriksson, Mio; Vengberg, Sofie; Winblad, Ulrika; Halford, Christina; Dahlström, Tobias

    2016-01-01

    Objectives While national quality registries (NQRs) are suggested to provide opportunities for systematic follow-up and learning opportunities, and thus clinical improvements, features in registries and contexts triggering such processes are not fully known. This study focuses on one of the world's largest stroke registries, the Swedish NQR Riksstroke, investigating what aspects of the registry and healthcare organisations facilitate or hinder the use of registry data in clinical quality improvement. Methods Following particular qualitative studies, we performed a quantitative survey in an exploratory sequential design. The survey, including 50 items on context, processes and the registry, was sent to managers, physicians and nurses engaged in Riksstroke in all 72 Swedish stroke units. Altogether, 242 individuals were presented with the survey; 163 responded, representing all but two units. Data were analysed descriptively and through multiple linear regression. Results A majority (88%) considered Riksstroke data to facilitate detection of stroke care improvement needs and acknowledged that their data motivated quality improvements (78%). The use of Riksstroke for quality improvement initiatives was associated (R2=0.76) with ‘Colleagues’ call for local results’ (p=<0.001), ‘Management Request of Registry data’ (p=<0.001), and it was said to be ‘Simple to explain the results to colleagues’ (p=0.02). Using stepwise regression, ‘Colleagues’ call for local results’ was identified as the most influential factor. Yet, while 73% reported that managers request registry data, only 39% reported that their colleagues call for the unit's Riksstroke results. Conclusions While an NQR like Riksstroke demonstrates improvement needs and motivates stakeholders to make progress, local stroke care staff and managers need to engage to keep the momentum going in terms of applying registry data when planning, performing and evaluating quality initiatives. PMID

  16. 75 FR 38145 - Announcing the New National Electronic Job Registry for Use in the H-2A Temporary Agricultural...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-07-01

    ... disclose on the job registry information or data subject to privacy, security, or privilege limitations... Employment and Training Administration Announcing the New National Electronic Job Registry for Use in the H... that the National Electronic Job Registry (job registry) in which H-2A job orders will be posted...

  17. 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

  18. Using registries to identify adverse events in rheumatic diseases.

    PubMed

    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

    2013-11-01

    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.

  19. Observing Huntington’s Disease: the European Huntington’s Disease Network’s REGISTRY

    PubMed Central

    Orth, Michael; Handley, Olivia J; Schwenke, Carsten; Dunnett, Stephen B.; Craufurd, David; Ho, Aileen K; Wild, Edward; Tabrizi, Sarah J; Landwehrmeyer, G. Bernhard; Bonelli, Raphael M.; Herranhof, Brigitte; Hödl, Anna; Koppitz, Michael; Magnet, Markus; Otti, Daniela; Painold, Annamaria; Reisinger, Karin; Flamez, Anja; Morez, Vera; de Raedt, Sylvie; Ribaï, Pascale; Verellen-Dumoulin, Christine; Vandenberghe, Wim; van Reijen, Dimphna; Hasholt, Lis; Hjermind, Lena E.; Jakobsen, Oda; Nørremølle, Anne; Sørensen, Sven Asger; Stokholm, Jette; Peippo, Maarit; Sipponen, Marjatta; Hiivola, Heli; Martikainen, Kirsti; Tuuha, Katri; Kosinski, Christoph Michael; Probst, Daniela; Sass, Christian; Schiefer, Johannes; Schlangen, Christiane; Werner, Cornelius J.; Priller, Josef; Prüß, Harald; Andrich, Jürgen; Hoffmann, Rainer; Kraus, Peter; Prehn, Christian; Saft, Carsten; Salmen, Stephan; Straßburger, Katrin; Lange, Herwig; Hunger, Ulrike; Löhle, Matthias; Schmidt, Simone; Storch, Alexander; Wolz, Anett; Wolz, Martin; Lammbeck, Johann; Zucker, Birgit; Hidding, Ute; Münchau, Alexander; Orth, Michael; Stubbe, Lars; Heinicke, Walburgis; Orth, Michael; Longinus, Bernhard; Möller, Jens Carsten; Rissling, Ida; Peinemann, Alexander; Städtler, Michael; Weindl, Adolf; Bohlen, Stefan; Lange, Herwig; Reilmann, Ralf; Beister, Antonie; Dose, Matthias; Leythaeuser, Gabriele; Marquard, Ralf; Schrenk, Caroline; Schuierer, Michele; Wiedemann, Alexandra; Ecker, Daniel; Landwehrmeyer, Bernhard; Lezius, Franziska; Trautmann, Sonja; Bertini, Elisabetta; Mechi, Claudia; Paganini, Marco; Piacentini, Sivia; Romoli, Maria; Sorbi, Sandro; Abbruzzese, Giovanni; di Poggio, Monica Bandettini; Di Maria, Emilio; Ferrandes, Giovanna; Mandich, Paola; Marchese, Roberta; Albanese, Alberto; Di Donato, Stefano; Mariotti, Caterina; Soliveri, Paola; Carlo, Rinaldi; Luigi, Di Maio; De Michele, Giuseppe; Rinaldi, Carlo; Salvatore, Elena; Tucci, Tecla; Ciarmiello, Andrea; Martino, Tiziana; Simonelli, Maria; Squitieri, Ferdinando; Bentivoglio, Anna Rita; Fasano, Alfonso; Frontali, Marina; Guidubaldi, Arianna; Ialongo, Tamara; Jacopini, Gioia; Loria, Giovanna; Piano, Carla; Romano, Silvia; Soleti, Francesco; Spadaro, Maria; Zinzi, Paola; Heiberg, Arvid; van Walsem, Marleen R; Bjørgo, Kathrine; Fannemel, Madelein; Lars Retterstøl, Per Gørvell.; Bjørnevoll, Inga; Sando, Sigrid Botne; Sitek, Emilia Jadwiga; Slawek, Jaroslaw; Soltan, Witold; Boczarska-Jedynak, Magdalena; Jasinska-Myga, Barbara; Opala, Gregorz; Rudzińska, Monika; Szczudlik, Andrzej; Wójcik, Magdalena; Banaszkiewicz, Krzysztof; Bryl, Anna; Ciesielska, Anna; Klimberg, Aneta; Kozubski, Wojciech; Marcinkowski, Jerzy; Sempołowicz, Pani Justyna; Zielonka, Daniel; Janik, Piotr; Kalbarczyk, Anna; Kwiecinski, Hubert; Jamrozik, Zygmunt; Antczak, Jakub; Witkowski, Grzegorz; Rakowicz, Maryla; Richter, Przemyslaw; Ryglewicz, Danuta; Zaremba, Jacek; Zdzienicka, Elzbieta; Costa, Christina; Coelho, Miguel; Ferreira, Joaquim J; Mestre, Tiago; Rosa, Mário M; Valadas, Anabela; Gago, Miguel; Garrett, Carolina; Guerra, Maria Rosalia; Bas, Jordi; Calopa, Matilde; Barberà, Miquel Aguilar; Badenes, Dolores; Casas, Laura; Arroyo, Sonia Escalante; Vara, Jorge Hernández; Krupinski, Jerzy; López, Judith; Obdulia, Marta; Ferrer, Pilar Quilez; Sebastián, Ana Rojo; Contreras, Silvia Romero; Carruesco, Gemma Tome; Cubo, Esther; Mariscal, Natividad; Sánchez, Jesús; Barrero, Francisco J; Morales, Blas; López-Sendón Moreno, José Luis; García, Rocío García-Ramos; Quiroga, Purificacion Pin; Villanueva, Clara; Ruíz-Espiga, Pedro-José García; Martínez, Asunción; Artiga, María José Saiz; Sánchez, Vicenta; Bascuñana, Mónica; Fatas, Marta; Ribas, Guillermo García; de Yébenes, Justo García; López Moreno, José Luis; Schwarz, Christine; Cubillo, Patricia Trigo; Arques, Penelope Navas; Gorospe, Aranzazú; Legarda, Inés; Torres Rodríguez, María José; Gaston, Itziar; Ramos-Arroyo, Maria A.; del Val, Javier López; Martinez, Laura; Burgunder, Jean-Marc; Romero, Irene; Schüpbach, Michael; Zaugg, Sabine Weber; van Hout, Monique S.E.; van Vugt, Jeroen P.P.; de Weert, A. Marit; Bolwijn, J.J.W.; Dekker, Meike; Leenders, K.L.; van Oostrom, Joost.C.H.; Bos, Reineke; Dumas, Eve; Jurgens, Caroline K.; Roos, Raymund A.C.; Witjes-Ané, Marie-Noëlle; Matheson, Kirsty; Rae, Daniela; Simpson, Sheila; Summers, Fiona; Ure, Alexandra; Curtis, Adrienne; Keylock, Jenny; Rickards, Hugh; Wright, Jan; Barker, Roger A.; Fisher, Kate; Goodman, Anna Olivia Goyder; Hill, Susan; Kershaw, Ann; Mason, Sarah; Paterson, Nicole; Raymond, Lucy; Bisson, Jon; Busse, Monica; Ellison-Rose, Lynda; Handley, Olivia; Dunnett, SB; Naji, Jenny; Price, Kathy; Rosser, Anne; Edwards, Maureen; De Sousa, Paul A.; Hughes, Teresa; McGill, Marie; Pearson, Pauline; Porteous, Mary; Zema, Adam; Brockie, Peter; Foster, Jillian; Johns, Nicola; McKenzie, Sue; Thomas, Gareth; Burrows, Liz; Fletcher, Amy; Laver, Fiona; Silva, Mark; Thomson, Aileen; Chu, Carol; Hobson, Emma; Jamieson, Stuart; Toscano, Jean; Wild, Sue; Yardumian, Pam; Bourne, Colin; Clayton, Carole; Dipple, Heather; Grant, Janet; Gross, Diana; Hallam, Caroline; Middleton, Julia; Murch, Ann; Andrews, Thomasin; Dougherty, Andrew; Kavalier, Fred; Golding, Charlotte; Lashwood, Alison; Robertson, Dene; Ruddy, Deborah; Whaite, Anna; Andrews, Thomasin; Bruno, Stefania; Golding, Charlotte; Henley, Susie; Novak, Marianne; O'Driscoll, Christine; Patel, Aakta; Rosser, Elisabeth; Tabrizi, Sarah; Taylor, Rachel; Warner, Thomas; Wild, Edward; Arran, Natalie; Craufurd, David; Fullam, Ruth; Howard, Liz; Huson, Susan; Partington-Jones, Lucy; Ritchie, Nichola; Snowden, Julie; Solom, Annie; Stopford, Cheryl; Thompson, Jennifer; Westmoreland, Leann; Nemeth, Andrea H; Siuda, Gill; Bandmann, Oliver; Bradbury, Alyson; Fillingham, Kay; Foustanos, Isabella; Quarrell, Oliver; Reynders, Hazel; Robertson, Lisa; Tidswell, Katharine

    2011-01-01

    Background: Huntington’s disease (HD) is a rare triplet repeat (CAG) disorder. Advanced, multi-centre, multi-national research frameworks are needed to study simultaneously multiple complementary aspects of HD. This includes the natural history of HD, its management and the collection of clinical information and biosamples for research. Methods: We report on cross-sectional data of the first 1766 participants in REGISTRY, the European Huntington’s Disease Network’s (EHDN), multi-lingual, multi-national prospective observational study of HD in Europe. Data collection (demographics, phenotype, genotype, medication, co-morbidities, biosamples) followed a standard protocol. Results: Phenotype, and the HD genotype, of manifest HD participants across different European regions was similar. Motor onset was most common (48%) with a non-motor onset in more than a third of participants. Motor signs increased, and cognitive abilities and functional capacity declined as the disease burden (CAGn-35.5) X age) increased. A life-time history of behavioural symptoms was common, but the behavioural score was not related to disease burden. One fifth of participants had severe psychiatric problems, e.g. suicidal ideation and attempts, and/or irritability/aggression, with psychosis being less common. Participants on anti-dyskinetic medication had a higher motor and lower cognitive score, were older, and more prone to physical trauma. A higher motor and a lower cognitive score predicted more advanced disease. Conclusions: The unparalleled collection of clinical data and biomaterials within the EHDN’s REGISTRY can expedite the search for disease modifiers (genetic and environmental) of age at onset and disease progression that could be harnessed for the development of novel treatments. PMID:20890398

  20. Japan Renal Biopsy Registry and Japan Kidney Disease Registry: Committee Report for 2009 and 2010.

    PubMed

    Sugiyama, Hitoshi; Yokoyama, Hitoshi; Sato, Hiroshi; Saito, Takao; Kohda, Yukimasa; Nishi, Shinichi; Tsuruya, Kazuhiko; Kiyomoto, Hideyasu; Iida, Hiroyuki; Sasaki, Tamaki; Higuchi, Makoto; Hattori, Motoshi; Oka, Kazumasa; Kagami, Shoji; Kawamura, Tetsuya; Takeda, Tetsuro; Hataya, Hiroshi; Fukasawa, Yuichiro; Fukatsu, Atsushi; Morozumi, Kunio; Yoshikawa, Norishige; Shimizu, Akira; Kitamura, Hiroshi; Yuzawa, Yukio; Matsuo, Seiichi; Kiyohara, Yutaka; Joh, Kensuke; Nagata, Michio; Taguchi, Takashi; Makino, Hirofumi

    2013-04-01

    The Japan Renal Biopsy Registry (J-RBR) was started in 2007 and the Japan Kidney Disease Registry (J-KDR) was then started in 2009 by the Committee for Standardization of Renal Pathological Diagnosis and the Committee for the Kidney Disease Registry of the Japanese Society of Nephrology. The purpose of this report is to describe and summarize the registered data from 2009 and 2010. For the J-KDR, data were collected from 4,016 cases, including 3,336 (83.1 %) by the J-RBR and 680 (16.9 %) other cases from 59 centers in 2009, and from 4,681 cases including 4,106 J-RBR cases (87.7 %) and 575 other cases (12.3 %) from 94 centers in 2010, including the affiliate hospitals. In the J-RBR, 3,165 native kidneys (94.9 %) and 171 renal grafts (5.1 %) and 3,869 native kidneys (94.2 %) and 237 renal grafts (5.8 %) were registered in 2009 and 2010, respectively. Patients younger than 20 years of age comprised 12.1 % of the registered cases, and those 65 years and over comprised 24.5 % of the cases with native kidneys in 2009 and 2010. The most common clinical diagnosis was chronic nephritic syndrome (55.4 % and 50.0 % in 2009 and 2010, respectively), followed by nephrotic syndrome (22.4 % and 27.0 %); the most frequent pathological diagnosis as classified by the pathogenesis was IgA nephropathy (31.6 % and 30.4 %), followed by primary glomerular diseases (except IgA nephropathy) (27.2 % and 28.1 %). Among the primary glomerular diseases (except IgA nephropathy) in the patients with nephrotic syndrome, membranous nephropathy was the most common histopathology in 2009 (40.3 %) and minor glomerular abnormalities (50.0 %) were the most common in 2010 in native kidneys in the J-RBR. Five new secondary and longitudinal research studies by the J-KDR were started in 2009 and one was started in 2010.

  1. National Kidney Registry: 213 transplants in three years.

    PubMed

    Veale, Jeffrey; Hil, Garet

    2010-01-01

    Since its establishment in 2008, the National Kidney Registry has facilitated 213 kidney transplants between unrelated living donors and recipients at 28 transplant centers. Rapid innovations in matching strategies, advanced computer technologies, good communication and an evolving understanding of the processes at participating transplant centers and histocompatibility laboratories are among the factors driving the success of the NKR. Virtual cross match accuracy has improved from 43% to 91% as a result of changes to the HLA typing requirements for potential donors and improved mechanisms to list unacceptable HLA antigens for sensitized patients. A uniform financial agreement among participating centers eliminated a major roadblock to facilitate unbalanced donor kidney exchanges among centers. The NKR transplanted 64% of the patients registered since 2008 and the average waiting time for those transplanted in 2010 was 11 months.

  2. 16 CFR 310.8 - Fee for access to the National Do Not Call Registry.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... Registry. 310.8 Section 310.8 Commercial Practices FEDERAL TRADE COMMISSION REGULATIONS UNDER SPECIFIC ACTS OF CONGRESS TELEMARKETING SALES RULE § 310.8 Fee for access to the National Do Not Call Registry. (a... access to telephone numbers within that area code that are included in the National Do Not Call...

  3. 36 CFR 704.1 - Films selected for inclusion in the National Film Registry.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 36 Parks, Forests, and Public Property 3 2014-07-01 2014-07-01 false Films selected for inclusion in the National Film Registry. 704.1 Section 704.1 Parks, Forests, and Public Property LIBRARY OF CONGRESS NATIONAL FILM REGISTRY OF THE LIBRARY OF CONGRESS § 704.1 Films selected for inclusion in...

  4. 36 CFR 704.1 - Films selected for inclusion in the National Film Registry.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 36 Parks, Forests, and Public Property 3 2010-07-01 2010-07-01 false Films selected for inclusion in the National Film Registry. 704.1 Section 704.1 Parks, Forests, and Public Property LIBRARY OF CONGRESS NATIONAL FILM REGISTRY OF THE LIBRARY OF CONGRESS § 704.1 Films selected for inclusion in...

  5. 36 CFR 704.1 - Films selected for inclusion in the National Film Registry.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 36 Parks, Forests, and Public Property 3 2012-07-01 2012-07-01 false Films selected for inclusion in the National Film Registry. 704.1 Section 704.1 Parks, Forests, and Public Property LIBRARY OF CONGRESS NATIONAL FILM REGISTRY OF THE LIBRARY OF CONGRESS § 704.1 Films selected for inclusion in...

  6. 36 CFR 704.1 - Films selected for inclusion in the National Film Registry.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 36 Parks, Forests, and Public Property 3 2013-07-01 2012-07-01 true Films selected for inclusion in the National Film Registry. 704.1 Section 704.1 Parks, Forests, and Public Property LIBRARY OF CONGRESS NATIONAL FILM REGISTRY OF THE LIBRARY OF CONGRESS § 704.1 Films selected for inclusion in...

  7. 36 CFR 704.1 - Films selected for inclusion in the National Film Registry.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 36 Parks, Forests, and Public Property 3 2011-07-01 2011-07-01 false Films selected for inclusion in the National Film Registry. 704.1 Section 704.1 Parks, Forests, and Public Property LIBRARY OF CONGRESS NATIONAL FILM REGISTRY OF THE LIBRARY OF CONGRESS § 704.1 Films selected for inclusion in...

  8. 49 CFR 195.64 - National Registry of Pipeline and LNG Operators.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 3 2013-10-01 2013-10-01 false National Registry of Pipeline and LNG Operators... Reporting § 195.64 National Registry of Pipeline and LNG Operators. (a) OPID Request. Effective January 1... Pipeline and LNG Operators in accordance with § 195.58. (b) OPID validation. An operator who has...

  9. 49 CFR 191.22 - National Registry of Pipeline and LNG operators.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 49 Transportation 3 2014-10-01 2014-10-01 false National Registry of Pipeline and LNG operators...-RELATED CONDITION REPORTS § 191.22 National Registry of Pipeline and LNG operators. (a) OPID Request. Effective January 1, 2012, each operator of a gas pipeline, gas pipeline facility, LNG plant or LNG...

  10. 49 CFR 195.64 - National Registry of Pipeline and LNG Operators.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 49 Transportation 3 2012-10-01 2012-10-01 false National Registry of Pipeline and LNG Operators... Reporting § 195.64 National Registry of Pipeline and LNG Operators. (a) OPID Request. Effective January 1... Pipeline and LNG Operators in accordance with § 195.58. (b) OPID validation. An operator who has...

  11. 49 CFR 191.22 - National Registry of Pipeline and LNG operators.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 49 Transportation 3 2012-10-01 2012-10-01 false National Registry of Pipeline and LNG operators...-RELATED CONDITION REPORTS § 191.22 National Registry of Pipeline and LNG operators. (a) OPID Request. Effective January 1, 2012, each operator of a gas pipeline, gas pipeline facility, LNG plant or LNG...

  12. 49 CFR 191.22 - National Registry of Pipeline and LNG operators.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 49 Transportation 3 2013-10-01 2013-10-01 false National Registry of Pipeline and LNG operators...-RELATED CONDITION REPORTS § 191.22 National Registry of Pipeline and LNG operators. (a) OPID Request. Effective January 1, 2012, each operator of a gas pipeline, gas pipeline facility, LNG plant or LNG...

  13. 49 CFR 191.22 - National Registry of Pipeline and LNG operators.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 3 2011-10-01 2011-10-01 false National Registry of Pipeline and LNG operators...-RELATED CONDITION REPORTS § 191.22 National Registry of Pipeline and LNG operators. (a) OPID Request. Effective January 1, 2012, each operator of a gas pipeline, gas pipeline facility, LNG plant or LNG...

  14. 49 CFR 195.64 - National Registry of Pipeline and LNG Operators.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 49 Transportation 3 2011-10-01 2011-10-01 false National Registry of Pipeline and LNG Operators... Reporting § 195.64 National Registry of Pipeline and LNG Operators. (a) OPID Request. Effective January 1... Pipeline and LNG Operators in accordance with § 195.58. (b) OPID validation. An operator who has...

  15. 49 CFR 195.64 - National Registry of Pipeline and LNG Operators.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 49 Transportation 3 2014-10-01 2014-10-01 false National Registry of Pipeline and LNG Operators... Reporting § 195.64 National Registry of Pipeline and LNG Operators. (a) OPID Request. Effective January 1... Pipeline and LNG Operators in accordance with § 195.58. (b) OPID validation. An operator who has...

  16. 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

  17. Comparison of Body Habitus in Patients With Pulmonary Arterial Hypertension Enrolled in the Registry to Evaluate Early and Long-term PAH Disease Management With Normative Values From the National Health and Nutrition Examination Survey

    PubMed Central

    Burger, Charles D.; Foreman, Aimee J.; Miller, Dave P.; Safford, Robert E.; McGoon, Michael D.; Badesch, David B.

    2011-01-01

    OBJECTIVE: To investigate the correlation between body mass index (BMI) and pulmonary artery systolic pressure in a large population of patients with pulmonary arterial hypertension (PAH). PATIENTS AND METHODS: The BMI of patients with group 1 PAH enrolled in the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) was compared with that of age- and sex-matched controls in the National Health and Nutrition Examination Survey (NHANES) to clarify whether obesity is linked with PAH. The diagnosis of PAH was defined in REVEAL by right-sided heart catheterization. Differences in BMI and the percentage of patients considered obese (BMI ≥30) and underweight (BMI <18.5) in various subgroups of patients enrolled in REVEAL from March 30, 2006, through September 11, 2007, were determined. RESULTS: Mean BMI was no different for patients with PAH (n=2141) than for the NHANES normal comparison group; however, the proportion of obese and underweight patients was increased in patients with PAH. Subgroup analysis demonstrated that subgroups with idiopathic PAH and those with PAH associated with drugs and toxins had both higher BMI and percentage of obese patients, whereas 3 other subgroups (those with PAH associated with congenital heart disease, connective tissue disease, and human immunodeficiency virus) had lower mean BMI. CONCLUSION: Mean BMI of the REVEAL patients was the same as that of the NHANES normal comparison group; however, there were higher percentages of obese and underweight patients in REVEAL. This discrepancy can be explained by the balancing effect of more overweight and underweight patients in different PAH subgroups. The reason for the increased frequency of obesity in idiopathic PAH is unknown, and additional study is needed. Trial Registration: clinicaltrials.gov Identifier: NCT00370214 PMID:21282484

  18. Need for a roadmap for development of a coordinated national registry programme.

    PubMed

    Wilkins, S; Best, R L; Evans, S M

    2015-11-01

    Clinical quality registries are an overlooked and under-funded arm of clinical research in Australia. Registries are databases for patients with a particular disease, or who undergo a procedure, or use a health resource. Registries, where properly funded and universally adopted, have provided substantial benefits to the quality of healthcare and, in some cases, have had demonstrable effect in reducing costs. There is a lack of a coordinated programme for both funding and development of registries in Australia. A coordinated effort is required to address key gaps in registry coverage and ensure registries comply with appropriate technical and operating principles, and target areas where registries can add value to the health system. This will ensure that Australia is competitive with its international peers in this dynamic environment.

  19. The Danish National Patient Registry: a review of content, data quality, and research potential

    PubMed Central

    Schmidt, Morten; Schmidt, Sigrun Alba Johannesdottir; Sandegaard, Jakob Lynge; Ehrenstein, Vera; Pedersen, Lars; Sørensen, Henrik Toft

    2015-01-01

    Background The Danish National Patient Registry (DNPR) is one of the world’s oldest nationwide hospital registries and is used extensively for research. Many studies have validated algorithms for identifying health events in the DNPR, but the reports are fragmented and no overview exists. Objectives To review the content, data quality, and research potential of the DNPR. Methods We examined the setting, history, aims, content, and classification systems of the DNPR. We searched PubMed and the Danish Medical Journal to create a bibliography of validation studies. We included also studies that were referenced in retrieved papers or known to us beforehand. Methodological considerations related to DNPR data were reviewed. Results During 1977–2012, the DNPR registered 8,085,603 persons, accounting for 7,268,857 inpatient, 5,953,405 outpatient, and 5,097,300 emergency department contacts. The DNPR provides nationwide longitudinal registration of detailed administrative and clinical data. It has recorded information on all patients discharged from Danish nonpsychiatric hospitals since 1977 and on psychiatric inpatients and emergency department and outpatient specialty clinic contacts since 1995. For each patient contact, one primary and optional secondary diagnoses are recorded according to the International Classification of Diseases. The DNPR provides a data source to identify diseases, examinations, certain in-hospital medical treatments, and surgical procedures. Long-term temporal trends in hospitalization and treatment rates can be studied. The positive predictive values of diseases and treatments vary widely (<15%–100%). The DNPR data are linkable at the patient level with data from other Danish administrative registries, clinical registries, randomized controlled trials, population surveys, and epidemiologic field studies – enabling researchers to reconstruct individual life and health trajectories for an entire population. Conclusion The DNPR is a valuable

  20. Proposal for a Prospective Registry for Moyamoya Disease in Japan

    PubMed Central

    KAZUMATA, Ken; ITO, Masaki; UCHINO, Haruto; NISHIHARA, Hiroshi; HOUKIN, Kiyohiro

    2017-01-01

    The number of clinical research papers published worldwide on moyamoya disease (MMD) has increased recently. However, the majority of the literature comprises retrospective single-center studies collecting data on small numbers of patients. Several multi-center studies are ongoing in Japan; however, the current data are insufficient for comprehensively outlining the various characteristics of MMD. To enhance our knowledge on epidemiologic, vascular, and genetic aspects of MMD, a prospective multicenter registry will be established in Japan that will help to streamline clinical research as well as improve clinical treatments and long-term outcomes. Patients with MMD or secondary moyamoya syndrome referred to the participating centers will be invited to the registry. Demographic and physiological parameters, along with neuroimaging data will be collected chronologically. Clinical events, including neurological, medical, and surgical interventions will be recorded. Whole blood samples will be collected. Extra- and intracranial vascular tissue, and/or cerebrospinal fluid will also be collected from patients who undergo surgical revascularization. These biospecimens will be stored at the repositories and utilized for genome-wide association studies for identifying genetic variants, as well as tissue-specific proteomic, and/or molecular analyses. Ethics approval will be obtained at all facilities collecting biospecimens. The registry will provide descriptive statistics on functional outcomes, surgical techniques used, medications, and neurological events stratified according to patients’ clinical characteristics. We expect this study to provide novel insights in the management of MMD patients and design better therapies. PMID:28070115

  1. Proposal for a Prospective Registry for Moyamoya Disease in Japan.

    PubMed

    Kazumata, Ken; Ito, Masaki; Uchino, Haruto; Nishihara, Hiroshi; Houkin, Kiyohiro

    2017-02-15

    The number of clinical research papers published worldwide on moyamoya disease (MMD) has increased recently. However, the majority of the literature comprises retrospective single-center studies collecting data on small numbers of patients. Several multi-center studies are ongoing in Japan; however, the current data are insufficient for comprehensively outlining the various characteristics of MMD. To enhance our knowledge on epidemiologic, vascular, and genetic aspects of MMD, a prospective multicenter registry will be established in Japan that will help to streamline clinical research as well as improve clinical treatments and long-term outcomes. Patients with MMD or secondary moyamoya syndrome referred to the participating centers will be invited to the registry. Demographic and physiological parameters, along with neuroimaging data will be collected chronologically. Clinical events, including neurological, medical, and surgical interventions will be recorded. Whole blood samples will be collected. Extra- and intra-cranial vascular tissue, and/or cerebrospinal fluid will also be collected from patients who undergo surgical revascularization. These biospecimens will be stored at the repositories and utilized for genome-wide association studies for identifying genetic variants, as well as tissue-specific proteomic, and/or molecular analyses. Ethics approval will be obtained at all facilities collecting biospecimens. The registry will provide descriptive statistics on functional outcomes, surgical techniques used, medications, and neurological events stratified according to patients' clinical characteristics. We expect this study to provide novel insights in the management of MMD patients and design better therapies.

  2. Surveillance of the Incidence of Non-Communicable Diseases (NCDs) with Sparse Resources: A Simulation Study Using Data from a National Diabetes Registry, Denmark, 1995-2004.

    PubMed

    Brinks, Ralph; Hoyer, Annika; Landwehr, Sandra

    2016-01-01

    We propose two new methods to estimate secular trends in the incidence of a chronic disease from a series of prevalence studies and mortality data. One method is a direct inversion formula, the second method is a least squares estimation. Both methods are validated in a simulation study based on data from a diabetes register. The results of the validation show that the proposed methods may be useful in epidemiological settings with sparse resources, where running a register or a series of follow-up studies is difficult or impossible.

  3. National Registry on Cardiac Electrophysiology 2007 and 2008.

    PubMed

    da Silva, Manuel Nogueira; Bonhorst, Daniel; de Sousa, João

    2009-11-01

    Clinical electrophysiology remains one of the most dynamic areas of cardiology, with continuing developments in equipping centers with more modern mapping and navigation systems. This has enabled an increase in the number and variety of interventions, resulting in significant improvements in results of therapeutic ablation of arrhythmias and prevention of sudden cardiac death. In this phase of transition towards implementation of a computerized national registry with nationwide data transmitted via the internet, publication of the registry in its previous form, although requiring more work, still seems justified, in order to appraise and disseminate qualitative and quantitative developments in this activity and enable comparisons with what is being done internationally, assess the centers' training capacity and inform national and European health authorities of the activities and real needs in this sector. The authors analyze the number and type of procedures performed during 2007 and 2008 based on a survey sent to centers performing diagnostic and interventional electrophysiology (16 centers in 2007 and 2008) and/or implanting cardioverter-defibrillators (ICDs) (19 centers in 2007 and 21 in 2008). Compared to 2006, one more center began interventional electrophysiology in 2007 and two centers began implanting ICDs in 2008. In the years under review, 2060 electrophysiological studies were performed in 2007 and 2007 were performed in 2008, of which 74 and 79.5% respectively were followed by therapeutic ablation, making totals of 1523 and 1596 ablations (increases of 10.7 and 4.6% from previous years). Atrioventricular nodal reentrant tachycardia was the main indication for ablation (28.4 and 28.7%), followed by accessory pathways (26.8 and 25.4%), atrial flutter (20.8 and 19.7%), atrial fibrillation (13.9 and 14.6%), ventricular tachycardia (4.7 and 5.1%), atrial tachycardia (2.8 and 2.6%) and atrioventricular junction ablation (2.7 and 3.9%). Regarding ICDs, a

  4. Towards a European registry of severe allergic reactions: current status of national registries and future needs.

    PubMed

    Worm, M; Timmermans, F; Moneret-Vautrin, A; Muraro, A; Malmheden Yman, I I; Lövik, M; Hattersley, S; Crevel, R

    2010-06-01

    The incidence of severe allergic reactions is largely unknown and information about triggering allergens, aggravating factors, demography of patients and medical care is lacking. A European wide registry could provide a powerful tool to improve the management of severe allergic reactions from both a medical and a public health perspective. Analysis of existing registries regarding the type and quality of data being collected was used to develop a plan for a pan-European registry, including the type of system to be used and the range of data to be entered. Surveillance will provide evidence for the efficacy of risk management measures and may identify the emergence of new allergenic foods, and aid monitoring of novel foods, ingredients and technologies. Patients need a clear indication of factors that may increase their risk of having an adverse reaction, which such a registry can help compile. Based on the collected data, food businesses will be able to develop educational programmes for allergen risk assessment and allergen risk communication. Finally, and most importantly preventive measures can be developed and government agencies receive population based data which may be relevant for legislative purposes.

  5. Implementation of a population-based epidemiological rare disease registry: study protocol of the amyotrophic lateral sclerosis (ALS) - registry Swabia

    PubMed Central

    2013-01-01

    Background The social and medical impact of rare diseases is increasingly recognized. Amyotrophic lateral sclerosis (ALS) is the most prevalent of the motor neuron diseases. It is characterized by rapidly progressive damage to the motor neurons with a survival of 2–5 years for the majority of patients. The objective of this work is to describe the study protocol and the implementation steps of the amyotrophic lateral sclerosis (ALS) registry Swabia, located in the South of Germany. Methods/Design The ALS registry Swabia started in October 2010 with both, the retrospective (01.10.2008-30.09.2010) and prospective (from 01.10.2010) collection of ALS cases, in a target population of 8.6 million persons in Southern Germany. In addition, a population based case–control study was implemented based on the registry that also included the collection of various biological materials. Retrospectively, 420 patients (222 men and 198 women) were identified. Prospectively data of ALS patients were collected, of which about 70% agreed to participate in the population-based case–control study. All participants in the case–control study provided also a blood sample. The prospective part of the study is ongoing. Discussion The ALS registry Swabia has been implemented successfully. In rare diseases such as ALS, the collaboration of registries, the comparison with external samples and biorepositories will facilitate to identify risk factors and to further explore the potential underlying pathophysiological mechanisms. PMID:23414001

  6. Reference Standards for Cardiorespiratory Fitness Measured With Cardiopulmonary Exercise Testing Using Cycle Ergometry: Data From the Fitness Registry and the Importance of Exercise National Database (FRIEND) Registry.

    PubMed

    Kaminsky, Leonard A; Imboden, Mary T; Arena, Ross; Myers, Jonathan

    2017-02-01

    The importance of cardiorespiratory fitness (CRF) is well established. This report provides newly developed standards for CRF reference values derived from cardiopulmonary exercise testing (CPX) using cycle ergometry in the United States. Ten laboratories in the United States experienced in CPX administration with established quality control procedures contributed to the "Fitness Registry and the Importance of Exercise: A National Database" (FRIEND) Registry from April 2014 through May 2016. Data from 4494 maximal (respiratory exchange ratio, ≥1.1) cycle ergometer tests from men and women (20-79 years) from 27 states, without cardiovascular disease, were used to develop these references values. Percentiles of maximum oxygen consumption (VO2max) for men and women were determined for each decade from age 20 years through age 79 years. Comparisons of VO2max were made to reference data established with CPX data from treadmill data in the FRIEND Registry and previously published reports. As expected, there were significant differences between sex and age groups for VO2max (P<.01). For cycle tests within the FRIEND Registry, the 50th percentile VO2max of men and women aged 20 to 29 years declined from 41.9 and 31.0 mLO2/kg/min to 19.5 and 14.8 mLO2/kg/min for ages 70 to 79 years, respectively. The rate of decline in this cohort was approximately 10% per decade. The FRIEND Registry reference data will be useful in providing more accurate interpretations for the US population of CPX-measured VO2max from exercise tests using cycle ergometry compared with previous approaches based on estimations of standard differences from treadmill testing reference values.

  7. National nephrectomy registries: Reviewing the need for population-based data.

    PubMed

    Pearson, John; Williamson, Timothy; Ischia, Joseph; Bolton, Damien M; Frydenberg, Mark; Lawrentschuk, Nathan

    2015-09-01

    Nephrectomy is the cornerstone therapy for renal cell carcinoma (RCC) and continued refinement of the procedure through research may enhance patient outcomes. A national nephrectomy registry may provide the key information needed to assess the procedure at a national level. The aim of this study was to review nephrectomy data available at a population-based level in Australia and to benchmark these data against data from the rest of the world as an examination of the national nephrectomy registry model. A PubMed search identified records pertaining to RCC nephrectomy in Australia. A similar search identified records relating to established nephrectomy registries internationally and other surgical registries of clinical importance. These records were reviewed to address the stated aims of this article. Population-based data within Australia for nephrectomy were lacking. Key issues identified were the difficulty in benchmarking outcomes and no ongoing monitoring of trends. The care centralization debate, which questions whether small-volume centers provide comparable outcomes to high-volume centers, is ongoing. Patterns of adherence and the effectiveness of existing protocols are uncertain. A review of established international registries demonstrated that the registry model can effectively address issues comparable to those identified in the Australian literature. A national nephrectomy registry could address deficiencies identified in a given nation's nephrectomy field. The model is supported by evidence from international examples and will provide the population-based data needed for studies. Scope exists for possible integration with other registries to develop a more encompassing urological or surgical registry. Need remains for further exploration of the feasibility and practicalities of initiating such a registry including a minimum data set, outcome indicators, and auditing of data.

  8. 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.

  9. 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

  10. The NIH Office of Rare Diseases Research Patient Registry Standard: A Report from the University of New Mexico’s Oculopharyngeal Muscular Dystrophy Patient Registry

    PubMed Central

    Daneshvari, Shamsi; Youssof, Sarah; Kroth, Philip J.

    2013-01-01

    Patient registries remove barriers to performing research by assembling patient cohorts and data in a systematic, efficient, and proactive manner. Consequently, registries are a valuable strategy for facilitating research and scientific discovery. Registries for rare diseases are arguably even more valuable since there is difficulty in assembling cohorts of adequate size for study. Recently, the NIH Office of Rare Diseases Research created a rare disease registry Standard to facilitate research across multiple registries. We implemented the Standard for the Oculopharyngeal Muscular Dystrophy patient registry created at the University of New Mexico Health Sciences Center. We performed a data element analysis for each Common Data Element defined in the Standard. Problems included the use of previous HL7 versions, non-structured data types, and a recent update to the Standard. Overall, the Standard is an excellent first step toward standardizing patient registries to facilitate work on broader questions and promote novel interdisciplinary collaborations. PMID:24551336

  11. Development of a disease registry for autoimmune bullous diseases: initial analysis of the pemphigus vulgaris subset.

    PubMed

    Shah, Amit Aakash; Seiffert-Sinha, Kristina; Sirois, David; Werth, Victoria P; Rengarajan, Badri; Zrnchik, William; Attwood, Kristopher; Sinha, Animesh A

    2015-01-01

    Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. Patients were recruited to the IPPF disease registry through direct mail, e-mail, advertisements, and articles in the IPPF-quarterly, -website, -Facebook webpage, and IPPF Peer Health Coaches to complete a 38-question survey. We present here the initial analysis of detailed clinical information collected on 393 PV patients. We report previously unrecognized gender differences in terms of lesion location, autoimmune comorbidity, and delay in diagnosis. The IPPF disease registry serves as a useful resource and guide for future clinical investigation.

  12. Conference Proceedings: “Down Syndrome: National Conference on Patient Registries, Research Databases, and Biobanks”

    PubMed Central

    Oster-Granite, Mary Lou; Parisi, Melissa A.; Abbeduto, Leonard; Berlin, Dorit S.; Bodine, Cathy; Bynum, Dana; Capone, George; Collier, Elaine; Hall, Dan; Kaeser, Lisa; Kaufmann, Petra; Krischer, Jeffrey; Livingston, Michelle; McCabe, Linda L.; Pace, Jill; Pfenninger, Karl; Rasmussen, Sonja A.; Reeves, Roger H.; Rubinstein, Yaffa; Sherman, Stephanie; Terry, Sharon F.; Whitten, Michelle Sie; Williams, Stephen; McCabe, Edward R.B.; Maddox, Yvonne T.

    2011-01-01

    A December 2010 meeting, “Down Syndrome: National Conference on Patient Registries, Research Databases, and Biobanks,” was jointly sponsored by the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) at the National Institutes of Health (NIH) in Bethesda, MD, and the Global Down Syndrome Foundation (GDSF)/Linda Crnic Institute for Down Syndrome based in Denver, CO. Approximately 70 attendees and organizers from various advocacy groups, federal agencies (Centers for Disease Control and Prevention, and various NIH Institutes, Centers, and Offices), members of industry, clinicians, and researchers from various academic institutions were greeted by Drs. Yvonne Maddox, Deputy Director of NICHD, and Edward McCabe, Executive Director of the Linda Crnic Institute for Down Syndrome. They charged the participants to focus on the separate issues of contact registries, research databases, and biobanks through both podium presentations and breakout session discussions. Among the breakout groups for each of the major sessions, participants were asked to generate responses to questions posed by the organizers concerning these three research resources as they related to Down syndrome and then to report back to the group at large with a summary of their discussions. This report represents a synthesis of the discussions and suggested approaches formulated by the group as a whole. PMID:21835664

  13. A national registry of haemoglobinopathies in Greece: deducted demographics, trends in mortality and affected births.

    PubMed

    Voskaridou, Ersi; Ladis, Vasilis; Kattamis, Antonis; Hassapopoulou, Eleni; Economou, Marina; Kourakli, Alexandra; Maragkos, Konstantinos; Kontogianni, Kalliopi; Lafioniatis, Stilianos; Vrettou, Eleni; Koutsouka, Freideriki; Papadakis, Alexandros; Mihos, Andreas; Eftihiadis, Eftihios; Farmaki, Kallistheni; Papageorgiou, Ourania; Tapaki, Georgia; Maili, Polixeni; Theohari, Maria; Drosou, Marouso; Kartasis, Zafeiris; Aggelaki, Maria; Basileiadi, Artemis; Adamopoulos, Ioannis; Lafiatis, Ioannis; Galanopoulos, Athanasios; Xanthopoulidis, Georgios; Dimitriadou, Efthimia; Mprimi, Agapi; Stamatopoulou, Maria; Haile, Elanso Damba; Tsironi, Maria; Anastasiadis, Athanasios; Kalmanti, Maria; Papadopoulou, Margarita; Panori, Evaggelia; Dimoxenou, Peristera; Tsirka, Antigoni; Georgakopoulos, Dimitrios; Drandrakis, Pantelis; Dionisopoulou, Dionisia; Ntalamaga, Androniki; Davros, Ioannis; Karagiorga, Markisia

    2012-09-01

    Haemoglobinopathies are the most common hereditary disorders in Greece. Although there is a successful national prevention program, established 35 years ago, there is lack of an official registry and collection of epidemiological data for haemoglobinopathies. This paper reports the results of the first National Registry for Haemoglobinopathies in Greece (NRHG), recently organized by the Greek Society of Haematology. NRHG records all patients affected by thalassaemia major (TM), thalassaemia intermedia (TI), "H" Haemoglobinopathy (HH) and sickle cell disease (SCD). Moreover, data about the annual rate of new affected births along with deaths, between 2000 and 2010, are reported. A total of 4,506 patients are registered all over the country while the number of affected newborns was significantly decreased during the last 3 years. Main causes for still having affected births are: (1) lack of medical care due to financial reasons or low educational level; (2) unawareness of time limitations for prenatal diagnosis (PD); due either to obstetricians' malpractice or to delayed demand of medical care of couples at risk; and (3) religious, social or bioethical reasons. Cardiac and liver disorders consist main causes for deaths while life expectancy of patients lengthened after 2005 (p < 0.01). The NRHG of patients affected by haemoglobinopathies in Greece provides useful data about the haemoglobinopathies in the Greek population and confirms the efficacy of the National Thalassaemia Prevention Program on impressively decreasing the incidence of TM and sickle cell syndromes.

  14. 77 FR 33264 - National Registry of Certified Medical Examiners Testing Providers Public Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-05

    ... need sign language interpretation or other assistance to participate, please contact Ms. Hamilton by... providers may apply on-line through the National Registry System to be a test delivery organization for...

  15. National and international registries of rare bleeding disorders

    PubMed Central

    Peyvandi, Flora; Spreafico, Marta

    2008-01-01

    Rare bleeding disorders (RBDs) are autosomal recessive disorders, representing 3–5% of all the inherited deficiencies of coagulation factors. Their frequency in the general population ranges from 1:500.000 to 1:2 millions. In countries with a high rate of consanguineous marriages RBDs occur more frequently, representing a significant clinical and social problem. Patients affected by RBDs have a wide spectrum of clinical symptoms that vary from a mild or moderate bleeding tendency to potentially serious or life-threatening haemorrhages. Current treatment is based on both replacement therapy and non-transfusional treatment. However, despite the existence of several concentrates, there is no Factor V concentrate available for the treatment of Factor V deficiency, yet. In 2004, to improve the understanding of RBDs prevalence, diagnosis and treatments, the Rare Bleeding Disorders database (RBDD, www.rbdd.org) was developed. The RBDD project allowed the collection of epidemiological information on 3,230 patients from 66 Centres scattered all over the world. Epidemiological data can also be derived from the annual survey of the World Federation of Hemophilia (www.wfh.org) and from other existing national registries. However, these data are not homogenous and global surveys provide a non-real picture of the distribution ofRBDs, as about 50% of data refers to European patients. Hence, we focused on Europe and, thanks to a European project (EN-RBD), we set up a network of 10 Treatment Centres to develop a homogeneous communication tool for inserting, managing and viewing information on RBD patients (www.rbdd.eu). This on-line database resulted to be a powerful tool to improve the quality of data collection. Preliminary results showed that a homogeneous and harmonized data collection using a unique model will help to have more accurate data for statistical analysis. PMID:19115503

  16. 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

  17. [Registries for rare diseases : OSSE - An open-source framework for technical implementation].

    PubMed

    Storf, Holger; Schaaf, Jannik; Kadioglu, Dennis; Göbel, Jens; Wagner, Thomas O F; Ückert, Frank

    2017-03-13

    Meager amounts of data stored locally, a small number of experts, and a broad spectrum of technological solutions incompatible with each other characterize the landscape of registries for rare diseases in Germany. Hence, the free software Open Source Registry for Rare Diseases (OSSE) was created to unify and streamline the process of establishing specific rare disease patient registries. The data to be collected is specified based on metadata descriptions within the registry framework's so-called metadata repository (MDR), which was developed according to the ISO/IEC 11179 standard. The use of a central MDR allows for sharing the same data elements across any number of registries, thus providing a technical prerequisite for making data comparable and mergeable between registries and promoting interoperability.With OSSE, the foundation is laid to operate linked patient registries while respecting strong data protection regulations. Using the federated search feature, data for clinical studies can be identified across registries. Data integrity, however, remains intact since no actual data leaves the premises without the owner's consent. Additionally, registry solutions other than OSSE can participate via the OSSE bridgehead, which acts as a translator between OSSE registry networks and non-OSSE registries. The pseudonymization service Mainzelliste adds further data protection.Currently, more than 10 installations are under construction in clinical environments (including university hospitals in Frankfurt, Hamburg, Freiburg and Münster). The feedback given by the users will influence further development of OSSE. As an example, the installation process of the registry for undiagnosed patients at University Hospital Frankfurt is described in more detail.

  18. Latin American Dialysis and Transplant Registry: Experience and contributions to end-stage renal disease epidemiology

    PubMed Central

    Cusumano, Ana Maria; Rosa-Diez, Guillermo Javier; Gonzalez-Bedat, Maria Carlota

    2016-01-01

    In 2015, 634387 million people (9% of the world’s population) resided in Latin America (LA), with half of those populating Brazil and Mexico. The LA Dialysis and Transplant Registry was initiated in 1991, with the aim of collecting data on renal replacement therapy (RRT) from the 20 LA-affiliated countries. Since then, the Registry has revealed a trend of increasing prevalence and incidence of end-stage kidney disease on RRT, which is ongoing and is correlated with gross national income, life expectancy at birth, and percentage of population that is older than 65 years. In addition, the rate of kidney transplantation has increased yearly, with > 70% being performed from deceased donors. According to the numbers reported for 2013, the rates of prevalence, incidence and transplantation were (in patients per million population) 669, 149 and 19.4, respectively. Hemodialysis was the treatment of choice (90%), and 43% of the patients undergoing this treatment was located in Brazil; in contrast, peritoneal dialysis prevailed in Costa Rica, El Salvador and Guatemala. To date, the Registry remains the only source of RRT data available to healthcare authorities in many LA countries. It not only serves to promote knowledge regarding epidemiology of end-stage renal disease and the related RRT but also for training of nephrologists and renal researchers, to improve understanding and clinical application of dialysis and transplantation services. In LA, accessibility to RRT is still limited and it remains necessary to develop effective programs that will reduce risk factors, promote early diagnosis and treatment of chronic kidney disease, and strengthen transplantation programs. PMID:27648403

  19. Latin American Dialysis and Transplant Registry: Experience and contributions to end-stage renal disease epidemiology.

    PubMed

    Cusumano, Ana Maria; Rosa-Diez, Guillermo Javier; Gonzalez-Bedat, Maria Carlota

    2016-09-06

    In 2015, 634387 million people (9% of the world's population) resided in Latin America (LA), with half of those populating Brazil and Mexico. The LA Dialysis and Transplant Registry was initiated in 1991, with the aim of collecting data on renal replacement therapy (RRT) from the 20 LA-affiliated countries. Since then, the Registry has revealed a trend of increasing prevalence and incidence of end-stage kidney disease on RRT, which is ongoing and is correlated with gross national income, life expectancy at birth, and percentage of population that is older than 65 years. In addition, the rate of kidney transplantation has increased yearly, with > 70% being performed from deceased donors. According to the numbers reported for 2013, the rates of prevalence, incidence and transplantation were (in patients per million population) 669, 149 and 19.4, respectively. Hemodialysis was the treatment of choice (90%), and 43% of the patients undergoing this treatment was located in Brazil; in contrast, peritoneal dialysis prevailed in Costa Rica, El Salvador and Guatemala. To date, the Registry remains the only source of RRT data available to healthcare authorities in many LA countries. It not only serves to promote knowledge regarding epidemiology of end-stage renal disease and the related RRT but also for training of nephrologists and renal researchers, to improve understanding and clinical application of dialysis and transplantation services. In LA, accessibility to RRT is still limited and it remains necessary to develop effective programs that will reduce risk factors, promote early diagnosis and treatment of chronic kidney disease, and strengthen transplantation programs.

  20. Design and Development of a Web-Based Saudi National Diabetes Registry

    PubMed Central

    Subhani, Shazia; Khalid, Al-Rubeaan

    2010-01-01

    Background Given that diabetes is an extremely common disorder in Saudi Arabia, the National Diabetes Registry was designed by King Saud University Hospital Diabetes Center in collaboration with King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia, in the year 2001. The aim of the registry is to identify risk factors related to diabetes and to provide statistics to public health programs and health care professionals for use in planning and evaluation. The registry was designed to provide information on the extent and nature of specific types of diabetes, diabetes complications, and treatment of diabetes in the Kingdom. The registry has been available since 2001, with major collaborations from 26 hospitals as part of Phase I in which 100,000 patient data is to be collected on a regional level from Ar-Riyadh before extending the program to other regions of Saudi Arabia. Methods The web application was designed using relational database techniques along with on-line help topics to assist users to get acquainted with application functionalities. All Internet forms were designed with validation checks and appropriate messages to ensure quality of data. The security measures established within the application ensure that only authorized users can gain access to the functionalities of the registry at allowed times. Administrative features were designed to manage the registry-related operations easily. Results The diabetes registry has been in operation for almost 10 years, and around 67,000 patients have been registered to date. The Web-application offers an anytime-anywhere access to the registry’s data, removing geographical boundaries and allowing the national registry to provide real-time data entry, updates, reporting, and mapping functionalities more easily. Conclusion Merging related information in the form of databases can provide improved health care operations through instant access to data, ease of managing complex data structures, and

  1. National kidney dialysis and transplant registries in Latin America: how to implement and improve them.

    PubMed

    González-Bedat, María Carlota; Rosa-Diez, Guillermo Javier; Fernández-Cean, Juan Manuel; Ordúñez, Pedro; Ferreiro, Alejandro; Douthat, Walter

    2015-09-01

    The Strategic Plan of the Pan American Health Organization, 2014-2019, Championing Health: Sustainable Development and Equityrecognizes that "Chronic kidney disease, caused mainly by complications of diabetes and hypertension, has increased in the Region." This Plan includes the first concrete goal on chronic kidney disease: to achieve a prevalence rate for renal replacement therapy of at least 700 patients per million population by 2019. National dialysis and transplant registries (DTR) are a useful tool for epidemiological research, health care planning, and quality improvement. Their success depends on the quality of their data and quality control procedures. This article describes the current situation of national DTRs in the Region and the content of their information and health indicators, and it offers recommendations for creating and maintaining them. It points to their heterogeneity or absence in some countries, in line with the inequities that patients face in access to renal replacement therapy. The complete lack of information in Caribbean countries prevents their inclusion in this communication, which requires immediate attention.

  2. [RD] PRISM Library: Patient Registry Item Specifications and Metadata for Rare Diseases.

    PubMed

    Richesson, Rachel; Shereff, Denise; Andrews, James

    2010-04-01

    Patient registries are important for understanding the causes and origins of rare diseases and estimating their impact; and they may prove critical developing new diagnostics and therapeutics. This paper introduces the [RD] PRISM resource , an NIH-funded project to develop a library of standardized question and answer sets to support rare disease research. The paper presents a project case-driven plan for creating a new registry using questions from an existing related registry, revising and expanding an existing registry, and showing interoperability of data collected from different registries and data sources. Each of the use cases involves the retrieval of indexed questions for re-use. Successful retrieval of questions can facilitate their re-use in registries, meaning new registries can be implemented more quickly, and the use of "standard" questions can be facilitated. The paper further discusses issues involved in encoding the sets with relevant data standards for interoperability and indexing encoded sets with metadata for optimal retrievability.

  3. The National Anesthesia Clinical Outcomes Registry: A Sustainable Model for the Information Age?

    PubMed Central

    Dutton, Richard P.

    2014-01-01

    Anesthesiologists care for patients of all ages, with all conceivable comorbidities, in every kind of health care facility. This leads to a significant challenge in the collection of data to describe the specialty, and in the development of evidence-based performance measures for anesthesiologists. Whereas narrowly defined medical specialties have developed registries based on manual abstraction of clinical data from the medical record (e.g., cardiac surgery), this approach would be prohibitively expensive for anesthesiology, and is unlikely to generate statistically useful data when major adverse outcomes occur a handful of times in tens of thousands of cases. The American Society of Anesthesiologists (ASA) addressed this challenge in 2008 by funding a related organization, the Anesthesia Quality Institute (AQI), to develop the National Anesthesia Clinical Outcomes Registry (NACOR). The technical development of this registry and the approach taken to define the specialty of anesthesiology and the performance of anesthesiologists may serve as a model for other specialty society efforts. PMID:25848607

  4. Integration of site-specific health information: Agency for Toxic Substances and Disease Registry health assessments

    SciTech Connect

    Lesperance, A.M.; Siegel, M.R.

    1990-12-01

    The Agency for Toxic Substances and Disease Registry is required to conduct a health assessment of any site that is listed on or proposed for the US Environmental Protection Agency's National Priorities List. Sixteen US Department of Energy (DOE) sites currently fall into this category. Health assessments contain a qualitative description of impacts to public health and the environment from hazardous waste sites, as well as recommendations for actions to mitigate or eliminate risk. Because these recommendations may have major impacts on compliance activities at DOE facilities, the health assessments are an important source of information for the monitoring activities of DOE's Office of Environmental Compliance (OEC). This report provides an overview of the activities involved in preparing the health assessment, its role in environmental management, and its key elements.

  5. Rationale and design of a multidisciplinary national real-world registry on carotid stenting: the Italian Registry for Carotid Stenting (RISC).

    PubMed

    Biasi, Giorgio M; Deleo, Gaetano; Froio, Alberto; Cremonesi, Alberto; Inglese, Luigi; Lavitrano, Marialuisa; Setacci, Carlo

    2006-04-01

    The Registro Italiano per lo Stenting Carotideo (RISC, Italian Registry for Carotid Stenting) has been organized by Italian specialists from different disciplines directly involved in the prevention of stroke due to carotid plaques through stenting of carotid lesions. The Registry has been endorsed by the national societies of 4 different specialties: vascular surgery, interventional cardiology, radiology, and neuroradiology. Each society contributed in the planning stage. The basis for the registry is to collect data on carotid stenting procedures performed by different specialists with different techniques in a "real-world" setting without the limitations of a randomized clinical trial. The Registry was funded to enroll at least 1200 patients over a minimum period of 36 months. The results will be analyzed using the intention-to-treat principle and are anticipated in late 2006. Primary endpoints of the registry are the 30-day combined death and stroke rate and the occurrence of restenosis and ipsilateral neurological deficit at 12 and 24 months. Considerable attention has been paid to the registry's quality control program to ensure scientific validation. An online database facilitates the collection of data with speed and accuracy.

  6. 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

  7. Total Ankle Replacement Survival Rates Based on Kaplan-Meier Survival Analysis of National Joint Registry Data.

    PubMed

    Bartel, Annette F P; Roukis, Thomas S

    2015-10-01

    National joint registry data provides unique information about primary total ankle replacement (TAR) survival. We sought to recreate survival curves among published national joint registry data sets using the Kaplan-Meier estimator. Overall, 5152 primary and 591 TAR revisions were included over a 2- to 13-year period with prosthesis survival for all national joint registries of 0.94 at 2-years, 0.87 at 5-years and 0.81 at 10-years. National joint registry datasets should strive for completion of data presentation including revision definitions, modes and time of failure, and patients lost to follow-up or death for complete accuracy of the Kaplan-Meier estimator.

  8. 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.

  9. The Norwegian Labour Inspectorate's Registry for Work-Related Diseases: data from 2006.

    PubMed

    Samant, Yogindra; Parker, David; Wergeland, Ebba; Wannag, Axel

    2008-01-01

    The Norwegian Labour Inspectorates (NLI's) Registry for Work-Related Diseases was established in 1920. Based on the principle of sentinel health events (SHE), its central purpose is to provide information to the NLI to enable workplace interventions and the prevention of hazardous exposures. Although physicians are required to report work-related diseases to the NLI, only 3% did so in 2006. There were 3392 cases of work-related diseases reported to the NLI by 561 physicians in 2006. Diseases of the ear (noise-induced hearing loss) comprised 59% (n=1987) of the cases, while 12% (n=398) of the cases were attributed to the diseases of the respiratory system and 7% (n =239) were diseases of the skin and subcutaneous tissue. Despite limitations, the registry continues to inform NLI's prevention strategies, supplements data concerning work-environment surveillance, and provides impetus for epidemiological studies.

  10. Internal disinhibition predicts 5‐year weight regain in the National Weight Control Registry (NWCR)

    PubMed Central

    Thomas, J. G.; Niemeier, H.; Wing, R. R.

    2016-01-01

    Summary Background Maintenance of weight loss remains elusive for most individuals. One potential innovative target is internal disinhibition (ID) or the tendency to eat in response to negative thoughts, feelings or physical sensations. Individuals high on ID do worse on average in standard behavioural treatment programmes, and recent studies suggest that disinhibition could play a significant role in weight regain. Purpose The purpose of the current study was to examine whether ID was associated with weight change over 5 years of follow‐up in the National Weight Control Registry, a registry of individuals who have successfully lost weight and maintained it. Methods From the National Weight Control Registry, 5,320 participants were examined across 5 years. Weight data were gathered annually. The disinhibition subscale of the Eating Inventory was used to calculate internal disinhibition and External Disinhibition (ED) and was collected at baseline, year 1, year 3 and year 5. Linear mixed models were used to estimate the weight loss maintained across follow‐up years 1 to 5 using ID and ED as baseline and prospective predictors. Results Internal disinhibition predicted weight regain in all analyses. ED interacted with ID, such that individuals who were high on ID showed greater weight regain if they were also higher on ED. Conclusions The ID scale could be a useful screening measure for risk of weight regain, given its brevity. Improved psychological coping could be a useful target for maintenance or booster interventions. PMID:27812382

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

    DTIC Science & Technology

    2013-03-01

    Parkinsonism , and another 12% reported multiple diagnoses of Parkinson’s disease and Parkinsonism . Table 2 below lists the cases by county. 15. Assessment...AD_________________ Award Number: W81XWH-07-1-0261 TITLE: California’s Parkinson’s Disease ...NUMBER California’s Parkinson’s Disease Registry Pilot Project – Coordination Center and Northern California Ascertainment 5b. GRANT NUMBER W81XWH

  12. A renal registry for Africa: first steps

    PubMed Central

    Davids, M. Razeen; Eastwood, John B.; Selwood, Neville H.; Arogundade, Fatiu A.; Ashuntantang, Gloria; Benghanem Gharbi, Mohammed; Jarraya, Faiçal; MacPhee, Iain A.M.; McCulloch, Mignon; Plange-Rhule, Jacob; Swanepoel, Charles R.; Adu, Dwomoa

    2016-01-01

    There is a dearth of data on end-stage renal disease (ESRD) in Africa. Several national renal registries have been established but have not been sustainable because of resource limitations. The African Association of Nephrology (AFRAN) and the African Paediatric Nephrology Association (AFPNA) recognize the importance of good registry data and plan to establish an African Renal Registry. This article reviews the elements needed for a successful renal registry and gives an overview of renal registries in developed and developing countries, with the emphasis on Africa. It then discusses the proposed African Renal Registry and the first steps towards its implementation. A registry requires a clear purpose, and agreement on inclusion and exclusion criteria, the dataset and the data dictionary. Ethical issues, data ownership and access, the dissemination of findings and funding must all be considered. Well-documented processes should guide data collection and ensure data quality. The ERA-EDTA Registry is the world's oldest renal registry. In Africa, registry data have been published mainly by North African countries, starting with Egypt and Tunisia in 1975. However, in recent years no African country has regularly reported national registry data. A shared renal registry would provide participating countries with a reliable technology platform and a common data dictionary to facilitate joint analyses and comparisons. In March 2015, AFRAN organized a registry workshop for African nephrologists and then took the decision to establish, for the first time, an African Renal Registry. In conclusion, African nephrologists have decided to establish a continental renal registry. This initiative could make a substantial impact on the practice of nephrology and the provision of services for adults and children with ESRD in many African countries. PMID:26798479

  13. The UK National Flap Registry (UKNFR): A National Database for all pedicled and free flaps in the UK.

    PubMed

    Hazari, Anita; Walton, Peter

    2015-12-01

    The UK National Flap Registry (UKNFR) is a cross-speciality National Clinical Audit with participation by the British Association of Plastic Reconstructive and Aesthetic Surgeons (BAPRAS), British Association of Head and Neck Oncologists (BAHNO), British Association of Oral and Maxillofacial Surgeons (BAOMS) and Association of Breast Surgery (ABS). The aim of UKNFR is to collect information about all major pedicled and free flap operations carried out in the UK and through that, assess the quality of care we provide for patients. This audit will allow appropriate comparison of clinical performance with national standards and provide useful data on changing trends. Participation in audit is integral to appraisal and revalidation in the UK.

  14. CERAD (Consortium to Establish a Registry for Alzheimer’s Disease) The first 20 years

    PubMed Central

    Fillenbaum, Gerda G.; van Belle, Gerald; Morris, John C.; Mohs, Richard C.; Mirra, Suzanne S.; Davis, Patricia C.; Tariot, Pierre N.; Silverman, Jeremy M.; Clark, Christopher M.; Welsh-Bohmer, Kathleen A.; Heyman, Albert

    2009-01-01

    The Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) was funded by the National Institute on Aging in 1986 to develop standardized, validated measures for the assessment of Alzheimer’s disease (AD). The present report describes the measures that CERAD developed during its first decade, and their continued use in their original and translated forms. These measures include clinical, neuropsychological, neuropathological and behavioral assessments of AD, and also assessment of family history and parkinsonism in AD. An approach to evaluating neuroimages did not meet the standards desired. Further evaluations which could not be completed because of lack of funding (but where some materials are available), include evaluation of very severe AD, and of service use and need by patient and caregiver. The information that was developed in the U.S. and abroad permits standardized assessment of AD in clinical practice, facilitates epidemiological studies, and provides information valuable for individual and public health planning. CERAD materials and data remain available for those wishing to use them. PMID:18631955

  15. United Kingdom Primary Sjogren's Syndrome Registry--a united effort to tackle an orphan rheumatic disease.

    PubMed

    Ng, Wan-Fai; Bowman, Simon J; Griffiths, Bridget

    2011-01-01

    Primary SS (pSS) is a multi-system autoimmune disease with a prevalence and health economic impact that are comparable with RA. However, pSS research has been relatively poorly supported. The creation of a large cohort of clinically well-characterized pSS patients will provide a catalyst and valuable resources to promote high-quality pSS research. In this review, we will describe the creation of such a cohort and the associated research biobank that is currently being established in the UK--entitled United Kingdom Primary Sjögren's Syndrome Registry (UKPSSR). We will discuss the strengths and weaknesses of the design of the registry and highlight the key challenges in the establishment of the registry and the strategies that we employ to overcome these barriers. Finally, we will consider the future development of the UKPSSR including utilization and maintenance of the cohort.

  16. Agency for Toxic Substances and Disease Registry Brownfields/ land-reuse site tool.

    PubMed

    Perlman, Gary D; Berman, Laurel; Leann, Kathryn; Bing, Lemley

    2012-12-01

    As part of our continuing effort to highlight innovative approaches to improving the health and environment of communities, the Journal is pleased to bring back the bimonthly column from the U.S. Agency for Toxic Substances and Disease Registry (ATSDR). The ATSDR, based in Atlanta, Georgia, is a federal public health agency of the U.S. Department of Health and Human Services and shares a common office of the Director with the National Center for Environmental Health at the Centers for Disease Control and Prevention (CDC). ATSDR serves the public by using the best science, taking responsive public health actions, and providing trusted health information to prevent harmful exposures and diseases related to toxic substances. The purpose of this column is to inform readers of ATSDR's activities and initiatives to better understand the relationship between exposure to hazardous substances in the environment and their impact on human health and how to protect public health. We believe that the column will provide a valuable resource to our readership by helping to make known the considerable resources and expertise that ATSDR has available to assist communities, states, and others to assure good environmental health practice for all is served. The conclusions of this article are those of the author(s) and do not necessarily represent the views of ATSDR, CDC, or the U.S. Department of Health and Human Services. Gary D. Perlman is an environmental health scientist for ATSDR. He is a commissioned officer with the U.S. Public Health Service and has been deployed in support of numerous environmental disasters including hurricanes Katrina, Rita, Isabelle, and Irene, as well as the Deepwater Horizon oil spill. Laurel Berman is the national brownfields coordinator with ATSDR. She coordinates the ATSDR Brownfields/Land-Reuse Health Initiative. Kathryn Leann Lemley Bing is an environmental health scientist and an ATSDR regional representative in Atlanta. She has specialized

  17. Positive predictive value of cardiovascular diagnoses in the Danish National Patient Registry: a validation study

    PubMed Central

    Sundbøll, Jens; Adelborg, Kasper; Munch, Troels; Frøslev, Trine; Sørensen, Henrik Toft; Bøtker, Hans Erik; Schmidt, Morten

    2016-01-01

    Objective The majority of cardiovascular diagnoses in the Danish National Patient Registry (DNPR) remain to be validated despite extensive use in epidemiological research. We therefore examined the positive predictive value (PPV) of cardiovascular diagnoses in the DNPR. Design Population-based validation study. Setting 1 university hospital and 2 regional hospitals in the Central Denmark Region, 2010–2012. Participants For each cardiovascular diagnosis, up to 100 patients from participating hospitals were randomly sampled during the study period using the DNPR. Main outcome measure Using medical record review as the reference standard, we examined the PPV for cardiovascular diagnoses in the DNPR, coded according to the International Classification of Diseases, 10th Revision. Results A total of 2153 medical records (97% of the total sample) were available for review. The PPVs ranged from 64% to 100%, with a mean PPV of 88%. The PPVs were ≥90% for first-time myocardial infarction, stent thrombosis, stable angina pectoris, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, takotsubo cardiomyopathy, arterial hypertension, atrial fibrillation or flutter, cardiac arrest, mitral valve regurgitation or stenosis, aortic valve regurgitation or stenosis, pericarditis, hypercholesterolaemia, aortic dissection, aortic aneurysm/dilation and arterial claudication. The PPVs were between 80% and 90% for recurrent myocardial infarction, first-time unstable angina pectoris, pulmonary hypertension, bradycardia, ventricular tachycardia/fibrillation, endocarditis, cardiac tumours, first-time venous thromboembolism and between 70% and 80% for first-time and recurrent admission due to heart failure, first-time dilated cardiomyopathy, restrictive cardiomyopathy and recurrent venous thromboembolism. The PPV for first-time myocarditis was 64%. The PPVs were consistent within age, sex, calendar year and hospital categories. Conclusions The validity of

  18. A Reference Equation for Normal Standards for VO2 max: Analysis from the Fitness Registry and the Importance of Exercise National Database (FRIEND Registry).

    PubMed

    Myers, Jonathan; Kaminsky, Leonard A; Lima, Ricardo; Chistle, Jeffrey; Ashley, Euan; Arena, Ross

    2017-04-01

    Existing normal standards for maximal oxygen uptake (VO2 max) are problematic because they tend to be population specific, lack normal distribution and portability, and are poorly represented by women. The objective of the current study was to apply the Fitness Registry and the Importance of Exercise: A National Data Base (FRIEND) Registry to improve upon previous regression formulas for normal standards for VO2 max using treadmill testing. Maximal treadmill tests were performed in 7783 healthy men and women (20-79years; maximal RER >1.0) from the FRIEND registry and a separate validation cohort of 1287 subjects. A regression equation for VO2 max was derived from the FRIEND registry and compared to the validation cohort and two commonly used equations (Wasserman and European). Age, gender, and body weight were the only significant predictors of VO2 max (multiple R=0.79, R(2)=0.62, p<0.001). The equation for predicting VO2 max was: [Formula: see text] Marked differences were observed in percentage predicted VO2 max achieved between commonly used reference equations, particularly among women, overweight and obese subjects. In the validation sample, the FRIEND equation closely paralleled measured VO2 max, with the validation group yielding a percent predicted VO2 max of 100.4% based on the FRIEND equation. An equation for age-predicted VO2 max derived from the FRIEND registry provided a lower average error between measured and predicted VO2 max than traditional equations, and thus may provide a more suitable normal standard relative to traditional equations.

  19. Pregnancy, Birth, and Infant Health Outcomes from the National Smallpox Health Vaccine in Pregnancy Registry, 2003-2006

    DTIC Science & Technology

    2008-01-01

    delivery, low birth weight , and other birth problems after exposure to smallpox vaccine in pregnancy have not been defined. Potential adverse effects on...preterm births (10.7%) among registry births, the average birth weight of singleton registry infants (3320 g), and the percentage of infants with low... birth weight (9.1%) may be compared with national data shown in table 2 [42]. Nearly half (47.9%) of all infants born to women in the Smallpox Vaccine in

  20. The National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC): Results from Phase I and Scientific Opportunities in Phase II

    PubMed Central

    Kroner, Barbara L.; Tolunay, H. Eser; Basson, Craig T.; Pyeritz, Reed E.; Holmes, Kathryn; Maslen, Cheryl L.; Milewicz, Dianna M.; LeMaire, Scott A.; Hendershot, Tabitha; Desvigne-Nickens, Patrice; Devereux, Richard B.; Dietz, Harry C.; Song, Howard; Ringer, Danny; Mitchell, Megan; Weinsaft, Jonathan W.; Ravekes, William; Menashe, Victor; Eagle, Kim A.

    2011-01-01

    Background Genetically triggered thoracic aortic conditions (GenTAC) represent an important problem for patients and their families. Accordingly, the National Heart, Lung and Blood Institute (NHLBI) established the first phase of its national GenTAC Registry in 2006. Enrollment and Diagnoses Between 2007 and 2010, six enrolling centers established the GenTAC I Registry consisting of 2,046 patients [(Marfan syndrome 576 (28.2%); bicuspid aortic valve disease 504 (24.6%), aneurysm or dissection age <50 years 369 (18%), and others.] Biologic samples for DNA analyses (WBC’s or saliva) are available in 97% and stored plasma is available in 60% of enrollees. Results Initial scientific inquiry using the GenTAC Registry has included validation studies of genetic causes for aortic syndromes, potential usefulness of TGFB blood levels in Marfan subjects, and current surgical approaches to ascending aortic conditions. Future Opportunity GenTAC II will allow biannual follow-up of GenTAC I enrollees for up to nine years, enrollment of an additional 1,500 subjects, further integration of imaging findings with clinical and genetic data through utilization of an imaging core lab, important validation of phenotype-genotype correlations through a phenotyping core lab, and integration of a scientific advisory committee to help define the full range and depth of the Registry’s scientific capabilities. The registry resources are available to the external scientific community through an application process accessible at https://gentac.rti.org. PMID:21982653

  1. 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.

  2. The National Neurosurgery Quality and Outcomes Database Qualified Clinical Data Registry: 2015 measure specifications and rationale.

    PubMed

    Parker, Scott L; McGirt, Matthew J; Bekelis, Kimon; Holland, Christopher M; Davies, Jason; Devin, Clinton J; Atkins, Tyler; Knightly, Jack; Groman, Rachel; Zyung, Irene; Asher, Anthony L

    2015-12-01

    Meaningful quality measurement and public reporting have the potential to facilitate targeted outcome improvement, practice-based learning, shared decision making, and effective resource utilization. Recent developments in national quality reporting programs, such as the Centers for Medicare & Medicaid Services Qualified Clinical Data Registry (QCDR) reporting option, have enhanced the ability of specialty groups to develop relevant quality measures of the care they deliver. QCDRs will complete the collection and submission of Physician Quality Reporting System (PQRS) quality measures data on behalf of individual eligible professionals. The National Neurosurgery Quality and Outcomes Database (N(2)QOD) offers 21 non-PQRS measures, initially focused on spine procedures, which are the first specialty-specific measures for neurosurgery. Securing QCDR status for N(2)QOD is a tremendously important accomplishment for our specialty. This program will ensure that data collected through our registries and used for PQRS is meaningful for neurosurgeons, related spine care practitioners, their patients, and other stakeholders. The 2015 N(2)QOD QCDR is further evidence of neurosurgery's commitment to substantively advancing the health care quality paradigm. The following manuscript outlines the measures now approved for use in the 2015 N(2)QOD QCDR. Measure specifications (measure type and descriptions, related measures, if any, as well as relevant National Quality Strategy domain[s]) along with rationale are provided for each measure.

  3. Strategies to achieve sustainability and quality in birth defects registries: the experience of the National Registry of Congenital Anomalies of Argentina.

    PubMed

    Groisman, Boris; Bidondo, Maria Paz; Gili, Juan Antonio; Barbero, Pablo; Liascovich, Rosa

    2013-01-01

    In many low-and middle-income countries, birth defects are not considered a public health priority and are perceived by the medical community as rare, unpreventable events. In this context, a registry of birth defects should address not only the collection, analysis, and dissemination of information but also contribute to local interventions like prevention, diagnosis, and treatment. We describe the National Registry of Congenital Anomalies of Argentina (RENAC) in terms of case definition, data collection, quality assurance, and data sending, coding, analysis, and information dissemination and we present the strategies used to ensure its sustainability. We emphasize strategies for motivating the people collecting data, such as training activities, participation in research projects, returning the processed data, making useful clinical information available, giving non-monetary rewards, and linking cases to genetic services.

  4. Immunization registries can be building blocks for national health information systems.

    PubMed

    Hinman, Alan R; Ross, David A

    2010-04-01

    Electronic health records and health information exchanges are necessary components of the information infrastructure to support a reformed health care system. However, they are not sufficient by themselves. Merely summing data from electronic health records together will not provide a comprehensive picture of the population, which is essential for tracking disease trends and treatment outcomes. Public health information systems such as immunization registries are an essential component of the information infrastructure and will allow assessment of the impact of changes in health care on the population as a whole.

  5. Quality control of a national bone marrow donor registry: results of a pilot study and proposal for a standardized approach.

    PubMed

    Tiercy, J-M; Stadelmann, S; Chapuis, B; Gratwohl, A; Schanz, U; Seger, R A; Faveri, G Nicoloso de; Kern, M; Morell, A; Schwabe, R; Schneider, P

    2003-09-01

    Unrelated hematopoietic stem cell transplantation (HSCT) is a recognized therapy for hematological diseases and over 8 million HLA-typed donors are ready to donate. Increased international exchanges and rapid requests through the Bone Marrow Donor Worldwide (BMDW) ask for standardized quality assurance. Since no such standards have been established to date, we tested a pilot program in order to evaluate donor availability and quality of HLA typing of the Swiss Registry. The 18500 donors of the registry have been analyzed by serology for HLA-AB and by molecular typing for HLA-DR. Through three successive annual quality control (QC) exercises, a total of 114 donor requests were sent to 13 blood transfusion centers responsible for donor recruitment asking for a blood sample. Donors were randomly selected according to recruitment periods (1988-1993; 1994-1997; 1998-2000), and to homozygosity for HLA-A and/or -B antigens. An additional 80 frozen blood samples from the repository corresponding to the three periods (n=26) and to the 2001 period (n=54) were also included in the HLA study. HLA-AB typings were done by polymerase chain reaction-sequence specific primers (PCR-SSP) and all discrepancies were retyped. The results showed that 79 samples provided by 69.3% of the requested donors were received within 14 days, and 19 samples (16.7%) were received in >14 days. Altogether, an 86% rate of donor availability was observed, independent of the recruitment period. Among the requested donors, 16 (14%) were not available: for medical reasons (two), for personal reasons (eight), for loss (one), and for an unknown reason (five). The HLA-A/B DNA typing results of 166 homozygous and 12 heterozygous blood samples showed that 437/439 (99.5%) of the assigned A/B antigens were correct. However in 36/178 donors (20.2%) an HLA-A or -B antigen had been missed (34 donors) or misassigned (two donors) by serology, with a decreasing discrepancy rate of 30% (1988-1993) to 18.5% in 2001

  6. The Global Network Maternal Newborn Health Registry: a multi-national, community-based registry of pregnancy outcomes

    PubMed Central

    2015-01-01

    Background The Global Network for Women's and Children's Health Research (Global Network) supports and conducts clinical trials in resource-limited countries by pairing foreign and U.S. investigators, with the goal of evaluating low-cost, sustainable interventions to improve the health of women and children. Accurate reporting of births, stillbirths, neonatal deaths, maternal mortality, and measures of obstetric and neonatal care is critical to efforts to discover strategies for improving pregnancy outcomes in resource-limited settings. Because most of the sites in the Global Network have weak registration within their health care systems, the Global Network developed the Maternal Newborn Health Registry (MNHR), a prospective, population-based registry of pregnancies at the Global Network sites to provide precise data on health outcomes and measures of care. Methods Pregnant women are enrolled in the MNHR if they reside in or receive healthcare in designated groups of communities within sites in the Global Network. For each woman, demographic, health characteristics and major outcomes of pregnancy are recorded. Data are recorded at enrollment, the time of delivery and at 42 days postpartum. Results From 2010 through 2013 Global Network sites were located in Argentina, Guatemala, Belgaum and Nagpur, India, Pakistan, Kenya, and Zambia. During this period, 283,496 pregnant women were enrolled in the MNHR; this number represented 98.8% of all eligible women. Delivery data were collected for 98.8% of women and 42-day follow-up data for 98.4% of those enrolled. In this supplement, there are a series of manuscripts that use data gathered through the MNHR to report outcomes of these pregnancies. Conclusions Developing public policy and improving public health in countries with poor perinatal outcomes is, in part, dependent upon understanding the outcome of every pregnancy. Because the worst pregnancy outcomes typically occur in countries with limited health registration

  7. National Hematopoietic Stem Cells Transplant Registry in Poland: Nationwide Internet Reporting System and Results.

    PubMed

    Łęczycka, A; Dudkiewicz, M; Czerwiński, J; Malanowski, P; Żalikowska-Hołoweńko, J; Danielewicz, R

    2016-06-01

    History of hematopoietic stem cell transplantations in Poland begins in early 1980s; the 1st bone marrow allotransplantation was performed in 1983 in the Central Clinical Hospital of the Military Medical Academy in Warsaw. Following years brought the 1st autologous stem cell transplantations. Ten years later, unrelated bone marrow transplantation was performed for the 1st time by the team of the Hematology and Blood and Marrow Transplantation Unit in Katowice. Since then, hematopoietic stem cell transplantation developed to be standard procedure and one of the most important therapies applied in leukemia treatment. The number of allotransplantations in Poland has grown significantly in the past 2 decades, which generated new needs and problems. In 2005, based on a new Transplant Law, a National Transplants Registry was created. Its main role is to collect data (registration of procedures and follow-up data) related to every transplantation case for stem cells and tissues as well as for organs. We present statistics concerning stem cell transplantations performed in Poland, as collected in the National Transplants Registry in the years 2006-2014. There are 18 centers transplanting hematopoietic stem cells in Poland. The total number of hematopoietic stem cell transplantations performed in 2006-2014 was 3,537, with allotransplantations from relatives accounted for 1,491 and from unrelated donors for 2,046. The main indication for allotransplantation in past years was acute leukemia.

  8. Malaysian Cardiothoracic Surgery Registry--a patient registry to evaluate the health outcomes of patients undergoing surgery for cardiothoracic diseases in Malaysia.

    PubMed

    Anas, R; Rahman, I; Jahizah, H; Hassan, A; Ezani, T; Jong, Y H; Norzalina, E; Ziyadi, G; Balan, S; Ramadan, J; Lim, T O; Jamaiyah, H; Hidayah, H

    2008-09-01

    The formulation of the Cardiothoracic Registry. Cardiothoracic surgery is the field of medicine involved in surgical treatment of diseases affecting organs inside the thorax (the chest). It is a general treatment of conditions of the heart (heart disease) and lungs (lung disease). In Malaysia, due to lack of data collection we do not have estimates of number and outcome of such procedure in the country. Western figures are often used as our reference values and this may not accurately reflect our Malaysian population. The Malaysian Cardiothoracic Surgery Registry (MyCARE) by the Ministry of Health will be a valuable tool to provide timely and robust data of cardiology practice, its safety and cost effectiveness and most importantly the outcome of these patients in the Malaysian setting.

  9. Ethical issues involved in establishing a registry for familial Alzheimer's disease.

    PubMed

    Godard, B; Knoppers, B M; Glass, K; Grenon, M; Bouchard, R; Bouvier, M; Goulet, J; Gauvreau, D

    1994-01-01

    In January 1992, the IMAGE Project extended the establishment of its registry of familial Alzheimer cases to all areas of the province of Quebec, for collection of epidemiological and clinical data, as well as biological samples. The aim is to study genetic transmission patterns of Alzheimer's disease (AD) and to provide a sampling framework for further etiologic and risk factor studies. The IMAGE registry already includes data of a population-based study in the Saguenay-Lac-St-Jean area; the project to collect data on familial AD (FAD) cases across the province of Quebec is known as the ALGENE Initiative. The registry is thus a collection of "AD families" for both familial and sporadic cases. The establishment of the registry involves several steps in the field work: recruitment and selection of families; collection of information on family medical history; selection of informative families and genetic testing for AD/FAD by linkage analysis. As AD is not homogeneous in its etiology and since we do not know if, in the event that genetics is involved in AD whether or not penetrance of the gene(s) is high, we must be aware of the "genetic horizons" of AD in collecting and conserving data on families of cases, and in the genetic testing for AD/FAD by linkage analysis. Families who choose genetic testing must be aware of the implications of our undertaking, assured of the confidentiality of the test and, at the same time, they must understand its limitations. The experimental nature of our research project raises ethical dilemmas. This article examines these initial considerations of the field work involved in developing a registry pertaining to genetic testing for AD/FAD by linkage analysis and offers some preliminary observations on the experience of the first year of this project.

  10. 77 FR 2126 - Pipeline Safety: Implementation of the National Registry of Pipeline and Liquefied Natural Gas...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-01-13

    ... Registry of Pipeline and Liquefied Natural Gas Operators AGENCY: Pipeline and Hazardous Materials Safety... registry of pipeline and liquefied natural gas operators. FOR FURTHER INFORMATION CONTACT: Jamerson Pender... 72878), titled: ``Pipeline Safety: Updates to Pipeline and Liquefied Natural Gas Reporting...

  11. Evaluation of ICD-10 algorithms to identify hypopituitary patients in the Danish National Patient Registry

    PubMed Central

    Berglund, Agnethe; Olsen, Morten; Andersen, Marianne; Nielsen, Eigil Husted; Feldt-Rasmussen, Ulla; Kistorp, Caroline; Gravholt, Claus Højbjerg; Stochhholm, Kirstine

    2017-01-01

    Objective Routinely collected health data may be valuable sources for conducting research. This study aimed to evaluate the validity of algorithms detecting hypopituitary patients in the Danish National Patient Registry (DNPR) using medical records as reference standard. Study design and setting Patients with International Classification of Diseases (10th edition [ICD-10]) diagnoses of hypopituitarism, or other diagnoses of pituitary disorders assumed to be associated with an increased risk of hypopituitarism, recorded in the DNPR during 2000–2012 were identified. Medical records were reviewed to confirm or disprove hypopituitarism. Results Hypopituitarism was confirmed in 911 patients. In a candidate population of 1,661, this yielded an overall positive predictive value (PPV) of 54.8% (95% confidence interval [CI]: 52.4–57.3). Using algorithms searching for patients recorded at least one, three or five times with a diagnosis of hypopituitarism (E23.0x) and/or at least once with a diagnosis of postprocedural hypopituitarism (E89.3x), PPVs gradually increased from 73.3% (95% CI: 70.6–75.8) to 83.3% (95% CI: 80.7–85.7). Completeness for the same algorithms, however, decreased from 90.8% (95% CI: 88.7–92.6) to 82.9% (95% CI: 80.3–85.3) respectively. Including data of hormone replacement in the same algorithms PPVs increased from 73.2% (95% CI: 70.6–75.7) to 82.6% (95% CI: 80.1–84.9) and completeness decreased from 94.3% (95% CI: 92.6–95.7) to 89.7% (95% CI: 87.5–91.6) with increasing records of E23.0x. Conclusion The DNPR is a valuable data source to identify hypopituitary patients using a search criteria of at least five records of E23.0x and/or at least one record of E89.3x. Completeness is increased when including hormone replacement data in the algorithm. The consequences of misclassification must, however, always be considered. PMID:28223847

  12. Incidence of primary breast cancer in Iran: Ten-year national cancer registry data report.

    PubMed

    Jazayeri, Seyed Behzad; Saadat, Soheil; Ramezani, Rashid; Kaviani, Ahmad

    2015-08-01

    Breast cancer is the leading type of malignancy and the leading cause of cancer-related deaths in women worldwide. The screening programs and advances in the treatment of patients with breast cancer have led to an increase in overall survival. Cancer registry systems play an important role in providing basic data for research and the monitoring of the cancer status. In this study, the results of the 10-year national cancer registry (NCR) of Iran in breast cancer are reviewed. NCR database records were searched for primary breast cancer records according to ICD-O-3 coding and the cases were reviewed. A total of 52,068 cases were found with the coding of primary breast cancer. Females constituted 97.1% of the cases. Breast cancer was the leading type of cancer in Iranian females, accounting for 24.6% of all cancers. The mean age of the women with breast cancer was 49.6 years (95%CI 49.5-49.6). Most of the cases (95.7%) were registered as having invasive pathologies (behavior code 3). The most common morphology of primary breast cancer was invasive ductal carcinoma (ICD-O 8500/3) followed by invasive lobular carcinoma (ICD-O 8520/3) with relative frequencies of 77.8% and 5.2%, respectively. The average annual crude incidence of primary breast cancer in females was 22.6 (95%CI 22.1-23.1) per 100,000 females, with an age-standardized rate (ASR) of 27.4 (95%CI 22.5-35.9). There were no data on survival, staging or immunohistochemical marker(s) of the breast-cancer-registered cases. The incidence of breast cancer in Iran is lower than in low-middle-income neighboring countries. The NCR data registry of breast cancer is not accurate in monitoring the effect of screening programs or determining the current status of breast cancer in Iran. Screening programs of breast cancer in Iran have failed to enhance the detection of the patients with in situ lesion detection. A quality breast cancer registry and a screening program for breast cancer are both needed.

  13. Hematologic malignancies in South Africa 2000-2006: analysis of data reported to the National Cancer Registry.

    PubMed

    Schonfeld, Sara J; Erdmann, Friederike; Wiggill, Tracey; Singh, Elvira; Kellett, Patricia; Babb, Chantal; Schüz, Joachim

    2016-04-01

    Little is known about the incidence patterns of hematologic malignancies in Sub-Saharan Africa, including South Africa. We estimated incidence rates of pathology-confirmed adult cases of leukemia, myeloma and related diseases (myeloma), Hodgkin lymphoma (HL), and non-Hodgkin lymphoma (NHL) reported to the National Cancer Registry of South Africa (NCR) between 2000 and 2006, by age, gender, and population group (Black, White, Coloured, Asian/Indian). Gender-specific age-standardized rates were calculated overall and by population group and incidence rate ratios (IRRs) were estimated using Poisson regression models. Between 2000 and 2006, there were 14662 cases of leukemia, myeloma, HL, and NHL reported to the registry. Incidence rates of reported hematologic malignancies were generally 20-50% higher among males than females. Our analyses suggested marked differences in the rates of reported hematologic malignancies by population group which were most pronounced when comparing the White versus Black population groups (IRRs ranging from 1.6 for myeloma to 3.8 for HL for males and females combined). Challenges related to diagnosis and reporting of cancers may play a role in the patterns observed by population group while the set-up of the NCR (pathology-based) could lead to some degree of under-ascertainment in all groups. This is the first country-wide report of the incidence of hematologic malignancies in South Africa. Despite challenges, it is important to analyze and report available national cancer incidence data to raise awareness of the cancer burden and to characterize patterns by demographic characteristics so as ultimately to improve the provision of cancer-related health care.

  14. Down syndrome: issues to consider in a national registry, research database and biobank.

    PubMed

    McCabe, Linda L; McCabe, Edward R B

    2011-01-01

    As the quality of life for individuals with Down syndrome continues to improve due to anticipatory healthcare, early intervention, mainstreaming in schools, and increased expectations, the lack of basic information regarding individuals with Down syndrome is being recognized, and the need to facilitate research through a national registry, research database and biobank is being discussed. We believe that there should not be ownership of the samples and information, but instead prefer stewardship of the samples and information to benefit the participants who provided them. We endorse a model with data and sample managers and a research review board to interface between the investigators and participants. Information and samples would be coded, and only a few data managers would know the relationship between the codes and identifying information. Research results once published should be included in an online newsletter. If appropriate, individual results should be shared with participants. A Down syndrome registry, research database and biobank should be accountable to participants, families, medical care providers, government, and funding sources.

  15. Cancer Incidence in Egypt: Results of the National Population-Based Cancer Registry Program

    PubMed Central

    Ibrahim, Amal S.; Khaled, Hussein M.; Mikhail, Nabiel NH; Baraka, Hoda; Kamel, Hossam

    2014-01-01

    Background. This paper aims to present cancer incidence rates at national and regional level of Egypt, based upon results of National Cancer Registry Program (NCRP). Methods. NCRP stratified Egypt into 3 geographical strata: lower, middle, and upper. One governorate represented each region. Abstractors collected data from medical records of cancer centers, national tertiary care institutions, Health Insurance Organization, Government-Subsidized Treatment Program, and death records. Data entry was online. Incidence rates were calculated at a regional and a national level. Future projection up to 2050 was also calculated. Results. Age-standardized incidence rates per 100,000 were 166.6 (both sexes), 175.9 (males), and 157.0 (females). Commonest sites were liver (23.8%), breast (15.4%), and bladder (6.9%) (both sexes): liver (33.6%) and bladder (10.7%) among men, and breast (32.0%) and liver (13.5%) among women. By 2050, a 3-fold increase in incident cancer relative to 2013 was estimated. Conclusion. These data are the only available cancer rates at national and regional levels of Egypt. The pattern of cancer indicated the increased burden of liver cancer. Breast cancer occupied the second rank. Study of rates of individual sites of cancer might help in giving clues for preventive programs. PMID:25328522

  16. 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.

  17. A new data management system for the French National Registry of human alveolar echinococcosis cases.

    PubMed

    Charbonnier, Amandine; Knapp, Jenny; Demonmerot, Florent; Bresson-Hadni, Solange; Raoul, Francis; Grenouillet, Frédéric; Millon, Laurence; Vuitton, Dominique Angèle; Damy, Sylvie

    2014-01-01

    Alveolar echinococcosis (AE) is an endemic zoonosis in France due to the cestode Echinococcus multilocularis. The French National Reference Centre for Alveolar Echinococcosis (CNR-EA), connected to the FrancEchino network, is responsible for recording all AE cases diagnosed in France. Administrative, epidemiological and medical information on the French AE cases may currently be considered exhaustive only on the diagnosis time. To constitute a reference data set, an information system (IS) was developed thanks to a relational database management system (MySQL language). The current data set will evolve towards a dynamic surveillance system, including follow-up data (e.g. imaging, serology) and will be connected to environmental and parasitological data relative to E. multilocularis to better understand the pathogen transmission pathway. A particularly important goal is the possible interoperability of the IS with similar European and other databases abroad; this new IS could play a supporting role in the creation of new AE registries.

  18. A new data management system for the French National Registry of human alveolar echinococcosis cases

    PubMed Central

    Charbonnier, Amandine; Knapp, Jenny; Demonmerot, Florent; Bresson-Hadni, Solange; Raoul, Francis; Grenouillet, Frédéric; Millon, Laurence; Vuitton, Dominique Angèle; Damy, Sylvie

    2014-01-01

    Alveolar echinococcosis (AE) is an endemic zoonosis in France due to the cestode Echinococcus multilocularis. The French National Reference Centre for Alveolar Echinococcosis (CNR-EA), connected to the FrancEchino network, is responsible for recording all AE cases diagnosed in France. Administrative, epidemiological and medical information on the French AE cases may currently be considered exhaustive only on the diagnosis time. To constitute a reference data set, an information system (IS) was developed thanks to a relational database management system (MySQL language). The current data set will evolve towards a dynamic surveillance system, including follow-up data (e.g. imaging, serology) and will be connected to environmental and parasitological data relative to E. multilocularis to better understand the pathogen transmission pathway. A particularly important goal is the possible interoperability of the IS with similar European and other databases abroad; this new IS could play a supporting role in the creation of new AE registries. PMID:25526544

  19. The Management of Iron Chelation Therapy: Preliminary Data from a National Registry of Thalassaemic Patients

    PubMed Central

    Ceci, Adriana; Mangiarini, Laura; Felisi, Mariagrazia; Bartoloni, Franco; Ciancio, Angela; Capra, Marcello; D'Ascola, Domenico; Cianciulli, Paolo; Filosa, Aldo

    2011-01-01

    Thalassaemia and other haemoglobinopathies constitute an important health problem in Mediterranean countries, placing a tremendous emotional, psychological, and economic burden on their National Health systems. The development of new chelators in the most recent years had a major impact on the treatment of thalassaemia and on the quality of life of thalassaemic patients. A new initiative was promoted by the Italian Ministry of Health, establishing a Registry for thalassaemic patients to serve as a tool for the development of cost-effective diagnostic and therapeutic approaches and for the definition of guidelines supporting the most appropriate management of the iron-chelating therapy and a correct use of the available iron-chelating agents. This study represents the analysis of the preliminary data collected for the evaluation of current status of the iron chelation practice in the Italian thalassaemic population and describes how therapeutic interventions can widely differ in the different patients' age groups. PMID:21738864

  20. ATSDR evaluation of health effects of chemicals. VI. Di(2-ethylhexyl)phthalate. Agency for Toxic Substances and Disease Registry.

    PubMed

    Fay, M; Donohue, J M; De Rosa, C

    1999-12-01

    Di(2-ethylhexyl)phthalate (also known as DEHP, bis(2-ethylhexyl)phthalate, or BEHP; CAS Registry Number 117-81-7) is a widely-used plasticizer. It is found in numerous plastic articles, such as paints, inks, floor tiles, upholstery, shower curtains, footwear, plastic bags, food-packaging materials, toys, and medical tubing. Not surprisingly, DEHP appears at many waste sites. As part of its mandate, the Agency for Toxic Substances and Disease Registry (ATSDR) prepares toxicological profiles on hazardous chemicals that are of greatest public health concern at Comprehensive Environmental Response, Compensation, and Liability Act (CERCLA) National Priority List (NPL) sites. These profiles comprehensively summarize toxicological and environmental information. This article constitutes the release of the bulk of ATSDR's profile for DEHP (ATSDR, 1993) into the mainstream scientific literature. An extensive listing of human and animal health effects, organized by route, duration, and endpoint, is presented. Toxicological information on toxicokinetics, biomarkers, interactions, sensitive subpopulations, reducing toxicity after exposure, and relevance to public health is also included. Environmental information encompasses physical properties, production and use, environmental fate, levels seen in the environment, analytical methods, and a listing of regulations. ATSDR, at the behest of Congress and therefore the citizenry, prepares these profiles to inform the public about site contaminants.

  1. 75 FR 51075 - National Registry of Evidence-Based Programs and Practices (NREPP): Open Submission Period for...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-18

    ... Practices (NREPP): Open Submission Period for Fiscal Year 2011 Background The Substance Abuse and Mental... HUMAN SERVICES Substance Abuse and Mental Health Services Administration National Registry of Evidence-Based Programs and Practices (NREPP): Open Submission Period for Fiscal Year 2011 AGENCY:...

  2. A Squandered Opportunity?: A Review of SAMHSA's National Registry of Evidence-Based Programs and Practices for Offenders

    ERIC Educational Resources Information Center

    Wright, Benjamin J.; Zhang, Sheldon X.; Farabee, David

    2012-01-01

    In the past decade, the push for evidence-based programs has taken on unprecedented prominence in the fields of substance abuse and correctional treatment as a key determinant for intervention funding. The National Registry of Evidence-based Programs and Practices (NREPP), managed and funded by the Substance Abuse and Mental Health Services…

  3. Gender and Geographic Differences in Developmental Delays among Young Children: Analysis of the Data from the National Registry in Taiwan

    ERIC Educational Resources Information Center

    Lai, Der-Chung; Tseng, Yen-Cheng; Guo, How-Ran

    2011-01-01

    Although developmental delays are not uncommon in children, the incidence is seldom assessed, and the reported prevalence varies widely. In Taiwan, the government mandates the reporting of suspected cases. Using the national registry data, we conducted a study to estimate the incidence and prevalence of developmental delays in young children in…

  4. Hospital for joint diseases participates in international spine registry Spine Tango after successful pilot study.

    PubMed

    Röder, Christoph; Errico, Thomas J; Spivak, Jeffrey M; Murray, M; Protopsaltis, T; Lis, A; Nordin, Margareta; Bendo, John

    2012-01-01

    Spine Tango is currently the only international spine registry in existence. It was developed under the auspices of Eurospine, the Spine Society of Europe, and is hosted at the University of Bern, Switzerland. The HJD Spine Center successfully tested Spine Tango during a 3-month pilot study and has since expanded documentation activities to more surgeons. Workflow integration and dedicated research staff are key factors for such an endeavor. Participation enables benchmarking against national and international peers and outcome research and quality assurance of surgical and non-surgical treatments.

  5. An ontology-based annotation of cardiac implantable electronic devices to detect therapy changes in a national registry.

    PubMed

    Rosier, Arnaud; Mabo, Philippe; Chauvin, Michel; Burgun, Anita

    2015-05-01

    The patient population benefitting from cardiac implantable electronic devices (CIEDs) is increasing. This study introduces a device annotation method that supports the consistent description of the functional attributes of cardiac devices and evaluates how this method can detect device changes from a CIED registry. We designed the Cardiac Device Ontology, an ontology of CIEDs and device functions. We annotated 146 cardiac devices with this ontology and used it to detect therapy changes with respect to atrioventricular pacing, cardiac resynchronization therapy, and defibrillation capability in a French national registry of patients with implants (STIDEFIX). We then analyzed a set of 6905 device replacements from the STIDEFIX registry. Ontology-based identification of therapy changes (upgraded, downgraded, or similar) was accurate (6905 cases) and performed better than straightforward analysis of the registry codes (F-measure 1.00 versus 0.75 to 0.97). This study demonstrates the feasibility and effectiveness of ontology-based functional annotation of devices in the cardiac domain. Such annotation allowed a better description and in-depth analysis of STIDEFIX. This method was useful for the automatic detection of therapy changes and may be reused for analyzing data from other device registries.

  6. Recent Progress of the ARegPKD Registry Study on Autosomal Recessive Polycystic Kidney Disease

    PubMed Central

    Ebner, Kathrin; Schaefer, Franz; Liebau, Max Christoph; Eid, L. A.

    2017-01-01

    Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disease with a severe phenotype often presenting prenatally or in early childhood. With its obligate renal and hepatic involvement, ARPKD is one of the most important indications for liver and/or kidney transplantation in childhood. Marked phenotypic variability is observed, the genetic basis of which is largely unknown. Treatment is symptomatic and largely empiric as evidence-based guidelines are lacking. Therapeutic initiatives for ARPKD face the problem of highly variable cohorts and lack of clinical or biochemical risk markers without clear-cut clinical end points. ARegPKD is an international, multicenter, retro- and prospective, observational study to deeply phenotype patients with the clinical diagnosis of ARPKD. Initiated in 2013 as a web-based registry (www.aregpkd.org), ARegPKD enrolls patients across large parts of Europe and neighboring countries. By January 2017, more than 400 patients from 17 mostly European countries have been registered in the ARPKD registry study with significant follow-up data. Due to comprehensive retro- and prospective data collection and associated biobanking, ARegPKD will generate a unique ARPKD cohort with detailed longitudinal clinical characterization providing a basis for future clinical trials as well as translational research. Hence, ARegPKD is hoped to contribute to the pathophysiological understanding of the disease and to the improvement of clinical management. PMID:28296980

  7. Congenital malformations in Ecuadorian children: urgent need to create a National Registry of Birth Defects

    PubMed Central

    González-Andrade, Fabricio; López-Pulles, Ramiro

    2010-01-01

    Aim This study sets out (a) to estimate the prevalence of admissions by birth defects, using the official database of hospitals of Ecuador; and (b) to set the basis for a new National Register of Birth Defects in Ecuador that works as a program for the clinical and epidemiological investigation of risk factors in the etiology of congenital anomalies in Ecuadorian hospitals, using a case-control methodological approach. This is the first report in their class. Methods The data used in this study are derived from the National Register of Hospital Admission/Discharges of the Instituto Nacional de Estadísticas y Censos; data of the Ministry of Public Health were also used. Ecuador does not have an official Medical Birth Registry or a Congenital Malformations Registry. Results A total of 51,375 discharges by congenital malformations were registered in a 7-year period. Of these, 16,679 admissions were of children aged less than 1 year of age, with a birth prevalence rate (BPR) of 72.33/10,000 births. 77% of the congenital defects registered comprise the 50 most common birth defects observed in this age group. Cleft lip was the most prevalent birth defect in children less than 1 year of age and the second most common defect in children 1 to 5 years of age. Unilateral cleft lip shows a BPR of 4.57/10,000 births; cardiac birth defects as a group have a BPR of 4.2; hydrocephalus a BPR of 3.77; and Down’s syndrome a BPR of 3.70. Undescended testicle was the most prevalent birth defect in children between 1 to 5 years. 9384 children under 1 year of age were male (55.9%) and 7053 were female (42.1%). BPR in males was 40.45 and in females 30.40. Conclusion This report documents the prevalence estimates for birth defects reported in the hospital discharge data. These estimates are important to 1) plan for health-care and education needs of the Ecuadorian population, 2) identify increased occurrences of birth defects in specific geographic regions, 3) serve as a reference point

  8. A Registry Framework Enabling Patient-Centred Care.

    PubMed

    Bellgard, Matthew I; Napier, Kathryn; Render, Lee; Radochonski, Maciej; Lamont, Leanne; Graham, Caroline; Wilton, Steve D; Fletcher, Sue; Goldblatt, Jack; Hunter, Adam A; Weeramanthri, Tarun

    2015-01-01

    Clinical decisions rely on expert knowledge that draws on quality patient phenotypic and physiological data. In this regard, systems that can support patient-centric care are essential. Patient registries are a key component of patient-centre care and can come in many forms such as disease-specific, recruitment, clinical, contact, post market and surveillance. There are, however, a number of significant challenges to overcome in order to maximise the utility of these information management systems to facilitate improved patient-centred care. Registries need to be harmonised regionally, nationally and internationally. However, the majority are implemented as standalone systems without consideration for data standards or system interoperability. Hence the task of harmonisation can become daunting. Fortunately, there are strategies to address this. In this paper, a disease registry framework is outlined that enables efficient deployment of national and international registries that can be modified dynamically as registry requirements evolve. This framework provides a basis for the development and implementation of data standards and enables patients to seamlessly belong to multiple registries. Other significant advances include the ability for registry curators to create and manage registries themselves without the need to contract software developers, and the concept of a registry description language for ease of registry template sharing.

  9. Establishment of a Population-based Registry of Inflammatory Bowel Diseases in Fars Province, Iran.

    PubMed

    Taghavi, Seyed Alireza; Bagheri Lankarani, Kamran; Moini, Maryam; Hamidpour, Laleh; Ardebili, Maryam; Mansoorabadi, Zahra

    2012-04-01

    BACKGROUND Inflammatory bowel diseases (IBD) are debilitating diseases that lead to a variety of problems in a patient's daily life and are a huge burden for the health care system. Since this group of diseases are multifactorial and complex, long-term longitudinal studies are clearly needed to understand them better. A population-based registry (IBD-FaR) has been established in Fars, a southern Iranian province, with the intent to create a reliable data source. This registry will be of considerable help in future planning of health care resources necessary to deal with IBD and to enable investigators to test their theories on the origin and/or treatment of IBD. METHODS This registry is managed by both the Gastroenterohepatology Research Center and Health Policy Research Center at Shiraz University of Medical Sciences. A governing committee is responsible for decisions regarding budget allocations and use of data. The designed questionnaire includes a consent form, basic history data, risk factors, related procedures, medical therapy, and follow-up data. The establishment process has two parallel phases: in the first phase, data is collected from numerous sources, including annual hospital discharge data, referral from university affiliated physicians and private practices, pathologic reports, death certificates, self-referral, and insurance system data. In the interview, the questionnaire is completed and blood samples are taken. The gathered data are entered in a custom-designed, computerized data base. In the second phase, annual follow up interviews will be conducted. New IBD patients are also being registered. This phase will continue indefinitely, in order to include new incident cases. RESULTS Briefly, from May 2011 until December 2011, there were 188 patients [94 (50%) females and 94 (50%) males] diagnosed with IBD who were registered in IBD-FaR. Patients' age range was between 15 and 80 years. A total of 164 (87.2%) patients out of 188 were registered as

  10. Danish Prostate Cancer Registry – methodology and early results from a novel national database

    PubMed Central

    Helgstrand, JT; Klemann, N; Røder, MA; Toft, BG; Brasso, K; Vainer, B; Iversen, P

    2016-01-01

    Background Systematized Nomenclature of Medicine (SNOMED) codes are computer-processable medical terms used to describe histopathological evaluations. SNOMED codes are not readily usable for analysis. We invented an algorithm that converts prostate SNOMED codes into an analyzable format. We present the methodology and early results from a new national Danish prostate database containing clinical data from all males who had evaluation of prostate tissue from 1995 to 2011. Materials and methods SNOMED codes were retrieved from the Danish Pathology Register. A total of 26,295 combinations of SNOMED codes were identified. A computer algorithm was developed to transcode SNOMED codes into an analyzable format including procedure (eg, biopsy, transurethral resection, etc), diagnosis, and date of diagnosis. For validation, ~55,000 pathological reports were manually reviewed. Prostate-specific antigen, vital status, causes of death, and tumor-node-metastasis classification were integrated from national registries. Results Of the 161,525 specimens from 113,801 males identified, 83,379 (51.6%) were sets of prostate biopsies, 56,118 (34.7%) were transurethral/transvesical resections of the prostate (TUR-Ps), and the remaining 22,028 (13.6%) specimens were derived from radical prostatectomies, bladder interventions, etc. A total of 48,078 (42.2%) males had histopathologically verified prostate cancer, and of these, 78.8% and 16.8% were diagnosed on prostate biopsies and TUR-Ps, respectively. Future perspectives A validated algorithm was successfully developed to convert complex prostate SNOMED codes into clinical useful data. A unique database, including males with both normal and cancerous histopathological data, was created to form the most comprehensive national prostate database to date. Potentially, our algorithm can be used for conversion of other SNOMED data and is available upon request. PMID:27729813

  11. Incidence of acute myocardial infarction in Islamic Republic of Iran: a study using national registry data in 2012.

    PubMed

    Ahmadi, A; Soori, H; Mehrabi, Y; Etemad, K; Samavat, T; Khaledifar, A

    2015-02-25

    Population-based data on myocardial infarction rates in the Islamic Republic of Iran have not been reported on a national or provincial scale. In a cross-sectional study, data were collected on 20 750 new cases of myocardial infarction (ICD10 codes I21-22) admitted to hospitals and registered by the Iranian Myocardial Infarction Registry in 2012. The crude and age-adjusted incidence for the 31 provinces and the whole country were directly calculated per 100 000 people using the WHO standard population. Overall, males comprised 72.4% of cases and had a significantly lower mean age at incidence than women [59.6 (SD 13.3) years versus 65.4 (SD 12.6) years]. The male:female incidence ratio was 2.63. The age-standardized myocardial infarction incidence rate was 73.3 per 100 000 in the whole country (95% CI: 72.3%-74.3%) and varied significantly from 24.5 to 152.5 per 100 000 across the 31 provinces. The study provides baseline data for monitoring and managing cardiovascular diseases in the country.

  12. Association of Pulmonary Tuberculosis and Diabetes in Mexico: Analysis of the National Tuberculosis Registry 2000–2012

    PubMed Central

    Delgado-Sánchez, Guadalupe; García-García, Lourdes; Castellanos-Joya, Martín; Cruz-Hervert, Pablo; Ferreyra-Reyes, Leticia; Ferreira-Guerrero, Elizabeth; Hernández, Andrés; Ortega-Baeza, Victor Manuel; Montero-Campos, Rogelio; Sulca, José Antonio; Martínez-Olivares, Ma. de Lourdes; Mongua-Rodríguez, Norma; Baez-Saldaña, Renata; González-Roldán, Jesús Felipe; López-Gatell, Hugo; Ponce-de-León, Alfredo; Sifuentes-Osornio, José; Jiménez-Corona, María Eugenia

    2015-01-01

    Background Tuberculosis (TB) remains a public health problem in Mexico while the incidence of diabetes mellitus type 2 (DM) has increased rapidly in recent years. Objective To describe the trends of incidence rates of pulmonary TB associated with DM and not associated with DM and to compare the results of treatment outcomes in patients with and without DM. Materials and Methods We analysed the National Tuberculosis Registry from 2000 to 2012 including patients with pulmonary TB among individuals older than 20 years of age. The association between DM and treatment failure was analysed using logistic regression, accounting for clustering due to regional distribution. Results In Mexico from 2000 to 2012, the incidence rates of pulmonary TB associated to DM increased by 82.64%, (p <0.001) in contrast to rates of pulmonary TB rate without DM, which decreased by 26.77%, (p <0.001). Patients with a prior diagnosis of DM had a greater likelihood of failing treatment (adjusted odds ratio, 1.34 (1.11–1.61) p <0.002) compared with patients who did not have DM. There was statistical evidence of interaction between DM and sex. The odds of treatment failure were increased in both sexes. Conclusion Our data suggest that the growing DM epidemic has an impact on the rates of pulmonary TB. In addition, patients who suffer from both diseases have a greater probability of treatment failure. PMID:26075393

  13. Low/No Calorie Sweetened Beverage Consumption in the National Weight Control Registry

    PubMed Central

    Catenacci, Victoria A.; Pan, Zhaoxing; Thomas, J. Graham; Ogden, Lorraine G.; Roberts, Susan A.; Wyatt, Holly R.; Wing, Rena R.; Hill, James O.

    2015-01-01

    Objective The aim of this cross-sectional study was to evaluate prevalence of and strategies behind low/no calorie sweetened beverage (LNCSB) consumption in successful weight loss maintainers. Methods An online survey was administered to 434 members of the National Weight Control Registry (NWCR, individuals who have lost ≥13.6 kg and maintained weight loss for > 1 year). Results While few participants (10%) consume sugar-sweetened beverages on a regular basis, 53% regularly consume LNCSB. The top five reasons for choosing LNCSB were for taste (54%), to satisfy thirst (40%), part of routine (27%), to reduce calories (22%) and to go with meals (21%). The majority who consume LNCSB (78%) felt they helped control total calorie intake. Many participants considered changing patterns of beverage consumption to be very important in weight loss (42%) and maintenance (40%). Increasing water was by far the most common strategy, followed by reducing regular calorie beverages. Conclusions Regular consumption of LNCSB is common in successful weight loss maintainers for various reasons including helping individuals to limit total energy intake. Changing beverage consumption patterns was felt to be very important for weight loss and maintenance by a substantial percentage of successful weight loss maintainers in the NWCR. PMID:25044563

  14. The completeness of chest X-ray procedure codes in the Danish National Patient Registry

    PubMed Central

    Hjertholm, Peter; Flarup, Kaare Rud; Guldbrandt, Louise Mahncke; Vedsted, Peter

    2017-01-01

    Objective The aim of this validation study was to assess the completeness of the registrations of chest X-rays (CXR) in two different versions of the Danish National Patient Registry (DNPR). Material and methods We included electronic record data on CXR performed on patients aged 40 to 99 years from nine radiology departments covering 20 Danish hospitals. From each department, we included data from three randomly selected weeks between 2004 and 2011 (reference standard). In two versions of the DNPR from the State Serum Institute (SSI) and Statistics Denmark, respectively, we investigated the proportion of registered CXR compared to the reference standard. Furthermore, we compared the completeness of the recorded data according to the responsible department (main department). Results We identified 11,235 patients and 12,513 CXR in the reference standard. The data from the SSI contained 12,265 (98%) CXR, whereas the data from Statistics Denmark comprised 9,151 (73.1%) CXR. The completeness of the SSI data was fairly constant across years, radiology departments, medical specialties, and age groups. The data from Statistics Denmark was almost complete in 2011 (95.8%). However, for the remaining study period, the data with radiology departments registered as the main department were lacking in the version from Statistics Denmark, and so the overall completeness was 73.1%. Conclusion The completeness of CXR registrations varied between 98% and 73% depending on the information source, and this should be considered when investigating radiology services in the basis of DNPR. PMID:28293121

  15. 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.

  16. The findings of the Agency for Toxic Substances and Disease Registry Medical Waste Tracking Act report.

    PubMed Central

    Lichtveld, M Y; Rodenbeck, S E; Lybarger, J A

    1992-01-01

    The Agency for Toxic Substances and Disease Registry (ATSDR) report "The Public Health Implications of Medical Waste: A Report to Congress" has been finalized and submitted to Congress. The report is a comprehensive review of all available data and information on the subject. Based on the data developed in the report, ATSDR concludes that the general public is not likely to be adversely affected by medical waste generated in the traditional health setting. However, the increase of in-home health care and other sources of nonregulated medical waste (e.g., intravenous drug users) provides opportunities for the general public to contact medical waste. In addition, ATSDR concludes that public health concerns exist for selected occupations involved with medical waste. These populations include janitorial and laundry workers, nurses, emergency medical personnel, and refuse workers. The ATSDR report also defines what material should be managed as medical waste and identifies research needs related to medical waste. PMID:1486856

  17. Females with Fabry disease frequently have major organ involvement: lessons from the Fabry Registry.

    PubMed

    Wilcox, William R; Oliveira, João Paulo; Hopkin, Robert J; Ortiz, Alberto; Banikazemi, Maryam; Feldt-Rasmussen, Ulla; Sims, Katherine; Waldek, Stephen; Pastores, Gregory M; Lee, Philip; Eng, Christine M; Marodi, Laszlo; Stanford, Kevin E; Breunig, Frank; Wanner, Christoph; Warnock, David G; Lemay, Roberta M; Germain, Dominique P

    2008-02-01

    Fabry disease (FD) is an X-linked lysosomal storage disease caused by alpha-galactosidase A deficiency. The Fabry Registry is a global clinical effort to collect longitudinal data on FD. In the past, most "carrier" females were usually thought to be clinically unaffected. A systematic effort has been made to enroll all FD females, regardless of symptomology. Of the 1077 enrolled females in the Registry, 69.4% had symptoms and signs of FD. The median age at symptom onset among females was 13 years, and even though 84.1% had a positive family history, the diagnosis was not made until a median age of 31 years. Twenty percent experienced major cerebrovascular, cardiac, or renal events, at a median age of 46 years. Among adult females with estimated glomerular filtration rate (eGFR) data (N=638), 62.5% had an eGFR <90 ml/min/1.73 m2 and 19.0% had eGFR <60 ml/min/1.73 m2. Proteinuria 300 mg/day was present in 39.0% of females, and 22.2% had >1 gram/day. Quality of life (QoL), as measured by the SF-36((R)) survey, was impaired at a later age than in males, but both genders experience significantly impaired QoL from the third decade of life onward. Thus, females with FD have a significant risk for major organ involvement and decreased QoL. Females should be regularly monitored for signs and symptoms of FD, and considered for enzyme replacement therapy.

  18. Management and risk factor control of coronary artery disease in elderly versus nonelderly: a multicenter registry

    PubMed Central

    Phrommintikul, Arintaya; Krittayaphong, Rungroj; Wongcharoen, Wanwarang; Boonyaratavej, Smonporn; Wongvipaporn, Chaiyasith; Tiyanon, Woraporn; Dinchuthai, Pakaphan; Kunjara-Na-Ayudhya, Rapeephon; Tatsanavivat, Pyatat; Sritara, Piyamitr

    2016-01-01

    Background Coronary artery disease (CAD) is a leading cause of death in elderly because aging is the important non-modifiable risk factors of atherosclerosis and also a predictor of poor outcomes. Underuse of guideline directed therapy may contribute to suboptimal risk factor control and worse outcomes in the elderly. We aimed to explore the management of CAD, risk factors control as well as goal attainment in elderly compared to nonelderly CAD patients. Methods The CORE-Thailand is an ongoing multicenter, prospective, observational registry of patients with high atherosclerotic risk in Thailand. The data of 4120 CAD patients enrolled in this cohort was analyzed comparing between the elderly (age ≥ 65 years) vs. nonelderly (age < 65 years). Results There were 2172 elderly and 1948 nonelderly patients. The elderly CAD patients had higher prevalence of hypertension, dyslipidemia, atrial fibrillation and chronic kidney disease. The proportion of patients who received coronary revascularization was not different between the elderly and nonelderly CAD patients. Antiplatelets were prescribed less in the elderly while statin was prescribed in the similar proportion. Goal attainments of risk factor control of glycemic control, low density lipoprotein cholesterol, and smoking cessation except the blood pressure goal were higher in the elderly CAD patients. Conclusions The CORE-Thailand registry showed the equity in the treatment of CAD between elderly and non-elderly. Elderly CAD patients had higher rate of goal attainment in risk factor control except blood pressure goal. The effects of goal attainment on cardiovascular outcomes will be demonstrated from ongoing cohort. PMID:28321237

  19. Validity of the Prescriber Information in the Danish National Prescription Registry.

    PubMed

    Rasmussen, Lotte; Valentin, Julie; Gesser, Katarina Margareta; Hallas, Jesper; Pottegård, Anton

    2016-10-01

    The aim of this study was to measure the validity of the prescriber information recorded in the Danish National Prescription Registry (DNPR). The prescriber information recorded in the pharmacies' electronic dispensing system was considered to represent the prescriber information recorded in the DNPR. Further, the problem of validity of the prescriber information pertains only to non-electronic prescriptions, as these are manually entered into the dispensing system. The recorded prescriber information was thus validated against information from a total of 2000 non-electronic prescriptions at five Danish community pharmacies. The validity of the recorded prescriber information was measured at the level of the individual prescriber and the prescriber type, respectively. The proportion of non-electronic prescriptions with incorrect registrations was 22.4% (95% confidence interval (CI): 20.6-24.3) when considering individual prescriber identifiers and 17.8% (95% CI: 16.1-19.5) when considering prescriber type. When excluding prescriptions specifically registered as 'missing prescriber identifier', the proportions decreased to 9.5% (95% CI: 8.2-11.0) and 4.1% (95% CI: 3.2-5.1), respectively. The positive predictive values for the classification of prescriber types were in the range of 94.0-99.2%, while the sensitivity ranged between 64.6% and 91.8%. With a maximum of 14% non-electronic prescriptions of all prescriptions in the DNPR in 2015, this corresponds to correct classification of prescriber types in the DNPR of at least 97.5%. In conclusion, the prescriber information in the DNPR was found to be valid, especially in recent years. Researchers should be aware of the low sensitivity towards prescriptions from private practicing specialists.

  20. The National Cardiovascular Data Registry Voluntary Public Reporting Program: An Interim Report From the NCDR Public Reporting Advisory Group.

    PubMed

    Dehmer, Gregory J; Jennings, Jonathan; Madden, Ruth A; Malenka, David J; Masoudi, Frederick A; McKay, Charles R; Ness, Debra L; Rao, Sunil V; Resnic, Frederic S; Ring, Michael E; Rumsfeld, John S; Shelton, Marc E; Simanowith, Michael C; Slattery, Lara E; Weintraub, William S; Lovett, Ann; Normand, Sharon-Lise

    2016-01-19

    Public reporting of health care data continues to proliferate as consumers and other stakeholders seek information on the quality and outcomes of care. Medicare's Hospital Compare website, the U.S. News & World Report hospital rankings, and several state-level programs are well known. Many rely heavily on administrative data as a surrogate to reflect clinical reality. Clinical data are traditionally more difficult and costly to collect, but more accurately reflect patients' clinical status, thus enhancing the validity of quality metrics. We describe the public reporting effort being launched by the American College of Cardiology and partnering professional organizations using clinical data from the National Cardiovascular Data Registry (NCDR) programs. This hospital-level voluntary effort will initially report process of care measures from the percutaneous coronary intervention (CathPCI) and implantable cardioverter-defibrillator (ICD) registries of the NCDR. Over time, additional process, outcomes, and composite performance metrics will be reported.

  1. The Brazilian Twin Registry.

    PubMed

    Ferreira, Paulo H; Oliveira, Vinicius C; Junqueira, Daniela R; Cisneros, Lígia C; Ferreira, Lucas C; Murphy, Kate; Ordoñana, Juan R; Hopper, John L; Teixeira-Salmela, Luci F

    2016-12-01

    The Brazilian Twin Registry (BTR) was established in 2013 and has impelled twin research in South America. The main aim of the initiative was to create a resource that would be accessible to the Brazilian scientific community as well as international researchers interested in the investigation of the contribution of genetic and environmental factors in the development of common diseases, phenotypes, and human behavior traits. The BTR is a joint effort between academic and governmental institutions from Brazil and Australia. The collaboration includes the Federal University of Minas Gerais (UFMG) in Brazil, the University of Sydney and University of Melbourne in Australia, the Australian Twin Registry, as well as the research foundations CNPq and CAPES in Brazil. The BTR is a member of the International Network of Twin Registries. Recruitment strategies used to register twins have been through participation in a longitudinal study investigating genetic and environmental factors for low back pain occurrence, and from a variety of sources including media campaigns and social networking. Currently, 291 twins are registered in the BTR, with data on demographics, zygosity, anthropometrics, and health history having been collected from 151 twins using a standardized self-reported questionnaire. Future BTR plans include the registration of thousands of Brazilian twins identified from different sources and collaborate nationally and internationally with other research groups interested on twin studies.

  2. Palivizumab prophylaxis of respiratory syncytial virus disease in 2000-2001: results from The Palivizumab Outcomes Registry.

    PubMed

    Parnes, Curt; Guillermin, Judith; Habersang, Rolf; Nicholes, Peggy; Chawla, Vijay; Kelly, Tammy; Fishbein, Judith; McRae, Patty; Goessler, Mary; Gatti, Antoinette; Calcagno, John A; Eki, Cheryl; Harris, Kristen A; Joyave, Joseph; McFarland, Kathy; Protter, Paul; Sullivan, Mary; Stanford, Allan; Lovett, Nancy; Ortiz, Marisol; Rojas, Sharon; Cyrus, Scott; Cyrus, Janell; Cohen, Stuart; Buchin, Debbie; Riordan, Linda; Zuniga, Monica; Shah, Rupa; Minard, Carmen; Quintin, Arden; Douglas, Glenda; van Houten, John; Freutner, Sharyn; Chartrand, Stephen; Nowatzke, Patsy; Romero, Jose; Rhodes, Torunn; Benoit, Michelle; Walter, Emmanuel; Walker, Leslie; DeBonnett, Laurie; Cross, Mia; Free, Teresa; Martin, Sharman; Shank, Karen; Guedes, Ben; Atkinson, Lee Ann; Halpin, George J; Rouse, Kathy; Hand, Ivan; Geiss, Donna; Marshall, James R; Burleson, Lois; Boland, Jim; Seybold, Kelsey; Hunter, Vicki; Unfer, Susan; Schmucker, Jackie; Gley, Margaret; Marcus, Michael; Thompson, Patricia; Milla, Paulino; Young, Connie; Zanni, Robert; Zinno, Virginia; Fetter-Zarzeka, Alexandra; Busey, Amanda; Sokunbi, Modupe A; Airington, Sherrie; Richard, Nancy; Muraligopal, Vellore; Lewis, Stephanie; Weber, F Thomas; Giordano, Beverly P; Linehan, Denise; Roach, Jane; Davis, Randle; Rzepka, Andrew A; Booth, Teri; Smeltzer, David; Walsh, Jeanne; Arispe, Emilio; Rowley, Rhonda; Bolling, Christopher; Botts, Tanya; Haskett, Kateri; Raby, Deana; Batiz, Evelyn; Gelfand, Andrew; Farrell, Lynn; Butler, Stephen; Colby, Linda; Schochet, Peter; Bentler, Julie; Hirsch, David; Wilkinson, Lisa; Aaronson, Allen; Bennett, Eleanora; Wingate, Julie; Quinn, Dawn; Komendowski, Katherine; Deckard, Marcia; Frogel, Michael; Nerwen, Cliff; Copenhaver, Steven; Prater, Michele; Wolsztein, Jacob; Mackey, Kristine; Benbow, Marshall; Naranjo, Marisela; Hensley, Sandra; Hayes, Cindy; Sadeghi, Hossein; Lawson, Sally May; McCall, Mark; Combs, Karla; Ledbetter, Joel; Sarnosky, Karen; Swafford, Cathy; Speer, Michael; Barton, Wendy J; Mink, J W; Lemm, Dianne; Hudak, Mark; Case, Elizabeth; Rowen, Judith; Fuentes, Sandra; Pane, Carly; Richardson, Leslie; Chavarria, Cesar; Cassino, Deanne; Ghaffari, Kourosh; Carroll, Carol; Lee, Haesoon; Guclu, Lydia; Johnson, Christopher; Blum, Valerie; Boron, Marnie L; Sorrentino, Mark; Hirsch, Robert L; Van Veldhuisen, Paul C; Smith, Carol

    2003-06-01

    The objective of the Registry was to characterize the population of infants receiving prophylaxis for respiratory syncytial virus (RSV) disease by describing the patterns and scope of usage of palivizumab in a cross section of US infants. RSV hospitalization outcomes were also described. The Palivizumab (Synagis, MedImmune, Inc., 25 West Watkins Mill Road, Gaithersburg, MD 20878) Outcomes Registry was a prospective multicenter survey conducted at 63 sites. Demographics, injection history, and RSV hospitalization outcomes were collected on 2,116 infants receiving palivizumab. Infants were enrolled in the Registry between September 1, 2000-March 1, 2001, at the time of their first injection. Infants born at less than 32 weeks of gestation accounted for 47% of infants enrolled, and those between 32-35 weeks accounted for 45%; approximately 8% were greater than 35 weeks of gestation. Lower RSV hospitalization rates were observed in infants who had greater adherence to regularly scheduled injections. Nearly one-half of all hospitalizations occurred within the first and second injection intervals, suggesting the importance of early RSV protection. The confirmed RSV hospitalization rate of all infants in the Registry was 2.9%; the rate was 5.8% in infants with chronic lung disease of infancy, and 2.1% in premature infants without chronic lung disease. In conclusion, these data support the continued effectiveness of palivizumab prophylaxis for severe RSV lower respiratory tract disease in a large cohort of high-risk infants from geographically diverse pediatric offices and clinics. The Palivizumab Outcomes Registry provides an opportunity to assess palivizumab utilization and clinical effectiveness in the US.

  3. Are all metal-on-metal hip revision operations contributing to the National Joint Registry implant survival curves?

    PubMed Central

    Sabah, S. A.; Henckel, J.; Koutsouris, S.; Rajani, R.; Hothi, H.; Skinner, J. A.; Hart, A. J.

    2016-01-01

    Aims The National Joint Registry for England, Wales and Northern Ireland (NJR) has extended its scope to report on hospital, surgeon and implant performance. Data linkage of the NJR to the London Implant Retrieval Centre (LIRC) has previously evaluated data quality for hip primary procedures, but did not assess revision records. Methods We analysed metal-on-metal hip revision procedures performed between 2003 and 2013. A total of 69 929 revision procedures from the NJR and 929 revised pairs of components from the LIRC were included. Results We were able to link 716 (77.1%) revision procedures on the NJR to the LIRC. This meant that 213 (22.9%) revision procedures at the LIRC could not be identified on the NJR. We found that 349 (37.6%) explants at the LIRC completed the full linkage process to both NJR primary and revision databases. Data completion was excellent (> 99.9%) for revision procedures reported to the NJR. Discussion This study has shown that only approximately one third of retrieved components at the LIRC, contributed to survival curves on the NJR. We recommend prospective registry-retrieval linkage as a tool to feedback missing and erroneous data to the NJR and improve data quality. Take home message: Prospective Registry – retrieval linkage is a simple tool to evaluate and improve data quality on the NJR. Cite this article: Bone Joint J 2016;98-B:33–9. PMID:26733513

  4. Dietary habits and weight maintenance success in high versus low exercisers in the National Weight Control Registry

    PubMed Central

    Ogden, Lorraine G.; Phelan, Suzanne; Thomas, J. Graham; Hill, James O; Wing, Rena R.; Wyatt, Holly R.

    2015-01-01

    Background The National Weight Control Registry (NWCR) was established to examine characteristics of successful weight loss maintainers. This study compares the diet and behavioral characteristics and weight regain trajectories of NWCR members with differing physical activity (PA) levels at baseline. Methods Participants (n=3591) were divided into 4 levels of self-reported PA at registry entry (<1000, 1000 to <2250, 2250 to <3500, and ≥3500 kcals/week). We compared self-reported energy intake (EI), macronutrient composition, eating behaviors (dietary restraint, hunger, and disinhibition), weight loss maintenance strategies, and 3 year weight regain between these 4 activity groups. Results Those with the highest PA at registry entry had lost the most weight, and reported lower fat intake, more dietary restraint, and greater reliance on several specific dietary strategies to maintain weight loss. Those in the lowest PA category maintained weight loss despite low levels of PA and without greater reliance on dietary strategies. There were no differences in odds of weight regain at year 3 between PA groups. Conclusions These findings suggest that there is not a “one size fits all strategy” for successful weight loss maintenance and that weight loss maintenance may require the use of more strategies by some individuals than others. PMID:24385447

  5. 77 FR 7167 - Global Rare Diseases Patient Registry and Data Repository (GRDR) Notice and Request for...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-10

    ... organizational component of the National Center for Advancing Translational Sciences (NCATS), National Institutes... through the patient organizations. Direct contact with the prospective participants would occur only after... community, ORDR believes that an open-science community for rare diseases is needed. Such a community...

  6. The Danish Heart Registry

    PubMed Central

    Özcan, Cengiz; Juel, Knud; Flensted Lassen, Jens; von Kappelgaard, Lene Mia; Mortensen, Poul Erik; Gislason, Gunnar

    2016-01-01

    Aim The Danish Heart Registry (DHR) seeks to monitor nationwide activity and quality of invasive diagnostic and treatment strategies in patients with ischemic heart disease as well as valvular heart disease and to provide data for research. Study population All adult (≥15 years) patients undergoing coronary angiography (CAG), percutaneous coronary intervention (PCI), coronary artery bypass grafting, and heart valve surgery performed across all Danish hospitals were included. Main variables The DHR contains a subset of the data stored in the Eastern and Western Denmark Heart Registries (EDHR and WDHR). For each type of procedure, up to 70 variables are registered in the DHR. Since 2010, the data quality protocol encompasses fulfillment of web-based validation rules of daily-submitted records and yearly approval of the data by the EDHR and WDHR. Descriptive data The data collection on procedure has been complete for PCI and surgery since 2000, and for CAG as of 2006. From 2000 to 2014, the number of CAG, PCI, and surgical procedures changed by 231%, 193%, and 99%, respectively. Until the end of 2014, a total of 357,476 CAG, 131,309 PCI, and 60,831 surgical procedures had been performed, corresponding to 249,445, 100,609, and 55,539 first-time patients, respectively. The DHR generally has a high level of completeness (1–missing) of each procedure (>90%) when compared to the National Patient Registry. Variables important for assessing the quality of care have a high level of completeness for surgery since 2000, and for CAG and PCI since 2010. Conclusion The DHR contains valuable data on cardiac invasive procedures, which makes it an important national monitoring and quality system and at the same time serves as a platform for research projects in the cardiovascular field. PMID:27822091

  7. The Measurement to Understand Reclassification of Disease of Cabarrus/Kannapolis (MURDOCK) Study Community Registry and Biorepository

    PubMed Central

    Bhattacharya, Sayanti; Dunham, Ashley A; Cornish, Melissa A; Christian, Victoria A; Ginsburg, Geoffrey S; Tenenbaum, Jessica D; Nahm, Meredith L; Miranda, Marie Lynn; Califf, Robert M; Dolor, Rowena J; Newby, L Kristin

    2012-01-01

    Current understanding of chronic diseases is based on crude clinical characterization, imaging studies, and laboratory testing that has evolved over decades. The Measurement to Understand Reclassification of Disease of Cabarrus/Kannapolis (MURDOCK) Study is a multi-tiered, longitudinal study designed to enable classification of chronic diseases using clinically annotated biospecimen collections, -omic technologies, electronic health records, and standard epidemiological methods. We expect that detailed molecular classification will improve mechanistic understanding of chronic diseases, augmenting discovery and testing of new treatments, and allowing refined selection of prevention and treatment strategies. The MURDOCK Study Community Registry and Biorepository will serve as a bridge for validation of initial exploratory studies, a platform for future prospective studies in targeted populations, and a resource of both data (analytical and clinical) and samples for cross-registry meta-analyses and comparative population studies. Participation of local health care providers and the Cabarrus County/Kannapolis, NC, community will facilitate future medical research and provide the opportunity to educate and inform the public about genomic research, actively engaging them in shaping the future of medical discovery and treatment of chronic diseases. We present the rationale and study design for the MURDOCK Community Registry and Biorepository and baseline characteristics of the first 6000 participants. PMID:23145214

  8. 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

  9. Lessons from 30 years' data of Korean end-stage renal disease registry, 1985-2015.

    PubMed

    Jin, Dong-Chan; Yun, Sung Ro; Lee, Seoung Woo; Han, Sang Woong; Kim, Won; Park, Jongha; Kim, Yong Kyun

    2015-09-01

    The Korean Society of Nephrology (KSN) launched a nationwide official survey program about dialysis therapy in 1985. Nowadays, the accumulated data for 30 years by this "Insan Prof. Min Memorial end-stage renal disease (ESRD) Registry" program have been providing the essential information for dialysis clinical practice, academic nephrology research, and health management policy. We reviewed 30 years of data to identify important changes and implications for the future improvement of dialysis therapy in Korea. Hemodialysis patients, especially diabetics and elderly patients have increased in number very rapidly during recent years in Korea. The Korean prevalence rate of ESRD patients was about 70% of the United States and about 50% of Japan according to the international comparisons in the annual data report of United States Renal Data System. The blood pressure control, anemia control, and dialysis adequacy have continuously improved year by year. The importance of calcium and phosphorus control has also been increasing because of the increase in long-term dialysis patients. In addition, chronic dialysis complications should be closely monitored and dialysis modifications, such as hemodiafiltration therapy, might be considered. Because of the increase of private clinics and nursing hospitals in dialysis practice, the role of dialysis specialists and continuing education are thought to be essential. For strict cost-effective dialysis control of increasing elderly, diabetic, and long-term dialysis patients, the KSN ESRD patient registration should be run by the KSN and health ministry in cooperation, in which the dialysis fee reimbursement should be accompanied.

  10. The Barth Syndrome Registry: distinguishing disease characteristics and growth data from a longitudinal study.

    PubMed

    Roberts, Amy E; Nixon, Connie; Steward, Colin G; Gauvreau, Kimberly; Maisenbacher, Melissa; Fletcher, Matthew; Geva, Judith; Byrne, Barry J; Spencer, Carolyn T

    2012-11-01

    Barth syndrome (BTHS); MIM accession # 302060) is a rare X-linked recessive cardioskeletal mitochondrial myopathy with features of cardiomyopathy, neutropenia, and growth abnormalities. The objectives of this study were to further elucidate the natural history, clinical disease presentation, and course, and describe growth characteristics for males with BTHS. Patients with a confirmed genetic diagnosis of BTHS are referred to the BTHS Registry through the Barth Syndrome Foundation, self-referral, or physician referral. This study is based on data obtained from 73 subjects alive at the time of enrollment that provided self-reported and/or medical record abstracted data. The mean age at diagnosis of BTHS was 4.04 ± 5.45 years. While the vast majority of subjects reported a history of cardiac dysfunction, nearly 6% denied any history of cardiomyopathy. Although most subjects had only mildly abnormal cardiac function by echocardiography reports, 70% were recognized as having cardiomyopathy in the first year of life and 12% have required cardiac transplantation. Of the 73 enrolled subjects, there have been five deaths. Growth curves were generated demonstrating a shift down for weight, length, and height versus the normative population with late catch up in height for a significant percentage of cases. This data also confirms a significant number of patients with low birth weight, complications in the newborn period, failure to thrive, neutropenia, developmental delay of motor milestones, and mild learning difficulties. However, it is apparent that the disease manifestations are variable, both over time for an individual patient and across the BTHS population.

  11. Long-Term Clinical Outcomes According to Previous Manifestations of Atherosclerotic Disease (from the FAST-MI 2010 Registry).

    PubMed

    Puymirat, Etienne; Aissaoui, Nadia; Lemesle, Gilles; Cottin, Yves; Coste, Pierre; Schiele, François; Ferrières, Jean; Simon, Tabassome; Danchin, Nicolas

    2017-03-01

    The prognosis of patients with acute myocardial infarction (AMI) has notably improved in the past 20 years. Using the French Registry of ST-Elevation and Non-ST-elevation Myocardial Infarction (FAST-MI) 2010 registry, we investigated whether previous manifestations of atherosclerotic disease (i.e., previous MI, or a history of any form of atherosclerotic disease) are at truly increased risk compared with those in whom AMI is the first manifestation of the disease. FAST-MI 2010 is a nationwide French registry including 3,079 patients with AMI, among whom 1,062 patients had a history of cardiovascular atherosclerotic disease and 498 patients had a history of MI. Overall, patients with a history of atherosclerotic disease (or MI) were older compared with patients without known cardiovascular disease (71 ± 13 vs 63 ± 14 years) and had higher cardiovascular risk profiles and co-morbidities. Using fully adjusted Cox multivariate analysis, previous manifestations of atherosclerotic disease were associated with higher 3-year mortality (hazard ratio 1.80, 95% confidence interval 1.40 to 2.31; p <0.001) as history of previous MI alone (hazard ratio 1.32, 95% confidence interval 1.00 to 1.73; p = 0.048). Similar results were found in patients discharged alive. In conclusion, previous cardiovascular atherosclerotic disease represents 1/3 of patients with AMI and are strongly associated with worse long-term clinical outcomes. Intensive follow-up and therapy should be encouraged in this high-risk population.

  12. Effects of Renal Denervation Documented in the Austrian National Multicentre Renal Denervation Registry

    PubMed Central

    Lambert, Thomas; Steinwender, Clemens; Weber, Thomas; Suppan, Markus; Brussee, Helmut; Koppelstätter, Christian; Kerschbaum, Julia; Watschinger, Bruno; Hohenstein-Scheibenecker, Katharina; Reindl-Schwaighofer, Roman; Sturmberger, Thomas; Kindslehner, Claudia; Weiss, Thomas Werner; Rohla, Miklos; Gruener, Peter; Maister, Petra; Auer, Johann; Dechant, Cornelia; Sykora, Josef; Krismer, Christoph; Glaser, Stefan; Zweiker, Robert

    2016-01-01

    Renal denervation (RDN) is a new procedure for treatment-resistant hypertensive patients. In order to monitor all procedures undergone in Austria, the Austrian Society of Hypertension established the investigator-initiated Austrian Transcatheter Renal Denervation (TREND) Registry. From April 2011 to September 2014, 407 procedures in 14 Austrian centres were recorded. At baseline, office and mean 24-h ambulatory blood pressure (ABP) were 171/94 and 151/89 mmHg, respectively, and patients were taking a median of 4 antihypertensive medications. Mean 24-h ABP changes after 2–6 weeks, 3, 6 and 12 months were -11/-6, -8/-4, -8/-5 and -10/-6 mmHg (p<0.05 at all measurements), respectively. The periprocedural complication rate was 2.5%. Incidence of long-term complications during follow-up (median 1 year) was 0.5%. Office BP and ABP responses showed only a weak correlation (Pearson coefficient 0.303). Based on the data from the TREND registry, ambulatory blood pressure monitoring in addition to office BP should be used for patient selection as well as for monitoring response to RDN. Furthermore, criteria for optimal patient selection are suggested. PMID:27529426

  13. JBEI Registry

    SciTech Connect

    Ham, Timothy

    2008-12-01

    The JBEI Registry is a software to store and manage to a database of biological parts. It is intended to be used as a web service that is accessed via a web browser. It is also capable of running as a desktop program for a single user. The registry software stores, indexes, categories, and allows users to enter, search, retrieve, and contruct biological constructs in silico. It is also able to communicate with other Registries for data sharing and exchange.

  14. [Role of cancer registries].

    PubMed

    Schaffer, P

    1995-05-01

    The first Cancer Registries were created in 1975 in France. Their ulterior development and their scientific production have been furthered by the apparition from 1986 under the aegis of the Health Ministry and of the INSERM, of a National Population Registry Committee. Cancer Registries have seriously contributed to a better knowledge of the cancer problem in our country and to describe the french specificities, in particular the importance of the mouth and pharynx cancers. They insure both a monitoring and an alert role; they also contribute to the medical supervision of the Chernobyl accident effects. French registries play a very active role concerning clinical research. They participate to many European studies of health care evaluation. In other respects, many etiological studies have been realized about professional risks of cancer, risks linked with nutritional habits, and on the etiologic role of the Tamoxifen. Finally, certain registries have created DNA banks. If nowadays their role in health planning remains modest, they very actively contribute in evaluating screening actions of breast, cervix and large bowel cancers. They also attracted the attention of Health Authorities on the cervix cancer screening's incoherencies. They evaluate the pilot project of the breast cancer and the registry of the Côte d'Or country evaluates the efficacity of a randomized colo rectal mass screening study. The main difficulties met by the registries are linked with the development of laws protecting more and more the individual freedoms, making it harder and harder the registration exhaustive character.

  15. Update to agency for toxic substances and disease registry 2012 report on assessment of biota exposure to mercury originating from Savannah River Site.

    SciTech Connect

    Kuhne, W.

    2015-08-10

    The purpose of this report is to 1) update previous Savannah River National Laboratory (SRNL) assessment reports (Kvartek et al. 1994 and Halverson et al. 2008) on the fate of mercury in the Savannah River Site (SRS) environment and 2) address comments and recommendations from the review of SRS by the Agency for Toxic Substances and Disease Registry (ATSDR) concerning the evaluation of exposures to contaminants in biota originating from the SRS. The ATSDR reviewed and evaluated data from SRS, South Carolina Department of Health & Environmental Control (SCDHEC) and the Georgia Department of Natural Resources (GDNR) concerning the non-radioactive contaminant mercury. This report will provide a response and update to conclusions and recommendations made by the ATSDR.

  16. Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at Sanford

    ClinicalTrials.gov

    2016-09-01

    Rare Disorders; Undiagnosed Disorders; Disorders of Unknown Prevalence; Cornelia De Lange Syndrome; Prenatal Benign Hypophosphatasia; Perinatal Lethal Hypophosphatasia; Odontohypophosphatasia; Adult Hypophosphatasia; Childhood-onset Hypophosphatasia; Infantile Hypophosphatasia; Hypophosphatasia; Kabuki Syndrome; Bohring-Opitz Syndrome; Narcolepsy Without Cataplexy; Narcolepsy-cataplexy; Hypersomnolence Disorder; Idiopathic Hypersomnia Without Long Sleep Time; Idiopathic Hypersomnia With Long Sleep Time; Idiopathic Hypersomnia; Kleine-Levin Syndrome; Kawasaki Disease; Leiomyosarcoma; Leiomyosarcoma of the Corpus Uteri; Leiomyosarcoma of the Cervix Uteri; Leiomyosarcoma of Small Intestine; Acquired Myasthenia Gravis; Addison Disease; Hyperacusis (Hyperacousis); Juvenile Myasthenia Gravis; Transient Neonatal Myasthenia Gravis; Williams Syndrome; Lyme Disease; Myasthenia Gravis; Marinesco Sjogren Syndrome(Marinesco-Sjogren Syndrome); Isolated Klippel-Feil Syndrome; Frasier Syndrome; Denys-Drash Syndrome; Beckwith-Wiedemann Syndrome; Emanuel Syndrome; Isolated Aniridia; Beckwith-Wiedemann Syndrome Due to Paternal Uniparental Disomy of Chromosome 11; Beckwith-Wiedemann Syndrome Due to Imprinting Defect of 11p15; Beckwith-Wiedemann Syndrome Due to 11p15 Translocation/Inversion; Beckwith-Wiedemann Syndrome Due to 11p15 Microduplication; Beckwith-Wiedemann Syndrome Due to 11p15 Microdeletion; Axenfeld-Rieger Syndrome; Aniridia-intellectual Disability Syndrome; Aniridia - Renal Agenesis - Psychomotor Retardation; Aniridia - Ptosis - Intellectual Disability - Familial Obesity; Aniridia - Cerebellar Ataxia - Intellectual Disability; Aniridia - Absent Patella; Aniridia; Peters Anomaly - Cataract; Peters Anomaly; Potocki-Shaffer Syndrome; Silver-Russell Syndrome Due to Maternal Uniparental Disomy of Chromosome 11; Silver-Russell Syndrome Due to Imprinting Defect of 11p15; Silver-Russell Syndrome Due to 11p15 Microduplication; Syndromic Aniridia; WAGR Syndrome; Wolf

  17. 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.

  18. A "pivot" Model to set up Large Scale Rare Diseases Information Systems: Application to the Fibromuscular Dysplasia Registry.

    PubMed

    Toubiana, Laurent; Ugon, Adrien; Giavarini, Alessandra; Riquier, Jérémie; Charlet, Jean; Jeunemaitre, Xavier; Plouin, Pierre-François; Jaulent, Marie-Christine

    2015-01-01

    The SIR-FMD project is a partnership between the Department of Genetics and Reference Centre for Rare Vascular Diseases at the Georges Pompidou European Hospital in Paris and the Medical Informatics and Knowledge Engineering Laboratory of Inserm. Its aim is to use an ontological approach to implement an information system for the French Fibromuscular Dysplasia Registry. The existing data was dispersed in numerous databases, which had been created independently. These databases have different structures and contain data of diverse quality. The project aims to provide generic solutions for the management of the communication of medical data. The secondary objective is to demonstrate the applicability of these generic solutions in the field of rare diseases (RD) in an operational context. The construction of the French FMD registry was a multistep process. A secure platform has been available since the beginning of November 2013. The medical records of 471 patients from the initial dataset provided by the HEGP-Paris, France have been included, and are accessible from a secure user account. Users are organized into a collaborative group, and can access patient groups. Each electronic patient record contains more than 2,200 items. The problem of semantic interoperability has become one of the major challenges for the development of applications requiring the sharing and reuse of data. The information system component of the SIR-FMD project has a direct impact on the standardisation of coding of rare diseases and thereby contributes to the development of e-Health.

  19. [National Cancer Registry. Significance of a reliable database in the implementation of the required structural changes of cancer care in Hungary].

    PubMed

    Kásler, Miklós; Ottó, Szabolcs; Sólyom, Olimpia

    2014-09-07

    The authors summarize the basic objectives and scope of the Hungarian Cancer Registry. They review more than 100-year history of the national cancer database and its effects on current cancer data collection activities, which is outstanding in Europe. The compilation deals with the development of information technology, covers points of principle and practical issues such as parallel display and evaluation of mortality and morbidity statistics and their national and international importance concerning public health. The authors underline that reliable data collection and services of the National Cancer Registry are important for the society because they are public health issues with a critical importance for a better understanding of risk factors, prevention and patient care. Restructuring and European harmonization of the Hungarian cancer system are inevitable using a reliable information exchange and service, taking into account national specificities and international requirements.

  20. Incidence of Congenital Heart Disease: The 9-Year Experience of the Guangdong Registry of Congenital Heart Disease, China

    PubMed Central

    Zhuang, Jian; Chen, Guanchun; Mai, Jinzhuang; Guo, Xiaoling; Ou, Yanqiu; Chen, Jimei; Gong, Wei; Gao, Xiangmin; Wu, Yong; Nie, Zhiqiang

    2016-01-01

    There are 16.5 million newborns in China annually. However, the incidence of congenital heart disease (CHD) has not been evaluated. In 2004, we launched an active province-wide hospital-based CHD registry in the Guangdong Province of southern China. In this study, we examined the incidence of CHD and its subtypes from 2004 to 2012 and compared our findings to the literature. Our results indicate there is an increasing trend of CHD incidence. The increase in incidence occurred mainly for single lesion and the most common subtypes (e.g., ventricular or atrial septal defect, patent ductus arteriosus). There were no increases found for multiple lesions or more complex subtypes. The proportion of CHD cases that were detected early (e.g., 1 week) increased over time. The incidence of CHD stabilized in 2010–2012 with the average cumulative incidences of 9.7, 9.9, and 11.1 per 1,000 live births at 1 week, 1 month, and 1 year, respectively. The incidences of CHD subtypes were comparable with recent international results. The data did not support previous reports that Asian children have a higher incidence of pulmonary outflow obstructions and lower incidence of transposition of the great arteries. However, there was a lower incidence of left ventricular outflow tract obstructions observed in our series. The increase in CHD incidence observed over time was due to improved detection and diagnosis. The true incidence of CHD in China was approximately 11.1 per 1,000 live births, which is higher than previously reported. PMID:27409588

  1. Breast and Colon Cancer Family Registries

    Cancer.gov

    The Breast Cancer Family Registry and the Colon Cancer Family Registry were established by the National Cancer Institute as a resource for investigators to use in conducting studies on the genetics and molecular epidemiology of breast and colon cancer.

  2. Safety and efficacy of endoscopic spray cryotherapy for Barrett's dysplasia: results of the National Cryospray Registry.

    PubMed

    Ghorbani, S; Tsai, F C; Greenwald, B D; Jang, S; Dumot, J A; McKinley, M J; Shaheen, N J; Habr, F; Coyle, W J

    2016-04-01

    Retrospective series have shown the efficacy of endoscopic spray cryotherapy in eradicating high-grade dysplasia (HGD) in Barrett's esophagus (BE); however, prospective data are lacking, and efficacy for low-grade dysplasia (LGD) is unclear. The aim of this study was to assess the efficacy and safety of spray cryotherapy in patients with LGD or HGD. A multicenter, prospective open-label registry enrolled patients with dysplastic BE. Spray cryotherapy was performed every 2-3 months until there was no endoscopic evidence of BE and no histological evidence of dysplasia, followed by surveillance endoscopies up to 2 years. Primary outcome measures were complete eradication of dysplasia (CE-D) and complete eradication of all intestinal metaplasia (CE-IM). Ninety-six subjects with Barrett's dysplasia (67% HGD; 65% long-segment BE; mean length 4.5 cm) underwent 321 treatments (mean 3.3 per subject). Mean age was 67 years, 83% were male. Eighty patients (83%) completed treatment with follow-up endoscopy (mean duration 21 months). In patients with LGD, rate of CE-D was 91% (21/23) and rate of CE-IM was 61% (14/23). In HGD, CE-D rate was 81% (46/57) and CE-IM was 65% (37/57). In patients with short-segment BE (SSBE) with any dysplasia, CE-D was achieved in 97% (30/31) and CE-IM in 77% (24/31). There were no esophageal perforations or related deaths. One subject developed a stricture, which did not require dilation. One patient was hospitalized for bleeding in the setting of non-steroidal anti-inflammatory drug use. In the largest prospective cohort to date, data suggest endoscopic spray cryotherapy is a safe and effective modality for eradication of BE with LGD or HGD, particularly with SSBE.

  3. Spatial analysis of myocardial infarction in Iran: National report from the Iranian myocardial infarction registry

    PubMed Central

    Ahmadi, Ali; Soori, Hamid; Mehrabi, Yadollah; Etemad, Koorosh

    2015-01-01

    Background: Myocardial infarction (MI) is a leading cause of mortality and morbidity in Iran. No spatial analysis of MI has been conducted to date. The present study was conducted to determine the pattern of MI incidence and to identify the associated factors in Iran by province. Materials and Methods: This study has two parts. One part is prospective and hospital-based, and the other part is an ecological study. In this study, the data of 20,750 new MI cases registered in Iranian Myocardial Infarction Registry in 2012 were used. For spatial analysis in global and local, spatial autocorrelation, Moran's I, Getis-Ord, and logistic regression models were used. Data were analyzed by Stata software and ArcGIS 9.3. Results: Based on autocorrelation coefficient, a specific pattern was observed in the distribution of MI incidence in different provinces (Moran's I: 0.75, P < 0.001). Spatial pattern of incidence was approximately the same in men and women. MI incidence was clustering in six provinces (North Khorasan, Yazd, Kerman, Semnan, Golestan, and Mazandaran). Out of the associated factors with clustered MI in six provinces, temperature, humidity, hypertension, smoking, and body mass index (BMI) could be mentioned. Hypertension, smoking, and BMI contributed to clustering with, respectively, 2.36, 1.31, and 1.31 odds ratio. Conclusion: Addressing the place-based pattern of incidence and clarifying their epidemiologic dimension, including spatial analysis, has not yet been implemented in Iran. Report on MI incidence rate by place and formal borders is useful and is used in the planning and prioritization in different levels of health system. PMID:26487871

  4. 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

  5. Chronobiology of Acute Aortic Dissection in the Marfan Syndrome (from the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions and the International Registry of Acute Aortic Dissection).

    PubMed

    Siddiqi, Hasan K; Luminais, Steven N; Montgomery, Dan; Bossone, Eduardo; Dietz, Harry; Evangelista, Arturo; Isselbacher, Eric; LeMaire, Scott; Manfredini, Roberto; Milewicz, Dianna; Nienaber, Christoph A; Roman, Mary; Sechtem, Udo; Silberbach, Michael; Eagle, Kim A; Pyeritz, Reed E

    2017-03-01

    Marfan syndrome (MFS) is an autosomal dominant connective tissue disease associated with acute aortic dissection (AAD). We used 2 large registries that include patients with MFS to investigate possible trends in the chronobiology of AAD in MFS. We queried the International Registry of Acute Aortic Dissection (IRAD) and the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) registry to extract data on all patients with MFS who had suffered an AAD. The group included 257 patients with MFS who suffered an AAD from 1980 to 2012. The chi-square tests were used for statistical testing. Mean subject age at time of AAD was 38 years, and 61% of subjects were men. AAD was more likely in the winter/spring season (November to April) than the other half of the year (57% vs 43%, p = 0.05). Dissections were significantly more likely to occur during the daytime hours, with 65% of dissections occurring from 6 a.m. to 6 p.m. (p = 0.001). Men were more likely to dissect during the daytime hours (6 a.m. to 6 p.m.) than women (74% vs 51%, p = 0.01). These insights offer a glimpse of the times of greatest vulnerability for patients with MFS who suffer from this catastrophic event. In conclusion, the chronobiology of AAD in MFS reflects that of AAD in the general population.

  6. The UK transcatheter aortic valve implantation registry; one of the suite of registries hosted by the National Institute for Cardiovascular Outcomes Research (NICOR).

    PubMed

    Ludman, Peter F

    2012-12-01

    This registry was set up to create a comprehensive record of all TAVI procedures performed in the United Kingdom since the introduction of the technique in 2007, to help drive quality improvement and provide data for research. Funding is independent of industry. All hospitals in the UK provide data for every consecutive patient where TAVI was attempted. The dataset includes variables defining patient demographic features, indications, procedural details and outcomes up to the time of hospital discharge. There are variables for follow up at 1 and 3 years assessing symptoms and life status. An updated dataset will be collected from 2013. Mortality is tracked centrally. Data entry is performed by clinical staff and data clerks. No external validation. The data are available for research by application to the UK TAVI Steering Group using a data sharing agreement which can be obtained at NICOR (www.ucl.ac.uk/nicor/).

  7. The Danish Stroke Registry

    PubMed Central

    Johnsen, Søren Paaske; Ingeman, Annette; Hundborg, Heidi Holmager; Schaarup, Susanne Zielke; Gyllenborg, Jesper

    2016-01-01

    Aim of database The aim of the Danish Stroke Registry is to monitor and improve the quality of care among all patients with acute stroke and transient ischemic attack (TIA) treated at Danish hospitals. Study population All patients with acute stroke (from 2003) or TIA (from 2013) treated at Danish hospitals. Reporting is mandatory by law for all hospital departments treating these patients. The registry included >130,000 events by the end of 2014, including 10,822 strokes and 4,227 TIAs registered in 2014. Main variables The registry holds prospectively collected data on key processes of care, mainly covering the early phase after stroke, including data on time of delivery of the processes and the eligibility of the individual patients for each process. The data are used for assessing 18 process indicators reflecting recommendations in the national clinical guidelines for patients with acute stroke and TIA. Patient outcomes are currently monitored using 30-day mortality, unplanned readmission, and for patients receiving revascularization therapy, also functional level at 3 months poststroke. Descriptive data Sociodemographic, clinical, and lifestyle factors with potential prognostic impact are registered. Conclusion The Danish Stroke Registry is a well-established clinical registry which plays a key role for monitoring and improving stroke and TIA care in Denmark. In addition, the registry is increasingly used for research. PMID:27843349

  8. Use of Anticoagulants and Antiplatelet Agents in Stable Outpatients with Coronary Artery Disease and Atrial Fibrillation. International CLARIFY Registry

    PubMed Central

    Fauchier, Laurent; Greenlaw, Nicola; Ferrari, Roberto; Ford, Ian; Fox, Kim M.; Tardif, Jean-Claude; Tendera, Michal; Steg, Ph. Gabriel

    2015-01-01

    Background Few data are available regarding the use of antithrombotic strategies in coronary artery disease patients with atrial fibrillation (AF) in everyday practice. We sought to describe the prevalence of AF and its antithrombotic management in a contemporary population of patients with stable coronary artery disease. Methods and Findings CLARIFY is an international, prospective, longitudinal registry of outpatients with stable coronary artery disease, defined as prior (≥12 months) myocardial infarction, revascularization procedure, coronary stenosis >50%, or chest pain associated with evidence of myocardial ischemia. Overall, 33,428 patients were screened, of whom 32,954 had data available for analysis at baseline; of these 2,229 (6.7%) had a history of AF. Median (interquartile range) CHA2DS2-VASc score was 4 (3, 5). Oral anticoagulation alone was used in 25.7%, antiplatelet therapy alone in 52.8% (single 41.8%, dual 11.0%), and both in 21.5%. OAC use was independently associated with permanent AF (p<0.001), CHA2DS2-VASc score (p=0.006), pacemaker (p<0.001), stroke (p=0.04), absence of angina (p=0.004), decreased left ventricular ejection fraction (p<0.001), increased waist circumference (p=0.005), and longer history of coronary artery disease (p=0.008). History of percutaneous coronary intervention (p=0.004) and no/partial reimbursement for cardiovascular medication (p=0.01, p<0.001, respectively) were associated with reduced oral anticoagulant use. Conclusions In this contemporary cohort of patients with stable coronary artery disease and AF, most of whom are theoretical candidates for anticoagulation, oral anticoagulants were used in only 47.2%. Half of the patients received antiplatelet therapy alone and one-fifth received both antiplatelets and oral anticoagulants. Efforts are needed to improve adherence to guidelines in these patients. Trial Registration ISRCTN registry of clinical trials: ISRCTN43070564. PMID:25915904

  9. The potential and limitations of data from population-based state cancer registries.

    PubMed

    Izquierdo, J N; Schoenbach, V J

    2000-05-01

    Cancer incidence varies markedly among states because of population heterogeneity regarding risk, genetic, and demographic factors. Population-based cancer registries are essential to monitoring cancer trends and control. The Centers for Disease Control and Prevention and the North American Association of Central Cancer Registries, through the National Program of Cancer Registries, are helping state registries generate more and better data nationwide. The National Program of Cancer Registries has supported the enhancement of 36 registries and the creation of 13 new registries in 45 states, 3 territories, and the District of Columbia, providing national standards for completeness, timeliness, and quality; financial support; and technical assistance. Users must be aware of diverse issues that influence collection and interpretation of cancer registry data, such as multiple cancer diagnoses, duplicate reports, reporting delays, misclassification of race/ethnicity, and pitfalls in estimations of cancer incidence rates. Attention to these issues and intense use of the available data for cancer surveillance will enable maximum societal benefit from the emerging network of population-based state cancer registries.

  10. Validation of primary metal-on-metal hip arthroplasties on the National Joint Registry for England, Wales and Northern Ireland using data from the London Implant Retrieval Centre

    PubMed Central

    Sabah, S. A.; Henckel, J.; Cook, E.; Whittaker, R.; Hothi, H.; Pappas, Y.; Blunn, G.; Skinner, J. A.; Hart, A. J.

    2015-01-01

    Arthroplasty registries are important for the surveillance of joint replacements and the evaluation of outcome. Independent validation of registry data ensures high quality. The ability for orthopaedic implant retrieval centres to validate registry data is not known. We analysed data from the National Joint Registry for England, Wales and Northern Ireland (NJR) for primary metal-on-metal hip arthroplasties performed between 2003 and 2013. Records were linked to the London Implant Retrieval Centre (RC) for validation. A total of 67 045 procedures on the NJR and 782 revised pairs of components from the RC were included. We were able to link 476 procedures (60.9%) recorded with the RC to the NJR successfully. However, 306 procedures (39.1%) could not be linked. The outcome recorded by the NJR (as either revised, unrevised or death) for a primary procedure was incorrect in 79 linked cases (16.6%). The rate of registry-retrieval linkage and correct assignment of outcome code improved over time. The rates of error for component reference numbers on the NJR were as follows: femoral head category number 14/229 (5.0%); femoral head batch number 13/232 (5.3%); acetabular component category number 2/293 (0.7%) and acetabular component batch number 24/347 (6.5%). Registry-retrieval linkage provided a novel means for the validation of data, particularly for component fields. This study suggests that NJR reports may underestimate rates of revision for many types of metal-on-metal hip replacement. This is topical given the increasing scope for NJR data. We recommend a system for continuous independent evaluation of the quality and validity of NJR data. Cite this article: Bone Joint J 2015;97-B:10–18. PMID:25568407

  11. The Value and Limitations of Guidelines, Expert Consensus, and Registries on the Management of Patients with Thoracic Aortic Disease

    PubMed Central

    Pacini, Davide; Murana, Giacomo; Leone, Alessandro; Di Marco, Luca; Pantaleo, Antonio

    2016-01-01

    Doctors are often faced with difficult decisions and uncertainty when patients need a certain treatment. They routinely rely on the scientific literature, in addition to their knowledge, experience, and patient preferences. Clinical practice guidelines are created with the intention of facilitating decision-making. They may offer concise instructions for the diagnosis, management (medical or surgical treatments), and prevention of specific diseases or conditions. All information included in the final version are the result of a systematic review of scientific articles and an assessment of the benefits and costs of alternative care options. The final document attempts to meet the needs of most patients in most circumstances and clinicians, aware of these recommendations, should always make individualized treatment decisions. In this review, we attempted to define the intent and applicability of clinical practice guidelines, expert consensus documents, and registry studies, focusing on the management of patients with thoracic aortic disease. PMID:27965917

  12. The Value and Limitations of Guidelines, Expert Consensus, and Registries on the Management of Patients with Thoracic Aortic Disease.

    PubMed

    Pacini, Davide; Murana, Giacomo; Leone, Alessandro; Di Marco, Luca; Pantaleo, Antonio

    2016-12-01

    Doctors are often faced with difficult decisions and uncertainty when patients need a certain treatment. They routinely rely on the scientific literature, in addition to their knowledge, experience, and patient preferences. Clinical practice guidelines are created with the intention of facilitating decision-making. They may offer concise instructions for the diagnosis, management (medical or surgical treatments), and prevention of specific diseases or conditions. All information included in the final version are the result of a systematic review of scientific articles and an assessment of the benefits and costs of alternative care options. The final document attempts to meet the needs of most patients in most circumstances and clinicians, aware of these recommendations, should always make individualized treatment decisions. In this review, we attempted to define the intent and applicability of clinical practice guidelines, expert consensus documents, and registry studies, focusing on the management of patients with thoracic aortic disease.

  13. South African National Cancer Registry: Effect of withheld data from private health systems on cancer incidence estimates

    PubMed Central

    Singh, E; Underwood, J M; Nattey, C; Babb, C; Sengayi, M; Kellett, P

    2015-01-01

    Background The National Cancer Registry (NCR) was established as a pathology-based cancer reporting system. From 2005 to 2007, private health laboratories withheld cancer reports owing to concerns regarding voluntary sharing of patient data. Objectives To estimate the impact of under-reported cancer data from private health laboratories. Methods A linear regression analysis was conducted to project expected cancer cases for 2005 – 2007. Differences between actual and projected figures were calculated to estimate percentage under-reporting. Results The projected NCR case total varied from 53 407 (3.8% net increase from actual cases reported) in 2005 to 54 823 (3.7% net increase) in 2007. The projected number of reported cases from private laboratories in 2005 was 26 359 (19.7% net increase from actual cases reported), 27 012 (18.8% net increase) in 2006 and 27 666 (28.4% net increase) in 2007. Conclusion While private healthcare reporting decreased by 28% from 2005 to 2007, this represented a minimal impact on overall cancer reporting (net decrease of <4%). PMID:26242527

  14. Prostate Cancer in South Africa: Pathology Based National Cancer Registry Data (1986–2006) and Mortality Rates (1997–2009)

    PubMed Central

    Babb, Chantal; Urban, Margaret; Kielkowski, Danuta; Kellett, Patricia

    2014-01-01

    Prostate cancer is one of the most common male cancers globally; however little is known about prostate cancer in Africa. Incidence data for prostate cancer in South Africa (SA) from the pathology based National Cancer Registry (1986–2006) and data on mortality (1997–2009) from Statistics SA were analysed. World standard population denominators were used to calculate age specific incidence and mortality rates (ASIR and ASMR) using the direct method. Prostate cancer was the most common male cancer in all SA population groups (excluding basal cell carcinoma). There are large disparities in the ASIR between black, white, coloured, and Asian/Indian populations: 19, 65, 46, and 19 per 100 000, respectively, and ASMR was 11, 7, 52, and 6 per 100 000, respectively. Prostate cancer was the second leading cause of cancer death, accounting for around 13% of male deaths from a cancer. The average age at diagnosis was 68 years and 74 years at death. For SA the ASIR increased from 16.8 in 1986 to 30.8 in 2006, while the ASMR increased from 12.3 in 1997 to 16.7 in 2009. There has been a steady increase of incidence and mortality from prostate cancer in SA. PMID:24955252

  15. Prostate cancer in South Africa: pathology based national cancer registry data (1986-2006) and mortality rates (1997-2009).

    PubMed

    Babb, Chantal; Urban, Margaret; Kielkowski, Danuta; Kellett, Patricia

    2014-01-01

    Prostate cancer is one of the most common male cancers globally; however little is known about prostate cancer in Africa. Incidence data for prostate cancer in South Africa (SA) from the pathology based National Cancer Registry (1986-2006) and data on mortality (1997-2009) from Statistics SA were analysed. World standard population denominators were used to calculate age specific incidence and mortality rates (ASIR and ASMR) using the direct method. Prostate cancer was the most common male cancer in all SA population groups (excluding basal cell carcinoma). There are large disparities in the ASIR between black, white, coloured, and Asian/Indian populations: 19, 65, 46, and 19 per 100 000, respectively, and ASMR was 11, 7, 52, and 6 per 100 000, respectively. Prostate cancer was the second leading cause of cancer death, accounting for around 13% of male deaths from a cancer. The average age at diagnosis was 68 years and 74 years at death. For SA the ASIR increased from 16.8 in 1986 to 30.8 in 2006, while the ASMR increased from 12.3 in 1997 to 16.7 in 2009. There has been a steady increase of incidence and mortality from prostate cancer in SA.

  16. Characteristics of Patients Diagnosed with Osteoporosis in South Korea: Results from the National Claim Registry

    PubMed Central

    Yoon, Hyun Koo

    2017-01-01

    Background Although osteoporosis is increasing medical issue in the elderly population, attempts to analyze the characteristics of patients diagnosed with osteoporosis are rare in South Korea. We investigated the characteristics of patients diagnosed with osteoporosis using Korea's National Health Insurance claims data, which includes almost Korean population. Methods We evaluated the patients who were diagnosed and treated with osteoporosis in medical institute in South Korea, using the national database by the Health Insurance Review & Assessment Service (HIRA). We evaluated the characteristic of patients and state of medical service utilization in 2010. Results The number of patients who were diagnosed with osteoporosis were 1,733,685, and almost of them (91.1%) were females. Fourteen point three percent of the patients had experienced osteoporosis-related fracture. The most commonly used type of institute and department was hospital and orthopedic surgery. Conclusions Considering increasing age population, basic information including characteristic of patients and current state of medical service utilization could be useful to perform appropriate management and treatment for osteoporosis patients in South Korea, using the National Health Insurance claims data. PMID:28326302

  17. Modern utilization of penile prosthesis surgery: a national claim registry analysis.

    PubMed

    Segal, R L; Camper, S B; Burnett, A L

    2014-01-01

    The objective of this study was to evaluate the modern utilization of penile prosthesis surgery based on data derived from national claim databases and contrast to an analysis of patients similarly treated at an academic center during a contemporaneous period. A retrospective claim analysis utilizing a national database (MarketScan, Thomson Reuters) was performed for Commercial insurer and Medicare databases between January 2000 and March 2011. A retrospective analysis of contemporaneous penile prosthesis implantation at the Johns Hopkins Hospital (JHH) was done. Population demographics, comorbidities, previous (ED) therapies and time from ED diagnosis to surgery were assessed. Median ages for patients undergoing penile prosthesis implantation were 58, 70 and 63 years for the Commercial, Medicare and JHH cohorts, respectively. For the claim databases (Commercial, Medicare, respectively), hypertension (72%, 78%), dyslipidemia (71%, 56%) and diabetes mellitus (45%, 40%) were predominant comorbidities, whereas for the JHH database prostate cancer (51%) and its management by prostatectomy (45%) or radiation (12%) were predominant. Previous use of PDE5 inhibitors was similar across databases (60, 58 and 69% for Commercial, Medicare and JHH cohorts, respectively), although previous use of non-oral ED therapies was greater in the JHH database. Median time to surgery from initial ED diagnosis was 2, 2 and 4 years for the Commercial, Medicare and JHH patients, respectively. Demographic variables and ED risk factors associated with penile prosthesis surgery at a national population-based level over a contemporary period were defined. Some differences in utilization trends of penile prosthesis surgery exist at a single institutional level.

  18. Predicting short-term survival after liver transplantation on eight score systems: a national report from China Liver Transplant Registry

    PubMed Central

    Ling, Qi; Dai, Haojiang; Zhuang, Runzhou; Shen, Tian; Wang, Weilin; Xu, Xiao; Zheng, Shusen

    2017-01-01

    To compare the performance of eight score systems (MELD, uMELD, MELD-Na. iMELD, UKELD, MELD-AS, CTP, and mCTP) in predicting the post-transplant mortality, we analyzed the data of 6,014 adult cirrhotic patients who underwent liver transplantation between January 2003 and December 2010 from the China Liver Transplant Registry database. In hepatitis B virus (HBV) group, MELD, uMELD and MELD-AS showed good predictive accuracies at 3-month mortality after liver transplantation; by comparison with other five models, MELD presented the best ability in predicting 3-month, 6-month and 1-year mortality, showing a significantly better predictive ability than UKELD and iMELD. In hepatitis C virus and Alcohol groups, the predictive ability did not differ significantly between MELD and other models. Patient survivals in different MELD categories were of statistically significant difference. Among patients with MELD score >35, a new prognostic model based on serum creatinine, need for hemodialysis and moderate ascites could identify the sickest one. In conclusion, MELD is superior to other score systems in predicting short-term post-transplant survival in patients with HBV-related liver disease. Among patients with MELD score >35, a new prognostic model can identify the sickest patients who should be excluded from waiting list to prevent wasteful transplantation. PMID:28198820

  19. National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

    ClinicalTrials.gov

    2016-12-19

    Marfan Syndrome; Turner Syndrome; Ehlers-Danlos Syndrome; Loeys-Dietz Syndrome; FBN1, TGFBR1, TGFBR2, ACTA2 or MYH11 Genetic Mutation; Bicuspid Aortic Valve Without Known Family History; Bicuspid Aortic Valve With Family History; Bicuspid Aortic Valve With Coarctation; Familial Thoracic Aortic Aneurysm and Dissections; Shprintzen-Goldberg Syndrome; Other Aneur/Diss of Thoracic Aorta Not Due to Trauma, <50yo; Other Congenital Heart Disease

  20. National disease management plans for key chronic non-communicable diseases in Singapore.

    PubMed

    Tan, C C

    2002-07-01

    In Singapore, chronic, non-communicable diseases, namely coronary heart disease, stroke and cancer, account for more than 60% of all deaths and a high burden of disability and healthcare expenditure. The burden of these diseases is likely to rise with our rapidly ageing population and changing lifestyles, and will present profound challenges to our healthcare delivery and financing systems over the next 20 to 30 years. The containment and optimal management of these conditions require a strong emphasis on patient education and the development of integrated models of healthcare delivery in place of the present uncoordinated, compartmentalised way of delivering healthcare. To meet these challenges, the Ministry of Health's major thrusts are disease control measures which focus mainly on primary prevention; and disease management, which coordinates the national effort to reduce the incidence of these key diseases and their predisposing factors and to ameliorate their long-term impact by optimising control to reduce mortality, morbidity and complications, and improving functional status through rehabilitation. The key initiatives include restructuring of the public sector healthcare institutions into two clusters, each comprising a network of primary health care polyclinics, regional hospitals and tertiary institutions. The functional integration of these healthcare elements within each cluster under a common senior administrative and professional management, and the development of common clinical IT systems will greatly facilitate the implementation of disease management programmes. Secondly, the Ministry is establishing National Disease Registries in coronary heart disease, cancer, stroke, myopia and kidney failure, which will be valuable sources of clinical and outcomes data. Thirdly, in partnership with expert groups, national committees and professional agencies, the Ministry will produce clinical practice guidelines which will assist doctors and healthcare

  1. National down syndrome patient database: Insights from the development of a multi-center registry study.

    PubMed

    Lavigne, Jenifer; Sharr, Christianne; Ozonoff, Al; Prock, Lisa Albers; Baumer, Nicole; Brasington, Campbell; Cannon, Sheila; Crissman, Blythe; Davidson, Emily; Florez, Jose C; Kishnani, Priya; Lombardo, Angela; Lyerly, Jordan; McCannon, Jessica B; McDonough, Mary Ellen; Schwartz, Alison; Berrier, Kathryn L; Sparks, Susan; Stock-Guild, Kara; Toler, Tomi L; Vellody, Kishore; Voelz, Lauren; Skotko, Brian G

    2015-11-01

    The Down Syndrome Study Group (DSSG) was founded in 2012 as a voluntary, collaborative effort with the goal of supporting evidenced-based health care guidelines for individuals with Down syndrome (DS). Since then, 5 DS specialty clinics have collected prospective, longitudinal data on medical conditions that co-occur with DS. Data were entered by clinical staff or trained designees into the National Down Syndrome Patient Database, which we created using REDCap software. In our pilot year, we enrolled 663 participants across the U.S., ages 36 days to 70 years, from multiple racial and ethnic backgrounds. Here we report: (i) the demographic distribution of participants enrolled, (ii) a detailed account of our database infrastructure, and (iii) lessons learned during our pilot year to assist future researchers with similar goals for other patient populations.

  2. Physical Trauma and Amyotrophic Lateral Sclerosis: A Population-Based Study Using Danish National Registries

    PubMed Central

    Seals, Ryan M.; Hansen, Johnni; Gredal, Ole; Weisskopf, Marc G.

    2016-01-01

    Prior studies have suggested that physical trauma might be associated with the development of amyotrophic lateral sclerosis (ALS). We conducted a population-based, individually matched case-control study in Denmark to assess whether hospitalization for trauma is associated with a higher risk of developing ALS. There were 3,650 incident cases of ALS in the Danish National Patient Register from 1982 to 2009. We used risk-set sampling to match each case to 100 age- and sex-matched population controls alive on the date of the case's diagnosis. Odds ratios and 95% confidence intervals were calculated using a conditional logistic regression model. History of trauma diagnosis was also obtained from the Danish Patient Register. When traumas in the 5 years prior to the index date were excluded, there was a borderline association between any trauma and ALS (odds ratio (OR) = 1.09, 95% confidence interval (CI): 0.99, 1.19). A first trauma before age 55 years was associated with ALS (OR = 1.22, 95% CI: 1.08, 1.37), whereas first traumas at older ages were not (OR = 0.97, 95% CI: 0.85, 1.10). Our data suggest that physical trauma at earlier ages is associated with ALS risk. Age at first trauma could help explain discrepancies in results of past studies of trauma and ALS. PMID:26825926

  3. National Human Radiobiological Tissue Repository (NHRTR) at the United States Transuranium and Uranium Registries

    DOE Data Explorer

    The NHRTR, one component of the USTUR, contains frozen tissues, tissue solutions, microscope slides, and paraffin blocks that were collected by the USTUR at the autopsy of workers with documented intakes of plutonium, americium, uranium, and thorium. The samples are available to qualified scientists for further research. Thousands of frozen, ashed, dried, and plastic embedded bone samples from the radium studies carried out by Argonne National Laboratory, Argonne Cancer Research Hospital, the Massachusetts Institute of Technology, and the New Jersey Radium Research Project are available and linked by case number to de-identified, published case data. These data include the person's source of exposure (dial painter, therapeutic injection, etc.), estimated body burden, radiochemical results, and medical history. Other samples, including organs and whole body donations, have come from volunteer donors who were impacted by elements such as plutonium, throium, etc. See the USTUR website for information on how to apply for research samples or how to become a volunteer donor. [Information taken from http://www.ustur.wsu.edu/NHRTR/index.html#

  4. 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.

  5. The National Spina Bifida Patient Registry: Profile of a Large Cohort of Participants from the First 10 Clinics

    PubMed Central

    Sawin, Kathleen J.; Liu, Tiebin; Ward, Elisabeth; Thibadeau, Judy; Schechter, Michael S.; Soe, Minn M.; Walker, William

    2015-01-01

    Objective To use data from the US National Spina Bifida Patient Registry (NSBPR) to describe variations in Contexts of Care, Processes of Care, and Health Outcomes among individuals with spina bifida (SB) receiving care in 10 clinics. Study design Reported here are baseline cross-sectional data representing the first visit of 2172 participants from 10 specialized, multidisciplinary SB clinics participating in the NSBPR. We used descriptive statistics, the Fisher exact test, χ2 test, and Wilcoxon rank-sum test to examine the data. Results The mean age was 10.1 (SD 8.1) years with slightly more female subjects (52.5%). The majority was white (63.4%) and relied upon public insurance (53.5%). One-third had sacral lesions, 44.8% had mid-low lumbar lesions, and 24.9% had high lumbar and thoracic lesions. The most common surgery was ventricular shunt placement (65.7%). The most common bladder-management technique among those with bladder impairment was intermittent catheterization (69.0%). Almost 14% experienced a pressure ulcer in the last year. Of those ages 5 years or older with bowel or bladder impairments, almost 30% were continent of stool; a similar percentage was continent of urine. Most variables were associated with type of SB diagnosis. Conclusion The NSBPR provides a cross section of a predominantly pediatric population of patients followed in specialized SB programs. There were wide variations in the variables studied and major differences in Context of Care, Processes of Care, and Health Outcomes by type of SB. Such wide variation and the differences by type of SB should be considered in future analyses of outcomes. PMID:25444012

  6. Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions (GenTAC)

    PubMed Central

    Song, Howard K.; Bavaria, Joseph E.; Kindem, Mark W.; Holmes, Kathryn W.; Milewicz, Dianna M.; Maslen, Cheryl L.; Pyeritz, Reed E.; Basson, Craig T.; Eagle, Kim; Tolunay, H. Eser; Kroner, Barbara L.; Dietz, Hal; Menashe, Victor; Devereux, Richard B.; Desvigne-Nickens, Patrice; Ravekes, William; Weinsaft, Jonathan W.; Brambilla, Donald; Stylianou, Mario P.; Hendershot, Tabitha; Mitchell, Megan S.; LeMaire, Scott A.

    2011-01-01

    Background Genetic disorders are an important cause of thoracic aortic aneurysms (TAAs) in young patients. Despite advances in the treatment of genetically triggered TAAs, the optimal syndrome-specific treatment approach remains undefined. We used data from the NIH-funded, multicenter National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) to characterize the contemporary surgical treatment of patients with genetically triggered TAAs. Methods GenTAC’s aim is to collect longitudinal clinical data and banked biospecimens from 2800 patients with genetically triggered TAAs. We analyzed data from all patients enrolled in GenTAC to date whose clinical data were available (n=606; mean age, 37.5 years). Results The patients’ primary diagnoses included Marfan syndrome (35.8%), bicuspid aortic valve with aneurysm (29.2%), and familial TAAs and dissections (10.7%). More than half of patients (56.4%) had undergone at least 1 operation; the most common indications were aneurysm (85.7%), valve dysfunction (65.8%), and dissection (25.4%). Surgical procedures included replacement of the aortic root (50.6%), ascending aorta (64.8%), aortic arch (27.9%), and descending or thoracoabdominal aorta (12.4%). Syndrome-specific differences in age, indications for surgery, and procedure type were identified. Conclusions Patients with genetically transmitted TAAs evaluated in tertiary care centers frequently undergo surgery. Aneurysm repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like TAAs themselves, complications of TAAs, including dissection and aortic valve dysfunction, are important indications for surgery. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of patients with TAAs. PMID:19699898

  7. Positive predictive value of cardiac examination, procedure and surgery codes in the Danish National Patient Registry: a population-based validation study

    PubMed Central

    Adelborg, Kasper; Sundbøll, Jens; Munch, Troels; Frøslev, Trine; Sørensen, Henrik Toft; Bøtker, Hans Erik; Schmidt, Morten

    2016-01-01

    Objective Danish medical registries are widely used for cardiovascular research, but little is known about the data quality of cardiac interventions. We computed positive predictive values (PPVs) of codes for cardiac examinations, procedures and surgeries registered in the Danish National Patient Registry during 2010–2012. Design Population-based validation study. Setting We randomly sampled patients from 1 university hospital and 2 regional hospitals in the Central Denmark Region. Participants 1239 patients undergoing different cardiac interventions. Main outcome measure PPVs with medical record review as reference standard. Results A total of 1233 medical records (99% of the total sample) were available for review. PPVs ranged from 83% to 100%. For examinations, the PPV was overall 98%, reflecting PPVs of 97% for echocardiography, 97% for right heart catheterisation and 100% for coronary angiogram. For procedures, the PPV was 98% overall, with PPVs of 98% for thrombolysis, 92% for cardioversion, 100% for radiofrequency ablation, 98% for percutaneous coronary intervention, and 100% for both cardiac pacemakers and implantable cardiac defibrillators. For cardiac surgery, the overall PPVs was 99%, encompassing PPVs of 100% for mitral valve surgery, 99% for aortic valve surgery, 98% for coronary artery bypass graft surgery, and 100% for heart transplantation. The accuracy of coding was consistent within age, sex, and calendar year categories, and the agreement between independent reviewers was high (99%). Conclusions Cardiac examinations, procedures and surgeries have high PPVs in the Danish National Patient Registry. PMID:27940630

  8. Lessons learned from the Pediatric Cardiomyopathy Registry (PCMR) Study Group.

    PubMed

    Wilkinson, James D; Westphal, Joslyn A; Bansal, Neha; Czachor, Jason D; Razoky, Hiedy; Lipshultz, Steven E

    2015-08-01

    Cardiomyopathy is a rare disorder of the heart muscle, affecting 1.13 cases per 100,000 children, from birth to 18 years of age. Cardiomyopathy is the leading cause of heart transplantation in children over the age of 1. The Pediatric Cardiomyopathy Registry funded in 1994 by the National Heart, Lung, and Blood Institute was established to examine the epidemiology of the disease in children below 18 years of age. More than 3500 children across the United States and Canada have been enrolled in the Pediatric Cardiomyopathy Registry, which has followed-up these patients until death, heart transplantation, or loss to follow-up. The Pediatric Cardiomyopathy Registry has provided the most in-depth illustration of this disease regarding its aetiology, clinical course, associated risk factors, and patient outcomes. Data from the registry have helped in guiding the clinical management of cardiomyopathy in children under 18 years of age; however, questions still remain regarding the most clinically effective diagnostic and treatment approaches for these patients. Future directions of the registry include the use of next-generation whole-exome sequencing and cardiac biomarkers to identify aetiology-specific treatments and improve diagnostic strategies. This article provides a brief synopsis of the work carried out by the Pediatric Cardiomyopathy Registry since its inception, including the current knowledge on the aetiologies, outcomes, and treatments of cardiomyopathy in children.

  9. 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

  10. Reference Standards for Cardiorespiratory Fitness Measured With Cardiopulmonary Exercise Testing: Data From the Fitness Registry and the Importance of Exercise National Database

    PubMed Central

    Kaminsky, Leonard A.; Arena, Ross; Myers, Jonathan

    2016-01-01

    Objective To develop standards for cardiorespiratory fitness by establishing reference values derived from cardiopulmonary exercise testing (CPX) in the United States. Patients and Methods Eight laboratories in the US experienced in CPX administration with established quality control procedures contributed data from January 1, 2014, through February 1, 2015, from 7783 maximal (respiratory exchange ratio, ≥1.0) treadmill tests from men and women (aged 20–79 years) without cardiovascular disease (CVD) to the Fitness Registry and the Importance of Exercise: A National Data Base (FRIEND). Percentiles of maximal oxygen consumption (V̇O2max) for men and women were determined for each decade from 20 years of age through 79 years of age. Comparisons of V̇O2maxwere made to reference data established with CPX data from Norway and to US reference data established without CPX measurements. Results There were significant differences between sex and age groups for V̇O2max. In FRIEND, the 50th percentile V̇O2max of men and women aged 20 to 29 years decreased from 48.0 and 37.6 mLO2·kg−1·min−1 to 24.4 and 18.3 mLO2·kg−1·min−1 for ages 70 to 79 years, respectively. The rate of decline in this cohort during a 5-decade period was approximately 10% per decade. Conclusion These are the first cardiorespiratory fitness reference data using measures obtained from CPX in the United States. FRIEND can be used to provide a more accurate interpretation of measured V̇O2max from maximal exercise tests for the US population compared with previous standards on the basis of workload-derived estimations. PMID:26455884

  11. Association of glomerular filtration rate with outcomes of acute stroke in type 2 diabetic patients: results from the China National Stroke Registry.

    PubMed

    Luo, Yang; Wang, Xianwei; Wang, Yilong; Wang, Chunxue; Wang, Haichen; Wang, David; Liu, Liping; Jia, Qian; Liu, Gaifen; Zhao, Xingquan; Wang, Yongjun

    2014-01-01

    OBJECTIVE We aim to explore whether a link exists between different levels of estimated glomerular filtration rate (eGFR) and poor outcomes of acute stroke in patients with type 2 diabetes. RESEARCH DESIGN AND METHODS Between 2007 and 2009, 6,261 patients with cerebrovascular events and diabetes were included in the final analysis from the China National Stroke Registry (CNSR) and substudy of CNSR (Abnormal Glucose Regulation in Patients with Acute Stroke Across China [ACROSS]).The period of follow-up was 1 year after stroke onset. eGFR was calculated with the Chinese modification of Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. The association between eGFR and poor stroke outcomes, including all-cause death, recurrent stroke, combined end point (stroke or death), and stroke disability, was evaluated by multivariate analysis with the adjustment for demographic and clinical features. RESULTS Of 4,836 patients with stroke, low eGFR (<45 mL/min/1.73 m(2)) occurred in 268 (5.5%) and high eGFR (≥120 mL/min/1.73 m(2)) in 387 (8.0%). The median value for eGFR in all patients was 92.6 mL/min/1.73 m(2). Low eGFR was independently associated with risks of all clinical outcomes in stroke/transient ischemic attack patients or patients with ischemic events, but not in patients with hemorrhagic stroke. Additionally, high eGFR was positively associated with an increased risk of adverse outcomes in all stroke subtypes, including hemorrhagic stroke. CONCLUSIONS Low and high eGFRs (<45 or ≥120 mL/min/1.73 m(2), respectively) are independent predictors of all-cause mortality and other poor outcomes after acute stroke in patients with type 2 diabetes.

  12. 78 FR 17213 - Agency for Toxic Substances and Disease Registry Availability of Final Toxicological Profile

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-20

    ....S. Department of Commerce, National Technical Information Service (NTIS), 5285 Port Royal Road, Springfield, Virginia 22161, telephone 1-800-553-6847 for a fee as determined by NTIS. Dated: March 13,...

  13. Validity of Race, Ethnicity, and National Origin in Population-Based Cancer Registries and Rapid Case Ascertainment Enhanced with a Spanish Surname List

    PubMed Central

    Clarke, Lisa C.; Rull, Rudolph P.; Ayanian, John Z.; Boer, Robert; Deapen, Dennis; West, Dee W.; Kahn, Katherine L.

    2015-01-01

    Background Accurate information regarding race, ethnicity, and national origins is critical for identifying disparities in the cancer burden. Objectives To examine the use of a Spanish surname list to improve the quality of race-related information obtained from rapid case ascertainment (RCA) and to estimate the accuracy of race-related information obtained from cancer registry records collected by routine reporting. Subjects . Self-reported survey responses of 3,954 participants from California enrolled in the Cancer Care Outcomes Research and Surveillance Consortium (CanCORS). Measures Sensitivity, specificity, positive predictive value (PPV), and percent agreement. We employed logistic regression to identify predictors of under-reporting and over-reporting of a race/ethnicity. Results Use of the Spanish surname list increased the sensitivity of RCA for Latino ethnicity from 37% to 83%. Sensitivity for cancer registry records collected by routine reporting was ≥95% for Whites, Blacks, and Asians, and specificity was high for all groups (86–100%). However, patterns of misclassification by race/ethnicity were found that could lead to biased cancer statistics for specific race/ethnicities. Discordance between self- and registry-reported race/ethnicity was more likely for women, Latinos, and Asians. Conclusion Methods to improve race and ethnicity data, such as using Spanish surnames in RCA and instituting data collection guidelines for hospitals, are needed to ensure minorities are accurately represented in clinical and epidemiological research. PMID:23938598

  14. Psychotropic Medication Use among Children with Autism Spectrum Disorders Enrolled in a National Registry, 2007-2008

    ERIC Educational Resources Information Center

    Rosenberg, Rebecca E.; Mandell, David S.; Farmer, Janet E.; Law, J. Kiely; Marvin, Alison R.; Law, Paul A.

    2010-01-01

    Patterns of current psychotropic medication use among 5,181 children with autism spectrum disorders (ASD) enrolled in a Web-based registry were examined. Overall, 35% used at least one psychotropic medication, most commonly stimulants, neuroleptics, and/or antidepressants. Those who were uninsured or exclusively privately insured were less likely…

  15. Lombardia GENS: a collaborative registry for monogenic diseases associated with stroke.

    PubMed

    Bersano, Anna; Baron, Pierluigi; Lanfranconi, Silvia; Trobia, Nadia; Sterzi, Roberto; Motto, Cristina; Comi, Giancarlo; Sessa, Maria; Martinelli-Boneschi, Filippo; Micieli, Giuseppe; Ferrarese, Carlo; Santoro, Patrizia; Parati, Eugenio; Boncoraglio, Giorgio; Padovani, Alessandro; Pezzini, Alessandro; Candelise, Livia

    2012-01-01

    The Italian region of Lombardy, with its existing stroke centers and high-technology laboratories, provides a favorable context for studying monogenic diseases associated with stroke. The Lombardia GENS project was set up to create a regional network for the diagnosis of six monogenic diseases associated with stroke: CADASIL, Fabry disease, MELAS, familial and sporadic hemiplegic migraine, hereditary cerebral amyloid angiopathy and Marfan syndrome. The network comprises 36 stroke centers and seven high-technology laboratories, performing molecular analysis. In this context, all stroke/TIA patients fulfilling clinical criteria for monogenic diseases are currently being included in an ongoing study. Demographic, clinical and family data and diagnostic criteria are collected using standardized forms. On the basis of stroke incidence in Lombardy and the reported prevalence of the diseases considered, we expect, during the course of the study, to collect datasets and DNA samples from more than 200 stroke patients suspected of having monogenic diseases. This will allow evaluation of the regional burden and better phenotype characterization of monogenic diseases associated with stroke.

  16. Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry.

    PubMed

    Leiva, Lily E; Zelazco, Marta; Oleastro, Matías; Carneiro-Sampaio, Magda; Condino-Neto, Antonio; Costa-Carvalho, Beatriz Tavares; Grumach, Anete Sevciovic; Quezada, Arnoldo; Patiño, Pablo; Franco, José Luis; Porras, Oscar; Rodríguez, Francisco Javier; Espinosa-Rosales, Francisco Javier; Espinosa-Padilla, Sara Elva; Almillategui, Diva; Martínez, Celia; Tafur, Juan Rodríguez; Valentín, Marilyn; Benarroch, Lorena; Barroso, Rosy; Sorensen, Ricardo U

    2007-01-01

    This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras, Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reveals that from a total of 3321 patients registered, the most common form of primary immunodeficiency disease was predominantly antibody deficiency (53.2%) with IgA deficiency reported as the most frequent phenotype. This category was followed by 22.6% other well-defined ID syndromes, 9.5% combined T- and B-cell inmunodeficiency, 8.6% phagocytic disorders, 3.3% diseases of immune dysregulation, and 2.8% complement deficiencies. All countries that participated in the first publication in 1998 reported an increase in registered primary immunodeficiency cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic granulomatous disease, and severe combined immunodeficiency between the first report and the present one shows an increase in the reporting of these diseases in all countries. In this report, the estimated minimal incidence of chronic granulomatous disease was between 0.72 and 1.26 cases per 100,000 births in Argentina, Chile, Costa Rica, and Uruguay and the incidence of severe combined immunodeficiency was 1.28 and 3.79 per 100,000 births in Chile and Costa Rica, respectively. However, these diseases are underreported in other participating countries. In addition to a better diagnosis of primary immunodeficiency diseases, more work on improving the registration of patients by each participating country and by countries that have not yet joined LAGID is still needed.

  17. Evaluation of a New Balloon Catheter for Difficult Calcified Lesions in Infrainguinal Arterial Disease: Outcome of a Multicenter Registry

    SciTech Connect

    Spaargaren, G. J.; Lee, M. J.; Reekers, J. A.; Overhagen, H. van; Schultze Kool, L. J.; Hoogeveen, Y. L.

    2009-01-15

    The purpose of this study was to assess the technical performance and immediate procedure outcome of a new balloon catheter in the treatment of calcified lesions in infrainguinal arterial disease. Seventy-five patients with infrainguinal arterial disease were prospectively entered into the registry. The catheter (ReeKross Clearstream, Ireland) is a 5- to 6-Fr balloon catheter with a rigid shaft intended for enhanced pushability. Only technical procedural outcome was recorded. Treated calcified lesions (range: 5-30 cm), assessed angiographically, were located in the superficial femoral, popliteal, and crural arteries. In 67 patients the lesion was an occlusion. Guidewire passage occurred subintimally in 68 patients. In 24 patients a standard balloon catheter was chosen as first treatment catheter: 5 failed to cross the lesion, 8 balloons ruptured, and in 11 patients there was an inadequate dilatation result. In only one of the five patients did subsequent use of the ReeKross catheter also fail in lesion crossing. The ReeKross was successful as secondary catheter in the other 23 cases. In 50 patients the ReeKross was used as primary catheter. In total the ReeKross crossed the lesions in 74 patients. After passage and dilatation with this catheter in 73 patients (1 failed true-lumen reentry), 19 had >30% residual lesions, of which 11 were not treated and 8 were successfully stented. No ReeKross balloons ruptured. We conclude that in the treatment of difficult calcified lesions in arterial stenotic or occlusive disease, the choice of a high-pushability angioplasty catheter, with more calcification-resistant balloon characteristics, like the ReeKross, warrants consideration.

  18. Update on the NAS-NRC Twin Registry.

    PubMed

    Page, William F

    2006-12-01

    The National Academy of Sciences-National Research Council (NAS-NRC) Twin Registry is one of the oldest, national population-based twin registries in the United States. It consists of 15,924 white male twin pairs born in the years 1917 to 1927 (inclusive), both of whom served in the armed forces, mostly during World War II. This article updates activity in this registry since the earlier 2002 article in Twin Research. The results of clinically based studies on dementia, Parkinson's disease, age-related macular degeneration, and primary osteoarthritis were published, as well as articles based on previously collected questionnaire data on chronic fatigue syndrome, functional limitations, and healthy aging. In addition, risk factor studies are being planned to merge clinical data with earlier collected risk factor data from questionnaires. Examination data from the subset of National Heart, Lung, and Blood Institute (NHLBI) twins resulted in a number of articles, including the relationship of endogenous sex hormones to coronary heart disease and morphological changes in aging brain structures. The NEO Five-Factor Personality Inventory (a paper-and-pencil self-administered questionnaire) has been fielded for the first time. A push to consolidate the various data holdings of the registry is being made.

  19. [The user´s reporting from the national registry of catheter aortic valve implantations (Czech TAVI Registry): the possibilities of the analytical reports based on the database system TrialDB2].

    PubMed

    Bláha, Milan; Kala, Petr; Klimeš, Daniel; Bernat, Ivo; Branny, Marian; Cervinka, Pavel; Horák, Jan; Kočka, Viktor; Mates, Martin; Němec, Petr; Pešl, Ladislav; Stípal, Roman; Sťásek, Josef; Zelízko, Michael

    2014-10-01

    Assessment of the treatment procedures and their results is increasingly important in current medicine. The emphasis is put on an effective use of the health technologies (HTA). Unlike randomised studies, which involve strictly selected groups of patients who meet inclusion and exclusion criterias, the multicentre clinical registries provide a real-life picture of the treatment safety and effectiveness. Well prepared registries involve both research database and a friendly user interface enabling collection of parametric and easily analyzable data. Although there are some technological aspects aiming to ensure a maximum quality of entered data, cooperation with the users and data managers is essential. Such a registry, otherwise meaningful, must provide answers to previously defined medical hypotheses. Regular feedback to users (so called benchmarking or reporting) is considered to be of key importance. The Czech TAVI Registry (CTR) is a good example of reaching all of the above defined criterias. This registry contains data of approximately 95 % of all transcatheter aortic valve implantations (TAVI) performed in the Czech Republic. It is based on a general system aimed at the design of clinical trials, namely the TrialDB2 (a database system for clinical registries developed by the Institute of Biostatistics and Analyses at the Masaryk University (IBA MU). CTR has been run as an English-language version under the auspices of the Czech Society of Cardiology and represents one of the top-quality registries maintained by IBA MU. This paper presents the currently available database systems and some reports from this particular registry.

  20. 76 FR 27070 - National Institute of Allergy and Infectious Diseases;

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-05-10

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... . Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis Panel,...

  1. [Optimization of registry of deaths from chronic kidney disease in agricultural communities in Central America].

    PubMed

    Escamilla-Cejudo, José Antonio; Báez, Jorge Lara; Peña, Rodolfo; Luna, Patricia Lorena Ruiz; Ordunez, Pedro

    2016-11-01

    Several Central American countries are seeing continued growth in the number of deaths from chronic kidney disease of nontraditional causes (CKDnT) among farm workers and there is underreporting. This report presents the results of a consensus process coordinated by the Pan American Health Organization/World Health Organization (PAHO/WHO), the United States Centers for Disease Control and Prevention (CDC), and the Latin American Society of Nephrology and Hypertension (SLANH). This consensus seeks to increase the probability of detecting and recording deaths from these causes. There has been recognition of the negative impact of the lack of a standardized instrument and the lack of training in the medical profession for adequate registration of the cause or causes of death. As a result of the consensus, the following has been proposed: temporarily use a code from the Codes for Special Purposes in the International Classification of Diseases (ICD-10); continue to promote use of the WHO international standardized instrument for recording causes and preceding events related to death; increase training of physicians responsible for filling out death certificates; take action to increase the coverage and quality of information on mortality; and create a decision tree to facilitate selection of CKDnT as a specific cause of death, while presenting the role that different regional and subregional mechanisms in the Region of the Americas should play in order to improve CKD and CKDnT mortality records.

  2. [Diagnostics and treatment of lower extremity peripheral arterial disease; guideline and registry].

    PubMed

    Vahl, A; Elsman, B; van Enst, A

    2016-01-01

    - Revision of the 2005 guideline 'Diagnostics and treatment of lower extremity peripheral arterial disease' and the development of an audit have instigated a degree of efficiency and transparency for the treatment of patients with peripheral arterial disease (PAD). - The key recommendations are that first-line treatment of patients, who - preferably by means of a consultation in a vascular laboratory - are diagnosed with intermittent claudication, is supervised exercise therapy and secondary prevention. Referral for second-line treatment only needs to occur when invasive therapy is considered, for example when there is insufficient improvement in symptoms or in patients who have or are developing critical ischemia. - In case of endovascular treatments it is not necessary to insert stents routinely; this can be reserved for cases where the angiographic result of the angioplasty is insufficient. - All patients with PAD are registered on the DAPA register ('Dutch audit for peripheral arterial disease'), which has two unique characteristics: patient reported outcome measures (PROMs) are recorded and a case mix correction is incorporated for the PROMs and amputation-free survival through linkage with the health insurance database.

  3. Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry

    PubMed Central

    Ortiz, Alberto; Abiose, Ademola; Bichet, Daniel G; Cabrera, Gustavo; Charrow, Joel; Germain, Dominique P; Hopkin, Robert J; Jovanovic, Ana; Linhart, Aleš; Maruti, Sonia S; Mauer, Michael; Oliveira, João P; Patel, Manesh R; Politei, Juan; Waldek, Stephen; Wanner, Christoph; Yoo, Han-Wook; Warnock, David G

    2016-01-01

    Background Agalsidase β is a form of enzyme replacement therapy for Fabry disease, a genetic disorder characterised by low α-galactosidase A activity, accumulation of glycosphingolipids and life-threatening cardiovascular, renal and cerebrovascular events. In clinical trials, agalsidase β cleared glycolipid deposits from endothelial cells within 6 months; clearance from other cell types required sustained treatment. We hypothesised that there might be a ‘lag time’ to clinical benefit after initiating agalsidase β treatment, and analysed the incidence of severe clinical events over time in patients receiving agalsidase β. Methods The incidence of severe clinical events (renal failure, cardiac events, stroke, death) was studied in 1044 adult patients (641 men, 403 women) enrolled in the Fabry Registry who received agalsidase β (average dose 1 mg/kg every 2 weeks) for up to 5 years. Results The incidence of all severe clinical events was 111 per 1000 person-years (95% CI 84 to 145) during the first 6 months. After 6 months, the incidence decreased and remained stable within the range of 40–58 events per 1000 patient-years. The largest decrease in incidence rates was among male patients and those aged ≥40 years when agalsidase β was initiated. Conclusions Contrary to the expected increased incidence of severe clinical events with time, adult patients with Fabry disease had decreased incidence of severe clinical events after 6 months treatment with agalsidase β 1 mg/kg every 2 weeks. Trial registration number NCT00196742. PMID:26993266

  4. Unravelling the myotonic dystrophy type 1 clinical spectrum: A systematic registry-based study with implications for disease classification.

    PubMed

    De Antonio, M; Dogan, C; Hamroun, D; Mati, M; Zerrouki, S; Eymard, B; Katsahian, S; Bassez, G

    2016-10-01

    The broad clinical spectrum of myotonic dystrophy type 1 (DM1) creates particular challenges for both medical care and design of clinical trials. Clinical onset spans a continuum from birth to late adulthood, with symptoms that are highly variable in both severity and nature of the affected organ systems. In the literature, this complex phenotype is divided into three grades (mild, classic, and severe) and four or five main clinical categories (congenital, infantile/juvenile, adult-onset and late-onset forms), according to symptom severity and age of onset, respectively. However, these classifications are still under discussion with no consensus thus far. While some specific clinical features have been primarily reported in some forms of the disease, there are no clear distinctions. As a consequence, no modifications in the management of healthcare or the design of clinical studies have been proposed based on the clinical form of DM1. The present study has used the DM-Scope registry to assess, in a large cohort of DM1 patients, the robustness of a classification divided into five clinical forms. Our main aim was to describe the disease spectrum and investigate features of each clinical form. The five subtypes were compared by distribution of CTG expansion size, and the occurrence and onset of the main symptoms of DM1. Analyses validated the relevance of a five-grade model for DM1 classification. Patients were classified as: congenital (n=93, 4.5%); infantile (n=303, 14.8%); juvenile (n=628, 30.7%); adult (n=694, 34.0%); and late-onset (n=326, 15.9%). Our data show that the assumption of a continuum from congenital to the late-onset form is valid, and also highlights disease features specific to individual clinical forms of DM1 in terms of symptom occurrence and chronology throughout the disease course. These results support the use of the five-grade model for disease classification, and the distinct clinical profiles suggest that age of onset and clinical form may

  5. Prospective observational cohort studies for studying rare diseases: the European PedNet Haemophilia Registry.

    PubMed

    Fischer, K; Ljung, R; Platokouki, H; Liesner, R; Claeyssens, S; Smink, E; van den Berg, H M

    2014-07-01

    Haemophilia is a rare disease. To improve knowledge, prospective studies of large numbers of subjects are needed. To establish a large well-documented birth cohort of patients with haemophilia enabling studies on early presentation, side effects and outcome of treatment. Twenty-one haemophilia treatment centres have been collecting data on all children with haemophilia with FVIII/IX levels up to 25% born from 2000 onwards. Another eight centres collected data on severe haemophilia A only. At baseline, details on delivery and diagnosis, gene mutation, family history of haemophilia and inhibitors are collected. For the first 75 exposure days, date, reason, dose and product are recorded for each infusion. Clinically relevant inhibitors are defined as follows: at least two positive inhibitor titres and a FVIII/IX recovery <66% of expected. For inhibitor patients, results of all inhibitor- and recovery tests are collected. For continued treatment, data on bleeding, surgery, prophylaxis and clotting factor consumption are collected annually. Data are downloaded for analysis annually. In May 2013, a total of 1094 patients were included: 701 with severe, 146 with moderate and 247 with mild haemophilia. Gene defect data were available for 87.6% of patients with severe haemophilia A. The first analysis, performed in May 2011, lead to two landmark publications. The outcome of this large collaborative research confirms its value for the improvement of haemophilia care. High-quality prospective observational cohorts form an ideal source to study natural history and treatment in rare diseases such as haemophilia.

  6. Stroke Trials Registry

    MedlinePlus

    ... News About Neurology Image Library Search The Internet Stroke Center Trials Registry Clinical Trials Interventions Conditions Sponsors ... a clinical trial near you Welcome to the Stroke Trials Registry Our registry of clinical trials in ...

  7. 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

  8. IgG4-Related Disease: Results From a Multicenter Spanish Registry

    PubMed Central

    Fernández-Codina, Andreu; Martínez-Valle, Fernando; Pinilla, Blanca; López, Cristina; DeTorres, Inés; Solans-Laqué, Roser; Fraile-Rodríguez, Guadalupe; Casanovas-Martínez, Arnau; López-Dupla, Miguel; Robles-Marhuenda, Ángel; Barragán-González, María Jesús; Cid, Maria Cinta; Prieto-González, Sergio; Brito-Zerón, Pilar; Cruces-Moreno, María Teresa; Fonseca-Aizpuru, Eva; López-Torres, Manuel; Gil, Judith; Núñez-Fernández, Manuel Jesús; Pardos-Gea, José; Salvador-Cervelló, Gonzalo

    2015-01-01

    Abstract IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients. To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients. A retrospective multicenter study was performed. Twelve hospitals across Spain included patients meeting the current 2012 consensus criteria on IgG4-RD diagnosis. Fifty-five patients were included in the study, 38 of whom (69.1%) were male. Median age at diagnosis was 53 years. Thirty (54.5%) patients were included in the Histologically Highly Suggestive IgG4-RD group and 25 (45.5%) in the probable IgG4-RD group. Twenty-six (47.3%) patients had more than 1 organ affected at presentation. The most frequently affected organs were: retroperitoneum, orbital pseudotumor, pancreas, salivary and lachrymal glands, and maxillary sinuses. Corticosteroids were the mainstay of treatment (46 patients, 83.6%). Eighteen patients (32.7%) required additional immunosuppressive agents. Twenty-four (43.6%) patients achieved a complete response and 26 (43.7%) presented a partial response (<50% of regression) after 22 months of follow-up. No deaths were attributed directly to IgG4-RD and malignancy was infrequent. This is the largest IgG4-RD series reported in Europe. Patients were middle-aged males, with histologically probable IgG4-RD. The systemic form of the disease was frequent, involving mainly sites of the head and abdomen. Corticosteroids were an effective first line treatment, sometimes combined with immunosuppressive agents. Neither fatalities nor malignancies were attributed to IgG4-RD. PMID:26266361

  9. Lessons from 30 years’ data of Korean end-stage renal disease registry, 1985–2015

    PubMed Central

    Jin, Dong-Chan; Yun, Sung Ro; Lee, Seoung Woo; Han, Sang Woong; Kim, Won; Park, Jongha; Kim, Yong Kyun

    2015-01-01

    The Korean Society of Nephrology (KSN) launched a nationwide official survey program about dialysis therapy in 1985. Nowadays, the accumulated data for 30 years by this “Insan Prof. Min Memorial end-stage renal disease (ESRD) Registry” program have been providing the essential information for dialysis clinical practice, academic nephrology research, and health management policy. We reviewed 30 years of data to identify important changes and implications for the future improvement of dialysis therapy in Korea. Hemodialysis patients, especially diabetics and elderly patients have increased in number very rapidly during recent years in Korea. The Korean prevalence rate of ESRD patients was about 70% of the United States and about 50% of Japan according to the international comparisons in the annual data report of United States Renal Data System. The blood pressure control, anemia control, and dialysis adequacy have continuously improved year by year. The importance of calcium and phosphorus control has also been increasing because of the increase in long-term dialysis patients. In addition, chronic dialysis complications should be closely monitored and dialysis modifications, such as hemodiafiltration therapy, might be considered. Because of the increase of private clinics and nursing hospitals in dialysis practice, the role of dialysis specialists and continuing education are thought to be essential. For strict cost-effective dialysis control of increasing elderly, diabetic, and long-term dialysis patients, the KSN ESRD patient registration should be run by the KSN and health ministry in cooperation, in which the dialysis fee reimbursement should be accompanied. PMID:26484037

  10. The national database of hospital-based cancer registries: a nationwide infrastructure to support evidence-based cancer care and cancer control policy in Japan.

    PubMed

    Higashi, Takahiro; Nakamura, Fumiaki; Shibata, Akiko; Emori, Yoshiko; Nishimoto, Hiroshi

    2014-01-01

    Monitoring the current status of cancer care is essential for effective cancer control and high-quality cancer care. To address the information needs of patients and physicians in Japan, hospital-based cancer registries are operated in 397 hospitals designated as cancer care hospitals by the national government. These hospitals collect information on all cancer cases encountered in each hospital according to precisely defined coding rules. The Center for Cancer Control and Information Services at the National Cancer Center supports the management of the hospital-based cancer registry by providing training for tumor registrars and by developing and maintaining the standard software and continuing communication, which includes mailing lists, a customizable web site and site visits. Data from the cancer care hospitals are submitted annually to the Center, compiled, and distributed as the National Cancer Statistics Report. The report reveals the national profiles of patient characteristics, route to discovery, stage distribution, and first-course treatments of the five major cancers in Japan. A system designed to follow up on patient survival will soon be established. Findings from the analyses will reveal characteristics of designated cancer care hospitals nationwide and will show how characteristics of patients with cancer in Japan differ from those of patients with cancer in other countries. The database will provide an infrastructure for future clinical and health services research and will support quality measurement and improvement of cancer care. Researchers and policy-makers in Japan are encouraged to take advantage of this powerful tool to enhance cancer control and their clinical practice.

  11. Impact of chronic obstructive pulmonary disease on Valve Academic Research Consortium-defined outcomes after transcatheter aortic valve implantation (from the FRANCE 2 Registry).

    PubMed

    Chopard, Romain; Meneveau, Nicolas; Chocron, Sidney; Gilard, Martine; Laskar, Marc; Eltchaninoff, Hélène; Iung, Bernard; Leprince, Pascal; Teiger, Emmanuel; Chevreul, Karine; Prat, Alain; Lievre, Michel; Leguerrier, Alain; Donzeau-Gouge, Patrick; Fajadet, Jean; Schiele, Francois

    2014-05-01

    The purposes of the present study were to determine the impact of chronic obstructive pulmonary disease (COPD) on Valve Academic Research Consortium-defined outcomes in patients undergoing transcatheter aortic valve implantation (TAVI). A total of 3,933 consecutive patients underwent TAVI from January 2010 to December 2011 in 34 centers and were included in the French national TAVI registry "FRANCE 2"; 895 (22.7%) had concomitant COPD, 3,038 (77.3%) did not. There were no significant differences in procedural characteristics or 30-day Valve Academic Research Consortium-defined outcomes between those with and without COPD. Multivariate regression analysis showed COPD to be an independent predictor of 1-year mortality and combined efficacy end point after adjustment for concomitant co-morbidities (hazard ratio 1.19, 95% confidence interval 1.005 to 1.41, p = 0.03 and hazard ratio 1.52, 95% confidence interval 1.29 to 1.79, p <0.001, respectively). The higher mortality rate at 1 year in patients with COPD was related to cardiovascular deaths (COPD 10.0% vs non-COPD 6.2%, p = 0.008). Subgroup analysis found that the effect of COPD on 1-year mortality rate was constant across different subgroups, especially the type of approach and the type of anesthesia subgroups. In conclusion, concomitant COPD in patients referred for TAVI characterizes a high-risk population. The excess in mortality is largely determined by a higher rate of cardiovascular deaths and exists regardless of the type of procedure performed and its results.

  12. Attitudes toward Potential Participant Registries.

    PubMed

    Grill, Joshua D; Holbrook, Andrew; Pierce, Aimee; Hoang, Dan; Gillen, Daniel L

    2017-01-01

    Difficult participant recruitment is a consistent barrier to successful medical research. Potential participant registries represent an increasingly common intervention to overcome this barrier. A variety of models for registries exist, but few data are available to instruct their design and implementation. To provide such data, we surveyed 110 cognitively normal research participants enrolled in a longitudinal study of aging and dementia. Seventy-four (67%) individuals participated in the study. Most (78%, CI: 0.67, 0.87) participants were likely to enroll in a registry. Willingness to participate was reduced for registries that required enrollment through the Internet using a password (26%, CI: 0.16, 0.36) or through email (38%, CI: 0.27, 0.49). Respondents acknowledged their expectations that researchers share information about their health and risk for disease and their concerns that their data could be shared with for-profit companies. We found no difference in respondent preferences for registries that shared contact information with researchers, compared to honest broker models that take extra precautions to protect registrant confidentiality (28% versus 30%; p = 0.46). Compared to those preferring a shared information model, respondents who preferred the honest broker model or who lacked model preference voiced increased concerns about sharing registrant data, especially with for-profit organizations. These results suggest that the design of potential participant registries may impact the population enrolled, and hence the population that will eventually be enrolled in clinical studies. Investigators operating registries may need to offer particular assurances about data security to maximize registry enrollment but also must carefully manage participant expectations.

  13. 50 CFR 600.1410 - Registry process.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... 50 Wildlife and Fisheries 12 2014-10-01 2014-10-01 false Registry process. 600.1410 Section 600.1410 Wildlife and Fisheries FISHERY CONSERVATION AND MANAGEMENT, NATIONAL OCEANIC AND ATMOSPHERIC... United States § 600.1410 Registry process. (a) A person may register through the NMFS web site at...

  14. 50 CFR 600.1410 - Registry process.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 50 Wildlife and Fisheries 10 2011-10-01 2011-10-01 false Registry process. 600.1410 Section 600.1410 Wildlife and Fisheries FISHERY CONSERVATION AND MANAGEMENT, NATIONAL OCEANIC AND ATMOSPHERIC... United States § 600.1410 Registry process. (a) A person may register through the NMFS web site at...

  15. 50 CFR 600.1410 - Registry process.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... 50 Wildlife and Fisheries 12 2013-10-01 2013-10-01 false Registry process. 600.1410 Section 600.1410 Wildlife and Fisheries FISHERY CONSERVATION AND MANAGEMENT, NATIONAL OCEANIC AND ATMOSPHERIC... United States § 600.1410 Registry process. (a) A person may register through the NMFS web site at...

  16. Improvement in Mortality Risk Prediction Following Percutaneous Coronary Intervention Through Addition of a “Compassionate Use” Variable to the National Cardiovascular Data Registry CathPCI® Dataset: A Study from the Massachusetts Angioplasty Registry

    PubMed Central

    Resnic, Frederic S.; Normand, Sharon-Lise T.; Piemonte, Thomas C.; Shubrooks, Samuel J.; Zelevinsky, Katya; Lovett, Ann; Ho, Kalon K.L.

    2011-01-01

    Objectives This study investigated the impact of adding novel elements to models predicting in-hospital mortality following percutaneous coronary interventions (PCIs). Background Massachusetts (MA) mandated public reporting of hospital-specific PCI mortality in 2003. In 2006, a physician advisory group recommended adding to the prediction models three attributes not collected by the National Cardiovascular Data Registry instrument. These “compassionate use” (CU) features included coma on presentation, active hemodynamic support during PCI, and cardiopulmonary resuscitation at PCI initiation. Methods From October 2005 through September 2007, PCI was performed during 29,784 admissions in MA non-federal hospitals. Of these, 5,588 involved patients with ST segment elevation myocardial infarction or cardiogenic shock. Cases with CU criteria identified were adjudicated by trained physician reviewers. Regression models with and without the CU composite variable (presence of any of the 3 features) were compared using areas under the receiver operator characteristic curves (AUC). Results Unadjusted mortality in this high-risk subset was 5.7%. Among these admissions, 96 (1.7%) had at least one CU feature, with 69.8% mortality. The adjusted odds ratio for in-hospital death for CU PCIs (vs. no CU criteria) was 27.3 (95% CI 14.5–47.6). Discrimination of the model improved after including CU, with AUC increasing from 0.87 to 0.90 (p<0.01), while goodness of fit was preserved. Conclusions A small proportion of patients at extreme risk for post-PCI mortality can be identified using pre-procedural factors not routinely collected, but that heighten predictive accuracy. Such improvements in model performance may result in greater confidence in reporting of risk-adjusted PCI outcomes. PMID:21329835

  17. Standardized cardiovascular data for clinical research, registries, and patient care: a report from the Data Standards Workgroup of the National Cardiovascular Research Infrastructure project.

    PubMed

    Anderson, H Vernon; Weintraub, William S; Radford, Martha J; Kremers, Mark S; Roe, Matthew T; Shaw, Richard E; Pinchotti, Dana M; Tcheng, James E

    2013-05-07

    Relatively little attention has been focused on standardization of data exchange in clinical research studies and patient care activities. Both are usually managed locally using separate and generally incompatible data systems at individual hospitals or clinics. In the past decade there have been nascent efforts to create data standards for clinical research and patient care data, and to some extent these are helpful in providing a degree of uniformity. Nonetheless, these data standards generally have not been converted into accepted computer-based language structures that could permit reliable data exchange across computer networks. The National Cardiovascular Research Infrastructure (NCRI) project was initiated with a major objective of creating a model framework for standard data exchange in all clinical research, clinical registry, and patient care environments, including all electronic health records. The goal is complete syntactic and semantic interoperability. A Data Standards Workgroup was established to create or identify and then harmonize clinical definitions for a base set of standardized cardiovascular data elements that could be used in this network infrastructure. Recognizing the need for continuity with prior efforts, the Workgroup examined existing data standards sources. A basic set of 353 elements was selected. The NCRI staff then collaborated with the 2 major technical standards organizations in health care, the Clinical Data Interchange Standards Consortium and Health Level Seven International, as well as with staff from the National Cancer Institute Enterprise Vocabulary Services. Modeling and mapping were performed to represent (instantiate) the data elements in appropriate technical computer language structures for endorsement as an accepted data standard for public access and use. Fully implemented, these elements will facilitate clinical research, registry reporting, administrative reporting and regulatory compliance, and patient care.

  18. Urological procedures in Central Europe and the current reality based on the national registries of Czech Republic, Hungary, and Poland (2012 status)

    PubMed Central

    Juszczak, Kajetan; Drewa, Tomasz; Hora, Milan; Nyirády, Peter; Sosnowski, Marek

    2016-01-01

    Introduction In recent years, the laparoscopic approach in oncologic urology seems more attractable to the surgeons. It is considered to have the same oncologic quality as open surgery, but is less invasive in patients. It is used widely in all of Europe, but with various frequency. The aim of the study was to present a various amount of oncourological procedures from three neighbouring countries – Poland, Czech Republic and Hungary. Prostatectomy, cystectomy, nephrectomy and tumorectomy (Nephron Sparing Procedures – NSS) were presented as a list of procedures prepared from the national registry. Material and methods The total amount of procedures was presented, as well as the LO (Lap to Open procedures) index, P/P (procedures/population) index, ratio of cystectomy/population, and cystectomy/TURBT. Results In the Czech Republic, the most complex procedures are performed (laparoscopic/robotic prostatectomy, NSS LAP, LAP nephrectomy) in the majority when analysing the country's population. In Hungary and Czech Republic, there are more laparoscopic/robotic radical prostatectomies performed, than open ones. In Poland the largest number of cystectomies is performed when analysing the country's population, but it is difficult to explain the much higher ratio of 6.57 TUR/one cystectomy. In the Czech Republic this procedure is performed in almost one quarter of the patients (23.36%). Interestingly, in Hungary the cystectomy with pouch creation is performed in about 67.65% cases. The highest reimbursement for surgical procedure is present in the Czech Republic with approximately 20–40% more than when compared to Poland or Hungary. Conclusions The definitive leader in Central Europe (based on the national registry) is the Czech Republic, where the most complex procedures are performed (laparoscopic/robotic prostatectomy, NSS LAP, LAP nephrectomy) in biggest amounts when analysing the country's population. Explanation of such circumstances, can be the higher

  19. One of a kind--the Pan African Clinical Trials Registry, a regional registry for Africa.

    PubMed

    Abrams, Amber L

    2011-01-01

    The 2004 Ministerial Summit on Health Research called on the World Health Organization to to establish a registry network with the intention of providing a single access point to identify trials. In 2007 the International Committee of Medical Journal Editors amended their support of this initiative stating that only trials registered prospectively on a member registry of the WHO's Network of Primary Registers would be published. The Pan African Clinical Trials Registry (www.pactr.org), was established in early 2007 as the AIDS, TB and Malaria (ATM) Clinical Trials Registry with the aim of piloting the concept of a registry that would cater to the specific needs of African trialists. In 2009 the ATM Registry expanded its remit to include all diseases for all regions of Africa; The Pan African Clinical Trials Registry became the first and is presently the only African member of the World Health Organization's Network of Primary Registers.

  20. Oral cancer in Libya and development of regional oral cancer registries: A review

    PubMed Central

    BenNasir, E.; El Mistiri, M.; McGowan, R.; Katz, R.V.

    2015-01-01

    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. PMID:26644751

  1. Considerations Before Establishing an Environmental Health Registry

    PubMed Central

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

    2016-01-01

    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

  2. Patient survival and safety with biologic therapy. Results of the Mexican National Registry Biobadamex 1.0.

    PubMed

    Ventura-Ríos, Lucio; Bañuelos-Ramírez, David; Hernández-Quiroz, María del Carmen; Robles-San Román, Manuel; Irazoque-Palazuelos, Fedra; Goycochea-Robles, María Victoria

    2012-01-01

    This work reports patient treatment survival and adverse events related to Biologic Therapy (BT), identified by a multicenter ambispective registry of 2047 rheumatic patients undergoing BT and including a control group of Rheumatoid Arthritis (RA) patients not using BT. The most common diagnoses were: RA 79.09%, Ankylosing Spondilytis 7.96%, Psoriatic Arthritis 4.40%, Systemic Lupus Erythematosus 3.37%, Juvenile Idiopathic Arthritis 1.17%. A secondary analysis included 1514 cases from the total sample and was performed calculating an incidence rate of any adverse events of 178 × 1000/BT patients per year vs 1009 × 1000/control group patients per year with a 1.6 RR (95% CI 1.4-1.9). For serious adverse events the RR was: 15.4 (95% CI 3.7-63.0, P<.0001). Global BT survival was 80% at 12 months, 61% at 24 months, 52% at 36 months and 45% at 48 months and SMR: 0.23 (95% CI 0.0-49.0) for BT vs 0.00 (95% CI 0.0-0.2) for the control group. In conclusion, BT was associated to a higher infection risk and adverse events, compared to other patients. Mortality using BT was not higher than expected for general population with same gender and age.

  3. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database.

    PubMed

    Fischbein, Rebecca; Saling, Julia R; Marty, Paige; Kropp, Denise; Meeker, James; Amerine, Jenna; Chyatte, Michelle Renee

    2015-09-01

    Chiari malformation (CM) is a condition in which cerebellar tonsillar ectopia may manifest with various clinical presentations. This study reports from the only national, online patient registry available, the symptoms, comorbid neurocognitive and psychological conditions, and diagnostic experiences of patients living with CM type I (CM I). The current research is one component of a large investigation designed to collect information from individuals with CM through the online Conquer Chiari Patient Registry questionnaire. Analyses included descriptive statistics to study body system impact and patient diagnostic experiences. Participants were 768 individuals with CM I and were predominantly female (86.8 %) and Caucasian (93.8 %) with an average age of 35 years. Pain was the most frequently reported symptom (76.69 %) experienced prior to diagnosis with headaches implicated most often (73.44 %). Neurocognitive comorbidities included memory difficulties (43.88 %) and aphasia (43.75 %) and psychological disorders such as depression (31.77 %) and anxiety disorders (19.92 %) were reported. Average time to diagnosis from first physician visit to diagnosis was 3.43 years, and only 8.46 % of patients had previous awareness of CM. CM I diagnosis was found incidentally for 24.87 % of participants. Common misdiagnoses were classified as psychological (19.26 %) and neurological (19.26 %). Fear was the most frequent emotion elicited at the time of correct diagnosis (42.19 %). CM I can be a challenging condition for patients and physicians, during both the search for diagnosis and management of symptoms. Patient and physician education about CM I may permit early intervention and the prevention of further deterioration and patient suffering.

  4. Bringing measurement and management science to the cath laboratory: the National Cardiovascular Data Registry (ACC-NCDR) and the Cardiac Catheterization Laboratory Continuous Quality Improvement Toolkit (ACC-CathKIT).

    PubMed

    Dehmer, Gregory J; Elma, MaryAnne; Hewitt, Kathleen; Brindis, Ralph G

    2004-01-01

    Diagnostic cardiac catheterization and percutaneous coronary interventions are widely performed for the evaluation and treatment of patients with cardiac disease. Because of high utilization, cost, and complication rates, invasive cardiac procedures are closely monitored and frequently measured using national benchmark databases and public reports. Before decision makers can accept these data and reports as accurate, it is necessary that the measurement process be performed correctly. However, collecting and measuring data is only the first step and does not automatically lead to improvements in quality. For an improvement to occur, a continuous quality improvement effort must exist to transform data into improved outcomes for patients. Recognizing the need to supply healthcare providers with methods and standards for measurement reporting and tools to assist facilities in the development of effective continuous quality improvement efforts, the American College of Cardiology developed the National Cardiovascular Data Registry (ACC-NCDR). Subsequently, the American College of Cardiology Foundation, in cooperation with the Society for Cardiovascular Angiography and Interventions, the American College of Cardiovascular Administrators, and several other professional organizations, developed the ACC-Cardiac Catheterization Laboratory Continuous Quality Improvement Toolkit (ACC-CathKIT). The development and usefulness of these products is described in this paper.

  5. Development of the SIOPE DIPG network, registry and imaging repository: a collaborative effort to optimize research into a rare and lethal disease.

    PubMed

    Veldhuijzen van Zanten, Sophie E M; Baugh, Joshua; Chaney, Brooklyn; De Jongh, Dennis; Sanchez Aliaga, Esther; Barkhof, Frederik; Noltes, Johan; De Wolf, Ruben; Van Dijk, Jet; Cannarozzo, Antonio; Damen-Korbijn, Carin M; Lieverst, Jan A; Colditz, Niclas; Hoffmann, Marion; Warmuth-Metz, Monika; Bison, Brigitte; Jones, David T W; Sturm, Dominik; Gielen, Gerrit H; Jones, Chris; Hulleman, Esther; Calmon, Raphael; Castel, David; Varlet, Pascale; Giraud, Géraldine; Slavc, Irene; Van Gool, Stefaan; Jacobs, Sandra; Jadrijevic-Cvrlje, Filip; Sumerauer, David; Nysom, Karsten; Pentikainen, Virve; Kivivuori, Sanna-Maria; Leblond, Pierre; Entz-Werle, Natasha; von Bueren, Andre O; Kattamis, Antonis; Hargrave, Darren R; Hauser, Péter; Garami, Miklos; Thorarinsdottir, Halldora K; Pears, Jane; Gandola, Lorenza; Rutkauskiene, Giedre; Janssens, Geert O; Torsvik, Ingrid K; Perek-Polnik, Marta; Gil-da-Costa, Maria J; Zheludkova, Olga; Shats, Liudmila; Deak, Ladislav; Kitanovski, Lidija; Cruz, Ofelia; Morales La Madrid, Andres; Holm, Stefan; Gerber, Nicolas; Kebudi, Rejin; Grundy, Richard; Lopez-Aguilar, Enrique; Zapata-Tarres, Marta; Emmerik, John; Hayden, Tim; Bailey, Simon; Biassoni, Veronica; Massimino, Maura; Grill, Jacques; Vandertop, William P; Kaspers, Gertjan J L; Fouladi, Maryam; Kramm, Christof M; van Vuurden, Dannis G

    2017-01-21

    Diffuse intrinsic pontine glioma (DIPG) is a rare and deadly childhood malignancy. After 40 years of mostly single-center, often non-randomized trials with variable patient inclusions, there has been no improvement in survival. It is therefore time for international collaboration in DIPG research, to provide new hope for children, parents and medical professionals fighting DIPG. In a first step towards collaboration, in 2011, a network of biologists and clinicians working in the field of DIPG was established within the European Society for Paediatric Oncology (SIOPE) Brain Tumour Group: the SIOPE DIPG Network. By bringing together biomedical professionals and parents as patient representatives, several collaborative DIPG-related projects have been realized. With help from experts in the fields of information technology, and legal advisors, an international, web-based comprehensive database was developed, The SIOPE DIPG Registry and Imaging Repository, to centrally collect data of DIPG patients. As for April 2016, clinical data as well as MR-scans of 694 patients have been entered into the SIOPE DIPG Registry/Imaging Repository. The median progression free survival is 6.0 months (95% Confidence Interval (CI) 5.6-6.4 months) and the median overall survival is 11.0 months (95% CI 10.5-11.5 months). At two and five years post-diagnosis, 10 and 2% of patients are alive, respectively. The establishment of the SIOPE DIPG Network and SIOPE DIPG Registry means a paradigm shift towards collaborative research into DIPG. This is seen as an essential first step towards understanding the disease, improving care and (ultimately) cure for children with DIPG.

  6. The FReedom from Ischemic Events - New Dimensions for Survival (FRIENDS) registry: design of a prospective cohort study of patients with advanced peripheral artery disease

    PubMed Central

    2013-01-01

    Background Advanced lower extremity peripheral artery disease (PAD), whether presenting as acute limb ischemia (ALI) or chronic critical limb ischemia (CLI), is associated with high rates of cardiovascular ischemic events, amputation, and death. Past research has focused on strategies of revascularization, but few data are available that prospectively evaluate the impact of key process of care factors (spanning pre-admission, acute hospitalization, and post-discharge) that might contribute to improving short and long-term health outcomes. Methods/Design The FRIENDS registry is designed to prospectively evaluate a range of patient and health system care delivery factors that might serve as future targets for efforts to improve limb and systemic outcomes for patients with ALI or CLI. This hypothesis-driven registry was designed to evaluate the contributions of: (i) pre-hospital limb ischemia symptom duration, (ii) use of leg revascularization strategies, and (iii) use of risk-reduction pharmacotherapies, as pre-specified factors that may affect amputation-free survival. Sequential patients would be included at an index “vascular specialist-defined” ALI or CLI episode, and patients excluded only for non-vascular etiologies of limb threat. Data including baseline demographics, functional status, co-morbidities, pre-hospital time segments, and use of medical therapies; hospital-based use of revascularization strategies, time segments, and pharmacotherapies; and rates of systemic ischemic events (e.g., myocardial infarction, stroke, hospitalization, and death) and limb ischemic events (e.g., hospitalization for revascularization or amputation) will be recorded during a minimum of one year follow-up. Discussion The FRIENDS registry is designed to evaluate the potential impact of key factors that may contribute to adverse outcomes for patients with ALI or CLI. Definition of new “health system-based” therapeutic targets could then become the focus of future

  7. Patient satisfaction with total knee replacement cannot be predicted from pre-operative variables alone: A cohort study from the National Joint Registry for England and Wales.

    PubMed

    Baker, P N; Rushton, S; Jameson, S S; Reed, M; Gregg, P; Deehan, D J

    2013-10-01

    Pre-operative variables are increasingly being used to determine eligibility for total knee replacement (TKR). This study was undertaken to evaluate the relationships, interactions and predictive capacity of variables available pre- and post-operatively on patient satisfaction following TKR. Using nationally collected patient reported outcome measures and data from the National Joint Registry for England and Wales, we identified 22 798 patients who underwent TKR for osteoarthritis between August 2008 and September 2010. The ability of specific covariates to predict satisfaction was assessed using ordinal logistic regression and structural equational modelling. Only 4959 (22%) of 22 278 patients rated the results of their TKR as 'excellent', despite the majority (71%, n = 15 882) perceiving their knee symptoms to be much improved. The strongest predictors of satisfaction were post-operative variables. Satisfaction was significantly and positively related to the perception of symptom improvement (operative success) and the post-operative EuroQol-5D score. While also significant within the models pre-operative variables were less important and had a minimal influence upon post-operative satisfaction. The most robust predictions of satisfaction occurred only when both pre- and post-operative variables were considered together. These findings question the appropriateness of restricting access to care based on arbitrary pre-operative thresholds as these factors have little bearing on post-operative satisfaction.

  8. Late-onset neutropenia after treatment with rituximab for rheumatoid arthritis and other autoimmune diseases: data from the AutoImmunity and Rituximab registry

    PubMed Central

    Salmon, J H; Cacoub, P; Combe, B; Sibilia, J; Pallot-Prades, B; Fain, O; Cantagrel, A; Dougados, M; Andres, E; Meyer, O; Carli, P; Pertuiset, E; Pane, I; Maurier, F; Ravaud, P; Mariette, X; Gottenberg, J E

    2015-01-01

    Objectives To evaluate the prevalence of late-onset neutropenia and its complications in patients treated with rituximab (RTX) for rheumatoid arthritis (RA) and other autoimmune diseases (AIDs) in a prospective registry. Methods The AutoImmunity and Rituximab registry is an independent 7-year prospective registry promoted by the French Society of Rheumatology. For each episode of neutropenia, data were validated by the clinician in charge of the patient. Results Among 2624 patients treated with RTX for refractory AIDs, and at least 1 follow-up visit (a total follow-up of 4179 patient-years in RA and 987 patient-years in AIDs), late-onset neutropenia was observed in 40 patients (25 RA (1.3% of patients with RA, 0.6/100 patient-years), and AIDs in 15 (2.3% of patients with AIDs, 1.5/100 patient-years)). 6 patients (15%) had neutrophils <500/mm3, 8 (20%) had neutrophils between 500 and 1000/mm3, and 26 (65%) had neutrophils between 1000 and 1500/mm3. Neutropenia occurred after a median period of 4.5 (3–6.5) months after the last RTX infusion in patients with RA, and 5 (3–6.5) months in patients with AIDs. 5 patients (12.5%), 4 of them with neutrophils lower than 500/mm3, developed a non-opportunistic serious infection and required antibiotics and granulocyte colony-stimulating factor injections, with a favourable outcome. After resolution of their RTX-related neutropenia, 19 patients (47.5%) were re-treated, and neutropenia reoccurred in 3 of them. Conclusions Late-onset neutropenia might occur after RTX and may result in serious infections. Thus, monitoring of white cell count should be performed after RTX. However, in this large registry of patients with AIDs, the frequency of RTX-induced neutropenia was much lower than that previously reported in patients treated for blood malignancies or AIDs. PMID:26509060

  9. Retrivability in The Danish National Hospital Registry of HIV and hepatitis B and C coinfection diagnoses of patients managed in HIV centers 1995–2004

    PubMed Central

    Obel, Niels; Reinholdt, Hanne; Omland, Lars H; Engsig, Frederik; Sørensen, Henrik T; Hansen, Ann-Brit E

    2008-01-01

    Background Hospital-based discharge registries are used increasingly for longitudinal epidemiological studies of HIV. We examined completeness of registration of HIV infections and of chronic hepatitis B (HBV) and hepatitis C (HCV) coinfections in the Danish National Hospital Registry (DNHR) covering all Danish hospitals. Methods The Danish HIV Cohort Study (DHCS) encompasses all HIV-infected patients treated in Danish HIV clinics since 1 January 1995. All 2,033 Danish patients in DHCS diagnosed with HIV-1 during the 10-year period from 1 January 1995 to 31 December 2004 were included in the current analysis. We used the DHCS as a reference to examine the completeness of HIV and of HBV and HCV coinfections recorded in DNHR. Cox regression analysis was used to estimate hazard ratios of time to diagnosis of HIV in DNHR compared to DHCS. Results Of the 2,033 HIV patients in DHCS, a total of 2,006 (99%) were registered with HIV in DNHR. Of these, 1,888 (93%) were registered in DNHR within one year of their first positive HIV test. A CD4 < 200 cells/μl, a viral load >= 100,000 copies/ml and being diagnosed after 1 January 2000, were associated with earlier registration in DNHR, both in crude and adjusted analyses. Thirty (23%) HIV patients registered with chronic HBV (n = 129) in DHCS and 126 (48%) of HIV patients with HCV (n = 264) in DHCS were registered with these diagnoses in the DNHR. Further 17 and 8 patients were registered with HBV and HCV respectively in DNHR, but not in DHCS. The positive predictive values of being registered with HBV and HCV in DHCS were thereby estimated to 0.88 and 0.97 and in DNHR to 0.32 and 0.54. Conclusion The study demonstrates that secondary data from national hospital databases may be reliable for identification of patients diagnosed with HIV infection. However, the predictive value of co-morbidity data may be low. PMID:18439245

  10. Validation of primary metal-on-metal hip arthroplasties on the National Joint Registry for England, Wales and Northern Ireland using data from the London Implant Retrieval Centre: a study using the NJR dataset.

    PubMed

    Sabah, S A; Henckel, J; Cook, E; Whittaker, R; Hothi, H; Pappas, Y; Blunn, G; Skinner, J A; Hart, A J

    2015-01-01

    Arthroplasty registries are important for the surveillance of joint replacements and the evaluation of outcome. Independent validation of registry data ensures high quality. The ability for orthopaedic implant retrieval centres to validate registry data is not known. We analysed data from the National Joint Registry for England, Wales and Northern Ireland (NJR) for primary metal-on-metal hip arthroplasties performed between 2003 and 2013. Records were linked to the London Implant Retrieval Centre (RC) for validation. A total of 67,045 procedures on the NJR and 782 revised pairs of components from the RC were included. We were able to link 476 procedures (60.9%) recorded with the RC to the NJR successfully. However, 306 procedures (39.1%) could not be linked. The outcome recorded by the NJR (as either revised, unrevised or death) for a primary procedure was incorrect in 79 linked cases (16.6%). The rate of registry-retrieval linkage and correct assignment of outcome code improved over time. The rates of error for component reference numbers on the NJR were as follows: femoral head category number 14/229 (5.0%); femoral head batch number 13/232 (5.3%); acetabular component category number 2/293 (0.7%) and acetabular component batch number 24/347 (6.5%). Registry-retrieval linkage provided a novel means for the validation of data, particularly for component fields. This study suggests that NJR reports may underestimate rates of revision for many types of metal-on-metal hip replacement. This is topical given the increasing scope for NJR data. We recommend a system for continuous independent evaluation of the quality and validity of NJR data.

  11. National policies and strategies for noncommunicable diseases.

    PubMed

    Mendis, Shanthi; Fuster, Valentin

    2009-11-01

    Good public policies are vital for protecting the health of populations. For the prevention and control of noncommunicable diseases (NCD), a single national policy is essential. Such a policy ensures clarity of vision and purpose, sets the platform for joint action to address risk factors and determinants that are shared by major NCDs, and provides coherence with other health and social policies. A national NCD policy also provides an overarching framework that enables governments to combine and balance synergistic public health strategies that target the whole population and those that target high-risk groups. Given that the adopted strategies are evidence-based and cost-effective, national NCD policy development can provide a strong basis for appropriate legislation and regulation in relation to tobacco control and promotion of healthy diet and physical activity. A national policy also serves as a vehicle to facilitate efficient and effective use of available public finances for NCD-related health care, while safeguarding equity. For the reasons alluded to above, a single national NCD policy is more appropriate than separate national polices for each individual NCD, particularly in low-income and middle-income countries that have a critical shortage of human and financial resources, including those necessary for policy development and implementation.

  12. United States Transuranium and Uranium Registries

    SciTech Connect

    Kathren, R.

    1993-02-28

    The United States Transuranium and Uranium Registries are unique human tissue research programs studying the distribution, dose, and possible biological effects of the actinide elements in man, with the primary goal of assuring the adequacy of radiation protection standards for these radionuclides. The Registries research is based on radiochemical analysis of tissues collected at autopsy from voluntary donors who have documented occupational exposure to the actinides. To date, tissues, or in some cases radioanalytical results only, have been obtained from approximately 300 individuals; another 464 living individuals have volunteered to participate in the Registries research programs and have signed premortem informed consent and autopsy permissions. The Registries originated at the National Plutonium Registry which was started in 1968 as a then Atomic Energy Commission project under the aegis of a prime contractor at the Hanford site. In 1970, the name was changed to the United States Transuranium Registry to reflect a broader involvement with the higher actinides. In 1978, an administratively separate parallel registry, the United States Uranium Registry, was formed to carry out similar studies among uranium fuel cycle workers.

  13. Rare diagnosis of IgG4-related systemic disease by lip biopsy in an international Sjögren syndrome registry.

    PubMed

    Baer, Alan N; Gourin, Christine G; Westra, William H; Cox, Darren P; Greenspan, John S; Daniels, Troy E

    2013-03-01

    IgG4-related disease has been recently defined as a distinct clinic-pathologic entity, characterized by dense IgG-4 plasmacytic infiltration of diverse organs, fibrosis, and tumefactive lesions. Salivary and lacrimal glands are a target of this disease and, when affected, may clinically resemble Küttner tumor, Mikulicz disease, or orbital inflammatory pseudotumor. In some patients, the disease is systemic, with metachronous involvement of multiple organs, including the pancreas, aorta, kidneys, and biliary tract. We report a 66-year-old man who presented with salivary gland enlargement and severe salivary hypofunction and was diagnosed with IgG4-related disease on the basis of a labial salivary gland biopsy. Additional features of his illness included a marked peripheral eosinophilia, obstructive pulmonary disease, and lymphoplasmacytic aortitis. He was evaluated in the context of a research registry for Sjögren syndrome and was the only 1 of 2594 registrants with minor salivary gland histopathologic findings supportive of this diagnosis.

  14. 75 FR 62406 - Plan To Develop a Genetic Testing Registry at the National Institutes of Health; Public Meeting...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-08

    ... 301-496-9839; or via postal service to Cathy Fomous, Ph.D., Office of Biotechnology Activities... meeting agenda and public comments, please contact Cathy Fomous, Ph.D., NIH Office of Biotechnology... Biotechnology Information (NCBI), part of the National Library of Medicine at NIH, is responsible for...

  15. Distinct risk factors of atrial fibrillation in patients with and without coronary artery disease: a cross-sectional analysis of the BOREAS-CAG Registry data

    PubMed Central

    Murakami, Naoto; Tanno, Masaya; Kokubu, Nobuaki; Nishida, Junichi; Nagano, Nobutaka; Ohnishi, Hirofumi; Akasaka, Hiroshi; Miki, Takayuki; Tsuchihashi, Kazufumi; Miura, Tetsuji

    2017-01-01

    Objective Although risk factors of atrial fibrillation (AF) in the general population have been characterised, their impacts on patients with specific diseases are unclear. Our aim was to determine whether risk factors of AF are different in patients with and those without coronary artery disease (CAD). Methods We enrolled 1871 consecutive patients who underwent coronary angiography for evaluation of symptoms suggestive of CAD in the BOREAS-CAG Registry between August 2014 and January 2015. After exclusion of patients with valvular heart disease or a history of PCI/cardiac surgery, 1150 patients contributed to multivariate logistic regression analysis to identify risk factors of AF. We also retrieved data for 361 consecutive patients with CAD admitted to Sapporo Medical University Hospital between April 2013 and July 2014 and analysed data for 166 patients using the same inclusion and exclusion criteria as those in the BOREAS-CAG Registry. Results Unexpectedly, CAD was independently associated with the absence of AF. The patients were then divided into a non-CAD group (n=576) and a CAD group (n=574) for further analysis. The brain natriuretic peptide level showed a strong association with AF regardless of the presence or absence of CAD. In the non-CAD group, lack of statin use was independently associated with AF, whereas high serum uric acid level was an independent explanatory variable of AF in the CAD group. The association of AF with uric acid was confirmed in a separate group of patients (n=166) enrolled in the CAD cohort in Sapporo Medical University Hospital. Conclusions Major risk factors of AF are different in patients with CAD and those without CAD. Patients with CAD are more likely to develop AF when the serum uric acid level is high, whereas no statin administration predicts development of AF in patients without CAD. PMID:28123767

  16. Database and Registry Research in Orthopaedic Surgery: Part 2: Clinical Registry Data.

    PubMed

    Pugely, Andrew J; Martin, Christopher T; Harwood, Jared; Ong, Kevin L; Bozic, Kevin J; Callaghan, John J

    2015-11-04

    The use of large-scale national databases for observational research in orthopaedic surgery has grown substantially in the last decade, and the data sets can be categorized as either administrative claims or clinical registries. Clinical registries contain secondary data on patients with a specific diagnosis or procedure. The data are typically used for patient outcome surveillance to improve patient safety and health-care quality. Registries used in orthopaedic research exist at the regional, national, and international levels, and many were designed to specifically collect outcomes relevant to orthopaedics, such as short-term surgical complications, longer-term outcomes (implant survival or reoperations), and patient-reported outcomes. Although heterogeneous, clinical registries-in contrast to claims data-typically have a more robust list of variables, with relatively precise prospective data input, management infrastructure, and reporting systems. Some weaknesses of clinical registries include a smaller number of patients, inconstant follow-up duration, and use of sampling methods that may limit generalizability. Within the U.S., national joint registry adoption has lagged international joint registries. Given the changing health-care environment, it is likely that clinical registries will provide valuable information that has the potential to influence clinical practice improvement and health-care policy in the future.

  17. Patient profile and periprocedural outcomes of bioresorbable vascular scaffold implantation in comparison with drug-eluting and bare-metal stent implantation. Experience from ORPKI Polish National Registry 2014–2015

    PubMed Central

    Rzeszutko, Łukasz; Tokarek, Tomasz; Siudak, Zbigniew; Dziewierz, Artur; Żmudka, Krzysztof

    2016-01-01

    Introduction There are limited data on the comparison of bioresorbable vascular scaffold (BVS) and drug-eluting stent (DES)/bare-metal stent (BMS) implantation in an unselected population of patients with coronary artery disease. Aim To compare the periprocedural outcomes and patient profile of BVS and DES/BMS implantation in an all-comer population from the ORPKI Polish National Registry. Material and methods A total of 141,324 consecutive patients from 151 invasive cardiology centers in Poland were included in this prospective registry between January 2014 and June 2015. Periprocedural data on patients with at least one BVS (Absorb, Abbott Vascular, Santa Clara, CA, USA), DES or BMS (all available types) implantation in de novo lesions during index percutaneous coronary intervention for stable angina (SA) or acute coronary syndrome were collected. Results Bioresorbable vascular scaffold was the most often used in patients with SA, in single-vessel disease and in younger male patients. Bioresorbable vascular scaffold implantation was significantly more often associated with periprocedural administration of ticagrelor/prasugrel (6.8% vs. 3.6%; p = 0.001) and use of intravascular ultrasound and optical coherence tomography in comparison with the DES/BMS group (2.8% vs. 0.6% and 1.8% vs. 0.1%, respectively; p = 0.001 for both). The incidence of periprocedural death was significantly lower in the BVS group than the DES/BMS group (0.04% vs. 0.32%; p = 0.02), but this difference was no longer significant after adjustment for covariates. On the other hand, coronary artery perforation occurred significantly more often during BVS delivery (0.31% vs. 0.12%; p = 0.01), and BVS implantation was identified as an independent predictor of coronary artery perforation in multivariate logistic regression analysis (OR = 6.728, 95% CI: 2.394–18.906; p = 0.001). Conclusions Patients treated with BVS implantation presented an acceptable safety and efficacy profile in comparison with

  18. Factors influencing revision risk following 15 740 single-brand hybrid hip arthroplasties: a cohort study from a National Joint Registry.

    PubMed

    Jameson, Simon S; Mason, James M; Baker, Paul N; Jettoo, Prithee; Deehan, David J; Reed, Mike R

    2013-08-01

    This retrospective cohort study of a National Joint Registry data examines survival time to revision following the commonest brand of primary hybrid THA, exploring risk factors independently associated with failure. Overall 5-year revision was 1.56%. In the final adjusted model, revision risk was significantly higher with standard polyethylene (PE) liners (metal-on-PE: hazard ratio [HR]=2.52, P=0.005, ceramic-on-PE: HR=2.99, P=0.025) when compared to metal-on-highly-cross-linked (XL) PE. Risk of revision with ceramic-on-ceramic bearings was borderline significant (HR=1.86, P=0.061). A significant interaction between age and acetabular shell type (solid or multi-hole) was found (P=0.022), suggesting that solid shells performed significantly better in younger patients. In summary, we found that there were significant differences in implant failure between different bearing surfaces and shell types after adjusting for a range of covariates.

  19. 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.

  20. Treatment and Survival of Medicare Beneficiaries with Colorectal Cancer: A Comparative Analysis Between a Rural State Cancer Registry and National Data.

    PubMed

    Rane, Pallavi B; Madhavan, S Suresh; Sambamoorthi, Usha; Sita, Kalidindi; Kurian, Sobha; Pan, Xiaoyun

    2017-02-01

    The aim was to examine and compare with "national" estimates, receipt of colorectal cancer (CRC) treatment in the initial phase of care and survival following a CRC diagnosis in rural Medicare beneficiaries. A retrospective study was conducted on fee-for-service Medicare beneficiaries diagnosed with CRC in 2003-2006, identified from West Virginia Cancer Registry (WVCR)-Medicare linked database (N = 2119). A comparative cohort was identified from Surveillance, Epidemiology, and End Results (SEER)-Medicare (N = 38,168). CRC treatment received was ascertained from beneficiaries' Medicare claims in the 12 months post CRC diagnosis or until death, whichever happened first. Receipt of minimally appropriate CRC treatment (MACT) was defined using recommended CRC treatment guidelines. All-cause and CRC-specific mortality in the 36-month period post CRC diagnosis were examined. Differences in usage of CRC surgery, chemotherapy, and radiation were observed between the 2 populations, with those from WVCR-Medicare being less likely to receive any type of CRC surgery (adjusted odds ratio [AOR] = 0.82; 95% confidence interval [CI] = [0.73-0.93]). Overall, those from WVCR-Medicare had a lower likelihood of receiving MACT, (AOR = 0.85; 95% CI = [0.76-0.96]) compared to their national counterparts. Higher hazard of CRC mortality was observed in the WVCR-Medicare cohort (adjusted hazard ratio = 1.26; 95% CI = [1.20-1.32]) compared to the SEER-Medicare cohort. Although more beneficiaries from WVCR-Medicare were diagnosed in early-stage CRC compared to their SEER-Medicare counterparts, they had a lower likelihood of receiving MACT and a higher hazard of CRC mortality. This study highlights the need for an increased focus on improving access to care at every phase of the CRC care continuum, especially for those from rural settings.

  1. The nationalization of a disease: a paradigm?

    PubMed Central

    Soviero, D J

    1986-01-01

    The early history of the Federal involvement in Hansen's Disease reflects the history of the Public Health Service itself. As a young and aggressive institution, the Public Health Service sought out contagious, infectious diseases that threatened the public health. National resources and national coordination were needed to fight the likes of malaria, hookworm, or smallpox. The customary attack would consist of a field study, determination of the etiology, the method of transmission, and, then, perhaps, preventive measures. An eradication campaign would follow. Leprosy fit perfectly into the model--a disease of unknown etiology, an unknown method of transmission, thought to be highly contagious, and no known cure. The United States launched a major investigation in Hawaii, where the disease was prevalent and its victims conveniently segregated. The investigation failed. The Public Health Service then turned toward segregation and isolation as a way to fulfill its public health role. A bureaucracy was established around the idea that victims of leprosy must be incarcerated for the good of the public. The institutionalization of the Public Health Service and the philosophy upon which its treatment of leprosy was based proved difficult to change when researchers in the field made major scientific breakthroughs in the 1940s. The realization that the disease was only feebly contagious, activities of patient organizations, and pressure from the media and the Congress did not achieve as dramatic results as the sulfone drugs did. The Public Health Service moved, but slowly. What are the lessons in all of this?. Images p401-a p402-a p403-a PMID:3090606

  2. Latin American Dialysis and Transplant Registry: 2008 prevalence and incidence of end-stage renal disease and correlation with socioeconomic indexes.

    PubMed

    Cusumano, Ana M; Garcia-Garcia, Guillermo; Gonzalez-Bedat, Maria C; Marinovich, Sergio; Lugon, Jocemir; Poblete-Badal, Hugo; Elgueta, Susana; Gomez, Rafael; Hernandez-Fonseca, Fabio; Almaguer, Miguel; Rodriguez-Manzano, Sandra; Freire, Nelly; Luna-Guerra, Jorge; Rodriguez, Gaspar; Bochicchio, Tommaso; Cuero, Cesar; Cuevas, Dario; Pereda, Carlos; Carlini, Raul

    2013-05-01

    In 2008, 563,294,000 people were living in Latin America (LA), of which 6.6% were older than 65. The region is going through a fast demographic and epidemiologic transition process, in the context of an improvement in socio-economic indices. The Latin American Dialysis and Renal Transplant Registry has collected data since 1991, through an annual survey completed by 20 affiliated National Societies. Renal replacement treatment (RRT) prevalence and incidence showed an increase year by year. The prevalence rate (in all modalities) correlated with the World Bank country classification by income and the epidemiologic transition stage the countries were experiencing. RRT prevalence and kidney transplantation rates correlated significantly with gross national income (GNI), health expenditure in constant dollars (HeExp), % older than 65, life expectancy at birth, and % of the population living in urban settings. Kidney transplantation increased also, year by year, with more than 50% of transplants performed using kidneys from deceased donors. Double transplants were performed in six countries. RRT prevalence and incidence increased in LA, and are associated with indexes reflecting higher and more evenly distributed national wealth (GNI and HeExp), and the stage of demographic and epidemiological transition.

  3. Latin American Dialysis and Transplant Registry: 2008 prevalence and incidence of end-stage renal disease and correlation with socioeconomic indexes

    PubMed Central

    Cusumano, Ana M; Garcia-Garcia, Guillermo; Gonzalez-Bedat, Maria C; Marinovich, Sergio; Lugon, Jocemir; Poblete-Badal, Hugo; Elgueta, Susana; Gomez, Rafael; Hernandez-Fonseca, Fabio; Almaguer, Miguel; Rodriguez-Manzano, Sandra; Freire, Nelly; Luna-Guerra, Jorge; Rodriguez, Gaspar; Bochicchio, Tommaso; Cuero, Cesar; Cuevas, Dario; Pereda, Carlos; Carlini, Raul

    2013-01-01

    In 2008, 563,294,000 people were living in Latin America (LA), of which 6.6% were older than 65. The region is going through a fast demographic and epidemiologic transition process, in the context of an improvement in socio-economic indices. The Latin American Dialysis and Renal Transplant Registry has collected data since 1991, through an annual survey completed by 20 affiliated National Societies. Renal replacement treatment (RRT) prevalence and incidence showed an increase year by year. The prevalence rate (in all modalities) correlated with the World Bank country classification by income and the epidemiologic transition stage the countries were experiencing. RRT prevalence and kidney transplantation rates correlated significantly with gross national income (GNI), health expenditure in constant dollars (HeExp), % older than 65, life expectancy at birth, and % of the population living in urban settings. Kidney transplantation increased also, year by year, with more than 50% of transplants performed using kidneys from deceased donors. Double transplants were performed in six countries. RRT prevalence and incidence increased in LA, and are associated with indexes reflecting higher and more evenly distributed national wealth (GNI and HeExp), and the stage of demographic and epidemiological transition. PMID:25018980

  4. 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

  5. Hemiarthroplasties after hip fractures in Norway and Sweden: a collaboration between the Norwegian and Swedish national registries.

    PubMed

    Gjertsen, Jan-Erik; Fenstad, Anne Marie; Leonardsson, Olof; Engesæter, Lars Birger; Kärrholm, Johan; Furnes, Ove; Garellick, Göran; Rogmark, Cecilia

    2014-01-01

    National registration of hemiarthroplasties after hip fractures has been established in both Norway and Sweden. We aimed to investigate differences in demographics, choice of implant selection, surgical approaches, and reoperations between the Norwegian Hip Fracture Register (NHFR) and the Swedish Hip Arthroplasty Register (SHAR). As part of the Nordic Arthroplasty Register Association (NARA) project a common hemiarthroplasty dataset has been established. 36,989 primary hemiarthroplasties (HAs) for acute hip fractures reported to NHFR (n = 12,761) and SHAR (n = 24,228) for the period 2005-2010 were included. Cemented prostheses were used in 78% of the operations in Norway and in 95% of the patients in Sweden. In Norway HAs almost exclusively had bipolar design (98%), whereas in Sweden HAs with unipolar design were used in 42% of the cases. Monoblock (non-modular) prostheses were uncommon, but still more frequently used in Sweden than in Norway (6.9% and 2.1% respectively). The lateral approach was more common in Norway (83%) than in Sweden (52%), where the posterior approach was used in 42% of the cases. The five-year survival of all HAs was 95.5% (95% CI: 94.8-96.2) in Norway and 94.8% (95% CI: 94.4-95.3) in Sweden. We concluded that surprisingly large differences between the two countries in demographics, implant design, and surgical technique had been revealed. This common dataset enables further investigations of the impact of these differences on revision rates and mortality.

  6. Adherence to guidelines: A national audit of the management of acute upper gastrointestinal bleeding. The REASON registry

    PubMed Central

    Lu, Yidan; Barkun, Alan N; Martel, Myriam

    2014-01-01

    OBJECTIVES: To assess process of care in nonvariceal upper gastrointestinal bleeding (NVUGIB) using a national cohort, and to identify predictors of adherence to ‘best practice’ standards. METHODS: Consecutive charts of patients hospitalized for acute upper gastrointestinal bleeding across 21 Canadian hospitals were reviewed. Data regarding initial presentation, endoscopic management and outcomes were collected. Results were compared with ‘best practice’ using established guidelines on NVUGIB. Adherence was quantified and independent predictors were evaluated using multivariable analysis. RESULTS: Overall, 2020 patients (89.4% NVUGIB, variceal in 10.6%) were included (mean [± SD] age 66.3±16.4 years; 38.4% female). Endoscopy was performed in 1612 patients: 1533 with NVUGIB had endoscopic lesions (63.1% ulcers; high-risk stigmata in 47.8%). Early endoscopy was performed in 65.6% and an assistant was present in 83.5%. Only 64.5% of patients with high-risk stigmata received endoscopic hemostasis; 9.8% of patients exhibiting low-risk stigmata also did. Intravenous proton pump inhibitor was administered after endoscopic hemostasis in 95.7%. Rebleeding and mortality rates were 10.5% and 9.4%, respectively. Multivariable analysis revealed that low American Society of Anesthesiologists score patients had fewer assistants present during endoscopy (OR 0.63 [95% CI 0.48 to 0.83), a hemoglobin level <70 g/L predicted inappropriate high-dose intravenous proton pump inhibitor use in patients with low-risk stigmata, and endoscopies performed during regular hours were associated with longer delays from presentation (OR 0.33 [95% CI 0.24 to 0.47]). CONCLUSION: There was variability between the process of care and ‘best practice’ in NVUGIB. Certain patient and situational characteristics may influence guideline adherence. Dissemination initiatives must identify and focus on such considerations to improve quality of care. PMID:25314356

  7. Kidney and liver transplantation in children with fibrocystic liver-kidney disease: data from the US Scientific Registry of Transplant Recipients: 1990-2010.

    PubMed

    Wen, Jessica W; Furth, Susan L; Ruebner, Rebecca L

    2014-11-01

    The natural history and survival of children with fibrocystic liver-kidney disease undergoing solid organ transplantation have infrequently been described. We report outcomes in a cohort of US children with fibrocystic liver-kidney disease receiving solid organ transplants over 20 yr. Retrospective cohort study of pediatric transplant recipients with diagnoses of fibrocystic liver-kidney disease from 1/1990 to 3/2010, using data from the SRTR. Subjects were categorized by the first transplanted organ: LT, KT, or SLK. Primary outcomes were death, re-transplant, transplant of the alternate organ, or initiation of dialysis. Seven hundred and sixteen subjects were transplanted in this period. Median age at first transplant was 9.7 yr. Of the LT, 14 (19%) required a second liver transplant at median of 0.2 yr, and five (7%) required kidney transplant or dialysis at a median of 9.0 yr. Of the KT, 188 (31%) required a second kidney transplant or dialysis at a median of 5.9 yr. Twenty-nine (5%) subsequently received liver transplant at a median of 6.0 yr. Among patients in this registry, far more children underwent kidney than liver transplants. The risk of subsequently needing transplantation of an alternate organ was low.

  8. Rising inequality in mortality among working-age men and women in Sweden: a national registry-based repeated cohort study, 1990–2007

    PubMed Central

    Kondo, Naoki; Rostila, Mikael; Yngwe, Monica Åberg

    2014-01-01

    Background In the past two decades, health inequality has persisted or increased in states with comprehensive welfare. Methods We conducted a national registry-based repeated cohort study with a 3-year follow-up between 1990 and 2007 in Sweden. Information on all-cause mortality in all working-age Swedish men and women aged between 30 and 64 years was collected. Data were subjected to temporal trend analysis using joinpoint regression to statistically confirm the trajectories observed. Results Among men, age-standardised mortality rate decreased by 38.3% from 234.9 to 145 (per 100 000 population) over the whole period in the highest income quintile, whereas the reduction was only 18.3% (from 774.5 to 632.5) in the lowest quintile. Among women, mortality decreased by 40% (from 187.4 to 112.5) in the highest income group, but increased by 12.1% (from 280.2 to 314.2) in the poorest income group. Joinpoint regression identified that the differences in age-standardised mortality between the highest and the lowest income quintiles decreased among men by 18.85 annually between 1990 and 1994 (p trend=0.02), whereas it increased later, with a 2.88 point increase per year (p trend <0.0001). Among women, it continuously increased by 9.26/year (p trend <0.0001). In relative terms, age-adjusted mortality rate ratios showed a continuous increase in both genders. Conclusions Income-based inequalities among working-age male and female Swedes have increased since the late 1990s, whereas in absolute terms the increase was less remarkable among men. Structural and behavioural factors explaining this trend, such as the economic recession in the early 1990s, should be studied further. PMID:25143429

  9. Ethnic differences in the presentation, treatment strategy, and outcomes of percutaneous coronary intervention (a report from the National Heart, Lung, and Blood Institute Dynamic Registry).

    PubMed

    Slater, James; Selzer, Faith; Dorbala, Sharmila; Tormey, Deborah; Vlachos, Helen A; Wilensky, Robert L; Jacobs, Alice K; Laskey, Warren K; Douglas, John S; Williams, David O; Kelsey, Sheryl F

    2003-10-01

    Information about the impact of race/ethnicity on adverse outcomes after percutaneous coronary intervention (PCI) in the modern era is limited. Using consecutive patients from the National Heart, Lung, and Blood Institute Dynamic Registry, this study investigated differences in clinical presentation, treatment strategy, and acute and long-term outcomes in 3,669 white, 446 black, 301 Hispanic, and 201 Asian patients who underwent PCI. All comparisons were made to whites. Blacks were more likely than whites to be younger, women, and to present with a higher prevalence of cardiovascular risk factors (hypertension, diabetes, and smoking). Hispanics tended to be younger, hypertensive, diabetic, and to be undergoing their first cardiovascular procedure. Asians were, on average, younger, men, and presented more often with hypertension and diabetes than whites. Although the rate of stent implantation was significantly lower in blacks compared with whites (63% vs 74%, p <0.001), angiographic and procedural success rates were high (> or =95%) and did not differ by race/ethnicity. In-hospital mortality (0.2% vs 1.7%, p <0.05) and death/myocardial infarction (MI)/coronary artery bypass grafting (CABG) (3.1% vs 5.5%, p <0.05) were lower in blacks. All other in-hospital complications were similar to whites. At 1 year, there were no statistical differences in cumulative adverse event rates by ethnicity; however by 2 years there was a modestly higher mortality rate (adjusted RR 1.87; 95% confidence interval 1.15 to 3.04) and adverse event rate (death/MI, death/MI/CABG) among black patients. Thus, although differences in patient demographics, clinical presentation, angiographic characteristics and treatment strategies did not impact the incidence of acute and 1-year adverse outcomes of non-whites, there appears to be a significant reduction in event-free survival among blacks by 2 years.

  10. Assessment and provision of rehabilitation among patients hospitalized with acute ischemic stroke in China: Findings from the China National Stroke Registry II.

    PubMed

    Bettger, Janet Prvu; Li, Zixiao; Xian, Ying; Liu, Liping; Zhao, Xingquan; Li, Hao; Wang, Chunxue; Wang, Chunjuan; Meng, Xia; Wang, Anxin; Pan, Yuesong; Peterson, Eric D; Wang, Yilong; Wang, Yongjun

    2017-04-01

    Background Stroke rehabilitation improves functional recovery among stroke patients. However, little is known about clinical practice in China regarding the assessment and provision of rehabilitation among patients with acute ischemic stroke. Aims We examined the frequency and determinants of an assessment for rehabilitation among acute ischemic stroke patients from the China National Stroke Registry II. Methods Data for 19,294 acute ischemic stroke patients admitted to 219 hospitals from June 2012 to January 2013 were analyzed. The multivariable logistic regression model with the generalized estimating equation method accounting for in-hospital clustering was used to identify patient and hospital factors associated with having a rehabilitation assessment during the acute hospitalization. Results Among 19,294 acute ischemic stroke patients, 11,451 (59.4%) were assessed for rehabilitation. Rates of rehabilitation assessment varied among 219 hospitals (IQR 41.4% vs 81.5%). In the multivariable analysis, factors associated with increased likelihood of a rehabilitation assessment ( p < 0.05) included disability prior to stroke, higher NIHSS on admission, receipt of a dysphagia screen, deep venous thrombosis prophylaxis, carotid vessel imaging, longer length of stay, and treatment at a hospital with a higher number of hospital beds (per 100 units). In contrast, patients with a history of atrial fibrillation and hospitals with higher number of annual stroke discharges (per 100 patients) were less likely to receive rehabilitation assessment during the acute stroke hospitalization. Conclusions Rehabilitation assessment among acute ischemic stroke patients was suboptimal in China. Rates varied considerably among hospitals and support the need to improve adherence to recommended care for stroke survivors.

  11. Influence of Differently Licensed KIR2DL1-Positive Natural Killer Cells in Transplant Recipients with Acute Leukemia: A Japanese National Registry Study.

    PubMed

    Arima, Nobuyoshi; Nakamura, Fumiaki; Yabe, Toshio; Tanaka, Junji; Fuji, Shigeo; Ohashi, Kazuteru; Fukuda, Takahiro; Miyamura, Koichi; Iwato, Koji; Eto, Tetsuya; Mori, Takehiko; Kobayashi, Naoki; Hoshino, Takumi; Kato, Chiaki; Kanamori, Heiwa; Nakamae, Hirohisa; Atsuta, Yoshiko; Morishima, Yasuo; Kanda, Yoshinobu

    2016-03-01

    Licensing by self MHC class I ligands is required for proper natural killer (NK) cell response. NK cells with inhibitory killer cell immunoglobulin-like receptors for nonself MHC exhibit transient alloreactivity after hematopoietic stem cell transplantation (HSCT). We analyzed 3866 recipients in the Japan national registry who underwent their first allogeneic HSCT for acute myeloid leukemia (AML) or acute lymphoblastic leukemia (ALL) from HLA-A, -B, and -DRB1 allele-genomatched unrelated donors. By classifying them into 5 independent groups based on HLA-C group matching and assumed donor NK cell status, we found that for HLA-C-matched HSCT for AML in HLA-C1/C1 recipients, in whom transient alloreactivity against HLA-C2-negative leukemic cells was expected, the relapse rate was significantly lower than it was in HLA-C-matched HSCT for AML in HLA-C1/C2 recipients (hazard ratio [HR], .72; P = .011). This difference was not observed in HLA-C-matched HSCT for ALL. Compared with HLA-C-matched HSCT, significantly higher mortality was observed in HLA-C1/C1 AML patients who received transplants from HLA-C-mismatched HLA-C1/C1 donors (HR, 1.37; P = .001) and in HLA-C1/C1 ALL patients who received transplants from HLA-C2-positive donors (HR, 2.13; P = .005). In conclusion, donor selection based on leukemic subtype and donor HLA-C group matching improves transplantation outcome after HLA-C-mismatched HSCT.

  12. A methodology for a minimum data set for rare diseases to support national centers of excellence for healthcare and research

    PubMed Central

    Choquet, Rémy; Maaroufi, Meriem; de Carrara, Albane; Messiaen, Claude; Luigi, Emmanuel; Landais, Paul

    2015-01-01

    Background Although rare disease patients make up approximately 6–8% of all patients in Europe, it is often difficult to find the necessary expertise for diagnosis and care and the patient numbers needed for rare disease research. The second French National Plan for Rare Diseases highlighted the necessity for better care coordination and epidemiology for rare diseases. A clinical data standard for normalization and exchange of rare disease patient data was proposed. The original methodology used to build the French national minimum data set (F-MDS-RD) common to the 131 expert rare disease centers is presented. Methods To encourage consensus at a national level for homogeneous data collection at the point of care for rare disease patients, we first identified four national expert groups. We reviewed the scientific literature for rare disease common data elements (CDEs) in order to build the first version of the F-MDS-RD. The French rare disease expert centers validated the data elements (DEs). The resulting F-MDS-RD was reviewed and approved by the National Plan Strategic Committee. It was then represented in an HL7 electronic format to maximize interoperability with electronic health records. Results The F-MDS-RD is composed of 58 DEs in six categories: patient, family history, encounter, condition, medication, and questionnaire. It is HL7 compatible and can use various ontologies for diagnosis or sign encoding. The F-MDS-RD was aligned with other CDE initiatives for rare diseases, thus facilitating potential interconnections between rare disease registries. Conclusions The French F-MDS-RD was defined through national consensus. It can foster better care coordination and facilitate determining rare disease patients’ eligibility for research studies, trials, or cohorts. Since other countries will need to develop their own standards for rare disease data collection, they might benefit from the methods presented here. PMID:25038198

  13. In axial spondyloarthritis, never smokers, ex-smokers and current smokers show a gradient of increasing disease severity - results from the Scotland Registry for Ankylosing Spondylitis (SIRAS).

    PubMed

    Jones, Gareth T; Ratz, Tiara; Dean, Linda E; Macfarlane, Gary J; Atzeni, Fabiola

    2016-11-29

    Objectives To examine the relationship between smoking, smoking cessation, and disease characteristics/quality of life (QoL) in spondyloarthritis. Methods The Scotland Registry for Ankylosing Spondylitis collects data from clinically diagnosed patients with spondyloarthritis. Clinical data, including Bath Ankylosing Spondylitis indices of disease activity (BASDAI) and function (BASFI), was obtained from medical records. Postal questionnaires provided information on smoking status and QoL (Ankylosing Spondylitis QoL questionnaire; ASQoL). Linear and logistic regression quantified the effect of smoking, and smoking cessation, on various disease-specific and QoL outcomes, adjusting for age, sex, deprivation, education and alcohol status. Results are presented as regression coefficients (β) or odds ratios (OR) with 95% confidence intervals. Results 946 participants provided data (male 73.5%, mean age 52yrs). Current smoking was reported by 22%, and 38% were ex-smokers. Ever smokers experienced poorer BASDAI (β = 0.5; 0.2 to 0.9) and BASFI (β = 0.8; 0.4 to 1.2), and reported worse QoL (ASQoL, β = 1.5; 0.7 to 2.3). Compared to current smokers, ex-smokers reported lower disease activity (BASDAI, β = -0.5; -1.0 to -0.04) and significantly better QoL (ASQoL, β = -1.2; -2.3 to -0.2). They also were more likely to have a uveitis history (OR = 2.4; 1.5 to 3.8). Conclusions Smokers with spondyloarthritis experience worse disease than never smokers. However, we provide new evidence that, among smokers, smoking cessation is associated with lower disease activity and better physical function and QoL. Clinicians should specifically promote smoking cessation as an adjunct to usual therapy in patients with spondyloarthritis. This article is protected by copyright. All rights reserved.

  14. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis.

    PubMed

    Iorio, A; Oliovecchio, E; Morfini, M; Mannucci, P M

    2008-05-01

    National haemophilia registries are powerful instruments to support health care and research. A national registry was established in Italy by the Ministry of Health until 1999. Since 2003 the Italian Association of Haemophilia Centres (AICE) started a new programme aiming at building up the Italian Registry of Haemophilia and Allied Disorders. The AICE identified an expert panel to steer the registry. A computer software to assist patient management was developed and all the AICE-affiliated haemophilia treatment centres (HTC) were prompted to adopt it. Twice a year a predefined set of anonymized data is centralized and merged into a national database. Duplicated entries are managed through a confidentiality sparing mechanism. The database covers sociodemographic, clinical, laboratory and treatment data. A subset of data are shared with the Ministry of Health (Istituto Superiore di Sanità,ISS).Overall, data were collected six times by 43 of 49 HTC; 41 centres updated their patients' records up to December 2006. The database contains 6632 unique records, 442 of them referring to dead patients. Database growth and missing data clearance showed a constantly positive trend over time. The database has collected records of the following alive patients - haemophilia A: 1364 severe, 398 moderate and 935 mild; haemophilia B: 231 severe, 138 moderate and 204 mild; von Willebrand's disease: 1208 type 1, 346 type 2 and 96 type 3. Inhibitor patients were 296 (of which 194 high responders and 65 low responders).The Italian registry run by AICE adds to the list of the available national haemophilia registries and is intended to establish treatment guidelines and foster research projects in Italy.

  15. Data available from birth and death registries and cancer registries in the United States

    SciTech Connect

    Wallin, B.L. |; Houser, A.R.; Merrill, D.W.; Selvin, S. |

    1994-01-01

    In the United States, cancer registries have been compiling data for decades, and state vital statistics offices have been compiling birth and death data for nearly a century. Although this information has been well used for disease surveillance and various studies, it could be better exploited by making it more readily available, reducing the duplication of effort that occurs when researchers at the private, city, county, state, and federal levels work separately on their data collection and disease investigations. This report summarizes the nationwide availability of birth and death records and cancer registry data, with particular emphasis on subcounty geographic detail, such as zip code and census tract. Birth and death data are available at the county level for the entire United States from the National Center for Health Statistics. However, county level data are inadequate for small area studies of potential environmental hazards. Hazards of current interest to the Department of Energy (DOE) include DOE facilities, nuclear power plants, and sources of electromagnetic radiation such as broadcasting towers and power lines.

  16. Positive and negative effects of IT on cancer registries.

    PubMed

    Mohammadzadeh, Niloofar; Safdari, Reza; Rahimi, Azin

    2013-01-01

    In the new millennium people are facing serious challenges in health care, especially with increasing non- communicable diseases (NCD). One of the most common NCDs is cancer which is the leading cause of death in developed countries and in developing countries is the second cause of death after heart diseases. Cancer registry can make possible the analysis, comparison and development of national and international cancer strategies and planning. Information technology has a vital role in quality improvement and facility of cancer registries. With the use of IT, in addition to gaining general benefits such as monitoring rates of cancer incidence and identifying planning priorities we can also gain specific advantages such as collecting information for a lifetime, creating tele medical records, possibility of access to information by patient, patient empowerment, and decreasing medical errors. In spite of the powerful role of IT, we confront various challenges such as general problems, like privacy of the patient, and specific problems, including possibility of violating patients rights through misrepresentation, omission of human relationships, and decrease in face to face communication between doctors and patients. By implementing appropriate strategies, such as identifying authentication levels, controlling approaches, coding data, and considering technical and content standards, we can optimize the use of IT. The aim of this paper is to emphasize the need for identifying positive and negative effects of modern IT on cancer registry in general and specific aspects as an approach to cancer care management.

  17. I RBH - First Brazilian Hypertension Registry

    PubMed Central

    Jardim, Paulo César Brandão Veiga; de Souza, Weimar Kunz Sebba Barroso; 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

    2016-01-01

    Background: 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. Objective: 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. Methods: 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. Results: The results will be presented after the final evaluation, which will occur at the end of a 1-year follow-up. Conclusion: 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

  18. Establishing a harmonized haemophilia registry for countries with developing health care systems.

    PubMed

    Alzoebie, A; Belhani, M; Eshghi, P; Kupesiz, A O; Ozelo, M; Pompa, M T; Potgieter, J; Smith, M

    2013-09-01

    Over recent decades tremendous progress has been made in diagnosing and treating haemophilia and, in resource-rich countries, life expectancy of people with haemophilia (PWH) is now close to that of a healthy person. However, an estimated 70% of PWH are not diagnosed or are undertreated; the majority of whom live in countries with developing health care systems. In these countries, designated registries for people with haemophilia are often limited and comprehensive information on the natural history of the disease and treatment outcomes is lacking. Taken together, this means that planning efforts for future treatment and care of affected individuals is constrained in countries where it is most needed. Establishment of standardized national registries in these countries would be a step towards obtaining reliable sociodemographic and clinical data for an entire country. A series of consensus meetings with experts from widely differing countries with different health care systems took place to discuss concerns specific to countries with developing health care systems. As a result of these discussions, recommendations are made on parameters to include when establishing and harmonizing national registries. Such recommendations should enable countries with developing health care systems to establish standardized national haemophilia registries. Although not a primary objective, the recommendations should also help standardized data collation on an international level, enabling treatment and health care trends to be monitored across groups of countries and providing data for advocacy purposes. Greater standardization of data collation should have implications for optimizing resources for haemophilia care both nationally and internationally.

  19. 76 FR 68615 - National Alzheimer's Disease Awareness Month, 2011

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-11-04

    ... eliminate Alzheimer's disease. Research funded by the National Institutes of Health has identified genetic... Documents#0;#0; ] Proclamation 8745 of November 1, 2011 National Alzheimer's Disease Awareness Month, 2011... heartbreak of watching a loved one struggle with Alzheimer's disease is a pain they know all too...

  20. 76 FR 35224 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-16

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Committee: National Institute of Allergy and Infectious Diseases Special Emphasis Panel, Disease...

  1. Body mass index and survival after diagnosis of invasive breast cancer: a study based on the Japanese National Clinical Database-Breast Cancer Registry.

    PubMed

    Kawai, Masaaki; Tomotaki, Ai; Miyata, Hiroaki; Iwamoto, Takayuki; Niikura, Naoki; Anan, Keisei; Hayashi, Naoki; Aogi, Kenjiro; Ishida, Takanori; Masuoka, Hideji; Iijima, Kotaro; Masuda, Shinobu; Tsugawa, Koichiro; Kinoshita, Takayuki; Nakamura, Seigo; Tokuda, Yutaka

    2016-06-01

    Few studies have reported the association between body mass index (BMI) and outcome among Asian breast cancer patients. We analyzed data for 20,090 female invasive breast cancer patients who had been followed-up for a median period of 6.7 years entered in the National Clinical Database-Breast Cancer Registry between 2004 and 2006. We used mainly the WHO criteria for BMI (kg/m(2) ) categories; <18.5 (underweight), ≥18.5-<21.8 (reference), ≥21.8-<25, ≥25-<30 (overweight), and ≥30 (obese). We divided normal weight patients into two subgroups because this category includes many patients compared to others. The timing of BMI measurement was not specified. The Cox proportional hazards model and cubic spline regression were used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs). Smoking, alcohol, and physical activity were not controlled. A total of 1418 all-cause, 937 breast cancer-specific deaths, and 2433 recurrences were observed. Obesity was associated with an increased risk of all-cause (HR: 1.46; 95% CI: 1.16-1.83) and breast cancer-specific death (HR: 1.47; 95% CI: 1.11-1.93) for all patients, and with all-cause (HR: 1.47; 95% CI: 1.13-1.92) and breast cancer-specific death (HR: 1.58; 95% CI: 1.13-2.20) for postmenopausal patients. Being underweight was associated with an increased risk of all-cause death for all (HR: 1.41; 95% CI: 1.16-1.71) and for postmenopausal patients (HR: 1.45; 95% CI: 1.15-1.84). With regard to subtype and menopausal status, obesity was associated with an increased risk of breast cancer-specific death for all cases of luminal B tumor (HR: 2.59; 95% CI: 1.51-4.43; Pheterogeneity of Luminal B vs. Triple negative = 0.016) and for postmenopausal patients with luminal B tumor (HR: 3.24; 95% CI: 1.71-6.17). Being obese or underweight is associated with a higher risk of death among female breast cancer patients in Japan.

  2. CTLA-4 gene polymorphism confers susceptibility to insulin-dependent diabetes mellitus (IDDM) independently from age and from other genetic or immune disease markers. The Belgian Diabetes Registry.

    PubMed

    Van der Auwera, B J; Vandewalle, C L; Schuit, F C; Winnock, F; De Leeuw, I H; Van Imschoot, S; Lamberigts, G; Gorus, F K

    1997-10-01

    Apart from genes in the HLA complex (IDDM1) and the variable number of tandem repeats in the 5' region of the insulin gene (INS VNTR, IDDM2), several other loci have been proposed to contribute to IDDM susceptibility. Recently, linkage and association have been shown between the cytotoxic T lymphocyte-associated protein 4 (CTLA-4) gene on chromosome 2q and IDDM. In a registry-based group of 525 recent-onset IDDM patients <40 years old we investigated the possible interactions of a CTLA-4 gene A-to-G transition polymorphism with age at clinical disease onset and with the presence or absence of established genetic (HLA-DQ, INS VNTR) and immune disease markers (autoantibodies against islet cell cytoplasm (ICA); insulin (IAA); glutamate decarboxylase (GAD65-Ab); IA-2 protein tyrosine phosphatase (IA-2-Ab)) determined within the first week of insulin treatment. In new-onset IDDM patients. G-allele-containing CTLA-4 genotypes (relative risk (RR)= 1.5; 95% confidence interval (CI) = 1.2-2.0; P < 0.005) were not preferentially associated with age at clinical presentation or with the presence of other genetic (HLA-DR3 or DR4 alleles; HLA-DQA1*0301-DQB1*0302 and/or DQA1*0501-DQB1*0201 risk haplotypes; INS VNTR I/I risk genotype) or immune (ICA, IAA, IA-2-Ab, GAD65-Ab) markers of diabetes. For 151 patients, thyrogastric autoantibodies (anti-thyroid peroxidase, anti-thyroid-stimulating hormone (TSH) receptor, anti-parietal cell, anti-intrinsic factor) were determined, but association between CTLA-4 risk genotypes and markers of polyendocrine autoimmunity could not be demonstrated before or after stratification for HLA- or INS-linked risk. In conclusion, the presence of a G-containing CTLA-4 genotype confers a moderate but significant RR for IDDM that is independent of age and genetic or immune disease markers.

  3. Pulmonary embolism and 3-month outcomes in 4036 patients with venous thromboembolism and chronic obstructive pulmonary disease: data from the RIETE registry

    PubMed Central

    2013-01-01

    Background Patients with chronic obstructive pulmonary disease (COPD) have a modified clinical presentation of venous thromboembolism (VTE) but also a worse prognosis than non-COPD patients with VTE. As it may induce therapeutic modifications, we evaluated the influence of the initial VTE presentation on the 3-month outcomes in COPD patients. Methods COPD patients included in the on-going world-wide RIETE Registry were studied. The rate of pulmonary embolism (PE), major bleeding and death during the first 3 months in COPD patients were compared according to their initial clinical presentation (acute PE or deep vein thrombosis (DVT)). Results Of the 4036 COPD patients included, 2452 (61%; 95% CI: 59.2-62.3) initially presented with PE. PE as the first VTE recurrence occurred in 116 patients, major bleeding in 101 patients and mortality in 443 patients (Fatal PE: first cause of death). Multivariate analysis confirmed that presenting with PE was associated with higher risk of VTE recurrence as PE (OR, 2.04; 95% CI: 1.11-3.72) and higher risk of fatal PE (OR, 7.77; 95% CI: 2.92-15.7). Conclusions COPD patients presenting with PE have an increased risk for PE recurrences and fatal PE compared with those presenting with DVT alone. More efficient therapy is needed in this subtype of patients. PMID:23865769

  4. [Hungarian Hypertension Registry].

    PubMed

    Kiss, István; Kékes, Ede

    2014-05-11

    Today, hypertension is considered endemic throughout the world. The number of individuals with high blood pressure and the increasing risk, morbidity and mortality caused by hypertension despite modern therapy do not decrease sufficiently. Hypertension has become a public health issue. Prevention and effective care require integrated datasets about many features, clinical presentation and therapy of patients with hypertension. The lack of this database in Hungary prompted the development of the registry which could help to provide population-based data for analysis. Data collection and processing was initiated by the Hungarian Society of Hypertension in 2002. Data recording into the Hungarian Hypertension Registry was performed four times (2002, 2005, 2007, 2011) and the registry currently contains data obtained from 108,473 patients. Analysis of these data indicates that 80% of the patients belong to the high or very high cardiovascular risk group. The registry provides data on cardiovascular risk of the hypertensive populations and the effectiveness of antihypertensive therapy in Hungary. Based on international experience and preliminary analysis of data from the Hungarian Hypertension Registry, establishment of hypertension registry may support the effectiveness of public health programs. A further step would be needed for proper data management control and the application of professional principles of evidence-based guidelines in the everyday practice.

  5. Systematic Review of Cerebral Palsy Registries/Surveillance Groups: Relationships between Registry Characteristics and Knowledge Dissemination

    PubMed Central

    Hurley, Donna S; Sukal-Moulton, Theresa; Gaebler-Spira, Deborah; Krosschell, Kristin J; Pavone, Larissa; Mutlu, Akmer; Dewald, Julius PA; Msall, Michael E

    2016-01-01

    The aims of this study were to provide a comprehensive summary of the body of research disseminated by Cerebral Palsy (CP) registries and surveillance programs from January 2009 through May 2014 in order to describe the influence their results have on our overall understanding of CP. Secondly, registries/surveillance programs and the work they produced were evaluated and grouped using standardized definitions and classification systems. Method A systematic review search in PubMed, CINAH and Embase for original articles published from 1 January 2009 to 20 May 2014 originating from or supported by population based CP registries and surveillance programs or population based national registries including CP were included. Articles were grouped by 2009 World CP Registry Congress aim, registry/surveillance program classification, geographical region, and the International Classification of Function, Disability and Health (ICF) domain. Registry variables were assessed using the ICF-CY classification. Results Literature searches returned 177 articles meeting inclusion criteria. The majority (69%) of registry/surveillance program productivity was related to contributions as a Resource for CP Research. Prevention (23%) and Surveillance (22%) articles were other areas of achievement, but fewer articles were published in the areas of Planning (17%) and Raising the Profile of CP (2%). There was a range of registry/surveillance program classifications contributing to this productivity, and representation from multiple areas of the globe, although most of the articles originated in Europe, Australia, and Canada. The domains of the ICF that were primarily covered included body structures and function at the early stages of life. Encouragingly, a variety of CP registry/surveillance program initiatives included additional ICF domains of participation and environmental and personal factors. Interpretation CP registries and surveillance programs, including novel non-traditional ones

  6. Establishing an institutional therapeutic apheresis registry.

    PubMed

    Mann, Steven A; McCleskey, Brandi; Marques, Marisa B; Adamski, Jill

    2016-12-01

    Apheresis was first performed as a therapeutic procedure in the 1950s. The first national therapeutic apheresis (TA) registry was established in Canada in 1981 and other national registries followed, including two attempts at establishing an international TA registry. There is no national registry in the United States. Our large, academic, tertiary hospital has a very active TA service. We created a TA database to track all procedures performed by the apheresis service by transferring data from paper appointment logs and the electronic medical records into a Microsoft Access database. Retrospective data from each TA procedure performed at UAB from January 1, 2003 through December 31, 2012 were entered, including the type of procedure, indication, date, and patient demographics. Microsoft Excel was used for data analysis. During the 10-year period, our TA service treated 1,060 patients and performed 11,718 procedures. Of these patients, 70% received therapeutic plasma exchange (TPE), 21% received extracorporeal photopheresis (ECP), 4.5% received red cell exchange (RCE), 4.2% received leukocytapheresis, and 0.6% underwent platelet depletion. Among the procedures, 54% were TPEs, 44% were ECPs, 1.3% were RCEs, 0.5% were leukocytaphereses, and 0.1% were platelet depletions. According to the current literature, national and international TA use is underreported. We believe that the UAB TA registry provides useful information about TA practices in our region and can serve as a model for other institutions. Furthermore, data from multiple institutional registries can be used for clinical research to increase the available evidence for the role of TA in various conditions. J. Clin. Apheresis 31:516-522, 2016. © 2015 Wiley Periodicals, Inc.

  7. National Institutes of Health Osteoporosis and Related Bone Diseases~National Resource Center

    MedlinePlus

    ... Publications View Publications View Publications View Publications Research Resources Clinical Trials www.clinicaltrials.gov MedlinePlus www.nlm. ... The NIH Osteoporosis and Related Bone Diseases ~ National Resource Center is supported by the National Institute of ...

  8. National Infectious Diseases Surveillance data of South Korea.

    PubMed

    Park, Sunhee; Cho, Eunhee

    2014-01-01

    The Korea Centers for Disease Control and Prevention (KCDC) operate infectious disease surveillance systems to monitor national disease incidence. Since 1954, Korea has collected data on various infectious diseases in accordance with the Infectious Disease Control and Prevention Act. All physicians (including those working in Oriental medicine) who diagnose a patient with an infectious disease or conduct a postmortem examination of an infectious disease case are obliged to report the disease to the system. These reported data are incorporated into the database of the National Infectious Disease Surveillance System, which has been providing web-based real-time surveillance data on infectious diseases since 2001. In addition, the KCDC analyzes reported data and publishes the Infectious Disease Surveillance Yearbook annually.

  9. Identification and outcomes of clinical phenotypes in amyotrophic lateral sclerosis/motor neuron disease: Australian National Motor Neuron Disease observational cohort

    PubMed Central

    Talman, Paul; Duong, Thi; Vucic, Steve; Mathers, Susan; Venkatesh, Svetha; Henderson, Robert; Rowe, Dominic; Schultz, David; Edis, Robert; Needham, Merrilee; Macdonnell, Richard; McCombe, Pamela; Birks, Carol; Kiernan, Matthew

    2016-01-01

    Objective To capture the clinical patterns, timing of key milestones and survival of patients presenting with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) within Australia. Methods Data were prospectively collected and were timed to normal clinical assessments. An initial registration clinical report form (CRF) and subsequent ongoing assessment CRFs were submitted with a completion CRF at the time of death. Design Prospective observational cohort study. Participants 1834 patients with a diagnosis of ALS/MND were registered and followed in ALS/MND clinics between 2005 and 2015. Results 5 major clinical phenotypes were determined and included ALS bulbar onset, ALS cervical onset and ALS lumbar onset, flail arm and leg and primary lateral sclerosis (PLS). Of the 1834 registered patients, 1677 (90%) could be allocated a clinical phenotype. ALS bulbar onset had a significantly lower length of survival when compared with all other clinical phenotypes (p<0.004). There were delays in the median time to diagnosis of up to 12 months for the ALS phenotypes, 18 months for the flail limb phenotypes and 19 months for PLS. Riluzole treatment was started in 78–85% of cases. The median delays in initiating riluzole therapy, from symptom onset, varied from 10 to 12 months in the ALS phenotypes and 15–18 months in the flail limb phenotypes. Percutaneous endoscopic gastrostomy was implemented in 8–36% of ALS phenotypes and 2–9% of the flail phenotypes. Non-invasive ventilation was started in 16–22% of ALS phenotypes and 21–29% of flail phenotypes. Conclusions The establishment of a cohort registry for ALS/MND is able to determine clinical phenotypes, survival and monitor time to key milestones in disease progression. It is intended to expand the cohort to a more population-based registry using opt-out methodology and facilitate data linkage to other national registries. PMID:27694488

  10. Design of comprehensive Alzheimer's disease centers to address unmet national needs.

    PubMed

    Trojanowski, John Q; Arnold, Steven E; Karlawish, Jason H; Brunden, Kurt; Cary, Mark; Davatzikos, Christos; Detre, John; Gaulton, Glen; Grossman, Murray; Hurtig, Howard; Jedrziewski, Kathryn; McCluskey, Leo; Naylor, Mary; Polsky, Daniel; Schellenberg, Gerard D; Siderowf, Andrew; Shaw, Leslie M; Van Deerlin, Vivianna; Wang, Li-San; Werner, Rachel; Xie, Sharon X; Lee, Virginia M-Y

    2010-03-01

    The problem of Alzheimer's disease (AD) exemplifies the challenges of dealing with a broad range of aging-related chronic disorders that require long-term, labor-intensive, and expensive care. As the baby boom generation ages and brain diseases become more prevalent, the need to confront the pending health care crisis is more urgent than ever before. Indeed, there is now a critical need to expand significantly the national effort to solve the problem of AD, with special focus on prevention. The Campaign to Prevent Alzheimer's Disease by 2020 (PAD2020) aims to create a new paradigm for planning and supporting the organization of worldwide cooperative research networks to develop new technologies for early detection and treatments of aging-related memory and motor impairments. PAD 2020 is developing an implementation plan to justify (1) increasing the federal budget for research, (2) developing novel national resources to discover new interventions for memory and motor disorders, and (3) creating innovative and streamlined decision-making processes for selecting and supporting new ideas. Since 1978 the National Institute on Aging or National Institute of Health (NIH) established an extensive national network of AD research facilities at academic institutions including AD Centers (ADCs), Consortium to Establish a Registry for AD, AD Cooperative Study (ADCS), AD Drug Discovery Program, National Alzheimer's Coordinating Center, National Cell Repository for AD, and AD Neuroimaging Initiative. However, despite the success of these programs and their critical contributions, they are no longer adequate to meet the challenges presented by AD. PAD 2020 is designed to address these changes by improving the efficiency and effectiveness of these programs. For example, the ADCs (P30s and P50s) can be enhanced by converting some into Comprehensive Alzheimer's Disease Centers (CADCs) to support not only research, but also by being demonstration projects on care/treatment, clinical

  11. 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

  12. Ticks Carrying Lyme Disease Confirmed in Eastern National Parks

    MedlinePlus

    ... html Ticks Carrying Lyme Disease Confirmed in Eastern National Parks U.S. National Park Service and CDC advise using insect repellents on ... Planning a hiking trip in an eastern U.S. national park? Better pack tick repellent -- a new study found ...

  13. Stereotactic Body Radiotherapy for Clinically Localized Prostate Cancer: Toxicity and Biochemical Disease-Free Outcomes from a Multi-Institutional Patient Registry

    PubMed Central

    Sharma, Sanjeev; Shumway, Richard; Perry, David; Bydder, Sean; Simpson, C. Kelley; D'Ambrosio, David

    2015-01-01

    Objectives: To report on initial patient characteristics, treatment practices, toxicity, and early biochemical disease-free survival (bDFS) of localized prostate cancer treated with stereotactic body radiotherapy (SBRT) and enrolled in the RSSearch® Patient Registry. Methods: A retrospective analysis was conducted on patients with clinically localized prostate cancer enrolled in RSSearch® from June 2006 - January 2015. Patients were classified as low-risk (PSA ≤ 10 ng/ml, T1c-T2a, Gleason score ≤ 6), intermediate-risk (PSA 10.1 - 20 ng/ml, T2b-T2c, or Gleason 7), or high-risk (PSA > 20 ng/ml, T3 or Gleason ≥ 8). Toxicity was reported using Common Toxicity Criteria for Adverse Events, version 3. Biochemical failure was assessed using the Phoenix definition (nadir + 2 ng/ml). The Kaplan-Meier analysis was used to calculate bDFS and association of patient and tumor characteristics with the use of SBRT. Results: Four hundred thirty-seven patients (189 low, 215 intermediate, and 33 high-risk) at a median of 69 years (range: 48-88) received SBRT at 17 centers. Seventy-eight percent of patients received 36.25 Gy/5 fractions, 13% received 37 Gy/5 fractions, 6% received 35 Gy/5 fractions, 3% received 38 Gy/4 fractions, and 5% received a boost dose of 19.5-29 Gy following external beam radiation therapy. Median follow-up was 20 months (range: 1–64 months). Genitourinary (GU) and gastrointestinal (GI) toxicities were minimal, with no acute or late Grade 3+ GU or GI toxicity. Late Grade 1 and 2 urinary frequency was 25% and 8%. Late Grade 1 and 2 proctitis was 3% and 2%. Median PSA decreased from 5.8 ng/ml (range: 0.3-43) to 0.88, 0.4, and 0.3 ng/ml at one, two, and three years. Two-year bDFS for all patients was 96.1%. Two-year bDFS was 99.0%, 94.5%, and 89.8% for low, intermediate, and high-risk patients (p < 0.0001). Two-year bDFS was 99.2%, 93.2%, and 90.4% for Gleason ≤ 6, Gleason 7, and Gleason ≥ 8 (p < 0.0001). Two-year bDFS was 96.4%, 97

  14. [German resuscitation registry : science and resuscitation research].

    PubMed

    Gräsner, J-T; Seewald, S; Bohn, A; Fischer, M; Messelken, M; Jantzen, T; Wnent, J

    2014-06-01

    Sudden death due to cardiac arrest represents one of the greatest challenges facing modern medicine, not only because of the massive number of cases involved but also because of its tremendous social and economic impact. For many years, the magic figure of 1 per 1000 inhabitants per year was generally accepted as an estimate of the annual incidence of sudden death in the industrialized world, with a survival rate of 6 %. This estimate was based on large numbers of published reports of local, regional, national and multinational experience in the management of cardiac arrest. Measuring the global incidence of cardiac arrest is challenging as many different definitions of patient populations are used. Randomized controlled trials (RCT) provide insights into the value of specific treatments or treatment strategies in a well-defined section of a population. Registries do not compete with clinical studies, but represent a useful supplement to them. Surveys and registries provide insights into the ways in which scientific findings and guidelines are being implemented in clinical practice. However, as with clinical studies, comprehensive preparations are needed in order to establish a registry. This is all the more decisive because not all of the questions that may arise are known at the time when the registry is established. The German resuscitation registry started in May 2007 and currently more than 230 paramedic services and hospitals take part. More than 45,000 cases of out-of-hospital cardiac arrest and in-hospital cardiac arrest are included. With this background the German resuscitation registry is one of the largest databases in emergency medicine in Germany. After 5 years of running the preclinical care dataset was revised in 2012. Data variables that reflect current or new treatment were added to the registry. The postresuscitation basic care and telephone cardiopulmonary resuscitation (CPR) datasets were developed in 2012 and 2013 as well. The German

  15. Linkage between the Danish National Health Service Prescription Database, the Danish Fetal Medicine Database, and other Danish registries as a tool for the study of drug safety in pregnancy

    PubMed Central

    Pedersen, Lars H; Petersen, Olav B; Nørgaard, Mette; Ekelund, Charlotte; Pedersen, Lars; Tabor, Ann; Sørensen, Henrik T

    2016-01-01

    A linked population-based database is being created in Denmark for research on drug safety during pregnancy. It combines information from the Danish National Health Service Prescription Database (with information on all prescriptions reimbursed in Denmark since 2004), the Danish Fetal Medicine Database, the Danish National Registry of Patients, and the Medical Birth Registry. The new linked database will provide validated information on malformations diagnosed both prenatally and postnatally. The cohort from 2008 to 2014 will comprise 589,000 pregnancies with information on 424,000 pregnancies resulting in live-born children, ∼420,000 pregnancies undergoing prenatal ultrasound scans, 65,000 miscarriages, and 92,000 terminations. It will be updated yearly with information on ∼80,000 pregnancies. The cohort will enable identification of drug exposures associated with severe malformations, not only based on malformations diagnosed after birth but also including those having led to termination of pregnancy or miscarriage. Such combined data will provide a unique source of information for research on the safety of medications used during pregnancy. PMID:27274312

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  1. Effects of Cardiac Medications for Patients With Obstructive Coronary Artery Disease by Coronary Computed Tomographic Angiography: Results from the Multicenter CONFIRM Registry

    PubMed Central

    Schulman-Marcus, Joshua; Hartaigh, Bríain ó; Giambrone, Ashley E; Gransar, Heidi; Valenti, Valentina; Berman, Daniel S.; Budoff, Matthew J.; Achenbach, Stephan; Al-Mallah, Mouaz; Andreini, Daniele; Cademartiri, Filippo; Callister, Tracy Q.; Chang, Hyuk-Jae; Chinnaiyan, Kavitha; Chow, Benjamin J. W.; Cury, Ricardo; Delago, Augustin; Hadamitzky, Martin; Hausleiter, Joerg; Feuchtner, Gudrun; Kim, Yong-Jin; Kaufmann, Philipp A.; Leipsic, Jonathon; Lin, Fay Y.; Maffei, Erica; Pontone, Gianluca; Raff, Gilbert; Shaw, Leslee J.; Villines, Todd C.; Dunning, Allison; Min, James K

    2014-01-01

    Objective This study sought to determine the correlation between baseline cardiac medications and cardiovascular outcomes in patients with obstructive coronary artery disease (CAD) diagnosed by coronary computed tomographic angiography (CCTA). Methods 1637 patients (mean age 64.8 ± 10.2 years, 69.6% male) with obstructive CAD from the CONFIRM (COronary CT Angiography EvaluatioN For Clinical Outcomes: An InteRnational Multicenter) registry were followed over the course of three years. Obstructive CAD was defined as a >50% stenosis in an epicardial vessel. Medications analyzed included statins, aspirin, beta-blockers, angiotensin converting enzyme (ACE) inhibitors, and angiotensin receptor blockers (ARBs). Using Cox proportional-hazards models, we calculated the hazard ratio (HR) with 95% confidence intervals (95% CIs) for incident major adverse cardiovascular events (MACE), defined as death, acute coronary syndrome, or myocardial infarction. Results At the time of CCTA, 59%, 54%, 40%, and 46% of patients were using statins, aspirin, beta-blockers, and ACE inhibitors or ARBs, respectively. Statins were associated with a 43% (95% CI = 0.38-0.87, p=0.008) lower adjusted risk of MACE. Following adjustment, aspirin, beta-blockers, ACE inhibitors and ARBs did not attenuate the risk of MACE. When restricted to patients with multivessel obstructive CAD, only statins were associated with lower risk of MACE. Conclusion In patients with obstructive CAD by CCTA, the baseline use of statins was associated with improved clinical outcomes. Other cardiac medications—including aspirin, beta-blockers, ACE inhibitors, and ARBs—were not associated with reduced risk of MACE. PMID:25479800

  2. Genetics Home Reference: Dent disease

    MedlinePlus

    ... 1 Genetic Testing Registry: Dent disease 2 Other Diagnosis and Management Resources (4 links) GeneReview: Dent Disease MedlinePlus Encyclopedia: Protein - Urine National Institute of Diabetes and Digestive and Kidney Diseases: Kidney Failure: Choosing a Treatment That's Right for ...

  3. Malignancies in Swedish persons with haemophilia: a longitudinal registry study.

    PubMed

    Lövdahl, Susanna; Henriksson, Karin M; Baghaei, Fariba; Holmström, Margareta; Berntorp, Erik; Astermark, Jan

    2016-09-01

    The aim of the study was to investigate, over time, the incidence of and mortality due to malignant diseases among persons with haemophilia, compared to matched controls. Persons with haemophilia A or B were enrolled via registries at each haemophilia centre, as well as from the National Patient Registry, and were compared to five sex and age-matched controls per patient. Data from the national Cancer Registry were linked to the study participants. A total of 1431 persons with haemophilia and 7150 matched controls were enrolled. Between the years 1972 and 2008, 164 malignancies were reported. The most common type of cancer among patients was prostate cancer, followed by haematologic malignancies, including lymphoma and leukaemia, which were significantly more frequent in patients [n = 35 (2.4%) vs. n = 60 (0.8%); P < 0.001]. Malignancies in bladder and other urinary organs were also significantly different [n = 21 (1.5%) vs. n = 46 (0.6%); P < 0.01]. The overall incidence rate ratio of malignancies per 1000 person-years compared to the controls was 1.3 [95% confidence interval (CI) 1.1, 1.6]. In subgroup analysis, the corresponding incidence rate ratios per 1000 person-years for persons with severe haemophilia was 1.7 (95% CI 0.9, 3.1) and that for mild/moderate haemophilia 1.1 (95% CI 0.8, 1.5). Swedish persons with haemophilia had a significantly higher incidence of malignant diseases than controls. These were primarily haematologic malignancies and cancer in urinary organs, and the difference independent of any co-infections with HIV and/or viral hepatitis. The findings indicate the importance of further studies and close follow-up of malignancies in persons with haemophilia.

  4. Impact of multivessel coronary artery disease and noninfarct-related artery revascularization on outcome of patients with ST-elevation myocardial infarction transferred for primary percutaneous coronary intervention (from the EUROTRANSFER Registry).

    PubMed

    Dziewierz, Artur; Siudak, Zbigniew; Rakowski, Tomasz; Zasada, Wojciech; Dubiel, Jacek S; Dudek, Dariusz

    2010-08-01

    The aim of the study was to assess the impact of multivessel coronary artery disease (MVD) and noninfarct-related artery (non-IRA) revascularization during index percutaneous coronary intervention (PCI) on outcomes of patients with ST-segment elevation myocardial infarction (STEMI). Data on 1,598 of 1,650 patients with complete angiographic data, with >or=1 significantly stenosed epicardial coronary artery, and without previous coronary artery bypass grafting were retrieved from the EUROTRANSFER Registry database. Patients with 1-, 2-, and 3-vessel disease made up 48.5%, 32.0%, and 19.5% of the registry population, respectively. Patients with MVD were less likely to achieve final Thrombolysis In Myocardial Infarction grade 3 flow (1- vs 2- vs 3-vessel disease, 93.6% vs 89.3% vs 87.9%, respectively, p = 0.003) and ST-segment resolution >50% within 60 minutes after PCI (1- vs 2- vs 3-vessel disease, 80.9% vs 77.5% vs 69.3%, respectively, p <0.001). They were also at higher risk of death during 1-year follow-up (1- vs 2- vs 3-vessel disease, 4.9% vs 7.4% vs 13.5%, respectively, p <0.001), and MVD was identified as an independent predictor of 1-year death. In 70 patients (9%) non-IRA PCI was performed during index PCI. These patients were at higher risk of 30-day and 1-year death compared to patients without non-IRA PCI, but this difference in mortality was no longer significant after adjustment for covariates. In conclusion, patients with MVD have decreased epicardial and myocardial reperfusion success and had worse prognosis after primary PCI for STEMI compared to patients with 1-vessel disease. In this large multicenter registry, non-IRA PCI during the index procedure was performed in 9% of patients with MVD and it was associated with increased 1-year mortality.

  5. Serious Infection and Mortality in Patients With Crohn's Disease: More Than 5 Years of Follow-Up in the TREAT™ Registry

    PubMed Central

    Lichtenstein, Gary R; Feagan, Brian G; Cohen, Russell D; Salzberg, Bruce A; Diamond, Robert H; Price, Samiyeh; Langholff, Wayne; Londhe, Anil; Sandborn, William J

    2012-01-01

    OBJECTIVES: The objective of this study was to contribute long-term safety data for infliximab and other therapies in Crohn's disease (CD). METHODS: We prospectively evaluated CD patients enrolled in the large, observational Crohn's Therapy, Resource, Evaluation, and Assessment Tool registry, established to compare infliximab safety with conventional nonbiological medications in CD. RESULTS: A total of 6,273 patients were enrolled and evaluated on or before 23 February 2010; 3,420 received infliximab (17,712 patient-years; 89.9% received ≥2 infusions) and 2,853 received other-treatments-only (13,251 patient-years). Mean length of patient follow-up was 5.2 years. More infliximab- than other-treatments-only-treated patients had moderate-to-severe (30.6% vs. 10.7%) or severe-to-fulminant (2.5% vs. 0.6%) disease severity (P<0.001). In the year before enrollment, more infliximab- than other-treatments-only-treated patients required surgical intervention (17.4% vs. 13.6%), medical hospitalization (14.2% vs. 8.8%), prednisone (47.8% vs. 31.4%), immunomodulators (52.0% vs. 32.1%), and narcotic analgesics (17.3% vs. 9.1%). Patient mortality was similar for infliximab- and other-treatments-only-treated patients (0.58 vs. 0.59/100 patient-years). In multivariate logistic regression analyses, treatment with prednisone (hazard ratio (HR)=2.14, 95% confidence interval (CI)=1.55, 2.95; P<0.001) or narcotic analgesics (HR=1.79, 95% CI=1.29, 2.48; P<0.001) and age (HR=1.08, 95% CI=1.07, 1.09; P<0.001) were associated with increased mortality risk. Neither infliximab nor immunomodulator treatment was associated with increased mortality risk. Factors independently associated with serious infections included moderate-to-severe disease activity (HR=2.24, 95% CI=1.57, 3.19; P<0.001), narcotic analgesic treatment (HR=1.98, 95% CI=1.44, 2.73; P<0.001), prednisone therapy (HR=1.57, 95% CI=1.17, 2.10; P=0.002), and infliximab treatment (HR=1.43, 95% CI=1.11, 1.84; P=0.006). CONCLUSIONS

  6. International Breast Implant Registry: a user report.

    PubMed

    Renner, C; Neuhann-Lorenz, C

    2006-01-01

    The International Breast Implant Registry (IBIR) was founded in 2002 under the auspices of the International Plastic, Reconstructive, and Aesthetic Surgery Foundation (IPRAF), the International Confederation for Plastic, Reconstructive, and Aesthetic Surgery (IPRAS), and the European and International Committee for Quality Assurance, Medical Technologies, and Devices in Plastic (EQUAM) on the basis of continuous discussion about the safety and compatibility of different breast implants. The IBIR aims to integrate and replace the already existing national breast implant registries. It also is assumed that the European Parliament, the Food and Drug Administration, and international organizations of plastic and aesthetic surgeons will postulate obligatory international breast implant registration. Currently, IBIR is in a pilot phase with the goal of understanding data collection issues and concerns in various countries whereby the data entered to date will be completely available in the final version. A well-established global registry represents an important tool of quality assurance. By publishing their experiences in applying the registry, the authors aim to encourage more plastic and aesthetic surgeons to submit their cases to the registry and thus enhance its value as a successful and powerful device.

  7. The cost of cancer registry operations: Impact of volume on cost per case for core and enhanced registry activities

    PubMed Central

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

    2016-01-01

    Background Cancer registration data is vital for creating evidence-based policies and interventions. Quantifying the resources needed for cancer registration activities and identifying potential efficiencies are critically important to ensure sustainability of cancer registry operations. Methods Using a previously validated web-based cost assessment tool, we collected activity-based cost data and report findings using 3 years of data from 40 National Program of Cancer Registry grantees. We stratified registries by volume: low-volume included fewer than 10,000 cases, medium-volume included 10,000–50,000 cases, and high-volume included >50,000 cases. Results Low-volume cancer registries incurred an average of $93.11 to report a case (without in-kind contributions) compared with $27.70 incurred by high-volume registries. Across all registries, the highest cost per case was incurred for data collection and abstraction ($8.33), management ($6.86), and administration ($4.99). Low- and medium-volume registries have higher costs than high-volume registries for all key activities. Conclusions Some cost differences by volume can be explained by the large fixed costs required for administering and performing registration activities, but other reasons may include the quality of the data initially submitted to the registries from reporting sources such as hospitals and pathology laboratories. Automation or efficiency improvements in data collection can potentially reduce overall costs. PMID:26702880

  8. Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry

    PubMed Central

    Ferrara, Giovanni; Carlson, Lisa; Palm, Andreas; Einarsson, Jonas; Olivesten, Cecilia; Sköld, Magnus; for the Swedish Idiopathic Pulmonary Fibrosis Registry Group

    2016-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is an emerging problem in the western world, being related to increasing age and implying significant costs for the diagnosis and management of affected patients. The epidemiology of IPF is not well understood. Methods To allow estimates of the problem and eventually to evaluate quality of the care of IPF patients in Sweden, a national IPF Registry was started in the autumn of 2014. Data on criteria used to diagnose IPF, demographics, lung function, and quality of life (measured with the King's Brief Interstitial Lung Disease Questionnaire, K-BILD) were reported directly to the registry, based at the coordinating centre (Karolinska University Hospital, Stockholm, Sweden) via a web-based platform. Results During the first year, the registry was implemented in 11 (33%) of the 33 respiratory units in the country. Seventy-one patients were registered between October 2014 and October 2015, 50 (70.4%) males and 21 (29.6%) females. Median age was 70 (range 47–86). The mean K-BILD score at the first inclusion in the registry was 54.3+9.5. Conclusions The main features of IPF patients in this first Swedish cohort were consistent with data published in the literature in main multinational randomized controlled trials. The K-BILD questionnaire showed that quality of life of patients with IPF and their perception of the disease are quite poor at the time of inclusion in the registry. PMID:27105945

  9. Associations between EBV serostatus and organ transplant type in PTLD risk: an analysis of the SRTR National Registry Data in the United States.

    PubMed

    Dharnidharka, V R; Lamb, K E; Gregg, J A; Meier-Kriesche, H-U

    2012-04-01

    In a prior multiorgan transplant database study, recipient Epstein-Barr virus (EBV) seronegativity was not associated with increased risk for posttransplant lymphoproliferative disorders (PTLD) in liver transplants (LTX), at variance with prior single center reports and with data from kidney and heart transplants (KTX and HTX). The Scientific Registry of Transplant Recipients (SRTR) in the United States is the only other registry with data on the required variables for comparison.Our study set comprised 112 756 KTX (580 PTLDs; 0.51%), 13 937 HTX (140 PTLDs; 1.0%) and 40 437 LTX (383 PTLDs; 0.95%) performed January 2003 onward. The unadjusted hazard ratio (HR) for PTLD if recipient EBV seronegative was 5.005 for KTX, 6.528 for HTX and 2.615 for LTX (p < 0.001 for all). In models adjusted for multiple covariates, the adjusted HR was 3.583 (p < 0.001) for KTX, 4.037 (p < 0.001) for HTX, 1.479 (p = 0.03) for LTX. Interaction models using EBV seropositive KTX as reference group showed significantly higher risk for all other EBV seronegative organ transplant groups and also for EBV seropositive LTX (AHR 2.053, p < 0.0001).Recipient EBV seronegativity is still significantly associated with risk for PTLD in LTX, though less so because of higher baseline risk in the EBV seropositive LTX group.

  10. The 2008 ERA–EDTA Registry Annual Report—a précis

    PubMed Central

    Stel, Vianda S.; van de Luijtgaarden, Moniek W.M.; Wanner, Christoph; Jager, Kitty J.

    2011-01-01

    Background. This study provides a summary of the 2008 ERA–EDTA Registry Report (this report is available at www.era-edta-reg.org). Methods. The data on renal replacement therapy (RRT) were available from 55 national and regional registries in 30 countries in Europe and bordering the Mediterranean Sea. Datasets with individual patient data were received from 36 registries, whereas 19 registries contributed data in aggregated form. We presented incidence and prevalence of RRT, and transplant rates. Survival analysis was solely based on individual patient records. Results. In 2008, the overall incidence rate of RRT for end-stage renal disease (ESRD) among all registries reporting to the ERA–EDTA Registry was 122 per million population (pmp), and the prevalence was 644 pmp. Incidence rates varied from 264 pmp in Turkey to 15 pmp in Ukraine. The mean age of patients starting RRT in 2008 ranged from 69 years in Dutch-speaking Belgium to 44 years in Ukraine. The highest prevalence of RRT for ESRD was reported by Portugal (1408 pmp) and the lowest by Ukraine (89 pmp). The prevalence of haemodialysis on 31 December 2008 ranged from 66 pmp (Ukraine) to 875 pmp (Portugal) and the prevalence of peritoneal dialysis from 8 pmp (Montenegro) to 115 pmp (Denmark). In Norway, 70% of the patients on RRT on 31 December 2008 were living with a functioning graft (572 pmp). In 2008, the number of transplants performed pmp was highest in Spain (Catalonia) (64 pmp), whereas the highest transplant rates with living-donor kidneys were reported from the Netherlands (25 pmp) and Norway (21 pmp). In the cohort 1999–2003, the unadjusted 1-, 2- and 5-year survival of patients on RRT was 80.8% (95% CI: 80.6–81.0), 69.1% (95% CI: 68.9–69.3) and 46.1% (95% CI: 45.9–46.3), respectively. PMID:21245934

  11. Multicenter Breast Cancer Collaborative Registry

    PubMed Central

    Sherman, Simon; Shats, Oleg; Fleissner, Elizabeth; Bascom, George; Yiee, Kevin; Copur, Mehmet; Crow, Kate; Rooney, James; Mateen, Zubeena; Ketcham, Marsha A.; Feng, Jianmin; Sherman, Alexander; Gleason, Michael; Kinarsky, Leo; Silva-Lopez, Edibaldo; Edney, James; Reed, Elizabeth; Berger, Ann; Cowan, Kenneth

    2011-01-01

    The Breast Cancer Collaborative Registry (BCCR) is a multicenter web-based system that efficiently collects and manages a variety of data on breast cancer (BC) patients and BC survivors. This registry is designed as a multi-tier web application that utilizes Java Servlet/JSP technology and has an Oracle 11g database as a back-end. The BCCR questionnaire has accommodated standards accepted in breast cancer research and healthcare. By harmonizing the controlled vocabulary with the NCI Thesaurus (NCIt) or Systematized Nomenclature of Medicine-Clinical Terms (SNOMED-CT), the BCCR provides a standardized approach to data collection and reporting. The BCCR has been recently certified by the National Cancer Institute’s Center for Biomedical Informatics and Information Technology (NCI CBIIT) as a cancer Biomedical Informatics Grid (caBIG®) Bronze Compatible product. The BCCR is aimed at facilitating rapid and uniform collection of critical information and biological samples to be used in developing diagnostic, prevention, treatment, and survivorship strategies against breast cancer. Currently, seven cancer institutions are participating in the BCCR that contains data on almost 900 subjects (BC patients and survivors, as well as individuals at high risk of getting BC). PMID:21918596

  12. Registry of hemophilia and other bleeding disorders in Syria.

    PubMed

    Ali, T; Schved, J F

    2012-11-01

    Creating a national registry for bleeding disorders is a major step in establishing a National Hemophilia Care Program in all countries. Creating such a registry which would contain accurate and regularly updated data, including laboratory analysis confirmed by a reference laboratory established at the Syrian Hemophilia Society. Blood samples were drawn and analysed in the Society reference laboratory for the following screening tests: prothrombin time (PT), APTT and coagulation factor assays. Inhibitor detection and VWF RiCof were performed depending on the result of the screening tests. HBs Ag, anti-HCV, anti-HIV 1+2 and syphilis tests were also performed to detect transfusion transmitted agents (TTA). Diagnosis of the bleeding disorder type was confirmed for 760 of these cases. Among the 760 confirmed patients, 82.5% had haemophilia. Among these, 89.6%were haemophilia A; 10.4% were haemophilia B; 8.3% had VWD; 9.2% had other rare bleeding disorders as follows: 1.2% FVII deficiency, 0.7% FV deficiency, 1.8% F1 deficiency, 0.4% FX deficiency, 1.4% platelets dysfunctions (mainly Glanzmann Thrombasthenia) and 3.7% had combined FVIII and FV deficiency. Eighty (21.3%) cases of 375 screened for transfusion transmitted agents were positive for at least one infection: 0.5% were HBsAg positive, 19.7% were anti-HCV positive, 0.8% had combined HBsAg and anti-HCV positivity and 0.3% was anti-Syphilis positive. All patients were negative for HIV1 and HIV2. The preliminary data presented here follow known data on haemophilia A, haemophilia B and VWD disease. This registry will certainly help in improving haemophilia care in Syria.

  13. Establishing a population-based patient-reported outcomes study (PROMs) using national cancer registries across two jurisdictions: the Prostate Cancer Treatment, your experience (PiCTure) study

    PubMed Central

    Drummond, F J; Kinnear, H; Donnelly, C; O'Leary, E; O'Brien, K; Burns, R M; Gavin, A; Sharp, L

    2015-01-01

    Objective To establish an international patient-reported outcomes (PROMs) study among prostate cancer survivors, up to 18 years postdiagnosis, in two countries with different healthcare systems and ethical frameworks. Design A cross-sectional, postal survey of prostate cancer survivors sampled and recruited via two population-based cancer registries. Healthcare professionals (HCPs) evaluated patients for eligibility to participate. Questionnaires contained validated instruments to assess health-related quality of life and psychological well-being, including QLQ-C30, QLQ-PR25, EQ-5D-5L, 21-question Depression, Anxiety and Stress Scale (DASS-21) and the Decisional Regret Scale. Setting Republic of Ireland (RoI) and Northern Ireland (NI). Primary outcome measures Registration completeness, predictors of eligibility and response, data missingness, unweighted and weighted PROMs. Results Prostate cancer registration was 80% (95% CI 75% to 84%) and 91% (95% CI 89% to 93%) complete 2 years postdiagnosis in NI and RoI, respectively. Of 12 322 survivors sampled from registries, 53% (n=6559) were classified as eligible following HCP screening. In the multivariate analysis, significant predictors of eligibility were: being ≤59 years of age at diagnosis (p<0.001), short-term survivor (<5 years postdiagnosis; p<0.001) and from RoI (p<0.001). 3348 completed the questionnaire, yielding a 54% adjusted response rate. 13% of men or their families called the study freephone with queries for assistance with questionnaire completion or to talk about their experience. Significant predictors of response in multivariate analysis were: being ≤59 years at diagnosis (p<0.001) and from RoI (p=0.016). Mean number of missing questions in validated instruments ranged from 0.12 (SD 0.71; EQ-5D-5L) to 3.72 (SD 6.30; QLQ-PR25). Weighted and unweighted mean EQ-5D-5L, QLQ-C30 and QLQ-PR25 scores were similar, as were the weighted and unweighted prevalences of depression, anxiety and

  14. Malaysian Psoriasis Registry--preliminary report of a pilot study using a newly revised registry form.

    PubMed

    Chang, C C; Gangaram, H B; Hussein, S H

    2008-09-01

    The Malaysian Psoriasis Registry, established in 1998, is the first skin disease clinical registry in Malaysia. It aims to provide useful data on various aspects of psoriasis. Following an extensive revision of the registry form in 2007, a total of 509 psoriasis patients from 10 government dermatologic centres were reviewed in a three month pilot study. The onset of psoriasis was during the second to fourth decade of life in the majority of patients. There was no sexual and ethnic predilection. A positive family history was present in 21.2%, and more common in patients with younger disease onset. The main aggravating factors of psoriasis were stress, sunlight and infection. Plaque psoriasis was the commonest clinical type (80.9%). Joint disease was present in 17.3% of patients, among which mono-/oligoarticular type being the commonest. Nail changes occurred in 68%. More psoriasis patients were overweight and obese compared to the normal population. The mean Dermatologic Life Quality Index (DLQI) score was 8.08 +/- 6.29, and changes during subsequent follow-up may reflect therapeutic effectiveness. This study enabled evaluation of the revised registry form and helped in identifying shortcomings in the implementation of the registry.

  15. Epidemiology and management of cardiac arrest: what registries are revealing.

    PubMed

    Gräsner, Jan-Thorsten; Bossaert, Leo

    2013-09-01

    Major European institutions report cardiovascular disease (CVD) as the first cause of death in adults, with cardiac arrest and sudden death due to coronary ischaemia as the primary single cause. Global incidence of CVD is decreasing in most European countries, due to prevention, lifestyle and treatment. Mortality of acute coronary events inside the hospital decreases more rapidly than outside the hospital. To improve the mortality of cardiac arrest outside the hospital, reliable epidemiological and process figures are essential: "we can only manage what we can measure". Europe is a patchwork of 47 countries (total population of 830 million), with a 10-fold difference in incidence of coronary heart disease between North and South, East and West, and a 5-fold difference in number of EMS-treated cardiac arrest (range 17-53/1000,000/year). Epidemiology of cardiac arrest should not be calculated as a European average, but it is appropriate to describe the incidence of cardiac arrest, the resuscitation process, and the outcome in each of the European regions, for benchmarking and quality management. Epidemiological reports of cardiac arrest should specify definitions, nominator (number of cases) and denominator (study population). Recently some regional registries in North America, Japan and Europe fulfilled these conditions. The European Registry of Cardiac Arrest (EuReCa) has the potential to achieve these objectives on a pan-European scale. For operational applications, the Utstein definition of "Cardiac arrest" is used which includes the potential of survival. For application in community health, the WHO definition of "sudden death" is frequently used, describing the mode of death. There is considerable overlap between both definitions. But this explains that no single method can provide all information. Integrating data from multiple sources (local, national, multinational registries and surveys, death certificates, post-mortem reports, community statistics, medical

  16. United States Transuranium and Uranium Registries. Annual report

    SciTech Connect

    Kathren, R.

    1993-02-28

    The United States Transuranium and Uranium Registries are unique human tissue research programs studying the distribution, dose, and possible biological effects of the actinide elements in man, with the primary goal of assuring the adequacy of radiation protection standards for these radionuclides. The Registries research is based on radiochemical analysis of tissues collected at autopsy from voluntary donors who have documented occupational exposure to the actinides. To date, tissues, or in some cases radioanalytical results only, have been obtained from approximately 300 individuals; another 464 living individuals have volunteered to participate in the Registries research programs and have signed premortem informed consent and autopsy permissions. The Registries originated at the National Plutonium Registry which was started in 1968 as a then Atomic Energy Commission project under the aegis of a prime contractor at the Hanford site. In 1970, the name was changed to the United States Transuranium Registry to reflect a broader involvement with the higher actinides. In 1978, an administratively separate parallel registry, the United States Uranium Registry, was formed to carry out similar studies among uranium fuel cycle workers.

  17. National Tay-Sachs and Allied Diseases Association, Inc.

    ERIC Educational Resources Information Center

    Exceptional Parent, 1977

    1977-01-01

    Reviewed are the history and organization, purpose and programs, and public services of the National Tay-Sachs and Allied Diseases Association, an organization geared toward eradicating Tay-Sachs disease (a hereditary disorder affecting primarily Jewish infants which generally leads to deterioration and death by the child's fifth year). (SBH)

  18. 77 FR 50139 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-08-20

    ... Diseases Special Emphasis Panel; Partnerships for Biodefense-- Diagnostics 1. Date: September 13, 2012... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special...

  19. 78 FR 28859 - National Institute of Diabetes and Digestive and Kidney Diseases Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-05-16

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... and Kidney Diseases Initial Review Group; Diabetes, Endocrinology and Metabolic Diseases B... Committee: National Institute of Diabetes and Digestive and Kidney Diseases Initial Review Group;...

  20. 77 FR 28890 - National Institute of Diabetes and Digestive and Kidney Diseases Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-05-16

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group; Kidney, Urologic and Hematologic Diseases D Subcommittee. Date... . Name of Committee: National Institute of Diabetes and Digestive and Kidney Diseases Initial...

  1. 75 FR 3741 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-22

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group; Kidney, Urologic and Hematologic Diseases D Subcommittee. Date... . Name of Committee: National Institute of Diabetes and Digestive and Kidney Diseases Initial...

  2. 78 FR 9063 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-07

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group; Kidney, Urologic and Hematologic Diseases D Subcommittee. Date... . Name of Committee: National Institute of Diabetes and Digestive and Kidney Diseases Initial...

  3. 77 FR 11140 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-24

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis Panel; Host Targeted Interventions As Therapeutics For Infectious Diseases. Date: March 13, 2012....

  4. 78 FR 12767 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-25

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis..., Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases Research,...

  5. 77 FR 59940 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-01

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... Allergy and Infectious Diseases Special Emphasis Panel; Targeting Inflammation and Immune Activations in... Infectious Diseases Research, National Institutes of Health, HHS) Dated: September 24, 2012. David...

  6. 77 FR 59940 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-01

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  7. 78 FR 24761 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-26

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis..., Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases...

  8. 78 FR 19276 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-29

    ..., Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases Research... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special...

  9. 78 FR 23771 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-22

    ....855, Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special...

  10. 77 FR 21789 - National Institute Of Allergy And Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-04-11

    ... HUMAN SERVICES National Institutes of Health National Institute Of Allergy And Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis..., Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases...

  11. 76 FR 6626 - National Institute of Allergy and Infectious Diseases; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-07

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases.... App.), notice is hereby given of meetings of the National Advisory Allergy and Infectious Diseases... Advisory Allergy and Infectious Diseases Council; Allergy, Immunology and Transplantation...

  12. 76 FR 81954 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-29

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Allergy and Infectious Diseases Special Emphasis Panel, Therapeutics for Neurotropic Biodefense Toxins...

  13. 78 FR 76847 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-19

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Allergy and Infectious Diseases Special Emphasis Panel; Limited Competition--Multicenter AIDS Cohort...

  14. 78 FR 63999 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-10-25

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Allergy and Infectious Diseases Special Emphasis Panel; Mechanisms of Cellular Immunity in the...

  15. 77 FR 16247 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-03-20

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... Allergy and Infectious Diseases, including consideration of personnel qualifications and performance, and... of Intramural Research, National Institute of Allergy and Infectious Diseases, NIH, Building 31,...

  16. 78 FR 19275 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-29

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases, including consideration of personnel qualifications and performance, and the competence..., National Institute of Diabetes and Digestive, and Kidney Diseases, National Institute of Health, Building...

  17. 77 FR 53208 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-08-31

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases, including consideration of personnel qualifications and performance, and the competence..., National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Health, Building...

  18. 78 FR 63996 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

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    2013-10-25

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... review and funding cycle. Name of Committee: National Institute of Allergy and Infectious...

  19. Towards better implementation of cancer screening in Europe through improved monitoring and evaluation and greater engagement of cancer registries.

    PubMed

    Anttila, Ahti; Lönnberg, Stefan; Ponti, Antonio; Suonio, Eero; Villain, Patricia; Coebergh, Jan Willem; von Karsa, Lawrence

    2015-01-01

    Proposals to improve implementation, monitoring and evaluation of breast, cervical and colorectal cancer screening programmes have been developed in a European project involving scientists and professionals experienced in cancer registration (EUROCOURSE). They call for a clear and more active role for cancer registries through better interfaces with cancer screening programmes and adapting data contents of cancer registries for evaluation purposes. Cancer registries are recognised as essential for adequate evaluation of cancer screening programmes, but they are not involved in screening evaluation in several European countries. This is a key barrier to improving the effectiveness of programmes across Europe. The variation in Europe in the implementation of cancer screening offers a unique opportunity to learn from best practices in collaboration between cancer registries and screening programmes. Population-based cancer registries have experience and tools in collecting and analysing relevant data, e.g. for diagnostic and therapeutic determinants of mortality. In order to accelerate improvements in cancer control we argue that cancer registries should take co-responsibility in promoting effective screening evaluation in Europe. Additional investments are vital to further development of infrastructures and activities for screening evaluation and monitoring in the national settings and also at the pan-European level. The EUROCOURSE project also aimed to harmonise implementation of the European quality assurance guidelines for cancer screening programmes across Europe through standardising routine data collection and analysis, and definitions for key performance indicators for screening registers. Data linkage between cancer and screening registers and other repositories of demographic data and cause of death and where available clinical registers is key to implementing the European screening standards and thereby reducing the burden of disease through early detection

  20. Establishment of electronic chart-based stroke registry system in a medical system in Taiwan.

    PubMed

    Lee, Tsong-Hai; Chang, Chien-Hung; Chang, Yeu-Jhy; Chang, Ku-Chou; Chung, Jacky

    2011-08-01

    To establish a prospective, real-time, self-sustainable stroke registry system, we incorporated a registry with an electronic chart to create an electronic chart-based stroke registry system in November 2006. The International Classification of Diseases Ninth Revision code (430-437) was used to auto-enroll stroke patients admitted to neurology departments. Clinical information was written by doctors, nursing information was recorded by nurses, and basic patient information was entered by administrative departments. Numerical data and the date and time of any studies were auto-downloaded from the hospital computer. In total, 212 items were auto-downloaded, including basic patient information, laboratory blood test and examination results, and the date and time of imaging and special intervention. The stroke scales (121 items, National Institutes of Health Stroke Scale, Barthel index, and modified Rankin scale) were designed to be auto-adjusted to reduce incompatibility. The 95 items with pull-down options were used to specify the contents. This registry system can be time-, labor- and money-saving with secured data accuracy.

  1. Modest familial risks for multiple sclerosis: a registry-based study of the population of Sweden.

    PubMed

    Westerlind, Helga; Ramanujam, Ryan; Uvehag, Daniel; Kuja-Halkola, Ralf; Boman, Marcus; Bottai, Matteo; Lichtenstein, Paul; Hillert, Jan

    2014-03-01

    Data on familial recurrence rates of complex diseases such as multiple sclerosis give important hints to aetiological factors such as the importance of genes and environment. By linking national registries, we sought to avoid common limitations of clinic-based studies such as low numbers, poor representation of the population and selection bias. Through the Swedish Multiple Sclerosis Registry and a nationwide hospital registry, a total of 28 396 patients with multiple sclerosis were identified. We used the national Multi-Generation Registry to identify first and second degree relatives as well as cousins, and the Swedish Twin Registry to identify twins of patients with multiple sclerosis. Crude and age corrected familial risks were estimated for cases and found to be in the same range as previously published figures. Matched population-based controls were used to calculate relative risks, revealing lower estimates of familial multiple sclerosis risks than previously reported, with a sibling recurrence risk (λs = 7.1; 95% confidence interval: 6.42-7.86). Surprisingly, despite a well-established lower prevalence of multiple sclerosis amongst males, the relative risks were equal among maternal and paternal relations. A previously reported increased risk in maternal relations could thus not be replicated. An observed higher transmission rate from fathers to sons compared with mothers to sons suggested a higher transmission to offspring from the less prevalent sex; therefore, presence of the so-called 'Carter effect' could not be excluded. We estimated the heritability of multiple sclerosis using 74 757 twin pairs with known zygosity, of which 315 were affected with multiple sclerosis, and added information from 2.5 million sibling pairs to increase power. The heritability was estimated to be 0.64 (0.36-0.76), whereas the shared environmental component was estimated to be 0.01 (0.00-0.18). In summary, whereas multiple sclerosis is to a great extent an inherited trait

  2. 76 FR 8753 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-15

    ...: National Institute of Allergy and Infectious Diseases Special Emphasis Panel; ``Integrated Preclinical... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  3. The Swiss Orthopaedic Registry.

    PubMed

    Röder, Christoph; El-Kerdi, A; Frigg, A; Kolling, C; Staub, L P; Bach, B; Müller, U

    2005-01-01

    Following the tradition of the IDES European Hip Registry inaugurated by M. E. Müller in the 1960s, the Institute for Evaluative Research in Orthopaedic Surgery at the University of Bern started a new era of data collection using internet technology (www.memdoc.org). With support of the Swiss Orthopaedic Society, the pilot of the Swiss Orthopaedic Registry was conducted, and in cooperation with different academic and non-academic centers the practicability of integrating the various data collection instruments into the daily clinical workflow was evaluated. Three different sizes of hip and knee questionnaires were compiled, covering the individual demands of the participating hospitals whereby the smaller questionnaires always represent a subset of the next larger one. Different types of data collection instruments are available: the online interface, optical mark reader paper questionnaires, and barcode sheets. Precise implant tracking is implemented by scanning the implant barcodes directly in the operating theaters and linking them to the clinical data set via a central server. In addition, radiographic information can be linked with the clinical data set. The pilot clinics suggested enhancements to the user interface and additional features for data management. Also, recommendations were made to simplify content in some instances and diversify in others. With a new software release and adapted questionnaires the Swiss Orthopaedic Registry was officially launched in Summer 2005.

  4. The draft "National Plan" to address Alzheimer's disease - National Alzheimer's Project Act (NAPA).

    PubMed

    Khachaturian, Zaven S; Khachaturian, Ara S; Thies, William

    2012-05-01

    This perspective updates the status of the "National Plan to Address Alzheimer's Disease" and the recommendations of the NAPA Advisory Council's Sub-committee on Research. Here, we identify some of the critical issues the future reiterations of the National Plan should consider during implementation phase of the plan. The Journal invites the scientific community to contribute additional ideas and suggestions towards a national research initiative.

  5. 45 CFR 2540.203 - When must I conduct a State criminal registry check and a National Sex Offender Public Web site...

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... check and a National Sex Offender Public Web site check on an individual in a covered position? 2540.203... National Sex Offender Public Web site check on an individual in a covered position? (a) The State criminal... enrolls in, or is hired by, your program on or after October 1, 2009. (b) The National Sex Offender...

  6. 45 CFR 2540.203 - When must I conduct a State criminal registry check and a National Sex Offender Public Web site...

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... check and a National Sex Offender Public Web site check on an individual in a covered position? 2540.203... National Sex Offender Public Web site check on an individual in a covered position? (a) The State criminal... enrolls in, or is hired by, your program on or after October 1, 2009. (b) The National Sex Offender...

  7. 45 CFR 2540.203 - When must I conduct a State criminal registry check and a National Sex Offender Public Web site...

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... check and a National Sex Offender Public Web site check on an individual in a covered position? 2540.203... National Sex Offender Public Web site check on an individual in a covered position? (a) The State criminal... enrolls in, or is hired by, your program on or after October 1, 2009. (b) The National Sex Offender...

  8. National Institutes of Health Osteoporosis and Related Bone Diseases~National Resource Center

    MedlinePlus

    ... Language Publications (en español) | Asian-Language Publications NIH Osteoporosis and Related Bone Diseases ~ NIH National Resource Center ... Font Size | S S M M L L Bone Basics Osteoporosis Osteogenesis Imperfecta Paget’s Disease of Bone Related Topics ...

  9. Autologous hematopoietic stem cell transplantation for pediatric multiple sclerosis: a registry-based study of the Autoimmune Diseases Working Party (ADWP) and Pediatric Diseases Working Party (PDWP) of the European Society for Blood and Marrow Transplantation (EBMT).

    PubMed

    Burman, J; Kirgizov, K; Carlson, K; Badoglio, M; Mancardi, G L; De Luca, G; Casanova, B; Ouyang, J; Bembeeva, R; Haas, J; Bader, P; Snowden, J; Farge, D

    2017-03-20

    Autologous hematopoietic stem cell transplantation (aHSCT) is a promising therapy for multiple sclerosis (MS), which has mainly been used in adults. The purpose of this study was to investigate efficacy and adverse events of aHSCT in the treatment of children with MS using data from the European Society for Blood and Marrow Transplantation registry. Twenty-one patients with a median follow-up time of 2.8 years could be identified. PFS at 3 years was 100%, 16 patients improved in expanded disability status scale score and only 2 patients experienced a clinical relapse. The procedure was generally well tolerated and only two instances of severe transplant-related toxicity were recorded. There was no treatment-related mortality, although one patient needed intensive care. aHSCT may be a therapeutic option for children with disease that does not respond to standard care.Bone Marrow Transplantation advance online publication, 20 March 2017; doi:10.1038/bmt.2017.40.

  10. Prospects for designating Alzheimer's disease research a national priority.

    PubMed

    Khachaturian, Zaven S

    2011-11-01

    This editorial evaluates the prospects of the National Alzheimer's Project Act (NAPA) succeeding to shape public policies that would substantially increase national expenditures for research on Alzheimer's disease. The essay identifies, in the context of 30-year history, some of the difficult challenges the NAPA Advisory Council must address and offers specific recommendations for an action plan by the Secretary, Department of Health and Human Services (DHHS).

  11. Quality control and assurance in hematopoietic stem cell transplantation data registries in Japan and other countries.

    PubMed

    Kuwatsuka, Yachiyo

    2016-01-01

    Observational studies from national and international registries with large volumes of patients are commonly performed to identify superior strategies for hematopoietic stem cell transplantation. Major international and national stem cell transplant registries collect outcome data using electronic data capture systems, and a systematic study support process has been developed. Statistical support for studies is available from some major international registries, and international and national registries also mutually collaborate to promote stem cell transplant outcome studies and transplant-related activities. Transplant registries additionally take measures to improve data quality to further improve the quality of outcome studies by utilizing data capture systems and manual data management. Data auditing can potentially even further improve data quality; however, human and budgetary resources can be limiting factors in system construction and audits of the Japanese transplant registry are not currently performed.

  12. 76 FR 13414 - Disease, Disability, and Injury Prevention and Control Special Emphasis Panel (SEP): Pilot...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-11

    ... HUMAN SERVICES Centers for Disease Control and Prevention Disease, Disability, and Injury Prevention and... Blackman, Ph.D., Scientific Review Officer, CDC, National Center for Chronic Disease Prevention and Health... Disease Control and Prevention and the Agency for Toxic Substances and Disease Registry. Dated: March...

  13. Analysis of data from the ERA-EDTA Registry indicates that conventional treatments for chronic kidney disease do not reduce the need for renal replacement therapy in autosomal dominant polycystic kidney disease.

    PubMed

    Spithoven, Edwin M; Kramer, Anneke; Meijer, Esther; Orskov, Bjarne; Wanner, Christoph; Caskey, Fergus; Collart, Frederic; Finne, Patrik; Fogarty, Damian G; Groothoff, Jaap W; Hoitsma, Andries; Nogier, Marie-Béatrice; Postorino, Maurizio; Ravani, Pietro; Zurriaga, Oscar; Jager, Kitty J; Gansevoort, Ron T

    2014-12-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of end-stage kidney failure, but is often identified early and therefore amenable to timely treatment. Interventions known to postpone the need for renal replacement therapy (RRT) in non-ADPKD patients have also been tested in ADPKD patients, but with inconclusive results. To help resolve this we determined changes in RRT incidence rates as an indicator for increasing effective renoprotection over time in ADPKD. We analyzed data from the European Renal Association-European Dialyses and Transplant Association Registry on 315,444 patients starting RRT in 12 European countries between 1991 and 2010, grouped into four 5-year periods. Of them, 20,596 were due to ADPKD. Between the first and last period the mean age at onset of RRT increased from 56.6 to 58.0 years. The age- and gender-adjusted incidence rate of RRT for ADPKD increased slightly over the four periods from 7.6 to 8.3 per million population. No change over time was found in the incidence of RRT for ADPKD up to age 50, whereas in recent time periods the incidence in patients above the age of 70 clearly increased. Among countries there was a significant positive association between RRT take-on rates for non-ADPKD kidney disease and ADPKD. Thus, the increased age at onset of RRT is most likely due to an increased access for elderly ADPKD patients or lower competing risk prior to the start of RRT rather than the consequence of effective emerging renoprotective treatments for ADPKD.

  14. 76 FR 28443 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-05-17

    ...: Microbiology, Infectious Diseases and AIDS Initial Review Group, Microbiology and Infectious Diseases Research... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  15. The New ADL Registry. ADL Registry Web Portal Changes

    DTIC Science & Technology

    2009-08-19

    Approaches 18 19 Primary ADL Registry Contributors Contributor Records Entry Date Navy eLearning (US Navy) 2,086 08/05/2008 Joint Knowledge Development...ADL Registry  http://adlregistry.adlnet.gov/  Navy eLearning Content Team  https://www.netc.navy.mil/ile  Joint Knowledge Online  http

  16. The growing number of hemophilia registries: Quantity vs. quality.

    PubMed

    Keipert, C; Hesse, J; Haschberger, B; Heiden, M; Seitz, R; van den Berg, H M; Hilger, A

    2015-05-01

    Registries for rare diseases provide a tool for obtaining an overview of the clinical situation and can be used to discover points of improvement and to monitor long-term safety. Registries could also become a powerful tool to provide supporting information for marketing authorization. There is an urgent need for a pan-European or global strategy that supports consistent data. Therefore, transparency in data collection, harmonization of the database structures, and the convergence of scientific approaches are required.

  17. 76 FR 11799 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-03

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases...: National Institute of Allergy and Infectious Diseases Special Emphasis Panel; Host-Pathogen...

  18. 75 FR 9911 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-04

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel, Seeding Team Science in Diabetes Endocrinology and Metabolic... of Committee: National Institute of Diabetes and Digestive and Kidney Diseases Special Emphasis...

  19. 75 FR 62546 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-12

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis Panel, Partnership for Development of New Therapeutics Classes for select Viral and Bacterial...

  20. 75 FR 62553 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-12

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis Panel; Partnership for Development of New Therapeutics Classes for Select Viral and Bacterial...

  1. 76 FR 11499 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-02

    ... Intramural Research, National Institute of Diabetes and Digestive, and Kidney Diseases, NIH, Bethesda, MD... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases, including consideration of personnel qualifications and performance, and the...

  2. 78 FR 9404 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-08

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... of Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and Transplantation...

  3. 78 FR 18996 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-28

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis.... (Catalogue of Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and...

  4. 76 FR 5596 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-02-01

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis....855, Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious...

  5. 78 FR 60294 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-10-01

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... . (Catalogue of Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and...

  6. 76 FR 64358 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-18

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and Transplantation Research;...

  7. 78 FR 63997 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-10-25

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and Transplantation Research;...

  8. 75 FR 65021 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-21

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... . (Catalogue of Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and...

  9. 76 FR 61719 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-05

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and Transplantation Research;...

  10. 75 FR 7486 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-02-19

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis....855, Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious...

  11. 77 FR 5035 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-01

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... . (Catalogue of Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and...

  12. 76 FR 70155 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-11-10

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis....855, Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious...

  13. 75 FR 49498 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-13

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... . (Catalogue of Federal Domestic Assistance Program Nos. 93.855, Allergy, Immunology, and...

  14. The National Tay Sachs and Allied Diseases Association.

    ERIC Educational Resources Information Center

    Zeitlin, Paula

    1986-01-01

    The National Tay-Sachs and Allied Diseases Association is involved in education, research, and prevention of Tay-Sachs, an inherited metabolic disorder which destroys the central nervous system, and over 30 related disorders. The group features a parent peer group network and a support group for carrier couples. (CL)

  15. Renal replacement therapy in Europe—a summary of the 2010 ERA–EDTA Registry Annual Report

    PubMed Central

    Kramer, Anneke; Stel, Vianda S.; Abad Diez, José Maria; Alonso de la Torre, Ramón; Bouzas Caamaño, Encarnación; Čala, Svjetlana; Cao Baduell, Higini; Castro de la Nuez, Pablo; Cernevskis, Harijs; Collart, Frederic; Couchoud, Cécile; de Meester, Johan; Djukanovic, Ljubica; Ferrer-Alamar, Manuel; Finne, Patrik; Fogarty, Damian; de los Ángeles García Bazaga, María; Garneata, Liliana; Golan, Eliezer; Gonzalez Fernández, Raquel; Heaf, James G.; Hoitsma, Andries; Ioannidis, George A.; Kolesnyk, Mykola; Kramar, Reinhard; Leivestad, Torbjørn; Limido, Aurelio; Lopot, Frantisek; Macario, Fernando; Magaz, Ángela; Martín-Escobar, Eduardo; Metcalfe, Wendy; Noordzij, Marlies; Ots-Rosenberg, Mai; Palsson, Runolfur; Piñera, Celestino; Postorino, Maurizio; Prutz, Karl G.; Ratkovic, Marina; Resic, Halima; Rodríguez Hernández, Aurelio; Rutkowski, Boleslaw; Serdengeçti, Kamil; Yebenes, Tomas Sierra; Spustová, Viera; Stojceva-Taneva, Olivera; Tomilina, Natalia A.; van de Luijtgaarden, Moniek W.M.; van Stralen, Karlijn J.; Wanner, Christoph; Jager, Kitty J.

    2013-01-01

    Background This study provides a summary of the 2010 European Renal Association–European Dialysis and Transplant Association (ERA–EDTA) Registry Annual Report (available at www.era-edta-reg.org). Methods This report includes data on renal replacement therapy (RRT) using data from the national and regional renal registries in 29 countries in Europe and bordering the Mediterranean Sea. Individual patient data were received from 27 registries, whereas 18 registries contributed data in aggregated form. We present incidence and prevalence of RRT, transplant rates, survival probabilities and expected remaining lifetimes. The latter two are solely based on individual patient records. Results In 2010, the overall incidence rate of RRT for end-stage renal disease (ESRD) among all registries reporting to the ERA–EDTA Registry was 123 per million population (pmp) (n = 91 798). The highest incidence rate was reported by Turkey (252pmp) and the lowest reported by Montenegro (21 pmp). The overall prevalence of RRT for ESRD at 31 December 2010 among all registries reporting to the ERA–EDTA Registry was 741 pmp (n = 551 005). The prevalence varied from 124 pmp in Ukraine to 1580 pmp in Portugal. The overall number of renal transplantations performed in 2010 among all registries was 29.2 pmp (n = 21 740). The highest overall transplant rate was reported from Spain, Cantabria (73 pmp), whereas the highest transplant rate for living donor kidneys was reported from the Netherlands (28 pmp). For patients who started RRT between 2001 and 2005, the unadjusted 5-year patient survival on RRT was 46.2% [95% confidence interval (CI) 46.0–46.3], and on dialysis 38.6% (95% CI 38.5–38.8). The unadjusted 5-year patient survival after the first renal transplantation performed between 2001 and 2005 was 86.6% (95% CI 86.1–87.1) for deceased donor kidneys and 94.1% (95% CI 93.4–94.8) for living donor kidneys. PMID:27818766

  16. International Quotidian Dialysis Registry: Annual report 2010.

    PubMed

    Lindsay, Robert M; Suri, Rita S; Moist, Louise M; Garg, Amit X; Cuerden, Meaghan; Langford, Sarah; Hakim, Raymond; Ofsthun, Norma J; McDonald, Stephen P; Hawley, Carmel; Caskey, Ferqus J; Couchoud, Cecile; Awaraji, Christian; Nesrallah, Gihad E

    2011-01-01

    The International Quotidian Dialysis Registry (IQDR) is a global initiative designed to study practices and outcomes associated with the use of hemodialysis (HD) regimens of increased frequency and/or duration. The IQDR grew out of the initiative that lead to the randomized prospective studies of nocturnal HD and short hours daily dialysis vs. conventional thrice weekly HD that are conducted by the Frequent Hemodialysis Network sponsored by the National Institutes of Health. These 2 separate studies are drawing to a close and the first results are expected to be reported later this year. These studies use surrogate outcomes for their primary endpoints as they are not powered to look at outcomes of mortality and hospitalization. The IQDR attempts to aggregate long-term follow-up data from centers utilizing alternative HD regimens worldwide and will have adequate statistical power to examine those important outcomes. To date, the IQDR has enrolled patients from Canada, the United States, Australia, New Zealand, and France and has linked with commercial databases and national registries. This sixth annual report of the IQDR describes: (1) An update on the governance structure; (2) The recommendations made at the first general meetings of the IQDR Scientific Committee and Advisory Board; (3) The status of those recommendations; (4) A summary of current data sources and participating registries; (5) The status of recruitment to date; (6) The creation of a specific Canadian IQDR data set and; (7) The current research agenda.

  17. Economic assessment of central cancer registry operations, Part III: Results from 5 programs.

    PubMed

    Tangka, Florence; Subramanian, Sujha; Beebe, Maggie Cole; Trebino, Diana; Michaud, Frances

    2010-01-01

    In this article, we report results from the cost analysis of 5 central cancer registries funded by the National Program of Cancer Registries (NPCR). To estimate the true economic costs of operating a cancer registry, we used a cost-assessment tool (CAT) to collect data on all registry activities, not just those funded by the NPCR. Data were collected on actual, rather than budgeted, expenditures, including personnel, consultants, information technology (IT) support, and other factors influencing costs. Factors that can affect registry costs include the amount of consolidation from abstract to incident cases, the method of data reporting, the number of edits that must be performed manually versus electronically, and the amount of interstate data exchange required of a registry. Expenditures were allocated to specific surveillance and data enhancement and analysis activities. Our study confirmed that cost per case varies across registry activities. The cost of surveillance activities per case ranges from $24.79 to $95.78 while the cost of data enhancement and analysis registry activities per reported cancer case ranges from $2.91 to $9.32. Total cost per reported cancer case also varies, ranging from $30 to slightly more than $100, with a median of $45.84. Further research using data from all NPCR-funded registries is required to assess reasons for this variation. Information gained from such an assessment will improve efficiency in registry operations and provide data to better quantify the funding requirements for expanding registry activities.

  18. 76 FR 3147 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-01-19

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel; Fellowships in Digestive Diseases and Nutrition. Date: February... Institute of Diabetes and Digestive and Kidney Diseases Special Emphasis Panel; Lactation and...

  19. 77 FR 34395 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-11

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel; Medication Adherence among Children with CKD: Ancillary Studies... Diseases and Nutrition Research; 93.849, Kidney Diseases, Urology and Hematology Research,...

  20. 75 FR 3472 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-21

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... of Allergy and Infectious Diseases Special Emphasis Panel; NIAID Clinical Trial Planning (R34) Grants..., Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases...

  1. EPA Facility Registry Service (FRS): RADINFO

    EPA Pesticide Factsheets

    This web feature service contains location and facility identification information from EPA's Facility Registry Service (FRS) for the subset of facilities that link to the Radiation Information Database (RADINFO). RADINFO contains information about facilities that are regulated by EPA for radiation and radioactivity. FRS identifies and geospatially locates facilities, sites or places subject to environmental regulations or of environmental interest. Using vigorous verification and data management procedures, FRS integrates facility data from EPA's national program systems, other federal agencies, and State and tribal master facility records and provides EPA with a centrally managed, single source of comprehensive and authoritative information on facilities. This data set contains the subset of FRS integrated facilities that link to RADINFO facilities once the RADINFO data has been integrated into the FRS database. Additional information on FRS is available at the EPA website https://www.epa.gov/enviro/facility-registry-service-frs

  2. Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management.

    PubMed

    Bierzynska, Agnieszka; McCarthy, Hugh J; Soderquest, Katrina; Sen, Ethan S; Colby, Elizabeth; Ding, Wen Y; Nabhan, Marwa M; Kerecuk, Larissa; Hegde, Shivram; Hughes, David; Marks, Stephen; Feather, Sally; Jones, Caroline; Webb, Nicholas J A; Ognjanovic, Milos; Christian, Martin; Gilbert, Rodney D; Sinha, Manish D; Lord, Graham M; Simpson, Michael; Koziell, Ania B; Welsh, Gavin I; Saleem, Moin A

    2017-04-01

    Steroid Resistant Nephrotic Syndrome (SRNS) in children and young adults has differing etiologies with monogenic disease accounting for 2.9-30% in selected series. Using whole exome sequencing we sought to stratify a national population of children with SRNS into monogenic and non-monogenic forms, and further define those groups by detailed phenotypic analysis. Pediatric patients with SRNS were identified via a national United Kingdom Renal Registry. Whole exome sequencing was performed on 187 patients, of which 12% have a positive family history with a focus on the 53 genes currently known to be associated with nephrotic syndrome. Genetic findings were correlated with individual case disease characteristics. Disease causing variants were detected in 26.2% of patients. Most often this occurred in the three most common SRNS-associated genes: NPHS1, NPHS2, and WT1 but also in 14 other genes. The genotype did not always correlate with expected phenotype since mutations in OCRL, COL4A3, and DGKE associated with specific syndromes were detected in patients with isolated renal disease. Analysis by primary/presumed compared with secondary steroid resistance found 30.8% monogenic disease in primary compared with none in secondary SRNS permitting further mechanistic stratification. Genetic SRNS progressed faster to end stage renal failure, with no documented disease recurrence post-transplantation within this cohort. Primary steroid resistance in which no gene mutation was identified had a 47.8% risk of recurrence. In this unbiased pediatric population, whole exome sequencing allowed screening of all current candidate genes. Thus, deep phenotyping combined with whole exome sequencing is an effective tool for early identification of SRNS etiology, yielding an evidence-based algorithm for clinical management.

  3. Registry data trends of total ankle replacement use.

    PubMed

    Roukis, Thomas S; Prissel, Mark A

    2013-01-01

    Joint arthroplasty registry data are meaningful when evaluating the outcomes of total joint replacement, because they provide unbiased objective information regarding survivorship and incidence of use. Critical evaluation of the registry data information will benefit the surgeon, patient, and industry. However, the implementation and acceptance of registry data for total ankle replacement has lagged behind that of hip and knee implant arthroplasty. Currently, several countries have national joint arthroplasty registries, with only some procuring information for total ankle replacement. We performed an electronic search to identify publications and worldwide registry databanks with pertinent information specific to total ankle replacement to determine the type of prostheses used and usage trends over time. We identified worldwide registry data from 33 countries, with details pertinent to total ankle replacement identified in only 6 countries. The obtained information was arbitrarily stratified into 3 distinct periods: 2000 to 2006, 2007 to 2010, and 2011. Within these study periods, the data from 13 total ankle replacement systems involving 3,980 ankles were identified. The vast majority (97%) of the reported ankle replacements were 3-component, mobile-bearing, uncemented prostheses. Three usage trends were identified: initial robust embracement followed by abrupt disuse, minimal use, and initial embracement followed by sustained growth in implantation. Before the widespread acceptance of new total ankle replacements, the United States should scrutinize and learn from the international registry data and develop its own national joint registry that would include total ankle replacement. Caution against the adoption of newly released prostheses, especially those without readily available revision components, is recommended.

  4. Gender and Geographic Differences in the Prevalence of Autism Spectrum Disorders in Children: Analysis of Data from the National Disability Registry of Taiwan

    ERIC Educational Resources Information Center

    Lai, Der-Chung; Tseng, Yen-Cheng; Hou, Yuh-Ming; Guo, How-Ran

    2012-01-01

    The prevalence of autism spectrum disorders (ASD) in the world has increased dramatically in the recent decades. However, data at the national level are limited, and geographic differences are seldom evaluated. According to the law, the local governments in Taiwan began to certify disabled residents and provide various services in 1980, and the…

  5. 78 FR 31952 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-05-28

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases..., zhuqing.li@nih.gov . Name of Committee: National Institute of Allergy and Infectious Diseases Special... Committee: National Institute of Allergy and Infectious Diseases Special Emphasis Panel; Clinical...

  6. 77 FR 55846 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-11

    ... . Name of Committee: National Institute of Diabetes and Digestive and Kidney Diseases Initial Review...: National Institute of Diabetes and Digestive and Kidney Diseases Initial Review Group; Digestive Diseases... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and...

  7. 76 FR 34086 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

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    2011-06-10

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis...@niaid.nih.gov . Name of Committee: National Institute of Allergy and Infectious Diseases...

  8. 75 FR 6043 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-02-05

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  9. 78 FR 737 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-01-04

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Infectious Diseases Research, National Institutes of Health, HHS) Dated: December 31, 2012. Melanie J....

  10. 78 FR 27409 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

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    2013-05-10

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis..., Microbiology and Infectious Diseases Research, National Institutes of Health, HHS) Dated: May 6, 2013....

  11. 77 FR 37422 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-21

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Infectious Diseases Research, National Institutes of Health, HHS) Dated: June 14, 2012. Jennifer S....

  12. 75 FR 51280 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-19

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  13. 77 FR 68136 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-11-15

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  14. 78 FR 25753 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-05-02

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis..., Microbiology and Infectious Diseases Research, National Institutes of Health, HHS) Dated: April 26, 2013....

  15. 76 FR 75552 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-02

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis....gov . Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis...

  16. 78 FR 76847 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

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    2013-12-19

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  17. 75 FR 13561 - National Institute of Allergy and Infectious Diseases; Notice of Meetings

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    2010-03-22

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases.... App.), notice is hereby given of meetings of the National Advisory Allergy and Infectious Diseases... personal privacy. Name of Committee: National Advisory Allergy and Infectious Diseases Council. Date:...

  18. 77 FR 16845 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

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    2012-03-22

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  19. 77 FR 297 - National Institute of Allergy And Infectious Diseases; Notice of Closed Meetings

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    2012-01-04

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  20. 78 FR 70065 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

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    2013-11-22

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis...@niaid.nih.gov . Name of Committee: National Institute of Allergy and Infectious Diseases...

  1. Genetics Home Reference: critical congenital heart disease

    MedlinePlus

    ... right ventricle, D-transposition of the great arteries , Ebstein anomaly, hypoplastic left heart syndrome , interrupted aortic arch, ... Testing Registry: Congenital heart disease Genetic Testing Registry: Ebstein's anomaly Genetic Testing Registry: Hypoplastic left heart syndrome ...

  2. 76 FR 4703 - Disease, Disability, and Injury Prevention and Control Special Emphasis Panel (SEP): Pregnancy...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-01-26

    ... HUMAN SERVICES Centers for Disease Control and Prevention Disease, Disability, and Injury Prevention and..., Scientific Review Officer, CDC, National Center for Chronic Disease Prevention and Health Promotion, Office... and Prevention and the Agency for Toxic Substances and Disease Registry. Dated: January 20,...

  3. 76 FR 12122 - Disease, Disability, and Injury Prevention and Control Special Emphasis Panel (SEP): The...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-04

    ... HUMAN SERVICES Centers for Disease Control and Prevention Disease, Disability, and Injury Prevention and...., Scientific Review Officer, CDC, National Center for Chronic Disease Prevention and Health Promotion, Office... and Prevention and the Agency for Toxic Substances and Disease Registry. Dated: February 25,...

  4. [The German National Disease Management Guideline "Chronic Heart Failure"].

    PubMed

    Weinbrenner, S; Langer, T; Scherer, M; Störk, S; Ertl, G; Muth, Ch; Hoppe, U C; Kopp, I; Ollenschläger, G

    2012-02-01

    Chronic heart failure (CHF) is an illness mostly affecting elderly people. In Germany CHF is one of the most common causes of death and at the same time one of the most common diagnosis in inpatient care. Due to the expected increase in life expectancy in the next few years experts predict a further step-up of the incidence. Against this background development of a national guideline on chronic heart failure was prioritised and accordingly the National Disease Management Guideline (NDMG) Chronic Heart Failure was developed by a multi- and interdisciplinary group. The guideline group comprised experts from all relevant scientific medical societies as well as a patient expert. The National Disease Management Guideline (NDMG) on Chronic Heart Failure aims at supporting patients and health care providers with respect to decisions on a specific health care problem by giving recommendations for actions. Recommendations are informed by the best available scientific evidence on this topic.Patients with CHF often suffer from multiple conditions. Due to this fact and the old age patients do have very complex and demanding health care needs. Thus accounting for co-morbidities is paramount in planning and providing health care for theses patients and communication between doctor and patient but also between all health care providers is crucial.Basic treatment strategies in chronic heart failure comprise management of risk factors and prognostic factors as well as appropriate consideration of co-morbidities accompanied by measures empowering patients in establishing a healthy life style and a self-dependant management of their illness.Psycho-social aspects have a very strong influence on patients' acceptance of the disease and their self-management. In addition they have a strong influence on therapy management of the treating physician thus they have to be addressed adequately during the consultation.The National Disease Management Guideline (NDMG) Chronic Heart Failure (CHF

  5. Infectious Disease and National Security: Strategic Information Needs

    DTIC Science & Technology

    2006-01-01

    forensic attribution. BioShield Signed into law by President Bush in July 2004, Project BioShield is an initiative to speed the development and procurement...vulnerabilities and determine potential consequences, and provide a national capability for conducting forensic analysis of evidence from biocrimes and...1999, pp. 54–64. Lounibos, L. Philip, “Invasions by Insect Vectors of Human Disease,” Annual Review of Entomology , Vol. 47, January 2002, pp. 233–266

  6. 77 FR 58851 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-09-24

    ..., and evaluation of individual intramural programs and projects conducted by the National Institute of... Transplantation Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of...

  7. 78 FR 71629 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-11-29

    ..., and evaluation of individual intramural programs and projects conducted by the NATIONAL INSTITUTE OF..., Microbiology and Infectious Diseases Research, National Institutes of Health, HHS) Dated: November 22,...

  8. 75 FR 18215 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-09

    ..., and evaluation of individual intramural programs and projects conducted by the National Institute of... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  9. 75 FR 2147 - National Institute of Diabetes and Digestive and Kidney Diseases, Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-01-14

    ... Committee: National Diabetes and Digestive and Kidney Diseases Advisory Council. Date: February 24, 2010... of Diabetes and Digestive and Kidney Diseases, 6707 Democracy Blvd., Room 715, MSC 5452, Bethesda, MD... Kidney Diseases Advisory Council; Diabetes, Endocrinology, and Metabolic Diseases Subcommittee....

  10. 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

  11. Clinical expression of facioscapulohumeral muscular dystrophy in carriers of 1–3 D4Z4 reduced alleles: experience of the FSHD Italian National Registry

    PubMed Central

    Nikolic, Ana; Ricci, Giulia; Sera, Francesco; Bucci, Elisabetta; Govi, Monica; Mele, Fabiano; Rossi, Marta; Ruggiero, Lucia; Vercelli, Liliana; Ravaglia, Sabrina; Brisca, Giacomo; Fiorillo, Chiara; Villa, Luisa; Maggi, Lorenzo; Cao, Michelangelo; D'Amico, Maria Chiara; Siciliano, Gabriele; Antonini, Giovanni; Santoro, Lucio; Mongini, Tiziana; Moggio, Maurizio; Morandi, Lucia; Pegoraro, Elena; Angelini, Corrado; Di Muzio, Antonio; Rodolico, Carmelo; Tomelleri, Giuliano; Grazia D'Angelo, Maria; Bruno, Claudio; Berardinelli, Angela; Tupler, Rossella

    2016-01-01

    Objectives Facioscapulohumeral muscular dystrophy type 1 (FSHD1) has been genetically linked to reduced numbers (≤8) of D4Z4 repeats at 4q35. Particularly severe FSHD cases, characterised by an infantile onset and presence of additional extra-muscular features, have been associated with the shortest D4Z4 reduced alleles with 1–3 repeats (1–3 DRA). We searched for signs of perinatal onset and evaluated disease outcome through the systematic collection of clinical and anamnestic records of de novo and familial index cases and their relatives, carrying 1–3 DRA. Setting Italy. Participants 66 index cases and 33 relatives carrying 1–3 DRA. Outcomes The clinical examination was performed using the standardised FSHD evaluation form with validated inter-rater reliability. To investigate the earliest signs of disease, we designed the Infantile Anamnestic Questionnaire (IAQ). Comparison of age at onset was performed using the non-parametric Wilcoxon rank-sum or Kruskal-Wallis test. Comparison of the FSHD score was performed using a general linear model and Wald test. Kaplan-Meier survival analysis was used to estimate the age-specific cumulative motor impairment risk. Results No patients had perinatal onset. Among index cases, 36 (54.5%) showed the first signs by 10 years of age. The large majority of patients with early disease onset (26 out of 36, 72.2%) were de novo; whereas the majority of patients with disease onset after 10 years of age were familial (16, 53.3%). Comparison of the disease severity outcome between index cases with age at onset before and over 10 years of age, failed to detect statistical significance (Wald test p value=0.064). Of 61 index cases, only 17 (27.9%) presented extra-muscular conditions. Relatives carrying 1–3 DRA showed a large clinical variability ranging from healthy subjects, to patients with severe motor impairment. Conclusions The size of the D4Z4 allele is not always predictive of severe clinical outcome. The high

  12. Trauma registries: history, logistics, limitations, and contributions to emergency medicine research.

    PubMed

    Zehtabchi, Shahriar; Nishijima, Daniel K; McKay, Mary Pat; Mann, N Clay

    2011-06-01

    Trauma registries have been designed to serve a number of purposes, including quality improvement, injury prevention, clinical research, and policy development. Since their inception over 30 years ago, there are increasingly more institutions with trauma registries, many of which submit data to a national trauma registry. The goal of this review is to describe the history, logistics, and characteristics of trauma registries and their contribution to emergency medicine and trauma research. Discussed in this review are the limitations of trauma registries, such as variability in quality and type of the collected data, absence of data pertaining to long-term and functional outcomes, prehospital information, and complications as well as other methodologic obstacles limiting the utility of registry data in clinical and epidemiologic research.

  13. Role of international registries in enhancing the care of familial hypercholesterolaemia.

    PubMed

    Hammond, Emma; Watts, Gerald F; Rubinstein, Yaffa; Farid, Waleed; Livingston, Michael; Knowles, Joshua W; Lochmüller, Hanns; Bellgard, Matthew; Dawkins, Hugh J S

    2013-06-01

    Familial hypercholesterolaemia (FH) is a relatively common genetic disorder associated with high risk of coronary heart disease that is preventable by early diagnosis and treatment. In a previous article, we reviewed the evidence for clinical management, models of care and health economic evaluations. The present commentary emphasises that collective action is needed to strengthen our approaches to evidence-based care, including better diagnosis and access to effective therapies. We detail how contemporary innovations in inter-operable, web-based, open-source and secure registries can provide the supporting infrastructure to: (i) address a current gap in the flow of data for measuring the quality of healthcare; (ii) support basic research through provision of high-quality, de-identified aggregate data; (iii) enable equitable access to clinical trials; and (iv) support efforts to disseminate evidence for best practice and information for care services. We describe how these aspects of enabling infrastructure will be incorporated into the development of a National FH Registry for Australasia, and proffer that a coordinated response to FH would be enhanced through a global network of inter-operable registries.

  14. 76 FR 52670 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-08-23

    ... Allergy and Infectious Diseases Special Emphasis Panel; NIAID Investigator Initiated Program Project... Transplantation Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of...

  15. 78 FR 68857 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-11-15

    ... personal privacy. Name of Committee: Microbiology, Infectious Diseases and AIDS Initial Review Group... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  16. 75 FR 62546 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-12

    ... personal privacy. Name of Committee: Microbiology, Infectious Diseases and AIDS Initial Review Group... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  17. 76 FR 21754 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-18

    ... Panel; Preclinical Services for the Development of Biopharmaceutical Products for Infectious Diseases... Research; 93.856, Microbiology and Infectious Diseases Research, National Institutes of Health, HHS)...

  18. The development of registries for surveillance of adult lead exposure, 1981 to 1992.

    PubMed Central

    Baser, M E

    1992-01-01

    OBJECTIVES. Since 1981, 15 states have established registries for surveillance of adult lead absorption, primarily based on reports of elevated blood lead levels from clinical laboratories. I review the status of the registries and recommend steps for further development. METHODS. Companies reported to the New York registry are compared with those cited by the Occupational Safety and Health Administration (OSHA). I present data on US workers and plants with potential lead exposures and blood tests, as well as review registries' reporting requirements. RESULTS. Registries identify many companies not cited by the Occupational Safety and Health Administration, but underreporting occurs because (1) reporting is usually not required from laboratories outside the state, (2) most registries use a blood lead reporting level of 1.21 mumol/L, which excludes many exposed workers, and (3) many companies with potential exposures do not have routine monitoring programs. CONCLUSIONS. Registries' reporting requirements and procedures should be standardized, including a blood lead reporting level of 0.72 mumol/L. Elevated blood lead levels should be a reportable condition nationwide, and a comprehensive national surveillance system should be established: clinical laboratories should be required to report cases to those states with lead registries or directly to the national adult lead registry. PMID:1636831

  19. Diagnosis and mortality in 47,XYY persons: a registry study

    PubMed Central

    2010-01-01

    Background Sex chromosomal abnormalities are relatively common, yet many aspects of these syndromes remain unexplored. For instance epidemiological data in 47,XYY persons are still limited. Methods Using a national Danish registry, we identified 208 persons with 47,XYY or a compatible karyotype, whereof 36 were deceased; all were diagnosed from 1968 to 2008. For further analyses, we identified age matched controls from the male background population (n = 20,078) in Statistics Denmark. We report nationwide prevalence data, data regarding age at diagnosis, as well as total and cause specific mortality data in these persons. Results The average prevalence was 14.2 47,XYY persons per 100,000, which is reduced compared to the expected 98 per 100,000. Their median age at diagnosis was 17.1 years. We found a significantly decreased lifespan from 77.9 years (controls) to 67.5 years (47,XYY persons). Total mortality was significantly increased compared to controls, with a hazard ratio of 3.6 (2.6-5.1). Dividing the causes of deaths according to the International Classification of Diseases, we identified an increased hazard ratio in all informative chapters, with a significantly increased ratio in cancer, pulmonary, neurological and unspecified diseases, and trauma. Conclusion We here present national epidemiological data regarding 47,XYY syndrome, including prevalence and mortality data, showing a significantly delay to diagnosis, reduced life expectancy and an increased total and cause specific mortality. PMID:20509956

  20. 75 FR 5602 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-02-03

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel, Hormones in Postmenopausal Women Ancillary Studies. Date... Diabetes and Digestive and Kidney Diseases Special Emphasis Panel, Training Grants in Digestive...

  1. 75 FR 65365 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-22

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel, Genetics of Nephropathy Ancillary Studies. Date: November 15... Digestive and Kidney Diseases Special Emphasis Panel, Consortium for Radiologic Imaging Studies...

  2. 75 FR 9231 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-01

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel; Metabolic Dysfunction Collaborative Interdisciplinary Science... Diabetes and Digestive and Kidney Diseases Special Emphasis Panel; CAMUS Trial. Date: April 2, 2010....

  3. 78 FR 58325 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-09-23

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel, Bariatric Surgery-- Related Ancillary Studies (R01s). Date... Digestive and Kidney Diseases Special Emphasis Panel, Regulatory Mechanisms in Intestinal Motility...

  4. 77 FR 62520 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-15

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel; Ancillary Studies to Major Ongoing Clinical Research Studies..., Diabetes, Endocrinology and Metabolic Research; 93.848, Digestive Diseases and Nutrition Research;...

  5. 77 FR 31628 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-05-29

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases..., Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases Research,...

  6. 76 FR 35224 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-16

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases..., Allergy, Immunology, and Transplantation Research; 93.856, Microbiology and Infectious Diseases...

  7. 75 FR 56552 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-16

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... need special assistance, such as sign language interpretation or other reasonable accommodations... Kidney Diseases Initial Review Group; Digestive Diseases and Nutrition C Subcommittee. Date: November...

  8. Cognitive evaluation of a physician data query tool for a national ICU registry: comparing two think aloud variants and their application in redesign.

    PubMed

    Peute, Linda W; de Keizer, Nicolette F; Jaspers, Monique W M

    2010-01-01

    Applying usability methods in formative evaluations of interactive healthcare information systems design is recognized as of extreme importance to the final success of these systems. However, it seems that the merits of specific methodological approaches for conducting these studies have received little attention. This study reports on a cognitive evaluation of a Physician Data Query Tool, which offers physicians the opportunity to query quality of care data collected by the Dutch National Intensive Care Evaluation (NICE) foundation. A comparison in terms of usefulness and utility of two variants of the Think Aloud method is addressed, the Concurrent and Retrospective Think Aloud. These methods are well known in the field of Human Computer Interaction in the context of usability evaluation. The results of this research indicate that though both methods have their disadvantages and benefits, in redesigning the Physician Data Query tool the Retrospective Think Aloud provided more useful input to the Tool's redesign. However, in deciding which method to apply in a formative evaluation study, end users' cognitive workload of performing the system's tasks and the system characteristics need to be considered as well.

  9. [After seven years of National Disease Management Guidelines: quo vadis?].

    PubMed

    Weinbrenner, Susanne; Conrad, Susann; Weikert, Beate; Kopp, Ina

    2010-01-01

    After seven years the National Disease Management Guidelines Programme (German DM-CPG Programme) that was established under the auspices of the German Medical Association, the National Association of Statutory Health Insurance Physicians and the Association of the Scientific Medical Societies in Germany has been widely accepted by both health care professionals and patients. DM-CPGs are available as tools for knowledge and quality management for widespread chronic diseases showing need for improvement in treatment pathways and coordination between health care providers. The main objective of the German DM-CPG Programme is to establish consensus among the medical professions on evidence-based key recommendations covering all sectors of health care provision and facilitating the coordination of care for the individual patient over time and across interfaces. German DM-CPGs provide a conceptual basis for disease management and integrative care aiming at the implementation of best practice recommendations for prevention, acute care, rehabilitation, chronic care and management aspects for high priority health care topics. Thus, representatives of all disciplines, professions and patients concerned with the topic of an individual German DM-CPG are involved in the development process. The methodology of guideline development is in accordance with international standards. However, the improvement of strategies for effective implementation and continuous update remain challenging. Future work will also focus on content-related aspects such as co-morbidity, gender and migration background.

  10. 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

  11. Lymphatic Anomalies Registry

    ClinicalTrials.gov

    2016-07-26

    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

  12. 75 FR 14605 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-26

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases, including consideration of personnel qualifications and performance, and the competence..., Director, Division of Intramural Research, National Institute of Diabetes and Digestive and Kidney...

  13. 76 FR 57747 - National Institute of Diabetes and Digestive and Kidney Diseases Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-09-16

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group; Kidney, Urologic and Hematologic Diseases D Subcommittee. Date....gov . Name of Committee: National Institute of Diabetes and Digestive and Kidney Diseases...

  14. 76 FR 4926 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-01-27

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group; Digestive Diseases and Nutrition C Subcommittee. Date: March 7-8... and Nutrition Research; 93.849, Kidney Diseases, Urology and Hematology Research, National...

  15. 75 FR 67989 - National Institute of Arthritis and Musculoskeletal and Skin Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-11-04

    ... Health National Institute of Arthritis and Musculoskeletal and Skin Diseases; Notice of Closed Meeting... Committee: National Institute of Arthritis and Musculoskeletal and Skin Diseases Special Emphasis Panel..., Musculoskeletal and Skin Diseases Research, National Institutes of Health, HHS) Dated: October 28, 2010....

  16. 75 FR 10488 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-08

    ... Autoimmune and Inflammatory Diseases. Date: April 30, 2010. Time: 11 a.m. to 3 p.m. Agenda: To review and... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special...

  17. 76 FR 30733 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-05-26

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group, Digestive Diseases and Nutrition C Subcommittee. Date: June 27-28... and Nutrition Research; 93.849, Kidney Diseases, Urology and Hematology Research, National...

  18. 75 FR 54895 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-09

    ... Allergy and Infectious Diseases Special Emphasis Panel, NHP Viral RNA Core. Date: September 27, 2010. Time... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special...

  19. 78 FR 57867 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-09-20

    ...: National Institute of Diabetes and Digestive and Kidney Diseases ] Special Emphasis Panel, PAR-11-043 NIDDK... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel, Digestive Diseases Centers. Date: November 21-22, 2013. Time:...

  20. 75 FR 57972 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-23

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... Allergy and Infectious Diseases, including consideration of personnel qualifications and performance, and... Research, National Institute of Allergy and Infectious Diseases, NIH, Building 31, Room 4A30, Bethesda,...

  1. 77 FR 53206 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-08-31

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis..., 301-594-1009, fdesilva@niaid.nih.gov . Name of Committee: National Institute of Allergy and...

  2. 77 FR 76296 - National Institute of Allergy and Infectious Diseases; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-12-27

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases.... App.), notice is hereby given of meetings of the National Advisory Allergy and Infectious Diseases... constitute a clearly unwarranted invasion of personal privacy. Name of Committee: National Advisory...

  3. 78 FR 20933 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-04-08

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis...- 7616, 301-496-2550, pjackson@niaid.nih.gov . Name of Committee: National Institute of Allergy...

  4. 76 FR 77241 - National Institute of Allergy and Infectious Diseases; Notice of Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-12

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases.... App.), notice is hereby given of meetings of the National Advisory Allergy and Infectious Diseases... constitute a clearly unwarranted invasion of personal privacy. Name of Committee: National Advisory...

  5. 78 FR 59707 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-09-27

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis...-2639, poeky@niaid.nih.gov . Name of Committee: National Institute of Allergy and Infectious...

  6. 75 FR 7488 - National Institute of Allergy and Infectious Diseases; Notice of Closed Meetings

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-02-19

    ... HUMAN SERVICES National Institutes of Health National Institute of Allergy and Infectious Diseases... personal privacy. Name of Committee: National Institute of Allergy and Infectious Diseases Special Emphasis..., Bethesda, MD 20892. 301-402-1464. eb237e@nih.gov . Name of Committee: National Institute of Allergy...

  7. Australia's notifiable diseases status, 2001: annual report of the National Notifiable Diseases Surveillance System.

    PubMed

    Blumer, Charlie; Roche, Paul; Spencer, Jenean; Lin, Ming; Milton, Alison; Bunn, Chris; Gidding, Heather; Kaldor, John; Kirk, Martyn; Hall, Rob; Della-Porta, Tony; Leader, Robyn; Wright, Phil

    2003-01-01

    In 2001 there were 104,187 notifications of communicable diseases in Australia reported to the National Notifiable Diseases Surveillance System (NNDSS). The number of notifications in 2001 was an increase of 16 per cent of those reported in 2000 (89,740) and the largest annual total since the NNDSS commenced in 1991. In 2001, nine new diseases were added to the list of diseases reported to NNDSS and four diseases were removed. The new diseases were cryptosporidiosis, laboratory-confirmed influenza, invasive pneumococcal disease, Japanese encephalitis, Kunjin virus infection, Murray Valley encephalitis virus infection, anthrax, Australian bat lyssavirus, and other lyssaviruses (not elsewhere classified). Bloodborne virus infections remained the most frequently notified disease (29,057 reports, 27.9% of total), followed by sexually transmitted infections (27,647, 26.5%), gastrointestinal diseases (26,086, 25%), vaccine preventable diseases (13,030 (12.5%), vectorborne diseases (5,294, 5.1%), other bacterial infections (1,978, 1.9%), zoonotic infections (1,091, 1%) and four cases of quarantinable diseases. In 2001 there were increases in the number of notifications of incident hepatitis C, chlamydial infections, pertussis, Barmah Forest virus infection and ornithosis. There were decreases in the number of notifications of hepatitis A, Haemophilus influenzae type b infections, measles, rubella, Ross River virus infections and brucellosis. This report also summarises data on communicable diseases from other surveillance systems including the Laboratory Virology and Serology Reporting Scheme and sentinel general practitioner schemes. In addition, this report comments on other important developments in communicable disease control in Australia in 2001.

  8. Alzheimer's disease research in the context of the national plan to address Alzheimer's disease.

    PubMed

    Snyder, Heather M; Hendrix, James; Bain, Lisa J; Carrillo, Maria C

    2015-01-01

    In 2012, the first National Plan to Address Alzheimer's Disease in the United States (U.S.) was released, a component of the National Alzheimer's Project Act legislation. Since that time, there have been incremental increases in U.S. federal funding for Alzheimer's disease and related dementia research, particularly in the areas of biomarker discovery, genetic link and related biological underpinnings, and prevention studies for Alzheimer's. A central theme in each of these areas has been the emphasis of cross-sector collaboration and private-public partnerships between government, non-profit organizations and for-profit organizations. This paper will highlight multiple private-public partnerships supporting the advancement of Alzheimer's research in the context of the National Plan to Address Alzheimer's.

  9. Renal replacement therapy in Europe: a summary of the 2012 ERA-EDTA Registry Annual Report

    PubMed Central

    Pippias, Maria; Stel, Vianda S.; Abad Diez, José Maria; Afentakis, Nikolaos; Herrero-Calvo, Jose Antonio; Arias, Manuel; Tomilina, Natalia; Bouzas Caamaño, Encarnación; Buturovic-Ponikvar, Jadranka; Čala, Svjetlana; Caskey, Fergus J.; Castro de la Nuez, Pablo; Cernevskis, Harijs; Collart, Frederic; Alonso de la Torre, Ramón; García Bazaga, Maria de los Ángeles; De Meester, Johan; Díaz, Joan Manuel; Djukanovic, Ljubica; Ferrer Alamar, Manuel; Finne, Patrik; Garneata, Liliana; Golan, Eliezer; González Fernández, Raquel; Gutiérrez Avila, Gonzalo; Heaf, James; Hoitsma, Andries; Kantaria, Nino; Kolesnyk, Mykola; Kramar, Reinhard; Kramer, Anneke; Lassalle, Mathilde; Leivestad, Torbjørn; Lopot, Frantisek; Macário, Fernando; Magaz, Angela; Martín-Escobar, Eduardo; Metcalfe, Wendy; Noordzij, Marlies; Palsson, Runolfur; Pechter, Ülle; Prütz, Karl G.; Ratkovic, Marina; Resić, Halima; Rutkowski, Boleslaw; Santiuste de Pablos, Carmen; Spustová, Viera; Süleymanlar, Gültekin; Van Stralen, Karlijn; Thereska, Nestor; Wanner, Christoph; Jager, Kitty J.

    2015-01-01

    Background This article summarizes the 2012 European Renal Association—European Dialysis and Transplant Association Registry Annual Report (available at www.era-edta-reg.org) with a specific focus on older patients (defined as ≥65 years). Methods Data provided by 45 national or regional renal registries in 30 countries in Europe and bordering the Mediterranean Sea were used. Individual patient level data were received from 31 renal registries, whereas 14 renal registries contributed data in an aggregated form. The incidence, prevalence and survival probabilities of patients with end-stage renal disease (ESRD) receiving renal replacement therapy (RRT) and renal transplantation rates for 2012 are presented. Results In 2012, the overall unadjusted incidence rate of patients with ESRD receiving RRT was 109.6 per million population (pmp) (n = 69 035), ranging from 219.9 pmp in Portugal to 24.2 pmp in Montenegro. The proportion of incident patients ≥75 years varied from 15 to 44% between countries. The overall unadjusted prevalence on 31 December 2012 was 716.7 pmp (n = 451 270), ranging from 1670.2 pmp in Portugal to 146.7 pmp in the Ukraine. The proportion of prevalent patients ≥75 years varied from 11 to 32% between countries. The overall renal transplantation rate in 2012 was 28.3 pmp (n = 15 673), with the highest rate seen in the Spanish region of Catalonia. The proportion of patients ≥65 years receiving a transplant ranged from 0 to 35%. Five-year adjusted survival for all RRT patients was 59.7% (95% confidence interval, CI: 59.3–60.0) which fell to 39.3% (95% CI: 38.7–39.9) in patients 65–74 years and 21.3% (95% CI: 20.8–21.9) in patients ≥75 years. PMID:26034584

  10. Epidemiology and Registry Studies of Stroke in Japan

    PubMed Central

    2013-01-01

    Stroke is the most prevalent cardiovascular disease in Japan. This review introduces two epidemiologic studies and four registry studies of stroke in Japan. The Hisayama Study was begun as a population-based prospective cohort study of cerebrovascular and cardiovascular diseases in 1961 in the town of Hisayama. Most of the deceased subjects of the study underwent autopsy examinations from the beginning of the study. Changes in stroke trends in the last 50 years were clarified by comparison of data from different study cohorts registered every 13 to 14 years. The Suita Study was based on a random sampling of Japanese urban residents. Several reports from this study showed the significance of pre-hypertension, as well as hypertension, as a risk factor for stroke by itself and in combination with other underlying characteristics. In addition, the Japan Multicenter Stroke Investigators' Collaboration (J-MUSIC), the Japan Standard Stroke Registry Study, the Fukuoka Stroke Registry, and the Stroke Acute Management with Urgent Risk-factor Assessment and Improvement (SAMURAI) rt-PA Registry are explained as registry studies involving Japanese stroke patients. PMID:24324936

  11. Epidemiology and registry studies of stroke in Japan.

    PubMed

    Toyoda, Kazunori

    2013-01-01

    Stroke is the most prevalent cardiovascular disease in Japan. This review introduces two epidemiologic studies and four registry studies of stroke in Japan. The Hisayama Study was begun as a population-based prospective cohort study of cerebrovascular and cardiovascular diseases in 1961 in the town of Hisayama. Most of the deceased subjects of the study underwent autopsy examinations from the beginning of the study. Changes in stroke trends in the last 50 years were clarified by comparison of data from different study cohorts registered every 13 to 14 years. The Suita Study was based on a random sampling of Japanese urban residents. Several reports from this study showed the significance of pre-hypertension, as well as hypertension, as a risk factor for stroke by itself and in combination with other underlying characteristics. In addition, the Japan Multicenter Stroke Investigators' Collaboration (J-MUSIC), the Japan Standard Stroke Registry Study, the Fukuoka Stroke Registry, and the Stroke Acute Management with Urgent Risk-factor Assessment and Improvement (SAMURAI) rt-PA Registry are explained as registry studies involving Japanese stroke patients.

  12. A Complex Contraception Registry

    ClinicalTrials.gov

    2014-12-02

    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

  13. Endoscopic Resection Multicenter Registry

    ClinicalTrials.gov

    2017-02-22

    Gastrointestinal Neoplasms; Gastrointestinal Disease; Gastric Cancer; Gastric Neoplasm; Gastric Polyp; Esophageal Neoplasms; Duodenal Neoplasms; Duodenal Polyp; Stomach Neoplasm; Stomach Polyp; Neoplasms; Colon Polyp; Colon Neoplasm

  14. [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

  15. 76 FR 60507 - National Institute of Diabetes and Digestive and Kidney Diseases Notice of Closed Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-09-29

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel; Digestive Diseases Core Centers. Date: December 2, 2011. Time: 8..., Digestive Diseases and Nutrition Research; 93.849, Kidney Diseases, Urology and Hematology...

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    2013-02-27

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    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Special Emphasis Panel, Fellowships in Digestive Diseases and Nutrition. Date: October 18... Diabetes and Digestive and Kidney Diseases Special Emphasis Panel. Kidney Disease Ancillary Studies....

  19. 78 FR 58322 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

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    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group, Digestive Diseases and Nutrition C Subcommittee. Date: October 23... Diseases and Nutrition Research; 93.849, Kidney Diseases, Urology and Hematology Research,...

  20. 76 FR 20692 - National Institute of Diabetes and Digestive and Kidney Diseases; Notice of Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-13

    ... HUMAN SERVICES National Institutes of Health National Institute of Diabetes and Digestive and Kidney... Kidney Diseases Initial Review Group; Diabetes, Endocrinology and Metabolic Diseases B Subcommittee. Date...; 93.848, Digestive Diseases and Nutrition Research; 93.849, Kidney Diseases, Urology and...