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  1. Vulval Crohn's disease: a clinical study of 22 patients.

    PubMed

    Laftah, Zainab; Bailey, Clare; Zaheri, Shirin; Setterfield, Jane; Fuller, Lucinda Claire; Lewis, Fiona

    2015-04-01

    Vulval Crohn's disease [VCD] is a challenging condition that can occur without gastrointestinal Crohn's disease [GCD]. We reviewed the clinical features and effects of therapy in a cohort of 22 patients with VCD to determine whether the presence of GCD affected the clinical presentation and treatment response. Of these, 64% had GCD and 65% of these presented with GCD prior to VCD. Fissuring, ulceration, and scarring were more common in those with VCD alone. Potent and ultra-potent topical steroids showed benefit in most patients. There was no clear evidence of sustained remission with metronidazole. Azathioprine was the most commonly used oral immunosuppressive agent, with an efficacy of 57%. Patients with both VCD and GCD had a better clinical response compared with those with VCD alone [70% and 25%, respectively]. Infliximab and adalimumab were effective in 56% and 71% of patients, respectively. Excision of redundant tissue was helpful in four patients. The absence of GCD may delay the diagnosis in women who present with vulval symptoms alone. The more active clinical features in those with VCD alone may represent a more aggressive condition, or the severity of the cutaneous disease may have been reduced by immunosuppression taken for GCD. Several patients obtained sustained remission in their GCD with anti-tumor necrosis factor alpha [anti-TNFα] agents while the VCD remained active.

  2. How to predict clinical relapse in inflammatory bowel disease patients

    PubMed Central

    Liverani, Elisa; Scaioli, Eleonora; Digby, Richard John; Bellanova, Matteo; Belluzzi, Andrea

    2016-01-01

    Inflammatory bowel diseases have a natural course characterized by alternating periods of remission and relapse. Disease flares occur in a random way and are currently unpredictable for the most part. Predictors of benign or unfavourable clinical course are required to facilitate treatment decisions and to avoid overtreatment. The present article provides a literature review of the current evidence on the main clinical, genetic, endoscopic, histologic, serologic and fecal markers to predict aggressiveness of inflammatory bowel disease and discuss their prognostic role, both in Crohn’s disease and ulcerative colitis. No single marker seems to be reliable alone as a flare predictor, even in light of promising evidence regarding the role of fecal markers, in particular fecal calprotectin, which has reported good results recently. In order to improve our daily clinical practice, validated prognostic scores should be elaborated, integrating clinical and biological markers of prognosis. Finally, we propose an algorithm considering clinical history and biological markers to intercept patients with high risk of clinical relapse. PMID:26811644

  3. Clinical and Sociodemographic Aspects of Inflammatory Bowel Disease Patients

    PubMed Central

    Delmondes, Leda Maria; Nunes, Marcelo Oliveira; Azevedo, Arthur Rangel; Oliveira, Murilo Matos de Santana; Coelho, Lorena Eugenia Rosa; Torres-Neto, Juvenal da Rocha

    2015-01-01

    Background In Brazil, there are few epidemiological studies available about the demographic and clinical aspects of inflammatory bowel disease (IBD). The aim of this study was to identify epidemiological and clinical characteristics of patients with IBD treated at the University Hospital (HU) of the Sergipe Federal University (UFS). Methods A cross-sectional descriptive study was conducted in HU/UFS from October 2011 to January 2014. The sample consisted of 87 patients with IBD, who registered in the coloproctology clinic. We applied a questionnaire with sociodemographic and clinical variables. Results Of the 87 patients, 40 (46%) had Crohn’s disease (CD) and 47 (54%) had ulcerative colitis (UC). Women had a higher prevalence of IBD. Data obtained were significant (P < 0.05) in the variables: age, origin and level of education. CD patients were younger (< 25 years old), had higher prevalence of smoking habits and were associated with urban origin, conjunctivitis, palpable mass, appendectomy and intestinal complications. UC was more prevalent in older individuals (> 25 years old), with rural origin, bloody diarrhea and rectal bleeding. Location and initial behavior of CD were ileum-colic (L3), inflammatory behavior and penetrating form of the disease. There is higher prevalence of proctitis and mild and severe forms of the UC among women. Osteoarticular and systemic manifestations predominated in both diseases. Conclusions IBD affected more women than men. The age, origin and level of education can interfere with early diagnosis. Demographic and clinical aspects were similar to the literature. Data differ in the time interval between the onset of symptoms and diagnosis, smoking habit, appendectomy and severity of UC for age and gender.

  4. Renal diseases in haemophilic patients: pathogenesis and clinical management.

    PubMed

    Esposito, Pasquale; Rampino, Teresa; Gregorini, Marilena; Fasoli, Gianluca; Gamba, Gabriella; Dal Canton, Antonio

    2013-10-01

    Haemophilia A and B are genetic X-linked bleeding disorders, caused by mutations in genes encoding factors VIII and IX, respectively. Clinical manifestations of haemophilia are spontaneous haemorrhage or acute bleeding caused by minor trauma, resulting in severe functional consequences that can culminate in a debilitating arthropathy. Life expectancy and quality of life of patients with haemophilia have dramatically improved over the last years, mainly for new therapeutic options and the awareness to the risk of HCV and HIV infections. Different clinical problems arise from this important change in history of patients with haemophilia. In particular, ageing-related diseases, such as diabetes, hypertension and cancer, and chronic viral infections are emerging as new challenges in this patient population. Among the different types of chronic illnesses, renal diseases are of special interest as they involve some difficult management issues. In fact, decisions regarding adequate preventive strategies and viral infection treatment, the choice of the dialytic modality, placement of vascular access and prescription of dialytic treatments are particularly complicated, because only few data are available. In this review, we discuss the pathogenesis of renal damage in patients with haemophilia, especially in those with blood-transmitted viral infections, and the major issues about the management of renal diseases, including problems related to dialytic treatment and kidney transplantation, providing practical algorithms to guide the clinical decision-making process.

  5. Treatment of Anemia in Patients with Heart Disease: A Clinical Practice Guideline

    MedlinePlus

    ... of Internal Medicine Summaries for Patients Treatment of Anemia in Patients With Heart Disease: A Clinical Practice ... Physicians The full report is titled “Treatment of Anemia in Patients With Heart Disease: A Clinical Practice ...

  6. Uncontrolled chronic disease: patient non-compliance or clinical mismanagement?

    PubMed

    Javors, Jonathan R; Bramble, Judith E

    2003-01-01

    A study group of 30 individuals was randomly chosen from 1,379 beneficiaries predicted to be at risk for health care complications at a large, Midwest, industrial company currently experiencing increased health care costs. All 30 individuals had one or more chronic illness, primarily diabetes, cardiovascular disease, or asthma. Through analysis of medical records, a self-reporting health risk assessment survey, and personal contact with both patients and clinicians, each study individual was assessed as to whether his disease(s) was under control, the individual was compliant with his treatment protocol, and whether the supervising clinician was following nationally accepted standards of care. Fewer than 50% of the individuals in the study group had their chronic illness(es) under control. Those individuals whose treatment adhered to national guidelines were significantly more likely to have their disease under control (p < 0.001). For this study, patient compliance was high and unrelated to whether their disease was under control. Behavioral (external) barriers were most often cited as the reason a clinical practitioner did not follow the appropriate national standard of care. Most clinicians were aware of and familiar with the guidelines; a few either did not agree with or misunderstood the guidelines. The results of this study suggest that changing clinical practice behaviors to better ensure compliance to national standards of care may make a substantial difference in chronic disease control.

  7. Uncontrolled chronic disease: patient non-compliance or clinical mismanagement?

    PubMed

    Javors, Jonathan R; Bramble, Judith E

    2003-01-01

    A study group of 30 individuals was randomly chosen from 1,379 beneficiaries predicted to be at risk for health care complications at a large, Midwest, industrial company currently experiencing increased health care costs. All 30 individuals had one or more chronic illness, primarily diabetes, cardiovascular disease, or asthma. Through analysis of medical records, a self-reporting health risk assessment survey, and personal contact with both patients and clinicians, each study individual was assessed as to whether his disease(s) was under control, the individual was compliant with his treatment protocol, and whether the supervising clinician was following nationally accepted standards of care. Fewer than 50% of the individuals in the study group had their chronic illness(es) under control. Those individuals whose treatment adhered to national guidelines were significantly more likely to have their disease under control (p < 0.001). For this study, patient compliance was high and unrelated to whether their disease was under control. Behavioral (external) barriers were most often cited as the reason a clinical practitioner did not follow the appropriate national standard of care. Most clinicians were aware of and familiar with the guidelines; a few either did not agree with or misunderstood the guidelines. The results of this study suggest that changing clinical practice behaviors to better ensure compliance to national standards of care may make a substantial difference in chronic disease control. PMID:14570385

  8. Clinical and genetic characteristics of Mexican Huntington's disease patients.

    PubMed

    Alonso, María Elisa; Ochoa, Adriana; Boll, Marie-Catherine; Sosa, Ana Luisa; Yescas, Petra; López, Marisol; Macias, Rosario; Familiar, Itziar; Rasmussen, Astrid

    2009-10-15

    We report the characteristics of 691 Mexican patients with Huntington's disease (HD). These patients, representing 401 families, constitute the largest series of Mexican HD cases as yet described in the literature. We found the clinical characteristics of these patients to be similar to those of other populations, but we observed a higher frequency of infantile cases, a shorter disease duration and a lower suicide rate. In 626 cases, for which molecular analyses were available, CAG-trinucleotide expansion size ranged from 37-106 repeats. The large number of CAG repeats (19.04 +/- 3.02) in normal alleles and the presence of new mutations suggest that the overall prevalence of HD in the Mexican population could be expected to be within range of, or higher than, that reported for Europeans.

  9. Clinical Assessment of Patients with Peripheral Arterial Disease

    PubMed Central

    Bailey, Marc A.; Griffin, Kathryn J.; Scott, D. Julian A.

    2014-01-01

    Peripheral arterial disease (PAD) describes the clinical manifestations of atherosclerosis affecting the circulation in the legs. The severity of PAD is classified according to symptom severity, time course, and anatomical distribution. The signs and symptoms of PAD reflect the degree of circulatory compromise and whether there has been a gradual reduction in the circulation or an abrupt, uncompensated decrease. Accurate clinical assessment underpins decisions on management strategy and should objectively assess the severity of the ischemia and need for revascularization. Clinical history should discriminate symptoms of PAD from other conditions presenting with leg pain, elucidate cardiovascular risk factors and the effect of symptoms on the patient's quality of life. Clinical examination includes signs of general cardiovascular disease and associated conditions before assessing the circulation and viability of the limb. Palpation of peripheral pulses must be augmented by determination of the ankle brachial pressure index using hand held Doppler. A whole patient approach to management is required and must include modification of cardiovascular risk status as well as dealing with the local circulatory manifestation of PAD. PMID:25435653

  10. Clinical assessment of patients with peripheral arterial disease.

    PubMed

    Bailey, Marc A; Griffin, Kathryn J; Scott, D Julian A

    2014-12-01

    Peripheral arterial disease (PAD) describes the clinical manifestations of atherosclerosis affecting the circulation in the legs. The severity of PAD is classified according to symptom severity, time course, and anatomical distribution. The signs and symptoms of PAD reflect the degree of circulatory compromise and whether there has been a gradual reduction in the circulation or an abrupt, uncompensated decrease. Accurate clinical assessment underpins decisions on management strategy and should objectively assess the severity of the ischemia and need for revascularization. Clinical history should discriminate symptoms of PAD from other conditions presenting with leg pain, elucidate cardiovascular risk factors and the effect of symptoms on the patient's quality of life. Clinical examination includes signs of general cardiovascular disease and associated conditions before assessing the circulation and viability of the limb. Palpation of peripheral pulses must be augmented by determination of the ankle brachial pressure index using hand held Doppler. A whole patient approach to management is required and must include modification of cardiovascular risk status as well as dealing with the local circulatory manifestation of PAD. PMID:25435653

  11. Functional capacity of Brazilian patients with Parkinson's disease (PD): relationship between clinical characteristics and disease severity.

    PubMed

    Barbieri, Fabio A; Rinaldi, Natalia M; Santos, Paulo Cezar R; Lirani-Silva, Ellen; Vitório, Rodrigo; Teixeira-Arroyo, Cláudia; Stella, Florindo; Gobbi, Lilian Teresa B

    2012-01-01

    The present study had three objectives: (a) to characterize the functional capacity of patients with PD, (b) to assess the relationship between the physical fitness components of functional capacity with clinical characteristics and disease severity, and (c) to compare the physical fitness components of functional capacity with clinical characteristics according to disease severity. The study included 54 patients with idiopathic PD who were distributed into two groups according to PD severity: unilateral group (n=35); and bilateral group (n=19). All patients underwent psychiatric assessment by means of the Hoehn and Yahr (HY) staging of PD, the Unified Parkinson's Disease Rating Scale (UPDRS), the Hospital Anxiety and Depression Scale (HADS-A and HADS-D, respectively), and The Mini-Mental State Examination (MMSE). The physical fitness components of functional capacity were evaluated over a 2-day period, using recommendations by the American Alliance for Health, Physical Education, Recreation and Dance, and the Berg Balance Scale (BBS). Pearson correlation coefficients and multiple regressions were calculated to test the correlation between functional capacity and clinical characteristics, and to predict clinical scores from physical performance, respectively. Clinical variables and physical component data were compared between groups using analysis of variance to determine the effects of disease severity. Patients with advanced disease showed low levels of functional capacity. Interestingly, patients with good functional capacity in one of the physical fitness components also showed good capacities in the other components. Disease severity is a major factor affecting functional capacity and clinical characteristics. Medical providers should take disease severity into consideration when prescribing physical activity for PD patients, since the relationship between functional capacity and clinical characteristics is dependent on disease severity. PMID:21963176

  12. Default patterns of patients attending clinics for sexually transmitted diseases.

    PubMed

    Mahony, J D; Bevan, J; Wall, B

    1978-04-01

    The influence of gender, propaganda, and treatment methods was studied in relation to default behaviour of patients with sexually transmitted diseases. The overall default rate of men and women was similar, but a larger proportion of men defaulted after the initial visit, while the biggest fall-out in women was after the second attendance at the clinic. The institution of a propaganda campaign was followed by a reduction in defaulting. The statistical significance of this is open to question, however: moreover the observed improvement in default rate was not maintained once the propaganda had been relaxed. Men treated for non-gonococcal urethritis by a regimen which included one injection a week for three weeks showed a highly significantly lower default rate compared with those who received tablets alone.

  13. [Clinical associations between retinal vascular diseases and cardiovascular diseases in patients with systemic atheromatosis].

    PubMed

    Stefănescu-Dima, Alin; Bătăiosu, Constantin; Sas, Teodor; Puianu, Mihaela

    2013-01-01

    A clinical study was conducted on a sample of 48 patients examined within 3 months. Of these, 27 patients were recruited by ophtalmologic criteria and 21 recruited by cardiologic criteria, 25% of these patients coming for routine check. They were investigated by ophthalmic examination, cardiological examination, imaging and laboratory examination. Testing has shown a strong link between cardiovascular disease and the eye of the patients investigated. The study demonstrated the need for interdisciplinary consultation for patients with vascular complaints in the carotid territory and a close correlation between the vascular and ophthalmologic pathology at this level.

  14. Clinical Analysis of Pulmonary Nocardiosis in Patients With Autoimmune Disease

    PubMed Central

    Li, Shan; Song, Xin yu; Zhao, Yu yue; Xu, Kai; Bi, Ya lan; Huang, Hui; Xu, Zuo jun

    2015-01-01

    Abstract Nocardiosis is an opportunistic infection that most commonly involves the lung; however, only a few case reports of autoimmune disease complicated by pulmonary nocardiosis exist in the literature. We conducted a retrospective analysis of 24 cases of both autoimmune disease and pulmonary nocardiosis at the Peking Union Medical College Hospital between 1990 and 2012. Fifty-two cases were hospitalized with nocardiosis, 24 of whom had at least 1 autoimmune disease before the diagnosis of pulmonary nocardiosis. The cohort patients consisted of 5 men and 19 women, with a mean age of 44.2 years. All were negative for human immunodeficiency virus. All but 1 patient had received immunosuppressants, including corticosteroids, cyclophosphamide, azathioprine, methotrexate, or hydroxychloroquine. Fever (87.5%), cough (83.3%), and sputum (79.2%) were the most common clinical manifestations. Ten cases were accompanied by subcutaneous nodules and/or cutaneous abscesses, and 4 had brain abscess. Half of them were lymphocytopenic. Thirteen of the 16 cases who underwent lymphocyte subtype analysis had decreased CD4+ T-cell counts. Nineteen cases had decreased serum albumin levels. Nocardia was isolated from sputum (13/24), bronchoalveolar lavage fluid (4/6), lung tissue (5/6), pleural effusions (3/5), skin or cutaneous pus (7/10), and brain tissue (1/1). The most common imaging findings were air-space opacities (83.3%), followed by nodules (62.5%), cavitations (45.8%), and masses (37.5%). Five were administered co-trimoxazole only, and the others were treated with 2 or more antibiotics. All 5 cases with skin abscesses and 2 of the 4 cases with brain abscesses were treated by surgical incision and drainage. None underwent thoracic surgery. Corticosteroid dosages were decreased in all cases, and cytotoxic agents were discontinued in some cases. Twenty-two cases recovered, and 2 died. Pulmonary nocardiosis associated with an underlying autoimmune disease showed a female

  15. Clinical and radiological correlations in patients with gestational trophoblastic disease*

    PubMed Central

    Lima, Lana de Lourdes Aguiar; Parente, Raphael Câmara Medeiros; Maestá, Izildinha; Amim Junior, Joffre; de Rezende Filho, Jorge Fonte; Montenegro, Carlos Antonio Barbosa; Braga, Antônio

    2016-01-01

    Gestational trophoblastic disease is an abnormality of pregnancy that encompasses a group of diseases that differ from each other in their propensity for regression, invasion, metastasis, and recurrence. In the past, it was common for patients with molar pregnancy to present with marked symptoms: copious bleeding; theca lutein cysts; uterus larger than appropriate for gestational age; early preeclampsia; hyperemesis gravidarum; and hyperthyroidism. Currently, with early diagnosis made by ultrasound, most patients are diagnosed while the disease is still in the asymptomatic phase. In cases of progression to trophoblastic neoplasia, staging-typically with Doppler flow studies of the pelvis and chest X-ray, although occasionally with computed tomography or magnetic resonance imaging-is critical to the choice of an appropriate antineoplastic therapy regimen. Because it is an unusual and serious disease that affects women of reproductive age, as well as because its appropriate treatment results in high cure rates, it is crucial that radiologists be familiar with gestational trophoblastic disease, in order to facilitate its early diagnosis and to ensure appropriate follow-up imaging. PMID:27777478

  16. Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease.

    PubMed

    Miura, Yukiko; Saito, Takefumi; Fujita, Kazutaka; Tsunoda, Yoshiya; Tanaka, Toru; Takoi, Hiroyuki; Yatagai, Yohei; Rin, Shigen; Sekine, Akimasa; Hayashihara, Kenji; Nei, Takahito; Azuma, Arata

    2014-10-20

    Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenidone, which is an antifibrotic agent, in five patients with scleroderma-related interstitial lung disease. All patients demonstrated an increase in vital capacity.

  17. Van Buchem disease: clinical, biochemical, and densitometric features of patients and disease carriers.

    PubMed

    van Lierop, Antoon H; Hamdy, Neveen A T; van Egmond, Martje E; Bakker, Egbert; Dikkers, Freek G; Papapoulos, Socrates E

    2013-04-01

    Van Buchem disease (VBD) is a rare bone sclerosing dysplasia caused by the lack of a regulatory element of the SOST gene, which encodes for sclerostin, an osteocyte-derived negative regulator of bone formation. We studied the demographic, clinical, biochemical, and densitometric features of 15 patients with VBD (12 adults and 3 children) and 28 related carriers of the gene mutation. The most common clinical findings in patients were facial palsy (100%) and various degrees of hearing impairment (93%); raised intracranial pressure had been documented in 20%. The clinical course of the disease appeared to stabilize in adulthood, with the majority of patients reporting no progression of symptoms or development of complications with time. Carriers of the disease had none of the clinical features or complications of the disease. Sclerostin could be detected in the serum in all but 1 VBD patients (mean 8.0 pg/mL; 95% confidence interval [CI], 4.9-11.0 pg/mL), and were lower than those of carriers (mean 28.7 pg/mL; 95% CI, 24.5-32.9 pg/mL; p < 0.001) and healthy controls (mean 40.0 pg/mL; 95% CI, 34.5-41.0 pg/mL; p < 0.). Serum procollagen type 1 amino-terminal propeptide (P1NP) levels were also significantly higher in adult patients (mean 96.0; 95% CI, 54.6-137.4 ng/mL versus mean 47.8; 95% CI, 39.4-56.2 ng/mL, p = 0.003 in carriers and mean 37.8; 95% CI, 34.5-41.0 ng/mL, p = 0.028 in healthy controls) and declined with age. Bone mineral density (BMD) was markedly increased in all patients (mean Z-score 8.7 ± 2.1 and 9.5 ± 1.9 at the femoral neck and spine, respectively); BMD of carriers was significantly lower than that of patients but varied widely (mean Z-scores 0.9 ± 1.0 and 1.3 ± 1.5 at the femoral neck and spine, respectively). Serum sclerostin levels were inversely correlated with serum P1NP levels (r = -0.39, p = 0.018) and BMD values (femoral neck r = -0.69, p < 0.001; lumbar spine r = -0

  18. Multiparametric magnetic resonance imaging predicts clinical outcomes in patients with chronic liver disease

    PubMed Central

    Pavlides, Michael; Banerjee, Rajarshi; Sellwood, Joanne; Kelly, Catherine J.; Robson, Matthew D.; Booth, Jonathan C.; Collier, Jane; Neubauer, Stefan; Barnes, Eleanor

    2016-01-01

    Background & Aims Multiparametric magnetic resonance (MR) imaging has been demonstrated to quantify hepatic fibrosis, iron, and steatosis. The aim of this study was to determine if MR can be used to predict negative clinical outcomes in liver disease patients. Methods Patients with chronic liver disease (n = 112) were recruited for MR imaging and data on the development of liver related clinical events were collected by medical records review. The median follow-up was 27 months. MR data were analysed blinded for the Liver Inflammation and Fibrosis score (LIF; <1, 1–1.99, 2–2.99, and ⩾3 representing normal, mild, moderate, and severe liver disease, respectively), T2∗ for liver iron content and proportion of liver fat. Baseline liver biopsy was performed in 102 patients. Results Liver disease aetiologies included non-alcoholic fatty liver disease (35%) and chronic viral hepatitis (30%). Histologically, fibrosis was mild in 54 (48%), moderate in 17 (15%), and severe in 31 (28%) patients. Overall mortality was 5%. Ten patients (11%) developed at least one liver related clinical event. The negative predictive value of LIF <2 was 100%. Two patients with LIF 2–2.99 and eight with LIF ⩾3 had a clinical event. Patients with LIF ⩾3 had a higher cumulative risk for developing clinical events, compared to those with LIF <1 (p = 0.02) and LIF 1–1.99 (p = 0.03). Cox regression analysis including all 3 variables (fat, iron, LIF) resulted in an enhanced LIF predictive value. Conclusions Non-invasive standardised multiparametric MR technology may be used to predict clinical outcomes in patients with chronic liver disease. PMID:26471505

  19. Clinical outcomes and management of inflammatory bowel disease in the older patient.

    PubMed

    Ha, Christina Y; Katz, Seymour

    2013-02-01

    The management of inflammatory bowel disease in the older patient extends beyond the gastrointestinal tract. Pre-existing comorbidities, polypharmacy, functional status and physical reserve can impact disease course, response to therapy and quality of life. Current therapeutic endpoints may not be as immediately applicable to the older IBD patient at higher risk for adverse outcomes. This review focuses on the latest studies addressing the natural history, clinical course and therapeutic outcomes among the older IBD cohort.

  20. Clinical strategies for complete denture rehabilitation in a patient with Parkinson disease and reduced neuromuscular control.

    PubMed

    Haralur, Satheesh B

    2015-01-01

    The dentist has a large role in geriatric health care for the ever increasing elder population with associated physical and neurological disorders. The Parkinson disease is progressive neurological disorder with resting tremor, bradykinesia, akinesia, and postural instability. The psychological components of disease include depression, anxiety, and cognitive deficiency. Poor oral hygiene, increased susceptibility for dental caries, and periodontal diseases predispose them to early edentulism. The number of Parkinson affected patients visiting dental clinic seeking complete denture is growing. This case report explains the steps involved in the complete denture rehabilitation of Parkinson patient. The effective prosthesis will help in alleviating functional, aesthetic, and psychological disabilities of the patient.

  1. Clinical and immunological data of nine patients with chronic mucocutaneous candidiasis disease.

    PubMed

    Dotta, Laura; Scomodon, Omar; Padoan, Rita; Timpano, Silviana; Plebani, Alessandro; Soresina, Annarosa; Lougaris, Vassilios; Concolino, Daniela; Nicoletti, Angela; Giardino, Giuliana; Licari, Amelia; Marseglia, Gianluigi; Pignata, Claudio; Tamassia, Nicola; Facchetti, Fabio; Vairo, Donatella; Badolato, Raffaele

    2016-06-01

    This paper describes the heterogeneous clinical phenotype of a cohort of nine patients diagnosed with heterozygous mutations in STAT1. We report data of extended immunophenotyping over time and we show lung damage in four patients. The increased phosphorylation of STAT1 in response to IFNγ and IFNα stimulation proves the gain-of-function nature of the defects. The data are supplemental to our original article concurrently published "Clinical heterogeneity of dominant chronic mucocutaneous candidiasis disease: presenting as treatment-resistant candidiasis and chronic lung disease" [1], where additional results and interpretation of our research can be found. PMID:26981552

  2. Reasons that prevent the inclusion of Alzheimer's disease patients in clinical trials

    PubMed Central

    Rollin-Sillaire, Adeline; Breuilh, Laetitia; Salleron, Julia; Bombois, Stéphanie; Cassagnaud, Pascaline; Deramecourt, Vincent; Mackowiak, Marie-Anne; Pasquier, Florence

    2013-01-01

    Aim To assess reasons that prevent Alzheimer's disease (AD) patients from being included in clinical trials. Methods In 2009, we reviewed the Lille Memory Clinic's case database to identify patients suitable for inclusion in four AD clinical trials. An initial selection was made on the basis of four criteria: (i) a diagnosis of AD (with or without white matter lesions [WML]), (ii) age, (iii) mini mental state examination (MMSE) score and (iv) symptomatic treatment of AD (cholinesterase inhibitors/memantine). Next, data on patients fulfilling these criteria were reviewed against all the inclusion/exclusion criteria for four clinical trials performed in 2009 at the Memory Clinic. Reasons for non-inclusion were analyzed. Results Two hundred and five patients were selected according to the four initial criteria. Reasons for subsequently not including some of patients in clinical trials were abnormalities on MRI (56.9%, 88.9% of which were WML), unauthorized medication (37.3%), the lack of a study partner/informant (37.1%), the presence of a non-authorized disease (24.4%), contraindication to MRI (9%), a change in diagnosis over time (3.9%), visual/auditory impairments (2.9%), alcohol abuse (2%) and an insufficient educational level (1%). Conclusion A high proportion of AD patients presented with vascular abnormalities on MRI. This was not unexpected, since the patients were selected from the database and, as shown in epidemiologic studies, cerebrovascular diseases are frequently associated with AD. The presence of a study partner is essential for enabling a patient to participate in clinical trials because of the need to record reliably primary and secondary outcomes. PMID:22891847

  3. Herpes Genitalis in Patients Attending a Clinic for Sexually Transmitted Diseases

    PubMed Central

    Lawee, David; Gutman, Mory; Hrytzay, Millie; McLachlin, Jeanette

    1983-01-01

    In a prospective study of 210 patients attending a hospital-based sexually transmitted disease clinic, we documented the prevalence of genital herpes infection (GHI) and its association with gonococcal infection (GI). Herpes simplex virus type 2 was cultured from 58% of symptomatic patients and 0.5% of asymptomatic patients. The ratio of GI to GHI was 41:31 by clinical criteria. The laboratory-confirmed ratio was 41:18. These ratios are much higher than those normally used to estimate the caseload of GHI. PMID:21283317

  4. Management and Prevention of Herpes Zoster in the Immunocompromised Inflammatory Bowel Disease Patient: A Clinical Quandary.

    PubMed

    Côté-Daigneault, Justin; Peerani, Farhad; MacMahon, Eithne; Delaporte, Emmanuel; Rahier, Jean-François; Colombel, Jean-Frédéric

    2016-10-01

    Crohn's disease (CD) and ulcerative colitis (UC), the 2 main clinical phenotypes of inflammatory bowel disease (IBD), are diseases that result from a dysregulated immune response to gut microbiota in genetically susceptible hosts. This aberrant immune response may intrinsically predispose IBD patients to infectious complications. Moreover, immunosuppressive medications used to treat IBD including corticosteroids, thiopurines, methotrexate, calcineurin inhibitors, anti-tumor necrosis factor (anti-TNF) agents and other biologics, further increase patients' susceptibility to opportunistic infections. Herpes zoster (HZ), also known as shingles, is an opportunistic viral reactivation often observed in IBD patients with several case reports demonstrating complicated or disseminated disease in those on immunosuppression. While HZ vaccination is recommended in all immunocompetent adults aged ≥60 years, as a live virus vaccine, it is currently contraindicated in IBD patients on anti-TNF therapy and in other significantly immunocompromised patient groups. While caution is still warranted in these circumstances, recent clinical data has emerged which has prompted us to review and examine the universal approach to HZ vaccination in the immunosuppressed IBD population. In the following narrative review, we will discuss and provide an overview of the clinical manifestations, incidence, management and prevention of HZ in the IBD patient.

  5. Management and Prevention of Herpes Zoster in the Immunocompromised Inflammatory Bowel Disease Patient: A Clinical Quandary.

    PubMed

    Côté-Daigneault, Justin; Peerani, Farhad; MacMahon, Eithne; Delaporte, Emmanuel; Rahier, Jean-François; Colombel, Jean-Frédéric

    2016-10-01

    Crohn's disease (CD) and ulcerative colitis (UC), the 2 main clinical phenotypes of inflammatory bowel disease (IBD), are diseases that result from a dysregulated immune response to gut microbiota in genetically susceptible hosts. This aberrant immune response may intrinsically predispose IBD patients to infectious complications. Moreover, immunosuppressive medications used to treat IBD including corticosteroids, thiopurines, methotrexate, calcineurin inhibitors, anti-tumor necrosis factor (anti-TNF) agents and other biologics, further increase patients' susceptibility to opportunistic infections. Herpes zoster (HZ), also known as shingles, is an opportunistic viral reactivation often observed in IBD patients with several case reports demonstrating complicated or disseminated disease in those on immunosuppression. While HZ vaccination is recommended in all immunocompetent adults aged ≥60 years, as a live virus vaccine, it is currently contraindicated in IBD patients on anti-TNF therapy and in other significantly immunocompromised patient groups. While caution is still warranted in these circumstances, recent clinical data has emerged which has prompted us to review and examine the universal approach to HZ vaccination in the immunosuppressed IBD population. In the following narrative review, we will discuss and provide an overview of the clinical manifestations, incidence, management and prevention of HZ in the IBD patient. PMID:27598743

  6. Clinical Utility of Serologic Testing for Celiac Disease in Asymptomatic Patients

    PubMed Central

    2011-01-01

    Executive Summary Objective The objective of this evidence-based analysis was to evaluate the clinical utility of serologic testing for celiac disease in asymptomatic individuals presenting with one of the non-gastrointestinal conditions evaluated in this report. The clinical utility was based on the effects of a gluten-free diet (GFD) on outcomes specific to each of these conditions. The prevalence of celiac disease in asymptomatic individuals and one of these non-gastrointestinal conditions was also evaluated. Clinical Need and Target Population Celiac Disease Celiac disease is an autoimmune disease characterized by a chronic inflammatory state of the proximal small bowel mucosa accompanied by structural and functional changes. Technology Under Evaluation Serologic Tests for Celiac Disease There are a number of serologic tests for celiac disease available. Serologic tests are automated with the exception of the anti-endomysial antibody test, which is more time-consuming and operator-dependent than the other tests. Research Questions What is the prevalence of asymptomatic celiac disease in patients presenting with one of the non-gastrointestinal conditions evaluated? What is the effect of the gluten-free diet on condition-specific outcomes in patients with asymptomatic celiac disease presenting with one of the non-gastrointestinal conditions evaluated? What is the clinical utility of serologic testing for celiac disease in asymptomatic patients presenting with one of the non-gastrointestinal conditions evaluated? The clinical utility was defined as the impact of the GFD on disease specific outcomes. What is the risk of all-cause mortality and lymphoma in individuals with asymptomatic celiac disease? What is the budget impact of serologic testing for celiac disease in asymptomatic subjects presenting with one of the non-gastrointestinal conditions evaluated? Research Methods Study Population The study population consisted of individuals with newly diagnosed celiac

  7. Clinical care of two patients with Ebola virus disease in the United States.

    PubMed

    Lyon, G Marshall; Mehta, Aneesh K; Varkey, Jay B; Brantly, Kent; Plyler, Lance; McElroy, Anita K; Kraft, Colleen S; Towner, Jonathan S; Spiropoulou, Christina; Ströher, Ute; Uyeki, Timothy M; Ribner, Bruce S

    2014-12-18

    West Africa is currently experiencing the largest outbreak of Ebola virus disease (EVD) in history. Two patients with EVD were transferred from Liberia to our hospital in the United States for ongoing care. Malaria had also been diagnosed in one patient, who was treated for it early in the course of EVD. The two patients had substantial intravascular volume depletion and marked electrolyte abnormalities. We undertook aggressive supportive measures of hydration (typically, 3 to 5 liters of intravenous fluids per day early in the course of care) and electrolyte correction. As the patients' condition improved clinically, there was a concomitant decline in the amount of virus detected in plasma.

  8. Clinical and immunological data of nine patients with chronic mucocutaneous candidiasis disease

    PubMed Central

    Dotta, Laura; Scomodon, Omar; Padoan, Rita; Timpano, Silviana; Plebani, Alessandro; Soresina, Annarosa; Lougaris, Vassilios; Concolino, Daniela; Nicoletti, Angela; Giardino, Giuliana; Licari, Amelia; Marseglia, Gianluigi; Pignata, Claudio; Tamassia, Nicola; Facchetti, Fabio; Vairo, Donatella; Badolato, Raffaele

    2016-01-01

    This paper describes the heterogeneous clinical phenotype of a cohort of nine patients diagnosed with heterozygous mutations in STAT1. We report data of extended immunophenotyping over time and we show lung damage in four patients. The increased phosphorylation of STAT1 in response to IFNγ and IFNα stimulation proves the gain-of-function nature of the defects. The data are supplemental to our original article concurrently published “Clinical heterogeneity of dominant chronic mucocutaneous candidiasis disease: presenting as treatment-resistant candidiasis and chronic lung disease” [1], where additional results and interpretation of our research can be found. PMID:26981552

  9. Unrevealed Depression Involves Dysfunctional Coping Strategies in Crohn's Disease Patients in Clinical Remission

    PubMed Central

    Viganò, Caterina; Calzolari, Roberta; Marinaccio, Paola Marianna; Bezzio, Cristina; Furfaro, Federica; Ba, Gabriella; Maconi, Giovanni

    2016-01-01

    Background and Aims. This study investigated the proportion of CD patients in clinical remission with clinical depression, and coping strategies in those with severe depressive disorders. Materials and Methods. One hundred consecutive CD patients in clinical remission were screened for anxiety and depression by using Hospital Anxiety and Depression Scale and patients with depressive symptoms were further investigated by means of Cognitive Behavioural Assessment 2.0 and Beck Depression Inventory (BDI). Afterwards the coping strategies were assessed through the Brief-COPE questionnaire. Results. Twenty-one patients had anxious symptoms and 16 had depressive symptoms with or without anxiety. Seven of these patients (43.8%) showed significant depressive symptoms. Compared to patients without psychiatric disorders, these patients showed significant lower score in “positive reframing” (p: 0.017) and in “planning” (p: 0.046) and higher score in “use of instrumental social support” (p < 0.001), in “denial” scale (p: 0.001), and in “use of emotional social support” (p: 0.003). Conclusions. Depressed CD patients in clinical remission may have dysfunctional coping strategies, meaning that they may not be able to implement functional strategies to manage at best stress related with their disease. PMID:26823663

  10. [Clinical aspects of the evolution of dental caries and periodontal disease in patients treated with corticosteroids].

    PubMed

    Lăcătuşu, St; Ghiorghe, Angela

    2004-01-01

    Patients treated with adrenal glucocorticoids may run a higher risk of dental caries, both as a result of their medical condition and of the physical and physiological effects of their pharmacotherapy. Our clinical study reports about patients treated with glucocorticoids who were also having an odonto-periodontal condition. They were examined and we found rampant caries and periodontal diseases. The slow evolution of asymptomatic periodontal disease encouraged destruction of teeth in root caries. The rampant caries were correlated with immunodeficiency and treatment of these caries must take into account the general treatment.

  11. Detection of Borreliae in Archived Sera from Patients with Clinically Suspect Lyme Disease

    PubMed Central

    Lee, Sin Hang; Vigliotti, Jessica S.; Vigliotti, Veronica S.; Jones, William; Shearer, David M.

    2014-01-01

    The diagnoses of Lyme disease based on clinical manifestations, serological findings and detection of infectious agents often contradict each other. We tested 52 blind-coded serum samples, including 20 pre-treatment and 12 post-treatment sera from clinically suspect Lyme disease patients, for the presence of residual Lyme disease infectious agents, using nested PCR amplification of a signature segment of the borrelial 16S ribosomal RNA gene for detection and direct DNA sequencing of the PCR amplicon for molecular validation. These archived sera were split from the samples drawn for the 2-tier serology tests performed by a CDC-approved laboratory, and are used as reference materials for evaluating new diagnostic reagents. Of the 12 post-treatment serum samples, we found DNA evidence of a novel borrelia of uncertain significance in one, which was also positive for the 2-tier serology test. The rest of the post-treatment sera and all 20 control sera were PCR-negative. Of the 20 pre-treatment sera from clinically suspect early Lyme disease patients, we found Borrelia miyamotoi in one which was 2-tier serology-negative, and a Borrelia burgdorferi in two—one negative and one positive for 2-tier serology. We conclude that a sensitive and reliable DNA-based test is needed to support the diagnosis of Lyme disease and Lyme disease-like borreliosis. PMID:24619223

  12. Detection of borreliae in archived sera from patients with clinically suspect Lyme disease.

    PubMed

    Lee, Sin Hang; Vigliotti, Jessica S; Vigliotti, Veronica S; Jones, William; Shearer, David M

    2014-03-11

    The diagnoses of Lyme disease based on clinical manifestations, serological findings and detection of infectious agents often contradict each other. We tested 52 blind-coded serum samples, including 20 pre-treatment and 12 post-treatment sera from clinically suspect Lyme disease patients, for the presence of residual Lyme disease infectious agents, using nested PCR amplification of a signature segment of the borrelial 16S ribosomal RNA gene for detection and direct DNA sequencing of the PCR amplicon for molecular validation. These archived sera were split from the samples drawn for the 2-tier serology tests performed by a CDC-approved laboratory, and are used as reference materials for evaluating new diagnostic reagents. Of the 12 post-treatment serum samples, we found DNA evidence of a novel borrelia of uncertain significance in one, which was also positive for the 2-tier serology test. The rest of the post-treatment sera and all 20 control sera were PCR-negative. Of the 20 pre-treatment sera from clinically suspect early Lyme disease patients, we found Borrelia miyamotoi in one which was 2-tier serology-negative, and a Borrelia burgdorferi in two-one negative and one positive for 2-tier serology. We conclude that a sensitive and reliable DNA-based test is needed to support the diagnosis of Lyme disease and Lyme disease-like borreliosis.

  13. Patient-reported outcomes as primary end points in clinical trials of inflammatory bowel disease.

    PubMed

    Williet, Nicolas; Sandborn, William J; Peyrin-Biroulet, Laurent

    2014-08-01

    The Food and Drug Administration (FDA) is moving from the Crohn's Disease Activity Index to patient-reported outcomes (PROs) and objective measures of disease, such as findings from endoscopy. PROs will become an important aspect of assessing activity of inflammatory bowel disease (IBD) and for labeling specific drugs for this disease. PROs always have been considered in the management of patients with rheumatoid arthritis or multiple sclerosis, and have included measurements of quality of life, disability, or fatigue. Several disease-specific scales have been developed to assess these PROs and commonly are used in clinical trials. Outcomes reported by patients in clinical trials of IBD initially focused on quality of life, measured by the Short-Form 36 questionnaire or disease-specific scales such as the Inflammatory Bowel Disease Questionnaire or its shorter version. Recently considered factors include fatigue, depression and anxiety, and work productivity, as measured by the Functional Assessment Chronic Illness Therapy-Fatigue, the Hospital Anxiety Depression, and the Work Productivity Activity Impairment Questionnaire, respectively. However, few data are available on how treatment affects these factors in patients with IBD. Although disability generally is recognized in patients with IBD, it is not measured. The international IBD disability index currently is being validated. None of the PROs currently used in IBD were developed according to FDA guidance for PRO development. PROs will be a major primary end point of future trials. FDA guidance is needed to develop additional PROs for IBD that can be incorporated into trials, to better compare patients' experience with different therapies. PMID:24534550

  14. The prevalence and clinical characteristics of anemia in Korean patients with inflammatory bowel disease

    PubMed Central

    Lee, Dae Sung; Bang, Ki Bae; Kim, Ji Yeon; Jung, Yoon Suk; Park, Jung Ho; Kim, Hong Joo; Cho, Yong Kyun; Sohn, Chong Il; Jeon, Woo Kyu; Kim, Byung Ik; Choi, Kyu Young

    2016-01-01

    Background/Aims Quality of life is closely related to anemia in patients with inflammatory bowel disease (IBD). Several studies have reported on anemia in patients with IBD in Western countries. This study investigated the prevalence and clinical characteristics of anemia in Korean patients with IBD. Methods We reviewed the medical records of 92 patients with ulcerative colitis (UC) and 76 patients with Crohn's disease (CD) who were followed regularly at a single tertiary medical center in Korea between January 2003 and December 2012. Hemoglobin (Hb) thresholds used to define anemia were <13.0 g/dL in men and <12.0 g/dL in women according to the World Health Organization criteria. We chose the lowest Hb level in each year as a representative value because Hb levels changed at each examination and anemia was associated with disease deterioration. The relationship between clinical variables and lowest Hb level was assessed. Results The prevalence of anemia was 36.3% in patients with UC and 41.6% in patients with CD. Anemia in patients with CD was associated with hospital admission, 5-aminosalicylate (5-ASA) and infliximab treatment in men. Anemia in patients with UC was associated with hospital admission, oral steroid use, thiopurine and infliximab treatment in men. Conclusions The prevalence of anemia in Korean patients with IBD was comparable to that of patients in Western countries. Anemia was associated with male patients with CD who were admitted to the hospital and received medications including 5-ASA and infliximab, and men with UC who were admitted to the hospital and received medications including oral steroids, thiopurine and infliximab. PMID:26884734

  15. Atrophy, hypometabolism and clinical trajectories in patients with amyloid-negative Alzheimer's disease.

    PubMed

    Chételat, Gaël; Ossenkoppele, Rik; Villemagne, Victor L; Perrotin, Audrey; Landeau, Brigitte; Mézenge, Florence; Jagust, William J; Dore, Vincent; Miller, Bruce L; Egret, Stéphanie; Seeley, William W; van der Flier, Wiesje M; La Joie, Renaud; Ames, David; van Berckel, Bart N M; Scheltens, Philip; Barkhof, Frederik; Rowe, Christopher C; Masters, Colin L; de La Sayette, Vincent; Bouwman, Femke; Rabinovici, Gil D

    2016-09-01

    See O'Sullivan and Vann (doi:10.1093/aww166) for a scientific commentary on this article.About 15% of patients clinically diagnosed with Alzheimer's disease do not show high tracer retention on amyloid positon emission tomography imaging. The present study investigates clinical and demographic features, patterns of brain atrophy and hypometabolism and longitudinal clinical trajectories of these patients. Forty amyloid-negative patients carrying a pre-scan diagnosis of Alzheimer's disease dementia from four centres were included (11/29 females/males; mean age = 67 ± 9). Detailed clinical histories, including the clinical diagnoses before and after the amyloid scan and at follow-up, were collected. Patients were classified according to their pre-scan clinical phenotype as amnestic (memory predominant), non-amnestic (predominant language, visuospatial or frontal symptoms), or non-specific (diffuse cognitive deficits). Demographic, clinical, neuropsychological, magnetic resonance imaging and (18)F-fluorodeoxyglucose positon emission tomography data were compared to 27 amyloid-positive typical Alzheimer's disease cases (14/13 females/males; mean age = 71 ± 10) and 29 amyloid-negative controls (15/14 females/males; mean age = 69 ± 12) matched for age, gender and education. There were 21 amnestic, 12 non-amnestic, and seven non-specific amyloid-negative Alzheimer's disease cases. Amyloid-negative subgroups did not differ in age, gender or education. After the amyloid scan, clinicians altered the diagnosis in 68% of amyloid-negative patients including 48% of amnestic versus 94% of non-amnestic and non-specific cases. Amnestic amyloid-negative cases were most often reclassified as frontotemporal dementia, non-amnestic as frontotemporal dementia or corticobasal degeneration, and non-specific as dementia with Lewy bodies or unknown diagnosis. The longer-term clinical follow-up was consistent with the post-scan diagnosis in most cases (90%), including in amnestic amyloid

  16. Atrophy, hypometabolism and clinical trajectories in patients with amyloid-negative Alzheimer's disease.

    PubMed

    Chételat, Gaël; Ossenkoppele, Rik; Villemagne, Victor L; Perrotin, Audrey; Landeau, Brigitte; Mézenge, Florence; Jagust, William J; Dore, Vincent; Miller, Bruce L; Egret, Stéphanie; Seeley, William W; van der Flier, Wiesje M; La Joie, Renaud; Ames, David; van Berckel, Bart N M; Scheltens, Philip; Barkhof, Frederik; Rowe, Christopher C; Masters, Colin L; de La Sayette, Vincent; Bouwman, Femke; Rabinovici, Gil D

    2016-09-01

    See O'Sullivan and Vann (doi:10.1093/aww166) for a scientific commentary on this article.About 15% of patients clinically diagnosed with Alzheimer's disease do not show high tracer retention on amyloid positon emission tomography imaging. The present study investigates clinical and demographic features, patterns of brain atrophy and hypometabolism and longitudinal clinical trajectories of these patients. Forty amyloid-negative patients carrying a pre-scan diagnosis of Alzheimer's disease dementia from four centres were included (11/29 females/males; mean age = 67 ± 9). Detailed clinical histories, including the clinical diagnoses before and after the amyloid scan and at follow-up, were collected. Patients were classified according to their pre-scan clinical phenotype as amnestic (memory predominant), non-amnestic (predominant language, visuospatial or frontal symptoms), or non-specific (diffuse cognitive deficits). Demographic, clinical, neuropsychological, magnetic resonance imaging and (18)F-fluorodeoxyglucose positon emission tomography data were compared to 27 amyloid-positive typical Alzheimer's disease cases (14/13 females/males; mean age = 71 ± 10) and 29 amyloid-negative controls (15/14 females/males; mean age = 69 ± 12) matched for age, gender and education. There were 21 amnestic, 12 non-amnestic, and seven non-specific amyloid-negative Alzheimer's disease cases. Amyloid-negative subgroups did not differ in age, gender or education. After the amyloid scan, clinicians altered the diagnosis in 68% of amyloid-negative patients including 48% of amnestic versus 94% of non-amnestic and non-specific cases. Amnestic amyloid-negative cases were most often reclassified as frontotemporal dementia, non-amnestic as frontotemporal dementia or corticobasal degeneration, and non-specific as dementia with Lewy bodies or unknown diagnosis. The longer-term clinical follow-up was consistent with the post-scan diagnosis in most cases (90%), including in amnestic amyloid

  17. Influence of clinical and demographic variables on quality of life in patients with Parkinson's disease

    PubMed Central

    Karlsen, K. H.; Larsen, J.; Tandberg, E.; Maland, J.

    1999-01-01

    OBJECTIVES—To identify the clinical and demographic factors that are associated with a poor quality of life in patients with Parkinson's disease.
METHODS—233 of a total of 245 patients identified in a community based study in a Norwegian county participated in the study. Quality of life was measured by the Nottingham Health Profile (NHP). The results were compared with those in 100 healthy elderly people. Clinical and demographic variables were determined during a semistructured interview and by clinical examination by a neurologist. Multiple regression analyses were used to determine which variables were associated with higher distress scores.
RESULTS—Patients with Parkinson's disease had higher distress scores than the healthy elderly people for all the NHP dimensions. The variables that most strongly predicted a high total NHP score were depressive symptoms, self reported insomnia, and a low degree of independence, measured by the Schwab and England scale. Severity of parkinsonism contributed, but to a lesser extent. Nearly half the patients with Parkinson's disease reported lack of energy, compared with a fifth of the control group. Severity of depressive symptoms and a higher score on the UPDRS motor subscale only partly accounted for this finding. Only 30% of the variation in NHP energy score was explained by the predictive variables identified in this study.
CONCLUSIONS—Parkinson's disease has a substantial impact on health related quality of life. Depressive symptoms and sleep disorders correlated strongly with high distress scores. Patients with Parkinson's disease should be examined for both conditions, which require treatment. Low energy was commonly reported and may be a separate entity of Parkinson's disease.

 PMID:10201412

  18. [Clinical and serologic course of patients with mixed connective tissue disease].

    PubMed

    López-Longo, F J; Fernández, J; Monteagudo, I; Rodríguez-Mahou, M; Sánchez-Atrio, A I; Pérez, T; Escalona, M; González, C M; Lapointe, N; Carreño, L

    1994-09-01

    The so called mixed connective tissue disease (MCTD), continues to be a controversial entity, while some authors considered it a good characterized disease, others think that is an undifferentiated connective tissue disease. OBJECTIVE. To analyse the clinical and serological evolution of a group of patients diagnosed of MCTD, with particular consideration to the meaning of anti-nRNP and anti-Sm antibodies. METHOD. We have studied 20 patients diagnosed of MCTD and 112 with systemic lupus erythematosus (SLE). Anti-nRNP and anti-Sm antibodies were detected through counter immunoelectrophoresis, immunoblotting and ELISA. RESULTS. After an average time of evolution of 10 years, 70% (14/20) of the patients diagnosed of MCTD fulfill criteria for SLE (6 cases), scleroderma (6 cases) or polymyositis (2 cases). Anti-nRNP response is persistent, directed mainly against the 70 Kd and A-nRNP polypeptides and qualitatively higher in MCTD in SLE (absorbencies 2.64 vs 1.25. The immunoblotting test detected anti-Sm antibodies in 5 patients (25%) and ELISA test in 14 (70%). CONCLUSIONS. Clinical and serological evolution suggest that MCTD is an undifferentiated connective tissue disease. Anti-nRNP antibodies are characteristic, although anti-Sm antibodies can be detected with ELISA regardless whether on not patients fulfill SLE criteria.

  19. Challenges in the clinical measurement of ocular surface disease in glaucoma patients.

    PubMed

    Pflugfelder, Stephen C; Baudouin, Christophe

    2011-01-01

    Ocular surface disease (OSD) is common among glaucoma patients. Clinical assessment of OSD can be challenging. This review focuses on some of the limitations relating to both subjective and objective measures of OSD, including dry eye. A survey of the literature was conducted to identify the caveats associated with different methods of assessing OSD. The effect of preservatives on the ocular surface, with respect to glaucoma patients in particular, was also reviewed. Objective methods for assessing ocular surface health and disease include the Schirmer test, tear break-up time, fluorescein turnover, corneal and conjunctival staining, tear osmolarity, and vital dyes. These measures all have limitations in terms of their ability to grade the severity of OSD. Previous studies using the OSD Index showed a mild-to-moderate correlation to dry eye disease severity. Other scoring systems for dry eye have shown a relationship to patient symptom scores or quality of life. Due to the challenges clinicians face concerning both subjective and objective ocular surface health assessments, discerning clinical improvement in ocular surface disease can be a challenge. Further research is needed in order to optimize existing clinical methods and/or identify alternative techniques for assessing OSD in the glaucoma population. PMID:22125404

  20. Challenges in the clinical measurement of ocular surface disease in glaucoma patients

    PubMed Central

    Pflugfelder, Stephen C; Baudouin, Christophe

    2011-01-01

    Ocular surface disease (OSD) is common among glaucoma patients. Clinical assessment of OSD can be challenging. This review focuses on some of the limitations relating to both subjective and objective measures of OSD, including dry eye. A survey of the literature was conducted to identify the caveats associated with different methods of assessing OSD. The effect of preservatives on the ocular surface, with respect to glaucoma patients in particular, was also reviewed. Objective methods for assessing ocular surface health and disease include the Schirmer test, tear break-up time, fluorescein turnover, corneal and conjunctival staining, tear osmolarity, and vital dyes. These measures all have limitations in terms of their ability to grade the severity of OSD. Previous studies using the OSD Index showed a mild-to-moderate correlation to dry eye disease severity. Other scoring systems for dry eye have shown a relationship to patient symptom scores or quality of life. Due to the challenges clinicians face concerning both subjective and objective ocular surface health assessments, discerning clinical improvement in ocular surface disease can be a challenge. Further research is needed in order to optimize existing clinical methods and/or identify alternative techniques for assessing OSD in the glaucoma population. PMID:22125404

  1. Prevalence of Chronic Kidney Disease among Patients Attending a Specialist Diabetes Clinic in Jamaica

    PubMed Central

    Ferguson, TS; Tulloch-Reid, MK; Younger-Coleman, NO; Wright-Pascoe, RA; Boyne, MS; Soyibo, AK; Wilks, RJ

    2015-01-01

    ABSTRACT Objectives: To estimate the prevalence of chronic kidney disease (CKD) among patients attending the University Hospital of the West Indies (UHWI) Diabetes Clinic and to determine the proportion of patients at high risk for adverse outcomes. Methods: We conducted a cross-sectional study among patients attending the UHWI Diabetes Clinic between 2009 and 2010. Trained nurses administered a questionnaire, reviewed dockets, and performed urinalyses. Estimated glomerular filtration rate (eGFR) was calculated using the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. Albuminuria was assessed using urine test strips for protein and microalbumin. Chronic kidney disease was defined as an eGFR < 60 ml/min/1.73m2 or albuminuria ≥ 30 mg/g creatinine. Risk of adverse outcome (all-cause mortality, cardiovascular disease and kidney failure) was determined using the Kidney Disease: Improving Global Outcome (KDIGO) 2012 prognosis grid. Results: Participants included 100 women and 32 men (mean age, 55.4 ± 12.9 years, mean duration of diabetes, 16.7 ± 11.7 years). Twenty-two per cent of participants had eGFR < 60 ml/min/1.73m2. Moderate albuminuria (30–300 mg/g) was present in 20.5% of participants and severe albuminuria (> 300 mg/g) in 62.1%. Overall prevalence of CKD was 86.3% (95% CI 80.4%, 92.2%). Based on KDIGO risk categories, 50.8% were at high risk and 17.4% at very high risk of adverse outcomes. Conclusion: Most patients at the UHWI Diabetes Clinic had CKD and were at high or very high risk of adverse outcomes. Further studies to determine the burden of CKD in other clinical settings and to identify the best strategies for preventing adverse outcomes in developing countries need to be conducted. PMID:26426170

  2. Association of nailfold capillary changes with disease activity, clinical and laboratory findings in patients with dermatomyositis

    PubMed Central

    Shenavandeh, Saeedeh; Zarei Nezhad, Maryam

    2015-01-01

    Background: The present study aimed to investigate the Nailfold Capillaroscopy (NC) features of the patients with dermatomyositis (DM) and its correlation with their disease activity indices, physical findings, and laboratory results. Methods: The present cross-sectional study was conducted on 27 DM patients above 16 years old who had referred to an(there are 3 clinics not one) outpatient rheumatology clinics from 2012 to 2013. Nailfold capillaroscopy and calculation of disease activity indices were performed separately for all the patients by two rheumatologists who were blinded to each other's results. Statistical analyses were performed using chi-square and Mann-Whitney U tests. Results: The mean age of the patients was 39.2±14.1 years with the mean disease duration of 13.1±15.2 months (range: 1-72 months). Myopathic electromyography (EMG) findings showed a strong association with scleroderma pattern (p=0.015). However, disease activity in each organ system and global disease activity showed no significant association between scleroderma pattern and other NC findings. (Disease activity in each organ system and also global disease activity were both assessed to see if they are associated with scleroderma pattern and other NC findings so if we use between it means we are looking for an association between scleroderma pattern and other NC findings and this is not what we have done and is wrong.) Conclusion: This study revealed no significant relationship between disease activity indices and NC features. Thus, it may be more precise to interpret the results of NC in conjunction with other physical and laboratory findings. PMID:26793626

  3. The French Gaucher’s disease registry: clinical characteristics, complications and treatment of 562 patients

    PubMed Central

    2012-01-01

    Background Clinical features, complications and treatments of Gaucher’s disease (GD), a rare autosomal–recessive disorder due to a confirmed lysosomal enzyme (glucocerebrosidase) deficiency, are described. Methods All patients with known GD, living in France, with ≥1 consultations (1980–2010), were included in the French GD registry, yielding the following 4 groups: the entire cohort, with clinical description; and its subgroups: patients with ≥1 follow-up visits, to investigate complications; recently followed (2009–2010) patients; and patients treated during 2009–2010, to examine complications before and during treatment. Data are expressed as medians (range) for continuous variables and numbers (%) for categorical variables. Results Among the 562 registry patients, 265 (49.6%) were females; 454 (85.0%) had type 1, 22 (4.1%) type 2, 37 (6.9%) perinatal–lethal type and 21 (3.9%) type 3. Median ages at first GD symptoms and diagnosis, respectively, were 15 (0–77) and 22 (0–84) years for all types. The first symptom diagnosing GD was splenomegaly and/or thrombocytopenia (37.6% and 26.3%, respectively). Bone-marrow aspiration and/or biopsy yielded the diagnosis for 54.7% of the patients, with enzyme deficiency confirming GD for all patients. Birth incidence rate was estimated at 1/50,000 and prevalence at 1/136,000. For the 378 followed patients, median follow-up was 16.2 (0.1–67.6) years. Major clinical complications were bone events (BE; avascular necrosis, bone infarct or pathological fracture) for 109 patients, splenectomy for 104, and Parkinson’s disease for 14; 38 patients died (neurological complications for 15 type-2 and 3 type-3 patients, GD complications for 11 type-1 and another disease for 9 type-1 patients). Forty-six had monoclonal gammopathy. Among 283 recently followed patients, 36 were untreated and 247 had been treated during 2009–2010; 216 patients received treatment in December 2010 (126 with imiglucerase, 45

  4. Cluster-Randomized Trial of Clinical Pharmacist Tobacco Cessation Counseling Among Patients with Cardiovascular Disease.

    PubMed

    Adams, Jody; Cymbala, Alicia A; Delate, Thomas; Kurz, Deanna; Olson, Kari L; Youngblood, Morgan; Zadvorny, Emily

    2015-08-01

    Optimal management of patients with cardiovascular disease (CVD) includes evaluation of risk factors using a team-based approach. Tobacco use often receives less attention than other CVD risk factors; therefore, utilization of nonphysician health care providers may be valuable in addressing tobacco use. The purpose of this trial was to assess the impact of brief, structured, telephone tobacco cessation counseling (BST) delivered by clinical pharmacists on tobacco cessation attempts compared to usual care. The BST consisted of 1 to 5 minutes discussing 3 key counseling points, including a recommendation to quit and education about cessation aids. This was a cluster-randomized trial of tobacco-using patients with CVD who were enrolled in a clinical pharmacist-managed, physician-directed, CVD disease state management service. Clinical pharmacists were randomized to provide usual care (control) or BST (intervention) to their tobacco-using patients during a 4-month period. Patients were surveyed 3 months later to assess their tobacco cessation attempts, use of tobacco cessation aids, and self-reported cessation. One hundred twenty patients were enrolled. Subjects were predominately white males, aged ≥65 years, with a history of myocardial infarction. One hundred and four subjects completed the follow-up survey. No differences were detected between the 36.2% and 38.6% of control and intervention subjects, respectively, reporting a tobacco cessation attempt (P=0.804) or in the other outcomes (all P>0.05). A BST delivered by clinical pharmacists may not adequately affect patient motivation enough to increase tobacco cessation attempts in tobacco-dependent patients with CVD. Future research is needed to evaluate other team-based strategies that can decrease tobacco use in patients with CVD. PMID:25647441

  5. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

    NASA Astrophysics Data System (ADS)

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-09-01

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862) while for subacute ILD patients (disease duration 3–6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.

  6. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

    PubMed Central

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-01-01

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3–6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM. PMID:27615411

  7. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis.

    PubMed

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-01-01

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3-6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM. PMID:27615411

  8. Development of anti-velaglucerase alfa antibodies in clinical trial-treated patients with Gaucher disease.

    PubMed

    Pastores, Gregory M; Turkia, Hadhami Ben; Gonzalez, Derlis E; Ida, Hiroyuki; Tantawy, Azza A G; Qin, Yulin; Qiu, Yongchang; Dinh, Quinn; Zimran, Ari

    2016-07-01

    Anti-drug antibodies may develop with biological therapies, possibly leading to a reduction of treatment efficacy and to allergic and other adverse reactions. Patients with Gaucher disease were tested for anti-drug antibodies every 6 or 12weeks in clinical studies of velaglucerase alfa enzyme replacement therapy, as part of a range of safety endpoints. In 10 studies between April 2004 and March 2015, 289 patients aged 2-84years (median 43years) were assessed for the development of anti-velaglucerase alfa antibodies. Sixty-four patients were treatment-naïve at baseline and 225 patients were switched to velaglucerase alfa from imiglucerase treatment. They received velaglucerase alfa treatment for a median of 36.4weeks (interquartile range 26.4-155.4weeks). Four patients (1.4%) became positive for anti-velaglucerase alfa IgG antibodies, two of whom had antibodies that were neutralizing in vitro, but there were no apparent changes in patients' platelet counts, hemoglobin levels or levels of CCL18 and chitotriosidase, suggestive of clinical deterioration after anti-velaglucerase alfa antibodies were detected, and no infusion-related adverse events were reported. Less than 2% of patients exposed to velaglucerase alfa tested positive for antibodies and there was no apparent correlation between anti-velaglucerase alfa antibodies and adverse events or pharmacodynamic or clinical responses. PMID:27282565

  9. Optimal management of Alzheimer’s disease patients: Clinical guidelines and family advice

    PubMed Central

    Haberstroh, Julia; Hampel, Harald; Pantel, Johannes

    2010-01-01

    Family members provide most of the patient care and administer most of the treatments to patients with Alzheimer’s disease (AD). Family caregivers have an important impact on clinical outcomes, such as quality of life (QoL). As a consequence of this service, family caregivers suffer high rates of psychological and physical illness as well as social and financial burdens. Hence, it is important to involve family caregivers in multimodal treatment settings and provide interventions that are both suitable and specifically tailored to their needs. In recent years, several clinical guidelines have been presented worldwide for evidence-based treatment of AD and other forms of dementia. Most of these guidelines have considered family advice as integral to the optimal clinical management of AD. This article reviews current and internationally relevant guidelines with emphasis on recommendations concerning family advice. PMID:20520788

  10. Phosphate Binders and Clinical Outcomes in Patients with Stage 5D Chronic Kidney Disease.

    PubMed

    Zoccali, Carmine; Mallamaci, Francesca; Cannata-Andía, Jorge

    2015-01-01

    Knowledge informing the prescription and the choice of phosphate binders in end stage kidney disease (ESKD) patients has a weak evidentiary base. To date, no placebo-controlled trial based on meaningful clinical endpoints (death, cardiovascular events, bone fractures) has been performed to test the efficacy of these drugs. By the same token, we still lack solid proof that noncalcium binders afford better clinical outcomes as compared with calcium-based binders. Without proper trials, clinical decisions about the treatment of hyperphosphatemia rest on experience and contingent clinical judgment. The use of huge doses of calcium-based binders typically prescribed in the nineties now appears unwarranted. The relationship between phosphate and the risk of death is U shaped and moderate hyperphosphatemia carries just a mild-to-moderate risk excess and may not be seen as a compelling indication for the prescription of phosphate binders. Placebo-controlled randomized clinical trials assessing whether non-calcium and calcium-based binders reduce the risk of death and cardiovascular disease events in ESKD patients remain a public health priority. PMID:26278591

  11. A Palpable Painless Axillary Mass as the Clinical Manifestation of Castleman's Disease in a Patient with Hepatitis C Disease

    PubMed Central

    Papazafiropoulou, Athanasia K.; Angelidi, Angeliki M.; Kousoulis, Antonis A.; Christofilidis, Georgios; Sagia, Chariklia; Kaftanidou, Liountmila; Manoloudaki, Kassiani; Tsavari, Aikaterini; Kranidiotis, Georgios; Kamaratos, Alexandros; Melidonis, Andreas

    2016-01-01

    Introduction. Castleman's disease (CD) is a rare lymphoproliferative disorder. CD is divided into two clinical subtypes: the most common unicentric and the less usual multicentric subtype. The majority of unicentric CD affects the mediastinum, while neck, abdomen, and axilla are less common locations. Case Presentation. Herein, we describe a rare case of unicentric CD in the right axilla in a 36-year-old white male with a medical history of hepatitis C virus infection admitted to our hospital due to palpation of a painless mass in the right axilla. Complete excision of the lesion was performed and, one year after the diagnosis, patient was free of the disease. Conclusions. Although infrequent, it is important to include CD in the differential diagnosis when evaluating axillary lymphadenopathy particularly in young patients with a low-grade inflammation process and chronic disease even in the absence of an abnormal blood picture or organomegaly. PMID:27313621

  12. A Palpable Painless Axillary Mass as the Clinical Manifestation of Castleman's Disease in a Patient with Hepatitis C Disease.

    PubMed

    Papazafiropoulou, Athanasia K; Angelidi, Angeliki M; Kousoulis, Antonis A; Christofilidis, Georgios; Sagia, Chariklia; Kaftanidou, Liountmila; Manoloudaki, Kassiani; Tsavari, Aikaterini; Kranidiotis, Georgios; Kamaratos, Alexandros; Melidonis, Andreas

    2016-01-01

    Introduction. Castleman's disease (CD) is a rare lymphoproliferative disorder. CD is divided into two clinical subtypes: the most common unicentric and the less usual multicentric subtype. The majority of unicentric CD affects the mediastinum, while neck, abdomen, and axilla are less common locations. Case Presentation. Herein, we describe a rare case of unicentric CD in the right axilla in a 36-year-old white male with a medical history of hepatitis C virus infection admitted to our hospital due to palpation of a painless mass in the right axilla. Complete excision of the lesion was performed and, one year after the diagnosis, patient was free of the disease. Conclusions. Although infrequent, it is important to include CD in the differential diagnosis when evaluating axillary lymphadenopathy particularly in young patients with a low-grade inflammation process and chronic disease even in the absence of an abnormal blood picture or organomegaly. PMID:27313621

  13. Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease.

    PubMed

    Lefaucheur, Jean-Pascal; Bachoud-Levi, Anne-Catherine; Bourdet, Catherine; Grandmougin, Thierry; Hantraye, Philippe; Cesaro, Pierre; Degos, Jean-Denis; Peschanski, Marc; Lisovoski, Fabrice

    2002-11-01

    Assessment programs recently designed to follow-up patients with Huntington's disease (HD) in therapeutic trials have not included electrophysiological testing in the list of mandatory examinations. This omission is likely due to the current lack of data establishing a clear correlation between the electrophysiological results and those of clinical assessment. We address this issue in a cohort of 36 patients at relatively early stages of the disease (I and II). Electrophysiological studies comprised the recording of palmar sympathetic skin responses (SSRs), blink reflexes (BRs), thenar long latency reflexes (LLRs), cortical somatosensory evoked potentials (SEPs), and electromyographic silent periods evoked by transcranial magnetic stimulation (SPs). Results were analyzed with reference to disease duration and staging and to specific cognitive, psychiatric, and motor alteration. SEPs were the most and very sensitive markers, because they were abnormal in 94% of patients. Except for LLRs, alteration of electrophysiological results increased in parallel to the evolution of the disease. Except for LLRs and SSR latency, electrophysiological results correlated with those of specific clinical examinations. In particular, an increased BR latency or a reduced amplitude of the N20 component of SEPs correlated with the extent of bradykinesia, whereas a reduced amplitude of SSRs or of the N30 component of SEPs correlated with hyperkinesia. Overall, electrophysiological tests, in particular SEPs and BRs, appeared sensitive and interesting in the follow-up of HD patients and correlated with various clinical parameters, suggesting that these easy to perform and noninvasive repeatable examinations could be added fruitfully to the assessment programs for HD. PMID:12465071

  14. Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities

    PubMed Central

    Kim, Hyun Jung; Lee, Hyo Eun; Yu, Jae Won

    2016-01-01

    Purpose Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. Methods We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. Results A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. Conclusion No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients. PMID:27610181

  15. Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities

    PubMed Central

    Kim, Hyun Jung; Lee, Hyo Eun; Yu, Jae Won

    2016-01-01

    Purpose Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. Methods We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. Results A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. Conclusion No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients.

  16. Genetic Markers Associated with Clinical Outcomes in Patients with Inflammatory Bowel Disease.

    PubMed

    Yamamoto-Furusho, Jesús K; Fonseca-Camarillo, Gabriela

    2015-11-01

    Genetic factors play a significant role in determining inflammatory bowel disease (IBD) susceptibility. Epidemiologic data support genetic contribution to the pathogenesis of IBD, which include familial aggregation, twin studies, and racial and ethnic differences in disease prevalence. Recently, several new genes have been identified to be involved in the genetic susceptibility to IBD. The characterization of novel genes potentially will lead to the identification of therapeutic agents and clinical assessment of phenotype and prognosis in patients with IBD. The development of genetic markers associated with clinical outcomes in patients with IBD will be very important in the future. The progress of molecular biology tools (microarrays, proteomics, and epigenetics) have progressed the field of the genetic markers discovery. The advances in bioinformatics coupled with cross-disciplinary collaborations have greatly enhanced our ability to retrieve, characterize, and analyze large amounts of data generated by the technological advances. The techniques available for markers development are genomics (single nucleotide polymorphism genotyping, pharmacogenetics, and gene expression analyses) and proteomics. This could be a potential great benefit in predicting the course of disease in individual patients and in guiding appropriate medical therapy.

  17. Clinical Correlates of Apathy in Patients Recently Diagnosed with Parkinson's Disease: The ANIMO Study

    PubMed Central

    Cubo, Esther; Benito-León, Julián; Coronell, Carlos; Armesto, Diana

    2012-01-01

    Objective Little is known about apathy in the early stages of Parkinson's disease (PD). We determined the clinical correlates of apathy in a large representative sample of patients recently diagnosed with PD (ANIMO study). Methods PD patients, diagnosed within 2 years of inclusion, were recruited in 102 outpatient clinics situated in 82 populations throughout Spain. Apathy was quantified using the Lille Apathy Rating Scale (LARS). Clinical comparisons and correlations were performed using nonparametric tests. Regression analyses were used to test the association of clinical variables with apathy. Results We recruited 557 PD patients (60.3% men) with a mean age of 68.8 ± 9.7 years, and UPDRS motor score of 21.1 ± 10.8. Apathy only was diagnosed in 186 (33.4%), and apathy and depression in 215 patients (38.6%). Patients with higher comorbidity (OR = 1.10, 95% CI 1.01−1.20, p = 0.001), motor impairment (OR = 1.07, 95% CI 1.03−1.10, p < 0.0001), and lower education (OR = 2.16, 95% CI 1.21−;3.85, p = 0.009) had higher odds of having apathy, in contrast to patients living in a rural environment (OR = 0.35, 95% CI 0.32–0.85, p = 0.01), and left predominant PD motor laterality (OR = 0.34, 95% CI 0.13–0.88, p = 0.01). LARS scores were significantly correlated with UPDRS motor scores (rs = 0.44, p < 0.001), predominantly with axial score (rs = 0.43, p < 0.001). Conclusions In PD, apathy is a very common and disabling nonmotor symptom separable from depression. Patients living in a rural environment, with lower comorbidity and motor impairment, higher education background, and left predominant PD motor laterality are at lower risk of suffering from apathy. PMID:22236943

  18. Clinical and genetic spectra in a series of Chinese patients with Charcot-Marie-Tooth disease.

    PubMed

    Wang, Rui; He, Jin; Li, Jin-Jing; Ni, Wang; Wu, Zhi-Ying; Chen, Wan-Jin; Wang, Yi

    2015-12-01

    The aim of this study was to determine the clinical features and frequencies of genetic subtypes in a series of patients with Charcot-Marie-Tooth (CMT) disease from Eastern China. Patients were divided into three subtypes, CMT1, CMT2 and hereditary neuropathy with liability to pressure palsy (HNPP), according to their electrophysiological manifestations. Multiplex ligation-dependent probe analysis (MLPA) was performed to detect duplications/deletions in the PMP22 gene. The coding regions and splice sites of the GJB1, MPZ, MFN2 and GDAP-1 genes were determined by direct sequencing. Among the 148 patients in the study, 37.2% of the cases had mutations in genes assessed. The mutation detection rate was higher in patients with family histories than in spontaneous cases. PMP22 duplication (13.5%) was predominant in this group of patients, followed by PMP22 deletion (11.5%), and point mutations in GJB1 (8.8%), MPZ (2.0%) and MFN2 (0.7%). Three novel mutations (c.151T>C and c.310 A>G in GJB1 and c.1516 C>G in MFN2) were detected. A small deletion in PMP22 exon 4 was detected in a patient with severe CMT1. Genetic tests have great value in CMT patients with family histories. The frequency of PMP22 duplications was lower in Asian patients than in others. We suggest that genetic testing strategies in CMT patients should be primarily based on electromyography data.

  19. Effect of Creatine Monohydrate on Clinical Progression in Patients With Parkinson Disease

    PubMed Central

    2015-01-01

    IMPORTANCE There are no treatments available to slow or prevent the progression of Parkinson disease, despite its global prevalence and significant health care burden. The National Institute of Neurological Disorders and Stroke Exploratory Trials in Parkinson Disease program was established to promote discovery of potential therapies. OBJECTIVE To determine whether creatine monohydrate was more effective than placebo in slowing long-term clinical decline in participants with Parkinson disease. DESIGN, SETTING, AND PATIENTS The Long-term Study 1, a multicenter, double-blind, parallel-group, placebo-controlled, 1:1 randomized efficacy trial. Participants were recruited from 45 investigative sites in the United States and Canada and included 1741 men and women with early (within 5 years of diagnosis) and treated (receiving dopaminergic therapy) Parkinson disease. Participants were enrolled from March 2007 to May 2010 and followed up until September 2013. INTERVENTIONS Participants were randomized to placebo or creatine (10 g/d) monohydrate for a minimum of 5 years (maximum follow-up, 8 years). MAIN OUTCOMES AND MEASURES The primary outcome measure was a difference in clinical decline from baseline to 5-year follow-up, compared between the 2 treatment groups using a global statistical test. Clinical status was defined by 5 outcome measures: Modified Rankin Scale, Symbol Digit Modalities Test, PDQ-39 Summary Index, Schwab and England Activities of Daily Living scale, and ambulatory capacity. All outcomes were coded such that higher scores indicated worse outcomes and were analyzed by a global statistical test. Higher summed ranks (range, 5–4775) indicate worse outcomes. RESULTS The trial was terminated early for futility based on results of a planned interim analysis of participants enrolled at least 5 years prior to the date of the analysis (n = 955). The median follow-up time was 4 years. Of the 955 participants, the mean of the summed ranks for placebo was 2360 (95

  20. Hepatitis C infection: A multi-faceted systemic disease with clinical, patient reported and economic consequences.

    PubMed

    Younossi, Zobair M; Birerdinc, Aybike; Henry, Linda

    2016-10-01

    Hepatitis C virus infection (HCV) affects approximately 170-200 million individuals globally. HCV is one of the primary causes of hepatocellular carcinoma (HCC) and cirrhosis and has been identified as the leading indication for liver transplantation in most Western countries. Because HCV is a systemic disease with hepatic, extrahepatic, economic and patient reported consequences, it is important for healthcare practitioners to understand the comprehensive and multi-faceted picture of this disease. In this context, it is important to fully appreciate the impact of HCV on the individual patient and the society. With the recent advent of the new generation of direct antiviral agents, the long standing goal of eradicating HCV in most infected patients has been accomplished. Therefore, now more than ever, it is critical to assess the total benefits of sustained virological response in a comprehensive manner. This should not be limited to the clinical benefits of HCV cure, but also to account for the improvement of patient reported health and economic outcomes of HCV cure. It is only through this comprehensive approach to HCV and its treatment that we will understand the full impact of this disease and the tremendous gains that have been achieved with the new antiviral regimens. PMID:27641981

  1. Hepatitis C infection: A multi-faceted systemic disease with clinical, patient reported and economic consequences.

    PubMed

    Younossi, Zobair M; Birerdinc, Aybike; Henry, Linda

    2016-10-01

    Hepatitis C virus infection (HCV) affects approximately 170-200 million individuals globally. HCV is one of the primary causes of hepatocellular carcinoma (HCC) and cirrhosis and has been identified as the leading indication for liver transplantation in most Western countries. Because HCV is a systemic disease with hepatic, extrahepatic, economic and patient reported consequences, it is important for healthcare practitioners to understand the comprehensive and multi-faceted picture of this disease. In this context, it is important to fully appreciate the impact of HCV on the individual patient and the society. With the recent advent of the new generation of direct antiviral agents, the long standing goal of eradicating HCV in most infected patients has been accomplished. Therefore, now more than ever, it is critical to assess the total benefits of sustained virological response in a comprehensive manner. This should not be limited to the clinical benefits of HCV cure, but also to account for the improvement of patient reported health and economic outcomes of HCV cure. It is only through this comprehensive approach to HCV and its treatment that we will understand the full impact of this disease and the tremendous gains that have been achieved with the new antiviral regimens.

  2. Clinical Factors and Disease Course Related to Diagnostic Delay in Korean Crohn's Disease Patients: Results from the CONNECT Study.

    PubMed

    Moon, Chang Mo; Jung, Sung-Ae; Kim, Seong-Eun; Song, Hyun Joo; Jung, Yunho; Ye, Byong Duk; Cheon, Jae Hee; Kim, You Sun; Kim, Young-Ho; Kim, Joo Sung; Han, Dong Soo

    2015-01-01

    Diagnostic delay frequently occurs in Crohn's disease (CD) patients because of diagnostic limitations. However, diagnostic delay and its related factors remain poorly defined. Therefore, we aimed to identify the predictors associated with diagnostic delay and to evaluate the impact of diagnostic delay on clinical course in a Korean CD patient cohort. We performed a multicenter retrospective analysis of 1,047 CD patients registered in the Crohn's Disease Clinical Network and Cohort study in Korea. The mean interval of diagnostic delay was 16.0 ± 33.1 months. Multivariate analysis showed that older age at diagnosis (≥40 years) (p = 0.014), concomitant upper gastrointestinal (UGI) disease (p = 0.012) and penetrating disease behavior at diagnosis (p = 0.001) were positively associated with long diagnostic delay (≥18 months). During the longitudinal follow-up, long diagnostic delay was independently predictive of further development of intestinal stenosis (hazard ratio [HR], 1.43; 95% confidence interval [CI], 1.07-1.93; p = 0.017), internal fistulas (HR, 1.62; 95% CI, 1.12-2.33; p = 0.011), and perianal fistulas (HR, 1.38; 95% CI, 1.06-1.80; p = 0.016). However, as for the risk of abscess formation, bowel perforation, and CD-related abdominal surgery, no significant association with diagnostic delay was observed. Older age at diagnosis, UGI involvement, and penetrating behavior are associated with long diagnostic delay in Korean CD patients. Moreover, diagnostic delay is associated with an increased risk of CD-related complications such as intestinal stenosis, internal fistulas, and perianal fistulas.

  3. Clinical and Molecular Epidemiology of Haemophilus influenzae Causing Invasive Disease in Adult Patients

    PubMed Central

    Puig, Carmen; Grau, Imma; Tubau, Fe; Calatayud, Laura; Pallares, Roman; Liñares, Josefina

    2014-01-01

    Objectives The epidemiology of invasive Haemophilus influenzae (Hi) has changed since the introduction of the Hi type b (Hib) vaccine. The aim of this study was to analyze the clinical and molecular epidemiology of Hi invasive disease in adults. Methods Clinical data of the 82 patients with Hi invasive infections were analyzed. Antimicrobial susceptibility, serotyping, and genotyping were studied (2008–2013). Results Men accounted for 63.4% of patients (whose mean age was 64.3 years). The most frequent comorbidities were immunosuppressive therapy (34.1%), malignancy (31.7%), diabetes, and COPD (both 22%). The 30-day mortality rate was 20.7%. The majority of the strains (84.3%) were nontypeable (NTHi) and serotype f was the most prevalent serotype in the capsulated strains. The highest antimicrobial resistance was for cotrimoxazole (27.1%) and ampicillin (14.3%). Twenty-three isolates (32.9%) had amino acid changes in the PBP3 involved in resistance. Capsulated strains were clonal and belonged to clonal complexes 6 (serotype b), 124 (serotype f), and 18 (serotype e), whereas NTHi were genetically diverse. Conclusions Invasive Hi disease occurred mainly in elderly and those with underlying conditions, and it was associated with a high mortality rate. NTHi were the most common cause of invasive disease and showed high genetic diversity. PMID:25379704

  4. Clinical applications of stereotactic radiation therapy for oligometastatic cancer patients: a disease-oriented approach

    PubMed Central

    Ricardi, Umberto; Badellino, Serena; Filippi, Andrea Riccardo

    2016-01-01

    Oligometastases from solid tumors are currently recognized as a distinct clinical entity, corresponding to an intermediate state between local and widespread disease. It has been suggested that local ablative therapies (including surgery, radiofrequency ablation and radiation therapy) play an important role in this setting, in combination or not with systemic therapies, particularly in delaying disease progression and hopefully in increasing the median survival time. Stereotactic body radiation therapy (SBRT) rapidly emerged in recent years as one of the most effective and less toxic local treatment modalities for lung, liver, adrenal, brain and bone metastases. The aim of this review was to focus on its clinical role for oligometastatic disease in four major cancer subtypes: lung, breast, colorectal and prostate. On the basis of the available evidence, SBRT is able to provide high rates of local tumor control without significant toxicity. Its global impact on survival is uncertain; however, in specific subpopulations of oligometastatic patients there is a trend towards a significant improvement in progression-free and overall survival rates; these important data might be used as a platform for clinical decision-making and establish the basis for the current and future prospective trials investigating its role with or without systemic treatments. PMID:26962198

  5. Clinical experience with infliximab biosimilar Remsima (CT-P13) in inflammatory bowel disease patients.

    PubMed

    Jahnsen, Jørgen

    2016-05-01

    Many reference biological therapies have now reached or are near to patent expiry, and therefore a number of biosimilars have been or will be developed. The term biosimilar can be defined as a biotherapeutic product that is similar in efficacy, safety and quality to the licensed reference product. Biosimilars may lead to a reduced price and significant cost savings for the health community and hopefully more patients globally will have easier access to biological therapy when indicated. CT-P13, which is a TNF-alfa inhibitor, is the first monoclonal antibody biosimilar being used in clinical practice. The drug is approved for all indications as an innovator product although clinical efficacy has only been demonstrated in rheumatic diseases. Until now the number of patients with inflammatory bowel disease (IBD) treated with CT-P13 is confined, but experience is continuously growing. Based on current data, CT-P13 seems to be efficacious and generally well tolerated in IBD especially in patients who are naïve to biological therapy. Knowledge with regard to interchangeability between CT-P13 and the originator infliximab is however, still rather sparse and more data are desired. Immunogenicity and long-term safety related to CT-P13 are other areas of great importance and good and reliable postmarketing pharmacovigilance is therefore required in the coming years. PMID:27134662

  6. Home-based pulmonary rehabilitation in patients with chronic obstructive pulmonary disease: a randomized clinical trial

    PubMed Central

    Dias, Fernanda Dultra; Sampaio, Luciana Maria Malosá; da Silva, Graziela Alves; Gomes, Évelim LF Dantas; do Nascimento, Eloisa Sanches Pereira; Alves, Vera Lucia Santos; Stirbulov, Roberto; Costa, Dirceu

    2013-01-01

    Introduction Pulmonary rehabilitation (PR) is a multidisciplinary program of care for patients with chronic obstructive pulmonary disease (COPD) with the goal of improving the functional capacity and quality of life, as well as maintaining the clinical stability of COPD sufferers. However, not all patients are available for such a program despite discomfort with their condition. The aim of this study was to evaluate the effects of a home-based PR (HBPR) program on functional ability, quality of life, and respiratory muscle strength and endurance. Patients and methods Patients with COPD according to the Global Initiative of Chronic Obstructive Lung Disease were randomized (double-blind) into two groups. One group performed a protocol at home with aerobic and muscle strength exercises and was called the intervention group; the other group received only instructions to perform breathing and stretching exercises, characterizing it as the control group (CG). We assessed the following variables at baseline and 2 months: exercise tolerance (incremental shuttle walk test and upper limb test), respiratory muscle (strength and endurance test), and health-related quality of life (Airways Questionnaire 20). Results There were no significant changes after the intervention in either of the two groups in exercise tolerance and quality of life. However, the intervention group had improved respiratory endurance compared with the CG, while the CG presented a decrease in the load sustained by the respiratory muscles after the HBPR. Conclusion A program of HBPR with biweekly supervision (although not enough to provide significant improvements in physical capacity or quality of life) played an important role in maintaining the stability of the clinical features of patients with COPD; the patients had no worsening of symptoms during the intervention period according to the daily log. PMID:24235824

  7. Full-mouth rehabilitation of a patient with gastroesophageal reflux disease: a clinical report.

    PubMed

    Guo, Juanli; Reside, Glenn; Cooper, Lyndon F

    2011-10-01

    Gastroesophageal reflux disease (GERD) is a chronic condition caused by stomach acid regurgitating into the esophagus or oral cavity, often causing heartburn. Tooth erosion and wear are common oral manifestations of GERD. This clinical report describes the full-mouth rehabilitation of a patient with over 30 years of GERD, causing wear of maxillary and mandibular anterior teeth, along with complications associated with past restorations. Full-mouth rehabilitation of natural teeth in conjunction with dental implants was selected as the treatment option. Ideal occlusal design and optimal esthetics, along with reinforcement of oral hygiene, ensure a favorable prognosis.

  8. Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14

    NASA Astrophysics Data System (ADS)

    Borjali, Davood

    Title: Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14 Kawasaki disease(KD) is a kind of vasculitis diagnosed by clinical manifestation and it caused acquired heart disease in children because of coronary arteries involvement. Method: patient divided to three group of American Japanese and incomplete and also study in two group according to fever days and then clinical features and laboratory data were checked. Result: A total of 150 patients were enrolled during the study period. number of patients with incomplete Kawasaki disease was 128 american group was 28 and Japanese was 4 patients, the most prevalent symptom was scaling of extremities(61 bladder most seen in group with fever more than five days. Keyword: Kawasaki , epidemiology , criteria

  9. The Clinical Utility of SUDOSCAN in Chronic Kidney Disease in Chinese Patients with Type 2 Diabetes

    PubMed Central

    Luk, Andrea O. Y.; Ozaki, Risa; Chung, Harriet H. Y.; Wong, Rebecca Y. M.; So, Wing-Yee; Chow, Francis C. C.

    2015-01-01

    There are gaps between recommendations on regular screening for diabetic kidney disease (DKD) and clinical practice especially in busy and low resource settings. SUDOSCAN (Impeto Medical, Paris, France) is a non-invasive technology for assessing sudomotor function using reverse iontophoresis and chronoamperometry which detects abnormal sweat gland function. Vasculopathy and neuropathy share common risk factors and we hypothesized that SUDOSCAN may be used to detect chronic kidney disease (CKD). Between 2012 and 2013, SUDOSCAN was performed in a consecutive cohort of 2833 Hong Kong Chinese adults with type 2 diabetes. Chronic kidney disease was defined as estimated glomerular filtration rate <60 ml/min/1.73m2. In this cross-sectional cohort (mean age 58.6±9.5 years, 55.7% male, median disease duration 8 [interquartile range 3–14] years), 5.8% had CKD. At a cut-off SUDOSCAN-DKD score of 53, the test had sensitivity of 76.7%, specificity of 63.4% and positive likelihood ratio of 2.1 to detect CKD. The area under receiver operating characteristic curve for CKD was 0.75 (95% confidence interval 0.72–0.79). Patients without CKD but low score had worse risk factors and complications than those with high score. We conclude that SUDOSCAN may be used to detect patients at risk of impaired renal function as part of a screening program in Chinese population, especially in outreach or low resource settings. PMID:26270544

  10. Clinical Factors Associated with Morbidity and Mortality in Patients Admitted with Sickle Cell Disease

    PubMed Central

    Galloway-Blake, K; Reid, M; Walters, C; Jaggon, J; Lee, MG

    2014-01-01

    ABSTRACT Objective: To determine the clinical factors associated with the length of hospitalization and mortality in patients with sickle cell disease (SCD). Methods: All patients with SCD admitted to the medical wards of the University Hospital of the West Indies, Jamaica, over a five-year period, January 1, 2005 to December 31, 2010, were reviewed. Data were extracted from hospital charts and comprised demographic and clinical information, investigations, interventions, duration of stay, pathological data and outcomes. Results: There were 105 patients reviewed; 84% were genotype Hb SS. Females accounted for 59% and males 41%. Overall mean age was 32.5 years (SD 13.7, range 12–66 years). The mean length of hospitalization was 10.2 days (SD 10.9, range 1–84 days). The main admission diagnoses were painful crisis, acute chest syndrome, severe anaemia, sepsis, hepatic sequestration, congestive cardiac failure and renal failure. The mean values for the following laboratory investigations were: haemoglobin 7.7 g/dL (SD 2.8), total white blood cell count 21.7 × 109/L (SD 14.2), platelet count 320 × 109/L (SD 191.9), blood urea 9.8 mmol/L (SD 11.9) and serum creatinine 198 umol/L (SD 267.9). Medical interventions included: blood transfusions in 20.9%, 55% received antibiotics and 74% received narcotic analgesia. There were 40 deaths with four autopsies done. The mortality rate for SCD was 38%. There were 189 repeat SCD admissions. Conclusion: Sickle cell disease still carries a high morbidity and mortality in patients admitted to hospital. Recurrent admissions are a concern, as they impact on patient's morbidity and quality of life. PMID:25867578

  11. Implementation strategies of Systems Medicine in clinical research and home care for cardiovascular disease patients.

    PubMed

    Montecucco, Fabrizio; Carbone, Federico; Dini, Frank Lloyd; Fiuza, Manuela; Pinto, Fausto J; Martelli, Antonietta; Palombo, Domenico; Sambuceti, Gianmario; Mach, François; De Caterina, Raffaele

    2014-11-01

    Insights from the "-omics" science have recently emphasized the need to implement an overall strategy in medical research. Here, the development of Systems Medicine has been indicated as a potential tool for clinical translation of basic research discoveries. Systems Medicine also gives the opportunity of improving different steps in medical practice, from diagnosis to healthcare management, including clinical research. The development of Systems Medicine is still hampered however by several challenges, the main one being the development of computational tools adequate to record, analyze and share a large amount of disparate data. In addition, available informatics tools appear not yet fully suitable for the challenge because they are not standardized, not universally available, or with ethical/legal concerns. Cardiovascular diseases (CVD) are a very promising area for translating Systems Medicine into clinical practice. By developing clinically applied technologies, the collection and analysis of data may improve CV risk stratification and prediction. Standardized models for data recording and analysis can also greatly broaden data exchange, thus promoting a uniform management of CVD patients also useful for clinical research. This advance however requires a great organizational effort by both physicians and health institutions, as well as the overcoming of ethical problems. This narrative review aims at providing an update on the state-of-art knowledge in the area of Systems Medicine as applied to CVD, focusing on current critical issues, providing a road map for its practical implementation.

  12. Clinical significance of screening for subclinical Cushing's disease in patients with pituitary tumors.

    PubMed

    Tamada, Daisuke; Kitamura, Tetsuhiro; Otsuki, Michio; Oshino, Satoru; Saitoh, Youichi; Shimomura, Iichiro

    2016-01-01

    Cushing's syndrome (CS) is a clinical state caused by chronic excess of glucocorticoid, and results in hypertension, impaired glucose tolerance, and dyslipidemia. Recently, a mild state of pituitary CS without typical Cushingoid appearance (subclinical Cushing's disease; SCD) has been identified. However, the true prevalence of SCD and its effect on metabolic disorders remain obscure. The aim of this prospective study was to determine the prevalence of SCD according to the guideline proposed by the working group of the Japanese Ministry of Health, Welfare and Labor, and to assess the outcome of surgery on metabolic disorders. The prevalence of SCD was investigated in 105 consecutive patients diagnosed with pituitary adenomas by MRI. ACTH-dependent hypercortisolism was diagnosed based on the results of the 0.5 mg dexamethasone suppression test (serum cortisol >3.0 μg/dL) plus one positive finding of the following two tests: midnight serum cortisol level >5.0 μg/dL or ACTH increase >50% after 1-deamino-5-D-arginine vasopressin (DDAVP) challenge. The final diagnosis of SCD was established by positive staining for ACTH in surgically-excised pituitary adenoma. Three patients (4.8%) were diagnosed with SCD among 62 patients with pituitary adenoma. Transsphenoidal adenomectomy partially resulted in improvement of blood pressure and glucose metabolism in SCD patients. Our results emphasize the importance of SCD screening in patients with pituitary tumors, especially in those patients with metabolic disorders.

  13. Biochemical and clinical effects of aspartame in patients with chronic, stable alcoholic liver disease.

    PubMed

    Hertelendy, Z I; Mendenhall, C L; Rouster, S D; Marshall, L; Weesner, R

    1993-05-01

    Aspartame is an artificial sweetener completely metabolized in the gut and absorbed as aspartate, phenylalanine, and methanol. Phenylalanine is thought to mediate or exacerbate hepatic encephalopathy, and an impaired liver may not be able to cope with the ammoniagenic properties of the amino acid constituents, or adequately metabolize methanol. Thus, we compared the clinical and biochemical effects of a single ingestion of aspartame (15 mg/kg) to skim milk (phenylalanine content equimolar to aspartame) and placebo in patients with chronic, alcoholic liver disease in a randomized, crossover study. Aspartame produced an elevation of plasma phenylalanine significantly greater than milk and placebo (Cmax 14.55 +/- 7.38, 10.95 +/- 4.95, 8.84 +/- 4.55 mumol/dl, respectively; p < 0.01). However, quantified encephalopathic changes were observed only with milk (p < 0.05). Plasma aspartate, methanol, formate, and ammonia levels remained unchanged after all treatments. The lack of clinical derangements in encephalopathic indices, methanol accumulation, or biochemical changes in liver status suggests that a single large dose of aspartame (representing 5 times the average daily intake of adults) may be used safely by patients with chronic, stable liver disease.

  14. Development of clinical practice guidelines for patients with comorbidity and multiple diseases.

    PubMed

    Bernabeu-Wittel, M; Alonso-Coello, P; Rico-Blázquez, M; Rotaeche Del Campo, R; Sánchez Gómez, S; Casariego Vales, E

    2014-01-01

    The management of patients with comorbidity and polypathology represents a challenge for all healthcare systems. Clinical practice guidelines (CPGs) have limitations when applied to this population. The aim of this study is to propose the terminology and methodology for optimally approach comorbidity and polypathology in the CPGs. Based on a literature review, we suggest a number of proposals for the approach in different phases of CPG preparation, with special attention to the inclusion of clusters of comorbidity in the initial questions the implementation of indirect evidence, the burden of disease management for patients and their environment, when establishing recommendations, as well as the strategies of dissemination and implementation. These proposals should be developed in greater depth with the implication of more agents in order to have valid and useful tools for this population.

  15. [Development of clinical practice guidelines for patients with comorbidity and multiple diseases].

    PubMed

    Bernabeu-Wittel, M; Alonso-Coello, P; Rico-Blázquez, M; Rotaeche del Campo, R; Sánchez Gómez, S; Casariego Vales, E

    2014-01-01

    The management of patients with comorbidity and polypathology represents a challenge for all healthcare systems. Clinical practice guidelines (CPGs) have limitations when applied to this population. The aim of this study is to propose the terminology and methodology for optimally approach comorbidity and polypathology in the CPGs. Based on a literature review, we suggest a number of proposals for the approach in different phases of CPG preparation, with special attention to the inclusion of clusters of comorbidity in the initial questions the implementation of indirect evidence, the burden of disease management for patients and their environment, when establishing recommendations, as well as the strategies of dissemination and implementation. These proposals should be developed in greater depth with the implication of more agents in order to have valid and useful tools for this population.

  16. Guidelines for the diagnosis, treatment and clinical monitoring of patients with juvenile and adult Pompe disease.

    PubMed

    Llerena Junior, Juan Clinton; Nascimento, Osvaldo Jm; Oliveira, Acary Souza B; Dourado Junior, Mario Emilio T; Marrone, Carlo D; Siqueira, Heloise Helena; Sobreira, Cláudia F R; Dias-Tosta, Elza; Werneck, Lineu Cesar

    2016-02-01

    Pompe disease (PD) is a potentially lethal illness involving irreversible muscle damage resulting from glycogen storage in muscle fiber and activation of autophagic pathways. A promising therapeutic perspective for PD is enzyme replacement therapy (ERT) with the human recombinant enzyme acid alpha-glucosidase (Myozyme®). The need to organize a diagnostic flowchart, systematize clinical follow-up, and establish new therapeutic recommendations has become vital, as ERT ensures greater patient longevity. A task force of experienced clinicians outlined a protocol for diagnosis, monitoring, treatment, genetic counseling, and rehabilitation for PD patients. The study was conducted under the coordination of REBREPOM, the Brazilian Network for Studies of PD. The meeting of these experts took place in October 2013, at L'Hotel Port Bay in São Paulo, Brazil. In August 2014, the text was reassessed and updated. Given the rarity of PD and limited high-impact publications, experts submitted their views. PMID:26690841

  17. Gorham's disease: clinical case.

    PubMed

    Sá, Pedro; Marques, Pedro; Oliveira, Carolina; Rodrigues, André Sá; Amorim, Nelson; Pinto, Rui

    2015-01-01

    Gorham's disease, also known as idiopathic massive osteolysis, is a rare pathological condition characterized by vascular proliferation that results in destruction and reabsorption of the bone matrix, of unknown etiology. It was first described by Jackson in 1838, but it was Gorham and Stout, in 1955, who defined this disease as a specific entity. It has variable clinical presentation and generally has progressive behavior. Controversy continues regarding the treatment and there is no standard treatment. This pathological condition generally presents a favorable prognosis. Here, a case of Gorham's disease with involvement of the left hip is presented, in a male patient without relevant antecedents.

  18. Gorham's disease: clinical case☆

    PubMed Central

    Sá, Pedro; Marques, Pedro; Oliveira, Carolina; Rodrigues, André Sá; Amorim, Nelson; Pinto, Rui

    2015-01-01

    Gorham's disease, also known as idiopathic massive osteolysis, is a rare pathological condition characterized by vascular proliferation that results in destruction and reabsorption of the bone matrix, of unknown etiology. It was first described by Jackson in 1838, but it was Gorham and Stout, in 1955, who defined this disease as a specific entity. It has variable clinical presentation and generally has progressive behavior. Controversy continues regarding the treatment and there is no standard treatment. This pathological condition generally presents a favorable prognosis. Here, a case of Gorham's disease with involvement of the left hip is presented, in a male patient without relevant antecedents. PMID:26229923

  19. Association Between Gastroesophageal Reflux Disease After Pneumatic Balloon Dilatation and Clinical Course in Patients With Achalasia

    PubMed Central

    Min, Yang Won; Lee, Jin Hee; Min, Byung-Hoon; Lee, Jun Haeng; Kim, Jae J; Rhee, Poong-Lyul

    2014-01-01

    Background/Aims The occurrence of gastroesophageal reflux disease (GERD) is known to be associated with lower post-treatment lower esophageal sphincter pressure in patients with achalasia. This study aimed to elucidate whether GERD after pneumatic balloon dilatation (PD) has a prognostic role and to investigate how the clinical course of GERD is. Methods A total of 79 consecutive patients who were first diagnosed with primary achalasia and underwent PD as an initial treatment were included in this retrospective study. Single PD was performed using a 3.0 cm balloon. The patients were divided into two groups: 1) who developed GERD after PD (GERD group) and 2) who did not develop GERD after PD (non-GERD group). GERD was defined as pathological acid exposure, reflux esophagitis or typical reflux symptoms. Results Twenty one patients (26.6%) developed GERD after PD during follow-up. There were no significant differences between the two groups in demographic or clinical factors including pre- and post-treatment manometric results. All patients in GERD group were well responsive to maintenance proton pump inhibitor therapy including on demand therapy or did not require maintenance. During a median follow-up of 17.8 months (interquartile range, 7.1–42.7 months), achalasia recurred in 15 patients (19.0%). However, the incidence of recurrence did not differ according to the occurrence of GERD after PD. Conclusions GERD often occurs after even a single PD for achalasia. However, GERD after PD is well responsive to PPI therapy. Our data suggest that GERD after PD during follow-up does not appear to have a prognostic role. PMID:24840373

  20. A clinical study of integrating acupuncture and Western medicine in treating patients with Parkinson's disease.

    PubMed

    Chen, Fang-Pey; Chang, Ching-Mao; Shiu, Jing-Huei; Chiu, Jen-Hwey; Wu, Ta-Peng; Yang, Jen-Lin; Kung, Yen-Ying; Chen, Fun-Jou; Chern, Chang-Ming; Hwang, Shinn-Jang

    2015-01-01

    Complementary therapy with acupuncture for Parkinson's disease (PD) has been studied for quite a long time, but the effectiveness of the treatment still remains unclear. The aim of this study is to evaluate the integrated effects of acupuncture treatment in PD patients who received western medicine. In the short-term acupuncture treatment study, 20 patients received acupuncture therapy twice a week in acupoints DU 20, GB 20, LI 11, LI 10, LI 4, GB 31, ST 32, GB 34 and GB 38 along with western medicine for 18 weeks, and 20 controlled patients received western medicine only. In the long-term acupuncture treatment, 13 patients received acupuncture treatment twice a week for 36 weeks. The outcome parameters include Unified Parkinson's disease rating scale (UPDRS), Beck Anxiety Inventory (BAI), Beck Depression Inventory-Version 2 (BDI-II), and WHO quality of life (WHOQOL). In the short-term clinical trial, a higher percentage of patients in the acupuncture group had score improvement in UPDRS total scores (55% vs. 15%, p = 0.019), sub-score of mind, behavior and mood (85% vs. 25%, p < 0.001), activity of daily living (65% vs. 15%, p = 0.003), mobility (40% vs. 15%, p = 0.155) and complication of treatment (75% vs. 15%, p < 0.001), BDI-II score (85% vs. 35%, p = 0.003), and WHOQOL score (65% vs. 15%, p = 0.003) when compared to control group at the end of the 18 weeks' follow up. After 36 weeks of long-term acupuncture treatment, the mean UPDRS total scores and sub-score of mentation, behavior and mood, sub-score of complications of therapy and BDI-II score decreased significantly when compared to the pretreatment baseline. In conclusion, acupuncture treatment had integrated effects in reducing symptoms and signs of mind, behavior, mood, complications of therapy and depression in PD patients who received Western medicine.

  1. Hepatitis B infection among patients attending a sexually transmitted diseases clinic in Rio de Janeiro, Brazil.

    PubMed

    Oliveira, L H; Silva, I R; Xavier, B L; Cavalcanti, S M

    2001-07-01

    Hepatitis B virus (HBV) has a low endemicity in Rio de Janeiro, Brazil. Sexual transmission must play an important role in this virus, but the prevalence and risk factors have never been properly investigated. The aim of this paper is to determine the prevalence and risk factors for HBV infection in patients attending a Sexually Transmitted Diseases Clinic of the Universidade Federal Fluminense, from the State of Rio de Janeiro, Brazil. In a retrospective study, HBV seroprevalence was investigated in 440 patients. Serum of each patient was assayed for antibodies against hepatitis B core antigen (anti-HBc), hepatitis B surface antigen (HBsAg) and antibodies against hepatitis B surface antigen (anti-HBs). Demographic and risk factor data were extracted from clinic notes. The overall seroprevalence of exposure markers for HBV (anti-HBc, HBsAg and anti-HBs) were 13%, 3.4% and 8.5% respectively. Homo/bisexual behaviour, anal intercourse, HIV infection, positive serology for syphilis and blood transfusion were predictors of the HBV exposure. Among demographic data, age and place of birth were associated with the anti-HBc seropositivity.

  2. Cataract surgery in patients with ocular surface disease: An update in clinical diagnosis and treatment.

    PubMed

    Afsharkhamseh, Neda; Movahedan, Asadolah; Motahari, Hooman; Djalilian, Ali R

    2014-07-01

    In this article we review essentials of diagnosis and management of ocular surface disease in patients who undergo cataract surgery. It is clearly shown that dry eye disease worsens following the cataract surgery in patients with prior history of ocular surface disease, Also new cases of dry eye might appear. Current strategies for the timely diagnosis and proper management of dry eye syndrome in the face of cataract surgery patients are mainly emphasized. To achieve the best outcome in cataract surgery, a healthy ocular surface is crucial. While ocular surface preparation is indispensable in patients with established ocular surface disease, it is also helpful in those with minimal signs or symptoms of surface disease. The current approach begins with early diagnosis and drastic management of ocular surface disease before cataract surgery using a stepwise regimen customized to each patient and disease severity. These measures are continued throughout and after the surgery. PMID:25278791

  3. Subthalamic nucleus stimulation in Parkinson's disease: clinical evaluation of 18 patients.

    PubMed

    Thobois, S; Mertens, P; Guenot, M; Hermier, M; Mollion, H; Bouvard, M; Chazot, G; Broussolle, E; Sindou, M

    2002-05-01

    The aim of the present study was to assess the efficacy and safety of chronic subthalamic nucleus deep-brain stimulation (STN-DBS) in patients with Parkinson's disease (PD). 18 consecutive severely affected PD patients were included (mean age, SD: 56.9+/-6 years; mean disease duration: 13.5+/-4.4 years). All the patients were evaluated clinically before and 6 months after the surgical procedure using the Unified Parkinson's Disease Rating Scale (UPDRS). Additionally, a 12 months follow-up was available in 14 patients. The target coordinates were determined by ventriculography under stereotactic conditions, followed by electrophysiology and intraoperative stimulation. After surgery, continuous monopolar stimulation was applied bilaterally in 17 patients at 2.9+/-0.4 V through 1 (n = 31) or 2 contacts (n = 3). One patient had bilateral bipolar stimulation. The mean frequency of stimulation was 140+/-16 Hz and pulse width 68+/-13 micros. Off medication, the UPDRS part III score (max = 108) was reduced by 55 % during on stimulation (score before surgery: 44.9+/-13.4 vs at 6 months: 20.2+/-10; p < 0.001). In the on medication state, no difference was noted between the preoperative and the postoperative off stimulation conditions (scores were respectively: 17.9+/-9.2 and 23+/-12.6). The severity of motor fluctuations and dyskinesias assessed by UPDRS IV was reduced by 76 % at 6 months (scores were respectively: 10.3+/-3 and 2.5+/-3; p < 0.001). Off medication, the UPDRS II or ADL score was reduced by 52.8 % during on stimulation (26.9+/-6.5 preop versus 12.7+/-7 at 6 months). The daily dose of antiparkinsonian treatment was diminished by 65.5 % (levodopa equivalent dose -- mg/D -- was 1045 +/- 435 before surgery and 360 +/- 377 at 6 months; p < 0.01). These results remained stable at 12 months for the 14 patients studied. Side effects comprised lower limb phlebitis (n = 2), pulmonary embolism (n = 1), depression (n = 6), dysarthria and freezing (n = 1), sialorrhea and

  4. Subthalamic nucleus stimulation in Parkinson's disease: clinical evaluation of 18 patients.

    PubMed

    Thobois, S; Mertens, P; Guenot, M; Hermier, M; Mollion, H; Bouvard, M; Chazot, G; Broussolle, E; Sindou, M

    2002-05-01

    The aim of the present study was to assess the efficacy and safety of chronic subthalamic nucleus deep-brain stimulation (STN-DBS) in patients with Parkinson's disease (PD). 18 consecutive severely affected PD patients were included (mean age, SD: 56.9+/-6 years; mean disease duration: 13.5+/-4.4 years). All the patients were evaluated clinically before and 6 months after the surgical procedure using the Unified Parkinson's Disease Rating Scale (UPDRS). Additionally, a 12 months follow-up was available in 14 patients. The target coordinates were determined by ventriculography under stereotactic conditions, followed by electrophysiology and intraoperative stimulation. After surgery, continuous monopolar stimulation was applied bilaterally in 17 patients at 2.9+/-0.4 V through 1 (n = 31) or 2 contacts (n = 3). One patient had bilateral bipolar stimulation. The mean frequency of stimulation was 140+/-16 Hz and pulse width 68+/-13 micros. Off medication, the UPDRS part III score (max = 108) was reduced by 55 % during on stimulation (score before surgery: 44.9+/-13.4 vs at 6 months: 20.2+/-10; p < 0.001). In the on medication state, no difference was noted between the preoperative and the postoperative off stimulation conditions (scores were respectively: 17.9+/-9.2 and 23+/-12.6). The severity of motor fluctuations and dyskinesias assessed by UPDRS IV was reduced by 76 % at 6 months (scores were respectively: 10.3+/-3 and 2.5+/-3; p < 0.001). Off medication, the UPDRS II or ADL score was reduced by 52.8 % during on stimulation (26.9+/-6.5 preop versus 12.7+/-7 at 6 months). The daily dose of antiparkinsonian treatment was diminished by 65.5 % (levodopa equivalent dose -- mg/D -- was 1045 +/- 435 before surgery and 360 +/- 377 at 6 months; p < 0.01). These results remained stable at 12 months for the 14 patients studied. Side effects comprised lower limb phlebitis (n = 2), pulmonary embolism (n = 1), depression (n = 6), dysarthria and freezing (n = 1), sialorrhea and

  5. [Adequacy of clinical interventions in patients with advanced and complex disease. Proposal of a decision making algorithm].

    PubMed

    Ameneiros-Lago, E; Carballada-Rico, C; Garrido-Sanjuán, J A; García Martínez, A

    2015-01-01

    Decision making in the patient with chronic advanced disease is especially complex. Health professionals are obliged to prevent avoidable suffering and not to add any more damage to that of the disease itself. The adequacy of the clinical interventions consists of only offering those diagnostic and therapeutic procedures appropriate to the clinical situation of the patient and to perform only those allowed by the patient or representative. In this article, the use of an algorithm is proposed that should serve to help health professionals in this decision making process.

  6. Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes.

    PubMed

    Fang, Lingling; Huang, Jia; Zhang, Qian; Chan, Raymond C K; Wang, Rong; Wan, Weiqing

    2016-08-01

    OBJECTIVE Dysexecutive syndrome is common in patients with moyamoya disease (MMD), a chronic cerebrovascular disease that is characterized by stenosis of the bilateral internal carotid arteries and progressive collateral revascularization, and MMD can be classified as ischemic or hemorrhagic according to the disease presentation and history. In this study, the authors aimed to determine which aspects of executive function are impaired in patients with MMD, in addition to the specific dysexecutive functions present among its clinical subtypes and the mechanisms underlying dysexecutive function in these patients. METHODS The authors administered 5 typical executive function tests (the Stroop test, the Hayling Sentence Completion Test [HSCT], the verbal fluency [VF] test, the N-back test, and the Sustained Attention to Response Task [SART]) to 49 patients with MMD and 47 IQ-, age-, education-, and social status-matched healthy controls. The dysexecutive questionnaire (DEX) was also used to assess participants' subjective feelings about their executive function. A total of 39 of the patients were evaluated by CT perfusion (CTP) before the assessments were performed, and the correlations among the performances of the patients on the above tests with the parameters of cerebral blood volume, cerebral blood flow (CBF), mean transit time (MTT), and time-to-peak (TTP) in the frontal lobes of these patients were also analyzed. RESULTS Many aspects of executive function in the patients with MMD were significantly poorer than those in the healthy controls, and the patients performed particularly poorer on the VF test, HSCT, N-back test, and SART. The patients with hemorrhagic MMD exhibited worse executive inhibition, executive processing, and semantic inhibition compared with those with ischemic MMD, but the latter group presented a worse working memory and poorer sustained attention. There were no significant differences in the DEX scores between the patients with MMD and

  7. Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes.

    PubMed

    Fang, Lingling; Huang, Jia; Zhang, Qian; Chan, Raymond C K; Wang, Rong; Wan, Weiqing

    2016-08-01

    OBJECTIVE Dysexecutive syndrome is common in patients with moyamoya disease (MMD), a chronic cerebrovascular disease that is characterized by stenosis of the bilateral internal carotid arteries and progressive collateral revascularization, and MMD can be classified as ischemic or hemorrhagic according to the disease presentation and history. In this study, the authors aimed to determine which aspects of executive function are impaired in patients with MMD, in addition to the specific dysexecutive functions present among its clinical subtypes and the mechanisms underlying dysexecutive function in these patients. METHODS The authors administered 5 typical executive function tests (the Stroop test, the Hayling Sentence Completion Test [HSCT], the verbal fluency [VF] test, the N-back test, and the Sustained Attention to Response Task [SART]) to 49 patients with MMD and 47 IQ-, age-, education-, and social status-matched healthy controls. The dysexecutive questionnaire (DEX) was also used to assess participants' subjective feelings about their executive function. A total of 39 of the patients were evaluated by CT perfusion (CTP) before the assessments were performed, and the correlations among the performances of the patients on the above tests with the parameters of cerebral blood volume, cerebral blood flow (CBF), mean transit time (MTT), and time-to-peak (TTP) in the frontal lobes of these patients were also analyzed. RESULTS Many aspects of executive function in the patients with MMD were significantly poorer than those in the healthy controls, and the patients performed particularly poorer on the VF test, HSCT, N-back test, and SART. The patients with hemorrhagic MMD exhibited worse executive inhibition, executive processing, and semantic inhibition compared with those with ischemic MMD, but the latter group presented a worse working memory and poorer sustained attention. There were no significant differences in the DEX scores between the patients with MMD and

  8. Clinically significant responses achieved with romidepsin across disease compartments in patients with cutaneous T-cell lymphoma

    PubMed Central

    Kim, Ellen J.; Kim, Youn H.; Rook, Alain H.; Lerner, Adam; Duvic, Madeleine; Reddy, Sunil; Robak, Tadeusz; Becker, Jürgen C.; Samtsov, Alexey; McCulloch, William; Waksman, Joel; Whittaker, Sean

    2015-01-01

    Cutaneous T-cell lymphoma (CTCL) is a rare heterogeneous group of non-Hodgkin lymphomas that arises in the skin but can progress to systemic disease (lymph nodes, blood, viscera). Historically, in clinical trials of CTCL there has been little consistency in how responses were defined in each disease “compartment”; some studies only assessed responses in the skin. The histone deacetylase inhibitor romidepsin is approved by the US Food and Drug Administration for the treatment of CTCL in patients who have received at least one prior systemic therapy. Phase II studies that led to approval used rigorous composite end points that incorporated disease assessments in all compartments. The objective of this analysis was to thoroughly examine the activity of romidepsin within each disease compartment in patients with CTCL. Romidepsin was shown to have clinical activity across disease compartments and is suitable for use in patients with CTCL having skin involvement only, erythroderma, lymphadenopathy and/or blood involvement. PMID:25791237

  9. Clinically significant responses achieved with romidepsin across disease compartments in patients with cutaneous T-cell lymphoma.

    PubMed

    Kim, Ellen J; Kim, Youn H; Rook, Alain H; Lerner, Adam; Duvic, Madeleine; Reddy, Sunil; Robak, Tadeusz; Becker, Jürgen C; Samtsov, Alexey; McCulloch, William; Waksman, Joel; Whittaker, Sean

    2015-01-01

    Cutaneous T-cell lymphoma (CTCL) is a rare heterogeneous group of non-Hodgkin lymphomas that arises in the skin but can progress to systemic disease (lymph nodes, blood, viscera). Historically, in clinical trials of CTCL there has been little consistency in how responses were defined in each disease "compartment"; some studies only assessed responses in the skin. The histone deacetylase inhibitor romidepsin is approved by the US Food and Drug Administration for the treatment of CTCL in patients who have received at least one prior systemic therapy. Phase II studies that led to approval used rigorous composite end points that incorporated disease assessments in all compartments. The objective of this analysis was to thoroughly examine the activity of romidepsin within each disease compartment in patients with CTCL. Romidepsin was shown to have clinical activity across disease compartments and is suitable for use in patients with CTCL having skin involvement only, erythroderma, lymphadenopathy and/or blood involvement.

  10. [Wilson's disease: clinical spectrum of liver disease].

    PubMed

    Ochoa Palominos, Alejandra; Ibáñez Samaniego, Luis; Catalina Rodríguez, María-Vega; Pajares Díaz, José; Clemente Ricote, Gerardo

    2013-02-01

    Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism,characterized by copper accumulation in the liver and brain. This rare entity, which has a broad clinical spectrum, is often difficult to diagnose and should therefore always be suspected in patients with liver disease of unclear cause. We describe two types of manifestation of liver disease in two patients; the first developed fulminant hepatic failure requiring urgent liver transplantation and the second showed advanced chronic liver disease and received standard medical treatment. The objective of this clinical observation is to analyze the diagnosis of Wilson's disease in two patients with distinct onset, illustrating the broad clinical spectrum of the disease, and its treatment.

  11. Personality traits in patients with Parkinson's disease: assessment and clinical implications.

    PubMed

    Poletti, Michele; Bonuccelli, Ubaldo

    2012-06-01

    This study reviews empirical evidence on the association between personality traits and Parkinson's disease (PD), with a twofold aim. First, to better identify non-motor symptoms, such as affective symptoms and personality changes, that could help to define the pre-motor phase of PD; second, to better understand the neurobiological bases of personality traits, a goal that is not fully accomplished by a purely anatomical approach. A literature review was performed on studies of personality traits in PD patients, in electronic databases ISI Web of Knowledge, Medline and PsychInfo, conducted in July 2011. We found evidence that the existence of a characteristic premorbid personality profile of PD patients is not actually sustained by robust empirical evidence, mainly due to the methodological bias of the retrospective assessment of personality; PD patients present a personality profile of low novelty seeking and high harm avoidance. We concluded that the definition of a pre-motor phase of PD, based on non-motor symptoms, should search for the presence of concomitant affective disorders and for a positive psychiatric history for affective disorders rather than for a typical personality profile or personality changes. The low novelty seeking profile is probably related to the dopaminergic deficit, while the high harm avoidance profile is probably associated with the presence of affective disorders. Clinical implications of these findings, in regard to personality assessment and pharmacological treatments in PD, are also discussed.

  12. Clinical features of pulmonary disease caused by rapidly growing mycobacteria. An analysis of 154 patients.

    PubMed

    Griffith, D E; Girard, W M; Wallace, R J

    1993-05-01

    The role of rapidly growing mycobacteria (RGM) as pulmonary pathogens has been unclear. We identified 154 cases of lung disease caused by RGM using the microbiologic and radiographic criteria of the American Thoracic Society (ATS) and availability of the causative organism for study. More than one third of patients had positive lung biopsy cultures. Patients were predominantly white (83%), female (65%) nonsmokers (66%), and they had prolonged periods from onset of symptoms to diagnosis of their disease. Cough was an almost universal presenting symptom, whereas constitutional symptoms became more important with progression of disease. Upper lobe infiltrates were most common (88%), with 77% of patients developing bilateral disease. Cavitation was present in only 16% of the patients. Specific underlying diseases were infrequent, but they included previously treated mycobacterial disease (18%), coexistent Mycobacterium avium complex (8%), cystic fibrosis (6%), and gastroesophageal disorders with chronic vomiting (6%). The majority of isolates (82%) were M. abscessus (formerly M. chelonae subsp. abscessus). Effective treatment for M. fortuitum long disease was accomplished with drug therapy, whereas surgical resection of localized disease was the only effective long-term therapy for M. abscessus. Although the disease was generally slowly progressive, 21 of 154 (14%) patients died as a consequence of progressive RGM lung disease and respiratory failure. RGM should be recognized as a cause of chronic mycobacterial lung disease, and respiratory isolates should be assessed carefully. PMID:8484642

  13. Dyslipidemia and cardiovascular disease risk profiles of patients attending an HIV treatment clinic in Harare, Zimbabwe.

    PubMed

    Zhou, Danai Tavonga; Kodogo, Vitaris; Chokuona, Kudzai Fortunate Vongai; Gomo, Exnevia; Oektedalen, Olav; Stray-Pedersen, Babill

    2015-01-01

    The chronic inflammation induced by human immunodeficiency virus (HIV) contributes to increased risk of coronary heart disease (CHD) in HIV-infected individuals. HIV-infected patients generally benefit from being treated with antiretroviral drugs, but some antiretroviral agents have side effects, such as dyslipidemia and hyperglycemia. There is general consensus that antiretroviral drugs induce a long-term risk of CHD, although the levels of that risk are somewhat controversial. The intention of this cross-sectional study was to describe the lipid profile and the long-term risk of CHD among HIV-positive outpatients at an HIV treatment clinic in Harare, Zimbabwe. Two hundred and fifteen patients were investigated (females n=165, mean age 39.8 years; males n=50; mean age 42.0 years). Thirty of the individuals were antiretroviral-naïve and 185 had been on antiretroviral therapy (ART) for a mean 3.9±3.4 years. All participants had average lipid and glucose values within normal ranges, but there was a small difference between the ART and ART-for total cholesterol (TC) and high-density lipoprotein (HDL). Those on a combination of D4T or ZDV/NVP/3TC and PI-based ART were on average oldest and had the highest TC levels. Framingham risk showed 1.4% prevalence of high CHD risk within the next ten years. After univariate analysis age, sex, TC/HDL ratio, HDL, economic earnings and systolic BP were associated with medium to high risk of CHD. After multivariate regression analysis and adjusting for age or sex only age, sex and economic earnings were associated with medium to high risk of CHD. There is small risk of developing CHD, during the next decade in HIV infected patients at an HIV treatment clinic in Harare.

  14. Effectiveness of an intervention promoting the female condom to patients at sexually transmitted disease clinics.

    PubMed Central

    Artz, L; Macaluso, M; Brill, I; Kelaghan, J; Austin, H; Fleenor, M; Robey, L; Hook, E W

    2000-01-01

    OBJECTIVES: This study evaluated a behavioral intervention designed to promote female condoms and reduce unprotected sex among women at high risk for acquiring sexually transmitted diseases (STDs). METHODS: The effect of the intervention on barrier use was evaluated with a pretest-posttest design with 1159 female STD clinic patients. RESULTS: Among participants with follow-up data, 79% used the female condom at least once and often multiple times. More than one third of those who completed the study used female condoms throughout follow-up. Use of barrier protection increased significantly after the intervention, and high use was maintained during a 6-month follow-up. To account for attrition, the use of protection by all subjects was projected under 3 conservative assumptions. The initial visit and termination visit projections suggest that use increased sharply after the intervention and declined during follow-up but remained elevated compared with the baseline. CONCLUSIONS: Many clients of public STD clinics will try, and some will continue, to use female condoms when they are promoted positively and when women are trained to use them correctly and to promote them to their partners. A behavioral intervention that promotes both female and male condoms can increase barrier use. PMID:10667185

  15. Activating Patients for Sustained Chronic Disease Self-Management: Thinking Beyond Clinical Outcomes.

    PubMed

    Dye, Cheryl J; Williams, Joel E; Evatt, Janet H

    2016-04-01

    This article describes the impact of an 8-week community program implemented by trained volunteers on the hypertension self-management of 185 patients who were batch randomized to intervention or wait-list control groups. Compared with control group participants, a higher proportion of treatment group participants moved from the cognitive to behavioral stages of motivational readiness for being physically active (P < .001), practicing healthy eating habits (P = .001), handling stress well (P = .001), and living an overall healthy lifestyle (P = .003). They also demonstrated a greater average increase in perceived competence for self-management, F(1.134) = 4.957, P = .028, η2 = .036, and a greater increase in mean hypertension-related knowledge, F(1.160) = 16.571, P < .0005, η(2) = .094. Enduring lifestyle changes necessary for chronic disease self-management require that psychosocial determinants of health behavior are instilled, which is typically beyond standard medical practice. We recommend peer-led, community-based programs as a complement to clinical care and support the increasing health system interest in promoting population health beyond clinical walls.

  16. Estimating the Ratio of Patients with a Certain Disease Between Hospitals for the Allocation of Patients to Clinical Trials Using Health Insurance Claims Data in Japan.

    PubMed

    Takeda, Toshihiro; Mihara, Naoki; Murata, Taizo; Shimai, Yoshie; Okada, Katsuki; Manabe, Shiro; Matsumura, Yasushi

    2016-01-01

    In clinical trials, investigating the ratio of patients with each disease who are treated in a hospital is important for determining the number of patients who are allocated to hospitals. The Japanese health insurance claims data includes standardized disease and medicine data. However, the disease data has some problems in terms of reliability, because the healed diseases are sometimes not deleted or because a disease that a patient does not actually have is registered to claim the cost of the examination. On the other hand, therapeutic medicines are administered to target particular diseases. In this study, we developed a system for estimating the number of patients with each disease using the disease data and the therapeutic medicine data. We converted the ICD-10 code to a 4-grade classification code so that we could predict the diseases in the shallow layer (e.g. gastrointestinal disease) when it was difficult to predict the precise diseases in the deep layer (e.g. gastric ulcers). A table showing the disease code and the corresponding therapeutic medicine code was provided by the Japan Pharmaceutical Information Center (JAPIC). We calculated the disease probability score from the diseases and therapeutic medicines and recorded the predicted disease. For the system evaluation, we used the health insurance claims data from Osaka University Hospital for January 2015. A total of 58,526 diseases were predicted from the health insurance claims data of 18,393 patients. One hundred twenty patients were randomly extracted for use in a chart review that was performed by an expert physician. Two hundred twenty-four of 329 predicted diseases, were correctly predicted; 56 were reasonably predicted, and 49 were incorrectly predicted. The main disease was correctly predicted in 71 patients. In conclusion, we could estimate the number of patients with each disease using the health insurance claims data with a certain degree of accuracy. PMID:27577441

  17. Estimating the Ratio of Patients with a Certain Disease Between Hospitals for the Allocation of Patients to Clinical Trials Using Health Insurance Claims Data in Japan.

    PubMed

    Takeda, Toshihiro; Mihara, Naoki; Murata, Taizo; Shimai, Yoshie; Okada, Katsuki; Manabe, Shiro; Matsumura, Yasushi

    2016-01-01

    In clinical trials, investigating the ratio of patients with each disease who are treated in a hospital is important for determining the number of patients who are allocated to hospitals. The Japanese health insurance claims data includes standardized disease and medicine data. However, the disease data has some problems in terms of reliability, because the healed diseases are sometimes not deleted or because a disease that a patient does not actually have is registered to claim the cost of the examination. On the other hand, therapeutic medicines are administered to target particular diseases. In this study, we developed a system for estimating the number of patients with each disease using the disease data and the therapeutic medicine data. We converted the ICD-10 code to a 4-grade classification code so that we could predict the diseases in the shallow layer (e.g. gastrointestinal disease) when it was difficult to predict the precise diseases in the deep layer (e.g. gastric ulcers). A table showing the disease code and the corresponding therapeutic medicine code was provided by the Japan Pharmaceutical Information Center (JAPIC). We calculated the disease probability score from the diseases and therapeutic medicines and recorded the predicted disease. For the system evaluation, we used the health insurance claims data from Osaka University Hospital for January 2015. A total of 58,526 diseases were predicted from the health insurance claims data of 18,393 patients. One hundred twenty patients were randomly extracted for use in a chart review that was performed by an expert physician. Two hundred twenty-four of 329 predicted diseases, were correctly predicted; 56 were reasonably predicted, and 49 were incorrectly predicted. The main disease was correctly predicted in 71 patients. In conclusion, we could estimate the number of patients with each disease using the health insurance claims data with a certain degree of accuracy.

  18. Assessing Alzheimer's disease patients with the Cohen-Mansfield Agitation Inventory: scoring and clinical implications.

    PubMed

    Weiner, Myron F; Tractenberg, Rochelle E; Jin, Shelia; Gamst, Anthony; Thomas, Ronald G; Koss, Elisabeth; Thal, Leon J

    2002-01-01

    We explored the applicability of the standard scoring of the Cohen-Mansfield Agitation Inventory (CMAI), a widely used nursing-home derived instrument, to community-dwelling persons with Alzheimer's disease (AD). Item responses to the CMAI were gathered from participants in two large clinical studies, one of which specifically included patients with behavioral disturbances. Confirmatory factor analysis in these two groups of well-characterized AD patients suggested that conventional CMAI subscoring did not adequately describe the responses of these two groups. Exploratory factor analysis indicated that the four CMAI subscores, based on a verbal-physical and aggressive-non-aggressive conceptualization of behavioral disturbance, did not fit community dwelling persons with AD. Based on cross-sectional and longitudinal analyses, there was suggestive evidence for three behavioral clusters, but these clusters did not achieve statistical significance Overall, the CMAI seemed best suited to describe the overall level rather than the specific subtypes of behavioral dyscontrol in community-dwelling persons with AD. PMID:11755457

  19. Sequential Waves of Gene Expression in Patients with Clinically Defined Dengue Illnesses Reveal Subtle Disease Phases and Predict Disease Severity

    PubMed Central

    Sun, Peifang; García, Josefina; Comach, Guillermo; Vahey, Maryanne T.; Wang, Zhining; Forshey, Brett M.; Morrison, Amy C.; Sierra, Gloria; Bazan, Isabel; Rocha, Claudio; Vilcarromero, Stalin; Blair, Patrick J.; Scott, Thomas W.; Camacho, Daria E.; Ockenhouse, Christian F.; Halsey, Eric S.; Kochel, Tadeusz J.

    2013-01-01

    Background Dengue virus (DENV) infection can range in severity from mild dengue fever (DF) to severe dengue hemorrhagic fever (DHF) or dengue shock syndrome (DSS). Changes in host gene expression, temporally through the progression of DENV infection, especially during the early days, remains poorly characterized. Early diagnostic markers for DHF are also lacking. Methodology/Principal Findings In this study, we investigated host gene expression in a cohort of DENV-infected subjects clinically diagnosed as DF (n = 51) and DHF (n = 13) from Maracay, Venezuela. Blood specimens were collected daily from these subjects from enrollment to early defervescence and at one convalescent time-point. Using convalescent expression levels as baseline, two distinct groups of genes were identified: the “early” group, which included genes associated with innate immunity, type I interferon, cytokine-mediated signaling, chemotaxis, and complement activity peaked at day 0–1 and declined on day 3–4; the second “late” group, comprised of genes associated with cell cycle, emerged from day 4 and peaked at day 5–6. The up-regulation of innate immune response genes coincided with the down-regulation of genes associated with viral replication during day 0–3. Furthermore, DHF patients had lower expression of genes associated with antigen processing and presentation, MHC class II receptor, NK and T cell activities, compared to that of DF patients. These results suggested that the innate and adaptive immunity during the early days of the disease are vital in suppressing DENV replication and in affecting outcome of disease severity. Gene signatures of DHF were identified as early as day 1. Conclusions/Significance Our study reveals a broad and dynamic picture of host responses in DENV infected subjects. Host response to DENV infection can now be understood as two distinct phases with unique transcriptional markers. The DHF signatures identified during day 1–3 may have

  20. Clinical significance of exercise-induced ST segment elevation. Correlative angiographic study in patients with ischaemic heart disease.

    PubMed Central

    de Feyter, P J; Majid, P A; van Eenige, M J; Wardeh, R; Wempe, F N; Roos, J P

    1981-01-01

    We have examined the relation between electrocardiographic ST elevation during treadmill exercise (greater than or equal to 1 mm, using the conventional 12 leads), the severity of coronary artery disease, and left ventricular wall motion abnormalities in 680 patients. They were divided into three groups: (1) 218 patients with clinically significant coronary artery disease, (2) 178 patients with clinically significant coronary artery disease, and (3) 284 patients with clinically significant coronary artery disease and previous myocardial infarction. ST elevation during exercise (predominantly in lead V2) was seen in two patients (1%) in group 1, three patients (2%) in group 2, and 147 patients (52%) in group 3. Coronary artery disease (number of vessels involved and severity of stenoses) was comparable in groups 2 and 3. All the patients in group 1 showed a normal left ventricular contraction pattern; 64% of the patients in group 2 showed wall motion abnormalities (predominantly hypokinesia) and 95% of group 3 (mainly akinesia, dyskinesia, or aneurysm). A strongly positive correlation was seen between the ST elevation and left ventricular dysfunction in patients belonging to group 3. The overall sensitivity and the specificity of the stress test in detecting wall motion abnormalities was 55% and 100% respectively. The sensitivity increased with deterioration in left ventricular function, reaching 81% and 90% in patients with dyskinesia and aneurysm, respectively. Maximal ST elevation (greater than or equal to 3 mm) was confined to the patients with dyskinesia or aneurysm. The incidence of ST elevation during exercise was also related to the location of previous infarction, showing a positive response in 85% of patients with anterior myocardial infarction and in only 33% with inferior myocardial infarction. We conclude that ST segment elevation during exercise in patients with previous myocardial infarction is a sensitive and a specific indicator of advanced left

  1. Impact of Helminth Infection on the Clinical and Microbiological Presentation of Chagas Diseases in Chronically Infected Patients

    PubMed Central

    Salvador, Fernando; Sulleiro, Elena; Sánchez-Montalvá, Adrián; Martínez-Gallo, Mónica; Carrillo, Eugenia; Molina, Israel

    2016-01-01

    Abstract Background Helminth infections are highly prevalent in tropical and subtropical countries, coexisting in Chagas disease endemic areas. Helminth infections in humans may modulate the host immune system, changing the Th1/Th2 polarization. This immunological disturbance could modify the immune response to other infections. The aim of this study is to evaluate the relationship between clinical, microbiological and epidemiological characteristics of Chagas disease patients, with the presence of helminth infection. Methods A prospective observational study was conducted at Vall d’Hebron University Hospital (Barcelona, Spain). Inclusion criteria were: age over 18 years, diagnosis of Chagas disease, and not having received specific treatment for Chagas disease previously to the inclusion. The study protocol included Chagas disease assessment (cardiac and digestive evaluation, detection of T. cruzi DNA measured by PCR in peripheral blood), and helminth infection diagnosis (detection of IgG anti-Strongyloides stercoralis by ELISA, microscopic examination of stool samples from three different days, and specific faecal culture for S. stercoralis larvae). Results Overall, 65 patients were included, median age was 38 years, 75.4% were women and most of them came from Bolivia. Cardiac and digestive involvement was present in 18.5% and 27.7% of patients respectively. T. cruzi PCR was positive in 28 (43.1%) patients. Helminth infection was diagnosed in 12 (18.5%) patients. No differences were observed in clinical and epidemiological characteristics between patients with and without helminth infection. Nevertheless, the proportion of patients with positive T. cruzi PCR was higher among patients with helminth infection compared with patients without helminth infection (75% vs 35.8%, p = 0.021). Conclusions We observed a high prevalence of S. stercoralis infection among chronic Chagas disease patients attended in our tropical medicine unit. Strongyloidiasis was associated

  2. Clinical Outcomes of Thirteen Patients with Acute Chagas Disease Acquired through Oral Transmission from Two Urban Outbreaks in Northeastern Brazil

    PubMed Central

    Bastos, Claudilson J. C.; Aras, Roque; Mota, Gildo; Reis, Francisco; Dias, Juarez Pereira; de Jesus, Robson Silva; Freire, Miralba Silva; de Araújo, Eline G.; Prazeres, Juliana; Grassi, Maria Fernanda Rios

    2010-01-01

    Background Outbreaks of orally transmitted Trypanosoma cruzi continue to be reported in Brazil and are associated with a high mortality rate, mainly due to myocarditis. Methods This study is a detailed report on the disease progression of acute Chagas disease in 13 patients who were infected during two micro-outbreaks in two northeastern Brazilian towns. Clinical outcomes as well as EKG and ECHO results are described, both before and after benznidazole treatment. Results Fever and dyspnea were the most frequent symptoms observed. Other clinical findings included myalgia, periorbital edema, headache and systolic murmur. Two patients died of cardiac failure before receiving benznidazole treatment. EKG and ECHO findings frequently showed a disturbance in ventricular repolarization and pericardial effusion. Ventricular dysfunction (ejection fraction <55%) was present in 27.3% of patients. After treatment, EKG readings normalized in 91.7% of patients. Ventricular repolarization abnormalities persisted in 50% of the patients, while sinus bradycardia was observed in 18%. The systolic ejection fraction normalized in two out of three patients with initially depressed ventricular function, while pericardial effusion disappeared. Conclusions Myocarditis is frequently found and potentially severe in patients with acute Chagas disease. Benznidazole treatment may improve clinical symptoms, as well as EKG and ECHO findings. PMID:20559542

  3. Disease Phenotype, Activity and Clinical Course Prediction Based on C-Reactive Protein Levels at Diagnosis in Patients with Crohn’s Disease: Results from the CONNECT Study

    PubMed Central

    Kwon, Jee Hye; Im, Jong Pil; Ye, Byong Duk; Cheon, Jae Hee; Jang, Hyun Joo; Lee, Kang Moon; Kim, You Sun; Kim, Sang Wook; Kim, Young Ho; Song, Geun Am; Han, Dong Soo; Kim, Won Ho; Kim, Joo Sung

    2016-01-01

    Background/Aims C-reactive protein (CRP) is an easily measured index of disease activity, but its ability to predict clinical course is controversial. We therefore designed a study to determine whether the CRP level at Crohn’s disease (CD) diagnosis is a valuable indicator of the disease phenotype, activity, and clinical course. Methods We retrospectively analyzed 705 CD patients from 32 institutions. The patients were classified into two groups according to CRP level. The patients’ demographic and clinical characteristics and their use of immunosuppressive or biological agents were recorded. Disease location and behavior, hospitalization, and surgery were analyzed. Results A high CRP was associated with younger age, steroid use, colonic or ileocolonic location, high CD activity index, and active inflammation at colonoscopy (p<0.001). As the disease progressed, patients with high CRP were more likely to exhibit strictures (p=0.027). There were significant differences in the use of 5-aminosalicylic acid, antibiotics, corticosteroids, azathioprine, and infliximab (p<0.001, p<0.001, p<0.001, p<0.001, and p=0.023, respectively). Hospitalization was also more frequent in patients with high CRP. Conclusions The CRP level at diagnosis is useful for evaluating the phenotype, activity, and clinical course of CD. Closer follow-up strategies, with early aggressive treatment, could be considered for patients with high CRP. PMID:27021506

  4. System analysis of gene mutations and clinical phenotype in Chinese patients with autosomal-dominant polycystic kidney disease

    PubMed Central

    Jin, Meiling; Xie, Yuansheng; Chen, Zhiqiang; Liao, Yujie; Li, Zuoxiang; Hu, Panpan; Qi, Yan; Yin, Zhiwei; Li, Qinggang; Fu, Ping; Chen, Xiangmei

    2016-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder mainly caused by mutation in PKD1/PKD2. However, ethnic differences in mutations, the association between mutation genotype/clinical phenotype, and the clinical applicable value of mutation detection are poorly understood. We made systematically analysis of Chinese ADPKD patients based on a next-generation sequencing platform. Among 148 ADPKD patients enrolled, 108 mutations were detected in 127 patients (85.8%). Compared with mutations in Caucasian published previously, the PKD2 mutation detection rate was lower, and patients carrying the PKD2 mutation invariably carried the PKD1 mutation. The definite pathogenic mutation detection rate was lower, whereas the multiple mutations detection rate was higher in Chinese patients. Then, we correlated PKD1/PKD2 mutation data and clinical data: patients with mutation exhibited a more severe phenotype; patients with >1 mutations exhibited a more severe phenotype; patients with pathogenic mutations exhibited a more severe phenotype. Thus, the PKD1/PKD2 mutation status differed by ethnicity, and the PKD1/PKD2 genotype may affect the clinical phenotype of ADPKD. Furthermore, it makes sense to detect PKD1/PKD2 mutation status for early diagnosis and prognosis, perhaps as early as the embryo/zygote stage, to facilitate early clinical intervention and family planning. PMID:27782177

  5. Identification of Novel Mutations in ABCA4 Gene: Clinical and Genetic Analysis of Indian Patients with Stargardt Disease

    PubMed Central

    Battu, Rajani; Verma, Anshuman; Hariharan, Ramesh; Krishna, Shuba; Kiran, Ravi; Jacob, Jemima; Ganapathy, Aparna; Ramprasad, Vedam L.; Kumaramanickavel, Govindasamy; Jeyabalan, Nallathambi; Ghosh, Arkasubhra

    2015-01-01

    Stargardt disease (STGD) is the leading cause of juvenile macular degeneration associated with progressive central vision loss, photophobia, and colour vision abnormalities. In this study, we have described the clinical and genetic features of Stargardt patients from an Indian cohort. The next generation sequencing was carried out in five clinically confirmed unrelated patients and their family members using a gene panel comprising 184 retinal specific genes. Sequencing results were analyzed by read mapping and variant calling in genes of interest, followed by their verification and interpretation. Genetic analysis revealed ABCA4 mutations in all of the five unrelated patients. Among these, four patients were found with compound heterozygous mutations and another one had homozygous mutation. All the affected individuals showed signs and symptoms consistent with the disease phenotype. We report two novel ABCA4 mutations in Indian patients with STGD disease, which expands the existing spectrum of disease-causing variants and the understanding of phenotypic and genotypic correlations. Screening for causative mutations in patients with STGD using panel of targeted gene sequencing by NGS would be a cost effective tool, might be helpful in confirming the precise diagnosis, and contributes towards the genetic counselling of asymptomatic carriers and isolated patients. PMID:25922843

  6. Correlation of Promis Scales and Clinical Measures Among Chronic Obstructive Pulmonary Disease Patients With and Without Exacerbations

    PubMed Central

    Irwin, Debra E.; Atwood, Charles A.; Hays, Ron D.; Spritzer, Karen; Liu, Honghu; Donohue, James F.; Leidy, Nancy Kline; Yount, Susan E.; DeWalt, Darren A.

    2014-01-01

    Purpose The Patient-Reported Outcomes Measurement Information System (PROMIS®) initiative was developed to advance the methodology of PROs applicable to chronic diseases. Chronic obstructive pulmonary disease (COPD) is a progressive chronic disease associated with poor health. This study was designed to examine the correlation of PROMIS health-related quality of life (HRQOL) scales and clinical measures among COPD patients. Methods A cross-sectional analysis was conducted comparing patients who were stable (n = 100) with those currently experiencing a COPD exacerbation (n=85). All PROMIS measures for adults available at the time of the study (2008), disease-targeted and other HRQOL instruments, health literacy, percent predicted FEV1, and a 6-minute walk distance were assessed when patients were considered clinically stable. Results Stable COPD patients reported significantly (p≤0.05) better health-related quality of life on PROMIS domains than patients experiencing an exacerbation. PROMIS domain scores were significantly (p≤0.01) correlated with each of legacy measures. Six-minute walk scores were most highly correlated with the PROMIS physical function domain scores (r=0.53) followed by the fatigue (r=-0.26), social (r=0.24) and to a lesser extent depression (r=-0.23) and anxiety (r=-0.22) domain scores. Percent predicted FEV1 score was significantly associated with PROMIS physical function scores (r=0.27). Conclusion This study provides support for the validity of the PROMIS measures in COPD patients. PMID:25307510

  7. Double-Blind Randomized Clinical Trial: Gluten versus Placebo Rechallenge in Patients with Lymphocytic Enteritis and Suspected Celiac Disease

    PubMed Central

    Carrasco, Anna; Ibarra, Montserrat; Temiño, Rocío; Salas, Antonio; Esteve, Maria

    2016-01-01

    Background The role of gluten as a trigger of symptoms in non-coeliac gluten sensitivity has been questioned. Aim To demonstrate that gluten is the trigger of symptoms in a subgroup of patients fulfilling the diagnostic criteria for non-coeliac gluten sensitivity (NCGS), which presented with lymphocytic enteritis, positive celiac genetics and negative celiac serology. Methods Double-blind randomized clinical trial of gluten vs placebo rechallenge. Inclusion criteria: >18 years of age, HLA-DQ2/8+, negative coeliac serology and gluten-dependent lymphocytic enteritis, and GI symptoms, with clinical and histological remission at inclusion. Eighteen patients were randomised: 11 gluten (20 g/day) and 7 placebo. Clinical symptoms, quality of life (GIQLI), and presence of gamma/delta+ cells and transglutaminase deposits were evaluated. Results 91% of patients had clinical relapse during gluten challenge versus 28.5% after placebo (p = 0.01). Clinical scores and GIQLI worsened after gluten but not after placebo (p<0.01). The presence of coeliac tissue markers at baseline biopsy on a gluten-free diet allowed classifying 9 out of the 18 (50%) patients as having probable ‘coeliac lite’ disease. Conclusion This proof-of-concept study indicates that gluten is the trigger of symptoms in a subgroup of patients fulfilling the diagnostic criteria for NCGS. They were characterized by positive celiac genetics, lymphocytic enteritis, and clinical and histological remission after a gluten-free diet. Trial Registration ClinicalTrials.gov NCT02472704 PMID:27392045

  8. Effect of Selenium Supplementation on Glutathione Peroxidase Enzyme Activity in Patients With Chronic Kidney Disease: A Randomized Clinical Trial

    PubMed Central

    Sedighi, Omid; Zargari, Mehryar; Varshi, Gharmohammad

    2014-01-01

    Background: Plasma selenium (Se) concentration and glutathione peroxidase (GSH-Pxs) enzyme activity of the patients with chronic kidney disease (CKD) are usually lower than healthy individuals; however, the effect of Se supplementation on the GSH-Pxs activity in those patients remains unclear. Objectives: This study aimed to assess the effect of Se supplementation on plasma Se concentration and red blood cell (RBC) GSH-Pxs activity in patients with different stages of CKD. Patients and Methods: In this randomized clinical trial, forty-five patients with CKD who attended in a nephrology clinic were recruited. The patients were randomly allocated into three groups according to their creatinine clearance rate and were supplemented with daily Se 200 mcg for three months. Plasma Se concentration and RBC GSH-Pxs activity were measured in each patient at the beginning and at the end of the study. This clinical trial was registered in the Iranian Registry of Clinical Trials (www.irct.ir) with registration number ID of IRCT201305318501N2. Results: Plasma Se concentration and RBC GSH-Pxs activity increased significantly in all three groups of patients with CKD (P < 0.05). There were no significant differences between three groups regarding baseline plasma Se (P = 0.268) and RBC GSH-Pxs activity (P = 0.741). Conclusions: Se supplementation can increase plasma Se concentration and RBC GSH-Pxs activity in patients with different stages of CKD. PMID:25032143

  9. A clinical study on the effectiveness of implant supported dental restoration in patients with chronic periodontal diseases.

    PubMed

    Jiang, B Q; Lan, J; Huang, H Y; Liang, J; Ma, X N; Huo, L D; Xu, X

    2013-02-01

    This study evaluated the efficacy of implant supported dental restorations in patients with chronic periodontal diseases at various stages within 2 years of completing treatment. 30 patients with periodontal diseases and 30 patients without periodontal diseases were studied. Total counts of 276 implant bodies were divided into group A (patients with periodontal diseases; a total of 149 implants) and group B (patients with healthy periodontium; a total of 127 implants). In group A subjects, periodontitis was treated prior to implant placement. The study focused on patients' modified sulcus bleeding index, modified plaque index, implant mobility index, periodontal probing depth and implant success rate 12 and 24 months after the completion of the treatment. The result show: there were no significant differences in implant success rate between groups A and B; modified sulcus bleeding index scores showed differences between the groups 24 months after treatment; there were no significant differences in other clinical indexes during the study between the groups; there were no significant differences in periodontal probing depth between the groups; modified plaque index and modified sulcus bleeding index were positively correlated in implant supported dental restoration patients with chronic periodontal diseases.

  10. Heterogeneous clinical spectrum of interstitial lung disease in patients with anti-EJ anti-synthetase syndrome: a case series.

    PubMed

    Giannini, Margherita; Notarnicola, Antonella; Dastmalchi, Maryam; Lundberg, Ingrid E; Lopalco, Giuseppe; Iannone, Florenzo

    2016-09-01

    Auto-antibodies against aminoacyl-tRNA-synthetases (anti-ARS Abs) represent the hallmark of the anti-synthetase syndrome that is defined as the clinical association of fever, Raynaud's phenomenon, myositis, interstitial lung disease (ILD), arthritis and mechanic's hands. Recently, differences in clinical features depending on specific anti-ARS Abs have been reported. We describe three cases of anti-EJ (anti-glycyl) antibody-positive patients presenting with ILD as a common feature, but with heterogeneous histopathological and radiographic patterns and with different responses to treatment. Relapsing-remittent fever, refractory muscle involvement and seronegative arthritis were also striking clinical manifestations.

  11. Clinical assessment and management of dyslipidemia in patients with chronic kidney disease.

    PubMed

    Nitta, Kosaku

    2012-08-01

    Chronic kidney disease (CKD) is a common cause of cardiovascular disease (CVD). Several factors contribute to the onset and progression of atherosclerosis and CVD in CKD patients. Most of the cases of coronary heart disease in the general population can be explained by traditional risk factors, whereas non-traditional risk factors, including oxidative stress, anemia, inflammation, malnutrition, vascular calcification, and endothelial dysfunction, have been proposed to play a central role in the pathogenesis of CVD in CKD patients. However, the precise mechanism of CVD initiation in CKD patients remains unclear. Lipid-lowering therapies may decrease proteinuria, and increase or maintain renal function. Because the serum levels of triglyceride-rich lipoproteins are increased in CKD patients, particularly in advanced stages, the serum non-HDL cholesterol level may be a better biomarker of dyslipidemia than the serum LDL cholesterol level in this population. A meta-analysis showed that statin therapy was associated with decreased albuminuria in comparison with a placebo. Moreover, lipid-lowering therapy with statins is effective in reducing the risk of CVD in the early stages of CKD, whereas the benefit of statins in patients with end-stage renal disease may be limited.

  12. Clinical outcomes of three Parkinson's disease patients treated with mandibular implant overdentures.

    PubMed

    Heckmann, S M; Heckmann, J G; Weber, H P

    2000-12-01

    Parkinson's disease (PD) often affects the oro-pharyngeal musculature, leading to problems with speaking, chewing and swallowing. The inevitable reduction in food and fluid intake contributes to the further deterioration of neurological symptoms. Parkinson's disease patients have great difficulties in adjusting to the use of complete dentures. It is the purpose of this report to evaluate the benefit of using dental implants combined with overdentures to improve chewing and predigestion capacity in severely handicapped PD patients. Three edentulous PD patients (2 male, 1 female; mean age 75.7 years; mean PD duration 4.3 years; PD severity grade III according to Hoehn and Yahr; mean edentulousness 19.3 years) complaining of poor chewing ability were included in this evaluation. One-stage dental implants were placed in the interforaminal region of the mandible. After completion of healing, new overdentures were fabricated. Custom-made non-rigid (resilient) telescopic attachments were used for retention of the overdentures on the implants. Follow-up examinations of the 3 patients were made between 28 and 42 months after the completion of treatment, and peri-implant tissue conditions as well as the patients' self-assessed satisfaction level were recorded. A modified gastrointestinal symptoms questionnaire, Hoehn and Yahr Scale and body weight measurements were used to monitor gastrointestinal impairment and PD severity. The peri-implant parameters indicated healthy soft tissue conditions and all Periotest values were in the negative range. The patients judged their chewing abilities to be greatly improved. Since placing the implants, PD severity had deteriorated to grade IV (Hoehn and Yahr scale) in 2 patients and was stable in 1 patient. The body weight had improved slightly in all patients (mean 2.2 kg). On the gastrointestinal scale, all patients had improved from a mean score of 8.7 to 5.7. Non-rigid telescopic attachments for overdenture stabilization are

  13. Obese sedentary patients with dyspnoea on exertion who are at low risk for coronary artery disease by clinical criteria have a very low prevalence of coronary artery disease.

    PubMed

    Bruckel, J T; Larsen, G; Benson, M R

    2014-06-01

    Dyspnoea, a much less specific symptom of ischaemia than chest discomfort, is common among obese patients. Patients with dyspnoea often undergo stress testing as part of their evaluation. We sought to examine the yield of stress testing in non-elderly, obese, sedentary patients with dyspnoea on exertion (DOE) as a chief complaint.We reviewed stress echocardiograms carried out on 203 patients in a stress testing laboratory at a major tertiary care centre. Of these, 81 (40%) fell into a group that was at low risk for coronary artery disease (CAD) by clinical criteria. Ischaemia was detected in two patients in the low-risk group (2.5%), and these results were likely false positives. In the higher risk group, 9.0% of functional tests showed ischaemia; after further testing, 2.5% of the higher risk patients were found to have obstructive coronary lesions. Clinical follow-up was performed for a mean of 815 days. New obstructive coronary disease was detected in 1.6% of all patients, and these patients were from the higher risk group. In obese sedentary patients with DOE but otherwise at low risk of coronary disease stress testing is of very low yield. DOE is generally not an anginal equivalent in this patient population.

  14. Novel anti-carbohydrate autoantibodies in patients with inflammatory bowel disease: are they useful for clinical practice?

    PubMed

    Malickova, Karin; Lukas, Milan; Donoval, Robert; Sandova, Petra; Janatkova, Ivana

    2006-01-01

    The objective of this study was to test the diagnostic accuracy of novel anti-carbohydrate assays in patients with inflammatory bowel disease, namely in Crohn's disease. These carbohydrate assays are based on oligosaccharide chitobioside carbohydrate - anti-chitobioside carbohydrate antibodies (ACCA), laminaribioside carbohydrate anti-laminaribioside carbohydrate antibodies (ALCA), and mannobioside carbohydrate - anti-mannobioside carbohydrate antibodies (AMCA). We compared these assays with the anti-Saccharomyces cerevisiae antibodies (ASCA) assay. The results of this study suggest that ASCA are still the best serological marker for Crohn's disease. Further studies are required to explore the clinical utility of ACCA, ALCA and AMCA.

  15. The emerging concept of chronic kidney disease without clinical proteinuria in diabetic patients.

    PubMed

    Halimi, J M

    2012-10-01

    The natural history of diabetic nephropathy was defined in the 1980s on the basis of longitudinal studies undertaken in patients with type 1 and type 2 diabetes. However, an increasing number of studies have indicated that certain diabetic patients do not present with the same evolution as was then defined: for example, some often have significant initial deterioration of glomerular filtration rate whereas, in others, microalbuminuria is reduced spontaneously. Chronic kidney disease (CKD) may be accompanied, rather than preceded, by macroalbuminuria, or it may develop in patients with microalbuminuria or even in those with albuminuria levels that revert to normal. CKD can also develop in patients whose albuminuria levels remain normal. Progression to macroalbuminuria is, in fact, less frequent than regression to normoalbuminuria or no change in microalbuminuria status in diabetic patients with microalbuminuria, especially in type 1 diabetes. Some experience progressive deterioration of renal function due to diabetes without developing significant proteinuria: this is seen fairly frequently and can affect 50% of patients with renal insufficiency. Such cases are more often older patients treated with renin-angiotensin system blockers who usually have a history of cardiovascular disease. Evolution to end-stage renal disease is slower in this subgroup of patients, although histological analyses may show surprisingly advanced glomerular lesions. The main parameters of surveillance remain regular monitoring of glycaemia, and control of blood pressure and the evolution of initial albuminuria levels. Nevertheless, why some patients exhibit conventional diabetic nephropathy while others have slower declines in renal function associated with normal albuminuria levels or microalbuminuria is unclear. It is hoped that the new pathological classification of diabetic nephropathy will help in our understanding of these discrepancies.

  16. Incidence and clinical significance of repetitive ventricular response in patients without identifiable organic heart disease.

    PubMed

    Treese, N; Geibel, A; Kasper, W; Meinertz, T; Pop, T; Meyer, J

    1984-10-01

    We determined the incidence of repetitive ventricular response (RVR) after programmed electrical stimulation and the incidence of spontaneous ventricular arrhythmias during 24 hr Holter monitoring in 38 patients in whom extensive non-invasive and invasive diagnostic tests had excluded abnormalities suggestive of organic heart disease. A standardized stimulation protocol with single (S1S2) and double (S1S2S3) extrastimuli during ventricular drive at cycle lengths of 600, 500 and 430 msec with a current strength below 5 mA at the right ventricular apex was employed. RVR occurred in 20 patients (58%) after S1S2 and in 30 patients (79%) after S1S2S3 stimulation. Eighteen patients (47%) showed RVR with 2 echo beats and 1 patient had 3 echo beats. RVR was due to bundle branch reentry (BBR) in 20 patients independent of the mode of stimulation. RVR due to intraventricular reentry (IVR) was found in 17 patients (47%) only after S1S2S3 stimulation. The incidence of both BBR and IVR was influenced by the basic ventricular driving rate, decreasing with shorter basic cycle lengths. 17 patients had no ventricular premature depolarizations (VPDs), 12 patients had uniform, 4 multiform (Lown III), 2 consecutive (Lown IVA) VPDs, and 1 patient had parasystolic rhythm. There was no relation to the incidence of repetitive ventricular response. We conclude that in patients without identifiable organic heart disease RVR with more than 2 consecutive beats is rarely found if single and double extrastimuli are employed during ventricular drive. Both bundle branch and intraventricular reentry with one or two echo beats are a common finding in this population without relation to the incidence of spontaneous ventricular arrhythmias.

  17. Patient's perceptions of chronic kidney disease and their association with psychosocial and clinical outcomes: a narrative review

    PubMed Central

    Clarke, Amy L.; Yates, Thomas; Smith, Alice C.; Chilcot, Joseph

    2016-01-01

    Patients with chronic kidney disease (CKD) form organized beliefs regarding their illness and treatment. These perceptions influence the coping strategies employed by an individual to manage his/her illness and may act as a predictor for his/her willingness to engage in self-management behaviours. While illness perceptions have been identified as predictors of non-adherence, depression and mortality in dialysis patients, there is a paucity of research in CKD patients not requiring renal replacement therapy. This narrative review synthesizes the existing literature regarding the role of illness perceptions and associated clinical and psychosocial outcomes in non-dialysis CKD patients. Studies were identified following database searches of AMED, BNI, CINAHL, EMBASE, Health Business Elite, HMIC, Medline, PsycINFO and Google Scholar in January 2016. Despite the small evidence base, existing studies indicate that negative illness perceptions are associated with disease progression and a number of psychosocial outcomes in non-dialysis CKD patients. Evidence from other clinical populations suggests that illness perceptions are modifiable through psychological intervention, which may be most effective if delivered early before beliefs have the chance to become more established. Therefore, targeting illness perceptions in the earlier stages of CKD may be optimal. Further studies are now required to ascertain the mechanisms through which illness perceptions predict psychosocial and clinical outcomes in CKD patients and to ultimately test the efficacy of illness perception–based interventions. PMID:27274839

  18. Effect of Peripheral Arterial Disease on Functional and Clinical Outcomes in Patients with Heart Failure From HF-ACTION

    PubMed Central

    Jones, W. Schuyler; Clare, Robert; Ellis, Stephen J.; Mills, James S.; Fischman, David L.; Kraus, William E.; Whellan, David J.; O'Connor, Christopher M.; Patel, Manesh R.

    2011-01-01

    Patients with peripheral arterial disease (PAD) have lower functional capacity and worse clinical outcomes than age and gender matched patients. Few data exist on the relationship of PAD with functional and clinical outcomes in heart failure (HF) patients. We sought to compare HF patients with and without PAD for baseline functional capacity, response to exercise training, and clinical outcomes. HF-ACTION was a randomized controlled trial comparing usual care to structured exercise training plus usual care in HF patients with an ejection fraction ≤ 35% and NYHA class II – IV heart failure symptoms. Cardiopulmonary exercise (CPX) testing occurred at enrollment, 3 months, and 1 year. Clinical follow-up occurred up to 4 years. Of the 2331 HF-ACTION patients, 157 (6.8%) had PAD. At baseline, HF patients with PAD had a lower exercise duration (8.0 vs. 9.8 minutes, p<0.001), lower peak oxygen consumption (VO2) (12.5 vs. 14.6 mL/kg/min, p<0.001), and shorter six minute walking distance (306 vs. 371 meters, p<0.001) compared to HF patients without PAD. At three months, HF patients with PAD had less improvement on CPX testing [exercise duration (0.5 vs. 1.1 minutes; p=0.002) and peak VO2 (mean change; 0.1 vs. 0.6 mL/kg/min; p=0.04)] compared to HF patients without PAD. PAD was an independent predictor of all-cause death or hospitalization [hazard ratio (95% CI); 1.31 (1.06 – 1.62), p=0.011]. PAD patients with HF have depressed baseline exercise capacity and decreased response to exercise training. In conclusion, PAD is an independent predictor of all-cause death or hospitalization in HF patients. PMID:21565325

  19. The antiplatelet effects of nitrates: is it of clinical significance in patients with cardiovascular disease?

    PubMed

    Zhou, Rui-Hai; Frishman, William H

    2010-01-01

    Organic nitrates have been used for over a century in cardiovascular therapy and are still widely used in the treatment of acute coronary syndromes, chronic angina pectoris, and congestive heart failure. Nitrates, together with sodium nitroprusside, generally referred to as nitrovasodilators, exert their biologic effects via the release of nitric oxide. They are also known as nitric oxide donors. The mechanism of action of these drugs is traditionally believed to lie in their arterial vasodilation and venodilation effects, resulting in an improvement of coronary artery blood supply and/or reduction of cardiac workload in the treatment of coronary artery disease and congestive heart failure. Recently it has been recognized that these drugs also have intrinsic antiplatelet and antithrombotic effects, demonstrated both in vitro and in vivo, which would add further rationale for the use of these drugs in atherothrombotic diseases. Research has shown that nitrovasodilators can nonselectively inhibit platelet aggregation induced by multiple stimuli. However, clinical trials have yielded conflicting results regarding clinical outcome, especially with long-term nitrate use. The potentially beneficial effects of nitrates could be negated by the development of tolerance and the generation of deleterious oxidative stress causing endothelial dysfunction during continuous nitrate administration. Much progress has been made in the development of new nitric oxide donors devoid of oxidant-generating properties. Novel combination therapies with nitrovasodilators plus antioxidants or agents with antioxidant properties have shown promise in reducing or reversing tolerance, potentiating antiplatelet effects, and improving clinical outcome. It is expected that clinical introduction of novel nitrovasodilator regimens will provide a new approach to the prevention and treatment of atherothrombotic diseases. Large-scale clinical trials will ultimately provide the evidence-based answers.

  20. The clinical characteristics of gastroesophageal reflux disease in patients with laryngeal symptoms who are referred to gastroenterology.

    PubMed

    Oh, J-H; Choi, M-G; Park, J-M; Lim, C-H; Cho, Y-K; Lee, I-S; Kim, S-W; Chung, I-S

    2013-07-01

    The prevalence of gastroesophageal reflux disease (GERD) has increased recently in Asia-Pacific countries. However, little is known about its prevalence and clinical characteristics in GERD patients with atypical symptoms in Asia. The aim of this study was to investigate the clinical characteristics of GERD in patients who had laryngeal symptoms in Korea. Data were gathered retrospectively from patients who presented with atypical symptoms, such as throat discomfort, globus pharyngeus, hoarseness, and chronic cough. They underwent a 24-hour ambulatory intraesophageal pH monitoring and filled in a validated reflux questionnaire. Overall, 128 patients (36 men and 92 women) with laryngeal symptoms were included. Of these 128, 43 patients (34%) had erosive esophagitis or pathological reflux from 24-hour ambulatory pH monitoring, and 24 (19%) had a positive Bernstein test or positive symptom index from 24-hour pH monitoring. Sixty-one patients (48%) had no evidence of reflux esophagitis on upper endoscopy and pathological acid reflux on 24-hour pH monitoring. Fifty-six patients (44%) had weekly heartburn or regurgitation. Typical symptoms and dyspepsia were significantly more common in patients with GERD who had laryngeal symptoms than non-GERD. Fifty-two percent of patients had laryngeal symptoms that were associated with GERD. The presence of typical reflux symptoms and dyspepsia are risk factors for GERD in patients who present with laryngeal symptoms.

  1. Clinical outcomes of kidney transplants on patients with end-stage renal disease secondary to lupus nephritis, polycystic kidney disease and diabetic nephropathy

    PubMed Central

    Nieto-Ríos, John Fredy; Builes-Rodriguez, Sheila Alexandra; Restrepo-Correa, Ricardo Cesar; Aristizabal-Alzate, Arbey; Ocampo-Kohn, Catalina; Serna-Campuzano, Angélica; Cardona-Díaz, Natalia; Giraldo-Ramirez, Nelson Darío; Zuluaga-Valencia, Gustavo Adolfo

    2016-01-01

    Background: Patients with lupus nephritis could progress to end-stage renal disease (10-22%); hence, kidney transplants should be considered as the treatment of choice for these patients. Objective: To evaluate the clinical outcomes after kidney transplants in patients with chronic kidney diseases secondary to lupus nephritis, polycystic kidney disease and diabetes nephropathy at Pablo Tobon Uribe Hospital. Methods: A descriptive and retrospective study performed at one kidney transplant center between 2005 and 2013. Results: A total of 136 patients, 27 with lupus nephritis (19.9%), 31 with polycystic kidney disease (22.8%) and 78 with diabetes nephropathy (57.4%), were included in the study. The graft survivals after one, three and five years were 96.3%, 82.5% and 82.5% for lupus nephritis; 90%, 86% and 76.5% for polycystic kidney disease and 91.7%, 80.3% and 67.9% for diabetes nephropathy, respectively, with no significant differences (p= 0.488); the rate of lupus nephritis recurrence was 0.94%/person-year. The etiology of lupus vs diabetes vs polycystic disease was not a risk factor for a decreased time of graft survival (Hazard ratio: 1.43; 95% CI: 0.52-3.93). Conclusion: Kidney transplant patients with end stage renal disease secondary to lupus nephritis has similar graft and patient survival success rates to patients with other kidney diseases. The complication rate and risk of recurrence for lupus nephritis are low. Kidney transplants should be considered as the treatment of choice for patients with end stage renal disease secondary to lupus nephritis. PMID:27226665

  2. Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID)

    PubMed Central

    2011-01-01

    Introduction Evidence from a number of open-label, uncontrolled studies has suggested that rituximab may benefit patients with autoimmune diseases who are refractory to standard-of-care. The objective of this study was to evaluate the safety and clinical outcomes of rituximab in several standard-of-care-refractory autoimmune diseases (within rheumatology, nephrology, dermatology and neurology) other than rheumatoid arthritis or non-Hodgkin's lymphoma in a real-life clinical setting. Methods Patients who received rituximab having shown an inadequate response to standard-of-care had their safety and clinical outcomes data retrospectively analysed as part of the German Registry of Autoimmune Diseases. The main outcome measures were safety and clinical response, as judged at the discretion of the investigators. Results A total of 370 patients (299 patient-years) with various autoimmune diseases (23.0% with systemic lupus erythematosus, 15.7% antineutrophil cytoplasmic antibody-associated granulomatous vasculitides, 15.1% multiple sclerosis and 10.0% pemphigus) from 42 centres received a mean dose of 2,440 mg of rituximab over a median (range) of 194 (180 to 1,407) days. The overall rate of serious infections was 5.3 per 100 patient-years during rituximab therapy. Opportunistic infections were infrequent across the whole study population, and mostly occurred in patients with systemic lupus erythematosus. There were 11 deaths (3.0% of patients) after rituximab treatment (mean 11.6 months after first infusion, range 0.8 to 31.3 months), with most of the deaths caused by infections. Overall (n = 293), 13.3% of patients showed no response, 45.1% showed a partial response and 41.6% showed a complete response. Responses were also reflected by reduced use of glucocorticoids and various immunosuppressives during rituximab therapy and follow-up compared with before rituximab. Rituximab generally had a positive effect on patient well-being (physician's visual analogue scale; mean

  3. Clinical, microbiological, and immunological effects of fructo‐oligosaccharide in patients with Crohn's disease

    PubMed Central

    Lindsay, J O; Whelan, K; Stagg, A J; Gobin, P; Al‐Hassi, H O; Rayment, N; Kamm, M A; Knight, S C; Forbes, A

    2006-01-01

    Background and aims The intestinal microbiota play a pivotal role in the inflammation associated with Crohn's disease through their interaction with the mucosal immune system. Some bifidobacteria species are immunoregulatory and induce increased dendritic cell interleukin 10 (IL‐10) release in vitro. Fructo‐oligosaccharides (FOS) increase faecal and mucosal bifidobacteria in healthy volunteers. The aim of this study was to assess the effect of FOS administration on disease activity, bifidobacteria concentrations, and mucosal dendritic cell function in patients with moderately active Crohn's disease. Patients and methods Ten patients with active ileocolonic Crohn's disease received 15 g of FOS for three weeks. Disease activity was measured using the Harvey Bradshaw index. Faecal and mucosal bifidobacteria were quantified by fluorescence in situ hybridisation, and mucosal dendritic cell IL‐10 and Toll‐like receptor (TLR) expression were assessed by flow cytometry of dissociated rectal biopsies. Results FOS induced a significant reduction in the Harvey Bradshaw index from 9.8 (SD 3.1) to 6.9 (3.4) (p<0.01). There was a significant increase in faecal bifidobacteria concentration from 8.8 (0.9) log10 to 9.4 (0.9) log10 cells/g dry faeces (p<0.001). The percentage of IL‐10 positive dendritic cells increased from 30 (12)% to 53 (10)% (p = 0.06). Finally, the percentage of dendritic cells expressing TLR2 and TLR4 increased from 1.7 (1.7)% to 36.8 (15.9)% (p = 0.08) and from 3.6 (3.6)% to 75.4 (3.4)% (p<0.001), respectively. Conclusions FOS supplementation increases faecal bifidobacteria concentrations and modifies mucosal dendritic cell function. This novel therapeutic strategy appears to decrease Crohn's disease activity in a small open label trial and therefore warrants further investigation. PMID:16162680

  4. [Clinical outcomes of 2 pediatric patients with Gaucher's disease in enzyme replacement therapy for 9 years].

    PubMed

    Quijada Fraile, Pilar; Martín Hernández, Elena; Teresa García-Silva, María

    2011-09-01

    We report two cases of type 1 Gaucher's disease in childhood and their outcomes after 9 years of enzyme replacement therapy. The first case concerns a 6-year-old boy who was diagnosed with Gaucher's disease after developing petechial exanthema, thrombocytopenia, anemia and hepatosplenomegaly, coinciding with chickenpox. The second case involves a 9-year-old girl who was referred to our unit. She had hepatosplenomegaly since 4-month-old and subsequently developed thrombocytopenia. Both patients have the N370S/L444P mutation. Enzyme replacement therapy was started with 60 U/kg imiglucerase every 2 weeks at the age of 6 and 9 years, respectively. In both patients, the therapeutic goals were achieved and maintained throughout treatment with a dose of 30 U/kg. PMID:22230125

  5. Self-management in patients with inflammatory bowel disease: strategies, outcomes, and integration into clinical care.

    PubMed

    Plevinsky, Jill M; Greenley, Rachel N; Fishman, Laurie N

    2016-01-01

    Self-management, including medication adherence, is associated with improved health and outcomes for patients with inflammatory bowel disease. The concept of self-management is complex, but can be divided into those aspects that involve the individual patient, those that involve the provider-patient relationship, and those that encompass the social environment. At the individual level, enhancing problem-solving skills and self-efficacy have both been shown to improve self-management tasks, particularly adherence to treatment. However, it is critical to consider these domains from a lifespan perspective because these processes by which self-management can be improved are distinct for children, adolescents, young adults, and adults. A particular emphasis is placed on strategies to improve self-management of older adolescents and young adults as they transition from pediatric to adult providers. The review concludes with recommendations for providers, including rationale and techniques for assessing and promoting patient self-efficacy, encouraging the development of problem-solving skills, improving the patient-provider relationship, and enhancing social support. Providers are encouraged to utilize elements of problem-solving skills training, engage in collaborative relationships with their patients, and offer their patients recommendations for how to increase the quality of their social support networks as ways of increasing overall self-management.

  6. Self-management in patients with inflammatory bowel disease: strategies, outcomes, and integration into clinical care.

    PubMed

    Plevinsky, Jill M; Greenley, Rachel N; Fishman, Laurie N

    2016-01-01

    Self-management, including medication adherence, is associated with improved health and outcomes for patients with inflammatory bowel disease. The concept of self-management is complex, but can be divided into those aspects that involve the individual patient, those that involve the provider-patient relationship, and those that encompass the social environment. At the individual level, enhancing problem-solving skills and self-efficacy have both been shown to improve self-management tasks, particularly adherence to treatment. However, it is critical to consider these domains from a lifespan perspective because these processes by which self-management can be improved are distinct for children, adolescents, young adults, and adults. A particular emphasis is placed on strategies to improve self-management of older adolescents and young adults as they transition from pediatric to adult providers. The review concludes with recommendations for providers, including rationale and techniques for assessing and promoting patient self-efficacy, encouraging the development of problem-solving skills, improving the patient-provider relationship, and enhancing social support. Providers are encouraged to utilize elements of problem-solving skills training, engage in collaborative relationships with their patients, and offer their patients recommendations for how to increase the quality of their social support networks as ways of increasing overall self-management. PMID:27601930

  7. Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus.

    PubMed

    Feng, X; Zou, Y; Pan, W; Wang, X; Wu, M; Zhang, M; Tao, J; Zhang, Y; Tan, K; Li, J; Chen, Z; Ding, X; Qian, X; Da, Z; Wang, M; Sun, L

    2014-03-01

    The objective of this study is to evaluate the association of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus (SLE) in a large, multicenter Chinese cohort. Medical records of 1898 SLE inpatients from 15 hospitals were reviewed and classified into three groups according to their ages at disease presentation. Categorical data were analyzed by chi-square test and potentially associated factors were tested by multinomial logistic regression. Among the patients studied, 259 (13.6%) were juvenile onset (≤18 years), 1444 (76.1%) were early onset (>18 and ≤45 years) and 195 (10.3%) were late onset (>45 years). Whenever manifestations occurred, most patients (>80%) were diagnosed within two years. Juvenile-onset patients were more likely to be untreated before admission (p < 0.001) and have mucocutaneous manifestations (p < 0.001), but musculoskeletal symptoms (p < 0.05) and leukopenia (p < 0.05) were less frequent, while comorbidities were much higher in patients with late-onset SLE (p < 0.001). Neuropsychiatric, cardiopulmonary, renal and gastrointestinal involvement, disease activity index and damage scores were similar among three groups. Anti-Sm antibodies were less prevalent in late-onset patients (p < 0.05) and antimalarial drugs were more often applied to juvenile-onset patients (p < 0.001). As expected, mortality was elevated in the late-onset SLE group (p < 0.05), in which nearly half died of infections, which was much higher than those in the other two groups (p < 0.001). Logistic regression confirmed that patients with juvenile- and early-onset disease were associated with high incidence of being untreated prior to admission, and with low incidence of comorbidities as well as deaths caused by infection compared to patients with late-onset lupus. Interestingly, our data showed that more patients with late-onset disease had a SLEDAI score change of >7 at discharge. In

  8. Demographics, clinical disease characteristics, and quality of life in a large cohort of psoriasis patients with and without psoriatic arthritis

    PubMed Central

    Truong, B; Rich-Garg, N; Ehst, BD; Deodhar, AA; Ku, JH; Vakil-Gilani, K; Danve, A; Blauvelt, A

    2015-01-01

    Innovation What is already known about the topic: psoriasis (PsO) is a common skin disease with major impact on quality of life (QoL). Patient-reported data on QoL from large number of PsO patients with and without psoriatic arthritis (PsA) are limited. What this study adds: In a large cohort referred to a university psoriasis center, patients with PsO and concomitant PsA (~30% in this group) had greater degrees of skin and nail involvement and experienced greater negative impacts on QoL. Despite large numbers of patients with moderate-to-severe disease, use of systemic therapy by community practitioners was uncommon. Background PsO and PsA are common diseases that have marked adverse impacts on QoL. The disease features and patient-reported QoL data comparing PsO and PsA patients are limited. Objective To identify and compare demographics, clinical disease characteristics, and QoL scores in a large cohort of PsO patients with and without PsA. Methods All PsO patients seen in a psoriasis specialty clinic, named the Center of Excellence for Psoriasis and Psoriatic Arthritis, were enrolled in an observational cohort. Demographic, QoL, and clinical data were collected from patient-reported questionnaires and from physical examinations performed by Center of Excellence for Psoriasis and Psoriatic Arthritis dermatologists and a rheumatologists. Cross sectional descriptive data were collected and comparisons between patients with PsO alone and those with concomitant PsA are presented. Results A total of 568 patients were enrolled in the database. Mean age of PsO onset was 28 years and mean disease duration was 18 years. Those with family history had an earlier onset of PsO by ~7 years. Mean body surface area involvement with PsO was 14%. Mean body mass index was 30.7. Prevalence of PsA was 29.8%. PsA patients had a higher mean body surface area compared to patients with PsO alone (16.7% vs 13.4%, P<0.05), higher prevalence of psoriatic nail changes (54.4% vs 36%, P<0

  9. Autoimmune thyroid disease in a cohort of Malaysian SLE patients: frequency, clinical and immunological associations.

    PubMed

    Ong, S G; Choy, C H

    2016-01-01

    Autoimmune thyroid disease (ATD) has been associated with other systemic autoimmune diseases. To date, there is limited data on thyroid disorders and autoimmune thyroid disease in Malaysia. The frequency of ATD among 189 systemic lupus erythematosus (SLE) patients was 6.3%, with 2.6% in the hyperthyroid group and 3.7% in the hypothyroid group. Hypothyroidism developed at a much younger mean age (24.3 years), suggesting that SLE might be a predisposing factor for the development of Hashimoto's thyroiditis. There was a higher rate of thyroid peroxidase antibody (TPO) positivity compared with anti-thyroglobulin antibody (Tg) in the hyperthyroid subgroup. This study also demonstrated a greater proportion of ATD patients who demonstrated high titres (≥ 1:6400) of TPO compared with high titres of Tg. Although there was an association between ATD and the presence of anti-Ro/SSA and/or anti-La/SSB antibodies, the absence of sicca symptoms and negative Schirmer's tests suggest a lack of association with secondary Sjogren's syndrome. A novel association between ATD and antiphospholipid syndrome (APS) was detected in our cohort. Hence we propose that patients affected by APS be routinely screened for ATD.

  10. Plasma levels of tumour necrosis factor and its soluble receptors correlate with clinical features and outcome of Hodgkin's disease patients.

    PubMed Central

    Warzocha, K.; Bienvenu, J.; Ribeiro, P.; Moullet, I.; Dumontet, C.; Neidhardt-Berard, E. M.; Coiffier, B.; Salles, G.

    1998-01-01

    A prospective study was performed to assess the use of plasma measurement of tumour necrosis factor (TNF), lymphotoxin alpha (LT alpha) and their soluble receptors (p55 and p75) for prognostic risk assignment in 61 patients with Hodgkin's disease. Plasma levels of TNF, p55 and p75, but not of LT alpha, were higher in Hodgkin's disease patients than in healthy controls. Plasma levels of TNF, p55 and p75 were associated with several prognostic factors for Hodgkin's disease, including those related to the host (age, performance status) and to the tumour (disease stage, extranodal site involvement, bulky tumour, serum levels of LDH and beta2-microglobulin, histology). Elevated plasma levels of TNF, p55 and p75 were also associated with several parameters reflecting an immune activation, including the presence of B symptoms, elevated serum levels of gammaglobulins, alkaline phosphatase and fibrinogen, as well as peripheral monocytosis, anaemia and low serum albumin levels. Finally, elevated TNF ligand receptor plasma markers were associated with a lower incidence of complete response to therapy and predicted shorter free-from-progression survival and overall survival of the patients. These results indicate that the plasma levels of TNF and its soluble receptors correlate with clinical features and outcome of patients with Hodgkin's disease. PMID:9649158

  11. Self-management in patients with inflammatory bowel disease: strategies, outcomes, and integration into clinical care

    PubMed Central

    Plevinsky, Jill M; Greenley, Rachel N; Fishman, Laurie N

    2016-01-01

    Self-management, including medication adherence, is associated with improved health and outcomes for patients with inflammatory bowel disease. The concept of self-management is complex, but can be divided into those aspects that involve the individual patient, those that involve the provider–patient relationship, and those that encompass the social environment. At the individual level, enhancing problem-solving skills and self-efficacy have both been shown to improve self-management tasks, particularly adherence to treatment. However, it is critical to consider these domains from a lifespan perspective because these processes by which self-management can be improved are distinct for children, adolescents, young adults, and adults. A particular emphasis is placed on strategies to improve self-management of older adolescents and young adults as they transition from pediatric to adult providers. The review concludes with recommendations for providers, including rationale and techniques for assessing and promoting patient self-efficacy, encouraging the development of problem-solving skills, improving the patient–provider relationship, and enhancing social support. Providers are encouraged to utilize elements of problem-solving skills training, engage in collaborative relationships with their patients, and offer their patients recommendations for how to increase the quality of their social support networks as ways of increasing overall self-management.

  12. Self-management in patients with inflammatory bowel disease: strategies, outcomes, and integration into clinical care

    PubMed Central

    Plevinsky, Jill M; Greenley, Rachel N; Fishman, Laurie N

    2016-01-01

    Self-management, including medication adherence, is associated with improved health and outcomes for patients with inflammatory bowel disease. The concept of self-management is complex, but can be divided into those aspects that involve the individual patient, those that involve the provider–patient relationship, and those that encompass the social environment. At the individual level, enhancing problem-solving skills and self-efficacy have both been shown to improve self-management tasks, particularly adherence to treatment. However, it is critical to consider these domains from a lifespan perspective because these processes by which self-management can be improved are distinct for children, adolescents, young adults, and adults. A particular emphasis is placed on strategies to improve self-management of older adolescents and young adults as they transition from pediatric to adult providers. The review concludes with recommendations for providers, including rationale and techniques for assessing and promoting patient self-efficacy, encouraging the development of problem-solving skills, improving the patient–provider relationship, and enhancing social support. Providers are encouraged to utilize elements of problem-solving skills training, engage in collaborative relationships with their patients, and offer their patients recommendations for how to increase the quality of their social support networks as ways of increasing overall self-management. PMID:27601930

  13. [Ischemic heart disease in elderly patients of behavioral type A (psychosocial and clinic functional changes, possibilities for correction)].

    PubMed

    Fedorets, V N

    2011-01-01

    833 patients aged 32 to 74 years who had coronary heart disease (CHD) were examined. Psychosocial and clinicofunctional features typical for elderly people of behavioral type A and with diagnosed CHD and ways for treatment optimization and disease prophylaxis are being under consideration. We studied the clinical efficiency and influence of Anaprilin, Metoprolol, Phenazepam, non-medical methods on psychoendocrine parameters in CHD elderly patients with behavioral type A. In addition to positive changes in the anginal syndrome sleep, decreases in hot temper, irritability, anxiety, phobic manifestations, hypochondrial trends, and neurotic asthenization, increased working and social orientation were improved. During Anaprilin, Metoprolol, Phenazepam therapy, cardialgias reduced or disappered, the incidence of arrhythmia decreased. There was a reduction in the personality profile in the neurotic triad and psychoasthenia scales, the reactive anxiety diminished significantly, and levels of aldosterone, cortisol, triiodothyronine, and thyroxine in the blood decreased. Thus, supplementation of Anapriline, Metoprolol, Phenazepam to the combined therapy for patients with CHD of behavioral type A positively effected to the clinical course of the disease. Configuration of the personality profile was leveled in patients with CHD of coronary type, signs of behavioral type A decreased. PMID:21809638

  14. Wormwood (Artemisia absinthium) suppresses tumour necrosis factor alpha and accelerates healing in patients with Crohn's disease - A controlled clinical trial.

    PubMed

    Krebs, Simone; Omer, Talib N; Omer, Bilal

    2010-04-01

    Suppression of tumour necrosis factor alpha (TNF-alpha) and other interleukins by wormwood (Artemisia absinthium) extracts were reported recently in in vitro studies. The aim of the present study was to find out if this effect can be also be observed in Crohn's Disease (CD) patients where TNF-alpha appears to play an important role. In a controlled trial, 10 randomly selected patients suffering from CD were given in addition to their basic CD therapy 3x750mg dried powdered wormwood for 6 weeks. Ten patients, also randomly selected who met the inclusion criteria served as control group. Minimum score of 200 on Crohn's Disease Activity Index (CDAI) was required at baseline for inclusion in each group. Patients who received infliximab or similar were excluded from the trial. TNF-alpha level in serum were measured at baseline, and after three and six weeks. During this period all concomitant CD medications was maintained at the baseline dose levels. Average serum TNF-alpha level fell from 24.5+/-3.5pg/ml at baseline to 8.0+/-2.5pg/ml after six weeks. The corresponding levels in the control group were 25.7+/-4.6 (week 0), and 21.1+/-3.2 (week 6). On the clinical side, CDAI scores fell from 275+/-15 to below 175+/-12 in wormwood group with remission of symptoms in eight patients (CDAI score below 170 or reduction by 70 points), compared to only two in the placebo group (CDAI of placebo group 282+/-11 at baseline and 230+/-14 on week 6). IBDQ also reflected accelerated clinical response with wormwood. Of clinical significance were the findings that wormwood also improved mood of the CD patients, as reflected in Hamilton's Depression Scale. These findings provide a base to test wormwood in clinical conditions thought to be mediated by increased production of pro-inflammatory cytokines such as TNF-alpha.

  15. Clinical presentation and cardiovascular risk profiles in patients with left main coronary artery disease in a middle eastern country.

    PubMed

    Gehani, A A; El-Menyar, Ayman; Elgendy, Islam; Abuzaid, Ahmed; Ahmed, Emad; Haque, Saiful

    2013-04-01

    We evaluated the prevalence and clinical profile of patients with left main coronary artery disease (LMCA) in Qatar between 2006 and 2010. Patients were divided into 2 groups: patients with LMCA and patients without LMCA but had severe 3-vessel disease (VeD) eligible for surgical revascularization. Among 7000 patients who underwent coronary angiography, 210 patients had significant LMCA and 200 patients with severe 3VeD were matched for age and sex. Diabetes mellitus and hypertension were comparable in the 2 groups. Presentations with myocardial infarction or heart failure were comparable in both groups. Isolated LMCA was 4-fold higher in women (P = .02). Dyslipidemia and smoking were more prevalent in patients with distal and proximal lesions, respectively. Renal failure was independent predictor of LMCA (adjusted odds ratio: 2.6; 95% confidence interval: 1.43-4.69). One-year mortality was higher in LMCA (P = .01). The LMCA carries high mortality. Certain cardiovascular risk factors were important predictors of stenosis site. PMID:22492251

  16. Clinical and neuroimaging differences between posterior cortical atrophy and typical amnestic Alzheimer’s disease patients at an early disease stage

    PubMed Central

    Peng, Guoping; Wang, Jianqin; Feng, Zhan; Liu, Ping; Zhang, Yafei; He, Fangping; Chen, Zhongqin; Zhao, Kui; Luo, Benyan

    2016-01-01

    To identify clinical and neuroimaging characteristics between posterior cortical atrophy (PCA) and typical amnestic Alzheimer’s disease (tAD) patients at an early disease stage, 16 PCA and 13 age-matched tAD patients were enrolled. Compared with tAD patients, PCA patients showed higher mean recognition and recall test scores, and lower mean calculation, spatial attention, shape discrimination, and writing test scores. Mean right hippocampal volume was larger in PCA patients compared with tAD patients, while cortical gray matter (GM) volume of bilateral parietal and occipital lobes was smaller in PCA patients. Further, when compared with tAD patients, significant hypometabolism was observed in bilateral parietal and occipital lobes, particularly the right occipitotemporal junction in PCA patients. Additionally, there were significant positive correlations in recognition and recall scores with hippocampal volumes. In PCA patients, calculation and visuospatial ability scores are positively associated with GM volume of parietal and occipital lobes. And only spatial attention and shape discrimination scores are positively associated with regional glucose metabolism of parietal and occipital lobes. Therefore, PCA patients display better recognition and recall scores, which are associated with larger hippocampal volumes and poorer performance in visual spatial tasks because of marked GM atrophy and hypometabolism of parietal and occipital lobes. PMID:27377199

  17. Prevalence and clinical significance of nonorgan specific antibodies in patients with autoimmune thyroiditis as predictor markers for rheumatic diseases.

    PubMed

    Elnady, Basant M; Kamal, Naglaa M; Shaker, Raneyah H M; Soliman, Amal F; Hasan, Waleed A; Alghamdi, Hamed A; Algethami, Mohammed M; Jajah, Mohamed Bilal

    2016-09-01

    Autoimmune diseases are considered the 3rd leading cause of morbidity and mortality in the industrialized countries. Autoimmune thyroid diseases (ATDs) are associated with high prevalence of nonorgan-specific autoantibodies, such as antinuclear antibodies (ANA), antidouble-stranded deoxyribonucleic acid (anti-dsDNA), antiextractable-nuclear antigens (anti-ENAs), rheumatoid factor (RF), and anticyclic-citrullinated peptides (anti-CCP) whose clinical significance is unknown.We aimed to assess the prevalence of various nonorgan-specific autoantibodies in patients with ATD, and to investigate the possible association between these autoantibodies and occurrence of rheumatic diseases and, if these autoantibodies could be considered as predictor markers for autoimmune rheumatic diseases in the future.This study had 2 phases: phase 1; in which 61 ATD patients free from rheumatic manifestations were assessed for the presence of these nonorgan-specific autoantibodies against healthy 61 control group, followed by 2nd phase longitudinal clinical follow-up in which cases are monitored systematically to establish occurrence and progression of any rheumatic disease in association to these autoantibodies with its influences and prognosis.Regarding ATD patients, ANA, anti-dsDNA, Anti-ENA, and RF were present in a percentage of (50.8%), (18%), (21.3%), and (34.4%), respectively, with statistically significance difference (P < 0.5) rather than controls. Nearly one third of the studied group (32.8%) developed the rheumatic diseases, over 2 years follow-up. It was obvious that those with positive anti-dsDNA had higher risk (2.45 times) to develop rheumatic diseases than those without. There was a statistically significant positive linear relationship between occurrence of disease in months and (age, anti-dsDNA, anti-CCP, RF, and duration of thyroiditis). Anti-dsDNA and RF are the most significant predictors (P < 0.0001).ATD is more associated with rheumatic diseases than

  18. Prevalence and clinical significance of nonorgan specific antibodies in patients with autoimmune thyroiditis as predictor markers for rheumatic diseases

    PubMed Central

    Elnady, Basant M.; Kamal, Naglaa M.; Shaker, Raneyah H.M.; Soliman, Amal F.; Hasan, Waleed A.; Alghamdi, Hamed A.; Algethami, Mohammed M.; Jajah, Mohamed Bilal

    2016-01-01

    Abstract Autoimmune diseases are considered the 3rd leading cause of morbidity and mortality in the industrialized countries. Autoimmune thyroid diseases (ATDs) are associated with high prevalence of nonorgan-specific autoantibodies, such as antinuclear antibodies (ANA), antidouble-stranded deoxyribonucleic acid (anti-dsDNA), antiextractable-nuclear antigens (anti-ENAs), rheumatoid factor (RF), and anticyclic-citrullinated peptides (anti-CCP) whose clinical significance is unknown. We aimed to assess the prevalence of various nonorgan-specific autoantibodies in patients with ATD, and to investigate the possible association between these autoantibodies and occurrence of rheumatic diseases and, if these autoantibodies could be considered as predictor markers for autoimmune rheumatic diseases in the future. This study had 2 phases: phase 1; in which 61 ATD patients free from rheumatic manifestations were assessed for the presence of these nonorgan-specific autoantibodies against healthy 61 control group, followed by 2nd phase longitudinal clinical follow-up in which cases are monitored systematically to establish occurrence and progression of any rheumatic disease in association to these autoantibodies with its influences and prognosis. Regarding ATD patients, ANA, anti-dsDNA, Anti-ENA, and RF were present in a percentage of (50.8%), (18%), (21.3%), and (34.4%), respectively, with statistically significance difference (P < 0.5) rather than controls. Nearly one third of the studied group (32.8%) developed the rheumatic diseases, over 2 years follow-up. It was obvious that those with positive anti-dsDNA had higher risk (2.45 times) to develop rheumatic diseases than those without. There was a statistically significant positive linear relationship between occurrence of disease in months and (age, anti-dsDNA, anti-CCP, RF, and duration of thyroiditis). Anti-dsDNA and RF are the most significant predictors (P < 0.0001). ATD is more associated with rheumatic

  19. Correlation of serum levels of macrophage migration inhibitory factor with disease severity and clinical outcome in dengue patients.

    PubMed

    Chen, Lien-Cheng; Lei, Huan-Yao; Liu, Ching-Chuan; Shiesh, Shu-Chu; Chen, Shun-Hua; Liu, Hsiao-Sheng; Lin, Yee-Shin; Wang, Shan-Tair; Shyu, Huey-Wen; Yeh, Trai-Ming

    2006-01-01

    Dengue virus infection can cause mild dengue fever (DF) or severe dengue hemorrhagic fever (DHF) and dengue shock syndrome (DSS). Cytokines are believed to be involved in the pathogenesis of dengue infection. However, the role of the pro-inflammatory cytokine macrophage migration inhibitory factor (MIF) in dengue infection is unclear. In this study, serum levels of MIF in adult dengue patients with different disease severity and clinical outcome were determined and compared with the levels of other cytokines, tumor necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6), IL-10, and interferon gamma (IFN-gamma), in the same patients. Serum levels of MIF, IL-6, and IL-10, but not IFN-gamma or TNF-alpha, were higher in all DHF patients who died than in DHF survivors and DF patients. We conclude that in addition to IL-6 and IL-10, elevated levels of serum MIF are a potential predictor of disease severity and clinical outcome in dengue patients.

  20. Clinical Decision Making With Myocardial Perfusion Imaging in Patients With Known or Suspected Coronary Artery Disease

    PubMed Central

    Cremer, Paul; Hachamovitch, Rory; Tamarappoo, Balaji

    2015-01-01

    Myocardial perfusion imaging (MPI) to diagnose coronary artery disease (CAD) is best performed in patients with intermediate pretest likelihood of disease; unfortunately, pretest likelihood is often overestimated, resulting in the inappropriate use of perfusion imaging. A good functional capacity often predicts low risk, and MPI for diagnosing CAD should be reserved for individuals with poor exercise capacity, abnormal resting electrocardiography, or an intermediate or high probability of CAD. With respect to anatomy-based testing, coronary CT angiography has a good negative predictive value, but stenosis severity correlates poorly with ischemia. Therefore decision making with respect to revascularization may be limited when a purely noninvasive anatomical test is used. Regarding perfusion imaging, the diagnostic accuracies of SPECT, PET, and cardiac magnetic resonance are similar, though fewer studies are available with cardiac magnetic resonance. PET coronary flow reserve may offer a negative predictive value sufficiently high to exclude severe CAD such that patients with mild to moderate reversible perfusion defects can forego invasive angiography. In addition, combined anatomical and perfusion-based imaging may eventually offer a definitive evaluation for diagnosing CAD, even in higher risk patients. Any remarkable findings on single-photon emission computed tomography and PET MPI studies are valuable for prognostication. Furthermore, assessment of myocardial blood flow with PET is particularly powerful for prognostication as it reflects the end result of many processes that lead to atherosclerosis. Decision making with respect to revascularization is limited for cardiac MRI and PET MPI. In contrast, retrospective radionuclide studies have identified an ischemic threshold, but randomized trials are needed. In patients with at least moderately reduced left ventricular systolic function, viable myocardium as assessed by PET or MRI, appears to identify patients

  1. [The clinical analysis of fulminant Wilson's disease in patients with hepatitis B virus infection: a report of 13 cases].

    PubMed

    Deng, H H; Xu, M

    2016-08-01

    To analyze the clinical features and prognosis of fulminant Wilson's disease (FWD) in patients with hepatitis B virus (HBV) infection. Twenty-seven patients were enrolled in Guangzhou Eighth People's Hospital from 2005 to 2015, including 13 FWD patients with HBV infection and 14 FWD patients without HBV infection. Clinical efficacy and survival rate were evaluated. Baseline biochemical data in two groups were comparable(P>0.05), including total bilirubin, prothrombin activity, serum albumin, alpha fetal protein, alanine transaminase, ceruloplasmin and 24 hours urine copper .Treatment in FWD group with HBV infection was ineffective, including 9(9/13) deaths and 4(4/13) patients receiveing liver transplants. However, 7(7/14)cases in the other group did not response to the treatment, including 6(6/14)deaths and 1(1/14)patient receiving liver transplant. The prognosis in the two groups is significantly different(P=0.006), which is much worse in FWD patients with HBV infection.

  2. [The clinical analysis of fulminant Wilson's disease in patients with hepatitis B virus infection: a report of 13 cases].

    PubMed

    Deng, H H; Xu, M

    2016-08-01

    To analyze the clinical features and prognosis of fulminant Wilson's disease (FWD) in patients with hepatitis B virus (HBV) infection. Twenty-seven patients were enrolled in Guangzhou Eighth People's Hospital from 2005 to 2015, including 13 FWD patients with HBV infection and 14 FWD patients without HBV infection. Clinical efficacy and survival rate were evaluated. Baseline biochemical data in two groups were comparable(P>0.05), including total bilirubin, prothrombin activity, serum albumin, alpha fetal protein, alanine transaminase, ceruloplasmin and 24 hours urine copper .Treatment in FWD group with HBV infection was ineffective, including 9(9/13) deaths and 4(4/13) patients receiveing liver transplants. However, 7(7/14)cases in the other group did not response to the treatment, including 6(6/14)deaths and 1(1/14)patient receiving liver transplant. The prognosis in the two groups is significantly different(P=0.006), which is much worse in FWD patients with HBV infection. PMID:27480558

  3. Exome Sequencing of Cell-Free DNA from Metastatic Cancer Patients Identifies Clinically Actionable Mutations Distinct from Primary Disease.

    PubMed

    Butler, Timothy M; Johnson-Camacho, Katherine; Peto, Myron; Wang, Nicholas J; Macey, Tara A; Korkola, James E; Koppie, Theresa M; Corless, Christopher L; Gray, Joe W; Spellman, Paul T

    2015-01-01

    The identification of the molecular drivers of cancer by sequencing is the backbone of precision medicine and the basis of personalized therapy; however, biopsies of primary tumors provide only a snapshot of the evolution of the disease and may miss potential therapeutic targets, especially in the metastatic setting. A liquid biopsy, in the form of cell-free DNA (cfDNA) sequencing, has the potential to capture the inter- and intra-tumoral heterogeneity present in metastatic disease, and, through serial blood draws, track the evolution of the tumor genome. In order to determine the clinical utility of cfDNA sequencing we performed whole-exome sequencing on cfDNA and tumor DNA from two patients with metastatic disease; only minor modifications to our sequencing and analysis pipelines were required for sequencing and mutation calling of cfDNA. The first patient had metastatic sarcoma and 47 of 48 mutations present in the primary tumor were also found in the cell-free DNA. The second patient had metastatic breast cancer and sequencing identified an ESR1 mutation in the cfDNA and metastatic site, but not in the primary tumor. This likely explains tumor progression on Anastrozole. Significant heterogeneity between the primary and metastatic tumors, with cfDNA reflecting the metastases, suggested separation from the primary lesion early in tumor evolution. This is best illustrated by an activating PIK3CA mutation (H1047R) which was clonal in the primary tumor, but completely absent from either the metastasis or cfDNA. Here we show that cfDNA sequencing supplies clinically actionable information with minimal risks compared to metastatic biopsies. This study demonstrates the utility of whole-exome sequencing of cell-free DNA from patients with metastatic disease. cfDNA sequencing identified an ESR1 mutation, potentially explaining a patient's resistance to aromatase inhibition, and gave insight into how metastatic lesions differ from the primary tumor.

  4. Clinical management of Krabbe disease.

    PubMed

    Escolar, Maria L; West, Tara; Dallavecchia, Alessandra; Poe, Michele D; LaPoint, Kathleen

    2016-11-01

    Krabbe disease (KD) is a rare neurodegenerative disorder caused by mutations in the gene encoding the galactocerebrosidase enzyme. The early- and late-infantile subtypes, which are the most common forms of the disease, are rapidly progressive and lead to early death, whereas the later-onset types are clinically heterogeneous. The only disease-modifying treatment currently available is hematopoietic stem cell transplantation, which is effective only when performed early in the course of the disease. Because most patients with KD are diagnosed too late for treatment, primary care physicians are faced with the challenge of caring for a child with severe neurologic impairment. This Review describes presenting symptoms, diagnosis, and disease manifestations of KD and provides basic guidelines for its management. Symptomatic treatment and supportive care that address the unique requirements of these patients can greatly improve the quality of life of patients and their families. © 2016 Wiley Periodicals, Inc.

  5. Clinical management of Krabbe disease.

    PubMed

    Escolar, Maria L; West, Tara; Dallavecchia, Alessandra; Poe, Michele D; LaPoint, Kathleen

    2016-11-01

    Krabbe disease (KD) is a rare neurodegenerative disorder caused by mutations in the gene encoding the galactocerebrosidase enzyme. The early- and late-infantile subtypes, which are the most common forms of the disease, are rapidly progressive and lead to early death, whereas the later-onset types are clinically heterogeneous. The only disease-modifying treatment currently available is hematopoietic stem cell transplantation, which is effective only when performed early in the course of the disease. Because most patients with KD are diagnosed too late for treatment, primary care physicians are faced with the challenge of caring for a child with severe neurologic impairment. This Review describes presenting symptoms, diagnosis, and disease manifestations of KD and provides basic guidelines for its management. Symptomatic treatment and supportive care that address the unique requirements of these patients can greatly improve the quality of life of patients and their families. © 2016 Wiley Periodicals, Inc. PMID:27638597

  6. [ANMCO/GICR-IACPR/SICI-GISE Consensus document: Clinical management of patients with stable coronary artery disease].

    PubMed

    Riccio, Carmine; Gulizia, Michele Massimo; Colivicchi, Furio; Di Lenarda, Andrea; Musumeci, Giuseppe; Faggiano, Pompilio Massimo; Abrignani, Maurizio Giuseppe; Rossini, Roberta; Fattirolli, Francesco; Valente, Serafina; Mureddu, Gian Francesco; Temporelli, Pier Luigi; Olivari, Zoran; Amico, Antonio Francesco; Casolo, Giancarlo; Fresco, Claudio; Menozzi, Alberto; Nardi, Federico

    2016-01-01

    Stable coronary artery disease is of epidemiological importance. It is becoming increasingly common due to the longer life expectancy, being strictly related to age and to advances in diagnostic techniques and pharmacological and non-pharmacological interventions.Stable coronary artery disease encompasses a variety of clinical and anatomic presentations, making the identification of its clinical and anatomical features challenging. Therapeutic interventions should be defined on an individual basis according to the patient's risk profile. To this aim, management flow-charts have been reviewed based on sustainability and appropriateness derived from recent evidence. Special emphasis has been placed on non-pharmacological interventions, stressing the importance of lifestyle changes, including smoking cessation, regular physical activity and diet. Adherence to therapy as an emerging risk factor is also discussed.

  7. [ANMCO/GICR-IACPR/SICI-GISE Consensus document: Clinical management of patients with stable coronary artery disease].

    PubMed

    Riccio, Carmine; Gulizia, Michele Massimo; Colivicchi, Furio; Di Lenarda, Andrea; Musumeci, Giuseppe; Faggiano, Pompilio Massimo; Abrignani, Maurizio Giuseppe; Rossini, Roberta; Fattirolli, Francesco; Valente, Serafina; Mureddu, Gian Francesco; Temporelli, Pier Luigi; Olivari, Zoran; Amico, Antonio Francesco; Casolo, Giancarlo; Fresco, Claudio; Menozzi, Alberto; Nardi, Federico

    2016-01-01

    Stable coronary artery disease is of epidemiological importance. It is becoming increasingly common due to the longer life expectancy, being strictly related to age and to advances in diagnostic techniques and pharmacological and non-pharmacological interventions.Stable coronary artery disease encompasses a variety of clinical and anatomic presentations, making the identification of its clinical and anatomical features challenging. Therapeutic interventions should be defined on an individual basis according to the patient's risk profile. To this aim, management flow-charts have been reviewed based on sustainability and appropriateness derived from recent evidence. Special emphasis has been placed on non-pharmacological interventions, stressing the importance of lifestyle changes, including smoking cessation, regular physical activity and diet. Adherence to therapy as an emerging risk factor is also discussed. PMID:27571333

  8. Clinical Profiles, Disease Outcome and Co-Morbidities among T. b. rhodesiense Sleeping Sickness Patients in Uganda

    PubMed Central

    Kato, Charles D.; Nanteza, Ann; Mugasa, Claire; Edyelu, Andrew; Matovu, Enock; Alibu, Vincent P.

    2015-01-01

    Background The acute form of Human African Trypanosomiasis (HAT, also known as Sleeping sickness) caused by Trypanosoma brucei rhodesiense has been shown to have a wide spectrum of focus specific clinical presentation and severity in East and Southern Africa. Indeed HAT occurs in regions endemic for other tropical diseases, however data on how these co-morbidities might complicate the clinical picture and affect disease outcome remains largely scanty. We here describe the clinical presentation, presence of co-infections, and how the latter impact on HAT prognosis. Methods and Findings We carried out a retrospective analysis of clinical data from 258 sleeping sickness patients reporting to Lwala hospital between 2005 and 2012. The mean patient age was 28.6 years with a significant number of cases below 18 years (p< 0.0001). About 93.4% of the cases were diagnosed as late stage (p< 0.0001). The case fatality rate was 10.5% with post treatment reactive encephalopathys reported in 7.9% of the cases, of whom 36.8% eventually died. Fever was significantly (p = 0.045) higher in patients under 18 years. Of the early stage patients, 26.7% and 6.7% presented with late stage signs of sleep disorder and mental confusion respectively. Among the co-infections, malaria was significantly more prevalent (28.9%; p< 0.0001) followed by urinary tract infections (4.2%). Co-infections were present in 14.3% of in-hospital deaths, 38.5% of which were recorded as Malaria. Malaria was significantly more common in patients under 18 years (45.5%; p< 0.02), and was reported in 60% of the fatal cases in this age group. Conclusions We show a wide spectrum of sleeping sickness clinical presentation and disease outcome that was apparently not significantly influenced by concurrent infections. It would thus be interesting to determine the host and/or parasite factors that might be responsible for the observed diverse clinical presentation. PMID:25719539

  9. Clinical effectiveness of low-power laser radiation and functioning of hemosalivatory barrier in patients with rheumatic diseases

    NASA Astrophysics Data System (ADS)

    Gladkova, Natalia D.; Karachistov, Alexander B.; Komarova, Lia G.; Alekseeva, Olga P.; Grunina, Elena A.

    1996-11-01

    We have estimated the clinical effectiveness of several regimes and ways of low power laser therapy (LT) on the basis of a double 'blind', placebo-controlling randomizing comparative test in 454 patients with rheumatic diseases (RD). LT for RD has a well-expressed placebo effect. The level of clinical effect of LT for RD is not so high. We couldn't achieve 'a considerable improvement' in any cases, 'an improvement' was secured in only 18 percent. LT should be viewed as a symptomatic means, with a primary anesthetic and feebly expressed anti-inflammatory effect, which can not influence the course of the rheumatoid process. Only in 15 percent of patients with RD, a sufficient functioning of hemo-salivary barrier was observed, the latter providing a reserve for adaption mechanism, which leads under the influence of stressor agents of medium strength not only to anesthetic, but also to moderately expressed anti- inflammatory effect.

  10. Gender-Specific Differences in Clinical Profile and Biochemical Parameters in Patients with Cushing's Disease: A Single Center Experience

    PubMed Central

    Zhu, Xiaoming; Zeng, Meifang; Zhuang, Yan; Zhou, Yiting; Zhang, Zhaoyun; Yang, Yehong; Wang, Yongfei; Ye, Hongying; Li, Yiming

    2015-01-01

    Cushing's disease (CD) is remarkably prevalent among females; however, more severe clinical presentation and adverse outcomes have been found in males. The purpose of this study was to investigate the overall clinical profile and biochemical parameters in patients with CD to identify the gender differences. Here we describe our series of CD patients referred to our medical center during 2012-2013. Among 73 cases, females presented a marked preponderance compared to males. Males had significantly higher ACTH, BMI, HbA1c, systolic blood pressure, and hemoglobin than females. For the first time, the incidence of fatty liver and hepatic function was also shown to be elevated in males. Multiple linear regression analysis was performed to further investigate the correlation of risk factors with hypokalemia, HbA1c, and systolic blood pressure. Gender and serum cortisol were associated with hypokalemia. Age, gender, and serum cortisol were significantly associated with HbA1c. Additionally, only gender was significantly associated with systolic blood pressure. Regarding clinical presentation, purple striae seemed to occur more frequently in males than in females. Thus, more severe clinical presentation, biochemical parameters, and complications were found in males than in females. Clinical professionals should pay more attention to the diagnosis and management of males with CD. PMID:26064114

  11. Clinical research on erythrocyte deformability with different doses of He-Ne exposure in patient with ischemia disease

    NASA Astrophysics Data System (ADS)

    Zhao, Yanping; Liu, Song-hao; Sun, Jinbo; Luo, Gangyue; Hua, Rong; Liu, Qianqin

    2005-01-01

    The aim of this study was to test human erythrocyte deformability with the exposure of erythrocyte from apoplexy patient and other ischemia diseases, contracted with normal donors' blood sample, and the doses-effect of Low-power He_Ne laser in vitro were discussed. Fresh blood sample from adult health donors and patients with different diseases such as apoplexy, diabetes, heart block etc in emergency department were collected and divided into different groups in which there were no less than 6 persons. Fresh human blood samples were irradiated with a He-Ne laser (Lamba=632.8nm), power output around 4.5MW, 9MW, 15mW, and 18mW, et al., exposure time from 7.5min, 15min, and 30min, operating in continuous wave. Measurements of human erythrocyte deformability were taken. Erythrocyte deformability appearance shown some different in the health contracted group and the other ischemia disease group. Some notice difference also shown among some disease group with nonirradiation and the same disease group with laser irradiation. The dose-effects of He-Ne laser therapy was discussed on the further research on the erythrocyte deformability of blood sample from patients with apoplexy disease treated with He-Ne laser at different doses, and a certain optimal doses which could take a beneficial effect in clinic were speculated on. This study revealed that the He-Ne laser have some different effects on erythrocyte deformability in vitro, which were related with the disease condition, red cell state, and outpower-doses, et al closely.

  12. Thiopurine withdrawal during sustained clinical remission in inflammatory bowel disease: relapse and recapture rates, with predictive factors in 237 patients

    PubMed Central

    Kennedy, N A; Kalla, R; Warner, B; Gambles, C J; Musy, R; Reynolds, S; Dattani, R; Nayee, H; Felwick, R; Harris, R; Marriott, S; Senanayake, S M; Lamb, C A; Al-Hilou, H; Gaya, D R; Irving, P M; Mansfield, J; Parkes, M; Ahmad, T; Cummings, J R F; Arnott, I D; Satsangi, J; Lobo, A J; Smith, M; Lindsay, J O; Lees, C W

    2014-01-01

    Background Thiopurines (azathioprine and mercaptopurine) remain integral to most medical strategies for maintaining remission in Crohn's disease (CD) and ulcerative colitis (UC). Indefinite use of these drugs is tempered by long-term risks. While clinical relapse is noted frequently following drug withdrawal, there are few published data on predictive factors. Aim To investigate the success of planned thiopurine withdrawal in patients in sustained clinical remission to identify rates and predictors of relapse. Methods This was a multicentre retrospective cohort study from 11 centres across the UK. Patients included had a definitive diagnosis of IBD, continuous thiopurine use ≥3 years and withdrawal when in sustained clinical remission. All patients had a minimum of 12 months follow-up post drug withdrawal. Primary and secondary end points were relapse at 12 and 24 months respectively. Results 237 patients were included in the study (129 CD; 108 UC). Median duration of thiopurine use prior to withdrawal was 6.0 years (interquartile range 4.4–8.4). At follow-up, moderate/severe relapse was observed in 23% CD and 12% UC patients at 12 months, 39% CD and 26% UC at 24 months. Relapse rate at 12 months was significantly higher in CD than UC (P = 0.035). Elevated CRP at withdrawal was associated with higher relapse rates at 12 months for CD (P = 0.005), while an elevated white cell count was predictive at 12 months for UC (P = 0.007). Conclusion Thiopurine withdrawal in the context of sustained remission is associated with a 1-year moderate-to-severe relapse rate of 23% in Crohn's disease and 12% in ulcerative colitis. PMID:25284134

  13. Physician - nurse practitioner teams in chronic disease management: the impact on costs, clinical effectiveness, and patients' perception of care.

    PubMed

    Litaker, David; Mion, Lorraine; Planavsky, Loretta; Kippes, Christopher; Mehta, Neil; Frolkis, Joseph

    2003-08-01

    Increasing demand to deliver and document therapeutic and preventive care sharpens the need for disease management strategies that accomplish these goals efficiently while preserving quality of care. The purpose of this study was to compare selected outcomes for a new chronic disease management program involving a nurse practitioner - physician team with those of an existing model of care. One hundred fifty-seven patients with hypertension and diabetes mellitus were randomly assigned to their primary care physician and a nurse practitioner or their primary care physician alone. Costs for personnel directly involved in patient management, calculated from hourly rates and encounter time with patients, and pre- and post-study glycosylated hemoglobin (HbA(1c)), high-density lipoprotein cholesterol (HDL-c), satisfaction with care and health-related quality of life (HRQoL) were assessed. Although 1-year costs for personnel were higher in the team-treated group, participants experienced significant improvements in mean HbA(1c) ( - 0.7%, p = 0.02) and HDL-c ( + 2.6 mg dL( - 1), p = 0.02). Additionally, satisfaction with care improved significantly for team-treated subjects in several sub-scales whereas the mean change over time in HRQoL did not differ significantly between groups. This study demonstrates the value of a complementary team approach to chronic disease management in improving patient-derived and clinical outcomes at modest incremental costs.

  14. Non-familial degenerative disease and atrophy of brainstem and cerebellum. Clinical and CT data in 47 patients.

    PubMed

    Staal, A; Meerwaldt, J D; van Dongen, K J; Mulder, P G; Busch, H F

    1990-03-01

    We studied the clinical features of 47 patients with a non-hereditary degenerative disease and with atrophy of brainstem or cerebellum or both in CT scanning. There was no relation between the CT findings and duration or severity of the disease, nor with the kind of the neurological signs which comprised ataxia, a hypokinetic rigid syndrome, oculomotor abnormalities, upper and lower motor neuron signs, orthostatic hypotension and dementia. The 2 main diagnoses were olivopontocerebellar atrophy (OPCA), or a combination of OPCA and striatonigral degeneration (SND). The differential diagnosis with Parkinson's disease and progressive supranuclear palsy was discussed. We concluded, that a CT scan is warranted in all cases of suspected Parkinson's disease, especially in those without tremor, and in cases of motoneuron disease with broad-based gait. In our patients with mainly hypokinesia and rigidity, levodopa treatment had no or brief beneficial effects. If ataxia predominated, OPCA appeared the most sensible diagnosis; if a hypokinetic-rigid syndrome predominated, the diagnoses SND plus OPCA appeared the most suitable. We assessed the degree of atrophy on CT subjectively, because an interobserver study of 60 normal CT scans, did not produce reliable measurements. PMID:2358820

  15. Non-familial degenerative disease and atrophy of brainstem and cerebellum. Clinical and CT data in 47 patients.

    PubMed

    Staal, A; Meerwaldt, J D; van Dongen, K J; Mulder, P G; Busch, H F

    1990-03-01

    We studied the clinical features of 47 patients with a non-hereditary degenerative disease and with atrophy of brainstem or cerebellum or both in CT scanning. There was no relation between the CT findings and duration or severity of the disease, nor with the kind of the neurological signs which comprised ataxia, a hypokinetic rigid syndrome, oculomotor abnormalities, upper and lower motor neuron signs, orthostatic hypotension and dementia. The 2 main diagnoses were olivopontocerebellar atrophy (OPCA), or a combination of OPCA and striatonigral degeneration (SND). The differential diagnosis with Parkinson's disease and progressive supranuclear palsy was discussed. We concluded, that a CT scan is warranted in all cases of suspected Parkinson's disease, especially in those without tremor, and in cases of motoneuron disease with broad-based gait. In our patients with mainly hypokinesia and rigidity, levodopa treatment had no or brief beneficial effects. If ataxia predominated, OPCA appeared the most sensible diagnosis; if a hypokinetic-rigid syndrome predominated, the diagnoses SND plus OPCA appeared the most suitable. We assessed the degree of atrophy on CT subjectively, because an interobserver study of 60 normal CT scans, did not produce reliable measurements.

  16. The Use of Cardiac Magnetic Resonance in Patients with Suspected Coronary Artery Disease: A Clinical Practice Perspective

    PubMed Central

    Chang, Sung-A

    2016-01-01

    Cardiac magnetic resonance imaging (CMR) is a useful diagnostic imaging modality in patients with known or suspected coronary artery disease (CAD). It provides unique information not available from other modalities, however, it is complex. CMR is not a single technique. Instead, it consists of multiple distinct techniques and a lack of understanding of which techniques to perform and how to interpret the findings in combination limits its efficacy and widespread use. Conversely, its multiparametric nature can provide a comprehensive assessment with the potential for higher accuracy than is achievable by other modalities. Moreover, its ability to directly assess myopathic processes often contributes insights that change patient management. In this article we provide a brief technical overview and focus on specific clinical scenarios in patients with known or suspected CAD. We highlight the multiparametric nature of CMR and discuss cases which illustrate the unique information that CMR can contribute. PMID:27358697

  17. Association of Ureaplasma urealyticum biovars with clinical outcome for neonates, obstetric patients, and gynecological patients with pelvic inflammatory disease.

    PubMed Central

    Abele-Horn, M; Wolff, C; Dressel, P; Pfaff, F; Zimmermann, A

    1997-01-01

    In this prospective study, the prevalence of the two Ureaplasma urealyticum biovars, parvo and T960, was determined in pregnant women and in gynecological patients colonized by ureaplasmas. Furthermore, we investigated the association of these biovars with gynecological complications and adverse pregnancy outcome. Isolates of U. urealyticum from 254 women were biotyped by a PCR method recently developed. The parvo biovar was found in 81% (206 of 254) of the patients, and the T960 biovar was found in 30% (76 of 254) of the patients; 6% (14 of 254) of the women were coinfected. Identical biovars were detected in mothers and their infants. Serial isolations or cultures from different sampling sites of the same individual revealed the same biovar. T960 was dominant in patients with pelvic inflammatory disease (57%) and patients who had had a miscarriage (42%), showed a higher rate of tetracycline resistance than did parvo isolates (55 versus 18%), and seemed to have more adverse effects on pregnancy outcome with regard to birth weight (2,500 versus 1,720 g), gestational age (35 versus 30 weeks), and preterm delivery (35 versus 77%). PMID:9114407

  18. Clinical utility of PKD2 mutation testing in a polycystic kidney disease cohort attending a specialist nephrology out-patient clinic

    PubMed Central

    2012-01-01

    Background ADPKD affects approximately 1:1000 of the worldwide population. It is caused by mutations in two genes, PKD1 and PKD2. Although allelic variation has some influence on disease severity, genic effects are strong, with PKD2 mutations predicting later onset of ESRF by up to 20 years. We therefore screened a cohort of ADPKD patients attending a nephrology out-patient clinic for PKD2 mutations, to identify factors that can be used to offer targeted gene testing and to provide patients with improved prognostic information. Methods 142 consecutive individuals presenting to a hospital nephrology out-patient service with a diagnosis of ADPKD and CKD stage 4 or less were screened for mutations in PKD2, following clinical evaluation and provision of a detailed family history (FH). Results PKD2 mutations were identified in one fifth of cases. 12% of non-PKD2 patients progressed to ESRF during this study whilst none with a PKD2 mutation did (median 38.5 months of follow-up, range 16–88 months, p < 0.03). A significant difference was found in age at ESRF of affected family members (non-PKD2 vs. PKD2, 54 yrs vs. 65 yrs; p < 0.0001). No PKD2 mutations were identified in patients with a FH of ESRF occurring before age 50 yrs, whereas a PKD2 mutation was predicted by a positive FH without ESRF. Conclusions PKD2 testing has a clinically significant detection rate in the pre-ESRF population. It did not accurately distinguish those individuals with milder renal disease defined by stage of CKD but did identify a group less likely to progress to ESRF. When used with detailed FH, it offers useful prognostic information for individuals and their families. It can therefore be offered to all but those whose relatives have developed ESRF before age 50. PMID:22863349

  19. Toward comprehensive management tailored to prognostic factors of patients with clinical stages I and II in Hodgkin's disease. The EORTC Lymphoma Group controlled clinical trials: 1964-1987.

    PubMed

    Tubiana, M; Henry-Amar, M; Carde, P; Burgers, J M; Hayat, M; Van der Schueren, E; Noordijk, E M; Tanguy, A; Meerwaldt, J H; Thomas, J

    1989-01-01

    From 1964 to 1987, the EORTC Lymphoma Group conducted four consecutive controlled clinical trials on clinical stages I and II Hodgkin's disease in which 1,579 patients were entered. From the onset the main aim of these trials was to identify the subsets of patients who could be treated safely by regional radiotherapy (RT). Therefore, several prognostic indicators were prospectively registered and progressively used in the trial protocols for the delineation of the favorable and unfavorable subgroups as soon as they were recognized of high predictive value. In the H2 trial (1972 to 1976), the histologic subtype was the only variable taken into account for the therapeutic strategy and the staging laparotomy findings were found to be of prognostic value only in patients with favorable prognostic indicators. In the H5 trial (1977 to 1982), patients were subdivided into two subgroups according to six prognostic indicators. Patients with favorable features were submitted to a staging laparotomy (lap); lap negative patients were randomized between mantle field RT and mantle field plus paraaortic RT. Disease free survival (DFS) and total survival (S) were similar in the two arms. Among patients with unfavorable features, DFS and S were significantly higher in the arm treated by combination of mechlorethamine, vincristine, procarbazine, prednisone (MOPP) chemotherapy (CT) and RT than in the arm treated by total nodal irradiation. Nevertheless, in patients below the age of 40, the overall survival rates were equivalent in the two arms. In the H6 trial, the delineation of the favorable subgroup was based on (a) absence of systemic symptoms and elevated ESR, (b) no more than one or two lymph node areas involved. The aim of the study was to assess the impact on survival of a therapeutic strategy including staging laparotomy. At a 4-year follow-up, no difference in survival was evidenced. In patients with unfavorable prognostic indicators, 3 MOPP-RT-3 MOPP were compared with 3

  20. A serological, parasitological and clinical evaluation of untreated Chagas disease patients and those treated with benznidazole before and thirteen years after intervention

    PubMed Central

    Machado-de-Assis, Girley Francisco; Diniz, Glaucia Alessio; Montoya, Roberto Araújo; Dias, João Carlos Pinto; Coura, José Rodrigues; Machado-Coelho, George Luiz Lins; Albajar-Viñas, Pedro; Torres, Rosália Morais; de Lana, Marta

    2013-01-01

    The etiological treatment of Chagas disease is recommended for all patients with acute or recent chronic infection, but controversies remain regarding the benefit of chemotherapy and interpretations of the parasitological cure after etiological treatment. This study compares the laboratory and clinical evaluations of Chagas disease patients who were diagnosed 13 years earlier. Fifty-eight Chagas disease patients (29 treated with benznidazole and 29 untreated) were matched at the time of treatment based on several variables. Conventional serology revealed the absence of seroconversion in all patients. However, lower serological titres were verified in the treated group, primarily among patients who had the indeterminate form of the disease. Haemoculture performed 13 years after the intervention was positive for 6.9% and 27.6% of the treated and untreated patients, respectively. Polymerase chain reaction tests were positive for 44.8% and 13.8% of the treated and untreated patients, respectively. Patients who presented with the indeterminate form of the disease at the beginning of the study exhibited less clinical progression (17.4%) compared with the untreated group (56.5%). Therefore, this global analysis revealed that etiological treatment with benznidazole may benefit patients with respect to the clinical progression of Chagas disease and the prognosis, particularly when administered to patients with the indeterminate form of the disease. PMID:24037109

  1. Prediction of individual clinical scores in patients with Parkinson's disease using resting-state functional magnetic resonance imaging.

    PubMed

    Hou, YanBing; Luo, ChunYan; Yang, Jing; Ou, RuWei; Song, Wei; Wei, QianQian; Cao, Bei; Zhao, Bi; Wu, Ying; Shang, Hui-Fang; Gong, QiYong

    2016-07-15

    Neuroimaging holds the promise that it may one day aid the clinical assessment. However, the vast majority of studies using resting-state functional magnetic resonance imaging (fMRI) have reported average differences between Parkinson's disease (PD) patients and healthy controls, which do not permit inferences at the level of individuals. This study was to develop a model for the prediction of PD illness severity ratings from individual fMRI brain scan. The resting-state fMRI scans were obtained from 84 patients with PD and the Unified Parkinson's Disease Rating Scale-III (UPDRS-III) scores were obtained before scanning. The RVR method was used to predict clinical scores (UPDRS-III) from fMRI scans. The application of RVR to whole-brain resting-state fMRI data allowed prediction of UPDRS-III scores with statistically significant accuracy (correlation=0.35, P-value=0.001; mean sum of squares=222.17, P-value=0.002). This prediction was informed strongly by negative weight areas including prefrontal lobe and medial occipital lobe, and positive weight areas including medial parietal lobe. It was suggested that fMRI scans contained sufficient information about neurobiological change in patients with PD to permit accurate prediction about illness severity, on an individual subject basis. Our results provided preliminary evidence, as proof-of-concept, to support that fMRI might be possible to be a clinically useful quantitative assessment aid in PD at individual level. This may enable clinicians to target those uncooperative patients and machines to replace human for a more efficient use of health care resources. PMID:27288771

  2. The diagnostic value of minor salivary gland biopsy in clinically diagnosed patients with Parkinson's disease: comparison with DAT PET scans.

    PubMed

    Gao, Liyan; Chen, Huimin; Li, Xin; Li, Fangfei; Ou-Yang, Qiaohong; Feng, Tao

    2015-09-01

    To investigate the predictive value of minor salivary gland biopsy in clinically diagnosed early stage Parkinson's disease (PD) patients, and to provide more evidence of minor salivary gland biopsy as a pathological diagnostic biomarker of PD. Thirteen patients with early stage PD and 13 age-matched controls were recruited. Hoehn and Yahr stage and Unified Parkinson's disease Rating Scale Part III were employed to evaluate their severity of the disease. All the subjects underwent minor salivary gland biopsy and (11)C-methyl-N-2β-carbomethoxy-3β-(4-fluorophenyl) tropane ((11)C-CFT) DAT-PET scan. Immunohistochemical staining for Lewy-type alpha-synucleinopathy using antibody against alpha-synuclein (α-Syn) was performed in the tissues obtained from minor salivary gland. Abnormal accumulation of α-Syn was found around the gland cells in 9 of the 13 patients with PD, but in none of the control subjects. The α-Syn immunoreactive structures were located in the periacinar space. Twelve clinically diagnosed PD patients showed asymmetrical and relatively severe reduction of (11)C-CFT uptake in the posterior putamen compared with the control. The sensitivity, specificity, positive predictive value and negative predictive value of minor salivary gland biopsy were 75, 100, 100 and 25%, respectively, when compared with the DAT-PET imaging. Our results suggest that minor salivary gland biopsy does not hold high diagnostic accuracy as DAT-PET, but still has the potential to be a useful pathologic biomarker for PD, which is worth more investigations.

  3. [Clinical symptoms of Alzheimer disease].

    PubMed

    Tariska, P; Urbanics, K; Knolmayer, J; Mészáros, A

    1995-04-23

    Data of patients suffering from Alzheimer's disease and checked out in the special unit named Memory Clinic functioning from 1992 in the National Institute of Psychiatry and Neurology are summarized. Age average of the 60 patients was 63 years, the first symptoms of the disease had appeared in 57 p.c. before the age of 65, so the classical presenile form of the ailment is represented too in the material. Predominance of multifocal cortical function disturbances in the symptomatology is characteristic, association of the depression is outstandingly frequent. The atypical features, or those characteristic in diseases of cerebrovascular origin are not infrequently seen (headache, dizziness, slight symptoms of pyramidal lesions). The absence of epileptic seizures It was interesting even in considering the data of the literature too. The main points of clinical diagnostics and differential diagnostics are demonstrated with the aid of case reports. The author's material is the first Hungarian publication in the topics of clinical symptoms of patients suffering from Alzheimer's disease that had been investigated with up-to-date methods. Occurrence of the disease of very great frequency could be supposed to occur at general practitioners, the importance of differential diagnostics and planning of the complex longlasting therapy is extremely great.

  4. Managing hyperphosphatemia in patients with chronic kidney disease on dialysis with ferric citrate: latest evidence and clinical usefulness

    PubMed Central

    Fadem, Stephen Z.; Kant, Kotagal S.; Bhatt, Udayan; Sika, Mohammed; Lewis, Julia B.; Negoi, Dana

    2015-01-01

    Ferric citrate is a novel phosphate binder that allows the simultaneous treatment of hyperphosphatemia and iron deficiency in patients being treated for end-stage renal disease with hemodialysis (HD). Multiple clinical trials in HD patients have uniformly and consistently demonstrated the efficacy of the drug in controlling hyperphosphatemia with a good safety profile, leading the US Food and Drug Administration in 2014 to approve its use for that indication. A concurrent beneficial effect, while using ferric citrate as a phosphate binder, is its salutary effect in HD patients with iron deficiency being treated with an erythropoietin-stimulating agent (ESA) in restoring iron that becomes available for reversing chronic kidney disease (CKD)-related anemia. Ferric citrate has also been shown in several studies to diminish the need for intravenous iron treatment and to reduce the requirement for ESA. Ferric citrate is thus a preferred phosphate binder that helps resolve CKD-related mineral bone disease and iron-deficiency anemia. PMID:26336594

  5. Managing hyperphosphatemia in patients with chronic kidney disease on dialysis with ferric citrate: latest evidence and clinical usefulness.

    PubMed

    Yagil, Yoram; Fadem, Stephen Z; Kant, Kotagal S; Bhatt, Udayan; Sika, Mohammed; Lewis, Julia B; Negoi, Dana

    2015-09-01

    Ferric citrate is a novel phosphate binder that allows the simultaneous treatment of hyperphosphatemia and iron deficiency in patients being treated for end-stage renal disease with hemodialysis (HD). Multiple clinical trials in HD patients have uniformly and consistently demonstrated the efficacy of the drug in controlling hyperphosphatemia with a good safety profile, leading the US Food and Drug Administration in 2014 to approve its use for that indication. A concurrent beneficial effect, while using ferric citrate as a phosphate binder, is its salutary effect in HD patients with iron deficiency being treated with an erythropoietin-stimulating agent (ESA) in restoring iron that becomes available for reversing chronic kidney disease (CKD)-related anemia. Ferric citrate has also been shown in several studies to diminish the need for intravenous iron treatment and to reduce the requirement for ESA. Ferric citrate is thus a preferred phosphate binder that helps resolve CKD-related mineral bone disease and iron-deficiency anemia. PMID:26336594

  6. The clinical usefulness of a web-based messaging system between patients with Crohn disease and their physicians

    PubMed Central

    Jeong, Da Eun; Kim, Kyeong Ok; Jang, Byung Ik; Kim, Eun Young; Jung, Jin Tae; Jeon, Seong Woo; Lee, Hyun Seok; Kim, Eun Soo; Park, Kyung Sik; Cho, Kwang Bum

    2016-01-01

    Abstract To avoid missing events associated with clinical activity, the authors previously developed a novel, web-based, self-reporting Crohn disease (CD) symptom diary. However, although this diary provided a means of self-checking based on responses to set questions based on Harvey–Bradshaw index scores, it was limited in terms of describing other specific symptoms. Thus, the authors added a space to the questionnaire, which allows patients to send clinicians questions or a description of unpredictable events. The aim of the present study was to assess the clinical usefulness of this messaging system by analyzing patients’ messages. The messaging system between patients and their doctors was included in a webpage created for recording patients’ symptom diaries (www.cdsd.or.kr). Using this system, patients can send messages easily at any time and doctors can read and respond to these messages immediately using a smart phone or computer. In the present study, the authors retrospectively reviewed 686 messages sent by 152 patients from July 2012 to July 2014 and patient medical records. Mean patient age was 29.0 ± 11.6 years and the male-to-female ratio was 99:53. Most messages regarded symptoms (381 messages, 55.5%), which was followed by self-reports about general condition (195 messages, 28.4%) and questions about treatment (71 messages, 10.3%). With respect to symptoms, abdominal pain was most common (145 cases, 21.1%) followed by hematochezia (36 cases, 5.2%). Problems about medication were the most frequently associated with treatment (65, 91.5%). Patients above 40 years showed a greater tendency to focus on symptoms and treatment (P = 0.025). The doctor answer rate was 56.3% (n = 386), and based on these responses, an early visit was needed in 28 cases (7.3%). Using this web-based messaging system, patients were able to obtain proper advice from their physicians without visiting clinics or searching the Internet, and in addition, 7.3% of messages

  7. The Connecticut Cardiovascular Consortium: a unique, state-wide research collaboration to advance clinical outcomes in patients with heart disease.

    PubMed

    Boden, W E; McKay, R G; Cabin, H S; Radford, M J; Krumholz, H M; Zaret, B L; Garner, L; Bull, M B; Fisherkeller, M; Kosinski, E J; Krauthamer, M J; Maljanian, R; McDowell, A V; Sands, M J; Schwartz, K V; Seltzer, J P; Hager, J D

    2001-10-01

    The establishment of "best clinical practices" founded upon evidence-based medicine has become an increasingly important priority. Frequently, management guidelines are derived from published research data and disseminated among practitioners to help optimize patient care. The ultimate clinical impact of these guidelines in the "real world," however, is often clouded by an incomplete assessment of patient outcomes throughout the continuum of health-care delivery models. In order to address this gap in clinical outcome assessment, we propose to establish the Connecticut Cardiovascular Consortium. The Consortium will consist of a collaborative partnership among all 31 Connecticut hospitals working in concert with Connecticut Office of Health Care Access (OHCA). The primary objective of the Consortium will be to assess, compare, and optimize clinical outcomes among Connecticut residents with cardiovascular disease. As an initial goal for the Consortium, we further propose to undertake a prospective, observational study of Connecticut residents who present with ST Segment Elevation Acute Myocardial Infarction (STEMI). Recent advances in pharmacologic and mechanical reperfusion for STEMI have resulted in a need to define the optimal use of these therapies in the community at large. The primary purpose of this study will be to determine the relative merits of different treatment patterns for STEMI with regard to the use of fibrinolytic therapy and percutaneous coronary intervention (PCI). Particular emphasis will be placed on assessing the relative benefits of urgent mechanical revascularization performed at the state's seven tertiary facilities with PCI capability compared to all other treatment modalities. Successful completion of this unique collaborative endeavor is expected to have significant impact on improved patient care and on current health-care policy for medical resource allocation. Moreover, continued collaboration of health-care providers within the

  8. [Characteristics of patients with chronic kidney disease referred to a nephrology outpatient clinic: results of Nefrodata study].

    PubMed

    Cupisti, Adamasco; Bottai, Anna; Bellizzi, Vincenzo; Brunori, Giuliano; Cianciaruso, Bruno; De Nicola, Luca; Oldrizzi, Lamberto; Quintaliani, Giuseppe; Santoro, Domenico; Di Iorio, Biagio Raffaele

    2015-01-01

    Chronic kidney disease is acknowledged as one of the most relevant disease for public health. Knowledge of epidemiology of CKD may allow public health interventions both for prevention and treatment in order to limit burden and management costs. Nefrodata is a multicentric, prospective, and observational study conducted in Italy, including patients with CKD followed in a specialist setting. The study uses a web-based data setting; it includes 1263 subjects with an estimate glomerular filtration rate (eGFR) less than 60 ml/min *1,73 sqm, followed in outpatient clinics in Italy. Patients' characteristics analysis evidences that old subjects (mean age of 70.3 13.4 years, 55% of them older than 70 years), with cardiovascular morbidity (50,6%) and diabetics (37%) have a high prevalence. With the reduction of residual renal function, prevalence of hyperphospatemia, metabolic acidosis, use of erythropoiesis-stimulating agents, Vitamin D, and diuretics increases. Also allopurinol and gastric-protective drugs are widely used. Fifty-four and eight % of patients with CKD stage 4 and 65.9% of patients with CKD stage 5 received indication on nutritional therapy. PMID:26005945

  9. Prevalence of Peripheral Arterial Disease among Adult Patients Attending Outpatient Clinic at a General Hospital in South Angola.

    PubMed

    Paquissi, Feliciano Chanana; Cuvinje, Arminda Bimbi Paquissi; Cuvinje, Almeida Bailundo

    2016-01-01

    Background. Peripheral arterial disease (PAD) is a common manifestation of atherosclerosis, whose prevalence is increasing worldwide, and is associated with all-cause mortality. However, no study has assessed this disease in Huambo. The aim of this study was to evaluate the prevalence of PAD in patients attending an outpatient clinic at a general hospital in Huambo, South Angola. Methods. A cross-sectional study, including 115 patients aged 40 years and older attending an outpatient service. The evaluation included a basic questionnaire for lifestyle and medical history and ankle-brachial index (ABI) measurement using hand-held Doppler. PAD was defined as an ABI ≤0.9 in either lower limb. Results. Of 115 patients, 62.60% were women with a median age of 52.5 (range of 40 to 91) years. The prevalence of PAD was 42.6% (95% confidence intervals [CI]: 95%: 33.91-52.17%). Among patients with PAD, 95.92% had mild disease and 4.08% moderate to severe disease. The main risk factor for PAD was age (≥60 years) (χ (2) = 3.917, P ≤ 0.05). The prevalence was slightly higher in men and hypertensive subjects, but without statistical significance with ORs of 1.5 (95% CI: 0.69-3.21) and 1.42 (95% CI: 0.64-3.17), respectively. Hypertension was also high in the group (66.95%). Conclusion. The prevalence of PAD was 42.6%, higher in those aged 60 years and older. More studies, with representative samples, are necessary to clarify PAD prevalence and associated risk factors. PMID:27293966

  10. Prevalence of Peripheral Arterial Disease among Adult Patients Attending Outpatient Clinic at a General Hospital in South Angola

    PubMed Central

    Paquissi, Feliciano Chanana; Cuvinje, Arminda Bimbi Paquissi; Cuvinje, Almeida Bailundo

    2016-01-01

    Background. Peripheral arterial disease (PAD) is a common manifestation of atherosclerosis, whose prevalence is increasing worldwide, and is associated with all-cause mortality. However, no study has assessed this disease in Huambo. The aim of this study was to evaluate the prevalence of PAD in patients attending an outpatient clinic at a general hospital in Huambo, South Angola. Methods. A cross-sectional study, including 115 patients aged 40 years and older attending an outpatient service. The evaluation included a basic questionnaire for lifestyle and medical history and ankle-brachial index (ABI) measurement using hand-held Doppler. PAD was defined as an ABI ≤0.9 in either lower limb. Results. Of 115 patients, 62.60% were women with a median age of 52.5 (range of 40 to 91) years. The prevalence of PAD was 42.6% (95% confidence intervals [CI]: 95%: 33.91–52.17%). Among patients with PAD, 95.92% had mild disease and 4.08% moderate to severe disease. The main risk factor for PAD was age (≥60 years) (χ2 = 3.917, P ≤ 0.05). The prevalence was slightly higher in men and hypertensive subjects, but without statistical significance with ORs of 1.5 (95% CI: 0.69–3.21) and 1.42 (95% CI: 0.64–3.17), respectively. Hypertension was also high in the group (66.95%). Conclusion. The prevalence of PAD was 42.6%, higher in those aged 60 years and older. More studies, with representative samples, are necessary to clarify PAD prevalence and associated risk factors. PMID:27293966

  11. Velaglucerase alfa (VPRIV) enzyme replacement therapy in patients with Gaucher disease: Long-term data from phase III clinical trials

    PubMed Central

    Hughes, Derralynn A; Gonzalez, Derlis E; Lukina, Elena A; Mehta, Atul; Kabra, Madhulika; Elstein, Deborah; Kisinovsky, Isaac; Giraldo, Pilar; Bavdekar, Ashish; Hangartner, Thomas N; Wang, Nan; Crombez, Eric; Zimran, Ari

    2015-01-01

    Type 1 Gaucher disease is an inherited lysosomal enzyme deficiency with variable age of symptom onset. Common presenting signs include thrombocytopenia, anemia, hepatosplenomegaly, bone abnormalities, and, additionally in children, growth failure. Fifty-seven patients aged 3–62 years at the baseline of two phase III trials for velaglucerase alfa treatment were enrolled in the single extension study. In the extension, they received every-other-week velaglucerase alfa intravenous infusions for 1.2–4.8 years at 60 U/kg, although 10 patients experienced dose reduction. No patient experienced a drug-related serious adverse event or withdrew due to an adverse event. One patient died following a convulsion that was reported as unrelated to the study drug. Only one patient tested positive for anti-velaglucerase alfa antibodies. Combining the experience of the initial phase III trials and the extension study, significant improvements were observed in the first 24 months from baseline in hematology variables, organ volumes, plasma biomarkers, and, in adults, the lumbar spine bone mineral density Z-score. Improvements were maintained over longer-term treatment. Velaglucerase alfa had a good long-term safety and tolerability profile, and patients continued to respond clinically, which is consistent with the results of the extension study to the phase I/II trial of velaglucerase alfa. EudraCT number 2008-001965-27; http://www.clinicaltrials.gov identifier NCT00635427. Am. J. Hematol. 90:584–591, 2015. © 2015 Wiley Periodicals, Inc. PMID:25801797

  12. Use of antiarrhythmic drug therapy and clinical outcomes in older patients with concomitant atrial fibrillation and coronary artery disease

    PubMed Central

    Steinberg, Benjamin A.; Broderick, Samuel H.; Lopes, Renato D.; Shaw, Linda K.; Thomas, Kevin L.; DeWald, Tracy A.; Daubert, James P.; Peterson, Eric D.; Granger, Christopher B.; Piccini, Jonathan P.

    2014-01-01

    Aims Atrial fibrillation (AF) and coronary artery disease (CAD) are common in older patients. We aimed to describe the use of antiarrhythmic drug (AAD) therapy and clinical outcomes in these patients. Methods and results We analysed AAD therapy and outcomes in 1738 older patients (age ≥65) with AF and CAD in the Duke Databank for cardiovascular disease. The primary outcomes were mortality and rehospitalization at 1 and 5 years. Overall, 35% of patients received an AAD at baseline, 43% were female and 85% were white. Prior myocardial infarction (MI, 31%) and heart failure (41%) were common. Amiodarone was the most common AAD (21%), followed by pure Class III agents (sotalol 6.3%, dofetilide 2.2%). Persistence of AAD was low (35% at 1 year). After adjustment, baseline AAD use was not associated with 1-year mortality [adjusted hazard ratio (HR) 1.23, 95% confidence interval (CI) 0.94–1.60] or cardiovascular mortality (adjusted HR 1.27, 95% CI 0.90–1.80). However, AAD use was associated with increased all-cause rehospitalization (adjusted HR 1.20, 95% CI 1.03–1.39) and cardiovascular rehospitalization (adjusted HR 1.20, 95% CI 1.01–1.43) at 1 year. This association did not persist at 5 years; however, these patients were at very high risk of death (55% for those >75 and on AAD) and all-cause rehospitalization (87% for those >75 and on AAD) at 5 years. Conclusions In older patients with AF and CAD, antiarrhythmic therapy was associated with increased rehospitalization at 1 year. Overall, these patients are at high risk of longer-term hospitalization and death. Safer, better-tolerated, and more effective therapies for symptom control in this high-risk population are warranted. PMID:24755440

  13. Nutrition Screening Tools and the Prediction of Clinical Outcomes among Chinese Hospitalized Gastrointestinal Disease Patients

    PubMed Central

    Wang, Fang; Chen, Wei; Bruening, Kay Stearns; Raj, Sudha

    2016-01-01

    Nutrition risk Screening 2002 (NRS-2002) and Subjective Global Assessment (SGA) are widely used screening tools but have not been compared in a Chinese population. We conducted secondary data analysis of a cross-sectional study which included 332 hospitalized gastrointestinal disease patients, collected by the Gastrointestinal department of Peking Union Medical College Hospital (PUMCH) in 2008. Results of NRS-2002 and SGA screening tools, complications, length of stay (LOS), cost, and death were measured. The agreement between the tools was assessed via Kappa (κ) statistics. The performance of NRS-2002 and SGA in predicting LOS and cost was assessed via linear regression. The complications and death prediction of tools was assessed using receiver operating characteristic (ROC) curves. NRS-2002 and SGA identified nutrition risk at 59.0% and 45.2% respectively. Moderate agreement (κ >0.50) between the two tools was found among all age groups except individuals aged ≤ 20, which only slight agreement was found (κ = 0.087). NRS-2002 (R square 0.130) and SGA (R square 0.140) did not perform differently in LOS prediction. The cost prediction of NRS-2002 (R square 0.198) and SGA (R square 0.190) were not significantly different. There was no difference between NRS-2002 (infectious complications: area under ROC (AUROC) = 0.615, death: AUROC = 0.810) and SGA (infectious complications: AUROC = 0.600, death: AUROC = 0.846) in predicting infectious complication and death, but NRS-2002 (0.738) seemed to perform better than SGA (0.552) in predicting non-infectious complications. The risk of malnutrition among patients was high. NRS-2002 and SGA have similar capacity to predict LOS, cost, infectious complications and death, but NRS-2002 performed better in predicting non-infectious complications. PMID:27490480

  14. Nutrition Screening Tools and the Prediction of Clinical Outcomes among Chinese Hospitalized Gastrointestinal Disease Patients.

    PubMed

    Wang, Fang; Chen, Wei; Bruening, Kay Stearns; Raj, Sudha; Larsen, David A

    2016-01-01

    Nutrition risk Screening 2002 (NRS-2002) and Subjective Global Assessment (SGA) are widely used screening tools but have not been compared in a Chinese population. We conducted secondary data analysis of a cross-sectional study which included 332 hospitalized gastrointestinal disease patients, collected by the Gastrointestinal department of Peking Union Medical College Hospital (PUMCH) in 2008. Results of NRS-2002 and SGA screening tools, complications, length of stay (LOS), cost, and death were measured. The agreement between the tools was assessed via Kappa (κ) statistics. The performance of NRS-2002 and SGA in predicting LOS and cost was assessed via linear regression. The complications and death prediction of tools was assessed using receiver operating characteristic (ROC) curves. NRS-2002 and SGA identified nutrition risk at 59.0% and 45.2% respectively. Moderate agreement (κ >0.50) between the two tools was found among all age groups except individuals aged ≤ 20, which only slight agreement was found (κ = 0.087). NRS-2002 (R square 0.130) and SGA (R square 0.140) did not perform differently in LOS prediction. The cost prediction of NRS-2002 (R square 0.198) and SGA (R square 0.190) were not significantly different. There was no difference between NRS-2002 (infectious complications: area under ROC (AUROC) = 0.615, death: AUROC = 0.810) and SGA (infectious complications: AUROC = 0.600, death: AUROC = 0.846) in predicting infectious complication and death, but NRS-2002 (0.738) seemed to perform better than SGA (0.552) in predicting non-infectious complications. The risk of malnutrition among patients was high. NRS-2002 and SGA have similar capacity to predict LOS, cost, infectious complications and death, but NRS-2002 performed better in predicting non-infectious complications. PMID:27490480

  15. Prediction of Angiographic Extent of Coronary Artery Disease on the Basis of Clinical Risk Scores in Patients of Unstable Angina

    PubMed Central

    Jain, Anoop; Rathore, Monika

    2015-01-01

    Background The correlation of clinical risk predictors and clinical risk scores: Thrombolysis in Myocardial Infarction (TIMI), Platelet Glycoprotein IIb-IIIa in Unstable Angina, Receptor Suppression Using Integrilin Therapy (PURSUIT) and Global Registry of Acute Coronary Events (GRACE) scores in Unstable Angina with angiographic extent of Coronary Artery Disease (CAD) is not known. Aim To know the correlation of clinical risk scores with angiographic extent of coronary artery disease. Materials and Methods This was a hospital based single centre, cross-sectional, observational, descriptive study conducted at a tertiary care teaching institute. One hundred and sixty patients with acute unstable angina were evaluated for presence of 9 clinical predictors and their 3 risk scores were calculated. All patients underwent coronary angiography. Correlation with Modified Gensini score and percentage stenosis in culprit artery was done. Statistical Analysis Data were summarized in the form of Mean, Standard Deviation and Proportions. Multiple linear regressions, Student’s t-test and Pearson’s coefficient ‘r’ were also used. Results Use of aspirin, age >= 65 years & presence of Congestive Heart Failure (CHF) were stronger predictors of Modified Gensini score. Presence of elevated enzymes and age >65 years were more significant predictors of percentage stenosis of culprit artery. GRACE score had better correlation with Modified Gensini score, PURSUIT score had more correlation with percentage stenosis in culprit artery. Conclusion Use of Aspirin, age >= 65 years, presence of CHF and presence of elevated enzymes are stronger predictors of extent of CAD. Hence we recommend that these factors be given more importance. GRACE and PURSUIT risk scores had more correlation with angiographic extent of CAD. PMID:26672410

  16. Interplay between patient global assessment, pain, and fatigue and influence of other clinical disease activity measures in patients with active rheumatoid arthritis.

    PubMed

    Egsmose, Emilie Lund; Madsen, Ole Rintek

    2015-07-01

    The interplay between patient-reported outcome measures in rheumatology is not well clarified. The objective of the study was to examine associations on the group level and concordance on the individual patient level between patient global assessment (PaGl), pain, and fatigue as scored on visual analog scales (VAS) in the daily clinic by patients with active rheumatoid arthritis (RA). Associations with other measures of disease activity were also examined. Traditional disease activity data on 221 RA patients with active disease planned to initiate biological treatment were extracted from the Danish DANBIO registry. Associations between VAS PaGl, pain, and fatigue (0-100) were examined using multiple regression analysis. Concordance between the VAS scores was expressed as the bias (mean difference between intra-individual scores) and the 95% lower and upper limits of agreement (LLoA; ULoA) according to the Bland-Altman method. Mean age was 57 ± 14 years, mean Disease Activity Score (DAS28-CRP4) 5.0 ± 0.9, and mean PaGl 63.6 ± 22.6. PaGl was most strongly predicted by pain and fatigue, pain by PaGl and fatigue, and fatigue by PaGl and pain (beta ranging from 0.17 to 0.69, p < 0.01-0.0001). More objective measures were not or far less predictive. LLoA;ULoA [bias] for PaGl vs. pain was -19.1; 29.5 [5.2], for PaGl vs. fatigue -22.8; 28.6 [2.9], and for fatigue vs. pain -29.2; 33.8 [2.3]. In conclusion, PaGl, pain, and fatigue were most strongly explained by each other, not by more objective clinical measures of disease activity and were nearly identical on the group level. On the individual patient level, however, differences between the scores varied considerably. The findings highlight the challenge of understanding and dealing with traditional patient-reported VAS measures when it comes to individual RA patients in the daily clinic.

  17. Chemotherapy-radiotherapy association in Hodgkin's disease, clinical stages IA, II/sub 2/A: results of a prospective clinical trial with 166 patients. [/sup 60/Co

    SciTech Connect

    Andrieu, J.M.; Montagnon, B.; Asselain, B.; Bayle-Weisgerber, C.; Chastang, C.; Teillet, F.; Bernard, J.

    1980-11-15

    One hundred sixty-six patients with clinical stages IA, II/sub 2/A Hodgkin's disease were treated between April 1972 and December 1976 with three courses of multiagent chemotherapy (methylchlorethamine, vincristine, procarbazine, prednisone) followed by mantle irradiation - excluding mediastinum for those with initial upper cervical presentation and absence of mediastinal involvement - or inverted Y radiotherapy. With a follow-up of 12 to 84 months, the overall survival is 93.5% and the overall relapse-free survival 89.9%. With chemotherapy-radiotherapy sequence, staging laparotomy is not indicated. Results and side effects of this treatment strategy are compared with those of other treatment policies.

  18. Clinical and radiological characteristics of 17 Chinese patients with pathology confirmed tumefactive demyelinating diseases: follow-up study.

    PubMed

    Yao, Jiarui; Huang, Dehui; Gui, Qiuping; Chen, Xiaolei; Lou, Xin; Wu, Lei; Cheng, Chen; Li, Jie; Wu, Weiping

    2015-01-15

    Tumefactive demyelinating disease is a rare inflammatory demyelinating disease (IDD) of the central nervous system (CNS). The literature lacks a clear and consistent description of the clinical and radiological spectrum of this disorder, and few Chinese cases have been studied. Here we report 17 Chinese patients, with pathology confirmed CNS IDD, who had distinct clinical and imaging features from those in previous reports. Median age at onset was 47 years, with a female to male ratio of 1.1:1. Multifocal lesions were present in nine cases (53%) on their pre-biopsy magnetic resonance imagings (MRIs), with locations predominantly involving periventricular white matter (41%), subcortical white matter (41%), juxtacortical regions (41%), and cortical gray matter (35%). Moderate to severe perilesional edema and/or mass effect were present in 35% of cases. A variety of enhancement patterns were observed; most were heterogeneous, including ring-like, patchy, venular-like, nodular, punctate, and diffuse in a decreased frequency. Perilesional restriction on diffusion-weighted images (DWI) were evident in 70% cases. Clinical course prior to biopsy was a first neurological event in 82% cases. During a median follow-up of 4.1 years, 76% of cases remained as isolated demyelinating syndrome, and 70% experienced a total or near-total recovery regardless of whether they received immunotherapy. Further studies are needed, especially concerning series with pathological confirmation and long-term follow-up information.

  19. Impact of imiglucerase supply shortage on clinical and laboratory parameters in Norrbottnian patients with Gaucher disease type 3.

    PubMed

    Machaczka, Maciej; Kämpe Björkvall, Cecilia; Wieremiejczyk, Joanna; Paucar Arce, Martin; Myhr-Eriksson, Kristina; Klimkowska, Monika; Hägglund, Hans; Svenningsson, Per

    2015-02-01

    A viral contamination of the production plant producing imiglucerase (Cerezyme™) resulted in an unpredicted worldwide shortage of global supplies during 2009-2010. The aim of the study was to describe the effects of dose reduction of enzyme replacement therapy (ERT) in adults with Norrbottnian form of Gaucher disease type 3 (N-GD3). There were ten adults with N-GD3 treated with imiglucerase in the county of Norrbotten in June 2009. Analyzed variables included plasma chitotriosidase activity and concentration of CCL18/PARC, whole blood hemoglobin concentration (Hb) and platelet count (PLT), as well as patients' body weight, subjective complaints and health status measured by the EuroQoL-5D questionnaire. The median duration of ERT shortage lasted for 14 months (10-20 months). The median percentage reduction of imiglucerase dose was 36 % (26-59 %). Hb decreased in four patients, PLT decreased in three patients, chitotriosidase increased in three patients (max. +22 % of baseline), and CCL18/PARC increased in six patients (+14 % to +57 %). The body weight was moderately decreased in one patient. No new bone events were noted. Self-assessment of individual patient's health status was stable in all but one patient. Our results suggest that moderate reduction of ERT dosage lasting for relatively short period of time can lead to worsening in biomarkers of adults with N-GD3. However, this worsening is infrequently translated to clinical worsening of patients. It is possible that CCL18/PARC has a higher sensitivity than chitotriosidase in monitoring of ERT dosing in GD3. PMID:25205209

  20. Acute clinical events in 299 homozygous sickle cell patients living in France. French Study Group on Sickle Cell Disease.

    PubMed

    Neonato, M G; Guilloud-Bataille, M; Beauvais, P; Bégué, P; Belloy, M; Benkerrou, M; Ducrocq, R; Maier-Redelsperger, M; de Montalembert, M; Quinet, B; Elion, J; Feingold, J; Girot, R

    2000-09-01

    A subset of 299 patients with homozygous sickle cell anaemia, enrolled in the cohort of the French Study Group on sickle cell disease (SCD), was investigated in this study. The majority of patients were children (mean age 10.1 +/- 5.8 yr) of first generation immigrants from Western and Central Africa, the others originated from the French West Indies (20.2%). We report the frequency of the main clinical events (mean follow-up 4.2 +/- 2.2 yr). The prevalence of meningitis-septicaemia and osteomyelitis was, respectively, 11.4% and 12% acute chest syndrome was observed in 134 patients (44.8%). Twenty patients (6.7%) developed stroke with peak prevalence at 10-15 yr of age. One hundred and seventy-two patients (58%) suffered from one or more painful sickle cell crises, while the others (42.5%) never suffered from pain. The overall frequency of acute anaemic episodes was 50.5%, (acute aplastic anaemia 46%; acute splenic sequestration 26%). A group of 27 patients were asymptomatic (follow-up > 3 yr). Epistatic mechanisms influencing SCD were studied. Coinherited alpha-thalassemia strongly reduced the risk of stroke (p <0.001) and increased that of painful crises (p < 0.02). There was a low prevalence of Senegal and Bantu (CAR) betas-chromosomes in patients with meningitis (p <0.04) and osteomyelitis (p < 0.03). Prevalence of Senegal betas-chromosomes was lower in the asymptomatic group of 27 patients (p < 0.02). The patients come from a population of unmixed immigrants in whom the beta-globin gene haplotype strongly reflects the geographic origin and identifies subgroups with a homogenous genetic background. Thus the observed effects might result more from differences in as yet unidentified determinants in the genetic background than from the direct linkage with differences in the beta-globin gene locus.

  1. The Submaximal Clinical Exercise Tolerance Test (SXTT) to Establish Safe Exercise Prescription Parameters for Patients with Chronic Disease and Disability

    PubMed Central

    Gappmaier, Eduard

    2012-01-01

    Purpose To describe how to perform a Submaximal Clinical Exercise Tolerance Test (SXTT) as part of an exercise evaluation in the physical therapy clinic to determine an appropriate exercise prescription and to establish safety of exercise for physical therapy clients. Summary of Key Points Physical activity is crucial for general health maintenance. An exercise evaluation includes a comprehensive patient history, physical examination, exercise testing, and exercise prescription. The SXTT provides important clinical data that form the foundation for an effective and safe exercise prescription. Observations obtained during the exercise evaluation will identify at-risk patients who should undergo further medical evaluation before starting an exercise program. Two case examples of SXTTs administered to individuals with multiple sclerosis are presented to demonstrate the application of these principles. Statement of Recommendations Due to their unique qualifications, physical therapists shall assume responsibility to design and monitor safe and effective physical activity programs for all clients and especially for individuals with chronic disease and disability. To ensure safety and efficacy of prescribed exercise interventions, physical therapists need to perform an appropriate exercise evaluation including exercise testing before starting their clients on an exercise program. PMID:22833706

  2. Consensus-based clinical practice recommendations for the examination and management of falls in patients with Parkinson's disease.

    PubMed

    van der Marck, Marjolein A; Klok, Margit Ph C; Okun, Michael S; Giladi, Nir; Munneke, Marten; Bloem, Bastiaan R

    2014-04-01

    Falls in Parkinson's disease (PD) are common and frequently devastating. Falls prevention is an urgent priority, but there is no accepted program that specifically addresses the risk profile in PD. Therefore, we aimed to provide consensus-based clinical practice recommendations that systematically address potential fall risk factors in PD. We developed an overview of both generic (age-related) and PD-specific factors. For each factor, we specified: best method of ascertainment; disciplines that should be involved in assessment and treatment; and which interventions could be engaged. Using a web-based tool, we asked 27 clinically active professionals from multiple relevant disciplines to evaluate this overview. The revised version was subsequently reviewed by 12 experts. Risk factors and their associated interventions were included in the final set of recommendations when at least 66% of reviewing experts agreed. These recommendations included 31 risk factors. Nearly all required a multidisciplinary team approach, usually involving a neurologist and PD-nurse specialist. Finally, the expert panel proposed to first identify the specific fall type and to tailor screening and treatment accordingly. A routine evaluation of all risk factors remains reserved for high-risk patients without prior falls, or for patients with seemingly unexplained falls. In conclusion, this project produced a set of consensus-based clinical practice recommendations for the examination and management of falls in PD. These may be used in two ways: for pragmatic use in current clinical practice, pending further evidence; and as the active intervention in clinical trials, aiming to evaluate the effectiveness and cost-effectiveness of large scale implementation.

  3. 13C-methacetin breath test correlates with clinical indices of liver disease severity in patients with primary biliary cirrhosis.

    PubMed

    Kochel-Jankowska, A; Hartleb, M; Jonderko, K; Kaminska, M; Kasicka-Jonderko, A

    2013-02-01

    This prospective study intended to ascertain if cytochrome P450 dependent liver function is affected in early and late histological stages of primary biliary cirrhosis (PBC). The study included 32 female PBC patients (mean age 55.4 years, range 33-70) and 16 aged-matched healthy women (mean age 52.6 years, range 38-65). In every subject a 13(C)-methacetin breath test (13(C)-MBT) was applied, and the results were related to histological Ludwig's staging system and several indices of liver disease severity comprising the MAYO-1, MAYO-2, MELD, and Child-Pugh score. The 13(C)-MBT differentiated healthy controls from the patients with Ludwig IV and Ludwig III histopathological stages of PBC. The most significant relationships (i.e. explaining >50% of the variance) were found between measurements of the momentary breath 13(C) elimination from 6 to 18 minutes as well as the 15-min or 30-min cumulative elimination and the MAYO-1 or MAYO-2 scores. The breath test poorly correlated with histopathological features of PBC, however, it accurately discriminated cirrhotic from non-cirrhotic patients (momentary breath 13(C) elimination at 40 min, AUROC 0,958). In conclusion, 13(C)-MBT correlates with clinical scoring systems, especially those specifically designed for PBC (Mayo model) and accurately recognizes the disease at the stage of cirrhosis up to 40 minutes of the test duration.

  4. Differences in Clinical Characteristics between Patients with Non-Erosive Reflux Disease and Erosive Esophagitis in Korea

    PubMed Central

    Ha, Na Rae; Lee, Oh Young; Yoon, Byung Chul; Choi, Ho Soon; Hahm, Joon Soo; Ahn, You Hern; Koh, Dong Hee

    2010-01-01

    Gastroesophageal reflux disease (GERD) is caused by abnormal reflux of gastric contents into the esophagus. GERD can be divided into two groups, erosive esophagitis and non-erosive reflux disease (NERD). The aim of this study was to compare the clinical characteristics of patients with erosive esophagitis to those with NERD. All participating patients underwent an upper endoscopy during a voluntary health check-up. The NERD group consisted of 500 subjects with classic GERD symptoms in the absence of esophageal mucosal injury during upper endoscopy. The erosive esophagitis group consisted of 292 subjects with superficial esophageal erosions with or without typical symptoms of GERD. Among GERD patients, male gender, high body mass index, high obesity degree, high waist-to-hip ratio, high triglycerides, alcohol intake, smoking and the presence of a hiatal hernia were positively related to the development of erosive esophagitis compared to NERD. In multivariated analysis, male gender, waist-to-hip ratio and the presence of a hiatal hernia were the significant risk factors of erosive esophagitis. We suggest that erosive esophagitis was more closely related to abdominal obesity. PMID:20808675

  5. Implementing electronic clinical reminders for lipid management in patients with ischemic heart disease in the veterans health administration: QUERI Series

    PubMed Central

    Sales, Anne; Helfrich, Christian; Ho, P Michael; Hedeen, Ashley; Plomondon, Mary E; Li, Yu-Fang; Connors, Alison; Rumsfeld, John S

    2008-01-01

    Background Ischemic heart disease (IHD) affects at least 150,000 veterans annually in the United States. Lowering serum cholesterol has been shown to reduce coronary events, cardiac death, and total mortality among high risk patients. Electronic clinical reminders available at the point of care delivery have been developed to improve lipid measurement and management in the Veterans Health Administration (VHA). Our objective was to report on a hospital-level intervention to implement and encourage use of the electronic clinical reminders. Methods The implementation used a quasi-experimental design with a comparison group of hospitals. In the intervention hospitals (N = 3), we used a multi-faceted intervention to encourage use of the electronic clinical reminders. We evaluated the degree of reminder use and how patient-level outcomes varied at the intervention and comparison sites (N = 3), with and without adjusting for self-reported reminder use. Results The national electronic clinical reminders were implemented in all of the intervention sites during the intervention period. A total of 5,438 patients with prior diagnosis of ischemic heart disease received care in the six hospitals (3 intervention and 3 comparison) throughout the 12-month intervention. The process evaluation showed variation in use of reminders at each site. Without controlling for provider self-report of use of the reminders, there appeared to be a significant improvement in lipid measurement in the intervention sites (OR 1.96, 95% CI 1.34, 2.88). Controlling for use of reminders, the amount of improvement in lipid measurement in the intervention sites was even greater (OR 2.35, CI 1.96, 2.81). Adjusting for reminder use demonstrated that only one of the intervention hospitals had a significant effect of the intervention. There was no significant change in management of hyperlipidemia associated with the intervention. Conclusion There may be some benefit to focused effort to implement electronic

  6. Primary care of the patient with chronic obstructive pulmonary disease-part 4: understanding the clinical manifestations of a progressive disease.

    PubMed

    Cooper, Christopher B; Dransfield, Mark

    2008-07-01

    This article reviews the main factors influencing the pathophysiology, symptoms, and progression of chronic obstructive pulmonary disease (COPD), including dynamic hyperinflation, exacerbations, and comorbid illness. Key clinical trials and reviews were identified. After formal presentations to a panel of pulmonary specialists and primary care physicians, a series of concepts, studies, and practical clinical implications related to COPD progression were integrated into this article, the last in a 4-part mini-symposium. The main points of roundtable consensus were as follows: (1) COPD is characterized by declining pulmonary function as classically measured by forced expiratory volume in 1 second (FEV(1)), but the complex pathophysiology and the rationale for bronchodilator therapy are actually better understood in terms of progressive hyperinflation, both at rest (static) and worsening during exercise (dynamic) and exacerbations; (2) although COPD progression is often thought of as inevitable and continuous, the clinical course is actually quite variable and probably influenced by the frequency of exacerbations; (3) preventing exacerbations with pharmacologic and nonpharmacologic care can influence overall morbidity; (4) comorbidities such as lung cancer, cardiovascular disease, and skeletal muscle dysfunction also contribute to declining patient health; and (5) surgical lung volume reduction and lung transplantation should be considered for selected patients with very severe COPD. We conclude that the concept of COPD as a gradual but relentlessly progressive illness that is best monitored via FEV(1) is outdated and likely compromises patient care. Many patients now being managed in primary care settings will benefit from an earlier, broad-based, and aggressive approach to management.

  7. The Utility of Fecal Calprotectin in the Real-World Clinical Care of Patients with Inflammatory Bowel Disease

    PubMed Central

    Abej, Esmail

    2016-01-01

    Objectives. To determine the relationship between fecal calprotectin (FCAL) and imaging studies and other biochemical inflammatory markers and the impact of FCAL measurements on decision-making in IBD patient management in usual clinical practice. Methods. 240 persons with IBD were enrolled. The correlation between FCAL values and other markers for disease activity such as serum albumin (alb), hemoglobin (Hg), and C-reactive protein (CRP) and diagnostic imaging or colonoscopy was examined. FCAL ≥ 250 mcg/g of stool was considered a positive result indicating active IBD. Results. 183 stool samples (76.3%) were returned. The return rate in the pediatric and adult cohorts was 91% (n = 82) and 67.3% (n = 101), respectively (P < 0.0001). Positive FCAL was associated with colonoscopy findings of active IBD (P < 0.05), low albumin (P < 0.05), anemia (P < 0.01), and elevated CRP (P < 0.01). There was no significant difference for FCAL results by outcomes on small bowel evaluation among the 21 persons with small bowel CD. Most persons (87.5%) with normal FCAL and no change in therapy remained in remission during subsequent 3 months. Conclusions. FCAL is a useful marker of disease activity and a valuable tool in managing persons with IBD in clinical practice. Clinicians have to be cautious in interpreting FCAL results in small bowel CD. PMID:27774443

  8. Feasibility and Clinical Outcomes of Peripheral Drug-Coated Balloon in High-Risk Patients with Femoropopliteal Disease

    PubMed Central

    Huang, Hsuan-Li; Juang, Jyh-Ming Jimmy; Chou, Hsin-Hua; Tsao, Chueh-Yung; Wu, Tien-Yu; Ko, Yu-Lin

    2015-01-01

    Background Clinical outcomes of the drug-coated balloon (DCB) procedure in high-risk patients with femoropopliteal (FP) disease have not been investigated sufficiently. Methods This retrospective, single-center study analyzed 87 patients (39% dialysis) and 97 affected legs (64% critical limb ischemia [CLI]) that underwent DCB for symptomatic FP disease from March 2013 to September 2014. Risk stratification was based on FeDCLIP (female, diabetes, dialysis, CLI, lesion length >150 mm and poor runoff) score. The DCB outcomes among the different risk groups were compared and factors predicting restenosis were analyzed during follow-up. Results Most of study participants (84%) were moderate to high-risk patients. The procedural success rate was 100% and the 30-day major adverse vascular event rate was 2.1%. The mean lesion length was 178 ± 106 mm and the mean follow-up time was 428 ± 145 (range 50–782) days. The binary restenosis-free and clinically driven target lesion revascularization (CD-TLR)-free rates at 12 months were 77.5% and 84.3%, respectively, for all participants. No significant differences were observed in 1-year binary restenosis and CD-TLR rates in the low-, moderate-, and high-risk groups (60%, 84%, and 73%: p = 0.396; 78%, 89%, and 80%: p = 0.635, respectively). In multivariate analysis, lesion length >150 mm (Hazard ratio [HR]: 8.00, 95% confidence interval (CI) 1.12 to 55.6, p = 0.038) and Rutherford class 6 (HR: 7.09, 95% CI, 1.15 to 43.5, p = 0.034) were identified as independent predictors of binary restenosis. Conclusions Despite general comorbidities and advanced limb ischemia, 1-year outcomes of DCB in high-risk patients with FP disease were effective. The DCB procedure holds promise to improve vessel patency; however, lesion length >150 mm and major tissue loss were independent predictors for binary restenosis after the treatment. PMID:26599128

  9. Clinically relevant RHD-CE genotypes in patients with sickle cell disease and in African Brazilian donors

    PubMed Central

    Gaspardi, Ane C.; Sippert, Emília A.; de Macedo, Mayra Dorigan; Pellegrino, Jordão; Costa, Fernando F.; Castilho, Lilian

    2016-01-01

    Background As a consequence of the homology and opposite orientation of RHD and RHCE, numerous gene rearrangements have occurred in Africans and resulted in altered RH alleles that predict partial antigens, contributing to the high rate of Rh alloimmunisation among patients with sickle cell disease (SCD). In this study, we characterised variant RH alleles encoding partial antigens and/or lacking high prevalence antigens in patients with SCD and in African Brazilian donors, in order to support antigen-matched blood for transfusion. Material and methods RH genotypes were determined in 168 DNA samples from SCD patients and 280 DNA samples from African Brazilian donors. Laboratory developed tests, RHD BeadChipTM, RHCE BeadChipTM, cloning and sequencing were used to determine RHD-CE genotypes among patients and African Brazilian blood donors. Results The distributions of RHD and RHCE alleles in donors and patients were similar. We found RHCE variant alleles inherited with altered RHD alleles in 25 out of 168 patients (15%) and in 22 out of 280 (7.8%) African Brazilian donors. The RHD and RHCE allele combinations found in the population studied were: RHD*DAR with RHCE*ceAR; RHD*weak D type 4.2.2 with RHCE*ceAR, RHD*weak D type 4.0 with RHCE*ceVS.01 and RHCE*ceVS.02; RHD*DIIIa with RHCE*ceVS.02. Thirteen patients and six donors had RHD-CE genotypes with homozygous or compound heterozygous alleles predicting partial antigens and/or lacking high prevalence antigens. Eleven patients were alloimmunised to Rh antigens. For six patients with RHD-CE genotypes predicting partial antigens, no donors with similar genotypes were found. Discussion Knowledge of the distribution and prevalence of RH alleles in patients with SCD and donors of African origin may be important for implementing a programme for RH genotype matching in SCD patients with RH variant alleles and clinically significant Rh antibodies. PMID:27177398

  10. Clinical Effect and Safety Profile of Recombinant Human Lysosomal Acid Lipase in Patients with Cholesteryl Ester Storage Disease

    PubMed Central

    Balwani, Manisha; Breen, Catherine; Enns, Gregory M; Deegan, Patrick B; Honzík, Tomas; Jones, Simon; Kane, John P; Malinova, Vera; Sharma, Reena; Stock, Eveline O; Valayannopoulos, Vassili; Wraith, J Edmond; Burg, Jennifer; Eckert, Stephen; Schneider, Eugene; Quinn, Anthony G

    2013-01-01

    Background & Aims Cholesteryl Ester Storage Disease, an inherited deficiency of lysosomal acid lipase, is an underappreciated cause of progressive liver disease with no approved therapy. Presenting features include dyslipidemia, elevated transaminases, and hepatomegaly. Methods To assess the clinical effects and safety of the recombinant human lysosomal acid lipase, sebelipase alfa, 9 patients received 4 once-weekly infusions (0.35, 1, or 3 mg·kg−1) in LAL-CL01 which is the first human study of this investigational agent. Patients completing LAL-CL01 were eligible to enroll in the extension study (LAL-CL04) in which they again received 4 once-weekly infusions of sebelipase alfa (0.35, 1, or 3 mg·kg−1) before transitioning to long term every other week infusions (1 or 3 mg·kg−1). Results Sebelipase alfa was well-tolerated with mostly mild adverse events unrelated sebelipase alfa. No anti-drug antibodies were detected. Transaminases decreased in patients in LAL-CL01 and increased between studies. In 7 patients receiving ongoing sebelipase alfa treatment in LAL-CL04, mean±SD decreases for alanine transaminase and aspartate aminotransferase at week 12 compared to the baseline values in LAL-CL01 were 46±21U/L (-52%) and 21±14U/L (-36%), respectively (p<0.05). Through week 12 of LAL-CL04, these 7 patients also showed mean decreases from baseline in total cholesterol of 44±41mg/dL (-22%; p=0.047), low density lipoprotein-cholesterol of 29±31mg/dL (-27%; p=0.078), and triglycerides of 50±38mg/dL (-28%, p=0.016) and increases in high density lipoprotein-cholesterol of 5mg/dL (15%; p=0.016). Conclusions These data establish that sebelipase alfa, an investigational enzyme replacement, in patients with Cholesteryl Ester Storage Disease is well tolerated, rapidly decreases serum transaminases and that these improvements are sustained with long term dosing and are accompanied by improvements in serum lipid profile. PMID:23348766

  11. Higher seroprevalence of hepatitis B virus antigen in patients with cystic hydatid disease than in patients referred to internal medicine clinics in Turkey.

    PubMed

    Gültepe, Bilge; Dülger, Ahmet Cumhur; Gültepe, İlhami; Karadas, Sevdegul; Ebinç, Senar; Esen, Ramazan

    2014-02-01

    Turkey remains an intermediate area for prevalence of hepatitis B virus (HBV) surface antigenemia. The sheep-raising areas of Turkey also pose a high risk for cystic hydatid disease (CHD). Both HBV infection and CHD are major public health issues particularly in eastern parts of Turkey; however, there is no data regarding HBV infection in patients who have had CHD. The aims of this study were to evaluate the association between HBV infection and CHD and suggest ways to reduce HBV infection which is still widespread in Turkey. A retrospective study was conducted with 94 adult patients with active CHD referred to the hepatology department, Yuzuncuyil University School of Medicine from December 2010 to December 2012. All subjects came from rural areas of the region and underwent ultrasonography of abdomen which detected CHD of the liver. All the patients were serologically positive for Echinococcus granulosus. The control group consisted of 500 patients (300 men and 200 women) referred to the internal medicine clinics for other reasons. The patients with CHD and in the control group were tested for the existence of HBs antigen according to the standard procedures. The seroprevalence of HBs antigen was significantly higher in patients with active CHD than those in the control group (12.7% vs 5.2%; P=0.0017). Our data indicate that there is significant association between HBV infection and CHD. All patients with CHD should be screened for HBV infection.

  12. Rare Diseases Clinical Research Network

    MedlinePlus

    ... RDCRN? Aims of the Rare Diseases Clinical Research Network Contact Us RDCRN Members Login Accessibility Disclaimer The Rare Diseases Clinical Research Network is an initiative of the Office of Rare ...

  13. Clinical, serologic, and genetic factors associated with pyoderma gangrenosum and erythema nodosum in inflammatory bowel disease patients

    PubMed Central

    Weizman, Adam; Huang, Brian; Berel, Dror; Targan, Stephan R.; Dubinsky, Marla; Fleshner, Phillip; Ippoliti, Andrew; Kaur, Manreet; Panikkath, Deepa; Brant, Steve; Oikonomou, Ioannis; Duerr, Rick; Rioux, John; Silverberg, Mark; Rotter, Jerome I; Vasiliauskas, Eric; Haritunians, Talin; Shih, David; Li, Dalin; Melmed, Gil Y.; McGovern, Dermot P.B.

    2014-01-01

    Objective Pyoderma gangrenosum (PG) and erythema nodosum (EN) are the most common cutaneous manifestations of inflammatory bowel disease (IBD) but little is known regarding their etiopathogenesis. Design We performed a case control study comparing characteristics between IBD patients with a documented episode of PG (PG+) and/or EN (EN+) with those without PG (PG-) and EN (EN-). Data on clinical features were obtained by chart review. IBD related serology was determined using ELISA and genome-wide data generated using Illumina technology. Standard statistical tests for association were used. Results We identified a total 92 cases of PG and 103 cases of EN with genetic and clinical characteristics, of which 64 PG and 55 EN were available for serological analyses. Fewer male subjects were identified in the PG(+) (OR 0.6, p=0.009) and EN(+) groups (OR 0.31, p=0<0.0001). Colonic disease, previous IBD related surgery, and non-cutaneous extra-intestinal manifestations were more common among both PG(+) and EN(+) patients compared to controls. PG(+) was associated with ANCA seropositivity (p=0.03) and higher ANCA level (p=0.02) in CD. Genetic associations with PG included known IBD loci (IL8RA (p=0.00003), and PRDM1 (0.03)) as well as with USP15 (4.8×10−6) and TIMP3 (5.6 ×10−7). Genetic associations with EN included known IBD susceptibility genes (PTGER4 (p=8.8×10−4), ITGAL (0.03)) as well as SOCS5 (9.64×10−6), CD207 3.14×10−6), ITGB3 (7.56×10−6) and rs6828740 (4q26)(p <5.0 × 10−8). Multivariable models using clinical, serologic, and genetic parameters predicted PG (AUC 0.8) and EN (AUC 0.97). Conclusion Cutaneous manifestations in IBD are associated with distinctive genetic characteristics as well as with the similar clinical characteristics including the development of other extra-intestinal manifestations suggesting shared and distinct etiologies. PMID:24487271

  14. Vitamin D-mediated calcium absorption in patients with clinically stable Crohn's disease: a pilot study

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Vitamin D is the critical hormone for intestinal absorption of calcium. Optimal calcium absorption is important for proper mineralization of bone in the prevention of osteoporosis and osteoporotic fractures, among other important functions. Diseases associated with gut inflammation, such as Crohn's ...

  15. An Intervention to Promote the Female Condom to Sexually Transmitted Disease Clinic Patients

    ERIC Educational Resources Information Center

    Artz, Lynn; Macaluso, Maurizio; Kelaghan, Joseph; Austin, Harland; Fleenor, Michael; Robey, Lawrence; Hook, III, Edward W.; Brill, Ilene

    2005-01-01

    This article describes a 1-hour behavioral intervention designed to promote female condoms and safer sex to women at a high risk for sexually transmitted diseases (STDs). The intervention includes a promotional videotape; a skills-oriented counseling session with a nurse clinician; assorted take-home items, including a videotape for men; and free…

  16. Pharmacokinetics, Pharmacodynamics and Clinical Use of SGLT2 Inhibitors in Patients with Type 2 Diabetes Mellitus and Chronic Kidney Disease.

    PubMed

    Scheen, André J

    2015-07-01

    Inhibitors of sodium-glucose cotransporters type 2 (SGLT2) are proposed as a novel approach for the management of type 2 diabetes mellitus. SGLT2 cotransporters are responsible for reabsorption of 90 % of the glucose filtered by the kidney. The glucuretic effect resulting from SGLT2 inhibition contributes to reduce hyperglycaemia and also assists weight loss and blood pressure reduction. Several SGLT2 inhibitors are already available in many countries (dapagliflozin, canagliflozin, empagliflozin) and in Japan (ipragliflozin, tofogliflozin). These SGLT2 inhibitors share similar pharmacokinetic characteristics with a rapid oral absorption, a long elimination half-life allowing once-daily administration, an extensive hepatic metabolism mainly via glucuronidation to inactive metabolites and a low renal elimination as a parent drug. Pharmacokinetic parameters are slightly altered in the case of chronic kidney disease (CKD). While no dose adjustment is required in the case of mild CKD, SGLT2 inhibitors may not be used or only at a lower daily dose in patients with moderate CKD. Furthermore, the pharmacodynamic response to SGLT2 inhibitors as assessed by urinary glucose excretion declines with increasing severity of renal impairment as assessed by a reduction in the estimated glomerular filtration rate. Nevertheless, the glucose-lowering efficacy and safety of SGLT2 inhibitors are almost comparable in patients with mild CKD as in patients with normal kidney function. In patients with moderate CKD, the efficacy tends to be dampened and safety concerns may occur. In patients with severe CKD, the use of SGLT2 inhibitors is contraindicated. Thus, prescribing information should be consulted regarding dosage adjustments or restrictions in the case of renal dysfunction for each SGLT2 inhibitor. The clinical impact of SGLT2 inhibitors on renal function and their potential to influence the course of diabetic nephropathy deserve attention because of preliminary favourable results

  17. SERIAL NECK ULTRASOUND IS MORE LIKELY TO IDENTIFY FALSE-POSITIVE ABNORMALITIES THAN CLINICALLY SIGNIFICANT DISEASE IN LOW-RISK PAPILLARY THYROID CANCER PATIENTS

    PubMed Central

    Yang, Samantha Peiling; Bach, Ariadne M.; Tuttle, R. Michael; Fish, Stephanie A.

    2016-01-01

    Objective American Thyroid Association (ATA) low-risk papillary thyroid cancer (PTC) patients without structural evidence of disease on initial posttreatment evaluation have a low risk of recurrence. Despite this, most patients undergo frequent surveillance neck ultrasound (US). The objective of the study was to evaluate the clinical utility of routine neck US in ATA low-risk PTC patients with no structural evidence of disease after their initial thyroid surgery. Methods We performed a retrospective review of 171 ATA low-risk PTC patients after total thyroidectomy, with or without radioactive iodine (RAI) ablation, who had a neck US without suspicious findings after therapy. The main outcome measure was a comparison of the frequency of finding false-positive US abnormalities and the frequency of identifying structural disease recurrence. Results Over a median follow-up of 8 years, 171 patients underwent a median of 5 neck US (range 2–17). Structural recurrence with low-volume disease (≤1 cm) was identified in 1.2% (2/171) of patients at a median of 2.8 years (range 1.6–4.1 years) after their initial diagnosis. Recurrence was associated with rising serum thyroglobulin (Tg) level in 1 of the 2 patients and was detected without signs of biochemical recurrence in the other patient. Conversely, false-positive US abnormalities were identified in 67% (114/171) of patients after therapy, leading to additional testing without identifying clinically significant disease. Conclusion In ATA low-risk patients without structural evidence of disease on initial surveillance evaluation, routine screening US is substantially more likely to identify false-positive results than clinically significant structural disease recurrence. PMID:26372300

  18. Improving physician's adherence to completing vaccination schedules for patients with type 2 diabetes attending non-communicable diseases clinics in West Bay Health Center, Qatar.

    PubMed

    Tawfik, Hassan; Bashwar, Zelaikha; Al-Ali, Amal; Salem, Mohamed; Abdelbagi, Isameldin

    2015-01-01

    Incomplete vaccination for patients with type 2 diabetes attending non-communicable diseases (NCD) clinics is an issue that could affect patient's health and wellness negatively and puts patients at high risk of serious diseases. We aimed to improve physicians adherence to complete vaccination schedule for patients with type 2 diabetes attending NCD clinics in west bay health center according to American Diabetes Association (ADA) recommendation by 25% by January 2015. In the pre-intervention phase: the quality improvement team designed a checklist to collect the percentage of physician's adherence of prescription of the recommended vaccination for patients with type 2 diabetes. The percentage of complete vaccination in patients with diabetes attending NCD clinic in West Bay Health Center was 20% . In the intervention phase the intervention was in the form of: the creation a vaccination form and attached to the (NCD) progress note; to distribute and remind the physicians about the ADA guidelines vaccination recommendations; a summary of the vaccination schedule developed and attached to (NCD) form; development of vaccination reminder posters and posters in the waiting area, nurse station, and physician clinics and education and orientation sessions for NCD clinic staff. In the post-intervention phase the average percentage of complete vaccination in patients with diabetes attending NCD clinic in West Bay Health Center increased to 69%.

  19. Improving physician's adherence to completing vaccination schedules for patients with type 2 diabetes attending non-communicable diseases clinics in West Bay Health Center, Qatar

    PubMed Central

    Tawfik, Hassan; Bashwar, Zelaikha; Al-Ali, Amal; Salem, Mohamed; Abdelbagi, Isameldin

    2015-01-01

    Incomplete vaccination for patients with type 2 diabetes attending non-communicable diseases (NCD) clinics is an issue that could affect patient's health and wellness negatively and puts patients at high risk of serious diseases. We aimed to improve physicians adherence to complete vaccination schedule for patients with type 2 diabetes attending NCD clinics in west bay health center according to American Diabetes Association (ADA) recommendation by 25% by January 2015. In the pre-intervention phase: the quality improvement team designed a checklist to collect the percentage of physician's adherence of prescription of the recommended vaccination for patients with type 2 diabetes. The percentage of complete vaccination in patients with diabetes attending NCD clinic in West Bay Health Center was 20% . In the intervention phase the intervention was in the form of: the creation a vaccination form and attached to the (NCD) progress note; to distribute and remind the physicians about the ADA guidelines vaccination recommendations; a summary of the vaccination schedule developed and attached to (NCD) form; development of vaccination reminder posters and posters in the waiting area, nurse station, and physician clinics and education and orientation sessions for NCD clinic staff. In the post-intervention phase the average percentage of complete vaccination in patients with diabetes attending NCD clinic in West Bay Health Center increased to 69%. PMID:26732463

  20. The use of health status questionnaires in the management of chronic obstructive pulmonary disease patients in clinical practice.

    PubMed

    van der Molen, Thys; Diamant, Zuzana; Kocks, Jan Willem H; Tsiligianni, Ioanna G

    2014-08-01

    Current guidelines recommend chronic obstructive pulmonary disease (COPD) management based on symptoms or health status assessment and lung function parameters. However, COPD is a complex and heterogeneous disease that needs an individualized approach for proper disease management. A structured consultation including health status assessment tools, such as the Clinical COPD Questionnaire and the COPD Assessment Test should improve the quality of the consultation, providing more information than symptoms alone. Both questionnaires are designed to provide the clinician information enabling a more personalized disease approach and subsequent management. Although both Clinical COPD Questionnaire and COPD Assessment Test have good discriminate properties, their use as prognostic markers of severity and their ability to modify disease management has not yet been fully established. New studies are needed to further determine their value on several disease outcomes.

  1. Long-Term Clinical Remission in Biologically Naïve Crohn's Disease Patients with Adalimumab Therapy, Including Analyses of Switch from Adalimumab to Infliximab.

    PubMed

    Mizoshita, Tsutomu; Tanida, Satoshi; Ozeki, Keiji; Katano, Takahito; Shimura, Takaya; Mori, Yoshinori; Kubota, Eiji; Kataoka, Hiromi; Kamiya, Takeshi; Joh, Takashi

    2016-01-01

    There is little evidence regarding the maintenance of long-term clinical remission by adalimumab (ADA) therapy in Crohn's disease (CD) patients naïve to anti-tumor necrosis factor treatment (naïve CD patients), since most CD patients are treated with ADA after infliximab (IFX) therapy. The long-term clinical response to ADA was retrospectively analyzed in 17 naïve CD patients for at least 24 months, and the serum trough IFX levels were evaluated in patients switching from ADA to IFX. Of the 17 naïve CD patients, 14 (82.4%) maintained long-term clinical remission with ADA therapy for at least 24 months, without serious adverse events. The clinical condition of 7 patients was observed for more than 36 months, and 3, 1, 1, and 2 cases maintained remission at months 42, 48, 54, and 60 after ADA therapy, respectively. Three patients (17.6%) switched from ADA to IFX less than 24 months after the start of ADA therapy, and they had remission, retaining trough levels of IFX higher than 1 μg/ml, occasionally by dose escalation. In conclusion, maintenance ADA therapy achieves long-term clinical remission in naïve CD patients. Switching from ADA to IFX is an important therapeutic option in CD patients showing loss of response to ADA, occasionally with dose escalation, based on the analysis of serum IFX trough levels. PMID:27462198

  2. Measuring disease activity in adults with systemic lupus erythematosus: the challenges of administrative burden and responsiveness to patient concerns in clinical research.

    PubMed

    Mikdashi, Jamal; Nived, Ola

    2015-01-01

    Measuring lupus disease activity accurately remains a challenging and demanding task given the complex multi-system nature of lupus, an illness known for its variability between patients and within the same patient over time. Many have attempted to define what disease activity means and how it should be measured, and several instruments were devised for a standardized assessment of disease activity and outcome domains in clinical research. Several of these measuring tools have been able to detect clinical improvement and have demonstrated adequate reliability, validity, and sensitivity to change in observational studies, and some were found to be useful in randomized controlled trials. However, several failed clinical trials have confronted these metrics, as they were not intended for clinical trials. The Outcome Measures Rheumatology group and the US Food and Drug Administration have recommended using measures of disease activity, cumulative organ damage, health-related quality of life, and adverse events as outcomes of interest. Composite responder indices that determine disease global improvement, ensure no significant worsening in unaffected organ systems, and include a physician's global assessment have been used in randomized clinical trials. Yet unmet therapeutic needs were further challenged by the complex content and psychometric information of the updated instruments, including increased administrative burden associated with demanding training and cost of instruments, and small effect size associated with responsiveness to patient concerns. Nevertheless, with the progress of novel targeted therapy, refining the disease activity metrics is essential. Selection of the disease activity endpoints which is a defining aspect of clinical trial design must be tailored to the outcome of interest and measured by a reliably rated scale characterized by minimal administrative burden. An optimal scale should be simple and practical and incorporate elements of patient

  3. Measuring disease activity in adults with systemic lupus erythematosus: the challenges of administrative burden and responsiveness to patient concerns in clinical research.

    PubMed

    Mikdashi, Jamal; Nived, Ola

    2015-01-01

    Measuring lupus disease activity accurately remains a challenging and demanding task given the complex multi-system nature of lupus, an illness known for its variability between patients and within the same patient over time. Many have attempted to define what disease activity means and how it should be measured, and several instruments were devised for a standardized assessment of disease activity and outcome domains in clinical research. Several of these measuring tools have been able to detect clinical improvement and have demonstrated adequate reliability, validity, and sensitivity to change in observational studies, and some were found to be useful in randomized controlled trials. However, several failed clinical trials have confronted these metrics, as they were not intended for clinical trials. The Outcome Measures Rheumatology group and the US Food and Drug Administration have recommended using measures of disease activity, cumulative organ damage, health-related quality of life, and adverse events as outcomes of interest. Composite responder indices that determine disease global improvement, ensure no significant worsening in unaffected organ systems, and include a physician's global assessment have been used in randomized clinical trials. Yet unmet therapeutic needs were further challenged by the complex content and psychometric information of the updated instruments, including increased administrative burden associated with demanding training and cost of instruments, and small effect size associated with responsiveness to patient concerns. Nevertheless, with the progress of novel targeted therapy, refining the disease activity metrics is essential. Selection of the disease activity endpoints which is a defining aspect of clinical trial design must be tailored to the outcome of interest and measured by a reliably rated scale characterized by minimal administrative burden. An optimal scale should be simple and practical and incorporate elements of patient

  4. Increased serum β2-microglobulin is associated with clinical and immunological markers of disease activity in systemic lupus erythematosus patients.

    PubMed

    Hermansen, M-L F; Hummelshøj, L; Lundsgaard, D; Hornum, L; Keller, P; Fleckner, J; Fox, B; Poulsen, L K; Jacobsen, S

    2012-09-01

    The objective of this study was to explore the relationship between serum levels of β2-microglobulin (β2MG), which some studies suggest reflect disease activity in systemic lupus erythematosus (SLE), and various clinical and immunological markers of disease activity in SLE. Twenty-six SLE patients and 10 healthy controls were included. Disease activity was assessed by: SLEDAI, 24 hr-proteinuria, circulating levels of complement C3, anti-double-stranded DNA (anti-dsDNA), β2MG and various pro-inflammatory and anti-inflammatory cytokines (IL-6, IL-8, IL-10, IL-18) measured with a multiplex assay, IFN-α assessed with a reporter gene assay, and a combined expression score of 12 IFN-α inducible genes in peripheral blood mononuclear cells. Median serum levels of β2MG were significantly higher in SLE patients vs controls (2.8 mg/L, range: 1.1-21.6 and 1.2 mg/L, range: 0.9-1.7, respectively, p < 0.001). β2MG was correlated with SLEDAI score (R = 0.68, p < 0.001), 24 hr-proteinuria (R = 0.64, p < 0.001), and complement C3 (R = -0.52, p = 0.007). The cytokines were significantly correlated with β2MG: IL-6 (R = 0.45, p = 0.02), IL-8 (R = 0.75, p < 0.001), IL-10 (R = 0.67, p < 0.001) and IL-18 (R = 0.71, p < 0.001) as were serum IFN-α (R = 0.45, p = 0.02) and the IFN-α inducible gene-score (R = 0.51, p = 0.01). The results support that β2MG may serve as a marker of disease activity in SLE. The correlations with the measured cytokines indicate that increased β2MG in SLE reflects immunological activity.

  5. European multicentre study to define disease activity criteria for systemic sclerosis.* I. Clinical and epidemiological features of 290 patients from 19 centres

    PubMed Central

    Della, R; Valentini, G; Bombardieri, S; Bencivelli, W; Silman, A; D'Angelo, S; Cerinic, M; Belch, J; Black, C; Becvar, R; Bruhlman, P; Cozzi, F; Czirjak, L; Drosos, A; Dziankowska, B; Ferri, C; Gabrielli, A; Giacomelli, R; Hayem, G; Inanc, M; McHugh, N; Nielsen, H; Scorza, R; Tirri, E; van den Hoogen, F H J; Vlachoyiannopoulo..., P

    2001-01-01

    OBJECTIVE—To investigate the existence of differences among European referral centres for systemic sclerosis (SSc) in the pattern of attendance and referral and in the clinical and therapeutical approaches.
METHODS—In 1995 the European Scleroderma Study Group initiated a multicentre prospective one year study whose aim was to define the disease activity criteria in SSc. During the study period each participating European centre was asked to enrol consecutive patients satisfying American College of Rheumatology criteria for SSc and to fill out for each of them a standardised clinical chart. Patients from various centres were compared and differences in epidemiological, clinical, and therapeutical aspects were analysed.
RESULTS—Nineteen different medical research centres consecutively recruited 290 patients. The patients could be divided into two subgroups: 173 with the limited (lSSc) and 117 with the diffuse (dSSc) form of the disease. The clinical and serological findings for the series of 290 patients seemed to be similar to data previously reported. However, when the data were analysed to elicit any differences between the participating centres, a high degree of variability emerged, in both epidemiological and clinical features and in the diagnostic and therapeutic approaches to the disease.
CONCLUSIONS—The clinical approach to SSc, not only in different countries but also in different centres within the same country, is not yet standardised. To overcome this problem, it will be necessary for the scientific community to draw up a standardised procedure for the management of patients with SSc. This would provide a common research tool for different centres engaged in research on this complex disease.

 PMID:11350847

  6. Clinical and laboratory characterization of 114 cases of Castleman disease patients from a single centre: paraneoplastic pemphigus is an unfavourable prognostic factor.

    PubMed

    Dong, Yujun; Wang, Mingyue; Nong, Lin; Wang, Lihong; Cen, Xinan; Liu, Wei; Zhu, Sainan; Sun, Yuhua; Liang, Zeyin; Li, Yuan; Ou, Jinping; Qiu, Zhixiang; Ren, Hanyun

    2015-06-01

    This study retrospectively collected the clinical and laboratory data of 114 patients with Castleman disease (CD) from a single medical centre. Clinical classification identified 62 patients (54·4%) with unicentric Castleman disease and 52 (45·6%) with multi-centric Castleman disease. Pathological classification revealed 68 cases (59·6%) of hyaline vascular variant, 16 (14·1%) mixed cellular variant (Mix) and 30 (26·3%) plasmacytic variant. Clinical complications occurred in 69 CD patients, including 37 cases of paraneoplastic pemphigus (PNP) and 25 cases with renal complications. Haematological involvement, pleural effusion and/or ascites and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) were also found. Univariate analysis showed that presence of clinical complications and PNP were both risk factors relating to CD patient survival. Prognostic factors showing P < 0·15 in univariate analysis and those with clinical significance were subjected to multivariate analysis using a Cox regression model. PNP presence and age over 40 years both significantly adversely affected survival. Thus, only presence of PNP was identified as an independent unfavourable survival risk factor in both univariate and multivariate analyses. Overall, the present data provide a panoramic description of CD cases and emphasize that the presence of PNP is an adverse prognostic factor.

  7. Brief Report: Enhancement of Patient Recruitment in Rheumatoid Arthritis Clinical Trials Using a Multi‐Biomarker Disease Activity Score as an Inclusion Criterion

    PubMed Central

    Bolce, Rebecca; Hambardzumyan, Karen; Saevarsdottir, Saedis; Forslind, Kristina; Petersson, Ingemar F.; Sasso, Eric H.; Hwang, C. C.; Segurado, Oscar G.; Geborek, Pierre

    2015-01-01

    Objective Rheumatoid arthritis (RA) clinical trials often exclude patients who have low C‐reactive protein (CRP) levels, which slows enrollment into the trial. The purpose of this study was to determine whether high Multi‐Biomarker Disease Activity (MBDA) scores (>44) in RA patients with low CRP levels (≤10 mg/liter) could be used as a complement to CRP levels >10 mg/liter to enhance patient recruitment without affecting clinical trial outcomes. Methods We evaluated patients from the Swedish Pharmacotherapy (SWEFOT) trial, which did not include any selection criteria for CRP levels. Clinical outcomes were assessed after 3 months of methotrexate (MTX) monotherapy in MTX‐naive RA patients (n = 220) and after 3–10 months of add‐on therapy in patients who were incomplete responders to MTX alone (MTX‐IR) (n = 127). Radiographic outcomes were assessed at 1 year in all patients. Within each cohort, the outcomes were compared between patients with a CRP level of ≤10 mg/liter and an MBDA score of >44 at the start of the respective treatment interval versus those with a CRP level of >10 mg/liter. Results Patients with both a CRP level of ≤10 mg/liter and an MBDA score of >44 at baseline had clinical and radiographic outcomes that were comparable to those in patients with a CRP level of >10 mg/liter at baseline. This broadened definition of the inclusion criteria identified an additional 24% of patients in the MTX‐naive cohort and 47% in the MTX‐IR cohort. Conclusion Patient recruitment into RA clinical trials may be substantially enhanced, without any decrease in clinical and radiographic outcomes, by using as an inclusion criterion “a CRP level of >10 mg/liter and/or an MBDA score of >44.” PMID:26213309

  8. Characteristics and Clinical Management of a Cluster of 3 Patients With Ebola Virus Disease, Including the First Domestically Acquired Cases in the United States

    PubMed Central

    Liddell, Allison M.; Davey, Richard T.; Mehta, Aneesh K.; Varkey, Jay B.; Kraft, Colleen S.; Tseggay, Gebre K.; Badidi, Oghenetega; Faust, Andrew C.; Brown, Katia V.; Suffredini, Anthony F.; Barrett, Kevin; Wolcott, Mark J.; Marconi, Vincent C.; Lyon, G. Marshall; Weinstein, Gary L.; Weinmeister, Kenney; Sutton, Shelby; Hazbun, Munir; Albariño, César G.; Reed, Zachary; Cannon, Debi; Ströher, Ute; Feldman, Mark; Ribner, Bruce S.; Lane, H. Clifford; Fauci, Anthony S.; Uyeki, Timothy M.

    2015-01-01

    Background More than 26 000 cases of Ebola virus disease (EVD) have been reported in western Africa, with high mortality. Several patients have been medically evacuated to hospitals in the United States and Europe. Detailed clinical data are limited on the clinical course and management of patients with EVD outside western Africa. Objective To describe the clinical characteristics and management of a cluster of patients with EVD, including the first cases of Ebola virus (EBOV) infection acquired in the United States. Design Retrospective clinical case series. Setting Three U.S. hospitals in September and October 2014. Patients First imported EVD case identified in the United States and 2 secondary EVD cases acquired in the United States in critical care nurses who cared for the index case patient. Measurements Clinical recovery, EBOV RNA level, resolution of Ebola viremia, survival with discharge from hospital, or death. Results The index patient had high EBOV RNA levels, developed respiratory and renal failure requiring critical care support, and died. Both patients with secondary EBOV infection had nonspecific signs and symptoms and developed moderate illness; EBOV RNA levels were moderate, and both patients recovered. Limitation Both surviving patients received uncontrolled treatment with multiple investigational agents, including convalescent plasma, which limits generalizability of the results. Conclusion Early diagnosis, prompt initiation of supportive medical care, and moderate clinical illness likely contributed to successful outcomes in both survivors. The inability to determine the potential benefit of investigational therapies and the effect of patient-specific factors that may have contributed to less severe illness highlight the need for controlled clinical studies of these interventions, especially in the setting of a high level of supportive medical care. Primary Funding Source None. PMID:25961438

  9. Sex differences in disease presentation, treatment and clinical outcomes of patients with hepatocellular carcinoma: a single-centre cohort study

    PubMed Central

    Ladenheim, Maya R; Kim, Nathan G; Nguyen, Pauline; Le, An; Stefanick, Marcia L; Garcia, Gabriel; Nguyen, Mindie H

    2016-01-01

    Background Although sex differences in hepatocellular carcinoma (HCC) risk are well known, it is unclear whether sex differences also exist in clinical presentation and survival outcomes once HCC develops. Methods We performed a retrospective cohort study of 1886 HCC patients seen in a US medical centre in 1998–2015. Data were obtained by chart review with survival data also by National Death Index search. Results The cohort consisted of 1449 male and 437 female patients. At diagnosis, men were significantly younger than women (59.9±10.7 vs 64.0±11.6, p<0.0001). Men had significantly higher rates of tobacco (57.7% vs 31.0%, p<0.001) and alcohol use (63.2% vs 35.1%, p<0.001). Women were more likely to be diagnosed by routine screening versus symptomatically or incidentally (65.5% vs 58.2%, p=0.03) and less likely to present with tumours >5 cm (30.2% vs 39.8%, p=0.001). Surgical and non-surgical treatment utilisation was similar for both sexes. Men and women had no significant difference in median survival from the time of diagnosis (median 30.7 (range=24.5–41.3) vs 33.1 (range=27.4–37.3) months, p=0.84). On multivariate analysis, significant predictors for improved survival included younger age, surgical or non-surgical treatment (vs supportive care), diagnosis by screening, tumour within Milan criteria and lower Model for End-Stage Liver Disease score, but not female sex (adjusted HR=1.01, CI 0.82 to 1.24, p=0.94). Conclusions Although men have much higher risk for HCC development, there were no significant sex differences in disease presentation or survival except for older age and lower tumour burden at diagnosis in women. Female sex was not an independent predictor for survival. PMID:27493763

  10. The effect of platelet autoantibodies on the course of the disease and clinical response of patients with idiopathic thrombocytopenic purpura.

    PubMed

    Sikorska, A; Konopka, L; Maślanka, K

    2008-02-01

    In this study, we evaluated the response to treatment of 409 idiopathic thrombocytopenic purpura (ITP) patients who were tested for the presence of platelet-associated autoantibodies by direct-platelet immunofluorescence test (PIFT) and for the presence of plasma antibodies directed against the GPIIb/IIIa, GPIb and GPIa/IIa by monoclonal antibody immobilization of platelet antigens (MAIPA). In patients with platelet autoantibodies in comparison with patients without antibodies more frequently were observed the chronic form of disease (83.5%vs. 68.5%) and severe symptoms of haemorrhage diathesis (17.3%vs. 6.9%). Evaluation of the treatment response (to corticosteroids, immunosuppressive drugs and splenectomy) referred to patients with complete response, e.g. complete remission defined as platelet count of >100 x 10(9)/l for at least 2 years. The percentage of complete response in the whole population of ITP patients, both with and without autoantibodies regardless of the method of treatment, was similar (about 54%). However, the presence of platelet autoantibodies had effect on patients treated with corticosteroids: complete response approximately 71% (36/51) of patients with autoantibodies and in 60% (72/120) of patients without antibodies, as well as in patients treated with immunosuppressive drugs (cyclophosphamide, azathioprine, vincristin and vinblastin); complete response approximately 51% (11/21) of patients with autoantibodies and in 34.8% (6/17) of patients without autoantibodies. The presence of autoantibodies had no effect on the response of splenectomy patients. PMID:18190469

  11. [Clinical and immunological study of the relationship of the digestive system chronic diseases and atherosclerosis in the basin of the abdominal aorta in elderly patients].

    PubMed

    Dolgushina, A I; Shaposhnik, I I; Volchegorskiĭ, I A

    2014-01-01

    Paper describes clinical and immunological study about the relationship between chronic diseases of the digestive system and atherosclerosis in the basin of the abdominal aorta in patients of elderly and senile age. There were revealed the structural and clinical features of the gastrointestinal tract diseases, depending on the extent of atherosclerosis in the basin of the abdominal aorta. Evaluation of the immune status included the determination of lymphocyte subpopulation composition, the functional state of neutrophils and cytokine levels. It is found that the progression of atherosclerosis in the basin of the abdominal aorta in patients of elderly and senile age with chronic diseases of the digestive system was accompanied by the activation of pro-inflammatory mechanisms of the immune system and the accompanying intensification of oxidative stress.

  12. [Influence of combined lacto-vegetarian diet and selective beta-blocking agents on clinical and metabolic indices in patients with coronary heart disease].

    PubMed

    Ieromuzo, A A; Medkova, I L; Nemytin, Iu V; Ivanov, A N

    2012-01-01

    Clinical, hemodynamic and metabolic parameters were investigated for 42 patients with coronary heart disease, after myocardial infarct, recieved selective beta-adrenoblockers. Patients were divided in two groups. The first group (24 patients) were given methoprolol (50 mg/daily) and antiatherogenic lacto vegetarian diet, the second (18 patients)--methoprolol (50 mg/daily) and standard mixed antiatherogenic diet. After the treatment, positive changes clinical and gemodynamic parameters were observed in both groups of patients. Among the clinical symptoms, a more pronounced decrease in blood pressure in the patients on vegetarian diet and a more significant increase in their exercise tolerance. The level of total cholesterol on the serum of blood decreased by 16%, low-density lipoproteins cholesterol decreased by 18%, the atherogenic coefficient (KA) decreased by 31%, only in vegetarian group. High-density lipoprotein cholesterol increased in vegetarian group, by 14% and decreased in control group. Balanced antiatherogenic lacto vegetarian diet in patients with coronary heart disease prevents the hyperlipedemic effect caused by the selective beta-adrenoblockers and it is an agent for preventing its negative effect on lipid metabolism.

  13. [Clinical diagnosis of HIV infection in patients with acute surgical diseases of the abdominal cavity organs and pulmonary tuberculosis].

    PubMed

    Nguen, V Kh; Stroganov, P V; Geshelin, S A

    2011-09-01

    The results of treatment of 81 patients, suffering tuberculosis and operated in emergency for an acute surgical diseases of the abdominal cavity organs, are adduced, in 29 of them--nonspecific diseases of nontuberculosis genesis were diagnosed. In 52 patients the indication for emergency operation performance were complications of abdominal tuberculosis (perforation of the tuberculosis ulcers of small intestine--in 37, the tuberculosis mesadenitis--in 15), of them in 34--pulmonary tuberculosis was in inactive phase, that's why the HIV presence was supposed. In 26 patients the diagnosis was confirmed, basing on serologic analysis data. The presence of intraabdominal catastrophe, caused by abdominal tuberculosis complications on inactive pulmonary tuberculosis background witnesses with 85.3% probability the HIV-infectioning of the patient.

  14. Clinical and epidemiological profile of elderly patients with Chagas disease followed between 2005-2013 by pharmaceutical care service in Ceará State, Northeastern Brazil.

    PubMed

    Pereira, Laíse dos Santos; Freitas, Erlane Chaves; Fidalgo, Arduína Sofia Ortet de Barros Vasconcelos; Andrade, Mônica Coelho; Cândido, Darlan da Silva; da Silva Filho, José Damião; Michailowsky, Vladimir; Oliveira, Maria de Fátima; Queiroz, José Ajax Nogueira

    2015-01-01

    By controlling the transmission of Chagas disease, the challenge of providing assistance to millions of infected patients that reach old age arises. In this study, the socioeconomic, demographic and comorbidity records of all elderly chagasic patients followed at the Pharmaceutical Care Service of the Chagas Disease Research Laboratory were assessed. The information related to the clinical form of the disease was obtained from medical records provided by the Walter Cantídio University Hospital. The profile of the studied population was: women (50.5%); mean age of 67 years; retired (54.6%); married (51.6 %); high illiteracy rate (40.2%); and family income equal to the minimum wage (51.5%). The predominant clinical forms of Chagas disease were cardiac (65.3%) and indeterminate (14.7%). The main electrocardiographic changes were the right bundle branch block (41.0%), associated or not with the anterosuperior left bundle branch block (27.4%). The average number of comorbidities per patient was 2.23 ± 1.54, with systemic arterial hypertension being the main one found (67.0%). It was found that the elderly comprise a vulnerable group of patients that associate aging with cardiac and/or digestive disorders resulting from the evolution of Chagas disease and other comorbidities, which requires special attention from health services to ensure more appropriate medical and social care. PMID:25923894

  15. CLINICAL AND EPIDEMIOLOGICAL PROFILE OF ELDERLY PATIENTS WITH CHAGAS DISEASE FOLLOWED BETWEEN 2005-2013 BY PHARMACEUTICAL CARE SERVICE IN CEARÁ STATE, NORTHEASTERN BRAZIL

    PubMed Central

    PEREIRA, Laíse dos Santos; FREITAS, Erlane Chaves; FIDALGO, Arduína Sofia Ortet de Barros Vasconcelos; ANDRADE, Mônica Coelho; CÂNDIDO, Darlan da Silva; da SILVA, José Damião; MICHAILOWSKY, Vladimir; OLIVEIRA, Maria de Fátima; QUEIROZ, José Ajax Nogueira

    2015-01-01

    By controlling the transmission of Chagas disease, the challenge of providing assistance to millions of infected patients that reach old age arises. In this study, the socioeconomic, demographic and comorbidity records of all elderly chagasic patients followed at the Pharmaceutical Care Service of the Chagas Disease Research Laboratory were assessed. The information related to the clinical form of the disease was obtained from medical records provided by the Walter Cantídio University Hospital. The profile of the studied population was: women (50.5%); mean age of 67 years; retired (54.6%); married (51.6 %); high illiteracy rate (40.2%); and family income equal to the minimum wage (51.5%). The predominant clinical forms of Chagas disease were cardiac (65.3%) and indeterminate (14.7%). The main electrocardiographic changes were the right bundle branch block (41.0%), associated or not with the anterosuperior left bundle branch block (27.4%). The average number of comorbidities per patient was 2.23 ± 1.54, with systemic arterial hypertension being the main one found (67.0%). It was found that the elderly comprise a vulnerable group of patients that associate aging with cardiac and/or digestive disorders resulting from the evolution of Chagas disease and other comorbidities, which requires special attention from health services to ensure more appropriate medical and social care. PMID:25923894

  16. Clinical Impact of Education Provision on Determining Advance Care Planning Decisions among End Stage Renal Disease Patients Receiving Regular Hemodialysis in University Malaya Medical Centre

    PubMed Central

    Hing (Wong), Albert; Chin, Loh Ee; Ping, Tan Li; Peng, Ng Kok; Kun, Lim Soo

    2016-01-01

    Introduction: Advance care planning (ACP) is a process of shared decision-making about future health-care plans between patients, health care providers, and family members, should patients becomes incapable of participating in medical treatment decisions. ACP discussions enhance patient's autonomy, focus on patient's values and treatment preferences, and promote patient-centered care. ACP is integrated as part of clinical practice in Singapore and the United States. Aim: To assess the clinical impact of education provision on determining ACP decisions among end-stage renal disease patients on regular hemodialysis at University Malaya Medical Centre (UMMC). To study the knowledge and attitude of patients toward ACP and end-of-life issues. Materials and Methods: Fifty-six patients were recruited from UMMC. About 43 questions pretest survey adapted from Lyon's ACP survey and Moss's cardiopulmonary resuscitation (CPR) attitude survey was given to patients to answer. An educational brochure is then introduced to these patients, and a posttest survey carried out after that. The results were analyzed using SPSS version 22.0. Results: Opinion on ACP, including CPR decisions, showed an upward trend on the importance percentage after the educational brochure exposure, but this was statistically not significant. Seventy-five percent of participants had never heard of ACP before, and only 3.6% had actually prepared a written advanced directive. Conclusion: The ACP educational brochure clinically impacts patients’ preferences and decisions toward end-of-life care; however, this is statistically not significant. Majority of patients have poor knowledge on ACP. This study lays the foundation for execution of future larger scale clinical trials, and ultimately, the incorporation of ACP into clinical practice in Malaysia. PMID:27803566

  17. [Charcot-Marie-Tooth disease associated with periaxin mutations (CMT4F): Clinical, electrophysiological and genetic analysis of 24 patients].

    PubMed

    Renouil, M; Stojkovic, T; Jacquemont, M L; Lauret, K; Boué, P; Fourmaintraux, A; Randrianaivo, H; Tallot, M; Mignard, D; Roelens, P; Tabailloux, D; Bernard, R; Cartault, F; Chane-Thien, E; Dubourg, O; Ferrer, X; Sole, G; Fournier, E; Latour, P; Lacour, A; Mignard, C

    2013-01-01

    Autosomal recessive Charcot-Marie-Tooth disease (AR-CMT) is often characterized by onset in early childhood and severe phenotype compared to the dominant forms. CMT disease associated with periaxin gene (PRX) is rare and characterized by demyelination limited to the major peripheral nerves. Following the discovery of a high frequency of a specific periaxin gene mutation (E1085fsX4 homozygote) in the Reunion Island, we examined all French patients known as carriers of the periaxin gene mutation. There were 24 patients. Eighteen were from the Reunion Island (6 families and 10 sporadic cases). The six remaining patients were in two families, each with two affected individuals, and two sporadic cases. The series included 17 female and seven male patients. Walking was acquired late, on average at 3.4±1.6 years. One patient never learned to walk. The Charcot Marie Tooth Neuropathy Score (CMTNS) averaged 24.5±8.1. Seven patients had been wheelchair-bound since the age of 24±22. Other symptoms were: scoliosis most often observed after the age of 12 years and sometimes complicated by a restrictive respiratory syndrome; foot deformity in 24 patients; strabismus; glaucoma; myopia. When conduction recordings are available, median nerve motor conduction was slow (<10m/s), associated with a major lengthening of distal latencies. Study of the periaxin gene should be considered in patients with severe demyelinating neuropathy associated with early infantile scoliosis. This disease leads to major disability (29% of patients in this series were wheelchair-bound) and to respiratory insufficiency. Genetic counselling is highly recommended for consanguineous families.

  18. Using an electronic self-management tool to support patients with chronic kidney disease (CKD): a CKD clinic self-care model.

    PubMed

    Ong, Stephanie W; Jassal, Sarbjit V; Porter, Eveline; Logan, Alexander G; Miller, Judith A

    2013-01-01

    New healthcare delivery models are needed to enhance the patient experience and improve quality of care for individuals with chronic conditions such as kidney disease. One potential avenue is to implement self-management strategies. There is growing evidence that self-management interventions help optimize various aspects of chronic disease management. With the increasing use of information technology (IT) in health care, chronic disease management programs are incorporating IT solutions to support patient self-management practices. IT solutions have the ability to promote key principles of self-management, namely education, empowerment, and collaboration. Positive clinical outcomes have been demonstrated for a number of chronic conditions when IT solutions were incorporated into self-management programs. There is a paucity of evidence for self-management in chronic kidney disease (CKD) patients. Furthermore, IT strategies have not been tested in this patient population to the same extent as other chronic conditions (e.g., diabetes, hypertension). Therefore, it is currently unknown if IT strategies will promote self-management behaviors and lead to improvements in overall patient care. We designed and developed an IT solution called My KidneyCare Centre to support self-management strategies for patients with CKD. In this review, we discuss the rationale and vision of incorporating an electronic self-management tool to support the care of patients with CKD.

  19. Gastroesophageal reflux disease: clinical features.

    PubMed

    Pettit, Michael

    2005-12-01

    Gastroesophageal reflux disease (GERD) is a chronic disease affecting up to 40% of people in the Western world. Risk factors associated with GERD include age and lifestyle habits, although the clinically relevant contribution of many of these factors is unclear. In GERD, refluxed gastric acid damages the oesophageal mucosa, generally when the pH falls below 4. GERD patients present a variety of symptoms, most commonly heartburn and regurgitation. Oesophageal complications associated with GERD include erosions, ulcers, peptic strictures, and Barrett's oesophagus which is implicated in the development of oesophageal adenocarcinoma. Diagnosis of GERD is problematic due to the range of symptoms which may be presented to the physician and symptom severity is frequently unrelated to disease severity. While endoscopic monitoring may be used to assess the presence and severity of GERD, a lack of visible damage does not necessarily indicate an absence of GERD. Techniques used to diagnose GERD include addition of an acid solution into the oesophagus in order to replicate symptoms (Bernstein test) or 24-hour intra-oesophageal pH monitoring. Proton pump inhibitors are effective in the treatment of GERD, acting to reduce the acidity of the gastric juice and hence reduce oesophageal damage and symptoms associated with GERD. Symptoms most indicative of GERD are those associated with erosive oesophagitis, including heartburn and acid regurgitation. Less common GERD-associated symptoms include chest pain, a range of ear, nose and throat conditions, and asthma. In contrast to perceptions of the disease as 'merely' heartburn, the impact on patients' quality of life can be profound. Increasing awareness of GERD by health care professionals has led to improved diagnosis and a greater appreciation of the need for maintenance therapy.

  20. The evaluation of clinical therapy effects of oral western medicine combined with magnetic pulse acupoint stimulation in treating elderly patients with coronary heart disease

    PubMed Central

    Fu, Xin; Guo, Li; Jiang, Zheng-Ming; Xu, Ai-Guo

    2015-01-01

    Objective: Treat the patients suffered from coronary heart disease with oral western medicine, combining with magnetic pulse acupoint stimulation, and observe the therapeutic effects of such combination therapy method. Methods: 56 old people with coronary heart disease are randomly divided into a treatment group and a control group. Both groups of patients are treated by the routine drugs, in addition, the patients of the treatment group are treated by magnetic pulse therapy additionally. Compare clinical symptoms, blood lipid and blood rheological indexes of the patients in the two groups when they are selected and after 30 days’ treatment. Results: after 30 days’ treatment, it is found that clinical symptoms, blood lipid and blood rheological indexes of the patients in the treatment group are significantly improved compared with those when they are selected and those of the control group (P<0.05). Conclusion: patients with coronary heart disease, treated by pulsed magnetic therapy and the conventional drug intervention, had relieved synptom, improve blood lipid and heart blood supply function. PMID:26309664

  1. The clinical significance of EBV DNA in the plasma and peripheral blood mononuclear cells of patients with or without EBV diseases.

    PubMed

    Kanakry, Jennifer A; Hegde, Aparna M; Durand, Christine M; Massie, Allan B; Greer, Amy E; Ambinder, Richard F; Valsamakis, Alexandra

    2016-04-21

    Epstein-Barr virus (EBV) is a ubiquitous virus that establishes a latent infection within the host and in some cases can lead to the development of EBV-associated lymphomas, lymphoproliferative disorders, hemophagocytic lymphohistiocytosis, solid tumors, and other diseases. We studied the clinical significance of detecting EBV DNA in the plasma and peripheral blood mononuclear cells (PBMCs) of 2146 patients who had blood specimens sent to the Johns Hopkins Hospital clinical laboratory for viral quantitative real-time polymerase chain reaction assay over a 5-year period. Within this largely immunocompromised and hospitalized cohort, 535 patients (25%) had EBV detected in plasma or PBMCs. When EBV was detected in the absence of an EBV(+)disease (n = 402), it was present only in PBMCs in 69% of cases. Immunocompromised patients were less likely to have EBV in plasma than in PBMCs in the absence of EBV(+)disease. In patients with active, systemic EBV(+)diseases (n = 105), EBV was detected in plasma in 99% of cases but detected in PBMCs in only 54%. Across a range of copy number cutoffs, EBV in plasma had higher specificity and sensitivity for EBV(+)disease as compared with EBV in PBMCs. EBV copy number in plasma distinguished untreated, EBV(+)lymphoma from EBV(+)lymphoma in remission and EBV(-)lymphoma, and also distinguished untreated, EBV(+)posttransplantation lymphoproliferative disorder (PTLD) from EBV(+)PTLD in remission and EBV(-)PTLD. EBV copy number quantification is a useful diagnostic marker across the spectrum of EBV(+)diseases, even among immunocompromised patients, with plasma specimens more indicative of EBV(+)disease than PBMCs.

  2. Observational clinical study of 22 adult-onset Pompe disease patients undergoing enzyme replacement therapy over 5years.

    PubMed

    Stepien, Karolina M; Hendriksz, Christian J; Roberts, Mark; Sharma, Reena

    2016-04-01

    Pompe disease is an autosomal recessive disease resulting from deficiency of the acid alpha-glucosidase (GAA). The late-onset Pompe Disease (LOPD) patients develop muscular and respiratory complications later in life. We describe a retrospective observational cohort study including 22 patients with LOPD. The cohort was assessed at baseline before Enzyme Replacement Therapy (ERT) with alglucosidase alpha (20mg/kg biweekly) was commenced and subsequently relevant information was collected at 2, 4 and 5years later. The median age of the patients at study entry was 44years (16-64years), with median disease duration of 11.5years (4-31years). At baseline, 10 patients (45%) could walk without support, 12 (55%) could walk with unilateral or bilateral support including 3/12 were wheelchair bound. Mean predicted FVC % was 55.7 (95% CI 45-66) of predicted normal at baseline and showed no significant change after 5years (54.6 (95% CI 43-66)), (all p=0.9815). Mean FVC % supine was 41.8 (95% CI 33.8-49) of predicted normal at baseline and remained significantly unchanged at 5years (48.4 (95% CI 37-59.6)), (all p=0.8680). The overnight non-invasive ventilator dependence increased by 18.2% as compared with baseline and requirement of mobility aids increased during this period by 5.2% as compared with the baseline. Mean walking distance at 6min walk test was 411.5 (95% CI 338-485) at baseline, 266.5 (95% CI 187-346) m at 2years, 238.6 (95% CI 162-315) m at 4years and 286.8 (95% CI 203-370) m at 5years (p=0.1981; ANOVA was completed only for 14 patients). A gradual decline in FVC% predicted was noted only in four cases and a decline in FVC% supine in two other. Only one patient showed a decline in both pulmonary function tests. In all remaining cases (17/22) respiratory function remains stable. In conclusion overall pulmonary function tests and mobility remained stable for 5years in majority of patients on ERT. However, in some patients they continued to decline in spite of ERT

  3. The clinical spectrum of hexosaminidase deficiency diseases.

    PubMed

    Johnson, W G

    1981-11-01

    Hexosaminidase deficiency diseases or GM2-gangliosidoses were originally described as infantile encephalopathies. Recently, hexosaminidase deficiencies have been found with different phenotypes, including juvenile and adult encephalopathies, cerebellar ataxias, and motor neuron diseases. Individual cases have resembled Ramsey-Hunt syndrome, olivopontocerebellar ataxia, Friedreich ataxia, amyotrophic lateral sclerosis, Kugelberg-Welander disease, Fazio-Londe disease, and Charcot-Marie-Tooth disease. Tremor, dystonia, spastic paresis, and psychosis have been seen. Since few diagnosable causes for these system atrophies are known, these patients should be tested for hexosaminidase deficiency. These recessive disorders fit a multiple loci/multiple alleles genetic scheme, and a clinical genetic classification is presented.

  4. Safety of the transpulmonary ultrasound contrast agent NC100100: a clinical and haemodynamic evaluation in patients with suspected or proved coronary artery disease

    PubMed Central

    Myreng, Y; Molstad, P; Ytre-Arne, K; Aas, M; Stoksflod, L; Nossen, J; Oftedal, B

    1999-01-01

    OBJECTIVE—To evaluate the clinical and haemodynamic safety of NC100100, a new transpulmonary ultrasound contrast agent intended for vascular use.
DESIGN—Pulmonary artery pressures and gas exchange, left ventricular and systemic blood pressure and ECG were measured at baseline, after saline injection, and after each of two increasing doses of NC100100 injected intravenously.
PATIENTS—30 patients who were evaluated for suspected coronary artery disease.
RESULTS—No change was detected in any of the haemodynamic variables, or in haematological or clinical chemical parameters. Blood gases were unchanged, as were heart rhythm and arterial oxygen saturation. No serious adverse reactions were reported.
CONCLUSIONS—NC100100 appeared to be haemodynamically inert and safe in patients with coronary artery disease.


Keywords: NC100100; ultrasound; safety PMID:10455084

  5. Home-based pulmonary rehabilitation improves clinical features and systemic inflammation in chronic obstructive pulmonary disease patients

    PubMed Central

    do Nascimento, Eloisa Sanches Pereira; Sampaio, Luciana Maria Malosá; Peixoto-Souza, Fabiana Sobral; Dias, Fernanda Dultra; Gomes, Evelim Leal Freitas Dantas; Greiffo, Flavia Regina; Ligeiro de Oliveira, Ana Paula; Stirbulov, Roberto; Vieira, Rodolfo Paula; Costa, Dirceu

    2015-01-01

    Chronic obstructive pulmonary disease (COPD) is a respiratory disease characterized by chronic airflow limitation that leads beyond the pulmonary changes to important systemic effects. COPD is characterized by pulmonary and systemic inflammation. However, increases in the levels of inflammatory cytokines in plasma are found even when the disease is stable. Pulmonary rehabilitation improves physical exercise capacity and quality of life and decreases dyspnea. The aim of this study was to evaluate whether a home-based pulmonary rehabilitation (HBPR) program improves exercise tolerance in COPD patients, as well as health-related quality of life and systemic inflammation. This prospective study was conducted at the Laboratory of Functional Respiratory Evaluation, Nove de Julho University, São Paulo, Brazil. After anamnesis, patients were subjected to evaluations of health-related quality of life and dyspnea, spirometry, respiratory muscle strength, upper limbs incremental test, incremental shuttle walk test, and blood test for quantification of systemic inflammatory markers (interleukin [IL]-6 and IL-8). At the end of the evaluations, patients received a booklet containing the physical exercises to be performed at home, three times per week for 8 consecutive weeks. Around 25 patients were enrolled, and 14 completed the pre- and post-HBPR ratings. There was a significant increase in the walked distance and the maximal inspiratory pressure, improvements on two components from the health-related quality-of-life questionnaire, and a decrease in plasma IL-8 levels after the intervention. The HBPR is an important and viable alternative to pulmonary rehabilitation for the treatment of patients with COPD; it improves exercise tolerance, inspiratory muscle strength, quality of life, and systemic inflammation in COPD patients. PMID:25848241

  6. The Clinical and Economic Impact of Exacerbations of Chronic Obstructive Pulmonary Disease: A Cohort of Hospitalized Patients

    PubMed Central

    Blasi, Francesco; Cesana, Giancarlo; Conti, Sara; Chiodini, Virginio; Aliberti, Stefano; Fornari, Carla; Mantovani, Lorenzo Giovanni

    2014-01-01

    Background Chronic Obstructive Pulmonary Disease (COPD) is a common disease with significant health and economic consequences. This study assesses the burden of COPD in the general population, and the influence of exacerbations (E-COPD) on disease progression and costs. Methods This is a secondary data analysis of healthcare administrative databases of the region of Lombardy, in northern Italy. The study included ≥ 40 year-old patients hospitalized for a severe E-COPD (index event) during 2006. Patients were classified in relation to the number and type of E-COPD experienced in a three-year pre-index period. Subjects were followed up until December 31st, 2009, collecting data on healthcare resource use and vital status. Results 15857 patients were enrolled –9911 males, mean age: 76 years (SD 10). Over a mean follow-up time of 2.4 years (1.36), 81% of patients had at least one E-COPD with an annual rate of 3.2 exacerbations per person-year and an all-cause mortality of 47%. A history of exacerbation influenced the occurrence of new E-COPD and mortality after discharge for an E-COPD. On average, the healthcare system spent 6725€ per year per person (95%CI 6590–6863). Occurrence and type of exacerbations drove the direct healthcare cost. Less than one quarter of patients presented claims for pulmonary function tests. Conclusions COPD imposes a substantial burden on healthcare systems, mainly attributable to the type and occurrence of E-COPD, or in other words, to the exacerbator phenotypes. A more tailored approach to the management of COPD patients is required. PMID:24971791

  7. Home-based pulmonary rehabilitation improves clinical features and systemic inflammation in chronic obstructive pulmonary disease patients.

    PubMed

    do Nascimento, Eloisa Sanches Pereira; Sampaio, Luciana Maria Malosá; Peixoto-Souza, Fabiana Sobral; Dias, Fernanda Dultra; Gomes, Evelim Leal Freitas Dantas; Greiffo, Flavia Regina; Ligeiro de Oliveira, Ana Paula; Stirbulov, Roberto; Vieira, Rodolfo Paula; Costa, Dirceu

    2015-01-01

    Chronic obstructive pulmonary disease (COPD) is a respiratory disease characterized by chronic airflow limitation that leads beyond the pulmonary changes to important systemic effects. COPD is characterized by pulmonary and systemic inflammation. However, increases in the levels of inflammatory cytokines in plasma are found even when the disease is stable. Pulmonary rehabilitation improves physical exercise capacity and quality of life and decreases dyspnea. The aim of this study was to evaluate whether a home-based pulmonary rehabilitation (HBPR) program improves exercise tolerance in COPD patients, as well as health-related quality of life and systemic inflammation. This prospective study was conducted at the Laboratory of Functional Respiratory Evaluation, Nove de Julho University, São Paulo, Brazil. After anamnesis, patients were subjected to evaluations of health-related quality of life and dyspnea, spirometry, respiratory muscle strength, upper limbs incremental test, incremental shuttle walk test, and blood test for quantification of systemic inflammatory markers (interleukin [IL]-6 and IL-8). At the end of the evaluations, patients received a booklet containing the physical exercises to be performed at home, three times per week for 8 consecutive weeks. Around 25 patients were enrolled, and 14 completed the pre- and post-HBPR ratings. There was a significant increase in the walked distance and the maximal inspiratory pressure, improvements on two components from the health-related quality-of-life questionnaire, and a decrease in plasma IL-8 levels after the intervention. The HBPR is an important and viable alternative to pulmonary rehabilitation for the treatment of patients with COPD; it improves exercise tolerance, inspiratory muscle strength, quality of life, and systemic inflammation in COPD patients.

  8. Patient Recruitment into a Multicenter Randomized Clinical Trial for Kidney Disease: Report of the Focal Segmental Glomerulosclerosis Clinical Trial (FSGS CT)

    PubMed Central

    Ferris, Maria; Norwood, Victoria; Radeva, Milena; Al-Uzri, Amira; Askenazi, David; Matoo, Tej; Pinsk, Maury; Sharma, Amita; Smoyer, William; Stults, Jenna; Vyas, Shefali; Weiss, Robert; Gipson, Debbie; Kaskel, Frederick; Friedman, Aaron; Moxey-Mims, Marva; Trachtman, Howard

    2015-01-01

    We describe the experience of the focal segmental glomerulosclerosis clinical trial (FSGS CT) in the identification and recruitment of participants into the study. This National Institutes of Health funded study, a multicenter open-label, randomized comparison of cyclosporine versus oral dexamethasone pulses plus mycophenolate mofetil, experienced difficulty and delays meeting enrollment goals. These problems occurred despite the support of patient advocacy groups and aggressive recruitment strategies. Multiple barriers were identified including: (1) inaccurate estimates of the number of potential incident FSGS patients at participating centers; (2) delays in securing one of the test agents; (3) prolonged time between IRB approval and execution of a subcontract (mean 7.5 ± 0.8 months); (4) prolonged time between IRB approval and enrollment of the first patient at participating sites (mean 19.6 ± 1.4 months); and (5) reorganization of clinical coordinating core infrastructure to align resources with enrollment. A web-based anonymous survey of site investigators revealed site-related barriers to patient recruitment. The value of a variety of recruitment tools was of marginal utility in facilitating patient enrollment. We conclude that improvements in the logistics of study approval and regulatory start-up and testing promising novel agents are important factors in promoting enrollment into randomized clinical trials in nephrology. PMID:23399084

  9. Clinical disease activity and acute phase reactant levels are discordant among patients with active rheumatoid arthritis: acute phase reactant levels contribute separately to predicting outcome at one year

    PubMed Central

    2014-01-01

    Introduction Clinical trials of new treatments for rheumatoid arthritis (RA) typically require subjects to have an elevated acute phase reactant (APR), in addition to tender and swollen joints. However, despite the elevation of individual components of the Clinical Disease Activity Index (CDAI) (tender and swollen joint counts and patient and physician global assessment), some patients with active RA may have normal erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) levels and thus fail to meet entry criteria for clinical trials. We assessed the relationship between CDAI and APRs in the Consortium of Rheumatology Researchers of North America (CORRONA) registry by comparing baseline characteristics and one-year clinical outcomes of patients with active RA, grouped by baseline APR levels. Methods This was an observational study of 9,135 RA patients who had both ESR and CRP drawn and a visit at which CDAI was >2.8 (not in remission). Results Of 9,135 patients with active RA, 58% had neither elevated ESR nor CRP; only 16% had both elevated ESR and CRP and 26% had either ESR or CRP elevated. Among the 4,228 patients who had a one-year follow-up visit, both baseline and one-year follow-up modified Health Assessment Questionnaire (mHAQ) and CDAI scores were lowest for patients with active RA but with neither APR elevated; both mHAQ and CDAI scores increased sequentially with the increase in number of elevated APR levels at baseline. Each individual component of the CDAI followed the same trend, both at baseline and at one-year follow-up. The magnitude of improvement in both CDAI and mHAQ scores at one year was associated positively with the number of APRs elevated at baseline. Conclusions In a large United States registry of RA patients, APR levels often do not correlate with disease activity as measured by joint counts and global assessments. These data strongly suggest that it is appropriate to obtain both ESR and CRP from RA patients at the initial

  10. Information for patients about inflammatory bowel disease.

    PubMed

    Mansfield, J C; Tanner, A R; Bramble, M G

    1997-01-01

    In inflammatory bowel disease it is important that patients understand their condition since this helps to improve long-term management of the disease. The aim of this study was to assess the information given to patients with inflammatory bowel disease about their condition, its treatment and the National Association for Colitis and Crohn's disease. Two surveys were performed, using anonymous questionnaires. One was of all association members in north-east England, the other was a sample of patients attending medical outpatients. The surveys showed that more patients heard of the National Association for Colitis and Crohn's disease from the media than from medical sources. Of patients seen in medical clinics, 75% would welcome more information about their disease. In four of the six participating centres less than half the patients had been told about the existence of a patients' association. There was considerable variation in the instructions on what action to take in the event of a relapse. These findings suggest that the opportunity offered by out-patient clinics to educate and inform patients is often wasted. Clinicians often neglect to mention the National Association for Colitis and Crohn's disease, especially to patients with long-standing disease. A higher priority should be given to providing patients with appropriate information on inflammatory bowel disease. Three simple audit standards for the organisation of outpatient clinic information are proposed. PMID:9131520

  11. High Resistance to Azithromycin in Clinical Samples from Patients with Sexually Transmitted Diseases in Guangxi Zhuang Autonomous Region, China

    PubMed Central

    Zhu, Bangyong; Li, Wei; Zhang, Jie; Huang, Geng; Cao, Juan; Tang, Zhongshu; Gan, Quan; Wei, Pingjiang

    2016-01-01

    Azithromycin is used as an alternative medicine in patients with syphilis who are intolerant to penicillin. Nevertheless, the report of treatment failure of azithromycin for patients with syphilis has raised concerns in China in the past years. In this study, 178 patients with early syphilis, who were treated in sexually transmitted infections clinics in four cities in Guangxi Zhuang Autonomous Region were enrolled to investigate the regional prevalence of Treponema pallidum strain resistant to azithromycin. Nested PCR was performed to amplify the 23S ribosomal RNA (23SrRNA) gene. The point mutation of A2058G in 23SrRNA, which confers Treponema pallidum resistance to azithromycin, was measured by endonuclease digestion of PCR amplification products using MboII. A2058G point mutation was detected in 91.0% (162/178; 95% CI, 86.8%, 95.2%) of the specimens, but no difference in prevalence of azithromycin resistance was found between the patients who had taken antibiotics before enrollment and the patients who had not (91.8% vs. 89.4%), nor between the patients with and without past sexually transmitted infections (87.1% vs. 93.1%). We concluded that azithromycin may not be suitable for syphilis as a treatment option in Guangxi Zhuang Autonomous Region because of the extremely high prevalence of resistance in the general syphilis population. PMID:27467164

  12. High Resistance to Azithromycin in Clinical Samples from Patients with Sexually Transmitted Diseases in Guangxi Zhuang Autonomous Region, China.

    PubMed

    Zhu, Bangyong; Bu, Jin; Li, Wei; Zhang, Jie; Huang, Geng; Cao, Juan; Tang, Zhongshu; Gan, Quan; Wei, Pingjiang

    2016-01-01

    Azithromycin is used as an alternative medicine in patients with syphilis who are intolerant to penicillin. Nevertheless, the report of treatment failure of azithromycin for patients with syphilis has raised concerns in China in the past years. In this study, 178 patients with early syphilis, who were treated in sexually transmitted infections clinics in four cities in Guangxi Zhuang Autonomous Region were enrolled to investigate the regional prevalence of Treponema pallidum strain resistant to azithromycin. Nested PCR was performed to amplify the 23S ribosomal RNA (23SrRNA) gene. The point mutation of A2058G in 23SrRNA, which confers Treponema pallidum resistance to azithromycin, was measured by endonuclease digestion of PCR amplification products using MboII. A2058G point mutation was detected in 91.0% (162/178; 95% CI, 86.8%, 95.2%) of the specimens, but no difference in prevalence of azithromycin resistance was found between the patients who had taken antibiotics before enrollment and the patients who had not (91.8% vs. 89.4%), nor between the patients with and without past sexually transmitted infections (87.1% vs. 93.1%). We concluded that azithromycin may not be suitable for syphilis as a treatment option in Guangxi Zhuang Autonomous Region because of the extremely high prevalence of resistance in the general syphilis population. PMID:27467164

  13. Impact of circulating cathepsin K on the coronary calcification and the clinical outcome in chronic kidney disease patients.

    PubMed

    Izumi, Yusuke; Hayashi, Mutsuharu; Morimoto, Ryota; Cheng, Xian Wu; Wu, Hongxian; Ishii, Hideki; Yasuda, Yoshinari; Yoshikawa, Daiji; Izawa, Hideo; Matsuo, Seiichi; Oiso, Yutaka; Murohara, Toyoaki

    2016-01-01

    Chronic kidney disease (CKD) is a cause of coronary artery calcification (CAC) and an independent predictor of major adverse cardiac and cerebrovascular events (MACCE). Cathepsin K (CatK) is a lysosomal cysteine protease which affects vascular calcification and glucose metabolism disorder. We investigated the relationships among CatK, CAC, diabetes mellitus (DM) and MACCE in CKD patients. 113 consecutive CKD patients were enrolled. Their CAC was evaluated by computed tomography. Their plasma CatK level was measured by ELISA. They were divided into two groups by CatK levels and followed up for up to 3 years. The impact of CatK was analyzed in all participants, diabetic patients and non-diabetic patients. Kaplan-Meier analysis demonstrated a significant higher incidence of MACCE in the high CatK group (P = 0.028). The CatK level was significantly higher in patients with MACCE compared to that in patients without MACCE (P = 0.034). Cox's model revealed the higher plasma CatK and BNP level as independent predictors of MACCE (P = 0.043 and P < 0.01, respectively). Only in non-diabetic patients, there was a significant correlation between CatK and CAC score, and high CatK group had a significant higher level of LDL-C and LDL-C/HDL-C ratio (P < 0.05 and P < 0.001, respectively) than low CatK group. And these lipid disorders were independent predictors of CatK elevation. In CKD patients, our results indicated an impact of higher CatK level on their MACCE. The significant association among the CatK level, CAC and MACCE was found in non-diabetic CKD patients.

  14. Leucocyte complement receptor 1 (CR1/CD35) transcript and its correlation with the clinical disease activity in rheumatoid arthritis patients

    PubMed Central

    Anand, D; Kumar, U; Kanjilal, M; Kaur, S; Das, N

    2014-01-01

    In view of the exaggerated complement activation in rheumatoid arthritis (RA) and significance of complement receptor 1 (CR1/CD35) as a complement regulatory protein (CRP), we aimed to determine the leucocyte-complement receptor 1 (L-CR1) transcript levels and the relationship of this protein with the clinical disease activity of RA patients. Sixty-six controls and 45 RA patients were enrolled. L-CR1 transcript levels were correlated with the levels of circulating immune complexes (CIC), C3, C4 and C3d in controls and patients and with disease activity score 28 (DAS28) in patients only. CIC levels were determined by polyethylene glycol (PEG) precipitation, C3 and C4 levels by nephlometry and C3d levels by enzyme-linked immunosorbent assay (ELISA). Eleven patients were recruited for follow-up of L-CR1 and DAS28 levels at weeks 0, 12 and 24. Appropriate statistical methods were used for the data analysis. L-CR1 (P < 0·01) transcript levels were decreased in patients compared to controls. L-CR1 levels correlated negatively with DAS28, CIC and C3d. DAS28 correlated positively with levels of CIC, C3 and C3d. Levels of CIC correlated positively with C3 and C3d. Levels of C3 correlated positively with C3d in patients and with C4 in both controls and patients. Levels of L-CR1 increased with decline in DAS28 scores in follow-up patients. Observations were statistically significant. Lower levels of L-CR1 transcript in patients compared to controls, their correlations with the levels of CIC, C3d and DAS28 at different time-points in RA patients suggest CR1 as a potential disease marker for RA. PMID:24433281

  15. Health-related quality of life in patients with pediatric onset of end-stage renal disease: state of the art and recommendations for clinical practice.

    PubMed

    Tjaden, Lidwien A; Grootenhuis, Martha A; Noordzij, Marlies; Groothoff, Jaap W

    2016-10-01

    Health-related quality of life (HRQoL) is increasingly recognized as a key outcome in both clinical and research settings in the pediatric population with end-stage renal disease (ESRD). This review aims to: (1) summarize the current knowledge on HRQoL and socioprofessional outcomes and (2) provide strategies for incorporation of HRQoL assessment into clinical practice. Studies report that pediatric patients with ESRD have significantly lower HRQoL scores compared with children with other chronic diseases. Patients treated by dialysis are at particularly high risk for impaired HRQoL. Furthermore, patients more often have impaired neurocognitive functioning and lower academic achievement. Important determinants of impaired HRQoL include medical factors (i.e., receiving dialysis, disabling comorbidities, cosmetic side effects, stunted growth), sociodemographic factors (i.e., female gender, non-Western background) and psychosocial factors (i.e., noneffective coping strategies). Contrary to the situation in childhood, adult survivors of pediatric ESRD report a normal mental HRQoL. Despite this subjective feeling of well-being, these patients have on average experienced significantly more difficulties in completing their education, developing intimate relationships, and securing employment. Several medical and psychosocial strategies may potentially improve HRQoL in children with ESRD. Regular assessment of HRQoL and neurocognitive functioning in order to identify areas in which therapies and interventions may be required should be part of standard clinical care.

  16. Clinical management of critically ill patients with Cushing's disease due to ACTH-secreting pituitary macroadenomas: effectiveness of presurgical treatment with pasireotide.

    PubMed

    Cannavo, S; Messina, E; Albani, A; Ferrau, F; Barresi, V; Priola, S; Esposito, F; Angileri, F

    2016-06-01

    The management of critically ill Cushing's disease (CD) patients is extremely challenging. Pasireotide is indicated for the treatment of CD patients when pituitary surgery is unfeasible or has not been curative, but no data are available about the use of this drug as pre-operative treatment in critically ill patients. We report the effects of presurgical pasireotide therapy in CD patients in whom hypercortisolism caused life-threatening hypokalemia, alkalosis, and cardio-respiratory complications precluding surgical approach. Clinical, biochemical, and radiological data of two critically ill patients with ACTH-secreting pituitary macroadenoma, before and during first-line presurgical pasireotide treatment (600 μg s.c. bid). During the first 21 days of treatment, pasireotide therapy induced a rapid, partial decrease of plasma ACTH, serum cortisol, and urinary free cortisol levels, with the consequent normalization of serum potassium concentration and arterial blood gases parameters, in both the patients. They did not experience unmanageable side effects and underwent endoscopic transsphenoidal surgery after 4 weeks of effective treatment. Pre-operative MRI evaluation did not show pituitary tumor shrinkage. Surgical cure of CD was obtained in the first patient, while debulking allowed the pharmacological control of hypercortisolism in the second case. We suggest that pasireotide can induce a rapid improvement of clinical and metabolic conditions in critically ill CD patients in whom surgical approach is considered hazardous and need to be delayed.

  17. Selected items from the Charcot-Marie-Tooth (CMT) Neuropathy Score and secondary clinical outcome measures serve as sensitive clinical markers of disease severity in CMT1A patients.

    PubMed

    Mannil, Manoj; Solari, Alessandra; Leha, Andreas; Pelayo-Negro, Ana L; Berciano, José; Schlotter-Weigel, Beate; Walter, Maggie C; Rautenstrauss, Bernd; Schnizer, Tuuli J; Schenone, Angelo; Seeman, Pavel; Kadian, Chandini; Schreiber, Olivia; Angarita, Natalia G; Fabrizi, Gian Maria; Gemignani, Franco; Padua, Luca; Santoro, Lucio; Quattrone, Aldo; Vita, Giuseppe; Calabrese, Daniela; Young, Peter; Laurà, Matilde; Haberlová, Jana; Mazanec, Radim; Paulus, Walter; Beissbarth, Tim; Shy, Michael E; Reilly, Mary M; Pareyson, Davide; Sereda, Michael W

    2014-11-01

    This study evaluates primary and secondary clinical outcome measures in Charcot-Marie-Tooth disease type 1A (CMT1A) with regard to their contribution towards discrimination of disease severity. The nine components of the composite Charcot-Marie-Tooth disease Neuropathy Score and six additional secondary clinical outcome measures were assessed in 479 adult patients with genetically proven CMT1A and 126 healthy controls. Using hierarchical clustering, we identified four significant clusters of patients according to clinical severity. We then tested the impact of each of the CMTNS components and of the secondary clinical parameters with regard to their power to differentiate these four clusters. The CMTNS components ulnar sensory nerve action potential (SNAP), pin sensibility, vibration and strength of arms did not increase the discriminant value of the remaining five CMTNS components (Ulnar compound motor action potential [CMAP], leg motor symptoms, arm motor symptoms, leg strength and sensory symptoms). However, three of the six additional clinical outcome measures - the 10m-timed walking test (T10MW), 9 hole-peg test (9HPT), and foot dorsal flexion dynamometry - further improved discrimination between severely and mildly affected patients. From these findings, we identified three different composite measures as score hypotheses and compared their discriminant power with that of the CMTNS. A composite of eight components CMAP, Motor symptoms legs, Motor symptoms arms, Strength of Legs, Sensory symptoms), displayed the strongest power to discriminate between the clusters. As a conclusion, five items from the CMTNS and three secondary clinical outcome measures improve the clinical assessment of patients with CMT1A significantly and are beneficial for upcoming clinical and therapeutic trials. PMID:25085517

  18. Selected items from the Charcot-Marie-Tooth (CMT) Neuropathy Score and secondary clinical outcome measures serve as sensitive clinical markers of disease severity in CMT1A patients.

    PubMed

    Mannil, Manoj; Solari, Alessandra; Leha, Andreas; Pelayo-Negro, Ana L; Berciano, José; Schlotter-Weigel, Beate; Walter, Maggie C; Rautenstrauss, Bernd; Schnizer, Tuuli J; Schenone, Angelo; Seeman, Pavel; Kadian, Chandini; Schreiber, Olivia; Angarita, Natalia G; Fabrizi, Gian Maria; Gemignani, Franco; Padua, Luca; Santoro, Lucio; Quattrone, Aldo; Vita, Giuseppe; Calabrese, Daniela; Young, Peter; Laurà, Matilde; Haberlová, Jana; Mazanec, Radim; Paulus, Walter; Beissbarth, Tim; Shy, Michael E; Reilly, Mary M; Pareyson, Davide; Sereda, Michael W

    2014-11-01

    This study evaluates primary and secondary clinical outcome measures in Charcot-Marie-Tooth disease type 1A (CMT1A) with regard to their contribution towards discrimination of disease severity. The nine components of the composite Charcot-Marie-Tooth disease Neuropathy Score and six additional secondary clinical outcome measures were assessed in 479 adult patients with genetically proven CMT1A and 126 healthy controls. Using hierarchical clustering, we identified four significant clusters of patients according to clinical severity. We then tested the impact of each of the CMTNS components and of the secondary clinical parameters with regard to their power to differentiate these four clusters. The CMTNS components ulnar sensory nerve action potential (SNAP), pin sensibility, vibration and strength of arms did not increase the discriminant value of the remaining five CMTNS components (Ulnar compound motor action potential [CMAP], leg motor symptoms, arm motor symptoms, leg strength and sensory symptoms). However, three of the six additional clinical outcome measures - the 10m-timed walking test (T10MW), 9 hole-peg test (9HPT), and foot dorsal flexion dynamometry - further improved discrimination between severely and mildly affected patients. From these findings, we identified three different composite measures as score hypotheses and compared their discriminant power with that of the CMTNS. A composite of eight components CMAP, Motor symptoms legs, Motor symptoms arms, Strength of Legs, Sensory symptoms), displayed the strongest power to discriminate between the clusters. As a conclusion, five items from the CMTNS and three secondary clinical outcome measures improve the clinical assessment of patients with CMT1A significantly and are beneficial for upcoming clinical and therapeutic trials.

  19. Influenza-like-illness and clinically diagnosed flu: disease burden, costs and quality of life for patients seeking ambulatory care or no professional care at all.

    PubMed

    Bilcke, Joke; Coenen, Samuel; Beutels, Philippe

    2014-01-01

    This is one of the first studies to (1) describe the out-of-hospital burden of influenza-like-illness (ILI) and clinically diagnosed flu, also for patients not seeking professional medical care, (2) assess influential background characteristics, and (3) formally compare the burden of ILI in patients with and without a clinical diagnosis of flu. A general population sample with recent ILI experience was recruited during the 2011-2012 influenza season in Belgium. Half of the 2250 respondents sought professional medical care, reported more symptoms (especially more often fever), a longer duration of illness, more use of medication (especially antibiotics) and a higher direct medical cost than patients not seeking medical care. The disease and economic burden were similar for ambulatory ILI patients, irrespective of whether they received a clinical diagnosis of flu. On average, they experienced 5-6 symptoms over a 6-day period; required 1.6 physician visits and 86-91% took medication. An average episode amounted to €51-€53 in direct medical costs, 4 days of absence from work or school and the loss of 0.005 quality-adjusted life-years. Underlying illness led to greater costs and lower quality-of-life. The costs of ILI patients with clinically diagnosed flu tended to increase, while those of ILI patients without clinically diagnosed flu tended to decrease with age. Recently vaccinated persons experienced lower costs and a higher quality-of-life, but this was only the case for patients not seeking professional medical care. This information can be used directly to evaluate the implementation of cost-effective prevention and control measures for influenza. In particular to inform the evaluation of more widespread seasonal influenza vaccination, including in children, which is currently considered by many countries.

  20. Patients with collagen vascular disease and dyspnea. The value of gallium scanning and bronchoalveolar lavage in predicting response to steroid therapy and clinical outcome

    SciTech Connect

    Greene, N.B.; Solinger, A.M.; Baughman, R.P.

    1987-05-01

    Patients with collagen vascular disease with or without pulmonary symptoms were studied to determine the value of gallium scan and bronchoalveolar lavage (BAL) in predicting clinical outcome and response to steroid therapy. Thirty-six subjects, 20 with progressive dyspnea, were studied. Gallium uptake was seen in the lung in 17 of the 20 progressively dyspneic patient's and none of the 16 nonprogressive patients. The BAL fluid in the progressive patients had a higher percentage of neutrophils (13.4 percent +/- 2.88) and lymphocytes (16.1 percent +/- 2.75) than in the nonprogressive patients (neutrophils = 3.3 +/- 1.30 percent; lymphocytes = 5.6 +/- 1.57 percent. Of the 19 progressive patients who were treated with steroids or cyclophosphamide, six had only increased neutrophils in their BAL fluid and all died. The remaining 13 treated progressive patients had increased lymphocytes or a normal BAL (two patients): six had improvement in their vital capacity, six have had stable function, and one died. We found gallium scan and BAL useful in assessing progressive pulmonary fibrosis in collagen vascular disease.

  1. Maintaining Life-saving Testing for Patients With Infectious Diseases: Infectious Diseases Society of America, American Society for Microbiology, and Pan American Society for Clinical Virology Recommendations on the Regulation of Laboratory-developed Tests.

    PubMed

    Caliendo, Angela M; Couturier, Marc R; Ginocchio, Christine C; Hanson, Kimberly E; Miller, Melissa B; Walker, Kimberly E; Frank, Gregory M

    2016-07-15

    In 2014, the US Food and Drug Administration (FDA) proposed to regulate laboratory-developed tests (LDTs)-diagnostics designed, manufactured, and used within a single laboratory. The Infectious Diseases Society of America, the American Society for Microbiology, and the Pan American Society for Clinical Virology recognize that the FDA is committed to protecting patients. However, our societies are concerned that the proposed regulations will limit access to testing and negatively impact infectious diseases (ID) LDTs. In this joint commentary, our societies discuss why LDTs are critical for ID patient care, hospital infection control, and public health responses. We also highlight how the FDA's proposed regulation of LDTs could impair patient access to life-saving tests and stifle innovation in ID diagnostics. Finally, our societies make specific recommendations for the FDA's consideration to reduce the burden of the proposed new rules on clinical laboratories and protect patients' access to state-of-the art, quality LDTs. PMID:27118790

  2. Maintaining Life-saving Testing for Patients With Infectious Diseases: Infectious Diseases Society of America, American Society for Microbiology, and Pan American Society for Clinical Virology Recommendations on the Regulation of Laboratory-developed Tests.

    PubMed

    Caliendo, Angela M; Couturier, Marc R; Ginocchio, Christine C; Hanson, Kimberly E; Miller, Melissa B; Walker, Kimberly E; Frank, Gregory M

    2016-07-15

    In 2014, the US Food and Drug Administration (FDA) proposed to regulate laboratory-developed tests (LDTs)-diagnostics designed, manufactured, and used within a single laboratory. The Infectious Diseases Society of America, the American Society for Microbiology, and the Pan American Society for Clinical Virology recognize that the FDA is committed to protecting patients. However, our societies are concerned that the proposed regulations will limit access to testing and negatively impact infectious diseases (ID) LDTs. In this joint commentary, our societies discuss why LDTs are critical for ID patient care, hospital infection control, and public health responses. We also highlight how the FDA's proposed regulation of LDTs could impair patient access to life-saving tests and stifle innovation in ID diagnostics. Finally, our societies make specific recommendations for the FDA's consideration to reduce the burden of the proposed new rules on clinical laboratories and protect patients' access to state-of-the art, quality LDTs.

  3. The effect of lactulose supplementation on fecal microflora of patients with chronic kidney disease; a randomized clinical trial

    PubMed Central

    Tayebi-Khosroshahi, Hamid; Habibzadeh, Afshin; Niknafs, Bahram; Ghotaslou, Reza; Yeganeh Sefidan, Fatemeh; Ghojazadeh, Morteza; Moghaddaszadeh, Majid; Parkhide, Sahar

    2016-01-01

    Introduction: Lactulose is a prebiotic with bifidogenic and urea reduction effects. It can improve Bifidobacteria and Lactobacilli counts in healthy humans and it may possibly have similar effects in chronic kidney disease (CKD) patients. Objectives: To investigate the effect of lactulose on fecal microflora of patients with CKD. Patients and Methods: Thirty-two patients with stages 3 and 4 of CKD (43.8% male with mean age of 58.09±12.75 years) were randomly assigned to intervention (n=16) and control (n=16) groups. Patients in intervention group received 30 mm lactulose syrup three times a day for an 8-week period. Control group received placebo 30 mm three times a day. A fecal sample was obtained from all patients at the beginning and at the end of the study and Bifidobacteria and Lactobacilli was counted. Results: Creatinine (Cr) significantly decreased in intervention group (3.90±1.43 to 3.60±1.44, P=0.003) and increased in control group (3.87±2.08 to 4.11±1.99, P=0.03). Although Bifidobacterial and Lactobacilli counts were similar before intervention, they were significantly higher at the end of the study in lactulose group (P=0.01 and P=0.04, respectively). Lactulose led to significant increase in fecal Bifidobacterial counts (3.61±0.54 to 4.90±0.96, P<0.001) and Lactobacilli counts (2.79±1.00 to 3.87±1.13, P<0.001), while the change in placebo group was not significant. Conclusion: Lactulose administration will increase Bifidobacteria and Lactobacillus counts in patients with CKD. PMID:27689115

  4. The effect of lactulose supplementation on fecal microflora of patients with chronic kidney disease; a randomized clinical trial

    PubMed Central

    Tayebi-Khosroshahi, Hamid; Habibzadeh, Afshin; Niknafs, Bahram; Ghotaslou, Reza; Yeganeh Sefidan, Fatemeh; Ghojazadeh, Morteza; Moghaddaszadeh, Majid; Parkhide, Sahar

    2016-01-01

    Introduction: Lactulose is a prebiotic with bifidogenic and urea reduction effects. It can improve Bifidobacteria and Lactobacilli counts in healthy humans and it may possibly have similar effects in chronic kidney disease (CKD) patients. Objectives: To investigate the effect of lactulose on fecal microflora of patients with CKD. Patients and Methods: Thirty-two patients with stages 3 and 4 of CKD (43.8% male with mean age of 58.09±12.75 years) were randomly assigned to intervention (n=16) and control (n=16) groups. Patients in intervention group received 30 mm lactulose syrup three times a day for an 8-week period. Control group received placebo 30 mm three times a day. A fecal sample was obtained from all patients at the beginning and at the end of the study and Bifidobacteria and Lactobacilli was counted. Results: Creatinine (Cr) significantly decreased in intervention group (3.90±1.43 to 3.60±1.44, P=0.003) and increased in control group (3.87±2.08 to 4.11±1.99, P=0.03). Although Bifidobacterial and Lactobacilli counts were similar before intervention, they were significantly higher at the end of the study in lactulose group (P=0.01 and P=0.04, respectively). Lactulose led to significant increase in fecal Bifidobacterial counts (3.61±0.54 to 4.90±0.96, P<0.001) and Lactobacilli counts (2.79±1.00 to 3.87±1.13, P<0.001), while the change in placebo group was not significant. Conclusion: Lactulose administration will increase Bifidobacteria and Lactobacillus counts in patients with CKD.

  5. Immunoblastic lymphoma involving the bone marrow in a patient with alpha chain disease. Clinical and immunoelectron microscopic study.

    PubMed

    Reyes, F; Piquet, J; Gourdin, M F; Haioun, C; Intrator, L; Tulliez, M; Roberti, A; Rambaud, J C

    1985-03-01

    A patient is reported who had disseminated immunoblastic proliferation that emerged during the course of alpha chain disease. This proliferation was characterized by overt marrow invasion together with osseous and neurologic manifestations. On immunoelectron microscopic study, the malignant immunoblasts displayed varying degrees of cytoplasmic maturation and constituted a morphologic spectrum of alpha-chain-synthesizing cells, ranging from immature blasts without endoplasmic reticulum development to relatively mature plasmablasts; alpha chain was not expressed at the surface of these cells. The general features of the overt malignant stage of alpha chain disease are reviewed in reference to this unusual case. The implications of the cellular findings are discussed with regard to the maturation stage of malignant immunoblasts. PMID:3917845

  6. Person-centered medicine and good clinical practice: disease has to be cured, but the patient has to be healed.

    PubMed

    Jakovljević, Miro; Ostojić, Ljerka

    2015-12-01

    Contemporary medicine has been in the process of a paradigm shift. Instead of relatively broad pathological entities, population-based risk assesments, and non-specific "one-size-fits-all" therapies, a new paradigm of predictive, individualized and personalized care and targeted therapy is rolling on the horizon. Person-centered medicine is a term with different meanings which competes in some way to other terms like medicine of the person, patient-centered medicine, personalized and individualzed medicine, integrative and holistic medicine. Clear definition of theoretical framework and clinical practice of person-centered medicine is urgent to prevent dangereous increasing confusion. PMID:26657982

  7. Duration of salmeterol-induced bronchodilation in mechanically ventilated chronic obstructive pulmonary disease patients: a prospective clinical study

    PubMed Central

    Malliotakis, Polychronis; Linardakis, Manolis; Gavriilidis, George; Georgopoulos, Dimitris

    2008-01-01

    Introduction Delivery of bronchodilators with a metered-dose inhaler (MDI) and a spacer device in mechanically ventilated patients has become a widespread practice. However, except for the short-acting β2-agonist salbutamol, the duration of action of other bronchodilators, including long-acting β2-agonists, delivered with this technique is not well established. The purpose of this study was to examine the duration of bronchodilation induced by the long-acting β2-agonist salmeterol administered with an MDI and a spacer in a group of mechanically ventilated patients with exacerbation of chronic obstructive pulmonary disease (COPD). Methods Ten mechanically ventilated patients with acute exacerbation of COPD received four puffs of salmeterol (25 μg/puff). Salmeterol was administered with an MDI adapted to the inspiratory limb of the ventilator circuit using an aerosol cloud enhance spacer. Static and dynamic airway pressures, minimum (Rint) and maximum (Rrs) inspiratory resistance, and the difference between Rrs and Rint (ΔR) were measured before and at 15, 30, and 60 minutes as well as at 2, 3, 4, 6, 8, 10, and 12 hours after salmeterol administration. The overall effects of salmeterol on respiratory system mechanics and heart rate during the 12-hour study period were analyzed by nonparametric Wilcoxon signed rank test. Results Salmeterol caused a significant decrease in dynamic and static airway pressures, Rint, and Rrs. These changes were evident at 30 minutes and remained significant for 8 hours after salmeterol administration. The duration of bronchodilation varied significantly among patients, lasting in some patients more than 10 hours and wearing off in others in less than 6 hours. Conclusions It is concluded that four puffs of salmeterol delivered with an MDI and a spacer device induces significant bronchodilation in mechanically ventilated patients with COPD exacerbation, the duration of which is highly variable, precluding definite conclusions in

  8. Prevalence and Clinical Significance of Low T3 Syndrome in Non-Dialysis Patients with Chronic Kidney Disease

    PubMed Central

    Fan, Jingxian; Yan, Peng; Wang, Yingdeng; Shen, Bo; Ding, Feng; Liu, Yingli

    2016-01-01

    Background There are few data on the prevalence of low T3 (triiodothyronine) syndrome in patients with non-dialysis chronic kidney disease (CKD) and it is unclear whether low T3 can be used to predict the progression of CKD. Material/Methods We retrospectively studied 279 patients who had been definitively diagnosed with CKD, without needing maintenance dialysis. Thyroid function was analyzed in all enrolled subjects and the incidence of thyroid dysfunction (low T3 syndrome, low T4 syndrome, and subclinical hypothyroidism) in patients at different stages of CKD was determined. Results Glomerular filtration rate (GFR) of CKD patients was estimated as follows: 145 subjects (52%) had GFR <60 ml/min per 1.73 m2; 47 subjects (16.8%) had GFR between 30 and 59 ml/min per 1.73 m2, and 98 subjects (35.1%) had GFR <30 ml/min per 1.73 m2. Among all enrolled subjects, 4.7% (n=13) had subclinical hypothyroidism, 5.4% (n=15) had low T4 syndrome, and 47% (n=131) had low T3 syndrome. In 114 CKD patients in stages 3–5, serum T3 was positively related to protein metabolism (STP, PA, and ALB) and anemia indicators (Hb and RBC), and negatively related to inflammatory status (CRP and IL-6). Conclusions A high prevalence of low T3 syndrome was observed in CKD patients without dialysis, even in early stages (1 and 2). The increasing prevalence of low T3 as CKD progresses indicates its value as a predictor of worsening CKD. Furthermore, low T3 syndrome is closely associated with both malnutrition-inflammation complex syndrome (MICS) and anemia. PMID:27056188

  9. The clinical usefulness of ESR, CRP, and disease duration in ankylosing spondylitis: the product of these acute-phase reactants and disease duration is associated with patient's poor physical mobility.

    PubMed

    Chen, Chun-Hsiung; Chen, Hung-An; Liao, Hsien-Tzung; Liu, Chin-Hsiu; Tsai, Chang-Youh; Chou, Chung-Tei

    2015-07-01

    We evaluated the clinical usefulness of ESR, CRP, and disease duration in ankylosing spondylitis (AS) disease severity. There were 156 Chinese AS patients included in Taiwan. Patients completed the questionnaires, containing demographic data, disease activity (BASDAI), functional status (BASFI), and patient's global assessment (BASG). Meanwhile, patient's physical mobility (BASMI) and acute-phase reactants, including ESR and CRP levels were measured. Receiver operating characteristic (ROC) plot analysis was used to evaluate the performance of ESR, CRP, and disease duration in the AS patients. ESR mildly correlated with BASFI (r = 0.176, p = 0.028) and disease duration (r = 0.214, p = 0.008), and moderately correlated with BASMI (r = 0.427, p < 0.001). CRP moderately correlated with BASMI (r = 0.410, p < 0.001). By using ROC plot analysis, ESR, CRP, and disease duration showed the best and significant "area under the curve (AUC)", in distinguishing the AS patients with poor physical mobility (BASMI ≥ 3.6, the Median) (AUC = 0.748, 0.751 and 0.738, respectively, all p < 0.001), as compared to BASDAI, BASFI, and BASG. ESR × disease duration (AUC = 0.801, p < 0.001) and CRP × disease duration (AUC = 0.821, p < 0.001) showed higher AUC values than ESR or CRP alone in indicating poor physical mobility. For detecting poor physical mobility (BASMI ≥ 3.6) in the AS patients: ESR × disease duration (≥60.0 mm/h × year): sensitivity = 72.7 % and specificity = 72.8 %; CRP × disease duration (≥8.3 mg/dl × year): sensitivity = 72.7 % and specificity = 74.6 %. ESR, CRP, and disease duration are particularly related to AS patient's poor physical mobility. Combining the usefulness of acute-phase reactants and disease duration, the values of ESR × disease duration and CRP × disease duration demonstrate better association with poor physical mobility in AS patients.

  10. Procoagulant microparticles are increased in patients with Behçet's disease but do not define a specific subset of clinical manifestations.

    PubMed

    Mejía, Juan Carlos; Ortiz, Thaia; Tàssies, Dolors; Solanich, Xavier; Vidaller, Antonio; Cervera, Ricard; Reverter, Joan-Carles; Espinosa, Gerard

    2016-03-01

    Microparticles (MP) are considered a key component in the haemostatic response. Beyond their in vitro procoagulant properties, a number of pieces of evidence points to procoagulant MP as efficient effectors in the haemostatic response and as pathogenic markers of thrombotic disorders and vascular damage. The aim of the present study was to analyze the procoagulant activity of MP and its correlation with clinical manifestations focusing on vascular involvement in patients with Behçet's disease (BD). We analyzed 55 BD patients in inactive phase of the disease (26 men; mean age, 35 ± 15 years) of which 19 had previously suffered from thrombosis (deep venous thrombosis in 17 and ischemic stroke in 2), and 73 healthy controls matched for age and sex. Procoagulant MP were assessed by a functional assay. BD patients showed higher procoagulant MP values than controls (22.89 ± 15.74 nM versus 14.47 ± 7.34 nM; p < 0.0001). Conversely, we did not find differences in the levels of procoagulant MP according to the gender of patients (22.22 ± 16.23 nM for men versus 21.46 ± 16.47 for women; p = 0.846) or to previous and current treatments. Moreover, the plasmatic concentration of MP does not define any clinical phenotype and it was not related to the time of evolution of the disease. Although inactive BD patients had high values of procoagulant MP, they did not differentiate between BD patients with or without thrombosis. PMID:25711877

  11. Clinical response to eliglustat in treatment-naïve patients with Gaucher disease type 1: Post-hoc comparison to imiglucerase-treated patients enrolled in the International Collaborative Gaucher Group Gaucher Registry.

    PubMed

    Ibrahim, Jennifer; Underhill, Lisa H; Taylor, John S; Angell, Jennifer; Peterschmitt, M Judith

    2016-09-01

    Eliglustat is a recently approved oral therapy in the United States and Europe for adults with Gaucher disease type 1 who are CYP2D6 extensive, intermediate, or poor metabolizers (> 90% of patients) that has been shown to decrease spleen and liver volume and increase hemoglobin concentrations and platelet counts in untreated adults with Gaucher disease type 1 and maintain these parameters in patients previously stabilized on enzyme replacement therapy. In a post-hoc analysis, we compared the results of eliglustat treatment in treatment-naïve patients in two clinical studies with the results of imiglucerase treatment among a cohort of treatment-naïve patients with comparable baseline hematologic and visceral parameters in the International Collaborative Gaucher Group Gaucher Registry. Organ volumes and hematologic parameters improved from baseline in both treatment groups, with a time course and degree of improvement in eliglustat-treated patients similar to imiglucerase-treated patients. PMID:27408819

  12. Randomised clinical trial of an intensive intervention in the primary care setting of patients with high plasma fibrinogen in the primary prevention of cardiovascular disease

    PubMed Central

    2012-01-01

    Background We have studied the possible effects of an intensive lifestyle change program on plasma fibrinogen levels, in patients with no cardiovascular disease, with elevated levels of fibrinogen, normal cholesterol levels, and a moderate estimated risk of coronary heart disease (CHD) and we have also analysed whether the effect on fibrinogen is independent of the effect on lipids. Results This clinical trial was controlled, unblinded and randomized, with parallel groups, done in 13 Basic Health Areas (BHA) in l'Hospitalet de Llobregat (Barcelona) and Barcelona city. The study included 436 patients, aged between 35 and 75 years, with no cardiovascular disease, elevated levels of fibrinogen (> 300 mg/dl), cholesterol < 250 mg/dl, 218 of whom received a more intensive intervention consisting of advice on lifestyle and treatment. The follow-up frequency of the intervention group was every 2 months. The other 218 patients followed their standard care in the BHAs. Fibrinogen, plasma cholesterol and other clinical biochemistry parameters were assessed. The evaluation of the baseline characteristics of the patients showed that both groups were homogenous. Obesity and hypertension were the most prevalent risk factors. After 24 months of the study, statistically significant changes were seen between the adjusted means of the two groups, for the following parameters: fibrinogen, plasma cholesterol, systolic and diastolic blood pressure and body mass index. Conclusion Intensive intervention to achieve lifestyle changes has shown to be effective in reducing some of the estimated CHD factors. However, the effect of intensive intervention on plasma fibrinogen levels did not correlate with the variations in cholesterol. Trial Registration ClinicalTrials.gov: NCT01089530 PMID:22381072

  13. Safety and Efficacy of PDpoetin for Management of Anemia in Patients with end Stage Renal Disease on Maintenance Hemodialysis: Results from a Phase IV Clinical Trial.

    PubMed

    Javidan, Abbas Norouzi; Shahbazian, Heshmatollah; Emami, Amirhossein; Yekaninejad, Mir Saeed; Emami-Razavi, Hassan; Farhadkhani, Masoumeh; Ahmadzadeh, Ahmad; Gorjipour, Fazel

    2014-08-26

    Recombinant human erythropoietin (rHuEPO) is available for correcting anemia. PDpoetin, a new brand of rHuEPO, has been certified by Food and Drug Department of Ministry of Health and Medical Education of Iran for clinical use in patients with chronic kidney disease. We conducted this post-marketing survey to further evaluate the safety and efficacy of PDpoetin for management of anemia in patients on maintenance hemodialysis. Patients from 4 centers in Iran were enrolled for this multicenter, open-label, uncontrolled phase IV clinical trial. Changes in blood chemistry, hemoglobin and hematocrit levels, renal function, and other characteristics of the patients were recorded for 4 months; 501 of the patients recruited, completed this study. Mean age of the patients was 50.9 (±16.2) years. 48.7% of patients were female. Mean of the hemoglobin value in all of the 4 centers was 9.29 (±1.43) g/dL at beginning of the study and reached 10.96 (±2.23) g/dL after 4 months and showed significant increase overall (P<0.001). PDpoetin dose was stable at 50-100 U/kg thrice weekly. Hemorheologic disturbancesand changes in blood electrolytes was not observed. No case of immunological reactions to PDpoetin was observed. Our study, therefore, showed that PDpoetin has significantly raised the level of hemoglobin in the hemodialysis patients (about 1.7±0.6 g/dL). Anemia were successfully corrected in 49% of patients under study. Use of this biosimilar was shown to be safe and effective for the maintenance of hemoglobin in patients on maintenance hemodialysis.

  14. Safety and Efficacy of PDpoetin for Management of Anemia in Patients with end Stage Renal Disease on Maintenance Hemodialysis: Results from a Phase IV Clinical Trial

    PubMed Central

    Javidan, Abbas Norouzi; Shahbazian, Heshmatollah; Emami, Amirhossein; Yekaninejad, Mir Saeed; Emami-Razavi, Hassan; Farhadkhani, Masoumeh; Gorjipour, Fazel

    2014-01-01

    Recombinant human erythropoietin (rHuEPO) is available for correcting anemia. PDpoetin, a new brand of rHuEPO, has been certified by Food and Drug Department of Ministry of Health and Medical Education of Iran for clinical use in patients with chronic kidney disease. We conducted this post-marketing survey to further evaluate the safety and efficacy of PDpoetin for management of anemia in patients on maintenance hemodialysis. Patients from 4 centers in Iran were enrolled for this multicenter, open-label, uncontrolled phase IV clinical trial. Changes in blood chemistry, hemoglobin and hematocrit levels, renal function, and other characteristics of the patients were recorded for 4 months; 501 of the patients recruited, completed this study. Mean age of the patients was 50.9 (±16.2) years. 48.7% of patients were female. Mean of the hemoglobin value in all of the 4 centers was 9.29 (±1.43) g/dL at beginning of the study and reached 10.96 (±2.23) g/dL after 4 months and showed significant increase overall (P<0.001). PDpoetin dose was stable at 50-100 U/kg thrice weekly. Hemorheologic disturbancesand changes in blood electrolytes was not observed. No case of immunological reactions to PDpoetin was observed. Our study, therefore, showed that PDpoetin has significantly raised the level of hemoglobin in the hemodialysis patients (about 1.7±0.6 g/dL). Anemia were successfully corrected in 49% of patients under study. Use of this biosimilar was shown to be safe and effective for the maintenance of hemoglobin in patients on maintenance hemodialysis. PMID:25317316

  15. Assessment of Interleukin 16 Serum Levels and Skin Expression in Psoriasis Patients in Correlation with Clinical Severity of the Disease

    PubMed Central

    Purzycka-Bohdan, Dorota; Szczerkowska-Dobosz, Aneta; Zablotna, Monika; Wierzbicka, Justyna; Piotrowska, Anna; Zmijewski, Michal A.; Nedoszytko, Boguslaw; Nowicki, Roman

    2016-01-01

    Interleukin 16 (IL-16) has been described as a significant cytokine involved in the recruitment of CD4+ cells during inflammation; however, its potential role in psoriasis has not been defined. Our aim was to investigate the IL-16 serum levels and IL-16 mRNA skin expression in psoriasis patients in correlation with disease severity and mRNA skin expression for CD4. Moreover, the IL-16 skin localization was assessed and the -295 T/C IL-16 polymorphism was analyzed. For this exploratory, observational, and cross-sectional study, 97 unrelated patients with chronic plaque type psoriasis and 104 healthy controls were enrolled. IL-16 serum levels were significantly increased in patients compared with controls (P = 0.000022) and positively correlated with Psoriasis Area and Severity Index (r = 0.34, P = 0.0007), Body Surface Area (r = 0.34, P = 0.01) and were significantly higher in individuals with moderate to severe psoriasis (P = 0.0029). There was no significant correlation between IL-16 serum levels and Dermatology Quality of Life Index and no differences in genotype and allele frequencies for -295 T/C IL-16 polymorphism. The expression of IL-16 (mRNA and protein) was elevated in the margin of psoriatic skin while statistically significant increase in IL-16 immunoreactivity, but not in mRNA level, was observed within plaques. Furthermore, the IL-16 mRNA levels within psoriatic lesions positively correlated with the levels of CD4 mRNA, but not with Psoriasis Area and Severity Index. In conclusion, our data revealed an association between circulating IL-16 and severity of psoriasis which indicates that this cytokine could serve as a potential marker of disease activity. However, further investigations are required. PMID:27788245

  16. Long-term outcome in BRAFV600E melanoma patients treated with vemurafenib: Patterns of disease progression and clinical management of limited progression

    PubMed Central

    Puzanov, Igor; Amaravadi, Ravi K.; McArthur, Grant A.; Flaherty, Keith T.; Chapman, Paul B.; Sosman, Jeffrey A.; Ribas, Antoni; Shackleton, Mark; Hwu, Patrick; Chmielowski, Bartosz; Nolop, Keith B.; Lin, Paul S.; Kim, Kevin B.

    2016-01-01

    Introduction Vemurafenib induces tumour regression in most patients with BRAFV600E-mutant melanoma; eventually, most experience progressive disease (PD). Long-term follow-up of patients with BRAFV600E melanoma treated in the phase 1 vemurafenib trial is reported. Methods Patients received vemurafenib 240–1120 mg (dose escalation cohort) or 960 mg (extension cohort) orally twice daily. Clinical response was evaluated every 8 weeks by RECIST. Patients with PD amenable to local therapy (surgery or radiotherapy) were allowed to continue vemurafenib after progression. Overall survival (OS) from time of treatment initiation and from PD was estimated. Sites of PD were recorded. Results Forty-eight patients (escalation cohort, n = 16; extension cohort, n = 32) received therapeutic doses of vemurafenib (≥240 mg twice daily). Forty-three patients had PD by the time of this analysis, and 5 remained progression free (follow-up time, 1.2–56.1 months). Median OS was 14 months (range, 1.2–56.1); 3- and 4-year melanoma-specific survival rate in the extension cohort was 26% and 19%, respectively. Median OS was 26.0 months (range, 7.7–56.1) among 20 patients who continued vemurafenib after local therapy. Median treatment duration beyond initial PD was 3.8 months (range, 1.1–26.6). In the extension cohort, 6 and 5 patients were alive after 3 and 4 years, respectively, on vemurafenib monotherapy. Conclusions Some patients with melanoma achieved long-term survival with vemurafenib monotherapy. Continuation of vemurafenib after PD might be beneficial in some patients because remaining disease might continue to respond to BRAF inhibition. PMID:25980594

  17. Development of Clinical Trial Assessments for the Study of Interstitial Lung Disease in Patients who have Connective Tissue Diseases—Methodological Considerations

    PubMed Central

    Huscher, Dörte; Saketkoo, Lesley Ann; Pittrow, David; Khanna, Dinesh

    2010-01-01

    This review article discusses the proposed methodology that will be utilized to develop core set items for connective tissue disease-associated interstitial lung disease (CTD-ILD). CTD-ILD remain an important enigma in clinical medicine. No consensus exists on measurement of disease activity or what constitutes a significant response to therapeutic interventions. Lack of appropriate measures inhibit effective drug development and hamper regulatory evaluation of candidate therapies. An interdisciplinary and international Steering Committee (SC) will oversee the execution of a 3-tier Delphi exercise involving experts in CTD and ILD. In parallel to the Delphi, qualitative information will be gathered from patients with ILD using focus groups. These data will subsequently be used to construct surveys to collect quantitative response from patients with ILD. The final Delphi and Patient Perspective results are to be scrutinized by SC and specialty sub-groups (including patient advocates) for truth, discrimination and feasibility – the OMERACT filters. Through application of Nominal Group technique, a core set of outcome measures will be proposed. Subsequent exercises will evaluate the applicability of a proposed core set to the unique issues posed by individual CTDs in addition to guidelines on screening, prognostication and damage scoring. PMID:20676224

  18. [Connection of endothelial dysfunction and level of blood leukocytes with clinical course of stable angina pectoris in patients with ischemic heart disease].

    PubMed

    Lyzohub, V H; Savchenko, O V; Bondarchuk, O M; Dykukha, I S; Voloshyna, O O; Bohdan, T V; Koval'chuk, S M; Moshkovs'ka, Iu O

    2008-01-01

    The authors presents in the article information on influence of endothelial dysfunction and level of blood leukocytes with clinical course of stable angina pectoris in patients with ischemic heart disease (IHD). 104 male patients aged from 36 to 69 (on average 54.9+/-2.1 years) with IHD and stage I-III stable angina pectoris have been observed. The level of leukocytes, ECG daily monitoring, endotelial function with the help of Celermaera-Sorensena test and plasma concentration of endothelin I have been studied in patients. It was established that there is no significant different (P>0.05) in the level of leukocytes between health persons and patients with IHD independently of functional class of angina pectoris. Patients with IHD had higher level of endothelin I in their blood serum than those in a control group. Patients with IHD have a complicated endothelial-non-dependent vasodilation and an increased thickness of the complex intima-media, there is a negative correlation bond between these characteristics. Patients III functional class of angina pectoris has also reliably decreased endothelial-dependent vasodilation by 37%. Frequency of ventricular extrasystoles in patients with IHD and endothelial dysfunction increases by 12 times.

  19. Clinical Genetics of Alzheimer's Disease

    PubMed Central

    Zou, Zhangyu; Liu, Changyun; Che, Chunhui; Huang, Huapin

    2014-01-01

    Alzheimer's disease (AD) is the most common progressive neurodegenerative disease and the most common form of dementia in the elderly. It is a complex disorder with environmental and genetic components. There are two major types of AD, early onset and the more common late onset. The genetics of early-onset AD are largely understood with mutations in three different genes leading to the disease. In contrast, while susceptibility loci and alleles associated with late-onset AD have been identified using genetic association studies, the genetics of late-onset Alzheimer's disease are not fully understood. Here we review the known genetics of early- and late-onset AD, the clinical features of EOAD according to genotypes, and the clinical implications of the genetics of AD. PMID:24955352

  20. Clinical Value of Natriuretic Peptides in Predicting Time to Dialysis in Stage 4 and 5 Chronic Kidney Disease Patients

    PubMed Central

    Sundqvist, Sofia; Larson, Thomas; Cauliez, Bruno; Bauer, Fabrice; Dumont, Audrey; Le Roy, Frank; Hanoy, Mélanie; Fréguin-Bouilland, Caroline; Godin, Michel

    2016-01-01

    Background Anticipating the time to renal replacement therapy (RRT) in chronic kidney disease (CKD) patients is an important but challenging issue. Natriuretic peptides are biomarkers of ventricular dysfunction related to poor outcome in CKD. We comparatively investigated the value of B-type natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) as prognostic markers for the risk of RRT in stage 4 and 5 CKD patients, and in foretelling all-cause mortality and major cardiovascular events within a 5-year follow-up period. Methods Baseline plasma BNP (Triage, Biosite) and NT-proBNP (Elecsys, Roche) were measured at inclusion. Forty-three patients were followed-up during 5 years. Kaplan-Meier analysis, with log-rank testing and hazard ratios (HR), were calculated to evaluate survival without RRT, cardiovascular events or mortality. The independent prognostic value of the biomarkers was estimated in separate Cox multivariate analysis, including estimated glomerular filtration rate (eGFR), creatininemia and comorbidities. Results During the first 12-month follow-up period, 16 patients started RRT. NT-proBNP concentration was higher in patients who reached endpoint (3221 ng/L vs 777 ng/L, p = 0.02). NT-proBNP concentration > 1345 ng/L proved significant predictive value on survival analysis for cardiovascular events (p = 0.04) and dialysis within 60 months follow-up (p = 0.008). BNP concentration > 140 ng/L was an independent predictor of RRT after 12 months follow-up (p<0.005), and of significant predictive value for initiation of dialysis within 60 months follow-up. Conclusions Our results indicate a prognostic value for BNP and NT-proBNP in predicting RRT in stage 4 and 5 CKD patients, regarding both short- and long-term periods. NT-proBNP also proved a value in predicting cardiovascular events. Natriuretic peptides could be useful predictive biomarkers for therapeutic guidance in CKD. PMID:27548064

  1. Epidemiology and clinical management of Legionnaires' disease.

    PubMed

    Phin, Nick; Parry-Ford, Frances; Harrison, Timothy; Stagg, Helen R; Zhang, Natalie; Kumar, Kartik; Lortholary, Olivier; Zumla, Alimuddin; Abubakar, Ibrahim

    2014-10-01

    Legionnaires' disease is an important cause of community-acquired and hospital-acquired pneumonia. Although uncommon, Legionnaires' disease continues to cause disease outbreaks of public health significance. The disease is caused by any species of the Gram-negative aerobic bacteria belonging to the genus Legionella; Legionella pneumophila serogroup 1 is the causative agent of most cases in Europe. In this Review we outline the global epidemiology of Legionnaires' disease, summarise its diagnosis and management, and identify research gaps and priorities. Early clinical diagnosis and prompt initiation of appropriate antibiotics for Legionella spp in all patients with community-acquired or hospital-acquired pneumonias is a crucial measure for management of the disease. Progress in typing and sequencing technologies might additionally contribute to understanding the distribution and natural history of Legionnaires' disease, and inform outbreak investigations. Control of Legionnaires' disease outbreaks relies on rapid ascertainment of descriptive epidemiological data, combined with microbiological information to identify the source and implement control measures. Further research is required to define the actual burden of disease, factors that influence susceptibility, key sources of infection, and differences in virulence between strains of Legionella species. Other requirements are improved, specific, sensitive, and rapid diagnostic tests to accurately inform management of Legionnaires' disease, and controlled clinical trials to ascertain the optimum antibiotics for treatment.

  2. Clinical outcomes at 12 months and risk of inflammatory bowel disease in patients with an intermediate raised fecal calprotectin: a ‘real-world’ view

    PubMed Central

    Chambers, Samantha; Malik, Ahmad; Lee, Bee; Sung, Edmond; Nwokolo, Chuka; Arasaradnam, Ramesh

    2016-01-01

    Objectives A recent systematic review confirmed the usefulness of fecal calprotectin (FC) in distinguishing organic (inflammatory bowel disease (IBD)) from non-organic gastrointestinal disease (irritable bowel syndrome (IBS)). FC levels <50 μg/g have a negative predictive value >92% to exclude organic gastrointestinal (GI) disease. Levels >250 μg/g correlate with endoscopic IBD disease activity; sensitivity 90%. We aimed to determine clinical outcomes in intermediate raised FC results (50–250 μg/g). Setting Primary care general practices in Coventry and Warwickshire, and 3 secondary care hospitals. Participants 443 FC results in adults (>16 years old) were reviewed from July 2012 to October 2013. Clinical data was collected from hospital databases and general practitioners. Long-term clinical data was available in 41 patients (out of 48). Primary and secondary outcome measures The number of new diagnoses of IBD, IBS and other diagnoses for the intermediate group. The number referred and discharged from secondary care. Results A new IBD diagnosis was made in 19% (n=8) of intermediate results (1% of normal and 38% of raised results). 5% (n=2) of intermediate results had known IBD in remission. A new IBS diagnosis was made in 27% (n=11) of intermediate results, while 34% (n=14) remained undiagnosed, although 8 of these were not referred to secondary care. Conclusions FC testing remains useful in aiding diagnosis of organic GI conditions. However, unlike negative and strongly positive FC results, intermediate FC results lead to a mixture of diagnoses. The OR of a new diagnosis of IBD for an intermediate result compared to normal FC result was 26.6, while an intermediate FC result gave an OR of 0.54 for a new IBS diagnosis compared to normal FC. For intermediate FC results, 1 in 3 patients remained in secondary care after 12 months with an OR of 3.6 compared to a normal FC result. PMID:27266773

  3. Visualization of endolymphatic hydrops with MR imaging in patients with Ménière's disease and related pathologies: current status of its methods and clinical significance.

    PubMed

    Naganawa, Shinji; Nakashima, Tsutomu

    2014-04-01

    Ménière's disease is an inner ear disorder characterized by vertigo attacks, fluctuating low-frequency hearing loss, ear fullness, and tinnitus. Endolymphatic hydrops has long been thought to be the pathological basis for Ménière's disease. Some patients have inner ear symptoms that do not match the diagnostic guidelines for Ménière's disease, and these are also thought to be related to endolymphatic hydrops. The diagnosis of endolymphatic hydrops is usually made based on clinical symptoms with some assistance from otological functional tests. Recently, the objective diagnosis of endolymphatic hydrops by MR imaging has become possible and many research results have been reported regarding the imaging methods, evaluation methods, the correlation between imaging results and functional otological tests and the correlation between imaging findings and clinical symptoms. In this article we summarize the development of current imaging methods, evaluation techniques and clinical reports based on a review of the literature. We also attempt to characterize the current significance and future directions of MR imaging of endolymphatic hydrops.

  4. The Effect of Follow up (Telenursing) on Liver Enzymes in Patients with Nonalcoholic Fatty Liver Disease: A Randomized Controlled Clinical Trial

    PubMed Central

    Fard, Sorur Javanmardi; Ghodsbin, Fariba; Kaviani, Mohammad Javad; Jahanbin, Iran; Bagheri, Zahra

    2016-01-01

    Background: Non-alcoholic fatty liver disease (NAFLD) is characterized by macro vesicular steatosis in the absence of alcohol. Patients with (NAFLD)need extensive education and support in their treatment. Our aim was to investigate the effect of telenursing on liver enzymes (ALT and AST) in patients with NAFLD. Methods: Our study is a randomized controlled clinical trial. In this study, 60 patients were enrolled from patients who referred to subspecialty gastrointestinal clinics affiliated to Shiraz University of Medical Sciences. Specialists confirmed their diseases by ultrasound and laboratory test. Simple randomization, based on random number table, was used to randomize the participants into intervention (N=30) and control (N=30) groups. Patients in both groups received dietary advice from a nutritionist and were trained to perform physical activities. Telephone intervention in the intervention group lasted for 12 weeks, in order to see the effect of follow up on the recommended diet and physical activities given by the specialist, while; the control group subjects were only followed up as usual by their physician. Results: The result of an independent t-test showed that the mean difference of liver Enzymes between the two groups was statistically significant (P<0.001). The difference of AST and ALT in the intervention and control groups was 18.03, -1.27 and 40.70, 1.52, respectively. Conclusion: We found out that; telenursing could have a positive effect on reduction of liver enzymes (ALT, AST) in patients with NAFLD. Trial Registration Number: IRCT2015040411691N5 PMID:27382590

  5. Future Care Planning for patients approaching end-of-life with advanced heart disease: an interview study with patients, carers and healthcare professionals exploring the content, rationale and design of a randomised clinical trial

    PubMed Central

    Denvir, Martin A; Highet, Gill; Robertson, Shirley; Cudmore, Sarah; Reid, Janet; Ness, Andrea; Hogg, Karen; Weir, Christopher; Murray, Scott; Boyd, Kirsty

    2014-01-01

    Objective To explore the optimal content and design of a clinical trial of an end-of-life intervention for advanced heart disease with patients, carers and healthcare professionals. Design Qualitative interview and focus group study. Setting Community and hospital-based focus groups and interviews. Participants Stable community-dwelling patients, informal carers (PC, n=15) and primary and secondary care based healthcare professionals (HCP, n=11). Results PC highlighted fragmentation of services and difficulty in accessing specialist care as key barriers to good care. They felt that time for discussion with HCP was inadequate within current National Health Service (NHS) healthcare systems. HCP highlighted uncertainty of prognosis, explaining mortality risk to patients and switching from curative to palliative approaches as key challenges. Patient selection, nature of the intervention and relevance of trial outcomes were identified by HCP as key challenges in the design of a clinical trial. Conclusions PC and HCP expressed a number of concerns relevant to the nature and content of an end-of-life intervention for patients with advanced heart disease. The findings of this study are being used to support a phase II randomised clinical trial of Future Care Planning in advanced heart disease. PMID:25023130

  6. High On-Aspirin Platelet Reactivity and Clinical Outcome in Patients With Stable Coronary Artery Disease: Results From ASCET (Aspirin Nonresponsiveness and Clopidogrel Endpoint Trial)

    PubMed Central

    Pettersen, Alf-Åge R.; Seljeflot, Ingebjørg; Abdelnoor, Michael; Arnesen, Harald

    2012-01-01

    Background Patients with stable coronary artery disease on single-antiplatelet therapy with aspirin are still at risk for atherothrombotic events, and high on-aspirin residual platelet reactivity (RPR) has been suggested as a risk factor. Methods and Results In this randomized trial, the association between platelet function determined by the PFA100 platelet function analyzer system (Siemens Healthcare Diagnostics, Germany) and clinical outcome in 1001 patients, all on single-antiplatelet therapy with aspirin (160 mg/d) was studied. Patients were randomized to continue with aspirin 160 mg/d or change to clopidogrel 75 mg/d. A composite end point of death, myocardial infarction, ischemic stroke, and unstable angina was used. At 2-year follow-up, 106 primary end points were registered. The prevalence of high RPR was 25.9%. High on-aspirin RPR did not significantly influence the primary end point in the aspirin group (13.3% versus 9.9%, P=0.31). However, in post hoc analysis, patients with von Willebrand factor levels or platelet count below median values and high on-aspirin RPR had a statistically significant higher end point rate than that of patients with low RPR (20% versus 7.5%, P=0.014, and 18.2% versus 10.8%, P=0.039, respectively). The composite end point rate in patients with high on-aspirin RPR treated with clopidogrel was not different from that of patients treated with aspirin (7.6% versus 13.3%, P=0.16). Conclusions In stable, aspirin-treated patients with coronary artery disease, high on-aspirin RPR did not relate to clinical outcome and did not identify a group responsive to clopidogrel. Post hoc subgroup analysis raised the possibility that high on-aspirin RPR might be predictive in patients with low von Willebrand factor or platelet count, but these findings will require confirmation in future studies. Clinical Trial Registration URL: http://www.clinicaltrials.gov Unique identifier: NCT00222261. (J Am Heart Assoc. 2012;1:e000703 doi: 10.1161/JAHA.112

  7. Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Disease

    PubMed Central

    Lee, Kyoung Hee; Choi, Dae Seob; Lee, Young Suk

    2016-01-01

    Hirayama disease, juvenile muscular atrophy of the distal upper limb, is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. This cervical myelopathy is associated with neck flexion. It should be suspected in young male patients with a chronic history of weakness and atrophy involving the upper extremities followed by clinical stability in few years. Herein, we report 2 cases of Hirayama disease on emphasis of diagnostic approach and describe the pathognomonic findings at flexion magnetic resonance imaging. PMID:27800001

  8. A nationwide survey of clinical characteristics, management, and outcomes of acute kidney injury (AKI) - patients with and without preexisting chronic kidney disease have different prognoses.

    PubMed

    Pan, Heng-Chih; Wu, Pei-Chen; Wu, Vin-Cent; Yang, Ya-Fei; Huang, Tao-Min; Shiao, Chih-Chung; Chen, Te-Chuan; Tarng, Der-Cherng; Lin, Jui-Hsiang; Yang, Wei-Shun; Sun, Chiao-Yin; Lin, Chan-Yu; Chu, Tzong-Shinn; Wu, Mai-Szu; Wu, Kwan-Dun; Chen, Yung-Chang; Huang, Chiu-Ching

    2016-09-01

    Acute kidney injury (AKI) is a common complication in hospitalized patients. The International Society of Nephrology implemented the "0 by 25" initiative aimed at preventing deaths from treatable AKI worldwide by 2025 and conducted a global snapshot survey in 2014. We joined in the project and conducted this study to compare the epidemiology, risk factors, and prognosis between patients with pure AKI and those with acute-on-chronic kidney disease (ACKD). In this study, we prospectively collected demographic parameters and data on clinical characteristics, baseline comorbidities, management, and outcomes of 201 AKI patients in 18 hospitals in Taiwan from September 2014 to November 2014. The in-hospital mortality rate was 16%. AKI was mostly attributed to sepsis (52%). Multivariate logistic regression indicated that oliguria was a positive independent predictor of in-hospital mortality, whereas preexisting CKD and exposure to nephrotoxic agents were negative independent predictors. The prevalence of vasopressor use, intensive care unit care, and mortality were significantly higher in pure AKI patients than in ACKD patients. Moreover, serum creatinine (SCr) levels significantly increased within 7 days after AKI diagnosis in nonsurvivors but not in survivors in the pure AKI group. By contrast, SCr levels were persistently lower in nonsurvivors than in survivors in the ACKD group during the same period. We thus determined that the prognosis of ACKD patients differed from that of pure AKI patients. Considering the CKD history in the future AKI staging system may improve prognosis prediction. PMID:27684854

  9. The clinical significance of HRCT in evaluation of patients with rheumatoid arthritis-associated interstitial lung disease: a report from China.

    PubMed

    Zou, Yu-Qiong; Li, Ya-Song; Ding, Xiao-Nan; Ying, Zhen-Hua

    2012-03-01

    The objective of this study is to describe the interstitial lung disease (ILD) in rheumatoid arthritis (RA) patients of China, and to study clinical significance of high-resolution computed tomography (HRCT) in evaluation and treatment. One hundred and ten Chinese patients (79 women and 31 man) diagnosed with RA between December 2008 to November 2009 were analyzed. According to the HRCT, 47 (42.73%) RA patients were diagnosed as ILD. Old age, smoking and pulmonary rales were closely related to ILD (P < 0.05). The main appearances of ILD were ground-glass (39.09%), honeycombing (4.55%), reticular patterns and consolidation (1.82%). Patients with reticular patterns and honeycombing were more likely to show the respiratory symptoms. It was also common to find other abnormal changes, such as fiber cord shadow (22.73%), lung markings fuzzy disorder (30%), pulmonary nodules (11.82%), emphysema (9.09%), bronchiectasis (3.64%), subpleural nodules (11.82%) and pleural thickening (24.55%). In treatment, honeycombing and subpleural nodules were more common in patients with methotrexate (MTX) and/or leflunomide treatment than without (P < 0.05). Other abnormal changes were no statistical significance (P > 0.05). Pulmonary involvement is common in RA patients, and it is suggested that HRCT could be a sensitive and useful way in evaluating the lung of RA patients.

  10. Clinical Practice of Two Measurements of Home Blood Pressure on Each Occasion in Patients with Chronic Kidney Disease

    PubMed Central

    Okada, Tomonari; Wada, Toshikazu; Nagaoka, Yume; Kanno, Yoshihiko

    2015-01-01

    Background/Aims Although several guidelines propose two or three measurements of home blood pressure (HBP) on each occasion, the actual status of multiple measurements is not clear in the practical management of hypertension. We surveyed the details regarding two measurements of HBP in patients with chronic kidney disease (CKD). Methods HBP was measured twice every morning and evening over 7 consecutive days in 175 CKD patients. The distribution of the differences between two BP values (2nd – 1st BP) and their association with BP parameters were evaluated. Results The 2nd – 1st morning systolic BP (SBP) and diastolic BP (DBP) differences were −2.3 ± 4.1 and −0.4 ± 2.6 mm Hg, respectively. The proportion of 2nd – 1st morning SBP differences >0 mm Hg was 31.7% in a total of 1,195 measurements. Eighty patients (45.7%) had days with a difference ≤-5 mm Hg and days with a difference ≥5 mm Hg in morning SBP during 7 days. The multivariate regression analysis of the SD values of 2nd – 1st morning SBP as a dependent variable showed that the SD value of the 1st morning SBP (β = 0.65, p < 0.001) was a significant determinant. Conclusion Although the 2nd SBP was 2-3 mm Hg lower than the 1st SBP in the population as a whole, various differences were found for each subject during 7 days. 2nd – 1st BP variability might be associated with day-by-day 1st BP variability. PMID:27194992

  11. Neurophysiological Aspects and their relationship to clinical and functional impairment in patients with Chronic Obstructive Pulmonary Disease

    PubMed Central

    de Miranda Rocco, Carolina Chiusoli; Sampaio, Luciana Maria Malosá; Stirbulov, Roberto; Corrêa, João Carlos Ferrari

    2011-01-01

    OBJECTIVE: The purpose was to assess functional (balance L–L and A–P displacement, sit‐to‐stand test (SST) and Tinetti scale – balance and gait) and neurophysiological aspects (patellar and Achilles reflex and strength) relating these responses to the BODE Index. INTRODUCTION: The neurophysiological alterations found in patients with chronic obstructive pulmonary disease (COPD) are associated with the severity of the disease. There is also involvement of peripheral muscle which, in combination with neurophysiological impairment, may further compromise the functional activity of these patients. METHODS: A cross‐sectional study design was used. Twenty‐two patients with moderate to very severe COPD (>60 years) and 16 age‐matched healthy volunteers served as the control group (CG). The subjects performed spirometry and several measures of static and dynamic balance, monosynaptic reflexes, peripheral muscle strength, SST and the 6‐minute walk test. RESULTS: The individuals with COPD had a reduced reflex response, 36.77±3.23 (p<0.05) and 43.54±6.60 (p<0.05), achieved a lower number repetitions on the SST 19.27±3.88 (p<0.05), exhibited lesser peripheral muscle strength on the femoral quadriceps muscle, 24.98±6.88 (p<0.05) and exhibited deficits in functional balance and gait on the Tinetti scale, 26.86±1.69 (p<0.05), compared with the CG. The BODE Index demonstrated correlations with balance assessment (determined by the Tinetti scale), r = 0.59 (p<0.05) and the sit‐to‐stand test, r = 0.78 (p<0.05). CONCLUSIONS: The individuals with COPD had functional and neurophysiological alterations in comparison with the control group. The BODE Index was correlated with the Tinetti scale and the SST. Both are functional tests, easy to administer, low cost and feasible, especially the SST. These results suggest a worse prognosis; however, more studies are needed to identify the causes of these changes and the repercussions that could result in their

  12. Impact of Clinical Guideline Recommendations on the Application of Coronary Computed Tomographic Angiography in Patients with Suspected Stable Coronary Artery Disease

    PubMed Central

    Zhou, Jia; Yang, Jun-Jie; Yang, Xia; Chen, Zhi-Ye; He, Bai; Du, Luo-Shan; Chen, Yun-Dai

    2016-01-01

    Background: Coronary computed tomographic angiography (CCTA) has been widely used in patients who are at intermediate risk for having stable coronary artery disease (SCAD), and 2013 European Society of Cardiology Guidelines on the Management of SCAD (2013G) recommended the appropriate application of CCTA. However, 2013G has not been subjected to systematic analyses for subsequent impact on clinical practice. Methods: A total of 5320 patients suspected with SCAD were enrolled and scheduled for CCTA from March 2013 to September 2014. For each patient, pretest probability of SCAD was calculated according to updated Diamond-Forrester model (UDFM). Appropriate CCTA or appropriate stress test was determined as described in the 2013G. A generalized estimating equation model was used to determine the trends in the half-monthly rate of appropriate CCTA. Results: Overall, only 61.37% of patients received appropriate CCTA, and there was insignificant change over time (P = 0.8701). The application of CCTA in patients who should have had a stress test accounted for most of the inappropriate CCTA before (22.29%) or after (19.98%) the publication of the 2013G. In all patients or any subgroup, no significant change in the adjusted half-monthly rate of appropriate CCTA was found after the publication of the 2013G (odds ratio, 1.002; 95% confidence interval, 0.982–1.021; P = 0.8678). Conclusions: These findings suggest that the 2013G have not, to date, been fully incorporated into clinical practice, and the clinical utilization of CCTA remains unreasonable to some extent. PMID:26830982

  13. The effect of disease-modifying therapies on brain atrophy in patients with clinically isolated syndrome: a systematic review and meta-analysis

    PubMed Central

    Tsivgoulis, Georgios; Katsanos, Aristeidis H.; Grigoriadis, Nikolaos; Hadjigeorgiou, Georgios M.; Heliopoulos, Ioannis; Papathanasopoulos, Panagiotis; Dardiotis, Efthimios; Kilidireas, Constantinos; Voumvourakis, Konstantinos

    2015-01-01

    Objectives: Brain atrophy is associated with cognitive deficits in patients with clinically isolated syndrome (CIS) and can predict conversion to clinical definite multiple sclerosis. The aim of the present meta-analysis was to evaluate the effect of disease-modifying drugs (DMDs) on brain atrophy in patients with CIS. Methods: Eligible placebo-control randomized clinical trials of patients with CIS that had reported changes in brain volume during the study period were identified by searching the MEDLINE, SCOPUS, and Cochrane Central Register of Controlled Trials (CENTRAL) databases. This meta-analysis adopted the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines for systematic reviews and meta-analyses. Results: Three eligible studies were identified, comprising 1362 patients. The mean percentage change in brain volume was found to be significantly lower in DMD-treated patients versus placebo-treated subgroups (standardized mean difference [SMD]: = −0.13, 95% confidence interval [CI]: −0.25, 0.01; p = 0.04). In the subgroup analysis of the two studies that provided data on brain-volume changes for the first (0–12 months) and second (13–24 months) year of treatment, DMD attenuated brain-volume loss in comparison with placebo during the second year (SMD = −0.25; 95% CI: −0.43, −0.07; p < 0.001), but not during the first year of treatment (SMD = −0.01; 95% CI: −0.27, 0.24; p = 0.93). No evidence of heterogeneity was found between estimates, while funnel-plot inspection revealed no evidence of publication bias. Conclusions: DMDs appear to attenuate brain atrophy over time in patients with CIS. The effect of DMDs on brain-volume loss is evident after the first year of treatment. PMID:26557896

  14. Clinical characteristics and one year outcomes in Chinese atrial fibrillation patients with stable coronary artery disease: a population-based study

    PubMed Central

    Bai, Ying; Zhu, Jun; Yang, Yan-Min; Liang, Yan; Tan, Hui-Qiong; Wang, Juan; Huang, Bi; Zhang, Han; Shao, Xing-Hui

    2016-01-01

    Background Atrial fibrillation (AF) and coronary artery disease (CAD) often coexist, however, the clinical characteristics and the impact of stable CAD on the outcomes in Chinese patients with AF has not been well understood. Methods Consecutive AF patients in 20 hospitals in China from November 2008 to October 2011 were enrolled. The primary endpoints included 1-year all-cause mortality, stroke, non-central nervous system (non-CNS) embolism, and major bleeding. Results A total of 1947 AF patients were analyzed, of whom 40.5% had stable CAD. The mean CHADS2 scores in CAD patients were significantly higher than that of non-CAD patients (2.4 ± 1.4 vs. 1.4 ± 1.2, P < 0.001). During follow-up period, warfarin use is low in both groups, with relatively higher proportion in non-CAD patients compared with CAD patients (22.3% vs. 10.7%, P < 0.001). Compared with non-CAD patients, CAD patients had higher one-year all-cause mortality (16.8% vs. 12.9%, P = 0.017) and incidence of stroke (9.0% vs. 6.4%, P = 0.030), while the non-CNS embolism and major bleeding rates were comparable between the two groups. After multivariate adjustment, stable CAD was independently associated with increased risk of 1-year all-cause mortality (HR = 1.35, 95% CI: 1.01−1 .80, P = 0.040), but not associated with stroke (HR = 1.07, 95% CI: 0.72–1.58, P = 0.736). Conclusions Stable CAD was prevalent in Chinese AF patients and was independently associated with increased risk of 1-year all-cause mortality. Chinese AF patients with stable CAD received inadequate antithrombotic therapy and this grim status of antithrombotic therapy needed to be improved urgently. PMID:27781056

  15. Hypertension in patients with Alzheimer's disease--prevalence, characteristics, and impact on clinical outcome. Experience of one neurology center in Poland.

    PubMed

    Warchol-Celinska, Ewa; Styczynska, Maria; Prejbisz, Aleksander; Przybylowska, Katarzyna; Chodakowska-Zebrowska, Malgorzata; Kurjata, Pawel; Piotrowski, Walerian; Polakowska, Maria; Kabat, Marek; Zdrojewski, Tomasz; Drygas, Wojciech; Januszewicz, Andrzej; Barcikowska, Maria

    2015-09-01

    The aim of the study was to evaluate hypertension (HT) prevalence, characteristics, and impact on clinical outcome in patients with Alzheimer's disease (AD). We evaluated 701 patients with AD (249 males, 452 females, and mean age 74.9 ± 7.5 years). As a group representing general population matched with regard to age, education level, and place of residence, we included 762 subjects (438 males, 324 females, and mean age 74.7 ± 4.4 years) from the Polish National Multicenter Health Survey (WOBASZ) studies. The patients with AD were characterized by lower systolic blood pressure (BP) and diastolic BP values (134 ± 21 vs. 151 ± 23 mm Hg, P < .001 and 77 ± 11 vs. 86 ± 12 mm Hg, P < .001, respectively) as well as lower HT prevalence (66% vs. 78.6%, P < .001) compared with the WOBASZ group. In long-term follow-up of AD group, HT and BP levels were not associated with the decline in cognitive functions nor the increased risk of death. Patients with AD were characterized by lower prevalence of HT and other vascular risk factors. BP levels and HT had no impact on clinical outcome. PMID:26210391

  16. A structured exercise programme during haemodialysis for patients with chronic kidney disease: clinical benefit and long-term adherence

    PubMed Central

    Anding, Kirsten; Bär, Thomas; Trojniak-Hennig, Joanna; Kuchinke, Simone; Krause, Rolfdieter; Rost, Jan M; Halle, Martin

    2015-01-01

    Objective Long-term studies regarding the effect of a structured physical exercise programme (SPEP) during haemodialysis (HD) assessing compliance and clinical benefit are scarce. Study design A single-centre clinical trial, non-randomised, investigating 46 patients with HD (63.2±16.3 years, male/female 24/22, dialysis vintage 4.4 years) performing an SPEP over 5 years. The SPEP (twice/week for 60 min during haemodialysis) consisted of a combined resistance (8 muscle groups) and endurance (supine bicycle ergometry) training. Exercise intensity was continuously adjusted to improvements of performance testing. Changes in endurance and resistance capacity, physical functioning and quality of life (QoL) were analysed over 1 year in addition to long-term adherence and economics of the programme over 5 years. Average power per training session, maximal strength tests (maximal exercise repetitions/min), three performance-based tests for physical function, SF36 for QoL were assessed in the beginning and every 6 months thereafter. Results 78% of the patients completed the programme after 1 year and 43% after 5 years. Participants were divided—according to adherence to the programme—into three groups: (1) high adherence group (HA, >80% of 104 training sessions within 12 months), (2) moderate adherence (MA, 60–80%), and 3. Low adherence group (LA, <60%)) with HA and MA evaluated quantitatively. One-year follow-up data revealed significant (p<0.05) improvement for both groups in all measured parameters: exercise capacity (HA: 55%, MA: 45%), strength (HA: >120%, MA: 40–50%), QoL in three scores of SF36 subscales and physical function in the three tests taken between 11% and 31%. Moreover, a quantitative correlation analysis revealed a close association (r=0.8) between large improvement of endurance capacity and weak physical condition (HA). Conclusions The exercise programme described improves physical function significantly and can be integrated

  17. Association between high biomarker probability of Alzheimer's disease and improvement of clinical outcomes after shunt surgery in patients with idiopathic normal pressure hydrocephalus.

    PubMed

    Kazui, Hiroaki; Kanemoto, Hideki; Yoshiyama, Kenji; Kishima, Haruhiko; Suzuki, Yukiko; Sato, Shunsuke; Suehiro, Takashi; Azuma, Shingo; Yoshimine, Toshiki; Tanaka, Toshihisa

    2016-10-15

    We examined the effect of the pathology of Alzheimer's disease (AD) on improvement of clinical symptoms after shunt surgery in patients with idiopathic normal pressure hydrocephalus (iNPH). Forty-four iNPH patients were classified into 18 patients with (iNPH/AD+) and 26 patients without (iNPH/AD-) combination with low amyloid β42 and high total tau in cerebrospinal fluid (CSF). We compared improvements after lumbo-peritoneal shunt surgery (LPS) between the two groups in Timed Up & Go Test, 10-m reciprocating walking test, Digit Symbol Substitution Test, attention test, delayed recall test, Mini-Mental State Examination, iNPH grading scale, Neuropsychiatric Inventory, Zarit Burden Interview, and other evaluations. Three months after LPS, gait, urination, overall cognition, psychomotor speed, attention, and neuropsychiatric symptoms significantly improved in both groups, but the improvement in delayed recall and reduction of caregiver burden were significantly greater in iNPH/AD- than iNPH/AD+. In addition, improvement in delayed recall score after LPS was significantly and negatively correlated with the probability of AD as judged by amyloid β42 and total tau levels in CSF. Three months after LPS, almost all of the triad symptoms decreased in iNPH patients with and without AD pathology but memory improved only in iNPH patients without AD pathology. PMID:27653897

  18. Intake of copper has no effect on cognition in patients with mild Alzheimer’s disease: a pilot phase 2 clinical trial

    PubMed Central

    Kessler, Holger; Bach, Daniela; Schneider-Axmann, Thomas; Supprian, Tillmann; Herrmann, Wolfgang; Haber, Manfred; Multhaup, Gerd; Falkai, Peter; Pajonk, Frank-Gerald

    2008-01-01

    Disturbed copper (Cu) homeostasis may be associated with the pathological processes in Alzheimer’s disease (AD). In the present report, we evaluated the efficacy of oral Cu supplementation in the treatment of AD in a prospective, randomized, double-blind, placebo-controlled phase 2 clinical trial in patients with mild AD for 12 months. Sixty-eight subjects were randomized. The treatment was well-tolerated. There were however no significant differences in primary outcome measures (Alzheimer’s Disease Assessment Scale, Cognitive subscale, Mini Mental Status Examination) between the verum [Cu-(II)-orotate-dihydrate; 8 mg Cu daily] and the placebo group. Despite a number of findings supporting the hypothesis of environmental Cu modulating AD, our results demonstrate that oral Cu intake has neither a detrimental nor a promoting effect on the progression of AD. PMID:18587525

  19. Clinical and Epidemiological Characteristics of Kawasaki Disease

    PubMed Central

    Shamsizadeh, Ahmad; Ziaei Kajbaf, Tahereh; Razavi, Maryam; Cheraghian, Bahman

    2014-01-01

    Background: Kawasaki disease (KD) is an acute multisystem vascular syndrome of unknown etiology that is the leading cause of acquired heart disease in children of developed counties. Objectives: We aimed to evaluate the epidemiological characteristics and clinical manifestations of KD in children residing in the southwest of Iran. Patients and Methods: In this retrospective study, we reviewed the medical records of all children with KD who had been admitted to the main children’s hospital of Ahvaz, southwest Iran, from March 2000 to March 2010. Data regarding clinical and epidemiological characteristics, management, and the outcome of disease for each patient were obtained. The patients were divided into cardiac and non-cardiac groups based on echocardiographic results. Results: In total, 104 patients with KD (66 boys and 38 girls) were enrolled in this study. The male to female ratio was 1.7:1. The mean ± SD age of the patients was 33.6 ± 24.2 months. Most (87.2%) cases were from urban areas. The disease occurred more frequently during winter and spring. Furthermore, 61.5% of the children had the criteria of classic KD, and 38.5% were labeled as incomplete KD. The mean ± SD of the duration of hospital stay was 6.9 ± 2.4 days. The mean time between illness and admission to the hospital was 6.47 ± 2.6 days. The most common sign was fever, followed by conjunctivitis and oral changes. In total, 20% of the patients had cardiac abnormalities. There was no significant statistical difference between the cardiac and non-cardiac groups according to age, sex, clinical manifestations, laboratory findings, and cessation of fever. The duration of hospital stay and the time between onset of illness and diagnosis were longer in the cardiac group. All patients received intravenous immunoglobulin and aspirin. Only one patient continued to have cardiac abnormalities after 6 months of follow-up. Conclusions: Kawasaki disease is not rare in southwest of Iran. The age, gender

  20. Risky Sexual Behavior, Bleeding Caused by Intimate Partner Violence, and Hepatitis C Virus Infection in Patients of a Sexually Transmitted Disease Clinic

    PubMed Central

    Chen, Meng-Jinn; Nochajski, Thomas H.; Testa, Maria; Zimmerman, Scott J.; Hughes, Patricia S.

    2009-01-01

    Objectives. We sought to investigate independent contributions of risky sexual behaviors and bleeding caused by intimate partner violence to prediction of HCV infection. Methods. We conducted a case–control study of risk factors among patients of a sexually transmitted disease clinic with and without HCV antibodies, group-matched by age. Results. Multivariate analyses indicated that Black race (odds ratio [OR] = 2.4; 95% confidence interval [CI] = 1.3, 4.4), injection drug use (OR = 20.3; 95% CI = 10.8, 37.8), sharing straws to snort drugs (OR = 1.8; 95% CI = 1.01, 3.0), sharing razors (OR = 7.8; 95% CI = 2.0, 31.0), and exposure to bleeding caused by intimate partner violence (OR = 5.5; 95% CI = 1.4, 22.8) contributed significantly to the prediction of HCV infection; risky sexual behavior and exposure to blood or sores during sexual intercourse did not. Conclusions. HCV risk among patients of a sexually transmitted disease clinic can be explained by direct blood exposure, primarily through injection drug use. Exposure to bleeding caused by intimate partner violence may be a previously unrecognized mechanism for HCV transmission associated with risky sexual behavior. PMID:19218181

  1. Effect of pantoprazole in patients with chronic laryngitis and pharyngitis related to gastroesophageal reflux disease: clinical, proximal, and distal pH monitoring results.

    PubMed

    Karoui, S; Bibani, N; Sahtout, S; Zouiten, L; Kallel, L; Matri, S; Serghini, M; Ben Mustapha, N; Boubaker, J; Besbes, G; Filali, A

    2010-05-01

    Few studies had evaluated the results of proton pump inhibitors on distal and proximal pH recording using a dual-channel probe. The aim of this study was to determine the clinical and pH-metric effect of treatment with pantoprazole 80 mg for 8 weeks in patients with ear, nose, and throat (ENT) manifestations of gastroesophageal reflux disease associated with pathological proximal acid exposure. We conducted a prospective open study. Patients included had to have chronic pharyngitis or laryngitis, and a pathological gastroesophagopharyngeal reflux. All patients received treatment with pantoprazole 80 mg daily for 8 weeks. One week after the end treatment, patients had a second ENT examination and a 24-hour pH monitoring using dual-channel probe. We included 33 patients (11 men, 22 women). A pathological distal acid reflux was found in 30 patients (91%). After treatment, the improvement of ENT symptoms was found in 51.5% of patients. Normalization of 24-hour proximal esophageal pH monitoring was observed in 22 patients (66%). After treatment, the overall distal acid exposure, the number of distal reflux events, and the number of reflux during more than 5 minutes were significantly decreased (respectively: 19.4% vs 7.2% [P < 0.0001], 62.7 vs 28.4 [P < 0.0001], and 10.4 vs 3.9 [P < 0.0001] ). Similarly, in proximal level, the same parameters were significantly decreased after treatment (respectively: 6.8% vs 1.6% [P < 0.0001], 32.6 vs 8.1 [P < 0.0001], and 3.4 vs 0.6 [P= 0.005] ). Treatment with pantoprazole reduced the frequency and severity of gastroesophagopharyngeal acid reflux in patients with chronic pharyngitis and laryngitis.

  2. Commentary on "a roadmap for the prevention of dementia II. Leon Thal Symposium 2008." Centers of excellence in Alzheimer's disease: it is time to better integrate patient care and clinical research to improve the prevention and treatment of Alzheimer's disease.

    PubMed

    Doody, Rachelle S

    2009-03-01

    Despite enormous worldwide public and private interest in improving the prevention and treatment of Alzheimer's disease (AD), we have not made as much of an impact as we would like, and the number of affected individuals continues to grow. Even more alarmingly, whereas global efforts to identify AD cases and to develop new treatments are increasing, patient-care options are disappearing, so that even if a highly efficacious therapy or prevention approach arose, it would not be used effectively. As a first step toward organizing a better way forward, we should establish AD centers of excellence that mandate both patient care and research in the same setting. These centers would benefit from changes in public health policies related to chronic-disease surveillance, Center for Medicare and Medicaid Services funding for the care of chronic diseases, institutional review boards, good clinical practice guidelines, National Institutes of Health regulations for the use of research funds, Food and Drug Administration guidelines for the approval of AD drugs, and Department of Commerce regulations related to patent protection of AD diagnostic aids and treatments. This new form of AD centers of excellence would also provide direct care to many patients and their families, model care for communities and medical trainees, enhance the voluntary recruitment of AD patients to clinical trials, and improve our understanding of AD and its management.

  3. Effect of Oral Eliglustat vs Placebo on Spleen Volume in Patients with Splenomegaly and Gaucher Disease Type 1: The ENGAGE Randomized Clinical Trial

    PubMed Central

    Mistry, Pramod K.; Lukina, Elena; Turkia, Hadhami Ben; Amato, Dominick; Baris, Hagit; Dasouki, Majed; Ghosn, Marwan; Mehta, Atul; Packman, Seymour; Pastores, Gregory; Petakov, Milan; Assouline, Sarit; Balwani, Manisha; Danda, Sumita; Hadjiev, Evgueniy; Ortega, Andres; Shankar, Suma; Solano, Maria Helena; Ross, Leorah; Angell, Jennifer; Peterschmitt, M. Judith

    2016-01-01

    Importance In Gaucher disease type 1, inherited deficiency of acid-β-glucosidase underlies accumulation of glucosylceramide in lysosomes of macrophages and resultant hepatosplenomegaly, anemia, thrombocytopenia, and skeletal disease. The standard of care is lifelong intravenous enzyme replacement therapy. A safe, effective oral therapy appropriate for a broad spectrum of patients is an important unmet need. Objective To determine whether eliglustat, a novel oral substrate-reduction therapy, safely reverses clinical manifestations in previously untreated adults with Gaucher disease type 1. Design, Setting, and Participants Phase-3, randomized, double-blind, placebo-controlled, multinational trial conducted from November 2009 to July 2012 in eligible untreated patients with Gaucher disease type 1 who had splenomegaly plus thrombocytopenia and/or anemia. Interventions Patients were stratified by spleen volume and randomized 1:1 to receive eliglustat (50 or 100 mg twice daily) or placebo for 9 months. Main Outcome and Measures The primary efficacy endpoint was percent change in spleen volume from baseline to 9 months; secondary efficacy endpoints were change in hemoglobin and percent changes in liver volume and platelet count. Results Of 72 patients screened, 40 patients from 12 countries and 18 sites were enrolled. All had baseline splenomegaly and thrombocytopenia (mostly moderate or severe), most had mild to moderate hepatomegaly and moderate to severe bone marrow infiltration, and 20% had mild anemia. Least square mean spleen volume decreased by 27.8% (95% CI: −32.57, −22.97) in the eliglustat group (13.89 MN to 10.17 MN) compared to an increase of 2.3% (95% CI: −2.54, 7.06) in the placebo group (12.50 MN to 12.84 MN), for an overall treatment difference of −30% (95% CI: −36.82, −23.24, P<0.001). For the secondary endpoints, the least squares mean difference between groups all favored eliglustat over placebo with a 1.2 g/dL increase in hemoglobin level

  4. HRCT score and serum ferritin level are factors associated to the 1-year mortality of acute interstitial lung disease in clinically amyopathic dermatomyositis patients.

    PubMed

    Zou, Jing; Guo, Qiang; Chi, Jiachang; Wu, Huawei; Bao, Chunde

    2015-04-01

    The aim of this study is to evaluate the factors associated to 1-year mortality in clinically amyopathic dermatomyositis (CADM) patients with acute interstitial lung disease (ILD). A single center of 37 cases of Chinese patients with CADM was reviewed retrospectively in Renji hospital. All CADM patients were diagnosed with ILD; there were 24 cases of acute interstitial pneumonia (AIP) and 13 cases of acute exacerbation of non-acute interstitial pneumonia non-AIP. The clinical features, including blood tests, chest high-resolution computed tomography (HRCT) score, and lung function, were analyzed, respectively. Neutrophil lymphocyte ratio (NLR), serum ferritin level, serum lactate dehydrogenase (LDH) level, and HRCT score were statistically significant factors on univariate analysis. Multivariate analysis revealed that the overall HRCT score (HR 1.134, 95 % confidence interval 1.009-1.275, P = 0.017) and serum ferritin level (HR 1.001, 95 % confidence interval 1.002-1.007, P = 0.010) were independently significant factors of 1-year mortality. C statistic value of HRCT score (c statistic value 0.867, P < 0.0001) and serum ferritin level (c statistic value 0.808, P = 0.002) were statistically significant in the classification of non-survivors. Patients with calcineurin inhibitor presented a better outcome than those without calcineurin inhibitor (log-rank test, P = 0.006). HRCT score and serum ferritin level are factors associated to the 1-year mortality of acute ILD in CADM patients. Calcineurin inhibitor might improve the outcome of CADM patients with acute ILD.

  5. An exploratory double-blind, randomized clinical trial with selisistat, a SirT1 inhibitor, in patients with Huntington’s disease

    PubMed Central

    Süssmuth, Sigurd D; Haider, Salman; Landwehrmeyer, G Bernhard; Farmer, Ruth; Frost, Chris; Tripepi, Giovanna; Andersen, Claus A; Di Bacco, Marco; Lamanna, Claudia; Diodato, Enrica; Massai, Luisa; Diamanti, Daniela; Mori, Elisa; Magnoni, Letizia; Dreyhaupt, Jens; Schiefele, Karin; Craufurd, David; Saft, Carsten; Rudzinska, Monika; Ryglewicz, Danuta; Orth, Michael; Brzozy, Sebastian; Baran, Anna; Pollio, Giuseppe; Andre, Ralph; Tabrizi, Sarah J; Darpo, Borje; Westerberg, Goran

    2015-01-01

    Aims Selisistat, a selective SirT1 inhibitor is being developed as a potentially disease-modifying therapeutic for Huntington's disease (HD). This was the first study of selisistat in HD patients and was primarily aimed at development of pharmacodynamic biomarkers. Methods This was a randomized, double-blind, placebo-controlled, multicentre exploratory study. Fifty-five male and female patients in early stage HD were randomized to receive 10 mg or 100 mg of selisistat or placebo once daily for 14 days. Blood sampling, clinical and safety assessments were conducted throughout the study. Candidate pharmacodynamic markers included circulating soluble huntingtin and innate immune markers. Results Selisistat was found to be safe and well tolerated, and systemic exposure parameters showed that the average steady-state plasma concentration achieved at the 10 mg dose level (125 nm) was comparable with the IC50 for SirT1 inhibition. No adverse effects on motor, cognitive or functional readouts were recorded. While circulating levels of soluble huntingtin were not affected by selisistat in this study, the biological samples collected have allowed development of assay technology for use in future studies. No effects on innate immune markers were seen. Conclusions Selisistat was found to be safe and well tolerated in early stage HD patients at plasma concentrations within the anticipated therapeutic concentration range. PMID:25223731

  6. MAGnesium-oral supplementation to reduce PAin in patients with severe PERipheral arterial occlusive disease: the MAG-PAPER randomised clinical trial protocol

    PubMed Central

    Venturini, Monica Aida; Zappa, Sergio; Minelli, Cosetta; Bonardelli, Stefano; Lamberti, Laura; Bisighini, Luca; Zangrandi, Marta; Turin, Maddalena; Rizzo, Francesco; Rizzolo, Andrea; Latronico, Nicola

    2015-01-01

    Introduction Magnesium exerts analgaesic effects in several animal pain models, as well as in patients affected by acute postoperative pain and neuropathic chronic pain. There is no evidence that magnesium can modulate pain in patients with peripheral arterial occlusive disease (PAOD). We describe the protocol of a single-centre randomised double-blind clinical trial aimed at assessing the efficacy of oral magnesium supplementation in controlling severe pain in patients with advanced PAOD. Methods and analysis Adult patients affected by PAOD at stages III and IV of Lèriche-Fontaine classification, who are opioid-naïve, and who have been admitted to our Acute Pain Service for intractable pain, will be eligible. Patients will be randomised to the control group, treated with standard therapy (oxycodone and pregabalin) plus placebo for 2 weeks, or to the experimental group (standard therapy plus magnesium oxide). Patients will be evaluated on days 0, 2, 4, 6, 8, 12 and 14; the following information will being collected: daily oxycodone dose; average and maximum pain (Numerical Rating Scale); pain relief (Pain Relief Scale); characteristics of the pain (Neuropathic Pain Scale); impact of pain on the patient's daily activities (Brief Pain Inventory). The primary outcome will be oxycodone dosage needed to achieve satisfactory analgaesia on day 14. Secondary outcomes will be pain relief on day 2, time needed to achieve satisfactory analgaesia and time needed to achieve a pain reduction of 50%. A sample size calculation was performed for the primary outcome, which estimated a required sample size of 150 patients (75 per group). Ethics and dissemination Ethical approval of the study protocol has been obtained from Comitato Etico Provinciale di Brescia, Brescia, Italy. Trial results will be disseminated through scientific journal manuscripts and scientific conference presentations. Trial registration number NCT02455726. PMID:26674497

  7. [Clinical, biological, therapeutic and evolving profile of patients with HIV infection hospitalized at Infectious and tropical diseases unit in Abidjan (Ivory Coast)].

    PubMed

    Kra, O; Aba, Y T; Yao, K H; Ouattara, B; Abouo, F; Tanon, K A; Eholié, S; Bissagnené, E

    2013-02-01

    The objective of this study is to describe the clinical, biological, therapeutic and evolving current profile of hospitalized patients with HIV infection in the cohort of the Infectious and Tropical Diseases Unit (ITDU) in the aim to improve their care management. This is a retrospective study, conducted on medical data of hospitalized cases of patients with HIV infection in the ITDU at the teaching hospital of Treichville (Abidjan) from 2006 to 2007. During the two years, 447 patients were included in the study. Their average age was 39 years [18 years-86 years] and sex ratio was 0.69. Of the 447 patients, 35% were unemployed and 67% were new patients who had never undergone antiretroviral therapy (ART). The duration of drug exposure was less than 6 months in 59% of treated patients. The average time to initiate ART was seven weeks. Among naive patients 41.9% were lost to follow up, 35.9% were waiting for treatment and 22.1% waiting for baseline biological test to initiate ART. At the initiation of ART, 79.6% of patients had a CD4 count less than 200/mm(3). The reasons of hospitalization defining AIDS were dominated by tuberculosis (34.2%), cerebral toxoplasmosis (17.9%) and neuromeningeal cryptococcosis (8%). The main reasons of hospitalization in classifying non-AIDS were pyelonephritis (6.5%), bacterial pneumonia (5.4%) and undetermined infectious encephalitis (4.9%). Hospital mortality was 24.4%. The leading causes of death were tuberculosis (22.9%), cerebral toxoplasmosis (20.2%), undetermined infectious encephalitis (18.3%) and cryptococcal meningitis (13.7%). The profile of PLHIV in hospital is characterized by profound immunosuppression due to late diagnosis and high mortality associated with severe opportunistic infections and late initiation of ART.

  8. Clinical characteristics of 154 patients suspected of having Ebola virus disease in the Ebola holding center of Jui Government Hospital in Sierra Leone during the 2014 Ebola outbreak.

    PubMed

    Yan, T; Mu, J; Qin, E; Wang, Y; Liu, L; Wu, D; Jia, H; Li, Z; Guo, T; Wang, X; Qin, Y; Li, Y; Chen, S; Zhang, Y; Zhang, J; Wu, Y; Wang, S; Li, J

    2015-10-01

    This article sought to analyze the clinical features of 154 patients suspected of having Ebola virus disease (EVD) in an Ebola holding center in Sierra Leone from October 1 through November 9, 2014. We found that 108 of the 154 patients were confirmed with EVD. Eighty-five had known outcomes. Forty-nine of the 85 patients had been exposed to EVD. The average mortality rate was 60%. The mean interval between the onset of symptoms and hospitalization was 5.8 ± 3.3 days. The mean incubation period was 9.2 ± 6.7 days. Common symptoms of the EVD patients on admission were fatigue (85.2%), anorexia (84.3%), fever (75.9%), and headache (72.2%). Our data showed that the total symptoms of confirmed EVD patients were significantly higher than those of non-EVD patients (9 vs. 5.5; p < 0.001). The likelihood of EVD was 87.6% when a patient presented more than 6 out of 21 symptoms on admission. The survivors were significantly younger than non-survivors (24.0 ± 10.0 years vs. 31.3 ± 15.3 years; p = 0.016). The real-time polymerase chain reaction (PCR) analysis showed that, in the survivors, the virus load was significantly lower (Ct value: 25.2 ± 4.1 vs. 28.7 ± 5.7; p = 0.002). Multivariate analysis showed that age, fever, and viral load were independent predictors of mortality. Taken together, our data suggested that a cutoff of six symptoms could be used to predict patients with high or low risk of EVD. It seemed that age, fever, and viral load were the main risk factors associated with EVD mortality. PMID:26223324

  9. Shared Risk Factors for Cardiovascular Disease and Cancer: Implications for Preventive Health and Clinical Care in Oncology Patients.

    PubMed

    Johnson, Christopher B; Davis, Margot K; Law, Angeline; Sulpher, Jeffrey

    2016-07-01

    The cardiovascular toxicity of cancer therapy has raised awareness of the importance of heart disease in cancer care among oncologists and cardiologists, leading to the new interdisciplinary field of cardio-oncology. Evidence is accumulating to suggest that risk factors associated with cardiovascular disease are also related to an increased incidence of cancer and excess cancer mortality. We review the epidemiologic evidence that smoking, obesity, poor diet, and inactivity can cause both heart disease and cancer. The importance of cardiovascular disease and cardiovascular risk factors in adversely affecting oncological outcomes and leading to increased cancer mortality is discussed. Cardiotoxicity prediction tools that incorporate cardiac disease and risk factors are described. Raising awareness about shared risk factors for cancer and heart disease may result in more effective advocacy to promote healthy lifestyle changes through the combined efforts of the historically separate specialties of cardiology and oncology. PMID:27343745

  10. Clinical outcome of Crohn's disease: analysis according to the vienna classification and clinical activity.

    PubMed

    Veloso, F T; Ferreira, J T; Barros, L; Almeida, S

    2001-11-01

    The aim of this study was to describe the clinical course of Crohn's disease (CD) in a well-defined, homogeneous groups of patients. A total of 480 patients with CD were followed up from diagnosis up to 20 years. Definitions of patient subgroups were made according to the Vienna Classification. Markov chain analysis was used to estimate the probabilities of remissions and relapses during the disease course. Both age at diagnosis and behavior were associated with different disease locations. Patients with ileal disease had a greater need for surgical and a lesser need for immunosuppressive treatment; patients with ileocolonic disease were diagnosed at an earlier age and showed a lower probability of remaining in remission during the disease course; patients with colonic disease needed less surgical or steroid treatments; patients with intestinal penetrating disease were frequently submitted to abdominal surgery, whereas those with anal-penetrating disease often needed immunosuppressive treatment. Approximately 40% of the patients were in clinical remission at any time, but only about 10% maintained a long-term remission free of steroids after their initial presentation. A more benign clinical course could be predicted in patients who stay in remission in the year after diagnosis. The grouping of patients with CD according to the Vienna Classification and/or the clinical activity in the year after diagnosis is useful in predicting the subsequent course of disease. PMID:11720320

  11. Resilience in Patients with Ischemic Heart Disease

    PubMed Central

    de Lemos, Conceição Maria Martins; Moraes, David William; Pellanda, Lucia Campos

    2016-01-01

    Background Resilience is a psychosocial factor associated with clinical outcomes in chronic diseases. The relationship between this protective factor and certain diseases, such heart diseases, is still under-explored. Objective The present study sought to investigate the frequency of resilience in individuals with ischemic heart disease. Method This was a cross-sectional study with 133 patients of both genders, aged between 35 and 65 years, treated at Rio Grande do Sul Cardiology Institute - Cardiology University Foundation, with a diagnosis of ischemic heart disease during the study period. Sixty-seven patients had a history of acute myocardial infarction. The individuals were interviewed and evaluated by the Wagnild & Young resilience scale and a sociodemographic questionnaire. Results Eighty-one percent of patients were classified as resilient according to the scale. Conclusion In the sample studied, resilience was identified in high proportion among patients with ischemic heart disease. PMID:26815312

  12. Smart Technology in Lung Disease Clinical Trials.

    PubMed

    Geller, Nancy L; Kim, Dong-Yun; Tian, Xin

    2016-01-01

    This article describes the use of smart technology by investigators and patients to facilitate lung disease clinical trials and make them less costly and more efficient. By "smart technology" we include various electronic media, such as computer databases, the Internet, and mobile devices. We first describe the use of electronic health records for identifying potential subjects and then discuss electronic informed consent. We give several examples of using the Internet and mobile technology in clinical trials. Interventions have been delivered via the World Wide Web or via mobile devices, and both have been used to collect outcome data. We discuss examples of new electronic devices that recently have been introduced to collect health data. While use of smart technology in clinical trials is an exciting development, comparison with similar interventions applied in a conventional manner is still in its infancy. We discuss advantages and disadvantages of using this omnipresent, powerful tool in clinical trials, as well as directions for future research. PMID:26135330

  13. Smart Technology in Lung Disease Clinical Trials.

    PubMed

    Geller, Nancy L; Kim, Dong-Yun; Tian, Xin

    2016-01-01

    This article describes the use of smart technology by investigators and patients to facilitate lung disease clinical trials and make them less costly and more efficient. By "smart technology" we include various electronic media, such as computer databases, the Internet, and mobile devices. We first describe the use of electronic health records for identifying potential subjects and then discuss electronic informed consent. We give several examples of using the Internet and mobile technology in clinical trials. Interventions have been delivered via the World Wide Web or via mobile devices, and both have been used to collect outcome data. We discuss examples of new electronic devices that recently have been introduced to collect health data. While use of smart technology in clinical trials is an exciting development, comparison with similar interventions applied in a conventional manner is still in its infancy. We discuss advantages and disadvantages of using this omnipresent, powerful tool in clinical trials, as well as directions for future research.

  14. Moyamoya Disease: Epidemiology, Clinical Features, and Diagnosis

    PubMed Central

    Kim, Jong S.

    2016-01-01

    Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology remains unknown, recent genetic studies identified RNF213 in the 17q25-ter region as an important susceptibility gene of MMD among East Asian populations. Possibly because of genetic differences, MMD is relatively common in people living in East Asian countries such as Korea and Japan, compared to those in the Western Hemisphere. The prevalence of MMD appears to be slightly lower among Chinese, compared to Koreans or Japanese. There are two peaks of incidence with different clinical presentations, at around 10 years and 30-40 years. The peak appears to occur later in women than men. In children, ischemic symptoms, especially transient ischemic attacks, are predominant. Intellectual decline, seizures, and involuntary movements are also more common in this age group. In contrast, adult patients present with intracranial hemorrhage more often than pediatric patients. In patients with MMD, intracerebral hemorrhage is more often accompanied by intraventricular hemorrhage than in patients with hypertensive intracerebral hemorrhage. These different age peaks and different clinical presentations in each age group are also observed in MMD patients in the USA. Catheter angiography is the diagnostic method of choice. Magnetic resonance (MR) angiography and computed tomographic angiography are noninvasive diagnostic methods. High-resolution vessel wall MR imaging also helps diagnose MMD by revealing concentric vessel wall narrowing with basal collaterals. PMID:26846755

  15. 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

  16. Pharmacokinetics and clinical use of incretin-based therapies in patients with chronic kidney disease and type 2 diabetes.

    PubMed

    Scheen, André J

    2015-01-01

    The prevalence of chronic kidney disease (CKD) of stages 3-5 (glomerular filtration rate [GFR] <60 mL/min) is about 25-30 % in patients with type 2 diabetes mellitus (T2DM). While most oral antidiabetic agents have limitations in patients with CKD, incretin-based therapies are increasingly used for the management of T2DM. This review analyses (1) the influence of CKD on the pharmacokinetics of dipeptidyl peptidase-4 (DPP-4) inhibitors and glucagon-like peptide-1 (GLP-1) receptor agonists; and (2) the efficacy/safety profile of these agents in clinical practice when prescribed in patients with both T2DM and CKD. Most DPP-4 inhibitors (sitagliptin, vildagliptin, saxagliptin, alogliptin) are predominantly excreted by the kidneys. Thereby, pharmacokinetic studies showed that total exposure to the drug is increased in proportion to the decline of GFR, leading to recommendations for appropriate dose reductions according to the severity of CKD. In these conditions, clinical studies reported a good efficacy and safety profile in patients with CKD. In contrast, linagliptin is eliminated by a predominantly hepatobiliary route. As a pharmacokinetic study showed only minimal influence of decreased GFR on total exposure, no dose adjustment of linagliptin is required in the case of CKD. The experience with GLP-1 receptor agonists in patients with CKD is more limited. Exenatide is eliminated by renal mechanisms and should not be given in patients with severe CKD. Liraglutide is not eliminated by the kidney, but it should be used with caution because of the limited experience in patients with CKD. Only limited pharmacokinetic data are also available for lixisenatide, exenatide long-acting release (LAR) and other once-weekly GLP-1 receptor agonists in current development. Several case reports of acute renal failure have been described with GLP-1 receptor agonists, probably triggered by dehydration resulting from gastrointestinal adverse events. However, increasing GLP-1 may

  17. Subthalamic Nucleus Deep Brain Stimulation Modulate Catecholamine Levels with Significant Relations to Clinical Outcome after Surgery in Patients with Parkinson’s Disease

    PubMed Central

    Yamamoto, Tatsuya; Uchiyama, Tomoyuki; Higuchi, Yoshinori; Asahina, Masato; Hirano, Shigeki; Yamanaka, Yoshitaka; Kuwabara, Satoshi

    2015-01-01

    Aims Although subthalamic nucleus deep brain stimulation (STN-DBS) is effective in patients with advanced Parkinson’s disease (PD), its physiological mechanisms remain unclear. Because STN-DBS is effective in patients with PD whose motor symptoms are dramatically alleviated by L-3,4-dihydroxyphenylalanine (L-DOPA) treatment, the higher preoperative catecholamine levels might be related to the better clinical outcome after surgery. We aimed to examine the correlation between the preoperative catecholamine levels and postoperative clinical outcome after subthalamic nucleus deep brain stimulation. The effectiveness of STN-DBS in the patient who responded well to dopaminergic medication suggest the causal link between the dopaminergic system and STN-DBS. We also examined how catecholamine levels were modulated after subthalamic stimulation. Methods In total 25 patients with PD were enrolled (Mean age 66.2 ± 6.7 years, mean disease duration 11.6 ± 3.7 years). Mean levodopa equivalent doses were 1032 ± 34.6 mg before surgery. Cerebrospinal fluid and plasma catecholamine levels were measured an hour after oral administration of antiparkinsonian drugs before surgery. The mean Unified Parkinson’s Disease Rating Scale scores (UPDRS) and the Parkinson’s disease Questionnaire-39 (PDQ-39) were obtained before and after surgery. Of the 25 patients, postoperative cerebrospinal fluid and plasma were collected an hour after oral administration of antiparkinsonian drugs during on stimulation at follow up in 11 patients. Results Mean levodopa equivalent doses significantly decreased after surgery with improvement in motor functions and quality of life. The preoperative catecholamine levels had basically negative correlations with postoperative motor scores and quality of life, suggesting that higher preoperative catecholamine levels were related to better outcome after STN-DBS. The preoperative plasma levels of L-DOPA had significantly negative correlations with

  18. Sickle cell disease: clinical management.

    PubMed

    Ballas, S K

    1998-03-01

    Sickle cell syndromes are a group of inherited disorders of haemoglobin structure that have no cure in adults at the present time. Bone marrow transplantation in children has been shown to be curative in selected patients. The phenotypic expression of these disorders and their clinical severity vary greatly among patients and longitudinally in the same patient. They are multisystem disorders and influence all aspects of the life of affected individuals including social interactions, family relations, peer interaction, intimate relationships, education, employment, spiritual attitudes and navigating the complexities of the health care system, providers and their ancillary functions. The clinical manifestations of these syndromes are protean. In this review emphasis is placed on four sets of major complications of these syndromes and their management. The first set pertains to the management of anaemia and its sequelae; the second set addresses painful syndromes both acute and chronic; the third set discusses infections; the fourth section deals with organ failure. New experimental therapies for these disorders are briefly mentioned at the end. Efforts were made to include several tables and figures to clarify the message of this review.

  19. The care of patients with varicose veins and associated chronic venous diseases: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum.

    PubMed

    Gloviczki, Peter; Comerota, Anthony J; Dalsing, Michael C; Eklof, Bo G; Gillespie, David L; Gloviczki, Monika L; Lohr, Joann M; McLafferty, Robert B; Meissner, Mark H; Murad, M Hassan; Padberg, Frank T; Pappas, Peter J; Passman, Marc A; Raffetto, Joseph D; Vasquez, Michael A; Wakefield, Thomas W

    2011-05-01

    The Society for Vascular Surgery (SVS) and the American Venous Forum (AVF) have developed clinical practice guidelines for the care of patients with varicose veins of the lower limbs and pelvis. The document also includes recommendations on the management of superficial and perforating vein incompetence in patients with associated, more advanced chronic venous diseases (CVDs), including edema, skin changes, or venous ulcers. Recommendations of the Venous Guideline Committee are based on the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system as strong (GRADE 1) if the benefits clearly outweigh the risks, burden, and costs. The suggestions are weak (GRADE 2) if the benefits are closely balanced with risks and burden. The level of available evidence to support the evaluation or treatment can be of high (A), medium (B), or low or very low (C) quality. The key recommendations of these guidelines are: We recommend that in patients with varicose veins or more severe CVD, a complete history and detailed physical examination are complemented by duplex ultrasound scanning of the deep and superficial veins (GRADE 1A). We recommend that the CEAP classification is used for patients with CVD (GRADE 1A) and that the revised Venous Clinical Severity Score is used to assess treatment outcome (GRADE 1B). We suggest compression therapy for patients with symptomatic varicose veins (GRADE 2C) but recommend against compression therapy as the primary treatment if the patient is a candidate for saphenous vein ablation (GRADE 1B). We recommend compression therapy as the primary treatment to aid healing of venous ulceration (GRADE 1B). To decrease the recurrence of venous ulcers, we recommend ablation of the incompetent superficial veins in addition to compression therapy (GRADE 1A). For treatment of the incompetent great saphenous vein (GSV), we recommend endovenous thermal ablation (radiofrequency or laser) rather than high ligation and inversion stripping

  20. Comparison of Two Assays for Molecular Determination of Rifampin Resistance in Clinical Samples from Patients with Buruli Ulcer Disease

    PubMed Central

    Phillips, Richard Odame; Badziklou, Kossi; Piten, Ebekalisai; Maman, Issaka; Sarfo, Fred Stephen; Huber, Kristina Lydia; Rhomberg, Agata; Symank, Dominik; Wagner, Magdalena; Wiedemann, Franz; Nitschke, Jörg; Banla Kere, Abiba; Herbinger, Karl-Heinz; Adjei, Ohene; Löscher, Thomas; Bretzel, Gisela

    2014-01-01

    This study evaluates a novel assay for detecting rifampin resistance in clinical Mycobacterium ulcerans isolates. Although highly susceptible for PCR inhibitors in 50% of the samples tested, the assay was 100% M. ulcerans specific and yielded >98% analyzable sequences with a lower limit of detection of 100 to 200 copies of the target sequence. PMID:24478404

  1. The Effect of Symbiotic Supplementation on Liver Enzymes, C-reactive Protein and Ultrasound Findings in Patients with Non-alcoholic Fatty Liver Disease: A Clinical Trial

    PubMed Central

    Asgharian, Atefe; Askari, Gholamreza; Esmailzade, Ahmad; Feizi, Awat; Mohammadi, Vida

    2016-01-01

    Background: Regarding to the growing prevalence of nonalcoholic fatty liver disease (NAFLD), concentrating on various strategies to its prevention and management seems necessary. The aim of this study was to determine the effects of symbiotic on C-reactive protein (CRP), liver enzymes, and ultrasound findings in patients with NAFLD. Methods: Eighty NAFLD patients were enrolled in this randomized, double-blind, placebo-controlled clinical trial. Participants received symbiotic in form of a 500 mg capsule (containing seven species of probiotic bacteria and fructooligosaccharides) or a placebo capsule daily for 8 weeks. Ultrasound grading, CRP, and liver enzymes were evaluated at the baseline and the end of the study. Results: In the symbiotic group, ultrasound grade decreased significantly compared to baseline (P < 0.005) but symbiotic supplementation was not associated with changes in alanine aminotransferase (ALT) and aspartate transaminase (AST) levels. In the placebo group, there was no significant change in steatosis grade whereas ALT and AST levels were significantly increased (P = 0.002, P = 0.02, respectively). CRP values remained static in either group. Conclusions: Symbiotic supplementation improved steatosis in NAFLD patients and might be useful in the management of NAFLD or protective against its progression. PMID:27076897

  2. Clinical and radiological features of bone disease in long-term (15 or more years) hemodialysis patients.

    PubMed

    Bedani, P L; Orzincolo, C; Storari, A; Perini, L; Soffritti, S; Gilli, P

    1993-10-01

    Fifteen patients on regular dialytic treatment for more than 15 years were given X-rays of the skull, spine, shoulders, wrists, pelvis and knees with the purpose of studying the principal skeletal and articular alterations due or not due to the uraemic status. Serum calcium, phosphorus, parathyroid hormone, alkaline phosphatase and basal aluminium were recorded. Osteopenia was evident in all the patients. Ten of whom (67%) showed alterations due to hyperparathyroidism. Nine patients presented the marks of dialysis spondyloarthropathy; in 14/15 cases geodes were present in the wrists, humeral heads or hip-joints; in ten patients there were multiple amyloid lesions. Two patients with serum basal aluminum above 100 micrograms/L showed the typical radiographic marks of osteomalacia. The majority of the long-term survivors showed multifactorial osteo-articular alterations resulting mainly from the combination of hyperparathyroidism and dialysis-related amyloidosis. The less frequent joint alterations were represented by arthrosis, enthesopathy and chondrocalcinosis. Disability and decreased articular mobility resulted in being mainly due to amyloid osteo-arthropathy.

  3. Can Gait Signatures Provide Quantitative Measures for Aiding Clinical Decision-Making? A Systematic Meta-Analysis of Gait Variability Behavior in Patients with Parkinson's Disease.

    PubMed

    König, Niklas; Singh, Navrag B; Baumann, Christian R; Taylor, William R

    2016-01-01

    A disturbed, inconsistent walking pattern is a common feature of patients with Parkinson's disease (PwPD). Such extreme variability in both temporal and spatial parameters of gait has been associated with unstable walking and an elevated prevalence of falls. However, despite their ability to discretise healthy from pathological function, normative variability values for key gait parameters are still missing. Furthermore, an understanding of each parameter's response to pathology, as well as the inter-parameter relationships, has received little attention. The aim of this systematic literature review and meta-analysis was therefore to define threshold levels for pathological gait variability as well as to investigate whether all gait parameters are equally perturbed in PwPD. Based on a broader systematic literature search that included 13'195 titles, 34 studies addressed Parkinson's disease, presenting 800 PwPD and 854 healthy subjects. Eight gait parameters were compared, of which six showed increased levels of variability during walking in PwPD. The most commonly reported parameter, coefficient of variation of stride time, revealed an upper threshold of 2.4% to discriminate the two groups. Variability of step width, however, was consistently lower in PwPD compared to healthy subjects, and therefore suggests an explicit sensory motor system control mechanism to prioritize balance during walking. The results provide a clear functional threshold for monitoring treatment efficacy in patients with Parkinson's disease. More importantly, however, quantification of specific functional deficits could well provide a basis for locating the source and extent of the neurological damage, and therefore aid clinical decision-making for individualizing therapies. PMID:27445759

  4. Can Gait Signatures Provide Quantitative Measures for Aiding Clinical Decision-Making? A Systematic Meta-Analysis of Gait Variability Behavior in Patients with Parkinson's Disease

    PubMed Central

    König, Niklas; Singh, Navrag B.; Baumann, Christian R.; Taylor, William R.

    2016-01-01

    A disturbed, inconsistent walking pattern is a common feature of patients with Parkinson's disease (PwPD). Such extreme variability in both temporal and spatial parameters of gait has been associated with unstable walking and an elevated prevalence of falls. However, despite their ability to discretise healthy from pathological function, normative variability values for key gait parameters are still missing. Furthermore, an understanding of each parameter's response to pathology, as well as the inter-parameter relationships, has received little attention. The aim of this systematic literature review and meta-analysis was therefore to define threshold levels for pathological gait variability as well as to investigate whether all gait parameters are equally perturbed in PwPD. Based on a broader systematic literature search that included 13′195 titles, 34 studies addressed Parkinson's disease, presenting 800 PwPD and 854 healthy subjects. Eight gait parameters were compared, of which six showed increased levels of variability during walking in PwPD. The most commonly reported parameter, coefficient of variation of stride time, revealed an upper threshold of 2.4% to discriminate the two groups. Variability of step width, however, was consistently lower in PwPD compared to healthy subjects, and therefore suggests an explicit sensory motor system control mechanism to prioritize balance during walking. The results provide a clear functional threshold for monitoring treatment efficacy in patients with Parkinson's disease. More importantly, however, quantification of specific functional deficits could well provide a basis for locating the source and extent of the neurological damage, and therefore aid clinical decision-making for individualizing therapies. PMID:27445759

  5. [Clinical value of the sequential study of the uric acid in CSF in patients with cerebral diseases: Part I. Brain tumor and the effect of irradiation].

    PubMed

    Ueda, T; Wakisaka, S; Kinoshita, K; Adachi, H

    1984-03-01

    Uric acid is the end product of the purine metabolism in the human and is mainly excreted to the urine. The studies on cerebrospinal fluid (CSF) uric acid in patients with various neurological diseases were reported in the literature. In the present study the authors discussed the clinical value of the sequential study of the CSF uric acid content in patients with brain tumors. CSF was investigated for uric acid in 23 controls and 30 cases of brain tumor. The results were as follows: The mean value and standard deviation of the uric acid in CSF in controls was 0.23 +/- 0.13 mg/dl. The uric acid in CSF increased in patients with malignant brain tumor (0.49 +/- 0.22 mg/dl, p less than 0.005), but was in normal range in patients with benign brain tumor (0.32 +/- 0.13 mg/dl, 0.10 less than p less than 0.25). There was no significant correlation between CSF uric acid and CSF protein contents. Uric acid in the lumbar CSF was approximately 4 times higher than in the ventricular CSF in patients with brain tumor. The CSF uric acid had progressively increased during irradiation to the whole brain. The factors contributing to increase of the uric acid in CSF were thought to be increased permeability of blood-CSF barrier, global damage of brain tissue, increased nucleic acid catabolism in the central nervous system (CNS) for example in tumor, inflammation or immunoreaction, increased of xanthine, hypoxanthine or xanthine oxidase activity in the CNS, directly increased of plasma components into the CSF due to such as subarachnoid hemorrhage, intraventricular hemorrhage, bleeding in the tumor or surgical operation, dysfunction of the CSF dynamics.(ABSTRACT TRUNCATED AT 250 WORDS)

  6. Impact on Life Expectancy of Withdrawing Thiopurines in Patients with Crohn’s Disease in Sustained Clinical Remission: A Lifetime Risk-Benefit Analysis

    PubMed Central

    Kirchgesner, Julien; Beaugerie, Laurent; Carrat, Fabrice; Sokol, Harry; Cosnes, Jacques; Schwarzinger, Michaël

    2016-01-01

    Objective Long-term treatment with thiopurines is associated with a decreased risk of Crohn’s disease (CD) flare but an increased risk of various cancers depending on gender, age, and presence of extensive colitis. We evaluated risks and benefits of withdrawing thiopurines in patients with CD in prolonged remission. Methods We developed a Markov model assessing risks and benefits of withdrawing thiopurines compared to continuing thiopurines in a lifetime horizon. The model was stratified by age (35 and 65 years old at thiopurine withdrawal), gender and presence of extensive colitis. Parameter estimates were taken from French cohorts and hospital databases, cancer and death national registries and published literature. Life expectancy, rates of relapse, serious adverse events, and causes-of-death were evaluated. Results In patients without extensive colitis, continuing thiopurines increased life expectancy up to 0.03 years for 35 year-old men and women but decreased life expectancy down to 0.07 years for 65 year-old men and women. Withdrawal strategy became the preferred strategy at 40.6 years for men, and 45.7 years for women without extensive colitis. In patients with extensive colitis, continuation strategy was the preferred strategy regardless of age. Risk-benefit analysis was not modified by duration of CD activity. Conclusions Factors determining life expectancy associated with withdrawal or continuation of thiopurines in patients with CD and in sustained clinical remission vary substantially according to gender, age and presence of extensive colitis. Individual decisions to continue or withdraw thiopurines in patients with CD in sustained remission should take into account these parameters. PMID:27271176

  7. Treatment with oral paricalcitol in daily clinical practice for patients with chronic kidney disease stage 3–4: a preliminary study

    PubMed Central

    Hadjiyannakos, Dimitrios; Filiopoulos, Vassilis; Trompouki, Sofia; Sonikian, Makroui; Karatzas, Ioannis; Panagiotopoulos, Konstantinos; Vlassopoulos, Dimosthenis

    2013-01-01

    Background Active vitamin D is an effective treatment for secondary hyperparathyroidism (SHPT) in chronic kidney disease (CKD) patients often complicated by hypercalcaemia and hyperphosphataemia. Treatment with paricalcitol, a selective vitamin D receptor activator, has shown benefits by adequately reducing parathyroid hormone (PTH) levels with minimal changes in serum calcium (Ca) and phosphorus (P). The purpose of this study is to present data on the use of oral paricalcitol in real-life clinical practice in patients with CKD stage 3–4 and SHPT. Methods We studied 43 patients, M/F: 25/18, median age: 74 years (47–87), CKD stage 3/4: 16/27, with SHPT, who were prescribed oral paricalcitol at recommended doses for 6 months. Monthly measurements of serum intact PTH (iPTH), Ca, P, alkaline phosphatase (ALP), haemoglobin, albumin (ALB), lipid profile, proteinuria and 24-h urine creatinine clearance were performed 3 months before and 6 months after treatment initiation. Results Paricalcitol induced a significant, early and sustained, through the end of follow-up period, decrease in iPTH and ALP levels and an increase in serum ALB. No significant increase in Ca and P levels as well as in Ca × P product was observed during the study period. No significant changes were found in protein excretion, kidney function and the other measured parameters between baseline and last evaluation. Paricalcitol final median dose was 5 μg/week ranging between 3 and 7 μg/week. Conclusions In the context of real-life clinical practice, oral paricalcitol for 6 months is an effective, well-tolerated treatment of SHPT in CKD stage 3–4 with minimal effects on calcium and phosphorus metabolism. PMID:26019845

  8. Clinical characteristics of elderly patients with proton pump inhibitor-refractory non-erosive reflux disease from the G-PRIDE study who responded to rikkunshito

    PubMed Central

    2014-01-01

    Background The incidence and severity of gastroesophageal reflux disease (GERD) in Japan tends to increase in elderly women. Rikkunshito (RKT), a traditional Japanese medicine, acts as a prokinetic agent and improves gastric emptying and gastric accommodation. Our previous prospective randomized placebo-controlled study showed that RKT combined with a standard-dose of rabeprazole (RPZ) significantly improved the acid-related dysmotility symptoms (ARD) in elderly patients with proton pump inhibitor (PPI)-refractory non-erosive reflux disease (NERD). This study aimed to evaluate clinical characteristics of elderly PPI-refractory NERD patients with ARD symptoms who responded to RKT. Methods Two hundred forty-two patients with PPI-refractory NERD were randomly assigned to 8 weeks of either RPZ (10 mg/q.d.) + RKT (7.5 g/t.i.d.) (RKT group) or RPZ + placebo (PL group). Among them, 95 were elderly (≥65 years) with ARD (RKT group: n = 52; PL group: n = 43). We analyzed the changes using the 12 subscale score of frequency scale for the symptoms of GERD (FSSG) and 15 items of the Gastrointestinal Symptom Rating Scale at 4 and 8 weeks and compared the therapeutic efficacy between the 2 groups. Results There were no marked differences in baseline demographic or clinical characteristics in the 2 groups except for rate of current smoking. The FSSG score (mean ± SD at 0, 4, and 8 weeks) in both the RKT (16.0 ± 7.0; 9.9 ± 8.4; 7.0 ± 6.4) and PL (15.1 ± 6.4; 10.9 ± 6.7, 11.1 ± 8.5) groups significantly decreased after treatment. However, the degree of improvement of total and ARD scores of FSSG after the 8-week treatment was significantly greater in the RKT group than in the PL group. Combination therapy with RKT for 8 weeks showed significant improvement in 3 subscale scores (abdominal bloating, heavy feeling in stomach and sick feeling after meals) of the ARD domain and 1 subscale score (heartburn after meals) of the reflux symptom domain

  9. Interdisciplinary care clinics in chronic kidney disease.

    PubMed

    Johns, Tanya S; Yee, Jerry; Smith-Jules, Terrian; Campbell, Ruth C; Bauer, Carolyn

    2015-01-01

    The burden of chronic kidney disease (CKD) is substantial, and is associated with high hospitalization rates, premature deaths, and considerable health care costs. These factors provide strong rationale for quality improvement initiatives in CKD care. The interdisciplinary care clinic (IDC) has emerged as one solution to improving CKD care. The IDC team may include other physicians, advanced practice providers, nurses, dietitians, pharmacists, and social workers--all working together to provide effective care to patients with chronic kidney disease. Studies suggest that IDCs may improve patient education and preparedness prior to kidney failure, both of which have been associated with improved health outcomes. Interdisciplinary care may also delay the progression to end-stage renal disease and reduce mortality. While most studies suggest that IDC services are likely cost-effective, financing IDCs is challenging and many insurance providers do not pay for all of the services. There are also no robust long-term studies demonstrating the cost-effectiveness of IDCs. This review discusses IDC models and its potential impact on CKD care as well as some of the challenges that may be associated with implementing these clinics. PMID:26458811

  10. Using Electromagnetic Microwave Field Combined With Laserotherapy in Postoperative Period of Patients With Purulent-Inflammatory Diseases of the Hand in Outpatient Clinic

    NASA Astrophysics Data System (ADS)

    Rabenok, L.; Grimalsky, V.; Juárez-R., D.

    2008-08-01

    The results of a treatment of 51 patients with purulent-inflammatory diseases of the hand in outpatient clinic are analyzed; a new method of treatment using electromagnetic (EM) microwave field combined with laserotherapy was applied. A portable apparatus was used that operates in the millimeter (mm) wave range in 4 regimes of an intensity 2-10 mW / cm2 and a red laser of 0.65 μm wavelength of a low intensity 10-15 mW / cm2 . A peculiarity of the method was an absence of any antibacterial medicine. An exposure of an influence was 10 min. The total course included 5-7 applications. An influence of low intensity EM radiation was started 15-20 min before the operation. The action was to the center of purulent inflammation and to the biological active points of acupuncture (G14, E36). Starting from the first day since the operation, the low intensity EM radiation was applied to the biological active points combined with laserotherapy to the wound in the sedative regime (the repetition rate 9-10 Hz) during 10 min. Clinical symptomatology, radiographic findings, the results of microbiologic, morphologic studies were analyzed as well and were satisfactory (without any complications).

  11. Using Electromagnetic Microwave Field Combined With Laserotherapy in Postoperative Period of Patients With Purulent-Inflammatory Diseases of the Hand in Outpatient Clinic

    SciTech Connect

    Rabenok, L.; Grimalsky, V.; Juarez R, D.

    2008-08-11

    The results of a treatment of 51 patients with purulent-inflammatory diseases of the hand in outpatient clinic are analyzed; a new method of treatment using electromagnetic (EM) microwave field combined with laserotherapy was applied. A portable apparatus was used that operates in the millimeter (mm) wave range in 4 regimes of an intensity 2-10 mW/cm{sup 2} and a red laser of 0.65 {mu}m wavelength of a low intensity 10-15 mW/cm{sup 2}. A peculiarity of the method was an absence of any antibacterial medicine. An exposure of an influence was 10 min. The total course included 5-7 applications. An influence of low intensity EM radiation was started 15-20 min before the operation. The action was to the center of purulent inflammation and to the biological active points of acupuncture (G14, E36). Starting from the first day since the operation, the low intensity EM radiation was applied to the biological active points combined with laserotherapy to the wound in the sedative regime (the repetition rate 9-10 Hz) during 10 min. Clinical symptomatology, radiographic findings, the results of microbiologic, morphologic studies were analyzed as well and were satisfactory (without any complications)

  12. Genomic and Clinical Effects Associated with a Relaxation Response Mind-Body Intervention in Patients with Irritable Bowel Syndrome and Inflammatory Bowel Disease

    PubMed Central

    Jacquart, Jolene; Scult, Matthew A.; Slipp, Lauren; Riklin, Eric Isaac Kagan; Lepoutre, Veronique; Comosa, Nicole; Norton, Beth-Ann; Dassatti, Allison; Rosenblum, Jessica; Thurler, Andrea H.; Surjanhata, Brian C.; Hasheminejad, Nicole N.; Kagan, Leslee; Slawsby, Ellen; Rao, Sowmya R.; Macklin, Eric A.; Fricchione, Gregory L.; Benson, Herbert; Libermann, Towia A.; Korzenik, Joshua; Denninger, John W.

    2015-01-01

    Introduction Irritable Bowel Syndrome (IBS) and Inflammatory Bowel Disease (IBD) can profoundly affect quality of life and are influenced by stress and resiliency. The impact of mind-body interventions (MBIs) on IBS and IBD patients has not previously been examined. Methods Nineteen IBS and 29 IBD patients were enrolled in a 9-week relaxation response based mind-body group intervention (RR-MBI), focusing on elicitation of the RR and cognitive skill building. Symptom questionnaires and inflammatory markers were assessed pre- and post-intervention, and at short-term follow-up. Peripheral blood transcriptome analysis was performed to identify genomic correlates of the RR-MBI. Results Pain Catastrophizing Scale scores improved significantly post-intervention for IBD and at short-term follow-up for IBS and IBD. Trait Anxiety scores, IBS Quality of Life, IBS Symptom Severity Index, and IBD Questionnaire scores improved significantly post-intervention and at short-term follow-up for IBS and IBD, respectively. RR-MBI altered expression of more genes in IBD (1059 genes) than in IBS (119 genes). In IBD, reduced expression of RR-MBI response genes was most significantly linked to inflammatory response, cell growth, proliferation, and oxidative stress-related pathways. In IBS, cell cycle regulation and DNA damage related gene sets were significantly upregulated after RR-MBI. Interactive network analysis of RR-affected pathways identified TNF, AKT and NF-κB as top focus molecules in IBS, while in IBD kinases (e.g. MAPK, P38 MAPK), inflammation (e.g. VEGF-C, NF-κB) and cell cycle and proliferation (e.g. UBC, APP) related genes emerged as top focus molecules. Conclusions In this uncontrolled pilot study, participation in an RR-MBI was associated with improvements in disease-specific measures, trait anxiety, and pain catastrophizing in IBS and IBD patients. Moreover, observed gene expression changes suggest that NF-κB is a target focus molecule in both IBS and IBD—and that

  13. CDKD: a clinical database of kidney diseases

    PubMed Central

    2012-01-01

    Background The main function of the kidneys is to remove waste products and excess water from the blood. Loss of kidney function leads to various health issues, such as anemia, high blood pressure, bone disease, disorders of cholesterol. The main objective of this database system is to store the personal and laboratory investigatory details of patients with kidney disease. The emphasis is on experimental results relevant to quantitative renal physiology, with a particular focus on data relevant for evaluation of parameters in statistical models of renal function. Description Clinical database of kidney diseases (CDKD) has been developed with patient confidentiality and data security as a top priority. It can make comparative analysis of one or more parameters of patient’s record and includes the information of about whole range of data including demographics, medical history, laboratory test results, vital signs, personal statistics like age and weight. Conclusions The goal of this database is to make kidney-related physiological data easily available to the scientific community and to maintain & retain patient’s record. As a Web based application it permits physician to see, edit and annotate a patient record from anywhere and anytime while maintaining the confidentiality of the personal record. It also allows statistical analysis of all data. PMID:22540288

  14. Clinical manifestations and management of Gaucher disease

    PubMed Central

    Linari, Silvia; Castaman, Giancarlo

    2015-01-01

    Summary Gaucher disease is a rare multi-systemic metabolic disorder caused by the inherited deficiency of the lysosomal enzyme β-glucocerebrosidase, which leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages with damage to haematological, visceral and bone systems. Anaemia, thrombocytopenia, enlargement of liver and/or spleen, skeletal abnormalities (osteopenia, lytic lesions, pathological fractures, chronic bone pain, bone crisis, bone infarcts, osteonecrosis and skeletal deformities) are typical manifestations of the most prevalent form of the disease, the so-called non-neuronopathic type 1. However, severity and coexistence of different symptoms are highly variable. The determination of deficient β-glucocerebrosidase activity in leukocytes or fibroblasts by enzymatic assay is the gold standard for the diagnosis of Gaucher disease. Comprehensive and reproducible evaluation and monitoring of all clinically relevant aspects are fundamental for the effective management of Gaucher disease patients. Enzyme replacement therapy has been shown to be effective in reducing glucocerebroside storage burden and diminishing the deleterious effects caused by its accumulation. Tailored treatment plan for each patient should be directed to symptom relief, general improvement of quality of life, and prevention of irreversible damage. PMID:26604942

  15. A Pilot Clinical Trial to Objectively Assess the Efficacy of Electroacupuncture on Gait in Patients with Parkinson's Disease Using Body Worn Sensors

    PubMed Central

    Lei, Hong

    2016-01-01

    Background Gait disorder, a key contributor to fall and poor quality of life, represents a major therapeutic challenge in Parkinson’s disease (PD). The efficacy of acupuncture for PD remains unclear, largely due to methodological flaws and lack of studies using objective outcome measures. Objective To objectively assess the efficacy of electroacupuncture (EA) for gait disorders using body-worn sensor technology in patients with PD. Methods In this randomized pilot study, both the patients and assessors were masked. Fifteen PD patients were randomly assigned to an experimental group (n = 10) or to a control group (n = 5). Outcomes were assessed at baseline and after completion of three weekly EA treatments. Measurements included gait analysis during single-task habitual walking (STHW), dual-task habitual walking (DTHW), single-task fast walking (STFW), dual-task fast walking (DTFW). In addition, Unified Parkinson's Disease Rating Scale (UPDRS), SF-12 health survey, short Falls Efficacy Scale-International (FES-I), and visual analog scale (VAS) for pain were utilized. Results All gait parameters were improved in the experimental group in response to EA treatment. After adjustment by age and BMI, the improvement achieved statistical significant level for gait speed under STHW, STFW, and DTFW (9%-19%, p<0.05) as well as stride length during DTFW (9%, p = 0.037) and midswing speed during STFW (6%, p = 0.033). No significant changes were observed in the control group (p>0.110). The highest correlation between gait parameters and UPRDS scores at baseline was observed between gait speed during STFW and UPDRS II (r = -0.888, p = 0.004). The change in this gait parameter in response to active intervention was positively correlated with baseline UPDRS (r = 0.595, p = 0.057). Finally, comparison of responses to treatment between groups showed significant improvement, prominently in gait speed (effect size 0.32–1.16, p = 0.001). Conclusions This study provides the objective

  16. Alloimmunization in multitransfused liver disease patients: Impact of underlying disease

    PubMed Central

    Bajpai, Meenu; Gupta, Shruti; Jain, Priyanka

    2016-01-01

    Introduction: Transfusion support is vital to the management of patients with liver diseases. Repeated transfusions are associated with many risks such as transfusion-transmitted infection, transfusion immunomodulation, and alloimmunization. Materials and Methods: A retrospective data analysis of antibody screening and identification was done from February 2012 to February 2014 to determine the frequency and specificity of irregular red-cell antibodies in multitransfused liver disease patients. The clinical and transfusion records were reviewed. The data was compiled, statistically analyzed, and reviewed. Results: A total of 842 patients were included in our study. Alloantibodies were detected in 5.22% of the patients. Higher rates of alloimmunization were seen in patients with autoimmune hepatitis, cryptogenic liver disease, liver damage due to drugs/toxins, and liver cancer patients. Patients with alcoholic liver disease had a lower rate of alloimmunization. The alloimmunization was 12.7% (23/181) in females and 3.17% (21/661) in males. Antibodies against the Rh system were the most frequent with 27 of 44 alloantibodies (61.36%). The most common alloantibody identified was anti-E (11/44 cases, 25%), followed by anti-C (6/44 cases, 13.63%). Conclusion: Our findings suggest that alloimmunization rate is affected by underlying disease. Provision of Rh and Kell phenotype-matched blood can significantly reduce alloimmunization. PMID:27605851

  17. Alloimmunization in multitransfused liver disease patients: Impact of underlying disease

    PubMed Central

    Bajpai, Meenu; Gupta, Shruti; Jain, Priyanka

    2016-01-01

    Introduction: Transfusion support is vital to the management of patients with liver diseases. Repeated transfusions are associated with many risks such as transfusion-transmitted infection, transfusion immunomodulation, and alloimmunization. Materials and Methods: A retrospective data analysis of antibody screening and identification was done from February 2012 to February 2014 to determine the frequency and specificity of irregular red-cell antibodies in multitransfused liver disease patients. The clinical and transfusion records were reviewed. The data was compiled, statistically analyzed, and reviewed. Results: A total of 842 patients were included in our study. Alloantibodies were detected in 5.22% of the patients. Higher rates of alloimmunization were seen in patients with autoimmune hepatitis, cryptogenic liver disease, liver damage due to drugs/toxins, and liver cancer patients. Patients with alcoholic liver disease had a lower rate of alloimmunization. The alloimmunization was 12.7% (23/181) in females and 3.17% (21/661) in males. Antibodies against the Rh system were the most frequent with 27 of 44 alloantibodies (61.36%). The most common alloantibody identified was anti-E (11/44 cases, 25%), followed by anti-C (6/44 cases, 13.63%). Conclusion: Our findings suggest that alloimmunization rate is affected by underlying disease. Provision of Rh and Kell phenotype-matched blood can significantly reduce alloimmunization.

  18. Gaucher disease: clinical profile and therapeutic developments

    PubMed Central

    Cox, Timothy M

    2010-01-01

    era before enzyme therapy, bone marrow transplantation was shown to correct systemic disease in Gaucher patients by supplying a source of competent donor macrophages. As a radical advance on cell- or protein-replacement techniques, contemporary methods for transferring genes to autologous hematopoietic stem cells, and to the brain, merit further exploration. At present, the inflated pharmaceutical niche of Gaucher disease appears to be resilient, but if the remaining unmet needs of patients are to be convincingly addressed and commercial development sustained, courageous scientific investment and clinical experimentation will be needed. PMID:21209725

  19. Chronic liver disease in Kuala Lumpur, Malaysia: a clinical study.

    PubMed

    Kudva, M V; Zawawi, M M

    1990-08-01

    This study was undertaken to analyse the clinical spectrum of chronic liver disease (cirrhosis, and others with portal hypertension) in Kuala Lumpur. Eighty patients were diagnosed over a 6-year period. Twenty-two had biopsy proven cirrhosis while 58 others had portal hypertension with clinical and biochemical evidence of chronic liver disease. The commonest aetiology was alcohol (36%), followed by the idiopathic variety and hepatitis B. The male to female ratio was 4.4:1. Indians had a high prevalence of alcohol-associated chronic liver disease. Overall, ascites was the commonest presentation. Eight patients presented with hepatocellular carcinoma. Spontaneous bacterial peritonitis was diagnosed in 13% of patients undergoing abdominal paracentesis. Gallstones were detected in 37% of patients who underwent ultrasonography. Diabetes mellitus and peptic ulcer disease were noted in 22% and 31% of patients respectively.

  20. The effect of positive thinking training on the level of spiritual well-being among the patients with coronary artery diseases referred to Imam Reza specialty and subspecialty clinic in Shiraz, Iran: A randomized controlled clinical trial

    PubMed Central

    Ghodsbin, Fariba; Safaei, Marzieh; Jahanbin, Iran; Ostovan, Mohammad Ali; Keshvarzi, Sareh

    2015-01-01

    BACKGROUND Positive thinking which is derived from an optimistic view toward the universe and plays an important role in the incidence of better and a more targeted behavior among human beings. It can improve spiritual health in the individuals through increased communication with God and thanksgiving and accelerate the healing process. Accordingly, we aimed to evaluate the effect of positive thinking on the level of spiritual health in the patients with coronary artery disease (CAD) referred to Imam Reza specialty and subspecialty clinic in Shiraz, Iran. METHODS In this study randomized controlled clinical trial, we enrolled 90 patients with confirmed CAD referred to Imam Reza clinic, Shiraz, during April to July 2013. A blocking randomization method was used to randomize the final 90 participants into intervention (n = 45) and control groups (n = 45). After obtaining written informed consent, the participants were asked to complete two questionnaires. Data were collected using Ellison and Paloutzian’s spiritual well-being scale (SWBS) and a demographic questionnaire. The patients in the intervention group participated in 7 training sessions on positive thinking in which several topics were discussed. The SWBS questionnaire was completed two more times by the participants; once immediately after, and once 1 month after the intervention. 16 patients were excluded from the study due to different reasons, and finally the analysis was performed on 74 patients. RESULTS The mean ± standard deviation (SD) of spiritual well-being (SWB) increased from 88.71 ± 12.5 to 96.63 ± 12.58 in the intervention group; while, it decreased from 93.19 ± 17.55 to 94.45 ± 16.01 in the control group in the interval of before and 1 month after the intervention. We observed a statistically significant difference between the two groups regarding both variables of time and group (P < 0.001). CONCLUSION SWB is an important factor which should be considered in the treatment process, and

  1. The emergence of Parkinson disease among patients with Gaucher disease.

    PubMed

    Elstein, Deborah; Alcalay, Roy; Zimran, Ari

    2015-03-01

    In the last decade, several lines of evidence have been presented that document the clinical manifestations, genetic associations, and sub-cellular mechanisms of the inter-relatedness of β-glucocerebrosidase mutations and the emergence of Parkinson disease among carriers and patients with Gaucher disease. This review is an attempt to apprise the reader of the recent literature with the caveat that this is an area of intensive exploration that is constantly being updated because of the immediate clinical ramifications but also because of the impact on our understanding of Parkinson disease, and finally because of the unexpected inter-reactions between these entities on the molecular level. It has been an unexpected happenstance that it has been discovered that a rare monogenetic disease has an interface at many points with a neurological disorder of the elderly that has both familial and sporadic forms: to date there is no cure for either of these disorders.

  2. Patients with chronic pulmonary disease.

    PubMed

    Hong, Caron M; Galvagno, Samuel M

    2013-11-01

    Chronic pulmonary disease is common among the surgical population and the importance of a thorough and detailed preoperative assessment is monumental for minimizing morbidity and mortality and reducing the risk of perioperative pulmonary complications. These comorbidities contribute to pulmonary postoperative complications, including atelectasis, pneumonia, and respiratory failure, and can predict long-term mortality. The important aspects of the preoperative assessment for patients with chronic pulmonary disease, and the value of preoperative testing and smoking cessation, are discussed. Specifically discussed are preoperative pulmonary assessment and management of patients with chronic obstructive pulmonary disease, asthma, restrictive lung disease, obstructive sleep apnea, and obesity. PMID:24182721

  3. Molecular sources of residual cardiovascular risk, clinical signals, and innovative solutions: relationship with subclinical disease, undertreatment, and poor adherence: implications of new evidence upon optimizing cardiovascular patient outcomes

    PubMed Central

    Kones, Richard

    2013-01-01

    cholesterol levels are still inversely related to MCVE. The efflux capacity, or ability to relocate cholesterol out of macrophages, is believed to be a major antiatherogenic mechanism responsible for reduction in MCVE mediated in part by healthy HDL. HDL cholesterol is a complex molecule with antioxidative, anti-inflammatory, anti-thrombotic, antiplatelet, and vasodilatory properties, among which is protection of LDL from oxidation. HDL-associated paraoxonase-1 has a major effect on endothelial function. Further, HDL promotes endothelial repair and progenitor cell health, and supports production of nitric oxide. HDL from patients with cardiovascular disease, diabetes, and autoimmune disease may fail to protect or even become proinflammatory or pro-oxidant. Mendelian randomization and other clinical studies in which raising HDL cholesterol has not been beneficial suggest that high plasma levels do not necessarily reduce cardiovascular risk. These data, coupled with extensive preclinical information about the functional heterogeneity of HDL, challenge the “HDL hypothesis”, ie, raising HDL cholesterol per se will reduce MCVE. After the equivocal AIM-HIGH (Atherothrombosis Intervention in Metabolic Syndrome With Low HDL/High Triglycerides: Impact on Global Health Outcomes) study and withdrawal of two major cholesteryl ester transfer protein compounds, one for off-target adverse effects and the other for lack of efficacy, development continues for two other agents, ie, anacetrapib and evacetrapib, both of which lower LDL cholesterol substantially. The negative but controversial HPS2-THRIVE (the Heart Protection Study 2-Treatment of HDL to Reduce the Incidence of Vascular Events) trial casts further doubt on the HDL cholesterol hypothesis. The growing impression that HDL functionality, rather than abundance, is clinically important is supported by experimental evidence highlighting the conditional pleiotropic actions of HDL. Non-HDL cholesterol reflects the cholesterol in all

  4. Conjugated linoleic acid improves glycemic response, lipid profile, and oxidative stress in obese patients with non-alcoholic fatty liver disease: a randomized controlled clinical trial

    PubMed Central

    Ebrahimi-Mameghani, Mehrangiz; Jamali, Haleh; Mahdavi, Reza; Kakaei, Farzad; Abedi, Rana; Kabir-Mamdooh, Bita

    2016-01-01

    Aim To investigate if conjugated linoleic acid supplementation (CLA) affects metabolic factors and oxidative stress in non-alcoholic fatty liver disease (NAFLD). Methods The study was a randomized, controlled clinical trial conducted in specialized and subspecialized clinics of Tabriz University of Medical Sciences from January 2014 to March 2015. 38 obese NAFLD patients were randomly allocated into either the intervention group, receiving three 1000 mg softgel of CLA with a weight loss diet and 400 IU vitamin E, or into the control group, receiving only weight loss diet and 400 IU vitamin E for eight weeks. Dietary data and physical activity, as well as anthropometric, body composition, metabolic factors, and oxidative stress were assessed at baseline and at the end of the study. Results Weight, body composition, and serum oxidative stress, insulin, and lipid profile significantly improved in both groups, while hemoglobin A1c (HbA1c) levels (P = 0.004), total cholesterol to high density lipoprotein ratio (P = 0.008), low density lipoprotein to high density lipoprotein ratio (LDL/HDL) (P = 0.002), and alanine aminotransferase to aspartate aminotransferase (ALT/AST) ratio (P = 0.025) significantly decreased in the intervention group. At the end of the study, fat mass (P = 0.001), muscle mass (P = 0.023), total body water (P = 0.004), HbA1c (P < 0.001), triglycerides (P = 0.006), LDL/HDL ratio (P = 0.027), and ALT/AST ratio (P = 0.046) were significantly better in the CLA group than in the control group. Conclusion CLA improved insulin resistance, lipid disturbances, oxidative stress, and liver function in NAFLD. Therefore, it could be considered as an effective complementary treatment in NAFLD. Registration number: IRCT2014020516491N1. PMID:27586548

  5. THE PRINTO CRITERIA FOR CLINICALLY INACTIVE DISEASE IN JUVENILE DERMATOMYOSITIS

    PubMed Central

    Lazarevic, Dragana; Pistorio, Angela; Palmisani, Elena; Miettunen, Paivi; Ravelli, Angelo; Pilkington, Clarissa; Wulffraat, Nico; Malattia, Clara; Garay, Stella; Hofer, Michael; Quartier, Pierre; Dolezalova, Pavla; Calvo, Inmaculada; Ferriani, Virginia; Ganser, Gerd; Kasapcopur, Ozgur; Melo-Gomes, Jose Antonio; Reed, Ann M.; Wierzbowska, Malgorzata; Rider, Lisa G.; Martini, Alberto; Ruperto, Nicolino

    2016-01-01

    Objectives To develop data-driven criteria for clinically inactive disease on and off therapy for juvenile dermatomyositis (JDM). Methods The PRINTO database contains 275 patients with active JDM evaluated prospectively up to 24 months. Thirty-eight patients off therapy at 24 months were defined as clinically inactive and included in the reference group. These were compared with a random sample of 76 patients who had active disease at study baseline. Individual measures of muscle strength/endurance, muscle enzymes, physician's and parent's global disease activity/damage evaluations, inactive disease criteria derived from literature, and other ad hoc criteria, were evaluated for sensitivity, specificity, and Cohen's kappa agreement. Results The individual measures that best characterised inactive disease (sensitivity and specificity >0.8 and Cohen's K>0.8) were manual muscle testing (MMT)≥78, physician global assessment of muscle activity=0, physician global assessment of overall disease activity (PhyGloVAS) ≤0.2, the Childhood Myositis Assessment Scale (CMAS) ≥48, the Disease Activity Score (DAS) ≤3 and the Myositis Disease Activity Assessment Visual Analogue Scale (MYOACT) ≤0.2. The best combination of variables to classify a patient as being in a status of inactive disease on or off therapy is at least 3 out of 4 of the following criteria: creatine kinase ≤150, CMAS≥48, MMT≥78, and PhyGloVAS≤0.2. After 24 months, 30/31 (96.8%) patients were inactive off therapy and 69/145 (47.6%) were inactive on-therapy. Conclusion PRINTO established data-driven criteria, with clearly evidence-based cut off values, to identify JDM patients with clinically inactive disease. These criteria can be used in clinical trials, in research and in clinical practice. PMID:22736096

  6. Information Needs and Concerns of Patients with Inflammatory Bowel Disease: What Can We Learn from Participants in a Bilingual Clinical Cohort?

    PubMed Central

    Pittet, Valérie; Vaucher, Carla; Maillard, Michel H.; Girardin, Marc; de Saussure, Philippe; Burnand, Bernard; Rogler, Gerhard; Michetti, Pierre

    2016-01-01

    Background Inflammatory Bowel Disease (IBD) patients are confronted with needs and concerns related to their disease. Aim To explore information expectations of patients included in a national bilingual IBD cohort in Switzerland (SIBDC). Methods This is a mixed-methods study, comprising 1) a semi-narrative survey sent to 1506 patients from the SIBDC and 2) two focus groups conducted with 14 patients to explore and assess the relevance of the survey’s findings. Data collected within the framework of the SIBDC was used to characterize survey’s responders. Results 728 patients (48%) replied to the survey: 52.5% females, 56% Crohn’s disease (CD), 87% secondary/tertiary level educated, 70% full/part-time employed. On average, 47% of patients sought for information, regardless of the disease stage; 27% of them were dissatisfied with information received at the time of first symptoms. During flares, 43% were concerned about drugs and therapies; in remission, 57% had concerns on research and developments; 27% searched for information linked to daily disease management. Information-seeking increased when active disease, for CD with high levels of perceived stress (OR = 2.47; p = 0.003), and for all with higher posttraumatic stress symptoms. The focus groups confirmed a perceived lack of information about general functioning, disease course, treatments and their risks, extra-intestinal symptoms and manifestations. Conclusions Information remains insufficient for IBD patients. Lack of information in specific domains can potentially cause stress and hinder detection of symptoms. Better information should be considered as a potentially important component in improving patients’ outcomes in IBD. PMID:26939069

  7. Nitric oxide synthase (NOS3) variants and disease-free survival among treated and untreated breast cancer patients in a SWOG Clinical Trial (S8897)

    PubMed Central

    Choi, Ji-Yeob; Barlow, William E.; Albain, Kathy S.; Hong, Chi-Chen; Blanco, Javier G.; Livingston, Robert B.; Davis, Warren; Rae, James M.; Yeh, I-Tien; Hutchins, Laura F.; Ravdin, Peter M.; Martino, Silvana; Lyss, Alan P.; Osborne, C. Kent; Abeloff, Martin D.; Hayes, Daniel F.; Ambrosone, Christine B.

    2009-01-01

    Purpose Numerous chemotherapeutic agents are cytotoxic through generation of reactive species, and variability in genes related to oxidative stress may influence disease free survival (DFS). We examined relationships between DFS and variants in NOS3, as well as NQO1, NQO2 and CBR3 among treated and untreated breast cancer patients in a Southwest Oncology Group clinical trial (S8897). Experimental Design In the parent trial, women were assigned according to prognostic features; the high risk group was randomized to CMF or CAF ± tamoxifen, and the low risk group did not receive adjuvant therapy. We extracted DNA from normal lymph node tissue and examined functional polymorphisms in NOS3, NQO1, NQO2 and CBR3, in relation to DFS, using Cox proportional hazard model. Results There were significant interactions between DFS, adjuvant therapy, and NOS3 Glu298Asp and -786 polymorphisms, alone and in combination (p for interaction=0.008). When NOS3 genotypes were combined, women with genotypes encoding for lower NO who received chemotherapy had more than a two fold increase in hazard of progression (HR=2.32, 95% CI=1.26-4.25), while there was reduced risk for those who did not receive adjuvant therapy (HR=0.42, 95% CI=0.19-0.95). There were no associations between the other genotypes and DFS in either group. Conclusion Variants encoding lower activity of NOS3 may affect outcomes in breast cancer patients, with the direction of risk differing depending upon chemotherapy status. These results may mirror the known dual functions of NO and NOS, depending upon oxidative environment. PMID:19671875

  8. [Diverticular disease - clinical patterns and treatment].

    PubMed

    Lembcke, Bernhard; Kruis, Wolfgang

    2015-09-01

    Diverticulosis, diverticular disease and diverticulitis have come into focus again because new aspects concerning diagnosis, risk factors and treatment arose only recently which prompted a new Guideline released by the DGVS and DGAV summarising the current evidence. Along with the guideline's essentials for medical practice a diagnosis of diverticulitis is considered unsatisfactory unless a cross-sectional imaging method (either ultrasonography [US] or computed tomography [CT] ) has proven that the clinical findings and inflammation (CRP considered superior to WBC and temperature) are due to diverticular inflammation. For reasons of practicability and considering relevant legislation for radiation exposure protection, US is the primary - and usually effectual - diagnostic method of choice as it is equipotent to CT. While US offers better resolution and enables precise imaging exactly at the location of pain as well as reiterative application, the latter implies advantages in the case of a deep abscess or diverticulitis in difficult locations (e. g. the small pelvis). Clinical evidence and laboratory and imaging findings allow for distinguishing a large number of differential diagnoses and also form the basis of a new classification (classification of diverticular disease, CDD) which comprises all forms of diverticular disease, from diverticulosis to bleeding and to the different facettes of diverticulitis. This classification -which should be applied in any patient with the diagnosis of diverticular disease- is independent of specific diagnostic preferences and applicable both to conservative and operative treatment options. While the number of recurrent episodes is no longer a significant indicator for surgery in diverticulitis, severity and / or complications determine treatment options along with the patients preferences. According to first data, conservative treatment may waive antibiotics under certain circumstances, however they are indispensible in

  9. Evaluating the Efficacy of a Web-Based Program (Diapason) for Informal Caregivers of Patients With Alzheimer’s Disease: Protocol for a Randomized Clinical Trial

    PubMed Central

    Kerhervé, Hélène; de Rotrou, Jocelyne; Rouquette, Alexandra; Legouverneur, Grégory; Rigaud, Anne-Sophie

    2013-01-01

    Background Informal caregivers (CGs) of patients with Alzheimer’s disease are at risk of suffering from psychological and physical weakening. Several psychoeducational interventions have been designed to prevent stress and burden of caregivers. In France, despite health authorities’ recommendations, to our knowledge there is no rigorously assessed Web-based psychoeducational program to date. Objective The objective of our study was to assess the efficacy of a French Web-based psychoeducational program (called Diapason) with an unblinded randomized clinical trial. Methods In this protocol, 80 informal caregivers of patients followed at Broca Hospital are recruited offline and randomized in the experimental condition (EC) or the control condition (CC). The volunteers in EC have to visit a closed online user group at least once a week and validate one new session of this fully automated Web program, during 12 weeks. Each week a new thematic is added to the website. The participants in the CC receive usual care, and have access to the Diapason program after their participation (6 months). Face-to-face evaluations for both groups are planned every 3 months (M0–M3 and M6). The main objective of this program is to provide CGs with information on the disease process, how to prevent psychological strain (using anticipation and relaxation techniques), and offering a virtual space (forum) to discuss with other caregivers. The primary outcome of this study is the self-perceived stress, while self-efficacy, burden, depression, and self-perceived health status are defined as secondary outcomes. Other variables that might have an impact on the program efficacy are collected. Results This protocol was accepted for funding. The enrollment began in October 2011, and participants currently recruited will finish their evaluations in January 2014. The results are expected for June 2014. Conclusions Findings might provide empirical evidence on: (1) the feasibility of an Internet

  10. Deep brain stimulation of pallidal versus subthalamic for patients with Parkinson’s disease: a meta-analysis of controlled clinical trials

    PubMed Central

    Xu, Fan; Ma, Wenbin; Huang, Yongmin; Qiu, Zhihai; Sun, Lei

    2016-01-01

    Background Parkinson’s disease (PD) is a common neurodegenerative disorder that affects many people every year. Deep brain stimulation (DBS) is an effective nonpharmacological method to treat PD motor symptoms. This meta-analysis was conducted to evaluate the efficacy of subthalamic nucleus (STN)-DBS versus globus pallidus internus (GPi)-DBS in treating advanced PD. Methods Controlled clinical trials that compared STN-DBS to GPi-DBS for short-term treatment of PD in adults were researched up to November 2015. The primary outcomes were the Unified Parkinson’s Disease Rating Scale Section (UPDRS) III score and the levodopa-equivalent dosage (LED) after DBS. The secondary outcomes were the UPDRS II score and the Beck Depression Inventory (BDI) score. Results Totally, 13 studies containing 1,148 PD patients were included in this meta-analysis to compare STN-DBS versus GPi-DBS. During the off-medication state, the pooled weighted mean difference (WMD) of UPDRS III and II scores were −2.18 (95% CI =−5.11 to 0.74) and −1.96 (95% CI =−3.84 to −0.08), respectively. During the on-medication state, the pooled WMD of UPDRS III and II scores were 0.15 (95% CI =−1.14 to 1.44) and 1.01 (95% CI =0.12 to 1.89), respectively. After DBS, the pooled WMD of LED and BDI were −254.48 (95% CI =−341.66) and 2.29 (95% CI =0.83 to 3.75), respectively. Conclusion These results indicate that during the off-medication state, the STN-DBS might be superior to GPi-DBS in improving the motor function and activities of daily living for PD patients; but during the on-medication state, the opposite result is observed. Meanwhile, the STN-DBS is superior at reducing the LED, whereas the GPi-DBS shows a significantly greater reduction in BDI score after DBS. PMID:27382286

  11. Clinical Factors Associated with High-risk Carotid Plaque Features as Assessed by Magnetic Resonance Imaging in Patients with Established Vascular Disease (From the AIM-HIGH Study)

    PubMed Central

    Zhao, Xue-Qiao; Hatsukami, Thomas S.; Hippe, Daniel S.; Sun, Jie; Balu, Niranjan; Isquith, Daniel A.; Crouse, John R.; Anderson, Todd; Huston, John; Polissar, Nayak; O’Brien, Kevin; Yuan, Chun

    2014-01-01

    Association between clinical factors and high-risk plaque features such as thin or ruptured cap, intra-plaque hemorrhage (IPH), presence of Lipid Rich Necrotic Core (LRNC) and increased LRNC volume as assessed by Magnetic Resonance Imaging (MRI) was examined in patients with established vascular disease in AIM-HIGH. A total of 214 subjects underwent carotid MRI and had acceptable image quality for assessment of plaque burden, tissue contents and MRI-modified AHA lesion type by a Core Lab. We found that 77% of subjects had carotid plaques, 52% had lipid-containing plaques, and 11% had advanced, AHA type-VI lesions with possible surface defect, IPH or mural thrombus. Type-VI lesions were associated with older age (OR=2.6 per 5 years increase, p<0.001). After adjusting for age, these lesions were associated with history of cerebrovascular disease (OR=4.1, p=0.01), higher levels of Lipoprotein(a) (OR=2.0 per 1 SD increase, p=0.02) and larger %wall volume (%WV; OR=4.6 per 1 SD increase, p<0.001), but, were negatively associated with metabolic syndrome (OR=0.2, p=0.02). Presence of LRNC was associated with male gender (OR=3.2, p=0.02) and %WV (OR=3.8 per 1 SD, p<0.001), but, was negatively associated with diabetes (OR=0.4, p=0.02) and HDL-C levels (OR=0.7 per 1 SD, p=0.02). Increased %LRNC was associated with %WV (regression coefficient=0.36, p<0.001) and negatively associated with ApoA1 levels (regression coefficient=−0.20, p=0.03). In conclusions, older age, male gender, history of cerebrovascular disease, larger plaque burden, higher Lp(a), and lower HDL-C or ApoA1 have statistically significant associations with high-risk plaque features. Metabolic syndrome and diabetes showed negative associations in this population. PMID:25245415

  12. Reductions in disease activity in the AMPLE trial: clinical response by baseline disease duration

    PubMed Central

    Schiff, Michael; Weinblatt, Michael E; Valente, Robert; Citera, Gustavo; Maldonado, Michael; Massarotti, Elena; Yazici, Yusuf; Fleischmann, Roy

    2016-01-01

    Objectives To evaluate clinical response by baseline disease duration using 2-year data from the AMPLE trial. Methods Patients were randomised to subcutaneous abatacept 125 mg weekly or adalimumab 40 mg bi-weekly, with background methotrexate. As part of a post hoc analysis, the achievement of validated definitions of remission (Clinical Disease Activity Index (CDAI) ≤2.8, Simplified Disease Activity Index (SDAI) ≤3.3, Routine Assessment of Patient Index Data 3 (RAPID3) ≤3.0, Boolean score ≤1), low disease activity (CDAI <10, SDAI <11, RAPID3 ≤6.0), Health Assessment Questionnaire-Disability Index response and American College of Rheumatology responses were evaluated by baseline disease duration (≤6 vs >6 months). Disease Activity Score 28 (C-reactive protein) <2.6 or ≤3.2 and radiographic non-progression in patients achieving remission were also evaluated. Results A total of 646 patients were randomised and treated (abatacept, n=318; adalimumab, n=328). In both treatment groups, comparable responses were achieved in patients with early rheumatoid arthritis (≤6 months) and in those with later disease (>6 months) across multiple clinical measures. Conclusions Abatacept or adalimumab with background methotrexate were associated with similar onset and sustainability of response over 2 years. Patients treated early or later in the disease course achieved comparable clinical responses. Trial registration number NCT00929864, Post-results. PMID:27110385

  13. Disease-mongering through clinical trials.

    PubMed

    González-Moreno, María; Saborido, Cristian; Teira, David

    2015-06-01

    Our goal in this paper is to articulate a precise concept of at least a certain kind of disease-mongering, showing how pharmaceutical marketing can commercially exploit certain diseases when their best definition is given through the success of a treatment in a clinical trial. We distinguish two types of disease-mongering according to the way they exploit the definition of the trial population for marketing purposes. We argue that behind these two forms of disease-mongering there are two well-known problems in the statistical methodology of clinical trials (the reference class problem and the distinction between statistical and clinical significance). Overcoming them is far from simple.

  14. THERAPIES FOR CROHN'S DISEASE: a clinical update.

    PubMed

    Sobrado, Carlos Walter; Leal, Raquel Franco; Sobrado, Lucas Faraco

    2016-01-01

    The main objectives of clinical therapy in Crohn's disease are clinical and endoscopic remission without the use of corticosteroids for long periods of time, prevention of hospitalization and surgery, and improvement of quality of life. The main limitation of drug therapy is the loss of response over the long term, which makes incorporation of new drugs to the therapeutic arsenal necessary. This review analyses the main drugs currently used in clinical treatment of Crohn's disease. PMID:27438429

  15. Dynamics of neural networks: a proposed mechanism to account for changes in clinical symptomatology through time in patients with psychotic diseases.

    PubMed

    Dubrovsky, B

    2001-10-01

    The classical Kraepelinean dichotomy between manic depressive insanity and the schizophrenias has been recently challenged from clinical and neurobiological quarters. It is not so infrequent to see patients shift from a manic to a schizophrenic symptomatology and vice versa. This paper proposes neurobiological mechanisms as to how these changes may occur, based on recent data on the functioning of neural networks at different modes.

  16. Incidence of coronary artery disease in patients with valvular heart disease.

    PubMed Central

    Morrison, G W; Thomas, R D; Grimmer, S F; Silverton, P N; Smith, D R

    1980-01-01

    The case notes, cardiac catheterisation data, and coronary arteriograms of 239 patients investigated for valvular heart disease during a five year period were reviewed. Angina present in 13 of 95 patients with isolated mitral valve disease, 43 of 90 patients with isolated aortic valve disease, and 18 of 54 patients with combined mitral and aortic valve disease. Significant coronary artery disease was present in 85 per cent of patients with mitral valve disease and angina, but in only 33 per cent of patients with aortic valve disease and angina. Patients with no chest pain still had a high incidence of coronary artery disease, significant coronary obstruction being present in 22 per cent with mitral valve disease, 22 per cent with aortic valve disease, and 11 per cent with combine mitral and aortic valve disease. Several possible clinical markers of coronary artery disease were examined but none was found to be of practical help. There was, however, a significant inverse relation between severity of coronary artery disease and severity of valve disease in patients with aortic valve disease. Asymptomatic coronary artery disease is not uncommon in patients with valvular heart disease and if it is policy to perform coronary artery bypass grafting in such patients, routine coronary arteriography must be part of the preoperative investigation. PMID:7459146

  17. [The biological kinetics of biofilms of clinical strains of Staphylococcus aureus and Pseudomonas aeruginosa separated from patients with bronchopulmonary complications under traumatic disease of spinal cord].

    PubMed

    Ul'ianov, V Iu; Opredelentseva, S V; Shvidenko, I G; Norkin, I A; Korshunov, G V; Gladkova, E V

    2014-08-01

    The capacity and intensity of formation of microbial biofilms was analyzed in 24 strains of Staphylococcus aureus and Pseudomonas aeruginosa in static conditions of cultivation during 24, 48, 72 and 96 yours. The microorganisms were separated from patients with bronchopulmonary infectious complications in acute and early periods of traumatic disease of spinal cord. PMID:25552053

  18. [The biological kinetics of biofilms of clinical strains of Staphylococcus aureus and Pseudomonas aeruginosa separated from patients with bronchopulmonary complications under traumatic disease of spinal cord].

    PubMed

    Ul'ianov, V Iu; Opredelentseva, S V; Shvidenko, I G; Norkin, I A; Korshunov, G V; Gladkova, E V

    2014-08-01

    The capacity and intensity of formation of microbial biofilms was analyzed in 24 strains of Staphylococcus aureus and Pseudomonas aeruginosa in static conditions of cultivation during 24, 48, 72 and 96 yours. The microorganisms were separated from patients with bronchopulmonary infectious complications in acute and early periods of traumatic disease of spinal cord.

  19. [Piracetam and tocopherol acetate ability to potentiate clinical effect of antianginal drugs in presenile and senile patients with ischemic heart disease (unstable angina)].

    PubMed

    Pimenov, L T; Churshin, A D; Ezhov, A V

    1997-01-01

    Potentiating ability of piracetam and tocopherol acetate was studied outpatiently in aged patients with ischemic heart disease. It was found that the addition of piracetam and tocopherol acetate to conventional antianginal drug therapy brings higher response and exercise tolerance, contributes to more effective hemodynamic and energetic support of the exercise, to positive changes in the central and peripheral hemodynamics.

  20. Fatigue in liver disease: Pathophysiology and clinical management

    PubMed Central

    Swain, Mark G

    2006-01-01

    Fatigue is the most commonly encountered symptom in patients with liver disease, and it has a significant impact on their quality of life. However, although some progress has been made with regard to the understanding of the processes which may generate fatigue in general, the underlying cause(s) of liver disease-associated fatigue remain incompletely understood. The present review describes recent advances which have been made in our ability to measure fatigue in patients with liver disease in the clinical setting, as well as in our understanding of potential pathways which are likely important in the pathogenesis of fatigue associated with liver disease. Specifically, experimental findings suggest that fatigue associated with liver disease likely occurs as a result of changes in neurotransmission within the brain. In conclusion, a reasonable approach to help guide in the management of the fatigued patient with liver disease is presented. PMID:16550262

  1. Disease-Related Knowledge and Information Needs Among Inflammatory Bowel Disease Patients in Korea

    PubMed Central

    Yoo, Yang-Sook; Cha, Kyeong-Sook

    2015-01-01

    The aim of this study was to identify disease-related knowledge and information needs of patients with inflammatory bowel disease. The 313 patients (Crohn disease: n = 169, colitis: n = 144) presenting to an outpatient gastroenterology clinic of a tertiary care hospital in Seoul, Republic of Korea, were scored on their knowledge of Crohn disease and colitis and their information needs were assessed in the questionnaire. Patients with Crohn disease obtained a higher mean knowledge score than patients with colitis. The patients with Crohn disease had significantly higher scores about complications than patients with colitis. The patients with Crohn disease showed significantly higher mean scores relating to the patients' information needs than patients with colitis. The favorite topics of information needed were disease, medication, and diagnosis/operations. The patients with Crohn disease wanted more information than patients with colitis about medications used for treatment, daily life, and pregnancy. The effectiveness of the training and education given to patients can be maximized in this education system when the information about disease and medications for Crohn disease patients or information about disease and diet for colitis patients is primarily provided according to the degree of the patients' need for information. PMID:25159269

  2. Pharmacokinetics and bioavailability of zidovudine and its glucuronidated metabolite in patients with human immunodeficiency virus infection and hepatic disease (AIDS Clinical Trials Group protocol 062).

    PubMed Central

    Moore, K H; Raasch, R H; Brouwer, K L; Opheim, K; Cheeseman, S H; Eyster, E; Lemon, S M; van der Horst, C M

    1995-01-01

    The pharmacokinetics of zidovudine (ZDV) are established in patients with various stages of human immunodeficiency virus (HIV) disease. This study was conducted to determine the pharmacokinetic parameters of ZDV in patients with asymptomatic HIV infection and liver disease. HIV-infected volunteers with normal renal function were stratified according to the severity of liver disease (seven of eight were classified as mild). Each subject received a single intravenous dose of ZDV (120 mg) on the first day, followed by a single oral dose of ZDV (200 mg) on the second day. Blood samples were obtained over a 8-h collection interval, and concentrations of ZDV and its glucuronidated metabolite (GZDV) were determined by high-performance liquid chromatography. The following pharmacokinetic parameters were obtained after oral administration of ZDV to HIV-infected patients with mild hepatic disease; these values were compared with previously reported data in healthy volunteers. The area under the curve (AUC) (1,670 +/- 192 ng.h/ml), maximum concentration of drug in serum (1,751 +/- 180 ng/ml), and half-life (2.04 +/- 0.38 h) of ZDV were increased, while the apparent oral clearance (1.57 +/- 0.31 liter/h/kg of body weight) was decreased; AUC (7,685 +/- 1,222 ng.h/ml) and maximum concentration of drug in serum (5,220 +/- 1,350 ng/ml) of GZDV and the AUC ratio of GZDV to ZDV (2.79 +/- 0.43) after oral administration were decreased. ZDV absolute bioavailability was 0.75 +/- 0.15 in HIV-infected patients with hepatic disease. Although the ZDV apparent oral clearance was not impaired as significantly as in patients with biopsy-proven cirrhosis, our results suggest that ZDV, could accumulate in HIV-infected patients with mild hepatic disease because of impaired formation of GZDV. Patients with mild hepatic disease may require dosage adjustment to avoid accumulation of ZDV after extended therapy. PMID:8593010

  3. Clinical Trials in Rare Disease: Challenges and Opportunities

    PubMed Central

    Augustine, Erika F.; Adams, Heather R.; Mink, Jonathan W.

    2014-01-01

    The neuronal ceroid lipofuscinoses constitute one of many groups of rare childhood diseases for which disease-modifying treatments are non-existent. Disease-specific barriers to therapeutic success include incomplete understanding of disease pathophysiology and limitations of treatments that cannot adequately cross the blood-brain barrier to access the central nervous system. Therapeutic development in the neuronal ceroid lipofuscinoses shares many challenges with other rare diseases, such as incomplete understanding of natural history to inform trial design, need for alternatives to the randomized controlled clinical trial, requirement for more sensitive outcome measures to quantify disease, limited access to resources required to mount a clinical trial (including funding), and difficulties of recruiting a small sample to participation. Solutions to these barriers will require multicenter collaboration, partnership with patient organizations, training a new generation of researchers interested in rare diseases, and leveraging existing resources. PMID:24014509

  4. Molecular sources of residual cardiovascular risk, clinical signals, and innovative solutions: relationship with subclinical disease, undertreatment, and poor adherence: implications of new evidence upon optimizing cardiovascular patient outcomes

    PubMed Central

    Kones, Richard

    2013-01-01

    cholesterol levels are still inversely related to MCVE. The efflux capacity, or ability to relocate cholesterol out of macrophages, is believed to be a major antiatherogenic mechanism responsible for reduction in MCVE mediated in part by healthy HDL. HDL cholesterol is a complex molecule with antioxidative, anti-inflammatory, anti-thrombotic, antiplatelet, and vasodilatory properties, among which is protection of LDL from oxidation. HDL-associated paraoxonase-1 has a major effect on endothelial function. Further, HDL promotes endothelial repair and progenitor cell health, and supports production of nitric oxide. HDL from patients with cardiovascular disease, diabetes, and autoimmune disease may fail to protect or even become proinflammatory or pro-oxidant. Mendelian randomization and other clinical studies in which raising HDL cholesterol has not been beneficial suggest that high plasma levels do not necessarily reduce cardiovascular risk. These data, coupled with extensive preclinical information about the functional heterogeneity of HDL, challenge the “HDL hypothesis”, ie, raising HDL cholesterol per se will reduce MCVE. After the equivocal AIM-HIGH (Atherothrombosis Intervention in Metabolic Syndrome With Low HDL/High Triglycerides: Impact on Global Health Outcomes) study and withdrawal of two major cholesteryl ester transfer protein compounds, one for off-target adverse effects and the other for lack of efficacy, development continues for two other agents, ie, anacetrapib and evacetrapib, both of which lower LDL cholesterol substantially. The negative but controversial HPS2-THRIVE (the Heart Protection Study 2-Treatment of HDL to Reduce the Incidence of Vascular Events) trial casts further doubt on the HDL cholesterol hypothesis. The growing impression that HDL functionality, rather than abundance, is clinically important is supported by experimental evidence highlighting the conditional pleiotropic actions of HDL. Non-HDL cholesterol reflects the cholesterol in all

  5. [The clinical significance of selenium deficiency in patients from the Samara region with cardiovascular diseases and its correction with the preparation Selena].

    PubMed

    Kuznetsov, G P; Lebedev, P A

    1995-01-01

    Selenium concentration in blood, glutathionperoxidase (GSH-Px) activity in erythrocytes and lipid peroxides in plasma were studied in patients with ischemic heart disease, hypertrophic (HCMP) and dilated cardiomyopathy (DCMP). Patients with IHD, HCMP and DCMP revealed depressed activity of GSH-Px with activation of lipid peroxides associated with lowered selenium content in blood. The selenium content in blood was lower in patients with severe forms of IHD Daily intake of 300 micrograms selenium with "Selena" during a month increases selenium concentration by 71% in patients with IHD and DCMP. This increase change inversely with primary selenium concentration in plasma. Plasma Malone dialdehyde concentration decreased by 17%. The results obtained suggest participation of selenium in cardiomyopathy and IHD pathogenesis, thus forming a basis for selecting the patients with selenium deficiency for its further correction with "Selena."

  6. Lenalidomide as a disease-modifying agent in patients with del(5q) myelodysplastic syndromes: linking mechanism of action to clinical outcomes.

    PubMed

    Giagounidis, Aristoteles; Mufti, Ghulam J; Fenaux, Pierre; Germing, Ulrich; List, Alan; MacBeth, Kyle J

    2014-01-01

    Deletion of the long arm of chromosome 5, del(5q), is the most prevalent cytogenetic abnormality in patients with myelodysplastic syndromes (MDS). In isolation, it is traditionally associated with favorable prognosis compared with other subtypes of MDS. However, owing to the inherent heterogeneity of the disease, prognosis for patients with del(5q) MDS is highly variable depending on the presence of factors such as additional chromosomal abnormalities, >5 % blasts in the bone marrow (BM), or transfusion dependence. Over recent years, the immunomodulatory drug lenalidomide has demonstrated remarkable efficacy in patients with del(5q) MDS. Advances in the understanding of the pathogenesis of the disease have suggested that lenalidomide targets aberrant signaling pathways caused by haplosufficiency of specific genes in a commonly deleted region on chromosome 5 (e.g., SPARC, RPS14, Cdc25C, and PP2A). As a result, the agent specifically targets del(5q) clones while also promoting erythropoiesis and repopulation of the bone marrow in normal cells. This review discusses recent developments in the understanding of the mechanism of action of lenalidomide, and how this underlies favorable outcomes in patients with del(5q) MDS. In addition, we discuss how improved understanding of the mechanism of disease will facilitate clinicians' ability to predict/monitor response and identify patients at risk of relapse. PMID:24018623

  7. Lenalidomide as a disease-modifying agent in patients with del(5q) myelodysplastic syndromes: linking mechanism of action to clinical outcomes.

    PubMed

    Giagounidis, Aristoteles; Mufti, Ghulam J; Fenaux, Pierre; Germing, Ulrich; List, Alan; MacBeth, Kyle J

    2014-01-01

    Deletion of the long arm of chromosome 5, del(5q), is the most prevalent cytogenetic abnormality in patients with myelodysplastic syndromes (MDS). In isolation, it is traditionally associated with favorable prognosis compared with other subtypes of MDS. However, owing to the inherent heterogeneity of the disease, prognosis for patients with del(5q) MDS is highly variable depending on the presence of factors such as additional chromosomal abnormalities, >5 % blasts in the bone marrow (BM), or transfusion dependence. Over recent years, the immunomodulatory drug lenalidomide has demonstrated remarkable efficacy in patients with del(5q) MDS. Advances in the understanding of the pathogenesis of the disease have suggested that lenalidomide targets aberrant signaling pathways caused by haplosufficiency of specific genes in a commonly deleted region on chromosome 5 (e.g., SPARC, RPS14, Cdc25C, and PP2A). As a result, the agent specifically targets del(5q) clones while also promoting erythropoiesis and repopulation of the bone marrow in normal cells. This review discusses recent developments in the understanding of the mechanism of action of lenalidomide, and how this underlies favorable outcomes in patients with del(5q) MDS. In addition, we discuss how improved understanding of the mechanism of disease will facilitate clinicians' ability to predict/monitor response and identify patients at risk of relapse.

  8. [Clinical analysis of cat scratch disease].

    PubMed

    Yoshida, Hiroshi; Kusaba, Nobuhide; Sata, Michio

    2010-05-01

    We analyzed the clinical background of 63 patients with serologically confirmed cat scratch disease (CSD), Age range of the patients was 0 to 83 years old and mean age was 35.0 years old. Seasonal patterns of cases was observed. A number of patients with CSD was increased during the summer and fall. The peak incidence of CSD occurred in October. Infection followed direct cat or dog contact. Cat contact occurred in 61 cases (96.8%) and dog contact in 2 cases (3.2%). A specific contact with kittens occurred in 39 cases (61.9%). About 49.2% of patients had a cat scratch, 3.2% had a cat bite, 3.2% had a cat flea bite, 41.2% had no history of animal bite. The papule of inoculation site were seen in 27 cases (42.9%) of CSD. The upper extremities were the most likely locations for scratches. Sixty cases (95.2%) of CSD developed lymphadenopathy, 51.7% of the involved nodes were in the axillary, 31.7% were in the inguinal, 21.7% were in the cervical, 16.7% were in the elbow. The mean incubation period of patients with CSD was 18.9 days. The mean duration of lymphadenopathy after the treatment of antibiotics was 44.2 days. The mean value of white blood cell counts was 8130/microL. The mean value of C-reactive protein level was 2.83 mg/dL.

  9. Disease Severity, Quality of Life, and Psychiatric Morbidity in Patients With Psoriasis With Reference to Sociodemographic, Lifestyle, and Clinical Variables: A Prospective, Cross-Sectional Study From Lahore, Pakistan

    PubMed Central

    Khawaja, Abdul Rahman; Bokhari, Syed Muhammad Azam; Rasheed, Tariq; Shahzad, Atif; Hanif, Muhammad; Qadeer, Faisal

    2015-01-01

    Background: Psoriasis is an immune-mediated, chronic disease with a genetic background that involves skin, nails, and joints. The incidence of psoriasis varies from 2.0% to 4.0% depending on the geographical location, ethnic background, and environmental conditions. Recent research has proved that psoriasis is a systemic inflammatory disease with extensive systemic implications. Objectives of the study were to explore the severity of psoriasis, dermatology-related quality of life, and psychiatric health of the patients with reference to sociodemographic, lifestyle, and clinical characteristics. Method: Consecutive patients with psoriasis (ICD-10 criteria) from skin outpatient clinics of 3 tertiary care hospitals in Lahore, Pakistan, between November 1, 2012, and December 31, 2012, were assessed in this prospective cross-sectional study. The final sample includes 87 patients who were evaluated for severity of psoriasis (Psoriasis Area Severity Index [PASI]), dermatology-related quality of life (Dermatology Life Quality Index [DLQI]), and psychiatric morbidity (12-item General Health Questionnaire [GHQ-12]) and were assessed on 23 sociodemographic, lifestyle, and clinical variables. Results: Of the 23 variables, the PASI was significantly associated with education and habit of drinking alcohol (P < .05), the DLQI was significantly associated with disturbed eating (P < .05), and the GHQ-12 score was significantly associated with hair disease (P < .05), current income (P < .05), and disturbed eating and sleeping (P < .01). The PASI, DLQI, and GHQ-12 were not usually affected by sociodemographic, lifestyle, and clinical factors, except for some variables such as education of the patient, alcohol intake, eating and sleeping disturbance, and income status. A statistically significant correlation (P < .01) was found between all 3 scores (ie, PASI, DLQI, and GHQ-12). The correlation coefficients of the PASI with the DLQI and GHQ-12 are 0.345 and 0.460, respectively, and

  10. Use of Cox's Cure Model to Establish Clinical Determinants of Long-Term Disease-Free Survival in Neoadjuvant-Chemotherapy-Treated Breast Cancer Patients without Pathologic Complete Response.

    PubMed

    Asano, Junichi; Hirakawa, Akihiro; Hamada, Chikuma; Yonemori, Kan; Hirata, Taizo; Shimizu, Chikako; Tamura, Kenji; Fujiwara, Yasuhiro

    2013-01-01

    In prognostic studies for breast cancer patients treated with neoadjuvant chemotherapy (NAC), the ordinary Cox proportional-hazards (PH) model has been often used to identify prognostic factors for disease-free survival (DFS). This model assumes that all patients eventually experience relapse or death. However, a subset of NAC-treated breast cancer patients never experience these events during long-term follow-up (>10 years) and may be considered clinically "cured." Clinical factors associated with cure have not been studied adequately. Because the ordinary Cox PH model cannot be used to identify such clinical factors, we used the Cox PH cure model, a recently developed statistical method. This model includes both a logistic regression component for the cure rate and a Cox regression component for the hazard for uncured patients. The purpose of this study was to identify the clinical factors associated with cure and the variables associated with the time to recurrence or death in NAC-treated breast cancer patients without a pathologic complete response, by using the Cox PH cure model. We found that hormone receptor status, clinical response, human epidermal growth factor receptor 2 status, histological grade, and the number of lymph node metastases were associated with cure.

  11. Autosomal dominant polycystic kidney disease: recent advances in clinical management.

    PubMed

    Mao, Zhiguo; Chong, Jiehan; Ong, Albert C M

    2016-01-01

    The first clinical descriptions of autosomal dominant polycystic kidney disease (ADPKD) go back at least 500 years to the late 16 (th) century. Advances in understanding disease presentation and pathophysiology have mirrored the progress of clinical medicine in anatomy, pathology, physiology, cell biology, and genetics. The identification of PKD1 and PKD2, the major genes mutated in ADPKD, has stimulated major advances, which in turn have led to the first approved drug for this disorder and a fresh reassessment of patient management in the 21 (st) century. In this commentary, we consider how clinical management is likely to change in the coming decade. PMID:27594986

  12. Autosomal dominant polycystic kidney disease: recent advances in clinical management

    PubMed Central

    Mao, Zhiguo; Chong, Jiehan; Ong, Albert C. M.

    2016-01-01

    The first clinical descriptions of autosomal dominant polycystic kidney disease (ADPKD) go back at least 500 years to the late 16 th century. Advances in understanding disease presentation and pathophysiology have mirrored the progress of clinical medicine in anatomy, pathology, physiology, cell biology, and genetics. The identification of PKD1 and PKD2, the major genes mutated in ADPKD, has stimulated major advances, which in turn have led to the first approved drug for this disorder and a fresh reassessment of patient management in the 21 st century. In this commentary, we consider how clinical management is likely to change in the coming decade. PMID:27594986

  13. Comparison of 2-year clinical outcomes between diabetic versus nondiabetic patients with acute myocardial infarction after 1-month stabilization: Analysis of the prospective registry of DIAMOND (DIabetic acute myocardial infarctiON Disease) in Korea: an observational registry study.

    PubMed

    Hur, Seung-Ho; Won, Ki-Bum; Kim, In-Cheol; Bae, Jang-Ho; Choi, Dong-Ju; Ahn, Young-Keun; Park, Jong-Seon; Kim, Hyo-Soo; Choi, Rak-Kyeong; Choi, Donghoon; Kim, Joon-Hong; Han, Kyoo-Rok; Park, Hun-Sik; Choi, So-Yeon; Yoon, Jung-Han; Gwon, Hyeon-Cheol; Rha, Seung-Woon; Jang, Wooyeong; Bae, Jang-Whan; Hwang, Kyung-Kuk; Lim, Do-Sun; Jung, Kyung-Tae; Oh, Seok-Kyu; Lee, Jae-Hwan; Shin, Eun-Seok; Kim, Kee-Sik

    2016-06-01

    This study assessed the 2-year clinical outcomes of patients with diabetes mellitus (DM) after acute myocardial infarction (AMI) in a cohort of the DIAMOND (DIabetic Acute Myocardial infarctiON Disease) registry. Clinical outcomes were compared between 1088 diabetic AMI patients in the DIAMOND registry after stabilization of MI and 1088 nondiabetic AMI patients from the KORMI (Korean AMI) registry after 1 : 1 propensity score matching using traditional cardiovascular risk factors. Stabilized patients were defined as patients who did not have any clinical events within 1 month after AMI. Primary outcomes were the 2-year rate of major adverse cardiac events (MACEs), a composite of all-cause death, recurrent MI (re-MI), and target vessel revascularization (TVR). Matched comparisons revealed that diabetic patients exhibited significantly lower left ventricular ejection fraction (LVEF) and estimated glomerular filtration rate and smaller stent size. Diabetic patients exhibited significantly higher 2-year rates of MACE (8.0% vs 3.7%), all-cause death (3.9% vs 1.4%), re-MI (2.8% vs 1.2%), and TVR (3.5% vs 1.3%) than nondiabetic patients (all P < 0.01), and higher cumulative rates in Kaplan-Meier analyses of MACE, all-cause death, and TVR (all P < 0.05). A multivariate Cox regression analysis revealed that chronic kidney disease, LVEF < 35%, and long stent were independent predictors of MACE, and large stent diameter and the use of drug-eluting stents were protective factors against MACE. The 2-year MACE rate beyond 1 month after AMI was significantly higher in DM patients than non-DM patients, and this rate was associated with higher comorbidities, coronary lesions, and procedural characteristics in DM.

  14. Effect of dronedarone on clinical end points in patients with atrial fibrillation and coronary heart disease: insights from the ATHENA trial

    PubMed Central

    Pisters, Ron; Hohnloser, Stefan H.; Connolly, Stuart J.; Torp-Pedersen, Christian; Naditch-Brûlé, Lisa; Page, Richard L.; Crijns, Harry J.G.M.

    2014-01-01

    Aims This study aimed to assess safety and cardiovascular outcomes of dronedarone in patients with paroxysmal or persistent atrial fibrillation (AF) with coronary heart disease (CHD). Coronary heart disease is prevalent among AF patients and limits antiarrhythmic drug use because of their potentially life-threatening ventricular proarrhythmic effects. Methods and results This post hoc analysis evaluated 1405 patients with paroxysmal or persistent AF and CHD from the ATHENA trial. Follow-up lasted 2.5 years, during which patients received either dronedarone (400 mg twice daily) or a double-blind matching placebo. Primary outcome was time to first cardiovascular hospitalization or death due to any cause. Secondary end points included first hospitalization due to cardiovascular events. The primary outcome occurred in 350 of 737 (47%) placebo patients vs. 252 of 668 (38%) dronedarone patients [hazard ratio (HR) = 0.73; 95% confidence interval (CI) = 0.62–0.86; P = 0.0002] without a significant increase in number of adverse events. In addition, 42 of 668 patients receiving dronedarone suffered from a first acute coronary syndrome compared with 67 of 737 patients from the placebo group (HR = 0.67; 95% CI = 0.46–0.99; P = 0.04). Conclusion In this post hoc analysis, dronedarone on top of standard care in AF patients with CHD reduced cardiovascular hospitalization or death similar to that in the overall ATHENA population, and reduced a first acute coronary syndrome. Importantly, the safety profile in this subpopulation was also similar to that of the overall ATHENA population, with no excess in proarrhythmias. The mechanism of the cardiovascular protective effects is unclear and warrants further investigation. PMID:24072451

  15. Elderly patients and inflammatory bowel disease

    PubMed Central

    Nimmons, Danielle; Limdi, Jimmy K

    2016-01-01

    The incidence and prevalence of inflammatory bowel disease (IBD) is increasing globally. Coupled with an ageing population, the number of older patients with IBD is set to increase. The clinical features and therapeutic options in young and elderly patients are comparable but there are some significant differences. The wide differential diagnosis of IBD in elderly patients may result in a delay in diagnosis. The relative dearth of data specific to elderly IBD patients often resulting from their exclusion from pivotal clinical trials and the lack of consensus guidelines have made clinical decisions somewhat challenging. In addition, age specific concerns such as co-morbidity; loco-motor and cognitive function, poly-pharmacy and its consequences need to be taken into account. In applying modern treatment paradigms to the elderly, the clinician must consider the potential for more pronounced adverse effects in this vulnerable group and set appropriate boundaries maximising benefit and minimising harm. Meanwhile, clinicians need to make personalised decisions but as evidence based as possible in the holistic, considered and optimal management of IBD in elderly patients. In this review we will cover the clinical features and therapeutic options of IBD in the elderly; as well as addressing common questions and challenges posed by its management. PMID:26855812

  16. Clinical Scenarios in Chronic Kidney Disease: Cystic Renal Diseases.

    PubMed

    Meola, Mario; Samoni, Sara; Petrucci, Ilaria

    2016-01-01

    Cysts are frequently found in chronic kidney disease (CKD) and they have a different prognostic significance depending on the clinical context. Simple solitary parenchymal cysts and peripelvic cysts are very common and they have no clinical significance. At US, simple cyst appears as a round anechoic pouch with regular and thin profiles. On the other hand, hereditary polycystic disease is a frequent cause of CKD in children and adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are the best known cystic hereditary diseases. ADPKD and ARPKD show a diffused cystic degeneration with cysts of different diameters derived from tubular epithelium. Medullary cystic disease may be associated with tubular defects, acidosis and lithiasis and can lead to CKD. Acquired cystic kidney disease, finally, is secondary to progressive structural end-stage kidney remodelling and may be associated with renal cell carcinoma. PMID:27169740

  17. The effect of multiple micronutrient supplementation on quality of life in patients with symptomatic heart failure secondary to ischemic heart disease: a prospective case series clinical study.

    PubMed

    Wong, Ang-Peng; Mohamed, Abdul-Latiff; Niedzwiecki, Aleksandra

    2015-01-01

    Heart failure is a progressive cardiovascular disorder and, in most cases, begins with atherosclerosis and ischemic heart disease. The prognosis of patients with heart failure is poor, even with improvement on the management of all forms of ischemic heart disease. There have been studies on heart failure using a single nutrient or a combination of multiple nutrients. Results are mixed. The aim of this study was to assess the influence of multiple micronutrient supplementation using the quality of life measure on patients with heart failure secondary to ischemic heart disease. This prospective case series followed 12 patients for a period between 3 to 8 months, using the Minnesota Living with Heart Failure Questionnaire (MLHFQ) as the sole outcome measure. The primary outcome was a score change over time between the start and endpoint of treatment. Change in MLHFQ mean total score was 27.08 ± 20.43 and mean symptoms score was 4.67 ± 3.34. Paired t-test showed a difference between baseline and endpoint of treatment (P < 0.001), which was statistically significant. A high dose of multiple micronutrients may have beneficial effects on cardiac function in patients with symptomatic heart failure. This study indicates the need for long-term controlled studies to test the efficacy and safety of this economic approach in managing heart failure.

  18. The effect of multiple micronutrient supplementation on quality of life in patients with symptomatic heart failure secondary to ischemic heart disease: a prospective case series clinical study

    PubMed Central

    Wong, Ang-Peng; Mohamed, Abdul-Latiff; Niedzwiecki, Aleksandra

    2015-01-01

    Heart failure is a progressive cardiovascular disorder and, in most cases, begins with atherosclerosis and ischemic heart disease. The prognosis of patients with heart failure is poor, even with improvement on the management of all forms of ischemic heart disease. There have been studies on heart failure using a single nutrient or a combination of multiple nutrients. Results are mixed. The aim of this study was to assess the influence of multiple micronutrient supplementation using the quality of life measure on patients with heart failure secondary to ischemic heart disease. This prospective case series followed 12 patients for a period between 3 to 8 months, using the Minnesota Living with Heart Failure Questionnaire (MLHFQ) as the sole outcome measure. The primary outcome was a score change over time between the start and endpoint of treatment. Change in MLHFQ mean total score was 27.08 ± 20.43 and mean symptoms score was 4.67 ± 3.34. Paired t-test showed a difference between baseline and endpoint of treatment (P < 0.001), which was statistically significant. A high dose of multiple micronutrients may have beneficial effects on cardiac function in patients with symptomatic heart failure. This study indicates the need for long-term controlled studies to test the efficacy and safety of this economic approach in managing heart failure. PMID:26417534

  19. Clinical management of patients with hyperthyroidism

    SciTech Connect

    Cooper, D.S.; Ridgway, E.C.

    1985-09-01

    The clinical management of the hyperthyroid patient is controversial, because there is no perfect treatment. Factors that influence the choice of therapy include the patient's age, sex, and type of hyperthyroidism, as well as patient and physician preference.

  20. Improving clinical skills to support the emotional and psychological well-being of patients with end-stage renal disease: a qualitative evaluation of two interventions

    PubMed Central

    Taylor, Francesca; Combes, Gill; Hare, Jennifer

    2016-01-01

    Background Many patients with end-stage renal disease (ESRD) need and want improved emotional and psychological support. Explicit attention to patients' emotional issues during consultations can help, yet renal consultants rarely address emotional problems. This qualitative study aimed to evaluate whether two different low-cost interventions could individually enable consultants to talk with patients about their emotional concerns during routine outpatient consultations. Method One intervention involved patients using a Patient Issues Sheet to identify two to three issues they would like to talk about in their consultation and the second involved consultants asking patients a direct question about their emotional feelings. Consultants were trained to handle any emotional issues raised. Semi-structured interviews were conducted with five consultants and 36 ESRD patients from two UK renal units. Interviews were transcribed verbatim and analysed using the constant comparative method. Results Although consultants and patients tended to use the two interventions in different ways, they expressed generally positive views about how helpful the interventions were in promoting discussion of emotional issues. Consultants appreciated the training for facilitating empathetic handling of patients' emotional disclosures and containment of discussion. Most patients who raised emotional concerns were satisfied with their consultant's responses, while others were dissuaded from more explicit discussion by their consultant's concentration on physical considerations. Conclusions These qualitative study findings suggest that both interventions are feasible and acceptable and have the potential to help consultants improve emotional and psychological patient care, providing cognitive and behavioural tools to enable discussion of emotional issues during routine outpatient consultations. PMID:27274842

  1. Laparoscopic splenectomy in patients with hematologic diseases.

    PubMed Central

    Flowers, J L; Lefor, A T; Steers, J; Heyman, M; Graham, S M; Imbembo, A L

    1996-01-01

    OBJECTIVE. The authors review their initial experience with laparoscopic splenectomy in patients with hematologic diseases. Efficacy, morbidity, and mortality of the technique are presented, and other patient recovery parameters are discussed. SUMMARY BACKGROUND DATA. Laparoscopic splenectomy is performed infrequently and data regarding its safety and efficacy are scarce. Factors such as a high level of technical difficulty, the potential for sudden, severe hemorrhage, and slow accrual of operative experience due to a relatively limited number of procedures are responsible. The potential patient benefits from the development of a minimally invasive form of splenectomy are significant. METHODS. Clinical follow-up, a prospective longitudinal database, and review of medical records were analyzed for all patients referred for elective splenectomy for hematologic disease from March 1992 to March 1995. RESULTS. Laparoscopic splenectomy was attempted in 43 patients and successfully completed in 35 (81%). Therapeutic platelet response to splenectomy occurred in 82% of patients with immune thrombocytopenic purpura and hematocrit level increased in 60% of patients with autoimmune hemolytic anemia undergoing successful laparoscopic splenectomy. The morbidity rate was 11.6% (5 of 43 patients), and the mortality rate was 4.7% (2 of 43 patients). Return of gastrointestinal function occurred in patients 23.1 hours after laparoscopic splenectomy and 76 hours after conversion to open splenectomy (p < 0.05). Mean length of stay was 2.7 days after laparoscopic splenectomy and 6.8 days after conversion to open splenectomy (p < 0.05). CONCLUSION. Laparoscopic splenectomy may be performed with efficacy, morbidity, and mortality rates comparable to those of open splenectomy for hematologic diseases, and it appears to retain other patient benefits of laparoscopic surgery. Images Figure 2. Figure 3. Figure 4. Figure 5. Figure 6. PMID:8678613

  2. Asymmetrical Pedaling Patterns in Parkinson's Disease Patients

    PubMed Central

    Penko, Amanda L.; Hirsch, Joshua R.; Voelcker-Rehage, Claudia; Martin, Philip E.; Blackburn, Gordon; Alberts, Jay L.

    2015-01-01

    Background Approximately 1.5 million Americans are affected by Parkinson's disease [1] which includes the symptoms of postural instability and gait dysfunction. Currently, clinical evaluations of postural instability and gait dysfunction consist of a subjective rater assessment of gait patterns using items from the Unified Parkinson's Disease Rating Scale, and assessments can be insensitive to the effectiveness of medical interventions. Current research suggests the importance of cycling for Parkinson's disease patients, and while Parkinson's gait has been evaluated in previous studies, little is known about lower extremity control during cycling. The purpose of this study is to examine the lower extremity coordination patterns of Parkinson's patients during cycling. Methods Twenty five participants, ages 44-72, with a clinical diagnosis of idiopathic Parkinson's disease participated in an exercise test on a cycle ergometer that was equipped with pedal force measurements. Crank torque, crank angle and power produced by right and left leg were measured throughout the test to calculate Symmetry Index at three stages of exercise (20 Watt, 60 Watt, maximum performance). Findings Decreases in Symmetry Index were observed for average power output in Parkinson's patients as workload increased. Maximum power Symmetry Index showed a significant difference in symmetry between performance at both the 20 Watt and 60 Watt stage and the maximal resistance stage. Minimum power Symmetry Index did not show significant differences across the stages of the test. While lower extremity asymmetries were present in Parkinson's patients during pedaling, these asymmetries did not correlate to postural instability and gait dysfunction Unified Parkinson's Disease Rating Scale scores. Interpretation This pedaling analysis allows for a more sensitive measure of lower extremity function than the Unified Parkinson's Disease Rating Scale and may help to provide unique insight into current and

  3. [Clinical guidelines for infantile-onset Pompe disease].

    PubMed

    Pascual-Pascual, S I; Nascimento, A; Fernandez-Llamazares, C M; Medrano-Lopez, C; Villalobos-Pinto, E; Martinez-Moreno, M; Ley, M; Manrique-Rodriguez, S; Blasco-Alonso, J

    2016-09-16

    Infantile-onset Pompe disease has a fatal prognosis in the short term unless it is diagnosed at an early stage and enzyme replacement therapy is not started as soon as possible. A group of specialists from different disciplines involved in this disease have reviewed the current scientific evidence and have drawn up an agreed series of recommendations on the diagnosis, treatment and follow-up of patients. We recommend establishing enzyme treatment in any patient with symptomatic Pompe disease with onset within the first year of life, with a clinical and enzymatic diagnosis, and once the CRIM (cross-reactive immunological material) status is known.

  4. [Clinical guidelines for infantile-onset Pompe disease].

    PubMed

    Pascual-Pascual, S I; Nascimento, A; Fernandez-Llamazares, C M; Medrano-Lopez, C; Villalobos-Pinto, E; Martinez-Moreno, M; Ley, M; Manrique-Rodriguez, S; Blasco-Alonso, J

    2016-09-16

    Infantile-onset Pompe disease has a fatal prognosis in the short term unless it is diagnosed at an early stage and enzyme replacement therapy is not started as soon as possible. A group of specialists from different disciplines involved in this disease have reviewed the current scientific evidence and have drawn up an agreed series of recommendations on the diagnosis, treatment and follow-up of patients. We recommend establishing enzyme treatment in any patient with symptomatic Pompe disease with onset within the first year of life, with a clinical and enzymatic diagnosis, and once the CRIM (cross-reactive immunological material) status is known. PMID:27600742

  5. Defining Clinical Excellence in Adult Infectious Disease Practice.

    PubMed

    Chida, Natasha M; Ghanem, Khalil G; Auwaerter, Paul G; Wright, Scott M; Melia, Michael T

    2016-09-01

    Clinical excellence should be recognized, particularly in the current climate that appropriately prioritizes relationship-centered care. In order to develop a recognition model, a definition of clinical excellence must be created and agreed upon. A paradigm recently suggested by C. Christmas describes clinical excellence through the following domains: diagnostic acumen, professionalism and humanism, communication and interpersonal skills, skillful negotiation of the healthcare system, knowledge, taking a scholarly approach to clinical practice, and having passion for clinical medicine. This work references examples of infectious disease (ID) clinical excellence across Christmas' domains and, in doing so, both examines how the definition of clinical excellence applies to ID practice and highlights the importance of ID physicians. Emphasizing such aspirational standards may not only inspire trainees and practicing physicians to pursue their own fulfilling clinical ID careers, it may also encourage health systems to fully value outstanding ID physicians who labor tirelessly to provide patients with exceptional care. PMID:27419186

  6. Defining Clinical Excellence in Adult Infectious Disease Practice

    PubMed Central

    Chida, Natasha M.; Ghanem, Khalil G.; Auwaerter, Paul G.; Wright, Scott M.; Melia, Michael T.

    2016-01-01

    Clinical excellence should be recognized, particularly in the current climate that appropriately prioritizes relationship-centered care. In order to develop a recognition model, a definition of clinical excellence must be created and agreed upon. A paradigm recently suggested by C. Christmas describes clinical excellence through the following domains: diagnostic acumen, professionalism and humanism, communication and interpersonal skills, skillful negotiation of the healthcare system, knowledge, taking a scholarly approach to clinical practice, and having passion for clinical medicine. This work references examples of infectious disease (ID) clinical excellence across Christmas' domains and, in doing so, both examines how the definition of clinical excellence applies to ID practice and highlights the importance of ID physicians. Emphasizing such aspirational standards may not only inspire trainees and practicing physicians to pursue their own fulfilling clinical ID careers, it may also encourage health systems to fully value outstanding ID physicians who labor tirelessly to provide patients with exceptional care. PMID:27419186

  7. Defining Clinical Excellence in Adult Infectious Disease Practice.

    PubMed

    Chida, Natasha M; Ghanem, Khalil G; Auwaerter, Paul G; Wright, Scott M; Melia, Michael T

    2016-09-01

    Clinical excellence should be recognized, particularly in the current climate that appropriately prioritizes relationship-centered care. In order to develop a recognition model, a definition of clinical excellence must be created and agreed upon. A paradigm recently suggested by C. Christmas describes clinical excellence through the following domains: diagnostic acumen, professionalism and humanism, communication and interpersonal skills, skillful negotiation of the healthcare system, knowledge, taking a scholarly approach to clinical practice, and having passion for clinical medicine. This work references examples of infectious disease (ID) clinical excellence across Christmas' domains and, in doing so, both examines how the definition of clinical excellence applies to ID practice and highlights the importance of ID physicians. Emphasizing such aspirational standards may not only inspire trainees and practicing physicians to pursue their own fulfilling clinical ID careers, it may also encourage health systems to fully value outstanding ID physicians who labor tirelessly to provide patients with exceptional care.

  8. A Randomized, Double Blinded, Clinical Trial to Assess the Efficacy and Cost Effectiveness of Omeprazole Compared to Rabeprazole in the Maintenance Therapy of Patients With Gastroesophageal Reflux Disease

    PubMed Central

    Park, Jung Ho; Lee, Dong Ho; Sung, In Kyung

    2013-01-01

    Background/Aims The aim of this study was to evaluate the efficacy and cost efficiency of omeprazole 10 mg and rabeprazole 10 mg once daily for 24 weeks in the maintenance therapy. Methods This was a randomized, open-label study enrolling 279 patients with erosive esophagitis A or B (Los Angeles classification) and typical gastroesophageal reflux disease symptoms. Patients who showed complete endoscopic and symptomatic healing after 8 weeks of proton pump inhibitor treatment were randomly allocated to maintenance treatment with omeprazole 10 mg once daily or rabeprazole 10 mg once daily for 42 weeks. The primary efficacy endpoint was the proportion of patients with symptomatic remission at 42 weeks. Results At the end of 42 weeks of maintenance therapy, 96.4% of omeprazole and 95.1% of rabeprazole treated patients remained symptom free (P > 0.05). Two drugs were also comparable with regard to the severity and frequency of reflux symptoms during the maintenance phase (P > 0.05). By the cost-minimization analysis, the mean total costs per patient for remaining symptom-free for 6 months were 241,775 won for omeprazole and 287,115 won for rabeprazole, respectively. Conclusions Omeprazole 10 mg appeared to have similar efficacy in maintaining symptomatic remission as rabeprazole 10 mg, but was superior to rabeprazole 10 mg in terms of cost efficiency in the maintenance therapy of gastroesophageal reflux disease symptoms. PMID:23667753

  9. Clinical utility of neuronal cells directly converted from fibroblasts of patients for neuropsychiatric disorders: studies of lysosomal storage diseases and channelopathy.

    PubMed

    Kano, S; Yuan, M; Cardarelli, R A; Maegawa, G; Higurashi, N; Gaval-Cruz, M; Wilson, A M; Tristan, C; Kondo, M A; Chen, Y; Koga, M; Obie, C; Ishizuka, K; Seshadri, S; Srivastava, R; Kato, T A; Horiuchi, Y; Sedlak, T W; Lee, Y; Rapoport, J L; Hirose, S; Okano, H; Valle, D; O'Donnell, P; Sawa, A; Kai, M

    2015-01-01

    Methodologies for generating functional neuronal cells directly from human fibroblasts [induced neuronal (iN) cells] have been recently developed, but the research so far has only focused on technical refinements or recapitulation of known pathological phenotypes. A critical question is whether this novel technology will contribute to elucidation of novel disease mechanisms or evaluation of therapeutic strategies. Here we have addressed this question by studying Tay-Sachs disease, a representative lysosomal storage disease, and Dravet syndrome, a form of severe myoclonic epilepsy in infancy, using human iN cells with feature of immature postmitotic glutamatergic neuronal cells. In Tay-Sachs disease, we have successfully characterized canonical neuronal pathology, massive accumulation of GM2 ganglioside, and demonstrated the suitability of this novel cell culture for future drug screening. In Dravet syndrome, we have identified a novel functional phenotype that was not suggested by studies of classical mouse models and human autopsied brains. Taken together, the present study demonstrates that human iN cells are useful for translational neuroscience research to explore novel disease mechanisms and evaluate therapeutic compounds. In the future, research using human iN cells with well-characterized genomic landscape can be integrated into multidisciplinary patient-oriented research on neuropsychiatric disorders to address novel disease mechanisms and evaluate therapeutic strategies.

  10. Clinical utility of neuronal cells directly converted from fibroblasts of patients for neuropsychiatric disorders: studies of lysosomal storage diseases and channelopathy.

    PubMed

    Kano, S; Yuan, M; Cardarelli, R A; Maegawa, G; Higurashi, N; Gaval-Cruz, M; Wilson, A M; Tristan, C; Kondo, M A; Chen, Y; Koga, M; Obie, C; Ishizuka, K; Seshadri, S; Srivastava, R; Kato, T A; Horiuchi, Y; Sedlak, T W; Lee, Y; Rapoport, J L; Hirose, S; Okano, H; Valle, D; O'Donnell, P; Sawa, A; Kai, M

    2015-01-01

    Methodologies for generating functional neuronal cells directly from human fibroblasts [induced neuronal (iN) cells] have been recently developed, but the research so far has only focused on technical refinements or recapitulation of known pathological phenotypes. A critical question is whether this novel technology will contribute to elucidation of novel disease mechanisms or evaluation of therapeutic strategies. Here we have addressed this question by studying Tay-Sachs disease, a representative lysosomal storage disease, and Dravet syndrome, a form of severe myoclonic epilepsy in infancy, using human iN cells with feature of immature postmitotic glutamatergic neuronal cells. In Tay-Sachs disease, we have successfully characterized canonical neuronal pathology, massive accumulation of GM2 ganglioside, and demonstrated the suitability of this novel cell culture for future drug screening. In Dravet syndrome, we have identified a novel functional phenotype that was not suggested by studies of classical mouse models and human autopsied brains. Taken together, the present study demonstrates that human iN cells are useful for translational neuroscience research to explore novel disease mechanisms and evaluate therapeutic compounds. In the future, research using human iN cells with well-characterized genomic landscape can be integrated into multidisciplinary patient-oriented research on neuropsychiatric disorders to address novel disease mechanisms and evaluate therapeutic strategies. PMID:25732146

  11. Acute promyelocytic leukaemia (APL) in a patient with Crohn's disease and exposure to infliximab: a rare clinical presentation and review of the literature.

    PubMed

    Mohammad, Farhan; Vivekanandarajah, Abhirami; Haddad, Housam; Shutty, Christopher M; Hurford, Matthew T; Dai, Qun

    2014-01-01

    With the introduction of potent immunosuppressive and chemotherapeutic medications for various diseases, there is an increased incidence of therapy-related myeloid neoplasms. They are the result of mutational rearrangement and historically, have a grave prognosis compared with de novo myeloid neoplasms. We did a short review on various types of myeloid leukaemias reported after therapy with antitumour necrosis factor and also report, to the best of our knowledge, one among the very few cases of therapy-related acute promyelocytic leukaemia in a patient on infliximab therapy for refractory Crohn's disease. The patient responded well to the traditional treatment and is in complete remission for more than 5 years. PMID:24842356

  12. Peyronie's disease and low intensity shock wave therapy: Clinical outcomes and patient satisfaction rate in an open-label single arm prospective study in Australian men

    PubMed Central

    2015-01-01

    Purpose To evaluate the efficacy, safety and patient satisfaction outcomes following low intensity extracorporeal shock wave therapy (LiESWT) in men with Peyronie's disease (PD) using a standardised protocol. Materials and Methods In this open-label single arm prospective study, patients with PD were enrolled following informed consent. Patient demographics, change in penile curvature and plaque hardness, International Index of Erectile Function (IIEF)-5 score, and overall satisfaction score (on a 5-point scale) were recorded. Treatment template consists of 3000 shock waves to the Peyronie's plaque over 20 minutes, twice weekly for 6 weeks. Results The majority of patients have PD history longer than 6 months (mean, 12.8 months; range, 6-28 months). Two thirds of patients have received and failed oral medical therapy. There were improvements in penile curvature (more than 15 degrees in 33% of men), plaque hardness (60% of men) and penile pain (4 out of 6 men) following LiESWT. There was a moderate improvement in IIEF-5 score (>5 points reported in 20% of men). No complication was reported and the majority of patients were satisfied (rated 4 out of 5; 70% of men) and would recommend this therapy to others. Conclusions In a carefully selected group of men with PD, LiESWT appears to be safe, has moderate efficacy and is associated with high patient satisfaction rate in the short term. PMID:26568796

  13. Subcutaneous sarcoidosis: a clinical analysis of nine patients.

    PubMed

    Ando, Masaru; Miyazaki, Eishi; Hatano, Yutaka; Nishio, Suehiro; Torigoe, Chihiro; Yamasue, Mari; Mukai, Yutaka; Nureki, Shin-Ichi; Kadota, Jun-Ichi

    2016-09-01

    Subcutaneous sarcoidosis is the specific subset of cutaneous sarcoidosis frequently associated with systemic disease. However, the disease activity, severity, and prognosis have not yet been elucidated due to the limited number of reported cases. The purpose of this study was to identify the clinical, laboratory, and prognostic differences between subcutaneous sarcoidosis and other type of cutaneous sarcoidosis. All patients with sarcoidosis diagnosed histopathologically from 2000 to 2012 at our institution were enrolled. The clinical, laboratory, chest X-ray, and pulmonary function test results were retrospectively evaluated in the patients with cutaneous sarcoidosis. In the 130 patients with sarcoidosis, cutaneous sarcoidosis was diagnosed in 37 patients (28.4 %), and 9 (6.9 %) of these patients had subcutaneous sarcoidosis. The serum levels of soluble interleukin-2 receptor (sIL-2R) were significantly elevated in the group of patients with subcutaneous sarcoidosis in comparison to the patients with other types of cutaneous sarcoidosis, whereas there was no significant difference in the severity score between the two groups. Following a 2-year observation period, three patients were in remission, five patients demonstrated stable disease, and one patient had progressive disease. Subcutaneous sarcoidosis may be associated with the disease activity, although it was not found to be associated with the disease severity and it was not a predictive factor for the prognosis. Furthermore, the prevalence of subcutaneous sarcoidosis may be higher than that in previously reported series. PMID:27449952

  14. Outcomes of Bowel Resection in Patients with Crohn's Disease.

    PubMed

    Moghadamyeghaneh, Zhobin; Carmichael, Joseph C; Mills, Steven D; Pigazzi, Alessio; Stamos, Michael J

    2015-10-01

    There is limited data regarding outcomes of bowel resection in patients with Crohn's disease. We sought to investigate complications of such patients after bowel resection. The Nationwide Inpatient Sample databases were used to examine the clinical data of Crohn's patients who underwent bowel resection during 2002 to 2012. Multivariate regression analysis was performed to investigate outcomes of such patients. We sampled a total of 443,950 patients admitted with the diagnosis of Crohn's disease. Of these, 20.5 per cent had bowel resection. Among patients who had bowel resection, 51 per cent had small bowel Crohn's disease, 19.4 per cent had large bowel Crohn's disease, and 29.6 per cent had both large and small bowel Crohn's disease. Patients with large bowel disease had higher mortality risk compared with small bowel disease [1.8% vs 1%, adjusted odds ratio (AOR): 2.42, P < 0.01]. Risks of postoperative renal failure (AOR: 1.56, P < 0.01) and respiratory failure (AOR: 1.77, P < 0.01) were higher in colonic disease compared with small bowel disease but postoperative enteric fistula was significantly higher in patients with small bowel Crohn's disease (AOR: 1.90, P < 0.01). Of the patients admitted with the diagnosis of Crohn's disease, 20.5 per cent underwent bowel resection during 2002 to 2012. Although colonic disease has a higher mortality risk, small bowel disease has a higher risk of postoperative fistula.

  15. [Evaluation of the status of patients with severe infection, criteria for intensive care unit admittance. Spanish Society for Infectious Diseases and Clinical Microbiology. Spanish Society of Intensive and Critical Medicine and Coronary Units].

    PubMed

    Olaechea, Pedro M; Alvarez-Lerma, Francisco; Sánchez, Miguel; Torres, Antonio; Palomar, Mercedes; Fernández, Pedro; Miró, José M; Cisneros, José Miguel; Torres, Manuel

    2009-06-01

    Recent studies have shown that early attention in patients with serious infections is associated with a better outcome. Assistance in intensive care units (ICU) can effectively provide this attention; hence patients should be admitted to the ICU as soon as possible, before clinical deterioration becomes irreversible. The objective of this article is to compile the recommendations for evaluating disease severity in patients with infections and describe the criteria for ICU admission, updating the criteria published 10 years ago. A literature review was carried out, compiling the opinions of experts from the Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica (SEIMC, Spanish Society for Infectious Diseases and Clinical Microbiology) and the Sociedad Española de Medicina Intensiva, Crítica y Unidades Coronarias (SEMICYUC, Spanish Society for Intensive Medicine, Critical Care and Coronary Units) as well as the working groups for infections in critically ill patients (GEIPC-SEIMC and GTEI-SEMICYUC). We describe the specific recommendations for ICU admission related to the most common infections affecting patients, who will potentially benefit from critical care. Assessment of the severity of the patient's condition to enable early intensive care is stressed.

  16. Epoetin alfa. A review of its clinical efficacy in the management of anaemia associated with renal failure and chronic disease and its use in surgical patients.

    PubMed

    Dunn, C J; Wagstaff, A J

    1995-08-01

    Epoetin alfa is a recombinant form of erythropoietin, a glycoprotein hormone which stimulates red blood cell production by stimulating the activity of erythroid progenitor cells. This review discusses the use of the drug in the management of anaemia in diseases often associated with advancing age [renal failure, cancer, rheumatoid arthritis (RA) and other chronic diseases, and the myelodysplastic syndromes (MDS)] and in surgical patients. Intravenous and subcutaneous therapy with epoetin alfa raises haematocrit and haemoglobin levels, and reduces transfusion requirements, in anaemic patients with end-stage renal failure undergoing haemodialysis or peritoneal dialysis. The drug is also effective in the correction of anaemia in patients with chronic renal failure not yet requiring dialysis and does not appear to affect renal haemodynamics adversely or to precipitate the onset of end-stage renal failure. Response rates of 32 to 82% with epoetin alfa therapy have been reported in patients with anaemia associated with cancer or cytotoxic chemotherapy. Limited data in patients with anaemia associated with RA show correction of anaemia after epoetin alfa treatment. Response rates to the drug of 0 to 56% have been noted in patients with MDS. Epoetin alfa also reduces anaemia, increases the capacity for autologous blood donation and reduces the need for allogeneic blood transfusion in patients scheduled to undergo surgery. Hypertension occurs in 30 to 35% of patients with end-stage renal failure who receive epoetin alfa, but this can be managed successfully with correction of fluid status and antihypertensive medication where necessary, and is minimised by avoiding rapid increases in haematocrit. Although vascular access thrombosis has not been conclusively linked to therapy with the drug, increased heparinisation may be required when it is administered to patients on haemodialysis. Epoetin alfa does not appear to exert any direct cerebrovascular adverse effects. Thus

  17. Clinical presentation of metabolic liver disease.

    PubMed

    Odievre, M

    1991-01-01

    Some clinical clues should alert paediatricians to the possibility of metabolic liver diseases. They can be classified into three categories: (i) Manifestations due to hepatocellular necrosis, acute or subacute, which can reveal galactosaemia, hereditary fructose intolerance, tyrosinaemia type I, Wilson disease and alpha 1-antitrypsin deficiency. Symptoms and signs suggestive of Reye syndrome should lead to a study of fatty acid oxidation and urea cycle enzymes. All these manifestations may necessitate a rapid diagnosis and treatment when liver dysfunction is severe. (ii) Cholestatic jaundice can reveal alpha 1-antitrypsin deficiency, Byler's disease, cystic fibrosis, Niemann-Pick disease and some disorders of peroxisome biogenesis. (iii) Hepatomegaly can reveal disorders with liver damage but also storage diseases such as glycogen storage diseases, cholesteryl ester storage disease and, when associated with splenomegaly, lysosomal storage diseases. Appropriate investigations for recognizing all these entities are proposed.

  18. Clinical spectrum of chronic interstitial lung disease in children.

    PubMed

    Fan, L L; Mullen, A L; Brugman, S M; Inscore, S C; Parks, D P; White, C W

    1992-12-01

    To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortality rates.

  19. Frequency of disease-associated and other nuclear autoantibodies in patients of the German network for systemic scleroderma: correlation with characteristic clinical features

    PubMed Central

    2011-01-01

    Introduction In the present study, we analysed in detail nuclear autoantibodies and their associations in systemic sclerosis (SSc) patients included in the German Network for Systemic Scleroderma Registry. Methods Sera of 863 patients were analysed according to a standardised protocol including immunofluorescence, immunoprecipitation, line immunoassay and immunodiffusion. Results Antinuclear antibodies (ANA) were detected in 94.2% of patients. In 81.6%, at least one of the autoantibodies highly associated with SSc or with overlap syndromes with scleroderma features was detected, that is, anti-centromere (35.9%) or anti-topoisomerase I (30.1%), followed in markedly lower frequency by antibodies to PM-Scl (4.9%), U1-ribonucleoprotein (U1-RNP) (4.8%), RNA polymerases (RNAPs) (3.8%), fibrillarin (1.4%), Ku (1.2%), aminoacyl-transfer RNA synthetases (0.5%), To (0.2%) and U11-RNP (0.1%). We found that the simultaneous presence of SSc-associated autoantibodies was rare (1.6%). Furthermore, additional autoantibodies were detected in 55.4% of the patients with SSc, of which anti-Ro/anti-La, anti-mitochondrial and anti-p25/p23 antibodies were most frequent. The coexistence of SSc-associated and other autoantibodies was common (43% of patients). SSc-associated autoantibodies disclosed characteristic associations with clinical features of patients, some of which were previously not acknowledged. Conclusions This study shows that five autoantigens (that is, centromere, topoisomerase I, PM-Scl, U1-RNP and RNAP) detected more than 95% of the known SSc-associated antibody responses in ANA-positive SSc patients and characterise around 79% of all SSc patients in a central European cohort. These data confirm and extend previous data underlining the central role of the determination of ANAs in defining the diagnosis, subset allocation and prognosis of SSc patients. PMID:22018289

  20. MicroRNA-related polymorphisms in apoptosis pathway genes are predictive of clinical outcome in patients with limited disease small cell lung cancer

    PubMed Central

    Jiang, Wei; Bi, Nan; Zhang, Wen-Jue; Wu, Li-Hong; Liu, Li-Pin; Men, Yu; Wang, Jing-Bo; Liang, Jun; Hui, Zhou-Guang; Zhou, Zong-Mei; Wang, Lu-Hua

    2016-01-01

    We examined the impact of single nucleotide polymorphisms (SNPs) at miRNA binding sites in the 3′-UTRs of genes in the apoptosis pathway on the prognosis of patients with limited disease-small cell lung cancer (LD-SCLC). Twelve tagSNPs in seven genes were genotyped using blood samples from 146 LD-SCLC patients treated with chemoradiotherapy. Cox proportional hazard regression models and recursive partitioning analysis were performed to identify SNPs significantly associated with overall survival. Three SNPs, CASP8: rs1045494 (C > T), PIK3R1: rs3756668 (A > G) and CASP7: rs4353229 (T > C), were associated with longer overall survival in LD-SCLC patients after chemoradiotherapy. The adjusted hazard ratios (95% confidence intervals) were 0.480 (0.258–0.894), 0.405 (0.173–0.947) and 0.446 (0.247–0.802), respectively, and remained significant after multiple comparison correction. Moreover, subset analysis showed these SNPs were still predictive of overall survival in stage III patients. Recursive partitioning analysis enabled patients to be classified into three risk subgroups based on unfavorable genotype combinations of the rs1045494 and rs4353229 SNPs. These findings suggest miRNA-related polymorphisms in the apoptosis pathway may be useful biomarkers for selection of LD-SCLC patients likely to benefit from chemoradiotherapy. PMID:26988918

  1. A study of attitudes, beliefs and organisational barriers related to safe emergency oxygen therapy for patients with COPD (chronic obstructive pulmonary disease) in clinical practice and research

    PubMed Central

    O'Driscoll, B Ronan; Bakerly, Nawar Diar; Caress, Ann-Louise; Roberts, June; Gaston, Miriam; Newton, Mark; Yorke, Janelle

    2016-01-01

    Background Patients can be harmed by receiving too little or too much oxygen. There is ongoing disagreement about the use of oxygen in medical emergencies. Methods This was a mixed methods study (survey, telephone interviews and focus groups) involving patients, the public and healthcare professionals (HCPs). Results 62 patients with chronic obstructive pulmonary disease (COPD), 65 members of the public, 68 ambulance crew members, 22 doctors, 22 nurses and 10 hospital managers took part. For five factual questions about oxygen therapy, the average score for correct answers was 28% for patients with COPD, 33% for the general public and 75% for HCPs. The HCPs had an average score of 66% for five technical questions. Patients (79%) and members of the public (68%) were more likely than HCPs (36%) to believe that oxygen was beneficial in most medical emergencies and less likely to have concerns that it might harm some people (35%, 25% and 68%). All groups had complex attitudes about research into oxygen use in medical emergencies. Many participants would not wish for themselves or their loved ones to have their oxygen therapy determined by a randomised protocol, especially if informed consent was not possible in an emergency situation. Conclusions We have found low levels of factual knowledge about oxygen use among patients with COPD and the general public and many false beliefs about the potential benefits and harms of using oxygen. HCPs had a higher level of factual knowledge. All groups had complex attitudes towards research into emergency oxygen use. PMID:27252870

  2. Clinical research of holmium laser therapy in extramammary Paget's disease.

    PubMed

    Ziyao, Li; Deyong, Yang; Xiangyu, Che; Huafeng, Zong; Hafeez, Adnan; Jianbo, Wang; Xishuang, Song

    2014-11-01

    This study aims to investigate the safety and efficiency of the holmium laser therapy in extramammary Paget's disease. The clinical data of 61 patients was collected since 2002 to 2012, confirmed as non-subcutaneous invasive extramammary Paget's disease by biopsy and underwent surgery. All patients were divided into two groups. Group A included 30 patients who underwent the holmium laser therapy. Group B included 31 patients who underwent the traditional surgical therapy. The clinical data of all patients included preoperative, intraoperative, and postoperative management and follow-up records. Compared with the traditional operation group, the holmium laser group had a shorter operation time and was easier to perform. There were no significant differences between the two groups in cases of intraoperative and postoperative complications, the recurrence-free survival, and the disease-specific survival. But the holmium laser group had a longer recovery time than the traditional operation group in large and deep nidus. Multiple-factor analysis of prognostic parameters of 61 patients confirmed that any of these two methods chosen was not a prognostic parameter for recurrence-free survival. The holmium laser therapy might prove to be a preferable alternative to the traditional operative therapy of extramammary Paget's disease. However, the holmium laser therapy did not demonstrate to have an obvious advantage over traditional operative therapy in the recurrence-free survival and the disease-specific survival.

  3. Proteolipoprotein gene analysis in 82 patients with sporadic Pelizaeus-Merzbacher Disease: duplications, the major cause of the disease, originate more frequently in male germ cells, but point mutations do not. The Clinical European Network on Brain Dysmyelinating Disease.

    PubMed Central

    Mimault, C; Giraud, G; Courtois, V; Cailloux, F; Boire, J Y; Dastugue, B; Boespflug-Tanguy, O

    1999-01-01

    Pelizaeus-Merzbacher Disease (PMD) is an X-linked developmental defect of myelination affecting the central nervous system and segregating with the proteolipoprotein (PLP) locus. Investigating 82 strictly selected sporadic cases of PMD, we found PLP mutations in 77%; complete PLP-gene duplications were the most frequent abnormality (62%), whereas point mutations in coding or splice-site regions of the gene were involved less frequently (38%). We analyzed the maternal status of 56 cases to determine the origin of both types of PLP mutation, since this is relevant to genetic counseling. In the 22 point mutations, 68% of mothers were heterozygous for the mutation, a value identical to the two-thirds of carrier mothers that would be expected if there were an equal mutation rate in male and female germ cells. In sharp contrast, among the 34 duplicated cases, 91% of mothers were carriers, a value significantly (chi2=9. 20, P<.01) in favor of a male bias, with an estimation of the male/female mutation frequency (k) of 9.3. Moreover, we observed the occurrence of de novo mutations between parental and grandparental generations in 17 three-generation families, which allowed a direct estimation of the k value (k=11). Again, a significant male mutation imbalance was observed only for the duplications. The mechanism responsible for this strong male bias in the duplications may involve an unequal sister chromatid exchange, since two deletion events, responsible for mild clinical manifestations, have been reported in PLP-related diseases. PMID:10417279

  4. [Kawasaki disease: interdisciplinary and intersocieties consensus (clinical guidelines). Brief version].

    PubMed

    2016-08-01

    Kawasaki disease is an acute self-limiting systemic vasculitis. It is the most common cause of acquired heart disease, with the risk of developing coronary artery aneurysms, myocardial infarction and sudden death. Diagnosis is based on the presence of fever in addition to other clinical criteria. The quarter of the Kawasaki disease patients have "incomplete" presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Non-responders to standard therapy have shown a successful response with the use of corticosteroids and/or biological agents. The long-term management must be delineated according to the degree of coronary involvement in a multidisciplinary manner. To facilitate the pediatrician's diagnosis, treatment and monitoring of Kawasaki disease, a group of experts from the Argentine Society of Pediatrics and the Argentine Society of Cardiology carried out a consensus to develop practical clinical guidelines. PMID:27399018

  5. [Kawasaki disease: interdisciplinary and intersocieties consensus (clinical guidelines). Brief version].

    PubMed

    2016-08-01

    Kawasaki disease is an acute self-limiting systemic vasculitis. It is the most common cause of acquired heart disease, with the risk of developing coronary artery aneurysms, myocardial infarction and sudden death. Diagnosis is based on the presence of fever in addition to other clinical criteria. The quarter of the Kawasaki disease patients have "incomplete" presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Non-responders to standard therapy have shown a successful response with the use of corticosteroids and/or biological agents. The long-term management must be delineated according to the degree of coronary involvement in a multidisciplinary manner. To facilitate the pediatrician's diagnosis, treatment and monitoring of Kawasaki disease, a group of experts from the Argentine Society of Pediatrics and the Argentine Society of Cardiology carried out a consensus to develop practical clinical guidelines.

  6. Clinical differences between alcoholic liver disease and nonalcoholic fatty liver disease.

    PubMed

    Toshikuni, Nobuyuki; Tsutsumi, Mikihiro; Arisawa, Tomiyasu

    2014-07-14

    Alcoholic liver disease (ALD) and nonalcoholic fatty liver disease (NAFLD) are serious health problems worldwide. These two diseases have similar pathological spectra, ranging from simple hepatic steatosis to steatohepatitis, liver cirrhosis, and hepatocellular carcinoma. Although most subjects with excessive alcohol or food intake experience simple hepatic steatosis, a small percentage of individuals will develop progressive liver disease. Notably, both ALD and NAFLD are frequently accompanied by extrahepatic complications, including cardiovascular disease and malignancy. The survival of patients with ALD and NAFLD depends on various disease-associated conditions. This review delineates the clinical characteristics and outcomes of patients with ALD and NAFLD by comparing their epidemiology, the factors associated with disease susceptibility and progression, and the predictors and characteristics of outcomes. A comprehensive understanding of the characteristics and outcomes of ALD and NAFLD is imperative in the management of these chronic liver diseases. PMID:25024597

  7. Impact of high-dose atorvastatin therapy and clinical risk factors on incident aortic valve stenosis in patients with cardiovascular disease (from TNT, IDEAL, and SPARCL).

    PubMed

    Arsenault, Benoit J; Boekholdt, S Matthijs; Mora, Samia; DeMicco, David A; Bao, Weihang; Tardif, Jean-Claude; Amarenco, Pierre; Pedersen, Terje; Barter, Philip; Waters, David D

    2014-04-15

    Clinical trials have not provided evidence for a role of statin therapy in reducing aortic valve stenosis (AVS) severity in patients with documented AVS. However, whether statin therapy could prevent the onset of AVS is unknown. Our objectives were (1) to compare the incidence rates of AVS among patients treated with high-dose versus usual-dose statin or placebo and (2) to identify clinical risk factors associated with the development of AVS. We conducted post hoc analyses in 23,508 participants from 3 large-scale multicenter atorvastatin randomized blinded clinical trials: Treating to New Targets, the Incremental Decrease in End Points Through Aggressive Lipid Lowering, and the Stroke Prevention by Aggressive Reduction in Cholesterol Levels. The main outcome measure was the incidence of clinical AVS over a median follow-up of 4.9 years (82 cases). Among patients who developed AVS, 39 (47.6%) were treated with atorvastatin 80 mg and 43 (52.4%) were treated with lower dose statin (atorvastatin 10 mg in Treating to New Targets, simvastatin 20 to 40 mg in Incremental Decrease in End Points Through Aggressive Lipid Lowering, or placebo in Stroke Prevention by Aggressive Reduction in Cholesterol Levels; hazard ratio [HR] 0.91, 95% confidence interval [CI] 0.59 to 1.41, p=0.67). In multivariate analyses forcing treatment, sex, and race into the model, factors that were significantly associated with AVS included age (HR 2.17, 95% CI 1.61 to 2.93, p<0.0001 per 1-SD increment), diabetes (HR 1.67, 95% CI 1.00 to 2.80, p=0.05), vitamin K antagonist use (HR 3.25, 95% CI 2.06 to 5.16, p<0.0001), and previous statin use (HR 2.65, 95% CI 1.54 to 4.60, p=0.0008). In conclusion, random allocation to high-dose versus usual-dose statin therapy or placebo did not impact the incidence of AVS among patients without known AVS. Age, diabetes, vitamin K antagonists, and previous statin use were significant predictors of incident AVS in these high-risk patients.

  8. Comparison of clinical outcomes of Chinese men and women after coronary stenting for coronary artery disease: a multi-center retrospective analysis of 4,334 patients

    PubMed Central

    Shrestha, Rajiv; Xu, Jing; Xie, Dujiang; Liu, Zhizhong; Xu, Tian; Ye, Fei; Din, Shiqing; Qian, Xuesong; Yang, Song; Liu, Yueqiang; Li, Feng; Zhang, Aiping; Chen, Shaoliang

    2014-01-01

    Abstract The outcome differences between Chinese male and female patients within one-year follow-up after percutaneous coronary intervention (PCI) with stent remain unclear. The present study was aimed to compare clinical outcomes in such two populations. From May 1999 to December 2009, 4,334 patients with acute myocardial infarction (MI), unstable angina, stable angina, or silent ischemia, who underwent PCI, were registered at our centers. Among these, 3,089 were men and 1,245 were women. We compared these groups with respect to the primary outcomes of MI and secondary outcomes including a composite of major adverse cardiac events (MACE) including cardiac death, MI, target lesion revascularization, target vessel revascularization (TVR), stent thrombosis (ST), definite ST and probable ST at one-year follow-up. Chinese male patients had a higher MACE rate (13% vs. 10.7%, P  =  0.039), mainly led by TVR (9.09% vs. 6.98%, P = 0.024) at one year, which was significantly different than female patients. Chinese male and female patients showed a significant difference on MACEs. However, there was no significant difference with respect to MI between these groups. PMID:25332708

  9. Clinical and molecular insights into tuberous sclerosis complex renal disease.

    PubMed

    Siroky, Brian J; Yin, Hong; Bissler, John J

    2011-06-01

    Patients with tuberous sclerosis complex are at great risk of developing renal lesions as part of their disease. These lesions include renal cysts and tumors. Significant advances in understanding the cell biology of these renal lesions has already led to clinical trials demonstrating that pharmacological interventions are likely possible. This review focuses on the pathology of these renal lesions, their underlying cell biology, and the possible therapeutic strategies that may prove to significantly improve care for these patients.

  10. Clinical impact of post-progression survival on overall survival in patients with limited-stage disease small cell lung cancer after first-line chemoradiotherapy

    PubMed Central

    Kasahara, Norimitsu; Imai, Hisao; Kaira, Kyoichi; Mori, Keita; Wakuda, Kazushige; Ono, Akira; Taira, Tetsuhiko; Kenmotsu, Hirotsugu; Harada, Hideyuki; Naito, Tateaki; Murakami, Haruyasu; Endo, Masahiro; Nakajima, Takashi; Yamada, Masanobu; Takahashi, Toshiaki

    2015-01-01

    Background The effects of first-line chemoradiotherapy on overall survival (OS) may be confounded by subsequent lines of therapy in patients with limited-stage disease small cell lung cancer (LD-SCLC). Therefore, we aimed to determine the relationships between progression-free survival (PFS), post-progression survival (PPS) and OS after first-line chemoradiotherapy in LD-SCLC patients. Patients and methods. We retrospectively analyzed 71 LD-SCLC patients with performance status (PS) 0–2 who received first-line chemoradiotherapy and had disease recurrence between September 2002 and March 2013 at Shizuoka Cancer Center (Shizuoka, Japan). We determined the correlation between PFS and OS and between PPS and OS at the individual level. In addition, we performed univariate and multivariate analyses to identify significant prognostic factors of PPS. Results OS is more strongly correlated with PPS (Spearman’s r = 0.86, R2 = 0.72, p < 0.05) than PFS (Spearman’s r = 0.46, R2 = 0.38, p < 0.05). In addition, the response to second-line treatments, the presence of distant metastases at recurrence and the number of additional regimens after first-line chemoradiotherapy were significant independent prognostic factors for PPS. Conclusions PPS has more impact on OS than PFS in recurrent LD-SCLC patients with good PS at beginning of the treatment. Moreover, treatments administered after first-line chemoradiotherapy may affect their OS. However, larger multicenter studies are needed to validate these findings. PMID:26834529

  11. [Clinical condition and therapy of bone diseases].

    PubMed

    Miura, Kohji; Oznono, Keiichi

    2013-12-01

    Skeletal dysplasia is the term which represents disorders including growth and differentiation of bone, cartilage and ligament. A lot of diseases are included, and new disorders have been added. However, the therapy of most bone diseases is less well-established. Achondroplasia, hypochondroplasia, and osteogenesis imperfecta are most frequent bone diseases. There is no curative treatment for these diseases, however, supportive therapies are available ; for example, growth-hormone therapy for achondroplasia and hypochondroplasia, and bisphosphonate therapy for osteogenesis imperfecta. In addition, enzyme replacement therapy for hypophosphatasia is now on clinical trial.

  12. [Analysis of clinical pathway in changing and disabling neurological diseases].

    PubMed

    Cordesse, V; Jametal, T; Guy, C; Lefebvre, S; Roussel, M; Ruggeri, J; Schimmel, P; Holstein, J; Meininger, V

    2013-01-01

    Neurological diseases are characterized by the complexity of care and by a constant and changing disability. More and more frequently, their impact on the clinical pathway remains unknown. Seven postgraduate rehabilitation students (Master coordination du handicap, université Pierre-et-Marie-Curie, Paris) reconstructed the clinical pathway of 123 patients with various neurological diseases: multiple sclerosis, Alzheimer disease, amyotrophic lateral sclerosis, spinal trauma, Parkinson disease and brain tumors. There was a significant correlation between disease duration and the number of specialists involved in care, the number of prescribed drugs and the number of short-term hospitalizations; there was no correlation with age. This result suggests that with time an increasing number of complications related to the initial neurological disease developed. Hospitalization in rehabilitation units was highly correlated with the degree of disability and also with the help received by the patients during the course of their disease. This result suggests that these hospitalizations were a direct consequence of burn out among relatives. General practitioners (GP) were highly involved only during the initial part of the pathway, and their involvement rapidly declined thereafter, suggesting a probable relation with the specificities and the complexity of care for neurological diseases which induces a progressive transfer of responsibilities from the GP to the hospital. Social care was always incomplete and occurred too late during the course of the disease. The feeling by the patients that their care pathway was chaotic was highly correlated with the quality of the information given to the patient at the time of the announcement of their disease. This study confirms that cares for neurological diseases is highly specific and that expert centers and coordination networks are in a key position to ensure an efficient care pathway.

  13. [Clinical approach to a patient with rheumatoid arthritis].

    PubMed

    Perković, Dijana; Kaliterna, DuSanka Martinović; Krstulović, Daniela Marasović; Bozić, Ivona; Borić, Katarina; Radić, Mislav

    2014-01-01

    Rheumatoid arthritis (RA) is chronic inflammatory rheumatic disease which leads to joint damage, functional im- pairment and reduced quality of life. The disease should be recognized early when there is a "window of oppor- tunity" to apply adequate treatment which may prevent structural damage. As clinical presentation of RA is not always typical, great knowledge and clinical experience, including collaboration of rheumatologist, general practi- tioner and patient, are required. The treatment should be started immediately upon the diagnosis, while the choice of modality of treatment depends on the rheumatologist in accordance with the patient. The RA patients with the higher risk of aggressive disease need to be recognized because they require more aggressive treatment from the start. The goal of the treatment is remission or at least low disease activity. Current treatment of RA includes disease modifying antirheumatic drugs (DMARDs) synthetics and biologics, nonsteroidal antirheumatic drugs (NSAIDs), glucocorticoids, analgesics, and rarely cytostatics. The course of disease is usually fluctuating with the exchange of relapses and remissions. Recognition of the relapsing patient on time enables treatment intensification or modifications in treatment scheme. Special issue in RA represents glucocorticoid-induced osteoporosis (GIO) which should be prevented by usage of calcium and vitamin D supplements and treated by antiresorptive or osteoanabolic agents. Besides the treatment of the primary disease, the care of RA patients should consider comorbidities, side effects of treatment, complications of disease, and psychosocial aspects of chronic disease. PMID:25427391

  14. [Clinical approach to a patient with rheumatoid arthritis].

    PubMed

    Perković, Dijana; Kaliterna, DuSanka Martinović; Krstulović, Daniela Marasović; Bozić, Ivona; Borić, Katarina; Radić, Mislav

    2014-01-01

    Rheumatoid arthritis (RA) is chronic inflammatory rheumatic disease which leads to joint damage, functional im- pairment and reduced quality of life. The disease should be recognized early when there is a "window of oppor- tunity" to apply adequate treatment which may prevent structural damage. As clinical presentation of RA is not always typical, great knowledge and clinical experience, including collaboration of rheumatologist, general practi- tioner and patient, are required. The treatment should be started immediately upon the diagnosis, while the choice of modality of treatment depends on the rheumatologist in accordance with the patient. The RA patients with the higher risk of aggressive disease need to be recognized because they require more aggressive treatment from the start. The goal of the treatment is remission or at least low disease activity. Current treatment of RA includes disease modifying antirheumatic drugs (DMARDs) synthetics and biologics, nonsteroidal antirheumatic drugs (NSAIDs), glucocorticoids, analgesics, and rarely cytostatics. The course of disease is usually fluctuating with the exchange of relapses and remissions. Recognition of the relapsing patient on time enables treatment intensification or modifications in treatment scheme. Special issue in RA represents glucocorticoid-induced osteoporosis (GIO) which should be prevented by usage of calcium and vitamin D supplements and treated by antiresorptive or osteoanabolic agents. Besides the treatment of the primary disease, the care of RA patients should consider comorbidities, side effects of treatment, complications of disease, and psychosocial aspects of chronic disease. PMID:25507641

  15. Risk and clinical predictors of osteoporotic fracture in East Asian patients with chronic obstructive pulmonary disease: a population-based cohort study

    PubMed Central

    Lee, Ping-Hsueh; Chou, Po-Liang; Ku, Ming-Chang; Chen, Yu-Ching

    2016-01-01

    Introduction Osteoporosis is becoming an impending epidemic in the Asia-Pacific region. The association between risk of osteoporotic fracture (OTPF) and chronic obstructive pulmonary disease (COPD) in East Asian patients is yet to be fully examined. We conducted a nationwide population-based retrospective cohort study of 98,700 patients aged ≥50 years with or without COPD using a national administrative claims dataset. Materials and Methods The patients were divided into COPD and comparison groups comprising 19,740 and 78,960 patients, respectively. The groups were 1 to 4 matched for age, gender, index date, diabetes mellitus, pre-existing osteoporosis and chronic kidney disease. Information such as the geographic area where southern part represented more sunshine exposure, smoking-related diagnoses, alcohol use disorder, whether there was regular use of inhaled corticosteroids and oral corticosteroids, vitamin D prescriptions, Charlson-Deyo comorbidity index score, and other relevant medical comorbidities were extracted for analysis. They were followed up until OTPF or the end of the year 2013. The outcome measure was an osteoporotic vertebral fracture and other long-bone fractures. A multivariate Cox model was constructed to derive adjusted hazard ratios (aHR) for OTPF with corresponding 95% confidence intervals (CI) after controlling for age, sex, insurance premium category, vitamin D prescription, osteoporosis, and coronary heart disease (CHD). Kaplan–Meier curves of the probability of OTPF-free survival for each cohort were compared using the log-rank test. Patients with OTPF during the first follow-up year were excluded from the overall risk calculation. Contributing factors to the increased risk of OTPF in COPD patients were examined in a sensitivity analysis. Results After a total follow-up of 68,743 patient-years for the COPD group and 278,051 patient-years for the matched comparison group, the HR for OTPF was 1.24 (95% CI [1.02–1.51]; P = 0

  16. Kimura's disease in Malay patients.

    PubMed

    Shahrul, H; Baharudin, A; Effat, O

    2007-08-01

    Kimura's Disease (KD) is an uncommon, chronic inflammatory disorder of unknown etiology which is endemic in Orientals. It is characterized by painless, large solitary or multiple nodules in subcutis of head and neck region or the major salivary glands, associated with regional lymphadenopathy, blood eosinophilia and elevated IgE levels. Its treatment ranging from conservative observation in asymptomatic patient to surgical resection of the mass, corticotherapy and irradiation therapy for the symptomatic ones.

  17. Gaucher disease in Iraqi children (Clinical, diagnostic & therapeutic aspects)

    PubMed Central

    Thejeal, Rabab Farhan; Kadhum, Ausama Jamal

    2016-01-01

    Background and Objective: Gaucher disease is the most common inherited lysosomal storage disorder. It is a multi organ disease affecting bone marrow, liver, spleen, lungs, and other organs contributes to pancytopenia and massive hepatosplenomegaly. This study aimed to spotlight on clinical and laboratory characteristics of children with Gaucher disease to raise awareness among physicians about the disease and to evaluate the outcome of enzyme replacement therapy (ERT). Methods: Clinical courses were reviewed in 30 patients with age (2-22 years) with Gaucher disease. After starting (ERT), assessment of response included serial measurements of hematological parameters, spleen and liver sizes, symptoms and signs of bone disease, growth and severity scores were also evaluated. Results: The most presenting age group was (1 – 5) years (60%). Abdominal distension was the most common presenting symptom, Splenomegaly presented in all of the patients. A significant response to ERT was observed, weight and height increased, both liver and spleen sizes decreased. Hemoglobin level normalizedin (67%) of the anemic patients, platelet count normalized in (53.8%)after 6 months from (ERT), the mean of severity scoring index decreased with ERT from (10.2±5.8) to (7.8±5.7) after one year of treatment. Conclusion: Using ERT was safe and effective in the reversal of hematological complications and organomegaly in most of the patients. PMID:27182231

  18. Clinical Audits in Outpatient Clinics for Chronic Obstructive Pulmonary Disease: Methodological Considerations and Workflow

    PubMed Central

    López-Campos, Jose Luis; Abad Arranz, María; Calero Acuña, Carmen; Romero Valero, Fernando; Ayerbe García, Ruth; Hidalgo Molina, Antonio; Aguilar Pérez-Grovas, Ricardo Ismael; García Gil, Francisco; Casas Maldonado, Francisco; Caballero Ballesteros, Laura; Sánchez Palop, María; Pérez-Tejero, Dolores; Segado, Alejandro; Calvo Bonachera, Jose; Hernández Sierra, Bárbara; Doménech, Adolfo; Arroyo Varela, Macarena; González Vargas, Francisco; Cruz Rueda, Juan Jose

    2015-01-01

    Objectives Previous clinical audits for chronic obstructive pulmonary disease (COPD) have provided valuable information on the clinical care delivered to patients admitted to medical wards because of COPD exacerbations. However, clinical audits of COPD in an outpatient setting are scarce and no methodological guidelines are currently available. Based on our previous experience, herein we describe a clinical audit for COPD patients in specialized outpatient clinics with the overall goal of establishing a potential methodological workflow. Methods A pilot clinical audit of COPD patients referred to respiratory outpatient clinics in the region of Andalusia, Spain (over 8 million inhabitants), was performed. The audit took place between October 2013 and September 2014, and 10 centers (20% of all public hospitals) were invited to participate. Cases with an established diagnosis of COPD based on risk factors, clinical symptoms, and a post-bronchodilator FEV1/FVC ratio of less than 0.70 were deemed eligible. The usefulness of formally scheduled regular follow-up visits was assessed. Two different databases (resources and clinical database) were constructed. Assessments were planned over a year divided by 4 three-month periods, with the goal of determining seasonal-related changes. Exacerbations and survival served as the main endpoints. Conclusions This paper describes a methodological framework for conducting a clinical audit of COPD patients in an outpatient setting. Results from such audits can guide health information systems development and implementation in real-world settings. PMID:26544556

  19. Clinical and laboratory aspects of Legionnaire's disease.

    PubMed

    Strampfer, M J; Cunha, B A

    1987-12-01

    Legionnaires' disease is an illness with protean manifestations that are due to infection with Legionella pneumophila. It occurs both in epidemic and sporadic form and usually presents as an atypical pneumonia. Relative bradycardia, abnormal liver function test results, and a patient presenting with an atypical pneumonia should alert the clinician to the possibility of Legionella. The presence of systemic involvement, specifically neurological, gastrointestinal, and renal abnormalities, should further suggest the diagnosis. Patients may demonstrate multiple extrapulmonary manifestations of legionnaires' disease, sometimes without pneumonia. Several methods are available to aid the clinician in making the diagnosis of legionnaires' disease, and the use of all tests will increase the overall sensitivity.

  20. Clinical Evaluation of MP4CO: A Phase 1b Escalating-Dose, Safety and Tolerability Study in Stable Adult Patients with Sickle Cell Disease.

    PubMed

    Keipert, Peter E

    2016-01-01

    MP4CO, developed by Sangart Inc. (San Diego, CA), is a pegylated human hemoglobin-based carbon monoxide (CO) delivery agent and oxygen therapeutic that has shown potential to prevent and reverse red cell sickling. A double blind, comparator controlled, dose-escalation, Phase 1b study was conducted to assess the safety of MP4CO. Adult sickle cell patients with HbSS or S/β(0) Thal genotype who were not experiencing a painful crisis were randomized to receive either MP4CO or normal saline (NS) in a sequential series of six escalating dose cohorts (A-F). In each cohort, three patients received MP4CO (Treatment group) and one patient received NS (Controls). Single IV doses ranged from 15 mg/kg/dose (0.35 mL/kg infusion) to 172 mg/kg/dose (4 mL/kg infusion). Two cohorts received fractionated doses of 172 or 344 mg/kg (4-8 mL/kg, given as two IV infusions, 24 h apart). Overall, 16/24 patients (66.7 %) reported mild to moderate adverse events (AEs); with 13/18 (72 %) in MP4CO group vs. 3/6 (50 %) in NS Controls. No serious adverse events (SAEs) were experienced and no deaths occurred. Most common AEs (reported by >2 patients) included headaches (mild and transient), fatigue and rash at the application site of the Holter electrodes. No treatment-emergent abnormalities in clinical lab values were noted. Vital signs, ECG readings, and pulmonary pressures remained within normal limits. The maximum increase in blood CO-Hb level was ~2 %, which returned to pre-dosing levels within 8 h after dosing. The mean increase in free plasma Hb (an index of MP4CO dose) ranged from 0.20 to 0.35 g/dL in the two highest dose cohorts, with no significant change in total whole blood hemoglobin level. There was no symptomatic or clinical evidence of renal dysfunction in either group based on serum creatinine and urinary albumin results. Two patients had elevated renal biomarkers (β2M and NAG) at Hour 72, which normalized at follow-up visits. Both patients had documented intercurrent

  1. The CopenHeartSF trial—comprehensive sexual rehabilitation programme for male patients with implantable cardioverter defibrillator or ischaemic heart disease and impaired sexual function: protocol of a randomised clinical trial

    PubMed Central

    Johansen, Pernille Palm; Zwisler, Ann-Dorthe; Hastrup-Svendsen, Jesper; Frederiksen, Marianne; Lindschou, Jane; Winkel, Per; Gluud, Christian; Giraldi, Annamaria; Steinke, Elaine; Jaarsma, Tiny; Berg, Selina Kikkenborg

    2013-01-01

    Introduction Sexuality is an important part of people’s physical and mental health. Patients with heart disease often suffer from sexual dysfunction. Sexual dysfunction has a negative impact on quality of life and well-being in persons with heart disease, and sexual dysfunction is associated with anxiety and depression. Treatment and care possibilities seem to be lacking. Studies indicate that non-pharmacological interventions such as exercise training and psychoeducation possess the potential of reducing sexual dysfunction in patients with heart disease. The CopenHeartSF trial will investigate the effect of a comprehensive sexual rehabilitation programme versus usual care. Methods and analysis CopenHeartSF is an investigator-initiated randomised clinical superiority trial with blinded outcome assessment, with 1:1 central randomisation to sexual rehabilitation plus usual care versus usual care alone. Based on sample size calculations, 154 male patients with impaired sexual function due to implantable cardioverter defibrillator or ischaemic heart disease will be included from two university hospitals in Denmark. All patients receive usual care and patients allocated to the experimental intervention group follow a 12-week sexual rehabilitation programme consisting of an individualised exercise programme and psychoeducative consultation with a specially trained nurse. The primary outcome is sexual function measured by the International Index of Erectile Function. The secondary outcome measure is psychosocial adjustment to illness by the Psychosocial Adjustment to Illness Scale, sexual domain. A number of explorative analyses will also be conducted. Ethics and dissemination CopenHeartSF is approved by the regional ethics committee (no H-4-2012-168) and the Danish Data Protection Agency (no 2007-58-0015) and is performed in accordance with good clinical practice and the Declaration of Helsinki in its latest form. Registration Clinicaltrials.gov identifier: NCT01796353

  2. Extracorporeal Cardiac Shock Wave Therapy Ameliorates Clinical Symptoms and Improves Regional Myocardial Blood Flow in a Patient with Severe Coronary Artery Disease and Refractory Angina

    PubMed Central

    Prinz, Christian; Lindner, Oliver; Bitter, Thomas; Hering, Detlef; Burchert, Wolfgang; Horstkotte, Dieter; Faber, Lothar

    2009-01-01

    Different therapeutic options are being used for chronic coronary artery disease (CAD). We report about a 51-year-old female with CAD and refractory angina pectoris despite maximally tolerated medical therapy and after both percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG). The patient received cardiac shock wave therapy (CSWT) over a period of 6 month. There was no arrhythmia during or after treatment; enzyme levels were normal at all times. PET imaging showed a substantial improvement of myocardial stress perfusion. Since the patient reported that she now was fully capable to deal with her everyday life, further treatment options were postponed. Our case report suggests that ultrasound-guided CSWT is able to improve symptoms and perfusion in ischemic myocardium. PMID:19724656

  3. Rethinking dry eye disease: a perspective on clinical implications.

    PubMed

    Bron, Anthony J; Tomlinson, Alan; Foulks, Gary N; Pepose, Jay S; Baudouin, Christophe; Geerling, Gerd; Nichols, Kelly K; Lemp, Michael A

    2014-04-01

    Publication of the DEWS report in 2007 established the state of the science of dry eye disease (DED). Since that time, new evidence suggests that a rethinking of traditional concepts of dry eye disease is in order. Specifically, new evidence on the epidemiology of the disease, as well as strategies for diagnosis, have changed the understanding of DED, which is a heterogeneous disease associated with considerable variability in presentation. These advances, along with implications for clinical care, are summarized herein. The most widely used signs of DED are poorly correlated with each other and with symptoms. While symptoms are thought to be characteristic of DED, recent studies have shown that less than 60% of subjects with other objective evidence of DED are symptomatic. Thus the use of symptoms alone in diagnosis will likely result in missing a significant percentage of DED patients, particularly with early/mild disease. This could have considerable impact in patients undergoing cataract or refractive surgery as patients with DED have less than optimal visual results. The most widely used objective signs for diagnosing DED all show greater variability between eyes and in the same eye over time compared with normal subjects. This variability is thought to be a manifestation of tear film instability which results in rapid breakup of the tearfilm between blinks and is an identifier of patients with DED. This feature emphasizes the bilateral nature of the disease in most subjects not suffering from unilateral lid or other unilateral destabilizing surface disorders. Instability of the composition of the tears also occurs in dry eye disease and shows the same variance between eyes. Finally, elevated tear osmolarity has been reported to be a global marker (present in both subtypes of the disease- aqueous-deficient dry eye and evaporative dry eye). Clinically, osmolarity has been shown to be the best single metric for diagnosis of DED and is directly related to

  4. Rethinking dry eye disease: a perspective on clinical implications.

    PubMed

    Bron, Anthony J; Tomlinson, Alan; Foulks, Gary N; Pepose, Jay S; Baudouin, Christophe; Geerling, Gerd; Nichols, Kelly K; Lemp, Michael A

    2014-04-01

    Publication of the DEWS report in 2007 established the state of the science of dry eye disease (DED). Since that time, new evidence suggests that a rethinking of traditional concepts of dry eye disease is in order. Specifically, new evidence on the epidemiology of the disease, as well as strategies for diagnosis, have changed the understanding of DED, which is a heterogeneous disease associated with considerable variability in presentation. These advances, along with implications for clinical care, are summarized herein. The most widely used signs of DED are poorly correlated with each other and with symptoms. While symptoms are thought to be characteristic of DED, recent studies have shown that less than 60% of subjects with other objective evidence of DED are symptomatic. Thus the use of symptoms alone in diagnosis will likely result in missing a significant percentage of DED patients, particularly with early/mild disease. This could have considerable impact in patients undergoing cataract or refractive surgery as patients with DED have less than optimal visual results. The most widely used objective signs for diagnosing DED all show greater variability between eyes and in the same eye over time compared with normal subjects. This variability is thought to be a manifestation of tear film instability which results in rapid breakup of the tearfilm between blinks and is an identifier of patients with DED. This feature emphasizes the bilateral nature of the disease in most subjects not suffering from unilateral lid or other unilateral destabilizing surface disorders. Instability of the composition of the tears also occurs in dry eye disease and shows the same variance between eyes. Finally, elevated tear osmolarity has been reported to be a global marker (present in both subtypes of the disease- aqueous-deficient dry eye and evaporative dry eye). Clinically, osmolarity has been shown to be the best single metric for diagnosis of DED and is directly related to

  5. Challenges in clinical patient management.

    PubMed

    Aapro, Matti S

    2010-01-01

    The use of endocrine therapy in breast cancer represents one of the earliest molecular targeting strategies used in cancer treatment. Tamoxifen, a selective estrogen-receptor (ER) modulator, has been the standard of care for women with receptor-positive breast cancer for the last 30 years. Tamoxifen suppresses the estrogen-dependent growth of breast cancer cells by specifically targeting the ER. Because of estrogenic effects, tamoxifen does not increase the risk of osteoporosis, but it can lead to endometrial cancer and thromboembolism. The third-generation aromatase inhibitors (AIs) exert their tumor antiproliferative action by targeting an enzyme critical for estrogen biosynthesis. The AIs thus have a different mechanism of action than tamoxifen, and a different safety profile. The majority of adverse events (AEs) related to the AIs are mild to moderate. Most of these AEs are common to menopause and are predictable and manageable. This review looks at AI-associated side effects and current clinical management strategies, with a particular emphasis on managing bone health. Compliance with long-term therapy, strategies to improve adherence, and considerations in elderly patients with hormone-responsive breast cancer are also discussed.

  6. Infective endocarditis in patients with hepatic diseases.

    PubMed

    Seminari, E; De Silvestri, A; Ravasio, V; Ludovisi, S; Utili, R; Petrosillo, N; Castelli, F; Bassetti, M; Barbaro, F; Grossi, P; Barzaghi, N; Rizzi, M; Minoli, L

    2016-02-01

    Few data have been published regarding the epidemiology and outcome of infective endocarditis (IE) in patients with chronic hepatic disease (CHD). A retrospective analysis of the Studio Endocarditi Italiano (SEI) database was performed to evaluate the epidemiology and outcome of CHD+ patients compared with CHD- patients. The diagnosis of IE was defined in accordance with the modified Duke criteria. Echocardiography, diagnosis, and treatment procedures were in accordance with current clinical practice. Among the 1722 observed episodes of IE, 300 (17.4 %) occurred in CHD+ patients. The cause of CHD mainly consisted of chronic viral infection. Staphylococcus aureus was the most common bacterial species in CHD+ patients; the frequency of other bacterial species (S. epidermidis, streptococci, and enterococci) were comparable among the two groups. The percentage of patients undergoing surgery for IE was 38.9 in CHD+ patients versus 43.7 in CHD- patients (p = 0.06). Complications were more common among CHD+ patients (77 % versus 65.3 %, p < 0.001); embolization (43.3 % versus 26.1 %, p < 0.001) and congestive heart failure (42 % versus 34.1 %, p = 0.01) were more frequent among CHD+ patients. Mortality was comparable (12.5 % in CHD- and 15 % in CHD+ patients). At multivariable analysis, factors associated with hospital-associated mortality were having an infection sustained by S. aureus, a prosthetic valve, diabetes and a neoplasia, and CHD. Being an intravenous drug user (IVDU) was a protective factor and was associated with a reduced death risk. CHD is a factor worsening the prognosis in patients with IE, in particular in patients for whom cardiac surgery was required.

  7. Evaluation of a combined strategy directed towards health-care professionals and patients with chronic obstructive pulmonary disease (COPD): Information and health education feedback for improving clinical monitoring and quality-of-life

    PubMed Central

    2009-01-01

    Background Chronic obstructive pulmonary disease (COPD) is a health problem that is becoming increasingly attended-to in Primary Care (PC). However, there is a scarcity of health-care programs and studies exploring the implementation of Clinical Practice Guidelines (CPG). The principal objective of the present study is to evaluate the effectiveness of a combined strategy directed towards health-care professionals and patients to improve the grade of clinical control and the quality-of-life (QoL) of the patients via a feedback on their state-of-health. A training plan for the health-care professionals is based on CPG and health education. Method/Design Multi-centred, before-after, quasi experimental, prospective study involving an intervention group and a control group of individuals followed-up for 12 months. The patients receive attention from urban and semi-urban Primary Care Centres (PCC) within the administrative area of the Costa de Ponent (near Barcelona). All the pacients corresponding to the PCC of one sub-area were assigned to the intervention group and patients from the rest of sub-areas to the group control. The intervention includes providing data to the health-care professionals (clinician/nurse) derived from a clinical history and an interview. A course of training focused on aspects of CPG, motivational interview and health education (tobacco, inhalers, diet, physical exercise, physiotherapy). The sample random includes a total of 801 patients (≥ 40 years of age), recorded as having COPD, receiving attention in the PCC or at home, who have had at least one clinical visit, and who provided written informed consent to participation in the study. Data collected include socio-demographic characteristics, drug treatment, exacerbations and hospital admissions, evaluation of inhaler use, tobacco consumption and life-style and health-care resources consumed. The main endpoints are dyspnoea, according to the modified scale of the Medical Research Council

  8. Clostridium difficile: clinical disease and diagnosis.

    PubMed Central

    Knoop, F C; Owens, M; Crocker, I C

    1993-01-01

    Clostridium difficile is an opportunistic pathogen that causes a spectrum of disease ranging from antibiotic-associated diarrhea to pseudomembranous colitis. Although the disease was first described in 1893, the etiologic agent was not isolated and identified until 1978. Since clinical and pathological features of C. difficile-associated disease are not easily distinguished from those of other gastrointestinal diseases, including ulcerative colitis, chronic inflammatory bowel disease, and Crohn's disease, diagnostic methods have relied on either isolation and identification of the microorganism or direct detection of bacterial antigens or toxins in stool specimens. The current review focuses on the sensitivity, specificity, and practical use of several diagnostic tests, including methods for culture of the etiologic agent, cellular cytotoxicity assays, latex agglutination tests, enzyme immunoassay systems, counterimmunoelectrophoresis, fluorescent-antibody assays, and polymerase chain reactions. PMID:8358706

  9. Clinical differences in patients with Alzheimer's disease according to the presence or absence of anosognosia: implications for perceived quality of life.

    PubMed

    Conde-Sala, Josep L; Reñé-Ramírez, Ramón; Turró-Garriga, Oriol; Gascón-Bayarri, Jordi; Juncadella-Puig, Montserrat; Moreno-Cordón, Laura; Viñas-Diez, Vanesa; Garre-Olmo, Josep

    2013-01-01

    This study aimed to determine the factors that predict anosognosia in patients with Alzheimer's disease (AD) and to examine the effect of anosognosia on patient and caregiver perceptions of the patient's quality of life (QoL-p), using a cross-sectional design with 164 patients and their caregivers. Instruments of measurement included Anosognosia Questionnaire-Dementia, Geriatric Depression Scale, Quality of Life in AD (QoL-AD), Disability Assessment for Dementia, Neuropsychiatric Inventory, and the Global Deterioration Scale (GDS). A binary logistic regression analysis was performed to identify the factors that predict anosognosia, while a linear regression analysis was conducted to determine the factors associated with QoL-AD. The degree of anosognosia increased in line with GDS stage (F (2,161) = 41.3, p < 0.001). In the binary regression analysis, the variables that predicted anosognosia were more neuropsychiatric symptoms (OR = 1.11, 95% CI: 1.06-1.17, p < 0.001), deficits in ADL (OR = 0.88, 95% CI: 0.83-0.94, p < 0.001), less depression (OR = 0.66, 95% CI: 0.54-0.82, p < 0.001), and older age (OR = 1.08, 95% CI: 1.00-1.15, p = 0.027). With regards to QoL-p, the multiple linear regression analysis for patients (r2 = 0.486) showed that less depression (β = -0.52, p < 0.001) and greater anosognosia (β = 0.40, p < 0.001) explained 33% and 10% of the variance in QoL-AD, respectively. Greater anosognosia was associated with better perceived QoL-p, especially in advanced GDS stages. Anosognosia was associated with greater caregiver burden and a greater discrepancy between patient and caregiver ratings of QoL-p.

  10. [Lyme disease--clinical manifestations and treatment].

    PubMed

    Stock, Ingo

    2016-05-01

    Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings.

  11. [Lyme disease--clinical manifestations and treatment].

    PubMed

    Stock, Ingo

    2016-05-01

    Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings. PMID:27348896

  12. [Brief psychotherapy in clinical medicine patients].

    PubMed

    Knobel, M

    1992-01-01

    The criteria that "illness is biographical crisis od the individual" and that the only medicine is "personal medicine" is stressed. Clinical medicine, which covers medicine in its entirety, demands conceptual and doctrinal reaffirmations so that gradually the patient can come to be dealt with as a human being fron a holistic point of view, which commences with his complaint and consultation, continues with the interview and semiology, to finish with the diagnosis and therapy which, although in some cases it may be surgical, is still medical and integral. All the steps mentioned are bio-socio-cultural thus, whether in the practice of general clinical medicine or in the most specialized and technologically sophisticated clinical medicine, the animist component is not lacking and demands a minimum degree of "psychosomatic" Knowledge. The use of a psychotherapeutic technique is proposed which, while based on the psychoanalysis theory, is distanced technically from it as a "psychotherapy on limited time and goals", which abbreviates the disease, and is projected not as the "focus" of therapeutic work, but as a re-evaluation of the "life style" of each individual, and tends to help to develop a "project for life" suited to the possible personal, familiar and social well-being of the "patient". Technically speaking, this modality of brief psychotherapy is based on the nonuse of transferential interpretations, on impeding the regression od the patient, on facilitating a cognitice-affective development of his conflicts and thus obtain an internal object mutation which allows the transformation of the "past" into true history, and the "present" into vital perspectives. This technique is within reach of every health professional.

  13. Coronary artery disease in patients with cerebrovascular disease: a prospective study

    SciTech Connect

    Rokey, R.; Rolak, L.A.; Harati, Y.; Kutka, N.; Verani, M.S.

    1984-07-01

    Coronary artery disease is the cause of death in most patients who have transient ischemic attacks or stroke. Evaluation for this condition is not routinely performed in such patients, and no prospective studies have been reported. We prospectively examined 50 consecutive patients with transient ischemic attacks or mild stroke to determine the prevalence and importance of coronary artery disease. All patients were examined by a cardiologist and underwent both exercise thallium-201 scintigraphy and exercise radionuclide ventriculography. Sixteen patients were suspected to have coronary artery disease on the basis of clinical evaluation. In 15 of these the was confirmed by the nuclear scans. The remaining 34 patients had no clinical evidence of heart disease, yet 14 had abnormal cardiac scans. Twenty of 22 patients with abnormal scans who underwent cardiac catheterization had significant coronary artery disease or a cardiomyopathy. The discovery of heart disease altered clinical management in 13 patients. Overall, 29 of 50 patients had significant coronary artery disease, compared with a 7% prevalence of the condition in other patients of similar age at the same institution.

  14. Ticagrelor for the treatment of atherosclerotic disease: insights from the PARTHENON clinical development program.

    PubMed

    Held, Peter; Himmelmann, Anders; Ditmarsch, Marc

    2016-07-01

    Ticagrelor (P2Y12 receptor