Liver involvement in Gaucher disease - Review and clinical approach.

Science.gov (United States)

Adar, Tomer; Ilan, Yaron; Elstein, Deborah; Zimran, Ari

2018-02-01

Gaucher disease (GD), one of the most prevalent lysosomal storage diseases, is associated with glucocerebroside accumulation in cells of the monocyte-macrophage system in various organs, including the liver. Evaluating and managing liver disease in patients with Gaucher disease may be challenging. While hepatic involvement is common in Gaucher disease, its severity, and clinical significance span a wide spectrum, ranging from sub-clinical involvement to liver cirrhosis with its associated complications including portal hypertension. Apart from liver involvement in Gaucher disease, patients with may also suffer from other comorbidities involving the liver. That Gaucher disease itself can mimic hepatic lesions, affect laboratory tests used to characterize liver disease, and may be associated with non-cirrhotic portal hypertension, complicates the diagnostic approach even more. Better understanding of liver involvement in Gaucher disease can spare patients unnecessary invasive testing, and assist physicians in decision making when evaluating patients with Gaucher disease suspected for significant liver disease. This review describes the various clinical manifestations, laboratory and imaging abnormalities that may be encountered when following patients with Gaucher disease for liver involvement. The mechanism for liver disease are discussed, as well as the possible hepato-protective effect of glucocerebroside, and the a diagnostic and treatment approaches. Copyright © 2016 Elsevier Inc. All rights reserved.

An evaluation of neuropsychiatric symptoms in Parkinson's disease ...

African Journals Online (AJOL)

Hoehn-Yahr (HY) scale was used to evaluate the clinical stages of PD and Unified Parkinson's Disease Rating Scale (UPDRS) was used to evaluate the severity of clinical signs. Cognitive functions were evaluated by Mini-Mental State Examination (MMSE) and neuropsychiatric findings were evaluated by Beck Depression ...

Unexpected MRI findings in clinically suspected Legg-Calve-Perthes disease

Energy Technology Data Exchange (ETDEWEB)

Lobert, Philip F.; Dillman, Jonathan R.; Strouse, Peter J.; Hernandez, Ramiro J. [University of Michigan Health System, Department of Radiology, Section of Pediatric Radiology, C.S. Mott Children' s Hospital/F3503, Ann Arbor, MI (United States)

2011-03-15

In the setting of clinically suspected Legg-Calve-Perthes (LCP) disease and negative/equivocal radiographs, contrast-enhanced MRI can be performed to confirm the diagnosis. To determine the frequency of unexpected causes of hip pain as identified by MRI in children with clinically suspected LCP disease and negative/equivocal radiographs. All pediatric contrast-enhanced MRI examinations of the pelvis and hips performed between January 2000 and February 2009 to evaluate for possible LCP disease in the setting of negative/equivocal radiographs were identified. MRI examinations performed to evaluate for secondary avascular necrosis were excluded. Imaging reports were retrospectively reviewed for unexpected clinically important causes of hip pain. Thirty-six pediatric patients underwent contrast-enhanced MRI examinations for clinically suspected LCP disease in the setting of negative/equivocal radiographs. Twenty-two (61%) imaging studies were normal, while four (11%) imaging studies demonstrated findings consistent with LCP disease. Ten (28%) imaging studies revealed unexpected clinically important causes of hip pain, including nonspecific unilateral joint effusion and synovitis (n = 7, juvenile chronic arthritis was eventually diagnosed in 3 patients), sacral fracture (n = 1), apophyseal injury (n = 1), and femoral head subluxation (n = 1). MRI frequently reveals unexpected clinically important causes of hip pain in children with suspected LCP disease and negative/equivocal radiographs. (orig.)

Reporting and evaluation of HIV-related clinical endpoints in two multicenter international clinical trials

DEFF Research Database (Denmark)

Lifson, A; Rhame, F; Bellosa, W

2006-01-01

adjudication between reviewers before diagnostic certainty was assigned. CONCLUSION: Important requirements for HIV trials using clinical endpoints include objective definitions of "confirmed" and "probable," a formal reporting process with adequate information and supporting source documentation, evaluation......PURPOSE: The processes for reporting and review of progression of HIV disease clinical endpoints are described for two large phase III international clinical trials. METHOD: SILCAAT and ESPRIT are multicenter randomized HIV trials evaluating the impact of interleukin-2 on disease progression...... and death in HIV-infected patients receiving antiretroviral therapy. We report definitions used for HIV progression of disease endpoints, procedures for site reporting of such events, processes for independent review of reported events by an Endpoint Review Committee (ERC), and the procedure...

Clinical evaluation of Morgellons disease in a cohort of North American patients.

Science.gov (United States)

Fesler, Melissa C; Middelveen, Marianne J; Stricker, Raphael B

2018-04-23

Morgellons disease (MD) is a dermatological condition characterized by aberrant production of keratin and collagen fibers in skin. Although infection with Borrelia burgdorferi , the causative agent of Lyme disease (LD), has been associated with MD, relatively few studies have hitherto provided epidemiological evidence regarding this association. A cohort of 1000 seropositive North American LD patients was evaluated for the presence of MD. Patients were diagnosed with MD based on detection of microscopic fibers in skin lesions or under unbroken skin. Demographic and clinical features of MD patients were analyzed, and laboratory testing for tickborne coinfections and other infectious agents, was performed. Subjective and objective features of MD were analyzed using statistical methods. Of 1000 seropositive LD patients, 60 (6%) were diagnosed with MD. Of these 60 patients, 75% were female and 78% presented in the late disseminated stage of MD. All 60 patients (100%) were seropositive for B. burgdorferi infection. Tickborne coinfections in these patients included Babesia spp (62%), Bartonella and Rickettsia (25% each), Ehrlichia (15%) and Anaplasma (10%). Helicobacter pylori was detected in 12% of MD patients. In all, 77% of MD patients had one or more coinfections. This study confirms recent findings that MD occurs in a limited subset of LD patients. The clinical and genetic determinants of MD in LD patients require further study.

Celiac disease in Saudi children. Evaluation of clinical features and diagnosis

Directory of Open Access Journals (Sweden)

Anjum Saeed

2017-09-01

Full Text Available Objectives: To characterize the clinical presentations and diagnosis including serological tests and histopathological findings in children with celiac disease. Methods: All children (less than 18 years with confirmed celiac disease diagnosed over a 6 year period at a private tertiary care health care center in Riyadh, Saudi Arabia were studied retrospectively. Information collected included demographics, clinical presentation and diagnostic modalities with serology and small intestinal histology reported by Marsh grading. Results: A total of 59 children had confirmed celiac disease. Thirty (50.8% were male. Median age was 8 years (range 1 to 16 years. The mean duration of symptoms before diagnosis was 2.3 (±1.5 years. Classical disease was present only in 30.5%, whereas 69.5% had either non-classical presentations or belonged to high risk groups for celiac disease such as those with type-1 diabetes, autoimmune thyroiditis, Down syndrome and siblings. Failure to thrive was the most common presentation followed by short stature, abdominal pain and chronic diarrhea. Anti-tissue transglutaminase antibody was positive in 91.5%, and titers were no different between those with classical and non-classical disease. All had Marsh-graded biopsy findings consistent with celiac disease. Conclusion: Children with celiac disease usually present with non-classical features. A high index of suspicion needs to be maintained to consider this disorder in the diagnostic workup of pediatric patients. High risk group should be screened early to avoid complications associated with untreated celiac disease.

Histologic scoring indices for evaluation of disease activity in Crohn's disease

NARCIS (Netherlands)

Novak, Gregor; Parker, Claire E.; Pai, Rish K.; Macdonald, John K.; Feagan, Brian G.; Sandborn, William J.; D'Haens, Geert; Jairath, Vipul; Khanna, Reena

2017-01-01

Histologic assessment of mucosal disease activity has been increasingly used in clinical trials of treatment for Crohn's disease. However, the operating properties of the currently existing histologic scoring indices remain unclear. A systematic review was undertaken to evaluate the development and

Clinical trial network for the promotion of clinical research for rare diseases in Japan: muscular dystrophy clinical trial network.

Science.gov (United States)

Shimizu, Reiko; Ogata, Katsuhisa; Tamaura, Akemi; Kimura, En; Ohata, Maki; Takeshita, Eri; Nakamura, Harumasa; Takeda, Shin'ichi; Komaki, Hirofumi

2016-07-11

Duchenne muscular dystrophy (DMD) is the most commonly inherited neuromuscular disease. Therapeutic agents for the treatment of rare disease, namely "orphan drugs", have recently drawn the attention of researchers and pharmaceutical companies. To ensure the successful conduction of clinical trials to evaluate novel treatments for patients with rare diseases, an appropriate infrastructure is needed. One of the effective solutions for the lack of infrastructure is to establish a network of rare diseases. To accomplish the conduction of clinical trials in Japan, the Muscular dystrophy clinical trial network (MDCTN) was established by the clinical research group for muscular dystrophy, including the National Center of Neurology and Psychiatry, as well as national and university hospitals, all which have a long-standing history of research cooperation. Thirty-one medical institutions (17 national hospital organizations, 10 university hospitals, 1 national center, 2 public hospitals, and 1 private hospital) belong to this network and collaborate to facilitate clinical trials. The Care and Treatment Site Registry (CTSR) calculates and reports the proportion of patients with neuromuscular diseases in the cooperating sites. In total, there are 5,589 patients with neuromuscular diseases in Japan and the proportion of patients with each disease is as follows: DMD, 29 %; myotonic dystrophy type 1, 23 %; limb girdle muscular dystrophy, 11 %; Becker muscular dystrophy, 10 %. We work jointly to share updated health care information and standardized evaluations of clinical outcomes as well. The collaboration with the patient registry (CTSR), allows the MDCTN to recruit DMD participants with specific mutations and conditions, in a remarkably short period of time. Counting with a network that operates at a national level is important to address the corresponding national issues. Thus, our network will be able to contribute with international research activity, which can lead to

Development of criteria for evaluating clinical response in thyroid eye disease using a modified Delphi technique

DEFF Research Database (Denmark)

Douglas, Raymond S; Tsirbas, Angelo; Gordon, Mark

2009-01-01

OBJECTIVE: To identify components of a provisional clinical response index for thyroid eye disease using a modified Delphi technique. METHODS: The International Thyroid Eye Disease Society conducted a structured, 3-round Delphi exercise establishing consensus for a core set of measures for clinical...... parsed into 11 domains for the Delphi surveys. Eighty-four respondents participated in the Delphi 1 survey, providing 220 unique items. Ninety-two members (100% of the respondents from Delphi 1 plus 8 new participants) responded in Delphi 2 and rated the same 220 items. Sixty-four members (76......% of participants) rated 153 criteria in Delphi 3 (67 criteria were excluded because of redundancy). Criteria with a mean greater than 6 (1 = least appropriate to 9 = most appropriate) were further evaluated by the nominal group technique and provisional core measures were chosen. CONCLUSIONS: Using a Delphi...

Celiac disease in Saudi children. Evaluation of clinical features and diagnosis.

Science.gov (United States)

Saeed, Anjum; Assiri, Asaad; Assiri, Hebah; Ullah, Anhar; Rashid, Mohsin

2017-09-01

 Objectives: To characterize the clinical presentations and diagnosis including serological tests and histopathological findings in children with celiac disease. Methods: All children (less than 18 years) with confirmed celiac disease diagnosed over a 6 year period at a private tertiary care health care center in Riyadh,  Saudi Arabia were studied retrospectively. Information collected included demographics, clinical presentation and diagnostic modalities with serology and small intestinal histology reported by Marsh grading. Results: A total of 59 children had confirmed celiac disease. Thirty (50.8%) were male. Median age was 8 years (range 1 to 16 years). The mean duration of symptoms before diagnosis was 2.3 (±1.5) years. Classical disease was present only in 30.5%, whereas 69.5% had either non-classical presentations or belonged to high risk groups for celiac disease such as those with type-1 diabetes, autoimmune thyroiditis, Down syndrome and siblings. Failure to thrive was the most common presentation followed by short stature, abdominal pain and chronic diarrhea. Anti-tissue transglutaminase antibody was positive in 91.5%, and titers were no different between those with classical and non-classical disease. All had Marsh-graded biopsy findings consistent with celiac disease. Conclusion: Children with celiac disease usually present with non-classical features. A high index of suspicion needs to be maintained to consider this disorder in the diagnostic workup of pediatric patients. High risk group should be screened early to avoid complications associated with untreated celiac disease.

Clinical evaluation of chemokine and enzymatic biomarkers of Gaucher disease

NARCIS (Netherlands)

Deegan, Patrick B.; Moran, Mary Teresa; McFarlane, Ian; Schofield, J. Paul; Boot, Rolf G.; Aerts, Johannes M. F. G.; Cox, Timothy M.

2005-01-01

Purpose: Gaucher disease is an exemplary orphan disorder. Enzyme replacement therapy with imiglucerase is effective, but very expensive. To improve the assessment of severity of disease and responses to this costly treatment, we have evaluated several enzymatic biomarkers and a newly-described

Clinical evaluation of the 2nd generation radio-receptor assay for anti-thyrotropin receptor antibodies (TRAb) in Graves' disease

International Nuclear Information System (INIS)

Giovanella, L.; Ceriani, L.; Garancini, S.

2002-01-01

Full text: Detection of autoantibodies to the TSH receptor by radioreceptorial assays (RRA) is largely requested in clinical practice for the diagnosis of Graves' disease and its differentiation from diffuse thyroid autonomy. Additionally, TRAb measurement during antithyroid drug treatment can be useful to evaluate the risk of disease's relapse alter therapy discontinuation. Nevertheless, some patients affected by Graves' disease are TRAb negative when 1st generation assay is used. Recently a new RRA method for TRAb assay was developed by using human recombinant TSH-receptor and solid-phase technique. Aim of our work was the comparison between 1st and 2nd generation TRAb assays in Graves' disease patients and, particularly, the evaluation of 2nd generation test in a sub-group of patients affected by Graves' disease but with negative 1st generation TRAb assay. We evaluated the diagnostic performance of a newly developed 2nd generation TRAb assay (DYNOtest(r) TRAK human, BRAHMS Diagnostica GmbH, Germany) in 46 patients affected by Graves' disease with negative 1st generation TRAb assay (TRAK Assay(r), BRAHMS Diagnostica GmbH, Germany) . A control groups of 50 Graves' disease patients with positive 1st generation TRAb assay, 50 patients affected by Hashimoto's thyroiditis and 50 patients affected by nodular goiter were also examined. 41 out of 46 patients affected by Graves' disease with negative 1st generation TRAb assay showed a positive 2nd generation test. The overall sensitivity of the 2nd generation test was significantly improved respect the 1st generation assay in Graves' disease patients (χ 2 = 22.5, p<0.0001). 1 and 3 out of 50 patients affected by Hashimoto's thyroiditis were positive by 1st and 2nd generation TRAB assay, respectively. All these patients showed primary hypothyroidism. No differences resulted in euthyroid Hashimoto's thyroiditis sub-group and in nodular goiter control group. The 2nd generation TRAB assay is clearly more sensitive than the 1

Clinical application of SPECT and PET in cerebrovascular disease

International Nuclear Information System (INIS)

Ra, Young Shin

2003-01-01

Single photon emission computed tomography(SPECT) and positron emission tomography(PET) are modern imaging techniques that allow for both qualitative are quantitative assessment of hemodynamic changes in cerebrovascular diseases. SPECT has been becoming an indispensable method to investigate regional cerebral blood flow because equipment and isotope are easily available in most general hospitals. Acetazolamide stress SPECT has also been proved to be useful to evaluate the cerebrovascular reserve of occlusive cerebrovascular diseases and to select surgical candidate. PET has gained wide spread clinical use in the evaluation of the hemodynamic and metabolic consequences of extracranial or intracranial arterial obstructive disease despite its complexity and limited availability. PET has been established as an invaluable tool in the pathophysilogy investigation of acute ischemic stroke. The potentials, limitations, and clinical applications of SPECT and PET in various cerebrovascular diseases will be discussed in this article with reviews of literatures

Clinical application of SPECT and PET in cerebrovascular disease

Energy Technology Data Exchange (ETDEWEB)

Ra, Young Shin [Ulsan University College of Medicine, Seoul (Korea, Republic of)

2003-02-01

Single photon emission computed tomography(SPECT) and positron emission tomography(PET) are modern imaging techniques that allow for both qualitative are quantitative assessment of hemodynamic changes in cerebrovascular diseases. SPECT has been becoming an indispensable method to investigate regional cerebral blood flow because equipment and isotope are easily available in most general hospitals. Acetazolamide stress SPECT has also been proved to be useful to evaluate the cerebrovascular reserve of occlusive cerebrovascular diseases and to select surgical candidate. PET has gained wide spread clinical use in the evaluation of the hemodynamic and metabolic consequences of extracranial or intracranial arterial obstructive disease despite its complexity and limited availability. PET has been established as an invaluable tool in the pathophysilogy investigation of acute ischemic stroke. The potentials, limitations, and clinical applications of SPECT and PET in various cerebrovascular diseases will be discussed in this article with reviews of literatures.

Clinical Utility of Serologic Testing for Celiac Disease in Asymptomatic Patients

Science.gov (United States)

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

Using whole disease modeling to inform resource allocation decisions: economic evaluation of a clinical guideline for colorectal cancer using a single model.

Science.gov (United States)

Tappenden, Paul; Chilcott, Jim; Brennan, Alan; Squires, Hazel; Glynne-Jones, Rob; Tappenden, Janine

2013-06-01

To assess the feasibility and value of simulating whole disease and treatment pathways within a single model to provide a common economic basis for informing resource allocation decisions. A patient-level simulation model was developed with the intention of being capable of evaluating multiple topics within National Institute for Health and Clinical Excellence's colorectal cancer clinical guideline. The model simulates disease and treatment pathways from preclinical disease through to detection, diagnosis, adjuvant/neoadjuvant treatments, follow-up, curative/palliative treatments for metastases, supportive care, and eventual death. The model parameters were informed by meta-analyses, randomized trials, observational studies, health utility studies, audit data, costing sources, and expert opinion. Unobservable natural history parameters were calibrated against external data using Bayesian Markov chain Monte Carlo methods. Economic analysis was undertaken using conventional cost-utility decision rules within each guideline topic and constrained maximization rules across multiple topics. Under usual processes for guideline development, piecewise economic modeling would have been used to evaluate between one and three topics. The Whole Disease Model was capable of evaluating 11 of 15 guideline topics, ranging from alternative diagnostic technologies through to treatments for metastatic disease. The constrained maximization analysis identified a configuration of colorectal services that is expected to maximize quality-adjusted life-year gains without exceeding current expenditure levels. This study indicates that Whole Disease Model development is feasible and can allow for the economic analysis of most interventions across a disease service within a consistent conceptual and mathematical infrastructure. This disease-level modeling approach may be of particular value in providing an economic basis to support other clinical guidelines. Copyright © 2013 International

Celiac disease: clinical observations

Directory of Open Access Journals (Sweden)

Yu. A. Emel’yanova

2016-01-01

Full Text Available Presented clinical cases of patients with a diagnosis of gluten enteropathy in treatment in the department of gastroenterology Regional Clinical Hospital. The case is of interest to doctors of different specialties for the differential diagnosis of anemia and malabsorption syndrome, demonstrate both the classic version, and atypical forms of the disease course. Diagnosis of celiac disease is based on three key positions: clinical findings, histology and serological markers. The clinical picture of celiac disease is characterized by pronounced polymorphism, by going beyond the a gastroenterological pathology. For screening of gluten sensitive celiac typically used an antibody to tissue transglutaminase. Morphological research of the mucous membrane of the small intestine is the determining criterion in the diagnosis of celiac disease. The use of specific gluten-free diet leads to the positive dynamics of the disease and improve the quality of life of patients.

Clinical Course of Acute Pancreatitis in Chronic Kidney Disease ...

African Journals Online (AJOL)

Introduction: The aim of this study was to assess the clinical course, etiology and complications of acute pancreatitis among chronic kidney disease (CKD) patients in a tertiary care renal center in Karachi. Methods: We retrospectively evaluated the clinical course of CKD patients who presented to our emergency room with ...

Ethical clinical translation of stem cell interventions for neurologic disease

DEFF Research Database (Denmark)

Cote, David J; Bredenoord, Annelien L; Smith, Timothy R

2017-01-01

The application of stem cell transplants in clinical practice has increased in frequency in recent years. Many of the stem cell transplants in neurologic diseases, including stroke, Parkinson disease, spinal cord injury, and demyelinating diseases, are unproven-they have not been tested...... in prospective, controlled clinical trials and have not become accepted therapies. Stem cell transplant procedures currently being carried out have therapeutic aims, but are frequently experimental and unregulated, and could potentially put patients at risk. In some cases, patients undergoing such operations...... are not included in a clinical trial, and do not provide genuinely informed consent. For these reasons and others, some current stem cell interventions for neurologic diseases are ethically dubious and could jeopardize progress in the field. We provide discussion points for the evaluation of new stem cell...

Advances in clinical application of optical coherence tomography in vitreomacular interface disease

Directory of Open Access Journals (Sweden)

Xiao-Li Xing

2013-08-01

Full Text Available Vitreous macular interface disease mainly includes vitreomacular traction syndrome, idiopathic macular epiretinal membrane and idiopathic macular hole. Optical coherence tomography(OCTas a new tool that provides high resolution biopsy cross section image non traumatic imaging inspection, has a unique high resolution, no damage characteristics, and hence clinical widely used, vitreous macular interface for clinical disease diagnosis, differential diagnosis and condition monitoring and quantitative evaluation, treatment options, etc provides important information and reference value. Vitreous macular interface disease in OCT image of anatomical morphology characteristics, improve the clinical on disease occurrence and development of knowledge. We reviewed the advances in the application of OCT in vitreomacular interface disease.

Performance Evaluation of an Automated ELISA System for Alzheimer's Disease Detection in Clinical Routine.

Science.gov (United States)

Chiasserini, Davide; Biscetti, Leonardo; Farotti, Lucia; Eusebi, Paolo; Salvadori, Nicola; Lisetti, Viviana; Baschieri, Francesca; Chipi, Elena; Frattini, Giulia; Stoops, Erik; Vanderstichele, Hugo; Calabresi, Paolo; Parnetti, Lucilla

2016-07-22

The variability of Alzheimer's disease (AD) cerebrospinal fluid (CSF) biomarkers undermines their full-fledged introduction into routine diagnostics and clinical trials. Automation may help to increase precision and decrease operator errors, eventually improving the diagnostic performance. Here we evaluated three new CSF immunoassays, EUROIMMUNtrademark amyloid-β 1-40 (Aβ1-40), amyloid-β 1-42 (Aβ1-42), and total tau (t-tau), in combination with automated analysis of the samples. The CSF biomarkers were measured in a cohort consisting of AD patients (n = 28), mild cognitive impairment (MCI, n = 77), and neurological controls (OND, n = 35). MCI patients were evaluated yearly and cognitive functions were assessed by Mini-Mental State Examination. The patients clinically diagnosed with AD and MCI were classified according to the CSF biomarkers profile following NIA-AA criteria and the Erlangen score. Technical evaluation of the immunoassays was performed together with the calculation of their diagnostic performance. Furthermore, the results for EUROIMMUN Aβ1-42 and t-tau were compared to standard immunoassay methods (INNOTESTtrademark). EUROIMMUN assays for Aβ1-42 and t-tau correlated with INNOTEST (r = 0.83, p ratio measured with EUROIMMUN was the best parameter for AD detection and improved the diagnostic accuracy of Aβ1-42 (area under the curve = 0.93). In MCI patients, the Aβ1-42/Aβ1-40 ratio was associated with cognitive decline and clinical progression to AD.The diagnostic performance of the EUROIMMUN assays with automation is comparable to other currently used methods. The variability of the method and the value of the Aβ1-42/Aβ1-40 ratio in AD diagnosis need to be validated in large multi-center studies.

Evaluating peer teaching about chronic disease.

Science.gov (United States)

Guenther, Sylvia; Shadbolt, Narelle; Roberts, Chris; Clark, Tyler

2014-12-01

The primary care areas of priority (PCAP) activity was developed to engage medical students in learning about chronic disease management in a clinical context from their peers. It is one of several summative assessment tasks that occur during a primary care community term rotation in a graduate-entry medical programme. We evaluated the acceptability and effectiveness of the PCAP activity as a combined teaching, learning and assessment innovation. Evaluation and assessment data from students who rotated through the four community term blocks during the 2011-2012 academic year was analysed using both qualitative and quantitative methods. PCAP peer teaching activity is an effective format for teaching about the management of chronic conditions in the community Analysis indicated that 89 per cent (n = 148/166) of responding students rated the PCAP as satisfactory, good or excellent. The marking rubric contained 11 assessable components, including teaching skills, mastery of clinical knowledge, developing a management plan, disease prevention and health promotion opportunities, identifying patient safety issues, the impact of the clinical presentation on the patient and family, and consideration of health equity factors. Analysis of the assessment scores indicated that the majority of students achieved the specified learning outcomes. The PCAP peer teaching activity was an acceptable and effective format for teaching about the management of chronic conditions in the community, and is adaptable to other teaching contexts. Students enjoyed teaching and being taught by their peers, and assessment results indicated that they developed their clinical knowledge as well as their teaching ability regarding chronic disease management. © 2014 John Wiley & Sons Ltd.

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

Science.gov (United States)

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

A Review of the Centers for Disease Control and Prevention's Guidelines for the Clinical Laboratory Diagnosis of Lyme Disease.

Science.gov (United States)

Miraglia, Caterina M

2016-12-01

The purpose of this paper is to review information regarding the current guidelines for the clinical laboratory diagnosis of Lyme disease as set forth by the Centers for Disease Control and Prevention (CDC) to chiropractic physicians and to discuss the clinical utility of this testing. The CDC's website was reviewed to determine what their current recommendations are for the clinical laboratory testing of Lyme disease. The CDC's established guidelines recommend the use of a 2-tiered serologic testing algorithm for the evaluation of patients with suspected Lyme disease. This review provides doctors of chiropractic with information to remain current with the CDC's recommended guidelines for Lyme disease testing because patients may present to their office with the associated signs and symptoms of Lyme disease.

Clinical course and prognosis in patients with Gaucher disease and parkinsonism

Science.gov (United States)

Lopez, Grisel; Kim, Jenny; Wiggs, Edythe; Cintron, Dahima; Groden, Catherine; Tayebi, Nahid; Mistry, Pramod K.; Pastores, Gregory M.; Zimran, Ari; Goker-Alpan, Ozlem

2016-01-01

Objective: The goal of this study was to characterize the parkinsonian phenotype in patients with Gaucher disease (GD) who developed parkinsonism in order to evaluate clinical course and prognosis. Methods: This is a retrospective observational study conducted at the Clinical Center of the NIH, Bethesda, MD, over a period of 10 years. The study included 19 patients with GD and parkinsonism. The severity of Gaucher and parkinsonian symptoms was determined from clinical data including physical, neurologic, pathologic, and neurocognitive evaluations, family histories, imaging studies, olfactory testing, and validated questionnaires. Results: We found an earlier age at onset of parkinsonism and evidence of mild cognitive dysfunction in our cohort. Although the clinical course in some patients was similar to that of idiopathic Parkinson disease with a favorable levodopa response, others exhibited features characteristic of dementia with Lewy bodies. When we examined the patients as a group, we did not observe a uniformly aggressive form of parkinsonism after the initial onset of symptoms, contrary to other published reports. Conclusions: Appreciable clinical variation was seen in this cohort with GD and parkinsonism. Although some patients had early onset and prominent cognitive changes, others had a later, slower course, indicating that GBA1 mutations may not be a reliable prognostic indicator in Parkinson disease in clinical settings. PMID:27123476

ETIOLOGY, PATHOGENESIS AND CLINICAL DIAGNOSIS OF PEYRONIE’S DISEASE

Directory of Open Access Journals (Sweden)

Тарас Валерьевич Шатылко

2017-03-01

Full Text Available Peyronie’s disease remains an understudied progressing disease being  one of the relevant problems of modern urology and andrology. This condition may cause erectile dysfunction in men of fertile age and its negative impact on sexual function adversely affects patients’ quality of life. This article reviews epidemiology, pathophysiology and specifics of recording history and clinical diagnosis of Peyronie’s disease, that includes questionnaires, physical examination, evaluation of erectile function and penile deformity.

Early diagnosis of Alzheimer's disease. Clinical significance and future perspectives

International Nuclear Information System (INIS)

Buerger, K.; Teipel, S.J.; Hampel, H.

2000-01-01

Early diagnosis of Alzheimer's disease describes the recognition and diagnosis in patients with very mild dementia. Internationally accepted diagnostic criteria support the diagnosis based on clinical evaluation. Recent advances in structural and functional neuroimaging as well as studies on specific proteins in the cerebro-spinal fluid that are related to distinct pathophysiological disease processes are most promising approaches to defining biological markers of Alzheimer's disease. (orig.) [de

Bridging the Gap between Research and Clinical Practice in Asymptomatic Alzheimer's Disease.

Science.gov (United States)

Downing, A M; Yaari, R; Ball, D E; Selzler, K J; Devous, M D

2016-01-01

Due to the growing global health impact of Alzheimer's disease (AD), there is a greater need for interventions that prevent or delay the onset of clinical symptoms of this debilitating disease. Clinical trials for disease-modifying compounds in AD have shifted towards earlier stages in the spectrum of illness, including the stage prior to cognitive symptoms. A population of specific interest for clinical research includes individuals with evidence of Alzheimer's disease pathology who are asymptomatic (ADPa). The challenges and barriers regarding medical treatment of ADPa must be identified and addressed prior to the completion of a positive clinical trial in order to accelerate the translation of research findings to clinical practice. This report applies an existing public health impact model from Spencer and colleagues (2013) to evaluate the readiness of the clinical practice environment to treat ADPa individuals if a disease-modifying agent achieves approval. We contrast the current clinical practice environment with a potential future state through investigating the effectiveness, reach, feasibility, sustainability, and transferability of the practice of treating ADPa individuals.

Histologic scoring indices for evaluation of disease activity in Crohn's disease.

Science.gov (United States)

Novak, Gregor; Parker, Claire E; Pai, Rish K; MacDonald, John K; Feagan, Brian G; Sandborn, William J; D'Haens, Geert; Jairath, Vipul; Khanna, Reena

2017-07-21

Histologic assessment of mucosal disease activity has been increasingly used in clinical trials of treatment for Crohn's disease. However, the operating properties of the currently existing histologic scoring indices remain unclear. A systematic review was undertaken to evaluate the development and operating characteristics of available histologic disease activity indices in Crohn's disease. Electronic searches of MEDLINE, EMBASE, PubMed, and the Cochrane Library (CENTRAL) databases from inception to 20 July 2016 were supplemented by manual reviews of bibliographies and abstracts submitted to major gastroenterology meetings (Digestive Disease Week, United European Gastroenterology Week, European Crohn's and Colitis Organisation). Any study design (e.g. randomised controlled trial, cohort study, case series) that evaluated a histologic disease activity index in patients with Crohn's disease was considered for inclusion. Study participants included adult patients (> 16 years), diagnosed with Crohn's disease using conventional clinical, radiographic or endoscopic criteria. Two authors independently reviewed the titles and abstracts of the studies identified from the literature search. The full text of potentially relevant citations were reviewed for inclusion and the study investigators were contacted as needed for clarification. Any disagreements regarding study eligibility were resolved by discussion and consensus with a third author.Two authors independently extracted and recorded data using a standard form. The following data were recorded from each eligible study: number of patients enrolled; number of patients per treatment arm; patient characteristics: age and gender distribution; description of histologic disease activity index utilized; and outcomes such as content validity, construct validity, criterion validity, responsiveness, intra-rater reliability, inter-rater reliability, and feasibility. Sixteen reports of 14 studies describing 14 different numerical

Clinical evaluation of SPECT in cerebrovascular disease

International Nuclear Information System (INIS)

Oshibuchi, Masao; Satoh, Mitsutaka; Kanda, Tetsuro; Nishi, Fumiaki; Yamane, Kanji; Fujimatsu, Masahiko; Edamitsu, Satoshi; Anno, Yasuro; Ohtake, Hisashi.

1989-01-01

In 131 patients with cerebrovascular disease, regional cerebral blood flow were determined by 123 I-IMP (N-isopropyl ( 123 I)-iodoamphetamine) or 99m Tc-HM-PAO ( 99m Tc (d, 1)-hexamethyl propyleneamine oxime) SPECT and findings were compared with those of X-CT or MRI. The perfusion deficit detected by SPECT was larger than the deficit by X-CT or MRI in every case. The perfusion deficit area was more clearly demonstrated by SPECT than by X-CT or MRI in patients with acute cerebral infarction. The hypoperfusion area determined by 123 I-IMP SPECT was wider than that by 99m Tc-HM-PAO SPECT. The crossed cerebellar diaschisis was observed in 56 out of 131 cases (43%). The results of operation were quantitatively evaluated by 123 I-IMP SPECT in 25 patients. (author)

Clinical presentation of juvenile Huntington disease

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Ruocco Heloísa H.

2006-01-01

Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

Celiac disease in non-clinical populations of Japan.

Science.gov (United States)

Fukunaga, Mai; Ishimura, Norihisa; Fukuyama, Chika; Izumi, Daisuke; Ishikawa, Nahoko; Araki, Asuka; Oka, Akihiko; Mishiro, Tomoko; Ishihara, Shunji; Maruyama, Riruke; Adachi, Kyoichi; Kinoshita, Yoshikazu

2018-02-01

Celiac disease is a chronic autoimmune enteropathy caused by gluten ingestion. While its prevalence in Western countries is reported to be as high as 1%, the prevalence has not been evaluated in a large-scale study of a Japanese population. The aim of our study was to clarify the possible presence of celiac disease in a Japanese non-clinical population as well as in patients showing symptoms suggestive of the disease. Serum samples were collected from 2008 non-clinical adults and 47 patients with chronic unexplained abdominal symptoms between April 2014 and June 2016. The anti-tissue transglutaminase (TTG) immunoglobulin A antibody titer was determined as a screening test for celiac disease in all subjects, and individuals with a value of >2 U/mL subsequently underwent testing for the presence of serum endomysial IgA antibody (EMA) as confirmation. Those testing positive for EMA or with a high concentration (>10 U/mL) of TTG were further investigated by histopathological examinations of duodenal mucosal biopsy specimens and HLA typing tests. Of the 2008 non-clinical adults from whom serum samples were collected, 161 tested positive for TTG, and all tested negative for EMA. Four subjects who had a high TTG titer were invited to undergo confirmatory testing, and the histopathological results confirmed the presence of celiac disease in only a single case (0.05%). Of the 47 symptomatic patients, one (2.1%) was found to have a high TTG titer and was diagnosed with celiac disease based on duodenal histopathological findings. The presence of celiac disease in a non-clinical Japanese population was low at 0.05% and was rarely found in patients with unexplained chronic abdominal symptoms.

Evidence assessments and guideline recommendations in Lyme disease: the clinical management of known tick bites, erythema migrans rashes and persistent disease

Science.gov (United States)

Cameron, Daniel J; Johnson, Lorraine B; Maloney, Elizabeth L

2014-01-01

Evidence-based guidelines for the management of patients with Lyme disease were developed by the International Lyme and Associated Diseases Society (ILADS). The guidelines address three clinical questions – the usefulness of antibiotic prophylaxis for known tick bites, the effectiveness of erythema migrans treatment and the role of antibiotic retreatment in patients with persistent manifestations of Lyme disease. Healthcare providers who evaluate and manage patients with Lyme disease are the intended users of the new ILADS guidelines, which replace those issued in 2004 (Exp Rev Anti-infect Ther 2004;2:S1–13). These clinical practice guidelines are intended to assist clinicians by presenting evidence-based treatment recommendations, which follow the Grading of Recommendations Assessment, Development and Evaluation system. ILADS guidelines are not intended to be the sole source of guidance in managing Lyme disease and they should not be viewed as a substitute for clinical judgment nor used to establish treatment protocols. PMID:25077519

Evidence assessments and guideline recommendations in Lyme disease: the clinical management of known tick bites, erythema migrans rashes and persistent disease.

Science.gov (United States)

Cameron, Daniel J; Johnson, Lorraine B; Maloney, Elizabeth L

2014-09-01

Evidence-based guidelines for the management of patients with Lyme disease were developed by the International Lyme and Associated Diseases Society (ILADS). The guidelines address three clinical questions - the usefulness of antibiotic prophylaxis for known tick bites, the effectiveness of erythema migrans treatment and the role of antibiotic retreatment in patients with persistent manifestations of Lyme disease. Healthcare providers who evaluate and manage patients with Lyme disease are the intended users of the new ILADS guidelines, which replace those issued in 2004 (Exp Rev Anti-infect Ther 2004;2:S1-13). These clinical practice guidelines are intended to assist clinicians by presenting evidence-based treatment recommendations, which follow the Grading of Recommendations Assessment, Development and Evaluation system. ILADS guidelines are not intended to be the sole source of guidance in managing Lyme disease and they should not be viewed as a substitute for clinical judgment nor used to establish treatment protocols.

Clinical patterns in Parkinson's disease

NARCIS (Netherlands)

Rooden, Stephanie Maria van

2012-01-01

The clinical heterogeneity of Parkinson’s disease (PD) patients may reflect the existence of subtypes of the disease. PD subtypes have often been defined by a classification according to researcher-specified criteria, such as age-at-onset or predominant clinical motor features. The general objective

A critical evaluation of the clinical evidence for pomegranate preparations in the prevention and treatment of cardiovascular diseases.

Science.gov (United States)

Vlachojannis, Christian; Erne, Paul; Schoenenberger, Andreas W; Chrubasik-Hausmann, Sigrun

2015-04-01

This study attempts a critical evaluation of the clinical evidence behind the use of dietary pomegranate preparations in the prevention and treatment of cardiovascular diseases. A search of PubMed on August 10, 2014 identified 228 references, which yielded extractable data from 24 clinical studies of pomegranate preparations. Hand searching identified two further studies. The quality of the studies and evidence of effectiveness of pomegranate were assessed by an established set of conventional criteria. Overall, the study quality was poor. Even in the best studies, indications of benefit did not reach the conventional levels of statistical significance. The only study with a definitive design had a biochemical rather than a clinical endpoint: it showed the expected difference in blood concentrations of myeloperoxidase after a single dose of either pomegranate or placebo. Only 10 of the 26 studies provided HPLC data on the amounts of co-active ingredients in the preparations that were consumed by the subjects. If pomegranate has a role in the prevention and treatment of cardiovascular diseases, there is a pressing need for dose-finding and long-term confirmatory studies. The ultimate endpoint for definitive studies would be mortality, but reductions in blood pressure or demonstrable decreases in atherosclerotic plaques would be useful surrogates. Sample sizes for various assumptions are provided. Future studies need to prove the clinical benefit. Copyright © 2015 John Wiley & Sons, Ltd.

Clinical heterogeneity in Fabry disease

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G. N. Salogub

2015-01-01

Full Text Available Fabry disease is an X-linked, lysosomal storage disease (OMIM: 301500, caused by α-galactosidase A deficiency, resulting in accumulation of its substrates, glycosphingolipids, primarily – globotriaosylceramide, in the lysosomes of multiple cell types with multi-system clinical manifestations, even within the same family, including abnormalities of the central and peripheral nervous system, kidneys, heart, gastrointestinal tract, lungs, organ of vision. Clinical heterogeneity is often the reason of the delayed diagnosis. Nowadays enzyme replacement therapy has proved its efficiency in the treatment of Fabry disease. Including Fabry disease in the differential diagnosis of a large range of disorders is important because of its wide clinical heterogeneity and the possibility of an earlier intervention with a beneficial treatment.

Clinical evaluation of Parkinson's disease using sup 123 I-IMP SPECT

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Hayashi, Hiroko; Odano, Ikuo; Nishihara, Mamiko; Higuchi, Shouichi; Sakai, Kunio [Niigata Univ. (Japan). School of Medicine; Ishikawa, Atsushi

1989-11-01

N-isopropyl-p({sup 123}I)iodoamphetamine ({sup 123}I-IMP) SPECT and regional cerebral blood flow (rCBF) studies were performed in 20 patients with Parkinson's disease (PD) and 8 normal subjects. RCBF was measured by the arterial blood sampling method which used the microsphere model. We analized seven factors which might be related to the rCBF in PD, i.e., age, stage, duration of the disease, cerebral atrophy, severity of dementia, laterality of symptoms and motor disability score (MDS; the degree of akinesia, rigidity, tremor, gait disturbance, freezing and pulsion sign). Compared with normal subjects, global CBF (supratentorial mean rCBF) was reduced by 21.8% in PD. In particular, rCBF in the basal ganglia and that of frontal cortex were reduced by 25.3% and 24.8%, respectively. Distribution patterns of rCBF in PD were almost as same as those in normals except for cerebellum. The reduction of both rCBFs in the basal ganglia and parietal cortex significantly correlated with MDS (p<0.05, respectively). Especially, akinesia was closely correlated to the reduction of rCBF in the parietal cortex (p<0.02). Moreover, we observed a significant relationship between cerebral atrophy and reduction of rCBF in each region except for cerebellum. However, there was no significant correlation between the severity of dementia and reduction of rCBF, even in the frontal cortex or parietal cortex. These data show that the severity of dementia in PD may be connected with other factors except for rCBF. {sup 123}I-IMP SPECT study is a useful method for clinical evaluation of PD. (author).

Cushing's disease: diagnostic evaluation, therapeutics and prognostic

International Nuclear Information System (INIS)

Pereira, M.A.A.; Jugue, S.M.; Moura, O.M.D.; Gross, K.; Halpern, A.; Nicolau, W.; Liberman, B.; Bloise, W.; Mendonca, B.; Cabral, N.D.; Marino Junior, R.; Wajchenberg, B.L.

1992-01-01

Aspects as clinical, diagnosis, laboratory tests and radiological evaluations concerning Cushing's disease are analysed in 56 patients. Several options of non medicamental therapy are presented. The relationship between preoperative data (hormonal and radiological information), postoperative findings and hypophyseal adenomectomy is discussed. (M.A.C.)

Pre-clinical cognitive phenotypes for Alzheimer disease: a latent profile approach.

Science.gov (United States)

Hayden, Kathleen M; Kuchibhatla, Maragatha; Romero, Heather R; Plassman, Brenda L; Burke, James R; Browndyke, Jeffrey N; Welsh-Bohmer, Kathleen A

2014-11-01

Cognitive profiles for pre-clinical Alzheimer disease (AD) can be used to identify groups of individuals at risk for disease and better characterize pre-clinical disease. Profiles or patterns of performance as pre-clinical phenotypes may be more useful than individual test scores or measures of global decline. To evaluate patterns of cognitive performance in cognitively normal individuals to derive latent profiles associated with later onset of disease using a combination of factor analysis and latent profile analysis. The National Alzheimer Coordinating Centers collect data, including a battery of neuropsychological tests, from participants at 29 National Institute on Aging-funded Alzheimer Disease Centers across the United States. Prior factor analyses of this battery demonstrated a four-factor structure comprising memory, attention, language, and executive function. Factor scores from these analyses were used in a latent profile approach to characterize cognition among a group of cognitively normal participants (N = 3,911). Associations between latent profiles and disease outcomes an average of 3 years later were evaluated with multinomial regression models. Similar analyses were used to determine predictors of profile membership. Four groups were identified; each with distinct characteristics and significantly associated with later disease outcomes. Two groups were significantly associated with development of cognitive impairment. In post hoc analyses, both the Trail Making Test Part B, and a contrast score (Delayed Recall - Trails B), significantly predicted group membership and later cognitive impairment. Latent profile analysis is a useful method to evaluate patterns of cognition in large samples for the identification of preclinical AD phenotypes; comparable results, however, can be achieved with very sensitive tests and contrast scores. Copyright © 2014 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.

[Clinical and psychopathological factors associated with impulse control disorders in Parkinson's disease].

Science.gov (United States)

Sáez-Francàs, N; Martí Andrés, G; Ramírez, N; de Fàbregues, O; Álvarez-Sabín, J; Casas, M; Hernández-Vara, J

2016-05-01

Impulse control disorders (ICD) constitute a complication that may arise during the course of Parkinson's disease (PD). Several factors have been linked to the development of these disorders, and their associated severe functional impairment requires specific and multidisciplinary management. The objective of this study was to evaluate the frequency of ICDs and the clinical and psychopathological factors associated with the appearance of these disorders. Cross-sectional, descriptive, and analytical study of a sample of 115 PD patients evaluated to determine the presence of an ICD. Clinical scales were administered to assess disease severity, personality traits, and presence of psychiatric symptoms at the time of evaluation. Of the 115 patients with PD, 27 (23.48%) displayed some form of ICD; hypersexuality, exhibited by 14 (12.2%), and binge eating, present in 12 (10.1%), were the most common types. Clinical factors associated with ICD were treatment with dopamine agonists (OR: 13.39), earlier age at disease onset (OR: 0.92), and higher score on the UPDRS-I subscale; psychopathological factors with a significant association were trait anxiety (OR: 1.05) and impulsivity (OR: 1.13). ICDs are frequent in PD, and treatment with dopamine agonists is the most important risk factor for these disorders. High impulsivity and anxiety levels at time of evaluation, and younger age at disease onset, were also linked to increased risk. However, presence of these personality traits prior to evaluation did not increase risk of ICD. Copyright © 2015 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.

Clinical presentation and evaluation of dermatomyositis

Directory of Open Access Journals (Sweden)

Umaima Marvi

2012-01-01

Full Text Available Dermatomyositis (DM is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50-70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron′s papules, Gottron′s sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, "mechanic′s hands", palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35-40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.

Development, validation and clinical assessment of a short questionnaire to assess disease-related knowledge in inflammatory bowel disease patients.

LENUS (Irish Health Repository)

Keegan, Denise

2013-02-01

Only two inflammatory bowel disease (IBD) knowledge scales are available, both primarily aimed at evaluating the effectiveness of clinical education programs. The aim of this study was to develop and validate a short knowledge questionnaire for clinical and academic research purposes.

Evaluation of clinical pathology parameters in fecal PCR-positive or PCR-negative goats for Johne's disease.

Science.gov (United States)

Bonelli, Francesca; Fratini, F; Turchi, B; Cantile, C; Ebani, V V; Colombani, G; Galiero, A; Sgorbini, M

2017-10-01

Johne's disease (JD) is an economically important infectious disease of ruminants caused by Mycobacterium avium subsp. paratuberculosis (MAP). This study evaluated the differences in various hematological and biochemical parameters between healthy goats and goats with JD. Forty goats were chosen randomly from a herd endemic for JD. A complete physical examination was performed. Blood and fresh fecal samples were collected from each goat. A complete blood cell (CBC) count and a protein electrophoresis were performed. Polymerase chain reaction (PCR) on fecal samples was performed in order to divide goats into two groups: group A "positive PCR on feces"; and group B "control (negative)." A Student's t test was performed for each parameter to verify differences between groups A vs B. Twenty goats were included in each group. Clinical signs likely related to JD were found in the history of 4/40 (10%) goats, while 36/40 (90%) goats were reported to be asymptomatic. CBC and electrophoresis values were within reference intervals in both groups. No differences were found for CBC parameters between the two groups. Values for alpha 1, beta, gamma globulins, and total protein (TP) were statistically higher in group A vs those in group B, while those for albumin and albumin/globulin (A/G) ratio were lower. An increase in TP, hypoalbuminemia, and hypergammaglobulinemia has been reported in group A, while no abnormalities were found concerning CBC. JD-positive goats seem to show earlier clinical pathological alternations than clinical signs. Protein electrophoresis may help the diagnosis of JD in asymptomatic goat herds, acting as an economical screening method.

Clinical course of Crohn's disease first diagnosed at surgery for acute abdomen.

Science.gov (United States)

Latella, G; Cocco, A; Angelucci, E; Viscido, A; Bacci, S; Necozione, S; Caprilli, R

2009-04-01

The severity of clinical activity of Crohn's disease is high during the first year after diagnosis and decreases thereafter. Approximately 50% of patients require steroids and immunosuppressants and 75% need surgery during their lifetime. The clinical course of patients with Crohn's disease first diagnosed at surgery has never been investigated. To assess the clinical course of Crohn's disease first diagnosed at surgery for acute abdomen and to evaluate the need for medical and surgical treatment in this subset of patients. Hospital clinical records of 490 consecutive Crohn's disease patients were reviewed. Patients were classified according to the Vienna criteria. Sex, extraintestinal manifestations, family history of inflammatory bowel diseases, appendectomy, smoking habit and medical/surgical treatments performed during the follow-up period were assessed. Kaplan-Meier survival method and Cox proportional hazards regression model. Of the 490 Crohn's disease patients, 115 had diagnosis of Crohn's disease at surgery for acute abdomen (Group A) and 375 by conventional clinical, radiological, endoscopic and histologic criteria (Group B). Patients in Group A showed a low risk of further surgery (Log Rank test pacute abdomen showed a low risk for reintervention and less use of steroids and immunosuppressants during follow-up than those not operated upon at diagnosis. Early surgery may represent a valid approach in the initial management of patients with Crohn's disease, at least in the subset of patients with ileal and complicated disease.

Evaluation of tuberculosis diagnostics in children: 1. Proposed clinical case definitions for classification of intrathoracic tuberculosis disease. Consensus from an expert panel.

Science.gov (United States)

Graham, Stephen M; Ahmed, Tahmeed; Amanullah, Farhana; Browning, Renee; Cardenas, Vicky; Casenghi, Martina; Cuevas, Luis E; Gale, Marianne; Gie, Robert P; Grzemska, Malgosia; Handelsman, Ed; Hatherill, Mark; Hesseling, Anneke C; Jean-Philippe, Patrick; Kampmann, Beate; Kabra, Sushil Kumar; Lienhardt, Christian; Lighter-Fisher, Jennifer; Madhi, Shabir; Makhene, Mamodikoe; Marais, Ben J; McNeeley, David F; Menzies, Heather; Mitchell, Charles; Modi, Surbhi; Mofenson, Lynne; Musoke, Philippa; Nachman, Sharon; Powell, Clydette; Rigaud, Mona; Rouzier, Vanessa; Starke, Jeffrey R; Swaminathan, Soumya; Wingfield, Claire

2012-05-15

There is a critical need for improved diagnosis of tuberculosis in children, particularly in young children with intrathoracic disease as this represents the most common type of tuberculosis in children and the greatest diagnostic challenge. There is also a need for standardized clinical case definitions for the evaluation of diagnostics in prospective clinical research studies that include children in whom tuberculosis is suspected but not confirmed by culture of Mycobacterium tuberculosis. A panel representing a wide range of expertise and child tuberculosis research experience aimed to develop standardized clinical research case definitions for intrathoracic tuberculosis in children to enable harmonized evaluation of new tuberculosis diagnostic technologies in pediatric populations. Draft definitions and statements were proposed and circulated widely for feedback. An expert panel then considered each of the proposed definitions and statements relating to clinical definitions. Formal group consensus rules were established and consensus was reached for each statement. The definitions presented in this article are intended for use in clinical research to evaluate diagnostic assays and not for individual patient diagnosis or treatment decisions. A complementary article addresses methodological issues to consider for research of diagnostics in children with suspected tuberculosis.

Evaluation of Tuberculosis Diagnostics in Children: 1. Proposed Clinical Case Definitions for Classification of Intrathoracic Tuberculosis Disease. Consensus From an Expert Panel

Science.gov (United States)

Graham, Stephen M.; Ahmed, Tahmeed; Amanullah, Farhana; Browning, Renee; Cardenas, Vicky; Casenghi, Martina; Cuevas, Luis E.; Gale, Marianne; Gie, Robert P.; Grzemska, Malgosia; Handelsman, Ed; Hatherill, Mark; Hesseling, Anneke C.; Jean-Philippe, Patrick; Kampmann, Beate; Kabra, Sushil Kumar; Lienhardt, Christian; Lighter-Fisher, Jennifer; Madhi, Shabir; Makhene, Mamodikoe; Marais, Ben J.; McNeeley, David F.; Menzies, Heather; Mitchell, Charles; Modi, Surbhi; Mofenson, Lynne; Musoke, Philippa; Nachman, Sharon; Powell, Clydette; Rigaud, Mona; Rouzier, Vanessa; Starke, Jeffrey R.; Swaminathan, Soumya; Wingfield, Claire

2012-01-01

There is a critical need for improved diagnosis of tuberculosis in children, particularly in young children with intrathoracic disease as this represents the most common type of tuberculosis in children and the greatest diagnostic challenge. There is also a need for standardized clinical case definitions for the evaluation of diagnostics in prospective clinical research studies that include children in whom tuberculosis is suspected but not confirmed by culture of Mycobacterium tuberculosis. A panel representing a wide range of expertise and child tuberculosis research experience aimed to develop standardized clinical research case definitions for intrathoracic tuberculosis in children to enable harmonized evaluation of new tuberculosis diagnostic technologies in pediatric populations. Draft definitions and statements were proposed and circulated widely for feedback. An expert panel then considered each of the proposed definitions and statements relating to clinical definitions. Formal group consensus rules were established and consensus was reached for each statement. The definitions presented in this article are intended for use in clinical research to evaluate diagnostic assays and not for individual patient diagnosis or treatment decisions. A complementary article addresses methodological issues to consider for research of diagnostics in children with suspected tuberculosis. PMID:22448023

Clinical-histopathological correlation in a case of Coats' disease

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Zajdenweber Moyses E

2006-08-01

Full Text Available Abstract Background Coats' disease is a non-hereditary ocular disease, with no systemic manifestation, first described by Coats in 1908. It occurs more commonly in children and has a clear male predominance. Most patients present clinically with unilateral decreased vision, strabismus or leukocoria. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has some overlapping clinical manifestations. Case presentation A 4 year-old girl presented with a blind and painful right eye. Ocular examination revealed neovascular glaucoma, cataract and posterior synechiae. Although viewing of the fundus was impossible, computed tomography disclosed total exsudative retinal detachment in the affected eye. The eye was enucleated and subsequent histopathological evaluation confirmed the diagnosis of Coats' disease. Conclusion General pathologists usually do not have the opportunity to receive and study specimens from patients with Coats' disease. Coats' disease is one of the most important differential diagnoses of retinoblastoma. Therefore, It is crucial for the pathologist to be familiar with the histopathological features of the former, and distinguish it from the latter.

Optimizing chronic disease management mega-analysis: economic evaluation.

Science.gov (United States)

2013-01-01

As Ontario's population ages, chronic diseases are becoming increasingly common. There is growing interest in services and care models designed to optimize the management of chronic disease. To evaluate the cost-effectiveness and expected budget impact of interventions in chronic disease cohorts evaluated as part of the Optimizing Chronic Disease Management mega-analysis. Sector-specific costs, disease incidence, and mortality were calculated for each condition using administrative databases from the Institute for Clinical Evaluative Sciences. Intervention outcomes were based on literature identified in the evidence-based analyses. Quality-of-life and disease prevalence data were obtained from the literature. Analyses were restricted to interventions that showed significant benefit for resource use or mortality from the evidence-based analyses. An Ontario cohort of patients with each chronic disease was constructed and followed over 5 years (2006-2011). A phase-based approach was used to estimate costs across all sectors of the health care system. Utility values identified in the literature and effect estimates for resource use and mortality obtained from the evidence-based analyses were applied to calculate incremental costs and quality-adjusted life-years (QALYs). Given uncertainty about how many patients would benefit from each intervention, a system-wide budget impact was not determined. Instead, the difference in lifetime cost between an individual-administered intervention and no intervention was presented. Of 70 potential cost-effectiveness analyses, 8 met our inclusion criteria. All were found to result in QALY gains and cost savings compared with usual care. The models were robust to the majority of sensitivity analyses undertaken, but due to structural limitations and time constraints, few sensitivity analyses were conducted. Incremental cost savings per patient who received intervention ranged between $15 per diabetic patient with specialized nursing to

Frequency and clinical predictors of coronary artery disease in chronic renal failure renal transplant candidates.

Science.gov (United States)

de Albuquerque Seixas, Emerson; Carmello, Beatriz Leone; Kojima, Christiane Akemi; Contti, Mariana Moraes; Modeli de Andrade, Luiz Gustavo; Maiello, José Roberto; Almeida, Fernando Antonio; Martin, Luis Cuadrado

2015-05-01

Cardiovascular diseases are major causes of mortality in chronic renal failure patients before and after renal transplantation. Among them, coronary disease presents a particular risk; however, risk predictors have been used to diagnose coronary heart disease. This study evaluated the frequency and importance of clinical predictors of coronary artery disease in chronic renal failure patients undergoing dialysis who were renal transplant candidates, and assessed a previously developed scoring system. Coronary angiographies conducted between March 2008 and April 2013 from 99 candidates for renal transplantation from two transplant centers in São Paulo state were analyzed for associations between significant coronary artery diseases (≥70% stenosis in one or more epicardial coronary arteries or ≥50% in the left main coronary artery) and clinical parameters. Univariate logistic regression analysis identified diabetes, angina, and/or previous infarction, clinical peripheral arterial disease and dyslipidemia as predictors of coronary artery disease. Multiple logistic regression analysis identified only diabetes and angina and/or previous infarction as independent predictors. The results corroborate previous studies demonstrating the importance of these factors when selecting patients for coronary angiography in clinical pretransplant evaluation.

Update on the clinical management of Wilson's disease

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Hedera P

2017-01-01

Full Text Available Peter Hedera Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA Abstract: Wilson’s disease (WD, albeit relatively rare, is an important genetic metabolic disease because of highly effective therapies that can be lifesaving. It is a great imitator and requires a high index of suspicion for correct and timely diagnosis. Neurologic, psychiatric and hepatologic problems in WD are very nonspecific, and we discuss the most common clinical phenotypes. The diagnosis remains laboratory based, and here we review the most important challenges and pitfalls in laboratory evaluation of WD, including the emerging role of genetic testing in WD diagnosis. WD is a monogenic disorder but has very high allelic heterogeneity with >500 disease-causing mutations identified, and new insights into phenotype–genotype correlations are also reviewed. The gold standard of therapy is chelation of excessive copper, but many unmet needs exist because of possible clinical deterioration in treated patients and potential adverse effects associated with currently available chelating medications. We also review the most promising novel therapeutic approaches, including chelators targeting specific cell types, cell transplantation and gene therapy. Keywords: Wilson’s disease, copper, ATP7B, chelation, gene therapy

Clinical research on peri-implant diseases: consensus report of Working Group 4.

LENUS (Irish Health Repository)

Sanz, Mariano

2012-02-01

Two systematic reviews have evaluated the quality of research and reporting of observational studies investigating the prevalence of, the incidence of and the risk factors for peri-implant diseases and of experimental clinical studies evaluating the efficacy of preventive and therapeutic interventions.

Evaluating next-generation sequencing for direct clinical diagnostics in diarrhoeal disease

DEFF Research Database (Denmark)

Joensen, Katrine Grimstrup; Engsbro, A L Ø; Lukjancenko, Oksana

2017-01-01

The accurate microbiological diagnosis of diarrhoea involves numerous laboratory tests and, often, the pathogen is not identified in time to guide clinical management. With next-generation sequencing (NGS) becoming cheaper, it has huge potential in routine diagnostics. The aim of this study...... was to evaluate the potential of NGS-based diagnostics through direct sequencing of faecal samples. Fifty-eight clinical faecal samples were obtained from patients with diarrhoea as part of the routine diagnostics at Hvidovre University Hospital, Denmark. Ten samples from healthy individuals were also included...

AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND AMERICAN COLLEGE OF ENDOCRINOLOGY DISEASE STATE CLINICAL REVIEW: DIAGNOSIS OF RECURRENCE IN CUSHING DISEASE.

Science.gov (United States)

Fleseriu, Maria; Hamrahian, Amir H; Hoffman, Andrew R; Kelly, Daniel F; Katznelson, Laurence

2016-12-01

Recurrence of hypercortisolemia after initial treatment of Cushing disease (CD) is more common than previously thought, with a third of patients suffering a recurrence over their lifetime. Awareness of this high rate and delayed timeline (sometimes decades) of potential recurrence is critical and patients with CD should be monitored at regular intervals throughout their lives. In this manuscript, we review the complex evaluation needed for defining CD remission versus persistent disease after surgery, and focus on challenges in diagnosing early recurrent hypercortisolemia. Late night salivary cortisol appears to be an earlier predictor of recurrence when compared with urinary free cortisol (UFC) excretion. We also review the criteria suggested to define recurrence of hypercortisolemia in patients treated with medical therapy. Further research is needed to determine the optimal way to evaluate a patient with CD recurrence as well as the riskbenefit ratio of treatment in early, mild recurrent disease. ACTH = adrenocorticotropic hormone AI = adrenal insufficiency CD = Cushing disease CDDT = coupled dexamethasone desmopressin test CR = circadian rhythm CRH = corticotropin-releasing hormone GC = glucocorticoid GCR = global clinical response HPA = hypothalamic-pituitary-adrenal LDDST = low-dose dexamethasone suppression test LNSC = late-night salivary cortisol ODST = overnight dexamethasone suppression test TSS = trans-sphenoidal surgery.

Paediatric Inflammatory Bowel Disease: Clinical Presentation and Disease Location.

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Aziz, Danish Abdul; Moin, Maryum; Majeed, Atif; Sadiq, Kamran; Biloo, Abdul Gaffar

2017-01-01

To determine different clinical presentationsand disease location demarcatedby upper and lower gastrointestinal endoscopyand relevant histopathologyin children diagnosed with inflammatory bowel disease (IBD). This is 5 years (2010 to 2015) retrospective studyconducted at the Aga Khan University Hospitalenrolling65admitted children between 6 months to 15years from either gender, diagnosed with IBD on clinical presentation, endoscopy and biopsy. Different clinical presentations at the time of diagnosis were noted in different categories of the disease. All patients underwent upper and lower (up to the terminal ileum) endoscopy with multiple punch biopsies and histologic assessment of mucosal specimens. All endoscopies were done by paediatric gastroenterologists at endoscopy suite of the hospital and all specimens were reported by the pathology department. ESPGHAN revised criteria for the diagnosis of inflammatory bowel disease in children and an adolescent was used to standardize our diagnosis. Extent of disease on endoscopy and relevant histopathology of the biopsy samples were noted at the time of diagnosis. Data was summarized using mean, standard deviation, numbers and percentages for different variables. Total 56 children were enrolled according to inclusion criteria. There were 34children (61.53%) diagnosed with ulcerative colitis (UC), 10 patients (16.92%) had Crohn'sDisease (CD) and 11 (21.53%) patients were labeled as Indeterminate colitis (IC). Mean age at onset of symptoms was10.03±2.44 and mean age at diagnosis was11.10±2.36. Abdominal pain (80%) and chronic diarrhea (70%) were common symptoms in CD whereas bloody diarrhea (79.41%) and rectal bleeding(64.70%)were common presentation in UC. Patients diagnosed with indeterminate colitis(IC) had similar clinical features as in UC patients. Only 7% patients had some extra-intestinal features in the form of joint pain and/or uveitis. Aspartate aminotransferase level (95.18 ±12.89) was relatively high in

Can we Predict Disease Course with Clinical Factors?

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Vegh, Zsuzsanna; Kurti, Zsuzsanna; Golovics, Petra A; Lakatos, Peter L

2018-01-01

The disease phenotype at diagnosis and the disease course of Crohn's disease (CD) and ulcerative colitis (UC) show remarkable heterogeneity across patients. This review aims to summarize the currently available evidence on clinical and some environmental predictive factors, which clinicians should evaluate in the everyday practice together with other laboratory and imaging data to prevent disease progression, enable a more personalized therapy, and avoid negative disease outcomes. In recent population-based epidemiological and referral cohort studies, the evolution of disease phenotype of CD and UC varied significantly. Most CD and severe UC patients still require hospitalization or surgery/colectomy during follow-up. A change in the natural history of inflammatory bowel diseases (IBD) with improved outcomes in parallel with tailored positioning of aggressive immunomodulator and biological therapy has been suspected. According to the currently available literature, it is of major importance to refer IBD cases at risk for adverse disease outcomes as early during the disease course as possible. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer's Disease Database.

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Maddox, Ryan A; Blase, J L; Mercaldo, N D; Harvey, A R; Schonberger, L B; Kukull, W A; Belay, E D

2015-12-01

Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing. National Alzheimer's Coordinating Center (NACC) Minimum and Neuropathologic Data Set for 1984 to 2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer's Disease Center (ADC), and were coded as having an Alzheimer's disease clinical diagnosis or a nonprion disease etiology. For the eligible patients with neuropathology indicating prion disease, further clinical information, collected from the reporting ADC, determined whether prion disease was considered before autopsy. Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases. The proportion of patients with dementia with clinically unrecognized but autopsy-confirmed prion disease was small. Besides confirming clinically suspected cases, neuropathology is useful to identify unsuspected clinically atypical cases of prion disease. © The Author(s) 2015.

Wilson's disease: cranial MRI observations and clinical correlation

International Nuclear Information System (INIS)

Sinha, S.; Taly, A.B.; Prashanth, L.K.; Venugopal, K.S.; Arunodaya, G.R.; Swamy, H.S.; Ravishankar, S.; Vasudev, M.K.

2006-01-01

Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson's disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD. MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3±8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted. The mean duration of illness and treatment were 8.3±10.8 years and 7.5±7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), ''Face of giant panda'' sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P<0.001) but did not correlate with the duration of illness. MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease. (orig.)

Childhood Pompe disease: clinical spectrum and genotype in 31 patients

NARCIS (Netherlands)

Capelle, C.I. van; Meijden, J.C. van der; Hout, J.M. van den; Jaeken, J.; Baethmann, M.; Voit, T.; Kroos, M.A.; Derks, T.G.; Rubio-Gozalbo, M.E.; Willemsen, M.A.A.P.; Lachmann, R.H.; Mengel, E.; Michelakakis, H.; Jongste, J.C. de; Reuser, A.J.; Ploeg, A.T. van der

2016-01-01

BACKGROUND: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in Rotterdam, the

Childhood Pompe disease: Clinical spectrum and genotype in 31 patients

NARCIS (Netherlands)

C.I. van Capelle (Carine); J.C. van der Meijden (Chris); J.M.P. van den Hout (Johanna); J. Jaeken; M. Baethmann; T. Voit; M.A. Kroos (Marian); T.G.J. Derks (Terry G J); M.E. Rubio-Gozalbo (Estela); M.A. Willemsen (Michél); R. Lachmann (Robin); E. Mengel; H. Michelakakis (Helen); J.C. de Jongste (Johan); A.J.J. Reuser (Arnold); A.T. van der Ploeg (Ans)

2016-01-01

textabstractBackground: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in

Childhood Pompe disease : clinical spectrum and genotype in 31 patients

NARCIS (Netherlands)

van Capelle, C I; van der Meijden, J C; van den Hout, J M P; Jaeken, J; Baethmann, M; Voit, T; Kroos, M A; Derks, T G J; Rubio-Gozalbo, M E; Willemsen, M A; Lachmann, R H; Mengel, E; Michelakakis, H; de Jongste, J C; Reuser, A J J; van der Ploeg, A T

2016-01-01

Background: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in Rotterdam, the

Correlation of clinical data, anatomical site and disease stage in ...

African Journals Online (AJOL)

Objective: To evaluate the colorectal cancer clinical data with respect to the anatomical location and stage of disease. Design: Retrospective observational study. Setting: Kenyatta National Hospital (KNH), Nairobi, Kenya. Subjects: Two hundred and fifty three tumours were categorised as right colonic (RCC), left colonic ...

Rheumatoid arthritis disease activity measures: American College of Rheumatology recommendations for use in clinical practice.

Science.gov (United States)

Anderson, Jaclyn; Caplan, Liron; Yazdany, Jinoos; Robbins, Mark L; Neogi, Tuhina; Michaud, Kaleb; Saag, Kenneth G; O'Dell, James R; Kazi, Salahuddin

2012-05-01

Although the systematic measurement of disease activity facilitates clinical decision making in rheumatoid arthritis (RA), no recommendations currently exist on which measures should be applied in clinical practice in the US. The American College of Rheumatology (ACR) convened a Working Group (WG) to comprehensively evaluate the validity, feasibility, and acceptability of available RA disease activity measures and derive recommendations for their use in clinical practice. The Rheumatoid Arthritis Clinical Disease Activity Measures Working Group conducted a systematic review of the literature to identify RA disease activity measures. Using exclusion criteria, input from an Expert Advisory Panel (EAP), and psychometric analysis, a list of potential measures was created. A survey was administered to rheumatologists soliciting input. The WG used these survey results in conjunction with the psychometric analyses to derive final recommendations. Systematic review of the literature resulted in identification of 63 RA disease activity measures. Application of exclusion criteria and ratings by the EAP narrowed the list to 14 measures for further evaluation. Practicing rheumatologists rated 9 of these 14 measures as most useful and feasible. From these 9 measures, the WG selected 6 with the best psychometric properties for inclusion in the final set of ACR-recommended RA disease activity measures. We recommend the Clinical Disease Activity Index, Disease Activity Score with 28-joint counts (erythrocyte sedimentation rate or C-reactive protein), Patient Activity Scale (PAS), PAS-II, Routine Assessment of Patient Index Data with 3 measures, and Simplified Disease Activity Index because they are accurate reflections of disease activity; are sensitive to change; discriminate well between low, moderate, and high disease activity states; have remission criteria; and are feasible to perform in clinical settings. Copyright © 2012 by the American College of Rheumatology.

Management of celiac disease: from evidence to clinical practice

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Tiziana M. Attardo

2017-11-01

Full Text Available Celiac disease (CD is a complex polygenic disorder, which involves genetic factors human leukocyte complex (HLA and non-HLA genes, environmental factors, innate and adoptive immunity, and a robust chronic T-mediated autoimmune component. The main goal of the present monograph is to define a methodological approach for the disease, characterized by frequent late diagnosis, in order for the physician to become aware of the disease management, the diversity of the clinical presentation itself and in different patients. A unique attention is payed to the specific diagnostic tests to define a correct and accurate application of them, and in addition, to disease follow-up and possible complications. Moreover, a dedicated space is assigned to refractory CD, to potential CD and non-celiac gluten sensitivity. Legislative aspects of the celiac disease in Italy are addressed, too. The celiac disease guidelines and their evaluation by means of Appraisal of Guidelines, Research and Evaluation II instrument allow us to classify the different recommendations and to apply them according to the stakeholders’ involvement, pertinence, methodological accuracy, clarity and publishing independence. Finally, the most current scientific evidence is taken into account to create a complete updated monograph.

Clinical and fiberoptic endoscopic assessment of swallowing in patients with chronic obstructive pulmonary disease

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Macri, Marina Rodrigues Bueno

2014-01-01

Full Text Available Introduction: Chronic obstructive pulmonary disease is characterized by progressive and partially reversible obstruction of pulmonary airflow. Aim: To characterize swallowing in patients with chronic obstructive pulmonary disease and correlate the findings with the degree chronic obstructive pulmonary disease, heart and respiratory rate, oxygen saturation, and smoking. Method: We conducted a prospective cohort study of 19 patients (12 men and 7 women; age range, 50–85 years with confirmed medical diagnosis of chronic obstructive pulmonary disease. This study was performed in 2 stages (clinical evaluation and functional assessment using nasolaryngofibroscopy on the same day. During both stages, vital signs were checked by medical personnel. Results: Clinical evaluation of swallowing in all patients showed the clinical signs of cough. The findings of nasolaryngofibroscopy highlighted subsequent intraoral escape in 5 patients (26.5%. No patient had tracheal aspiration. There was no association of subsequent intraoral escape with degree of chronic obstructive pulmonary disease, heart and respiratory rate, oxygen saturation, or smoking. Conclusion: In patients with chronic obstructive pulmonary disease, there was a prevalence of oral dysphagia upon swallowing and nasolaryngofibroscopy highlighted the finding of subsequent intraoral escape. There was no correlation between intraoral escape and the degree of chronic obstructive pulmonary disease, heart and respiratory rate, oxygen saturation, or smoking.

Disease activity indices in coeliac disease: systematic review and recommendations for clinical trials.

Science.gov (United States)

Hindryckx, Pieter; Levesque, Barrett G; Holvoet, Tom; Durand, Serina; Tang, Ceen-Ming; Parker, Claire; Khanna, Reena; Shackelton, Lisa M; D'Haens, Geert; Sandborn, William J; Feagan, Brian G; Lebwohl, Benjamin; Leffler, Daniel A; Jairath, Vipul

2018-01-01

Although several pharmacological agents have emerged as potential adjunctive therapies to a gluten-free diet for coeliac disease, there is currently no widely accepted measure of disease activity used in clinical trials. We conducted a systematic review of coeliac disease activity indices to evaluate their operating properties and potential as outcome measures in registration trials. MEDLINE, EMBASE and the Cochrane central library were searched from 1966 to 2015 for eligible studies in adult and/or paediatric patients with coeliac disease that included coeliac disease activity markers in their outcome measures. The operating characteristics of histological indices, patient-reported outcomes (PROs) and endoscopic indices were evaluated for content and construct validity, reliability, responsiveness and feasibility using guidelines proposed by the US Food and Drug Administration (FDA). Of 19 123 citations, 286 studies were eligible, including 24 randomised-controlled trials. Three of five PROs identified met most key evaluative criteria but only the Celiac Disease Symptom Diary (CDSD) and the Celiac Disease Patient-Reported Outcome (CeD PRO) have been approved by the FDA. All histological and endoscopic scores identified lacked content validity. Quantitative morphometric histological analysis had better reliability and responsiveness compared with qualitative scales. Endoscopic indices were infrequently used, and only one index demonstrated responsiveness to effective therapy. Current best evidence suggests that the CDSD and the CeD PRO are appropriate for use in the definition of primary end points in coeliac disease registration trials. Morphometric histology should be included as a key secondary or co-primary end point. Further work is needed to optimise end point configuration to inform efficient drug development. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

UPLC-based metabonomic applications for discovering biomarkers of diseases in clinical chemistry.

Science.gov (United States)

Zhao, Ying-Yong; Cheng, Xian-Long; Vaziri, Nosratola D; Liu, Shuman; Lin, Rui-Chao

2014-10-01

Metabonomics is a powerful and promising analytic tool that allows assessment of global low-molecular-weight metabolites in biological systems. It has a great potential for identifying useful biomarkers for early diagnosis, prognosis and assessment of therapeutic interventions in clinical practice. The aim of this review is to provide a brief summary of the recent advances in UPLC-based metabonomic approach for biomarker discovery in a variety of diseases, and to discuss their significance in clinical chemistry. All the available information on UPLC-based metabonomic applications for discovering biomarkers of diseases were collected via a library and electronic search (using Web of Science, Pubmed, ScienceDirect, Springer, Google Scholar, etc.). Metabonomics has been used in clinical chemistry to identify and evaluate potential biomarkers and therapeutic targets in various diseases affecting the liver (hepatocarcinoma and liver cirrhosis), lung (lung cancer and pneumonia), gastrointestinal tract (colorectal cancer) and urogenital tract (prostate cancer, ovarian cancer and chronic kidney disease), as well as metabolic diseases (diabetes) and neuropsychiatric disorders (Alzheimer's disease and schizophrenia), etc. The information provided highlights the potential value of determination of endogenous low-molecular-weight metabolites and the advantages and potential drawbacks of the application of UPLC-based metabonomics in clinical setting. Copyright © 2014 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

MDS clinical diagnostic criteria for Parkinson's disease in China.

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Li, Jun; Jin, Miao; Wang, Li; Qin, Bin; Wang, Kang

2017-03-01

The Movement Disorder Society Clinical Diagnostic Criteria for Parkinson's disease (MDS-PD Criteria) was introduced by the Movement Disorder Society in 2015 for research purposes. However, its use for clinical diagnosis of Parkinson disease still needs further revision. This study compares the UK-Criteria versus MDS-PD Criteria in the clinical diagnosis of Parkinson disease referred to the China-Japan Friendship Hospital of Beijing, China. To compare the MDS-PD Criteria with the UK-Criteria and discuss the feasibility of the clinical application of MDS-PD Criteria as a general guide to clinical diagnosis of PD in Chinese PD patients. 150 patients of neurology clinic of China-Japan Friendship Hospital of Beijing were recruited in our research. They were divided into three groups: UK-Criteria group, MDS-PD Criteria group and a combined group of UK and MDS-PD Criteria. Clinical history was collected while physical and auxiliary examinations were done by a trained neurologist according to the corresponding criteria. An interrater reliability analysis using the Kappa statistic claimed substantial agreement (κ = 0.626) between the MDS-PD Criteria and the UK-Criteria. The differences between the diagnostic results of these two criteria were statistically significant by paired Chi-square test (p = 0.000). It was found that levodopa-induced dyskinesia had a good positive predictive value, while early bulbar impairment and inspiratory dysfunction presented a negative predictive value. The MDS-PD Criteria emphasize the importance of non-motor symptoms, keeping the motor symptoms as the core for the clinical diagnosis of PD, and establish categories of diagnosis features and levels of certainty which are more complete and organized to be used and replicated by non specialized physicians to evaluated patients with Parkinsonism. The higher sensitivity of MDS-PD Criteria compared with UK-Criteria is worth being widely used in clinical work.

Mitochondrial disease patient motivations and barriers to participate in clinical trials.

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Zarazuela Zolkipli-Cunningham

disease patient communities is essential to effectively design robust clinical trials that engage patients and enable meaningful evaluation of emerging treatment interventions.

The economic evaluation of pharmacotherapies for Parkinson's disease.

Science.gov (United States)

Coyle, D; Barbeau, M; Guttman, M; Baladi, J-F

2003-06-01

As well as the significant clinical effects of Parkinson's disease (PD), the disease places a high economic burden on society. Given the scarcity of health care resources, it is becoming increasingly necessary to demonstrate that new therapies for PD provide value for money in comparison with other potential interventions. This paper outlines the basic techniques of cost-effectiveness analysis and its application to PD. These techniques are illustrated by a recent economic evaluation of entacapone for use in Canada.

Recruitment of subjects into clinical trials for Alzheimer disease.

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Knebl, Janice A; Patki, Deepti

2010-09-01

Alzheimer disease is a devastating neurodegenerative disorder affecting millions of Americans. It reduces the ability of the individual to remain independent, places a burden on caregivers, and substantially increases healthcare costs. New treatments are being tested in numerous clinical trials with the goal of preventing or delaying the onset of Alzheimer disease, slowing or modifying the disease's course, or finding a cure for patients with the disease. Alzheimer disease research can successfully proceed only if individuals who have this illness are willing to participate in clinical trials. However, recruitment and retention of subjects in clinical trials for Alzheimer disease is a challenging task. Furthermore, because of reductions in decision-making capacities of individuals with Alzheimer disease, clinical trials also need to involve caregivers. The present article delineates unique hurdles encountered in the recruitment process for Alzheimer disease clinical trials. The article also identifies strategies for effective recruitment of subjects in Alzheimer disease clinical trials, including guidelines to help principal investigators and clinical research coordinators reach recruitment goals.

Regulations in the United States for cell transplantation clinical trials in neurological diseases

Institute of Scientific and Technical Information of China (English)

He Zhu; Yuanqing Tan; Qi Gu; Weifang Han; Zhongwen Li; Jason S Meyer; Baoyang Hu

2015-01-01

Objective: This study aimed to use a systematic approach to evaluate the current utilization, safety, and effectiveness of cell therapies for neurological diseases in human. And review the present regulations, considering United States (US) as a representative country, for cell transplantation in neurological disease and discuss the challenges facing the field of neurology in the coming decades. Methods:A detailed search was performed in systematic literature reviews of cellular‐based therapies in neurological diseases, using PubMed, web of science, and clinical trials. Regulations of cell therapy products used for clinical trials were searched from the Food and Drug Administration (FDA) and the National Institutes of Health (NIH). Results: Seven most common types of cell therapies for neurological diseases have been reported to be relatively safe with varying degrees of neurological recovery. And a series of regulations in US for cellular therapy was summarized including preclinical evaluations, sourcing material, stem cell manufacturing and characterization, cell therapy product, and clinical trials. Conclusions:Stem cell‐based therapy holds great promise for a cure of such diseases and will value a growing population of patients. However, regulatory permitting activity of the US in the sphere of stem cells, technologies of regenerative medicine and substitutive cell therapy are selective, theoretical and does not fit the existing norm and rules. Compiled well‐defined regulations to guide the application of stem cell products for clinical trials should be formulated.

Clinical diagnostic potentials of thyroid ultrasonography and scintigraphy; An evaluation

Energy Technology Data Exchange (ETDEWEB)

Torizuka, Tatsuo; Kasagi, Kanji; Hatabu, Hiroto; Misaki, Takashi; Iida, Yasuhiro; Konishi, Junji (Kyoto Univ. (Japan). Hospital); Endo, Keigo

1993-06-01

This prospective study was designed to evaluate the potential contributions of high resolution ultrasonography (US) and Tc-99m scintigraphy in the routine diagnosis of thyroid disease. The diagnostic impacts of US and Tc-99m scintigraphy results in 177 patients visiting our thyroid clinic were assessed and scored according to the following criteria: when the information provided by either test supported, confirmed or changed the initial clinical diagnosis, they received scores of 2, 3 and 4 respectively, while score 1 was given when the test itself was useless for the differential diagnosis. US identified focal lesions that both palpation and scintigraphy had failed to detect in 14 (12.1%) of 116 patients with diffuse thyroid diseases, suggesting the necessity of Hashimoto's thyroiditis, adenoma, adenocarcinoma and adenomatous goiter, and vice versa in the diagnosis of hyperthyroid and euthyroid Graves's diseases. Thus, the advantages of US over scintigraphy for morphological evaluation were confirmed. US was particularly useful for the differential diagnosis of adenomatous goiter from Hashimoto's thyroiditis or a single nodular disease. In contrast, scintigraphy gave functional images, being especially helpful for the differential diagnosis of thyrotoxicosis. (author).

Clinical evaluation of ischemic heart diagnosis

International Nuclear Information System (INIS)

Kamei, Fumio

1983-01-01

Attempt were made to detect the existence of myocardial ischemia by means of both radiographic and scintigraphic techniques. Firstly, a new polygraph was especially designed for selecting the arbitrary phases in a cardiac cycle at which the corresponding radiogram should be synchronously obtained. A comparative investigation on the difference between end-systolic and-diastolic cardiac transverse diameters revealed a remarkable difference of 3.6% in normal subjects and 0.6% in patients with ischemic heart disease. These data indicating the difference of overall heart size was reflected in local dyskinesis documentation of recently developed techniques. For daily clinical purposes, radiography of the chest based on synchronously selected phases would contribute to accurate diagnosis and treatment of heart disease. Secondly, scintigraphic display using intravenously injected thallium-201 was clinically applied. For detection of ischemia, comparative study was performed of initial image relative to selective coronary cineangiography and stress scintigraphy. The former indicated a good correlation of 90%, whereas the latter served to enhnace sensitivity. Sequential images (initial and delayed) facilitated the distinction of normal, necrotic, and ischemic areas. Scintigram was used for objective evaluation of coronary dilator (dilazep), either at immediate or follow-up stage. In the same way, it was also possible to indicate the effectiveness of sublingually given nitroglycerin by myocardial scintigram, where by significant increase of uptake was observed 20 minutes after administration. Rehabilitation after acute heart disease was discussed, especially on the peripheral effect. Ratio of the thigh muscle to myocardium shown in this study was useful fer objective evaluation. Another preliminary study is to separate normal coronary arteries from myocardial necrosis. (J.P.N.)

Clinical evaluation of ischemic heart diagnosis

Energy Technology Data Exchange (ETDEWEB)

Kamei, Fumio [Sendai Railway Hospital (Japan)

1983-09-01

Attempt were made to detect the existence of myocardial ischemia by means of both radiographic and scintigraphic techniques. Firstly, a new polygraph was especially designed for selecting the arbitrary phases in a cardiac cycle at which the corresponding radiogram should be synchronously obtained. A comparative investigation on the difference between end-systolic and-diastolic cardiac transverse diameters revealed a remarkable difference of 3.6% in normal subjects and 0.6% in patients with ischemic heart disease. These data indicating the difference of overall heart size was reflected in local dyskinesis documentation of recently developed techniques. For daily clinical purposes, radiography of the chest based on synchronously selected phases would contribute to accurate diagnosis and treatment of heart disease. Secondly, scintigraphic display using intravenously injected thallium-201 was clinically applied. For detection of ischemia, comparative study was performed of initial image relative to selective coronary cineangiography and stress scintigraphy. The former indicated a good correlation of 90%, whereas the latter served to enhance sensitivity. Sequential images (initial and delayed) facilitated the distinction of normal, necrotic, and ischemic areas. Scintigram was used for objective evaluation of coronary dilator (dilazep), either at immediate or follow-up stage. In the same way, it was also possible to indicate the effectiveness of sublingually given nitroglycerin by myocardial scintigram, where by significant increase of uptake was observed 20 minutes after administration. Rehabilitation after acute heart disease was discussed, especially on the peripheral effect. Ratio of the thigh muscle to myocardium shown in this study was useful for objective evaluation. Another preliminary study is to separate normal coronary arteries from myocardial necrosis.

Clinical outcome and radiographic results after operative treatment of Scheuermann's disease

NARCIS (Netherlands)

Poolman, R. W.; Been, H. D.; Ubags, L. H.

2002-01-01

The aim of this prospective study was to evaluate radiographic findings, patient satisfaction and clinical outcome, and to report complications and instrumentation failure after operative treatment of Scheuermann's disease using a combined anterior and posterior spondylodesis. The loss of sagittal

AACE/ACE Disease State Clinical Review: Medical Management of Cushing Disease.

Science.gov (United States)

Hamrahian, Amir H; Yuen, Kevin C J; Hoffman, Andrew R

2014-07-01

To review available medical therapies for patients with Cushing disease and to provide a roadmap for their use in clinical practice. PubMed searches were performed to identify all of the available published data on medical management of Cushing disease. Medical therapy is usually not the first-line treatment for patients with Cushing disease but may be used to improve clinical manifestations of Cushing disease in patients who are not suitable candidates for surgery, following unsuccessful surgery or recurrence, or as a "bridge therapy" in those who have undergone radiotherapy. Medical therapy may also be used in preoperative preparation of patients with severe disease. Current available medical options for patients with Cushing disease include centrally acting agents, steroidogenesis inhibitors, and a glucocorticoid receptor antagonists. At present, there are no head-to-head studies comparing the efficacy, tolerability, and safety of different U.S. Food and Drug Administration (FDA)- and non-FDA-approved drugs in patients with Cushing disease. With the initiation of new studies and the completion of ongoing clinical trials, the number of FDA-approved drugs for medical treatment of Cushing disease is expected to increase. Medical therapy has an important adjunctive role in the management of patients with Cushing disease. The decision to initiate medical treatment depends on many factors, including patient characteristics and preference. Long-term studies are needed to better define the clinical efficacy, safety, and tolerability of medical treatment of Cushing disease, including the role of combination therapies.

Haematology and Clinical Biochemistry Findings Associated with Equine Diseases - a Review

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Onyinyechukwu A. AGINA

2017-03-01

Full Text Available The course and outcome of a disease process is dependent upon factors such as the disease-causing agent and its cell tropism, defense mechanisms of the host, genetic resistance of the species or breed affected, as well as the age, nutritional status and hormonal levels of the affected animal. When haematology, clinical biochemistry and cytology test results are combined with other laboratory procedures, complete physical examination and also with the history of the patient, a veterinarian is well armed to arrive at a definitive diagnosis, make a certain prognosis (good, poor or guarded and can also make a concluding statement on the efficacy of the instituted therapy. In clinical biochemistry, demonstration of specific enzyme activity and concentration of analytes in serum/plasma facilitates the disease diagnosis. Also, evaluation of haematology, clinical biochemistry and diagnostic cytology tests can help establish the presence or absence of diseases of internal organs, and by serial performance of these tests, may help to determine whether a disease process remains static, progressive or regressive. This review therefore provides the haematological, serum biochemical and cytological characteristics of diseases caused by the main bacteria, viruses, fungi, protozoa, helminths, arthropods, nutritional deficiencies, endocrine disturbances, neoplasm, allergy, toxins (phytoxins and zootoxins and inorganic poisons in horses.

Clinical observation of radiation urinary bladder disease

International Nuclear Information System (INIS)

Jin Yuke; Liu Libo; Zhang Haiying; Liang Shuo; Chen Dawei; Wu Zhenfeng; Dong Lihua; Lu Xuejun

2004-01-01

Objective: Clinical characteristic, diagnosis and treatment of radiation urinary bladder disease induced by radiation therapy for cancers in the pelvis were inquired into for providing diagnostic basis. Methods: Statistical analysis for the clinical cases was carried out. Results: The incidence of radiation bladder diseases induced by radiation therapy of cervix cancer are about 0.8%-2.96%, with an average of 2.14%. Radiation bladder disease is divided into acute radiation cystitis, chronic radiation cystitis and radiation vesical fistula. Chronic radiation cystitis is seen most often in the clinic and its main clinical symptom is painless macroscopic hematuria, which is again subdivided into slight and severe degrees. Diagnosis should include history of exposure to radiation, which dose exceed the dose threshold, and typical clinical characteristics. Conclusion: The characteristics, types and diagnostic basis of radiation urinary bladder disease analyzed in this study can provide the reference for drawing up diagnostic standard

Evaluation of Methacholine Challenge Test Results in Chronic Cough Patients Referring to Clinic of Pulmonary Disease

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Derakhshan Deilami Gholamreza

2009-10-01

Full Text Available Chronic cough is a common problem in patients visiting physicians and its prevalence in different populations range from 3 to 40%. Postnasal drip, asthma and gastroesophageal reflux are the known cause of chronic cough. Although diagnosis of asthma is usually made by clinical signs and spirometeric results, methacholine challenge test is a good diagnostic test in patients who show normal physical examination and spirometeric results. In this study, the results of methacholine challenge test in chronic cough patients are investigated. This is a cross sectional study performed on patients suffering from chronic cough (over 8 weeks, who went to Pulmonary Disease Clinic of Imam Khomeini Hospital in 2006. Postnasal drip, gastroesophageal reflux was evaluated and ruled out in all patients. Then they were tested by methacholine inhalation using low to high doses of methacholine. The results of test was defined as 20% fall in FEV1 and its relationship with age, sex, history of allergic disease, family history of asthma and smoking status was investigated. 81 patients (36 female and 45 male entered this study who had mean age of 32.5 ± 13.06 years. 81.5% of patients had never smoked or closed contact with smokers, 6.2% were passive smokers, 8.6% were smokers and 3.7% had quit smoking. 37% had suffered from chronic cough less than 6 months, 11% for 6-11 months and 52% for more than 12 months. In 26% of patients, family history of asthma was present and 34.5% had a history of one type of allergy. In 29.5% the results of methacholine challenge test was positive, among them 45.8% showed an intense response and 54.2% a moderate response. The test results and its intensity had no statistically significant relationship with age, sex, smoking status, the duration of cough and family history of asthma, but the relationship between methacholine challenge test and the history of allergic disease was significant. Methacholine challenge test can be used as a

Patient satisfaction with nurse-led chronic kidney disease clinics: A multicentre evaluation.

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Coleman, Sonya; Havas, Kathryn; Ersham, Susanne; Stone, Cassandra; Taylor, Berndatte; Graham, Anne; Bublitz, Lorraine; Purtell, Louise; Bonner, Ann

2017-03-01

There is growing international evidence that nurse-led chronic kidney disease (CKD) clinics provide a comprehensive approach to achieving clinical targets effective in slowing the progression of CKD. Across Queensland, Australia, these clinics have been established in many renal outpatient departments although patient satisfaction with these clinics is unknown. To measure patient satisfaction levels with CKD nurse-led clinics. This was a cross-sectional study undertaken at five clinics located in metropolitan, regional and remote hospitals in Queensland. Participants were >18 years of age (no upper age limit) with CKD (non-dialysis) who attended CKD nurse-led clinics over a six month period (N = 873). They completed the Nurse Practitioner Patient Satisfaction questionnaire which was modified for CKD. The response rate was 64.3 % (n = 561); half of the respondents were male (55.5 %), there was a median age range of 71-80 years (43.5 %) and most respondents were pensioners or retired (84.2 %). While the majority reported that they were highly satisfied with the quality of care provided by the nurse (83.8 %), we detected differences in some aspects of satisfaction between genders, age groups and familiarity with the nurse. Overall, patients' comments were highly positive with a few improvements to the service being suggested; these related to car-parking, providing more practical support, and having accessible locations. In an era of person-centred care, it is important to measure patient satisfaction using appropriate and standardised questionnaires. Our results highlight that, to improve services, communication strategies should be optimised in nurse-led clinics. © 2017 European Dialysis and Transplant Nurses Association/European Renal Care Association.

Evaluation of a clinical decision support tool for osteoporosis disease management: protocol for an interrupted time series design.

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Kastner, Monika; Sawka, Anna; Thorpe, Kevin; Chignel, Mark; Marquez, Christine; Newton, David; Straus, Sharon E

2011-07-22

Osteoporosis affects over 200 million people worldwide at a high cost to healthcare systems. Although guidelines on assessing and managing osteoporosis are available, many patients are not receiving appropriate diagnostic testing or treatment. Findings from a systematic review of osteoporosis interventions, a series of mixed-methods studies, and advice from experts in osteoporosis and human-factors engineering were used collectively to develop a multicomponent tool (targeted to family physicians and patients at risk for osteoporosis) that may support clinical decision making in osteoporosis disease management at the point of care. A three-phased approach will be used to evaluate the osteoporosis tool. In phase 1, the tool will be implemented in three family practices. It will involve ensuring optimal functioning of the tool while minimizing disruption to usual practice. In phase 2, the tool will be pilot tested in a quasi-experimental interrupted time series (ITS) design to determine if it can improve osteoporosis disease management at the point of care. Phase 3 will involve conducting a qualitative postintervention follow-up study to better understand participants' experiences and perceived utility of the tool and readiness to adopt the tool at the point of care. The osteoporosis tool has the potential to make several contributions to the development and evaluation of complex, chronic disease interventions, such as the inclusion of an implementation strategy prior to conducting an evaluation study. Anticipated benefits of the tool may be to increase awareness for patients about osteoporosis and its associated risks and provide an opportunity to discuss a management plan with their physician, which may all facilitate patient self-management.

Evaluation of the Short Parkinson's Evaluation Scale: a new friendly scale for the evaluation of Parkinson's disease in clinical drug trials.

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Rabey, J M; Bass, H; Bonuccelli, U; Brooks, D; Klotz, P; Korczyn, A D; Kraus, P; Martinez-Martin, P; Morrish, P; Van Sauten, W; Van Hilten, B

1997-08-01

The extensive use of the Unified Parkinson's Disease Rating Scale (UPDRS) has revealed low interrater reliability in some items and redundancy in others. In view of these shortcomings, we have structured a new scale that includes a zero-to three-point scale for each item in the evaluation of PD. The mental axis includes memory, thought disorders, and depression. Activities of daily living (ADL) includes eight items: speech, eating, feeding, dressing, hygiene, handwriting, walking, and turning in bed. The motor examination includes eight items: speech, tremor, rest and posture, rigidity, finger tapping, arising from chair, gait, and postural stability. Complications of therapy were also included: dyskinesias, dystonia, motor fluctuations, and freezing episodes, collected by history. In addition, a global scoring for motor fluctuations that should complement the Hoehn and Yahr Scale was incorporated. In this report, we present a statistical analysis of the ADL, motor evaluation, and complications of therapy sections. Concerning the interrater reliability mean, Kendall's W values were >0.9 for most of the items in the Short Parkinson's Evaluation Scale (SPES). Kendall's W <0.8 (motor evaluation) was found for two items of the SPES and nine items of the UPDRS. The mean interrater reliability for both scales across all seven centers (seven Kendall's W for seven centers) (Mann-Whitney test) showed no statistical differences between the scales. Spearman's correlations between items of both scales were significant. Factor analysis of the SPES and UPDRS data revealed a four-factor solution that explained approximately 60% of the data. All participating centers found the SPES easier to apply and quicker to complete, when compared with the UPDRS. The results obtained strongly favor the introduction of SPES for clinical practice.

Evaluation of clinical characteristics and prognosis of chronic pulmonary aspergillosis depending on the underlying lung diseases: Emphysema vs prior tuberculosis.

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Koyama, Kazuya; Ohshima, Nobuharu; Suzuki, Junko; Kawashima, Masahiro; Okuda, Kenichi; Sato, Ryota; Suzukawa, Maho; Nagai, Hideaki; Matsui, Hirotoshi; Ohta, Ken

2015-11-01

There have been scarce data evaluating the differences of clinical characteristics and prognosis of chronic pulmonary aspergillosis (CPA) depending on underlying pulmonary diseases. We tried to clarify them in CPA patients who had pulmonary emphysema or previous pulmonary tuberculosis. We reviewed and evaluated CPA patients diagnosed between 2007 and 2013 with pulmonary emphysema (PE group; n = 29), with previous pulmonary tuberculosis (PT group; n = 47) and with combination of these 2 underlying conditions (CTE group; n = 24). In CT findings, fungus balls were rare in PE group (7% in PE group and 36% in PT group; p = 0.006). Compared with PT group, PE group patients exhibited more frequent preceding antibiotics administration (45% vs 11%; p = 0.002) and fever (52% vs 17%; p = 0.002), less frequent hemosputum (24% vs 57%; p = 0.008), and more frequent consolidations in imaging (79% vs 38%; p = 0.001) and respiratory failure (34% vs 13%; p = 0.020), possibly suggesting more acute clinical manifestations of CPA in emphysematous patients. Trend of the differences between PT and PE group was not changed when patients with fungal balls were excluded. Multivariate Cox regression analysis of risks for all-cause mortality revealed age (HR, 1.079; p = 0.002) and emphysema (HR, 2.45; p = 0.040) as risk factors. Assessment of underlying lung diseases is needed when we estimate prognosis and consider treatment of CPA patients. Particularly, emphysematous patients can be presented as refractory pneumonia and show poor prognosis. Copyright © 2015 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Patient-Reported Outcome Measures for Use in Clinical Trials and Clinical Practice in Inflammatory Bowel Diseases: A Systematic Review.

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de Jong, Marin J; Huibregtse, Roxanne; Masclee, Ad A M; Jonkers, Daisy M A E; Pierik, Marie J

2018-05-01

Mucosal inflammation must be carefully monitored to improve the long-term outcomes of patients with inflammatory bowel diseases (IBD). Patient-reported outcome measures (PROMs) are used increasingly to monitor disease activity in clinical practice and as endpoints in clinical trials. We performed a systematic review to provide an overview of the available PROMs on IBD activity and to evaluate their diagnostic value. A systematic search of the PubMed, Medline, Cochrane library, and Embase databases using defined keywords, identified 973 articles. These were screened by 2 independent reviewers, and 37 articles on development or validation of PROMs to assess IBD activity were identified for further analysis. Based on the recommendations of the Food and Drug Administration (FDA), the following measurement properties were evaluated: content, construct, and criterion validity; reliability; and responsiveness to change. In addition, data on ease of use in clinical practice were collected. Seventeen articles presenting 20 different PROMs were included the final analysis, although none met all the FDA-recommended criteria. Only 2 PROMs (patient-reported Harvey Bradshaw Index and Simple Clinical Colitis Activity Index scores) reported patient involvement during its development. Only 6 PROMs (patient-reported global assessment, patient assessment of disease activity, mobile health index for Crohn's disease, mobile health index for ulcerative colitis, patient-reported outcome derived from the Mayo score, and the 6-point Mayo score) were validated as markers of IBD activity, using findings from endoscopy as the reference standard; these PROMs identified patients with mucosal inflammation with area under the curve values of 0.63-0.82. The mobile health index for CD and UC scores had the best measurement properties for use in clinical practice and in clinical trials. In a systematic review, we identified more than 20 PROMS that have been developed and tested for their ability to

EVALUATION OF METABOLIC SYNDROME, ITS CORRELATION WITH CLINICAL AND ANGIOGRAPHIC PROFILE IN PATIENTS WITH CORONARY ARTERY DISEASE- A PROSPECTIVE STUDY AT TERTIARY HOSPITAL

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Eruvaram Srikanth

2016-10-01

Full Text Available BACKGROUND Coronary artery disease has a major share for cardiovascular disease in a developing country like India, which is in epidemic proportion. There are number of risk factors for development of coronary artery disease. According to INTERHEART study, there were 9 modifiable risk factors with population attributable risk of 90 percent in men and 94 percent in women. Metabolic syndrome cluster of risk factors, which include insulin resistance, subclinical inflammation, increased future risk of diabetes and coronary artery disease. In south Asian people are increased tendency to develop metabolic syndrome because of their high percentage of body fat, abdominal obesity and insulin resistance. Metabolic syndrome has more mortality and morbidity from CAD. It is desirable identifying this subset of patients, which could improve patient or physician adherence to risk-reducing behaviours or interventions and improve clinical outcomes. There are reports in literature on association inflammatory markers and insulin resistance with severity of disease in CAD. There are few studies, which correlated severity of CAD with SYNTAX score in patients with metabolic syndrome, so in these study prospectively evaluated clinical and angiographic profile in patients with CAD in subset patients with metabolic syndrome, CAD severity was assessed with SYNTAX scoring system and thrombus burden was evaluated. MATERIALS AND METHODS Among 101 patients who were diagnosed to have metabolic syndrome according to ATP III guidelines were evaluated in the study. All patients were evaluated by clinical examination including waist circumference, body mass index, routine blood investigations were carried out. Lipid profile, ECG and 2D echo was done. Then, patients were evaluated with coronary angiogram and among patients who underwent coronary angiography, the lesions were classified according to AHA/ACC classification into type A, type B and type C for assessing lesion

Limited accessibility to designs and results of Japanese large-scale clinical trials for cardiovascular diseases.

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Sawata, Hiroshi; Ueshima, Kenji; Tsutani, Kiichiro

2011-04-14

Clinical evidence is important for improving the treatment of patients by health care providers. In the study of cardiovascular diseases, large-scale clinical trials involving thousands of participants are required to evaluate the risks of cardiac events and/or death. The problems encountered in conducting the Japanese Acute Myocardial Infarction Prospective (JAMP) study highlighted the difficulties involved in obtaining the financial and infrastructural resources necessary for conducting large-scale clinical trials. The objectives of the current study were: 1) to clarify the current funding and infrastructural environment surrounding large-scale clinical trials in cardiovascular and metabolic diseases in Japan, and 2) to find ways to improve the environment surrounding clinical trials in Japan more generally. We examined clinical trials examining cardiovascular diseases that evaluated true endpoints and involved 300 or more participants using Pub-Med, Ichushi (by the Japan Medical Abstracts Society, a non-profit organization), websites of related medical societies, the University Hospital Medical Information Network (UMIN) Clinical Trials Registry, and clinicaltrials.gov at three points in time: 30 November, 2004, 25 February, 2007 and 25 July, 2009. We found a total of 152 trials that met our criteria for 'large-scale clinical trials' examining cardiovascular diseases in Japan. Of these, 72.4% were randomized controlled trials (RCTs). Of 152 trials, 9.2% of the trials examined more than 10,000 participants, and 42.8% examined between 1,000 and 10,000 participants. The number of large-scale clinical trials markedly increased from 2001 to 2004, but suddenly decreased in 2007, then began to increase again. Ischemic heart disease (39.5%) was the most common target disease. Most of the larger-scale trials were funded by private organizations such as pharmaceutical companies. The designs and results of 13 trials were not disclosed. To improve the quality of clinical

Limited accessibility to designs and results of Japanese large-scale clinical trials for cardiovascular diseases

Directory of Open Access Journals (Sweden)

Tsutani Kiichiro

2011-04-01

Full Text Available Abstract Background Clinical evidence is important for improving the treatment of patients by health care providers. In the study of cardiovascular diseases, large-scale clinical trials involving thousands of participants are required to evaluate the risks of cardiac events and/or death. The problems encountered in conducting the Japanese Acute Myocardial Infarction Prospective (JAMP study highlighted the difficulties involved in obtaining the financial and infrastructural resources necessary for conducting large-scale clinical trials. The objectives of the current study were: 1 to clarify the current funding and infrastructural environment surrounding large-scale clinical trials in cardiovascular and metabolic diseases in Japan, and 2 to find ways to improve the environment surrounding clinical trials in Japan more generally. Methods We examined clinical trials examining cardiovascular diseases that evaluated true endpoints and involved 300 or more participants using Pub-Med, Ichushi (by the Japan Medical Abstracts Society, a non-profit organization, websites of related medical societies, the University Hospital Medical Information Network (UMIN Clinical Trials Registry, and clinicaltrials.gov at three points in time: 30 November, 2004, 25 February, 2007 and 25 July, 2009. Results We found a total of 152 trials that met our criteria for 'large-scale clinical trials' examining cardiovascular diseases in Japan. Of these, 72.4% were randomized controlled trials (RCTs. Of 152 trials, 9.2% of the trials examined more than 10,000 participants, and 42.8% examined between 1,000 and 10,000 participants. The number of large-scale clinical trials markedly increased from 2001 to 2004, but suddenly decreased in 2007, then began to increase again. Ischemic heart disease (39.5% was the most common target disease. Most of the larger-scale trials were funded by private organizations such as pharmaceutical companies. The designs and results of 13 trials were not

High resolution pituitary gland MRI at 7.0 tesla: a clinical evaluation in Cushing's disease

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Rotte, Alexandra A.J. de; Groenewegen, Amy; Rutgers, Dik R.; Witkamp, Theo; Luijten, Peter R.; Hendrikse, Jeroen [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Zelissen, Pierre M.J. [University Medical Center Utrecht, Department of Internal Medicine (Section of Endocrinology), Utrecht (Netherlands); Meijer, F.J.A. [Radboud University Medical Center, Department of Radiology and Nuclear Medicine, Nijmegen (Netherlands); Lindert, Erik J. van [Radboud University Medical Center, Department of Neurosurgery, Nijmegen (Netherlands); Hermus, Ad [Radboud University Medical Center, Department of Internal Medicine (Section of Endocrinology), Nijmegen (Netherlands)

2016-01-15

To evaluate the detection of pituitary lesions at 7.0 T compared to 1.5 T MRI in 16 patients with clinically and biochemically proven Cushing's disease. In seven patients, no lesion was detected on the initial 1.5 T MRI, and in nine patients it was uncertain whether there was a lesion. Firstly, two readers assessed both 1.5 T and 7.0 T MRI examinations unpaired in a random order for the presence of lesions. Consensus reading with a third neuroradiologist was used to define final lesions in all MRIs. Secondly, surgical outcome was evaluated. A comparison was made between the lesions visualized with MRI and the lesions found during surgery in 9/16 patients. The interobserver agreement for lesion detection was good at 1.5 T MRI (κ = 0.69) and 7.0 T MRI (κ = 0.62). In five patients, both the 1.5 T and 7.0 T MRI enabled visualization of a lesion on the correct side of the pituitary gland. In three patients, 7.0 T MRI detected a lesion on the correct side of the pituitary gland, while no lesion was visible at 1.5 T MRI. The interobserver agreement of image assessment for 7.0 T MRI in patients with Cushing's disease was good, and lesions were detected more accurately with 7.0 T MRI. (orig.)

High resolution pituitary gland MRI at 7.0 tesla: a clinical evaluation in Cushing's disease

International Nuclear Information System (INIS)

Rotte, Alexandra A.J. de; Groenewegen, Amy; Rutgers, Dik R.; Witkamp, Theo; Luijten, Peter R.; Hendrikse, Jeroen; Zelissen, Pierre M.J.; Meijer, F.J.A.; Lindert, Erik J. van; Hermus, Ad

2016-01-01

To evaluate the detection of pituitary lesions at 7.0 T compared to 1.5 T MRI in 16 patients with clinically and biochemically proven Cushing's disease. In seven patients, no lesion was detected on the initial 1.5 T MRI, and in nine patients it was uncertain whether there was a lesion. Firstly, two readers assessed both 1.5 T and 7.0 T MRI examinations unpaired in a random order for the presence of lesions. Consensus reading with a third neuroradiologist was used to define final lesions in all MRIs. Secondly, surgical outcome was evaluated. A comparison was made between the lesions visualized with MRI and the lesions found during surgery in 9/16 patients. The interobserver agreement for lesion detection was good at 1.5 T MRI (κ = 0.69) and 7.0 T MRI (κ = 0.62). In five patients, both the 1.5 T and 7.0 T MRI enabled visualization of a lesion on the correct side of the pituitary gland. In three patients, 7.0 T MRI detected a lesion on the correct side of the pituitary gland, while no lesion was visible at 1.5 T MRI. The interobserver agreement of image assessment for 7.0 T MRI in patients with Cushing's disease was good, and lesions were detected more accurately with 7.0 T MRI. (orig.)

Polysomnographic Findings and Clinical Correlates in Huntington Disease: A Cross-Sectional Cohort Study

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Piano, Carla; Losurdo, Anna; Della Marca, Giacomo; Solito, Marcella; Calandra-Buonaura, Giovanna; Provini, Federica; Bentivoglio, Anna Rita; Cortelli, Pietro

2015-01-01

Study Objectives: To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD). Design: Cross-sectional cohort study. Setting: Sleep laboratory. Patients: Thirty HD patients, 16 women and 14 men (mean age 57.3 ± 12.2 y); 30 matched healthy controls (mean age 56.5 ± 11.8 y). Interventions: Subjective sleep evaluation: Epworth Sleepiness Scale (ESS); Berlin's Questionnaire, interview for restless legs syndrome (RLS), questionnaire for REM sleep behavior disorder (RBD). Clinical evaluation: disease duration, clinical severity (unified Huntington disease motor rating scale [UHDMRS]), genetic tests. Laboratory-based full-night attended video-polysomnography (V-PSG). Measurements and Results: The duration of the disease was 9.4 ± 4.4 y, UHMDRS score was 55.5 ± 23.4, CAG repeats were 44.3 ± 4.1. Body mass index was 21.9 ± 4.0 kg/m2. No patients or caregivers reported poor sleep quality. Two patients reported symptoms of RLS. Eight patients had an ESS score ≥ 9. Eight patients had high risk of obstructive sleep apnea. At the RBD questionnaire, two patients had a pathological score. HD patients, compared to controls, showed shorter sleep, reduced sleep efficiency index, and increased arousals and awakenings. Four patients presented with sleep disordered breathing (SDB). Periodic limb movements (PLMs) during wake and sleep were observed in all patients. No episode of RBD was observed in the V-PSG recordings, and no patients showed rapid eye movement (REM) sleep without atonia. The disease duration correlated with ESS score (P Marca G, Solito M, Calandra-Buonaura G, Provini F, Bentivoglio AR, Cortelli P. Polysomnographic findings and clinical correlates in Huntington disease: a cross-sectional cohort study. SLEEP 2015;38(9):1489–1495. PMID:25845698

An Update on Renal Artery Denervation and Its Clinical Impact on Hypertensive Disease

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Aditya Bhat

2015-01-01

Full Text Available Hypertension is a globally prevalent condition, with a heavy clinical and economic burden. It is the predominant risk factor for premature cardiovascular and cerebrovascular disease, and is associated with a variety of clinical disorders including stroke, congestive cardiac failure, ischaemic heart disease, chronic renal failure, and peripheral arterial disease. A significant subset of hypertensive patients have resistant hypertensive disease. In this group of patients, catheter-based renal artery denervation has emerged as a potential therapy, with favourable clinical efficacy and safety in early trials. Additional benefits of this therapy are also being identified and include effects on left ventricular remodeling, cardiac performance, and symptom status in congestive cardiac failure. Utility of renal denervation for the management of resistant hypertension, however, has become controversial since the release of the Symplicity HTN-3 trial, the first large-scale blinded randomised study investigating the efficacy and safety of renal artery denervation. The aim of this paper is to evaluate the history, utility, and clinical efficacy of renal artery denervation technology, including an in-depth appraisal of the current literature and principal trials.

An Update on Renal Artery Denervation and Its Clinical Impact on Hypertensive Disease

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Kuang, Ye Min; Gan, Gary C. H.; Burgess, David; Denniss, Alan Robert

2015-01-01

Hypertension is a globally prevalent condition, with a heavy clinical and economic burden. It is the predominant risk factor for premature cardiovascular and cerebrovascular disease, and is associated with a variety of clinical disorders including stroke, congestive cardiac failure, ischaemic heart disease, chronic renal failure, and peripheral arterial disease. A significant subset of hypertensive patients have resistant hypertensive disease. In this group of patients, catheter-based renal artery denervation has emerged as a potential therapy, with favourable clinical efficacy and safety in early trials. Additional benefits of this therapy are also being identified and include effects on left ventricular remodeling, cardiac performance, and symptom status in congestive cardiac failure. Utility of renal denervation for the management of resistant hypertension, however, has become controversial since the release of the Symplicity HTN-3 trial, the first large-scale blinded randomised study investigating the efficacy and safety of renal artery denervation. The aim of this paper is to evaluate the history, utility, and clinical efficacy of renal artery denervation technology, including an in-depth appraisal of the current literature and principal trials. PMID:26495305

STRUCTURED CLINICAL EVALUATION

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Arabela Maria Barbosa Sampaio

2014-05-01

Full Text Available In a world experiencing profound technological and socio-political changes in areas of knowledge and capacity, healthcare can not remain static. A new kind of professional is required, whose practice is based on ethics, scientific standards, integrity, citizenship, and health promotion, who develops skills beyond healthcare, such as decision making, communication,leadership, management, and continuing education. No single method can assess all of these elements (knowledge, skills, and attitudes, and only a combination of methods is able to produce a valid evaluation. An alternative method exists: structured clinical assessments based on observation of "to do, or how to do" that aim to complete this evaluation by focusing attention on the performance of specific skills. In order to broaden the scope of evaluation methods that have been used in health education, this article, a literature review, intends to offer readers an overview of the diverse types of structured clinical evaluation, emphasizing Objective Structured Clinical Examination, the most widely used in Brazil, with a goal of advancing opportunities for health professionals to make use of this evaluative tool.

[Clinical Tests Testing New Therapies for Stargardt Disease].

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Kousal, B; Ďuďáková, Ľ; Hlavatá, L; Lišková, P

2016-02-01

To provide information on currently ongoing clinical trials for Stargardt disease. We have searched the clinical trial register (www.clinicaltrials.gov) for the keyword "Stargardt" and list active ongoing studies. There are currently eight registered clinical trials enrolling patients with Stargardt disease; all in phase I or II aiming at four mechanisms of action: inhibition of the production of vitamin A toxic dimers, gene therapy restoring wild type transcription of the ABCA4 gene, neuroprotection preventing retinal cells from oxidative damage, and replacement of the damaged retinal pigment epithelium using stem cell therapy. The basic prerequisite for enrolment in the vast majority of clinical trials is confirmation of the clinical diagnosis by mutational analysis. The wide variety of therapies that are registered as clinical trials for Stargardt disease significantly raises the possibility that effective treatments will be available in the near future for this currently incurable condition and that molecular genetic testing should be increasingly considered. Stargardt disease, clinical trial, ABCA4, mutation.

Initial experience with lung-MRI at 3.0 T: Comparison with CT and clinical data in the evaluation of interstitial lung disease activity

International Nuclear Information System (INIS)

Lutterbey, G.; Grohe, C.; Gieseke, J.; Falkenhausen, M. von; Morakkabati, N.; Wattjes, M.P.; Manka, R.; Trog, D.; Schild, H.H.

2007-01-01

Objectives: We evaluated the feasibility of highfield lung-MRI at 3.0 T. A comparison with Computed Tomography (CT) and clinical data regarding the assessment of inflammatory activity in patients with diffuse lung disease was performed. Material and methods: Prospective evaluation of 21 patients (15 males, 6 females, 43-80 y) with diffuse lung diseases who underwent clinical work-up inclusive laboratory tests, lung-function tests and transbronchial biopsy. After routine helical CT (additional 12 HRCT) a lung-MRI (3.0 Intera, Philips Medical Systems, Best, The Netherlands) using a T2-weighted, cardiac and respiratory triggered Fast-Spinecho-Sequence (TE/TR = 80/1500-2500 ms, 22 transverse slices, 7/2 mm slice-thickness/-gap) was performed. A pneumologist classified the cases into two groups: A = temporary acute interstitial disease or chronic interstitial lung disease with acute episode or superimposed infection/B = burned out interstitial lung disease without activity. Two blinded CT-radiologists graded the cases in active/inactive disease on the basis of nine morphological criteria each. A third radiologist rated the MRI-cases as active/inactive, depending on the signal-intensities of lung tissues. Results: The pneumologist classified 14 patients into group A and 7 patients into group B. Using CT, 6 cases were classified as active, 15 cases as inactive disease. With MRI 12 cases were classified as active and 9 cases as inactive. In the complete group of 21 patients MRI decisions and CT decisions respectively were false positive/false negative/correct in 2/4/15 respectively 0/8/13 cases. Correct diagnoses were obtained in 72% (MRI) respectively 62% (CT). In the subgroup of 12 cases including HRCT, MRI respectively CT were false positive/false negative/correct in 2/1/9 respectively 0/5/7 cases. Correct diagnoses were obtained in 75% (MRI) respectively 58% (CT). Conclusion: Highfield MRI of the lung is feasible and performed slightly better compared to CT in the

Initial experience with lung-MRI at 3.0 T: Comparison with CT and clinical data in the evaluation of interstitial lung disease activity

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Lutterbey, G. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany)]. E-mail: goetz.lutterbey@ukb.uni-bonn.de; Grohe, C. [Department of Internal Medicine, University of Bonn (Germany); Gieseke, J. [PHILIPS Medical Systems, Best (Netherlands); Falkenhausen, M. von [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Morakkabati, N. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Wattjes, M.P. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Manka, R. [Department of Internal Medicine, University of Bonn (Germany); Trog, D. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany); Schild, H.H. [Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn (Germany)

2007-02-15

Objectives: We evaluated the feasibility of highfield lung-MRI at 3.0 T. A comparison with Computed Tomography (CT) and clinical data regarding the assessment of inflammatory activity in patients with diffuse lung disease was performed. Material and methods: Prospective evaluation of 21 patients (15 males, 6 females, 43-80 y) with diffuse lung diseases who underwent clinical work-up inclusive laboratory tests, lung-function tests and transbronchial biopsy. After routine helical CT (additional 12 HRCT) a lung-MRI (3.0 Intera, Philips Medical Systems, Best, The Netherlands) using a T2-weighted, cardiac and respiratory triggered Fast-Spinecho-Sequence (TE/TR = 80/1500-2500 ms, 22 transverse slices, 7/2 mm slice-thickness/-gap) was performed. A pneumologist classified the cases into two groups: A = temporary acute interstitial disease or chronic interstitial lung disease with acute episode or superimposed infection/B = burned out interstitial lung disease without activity. Two blinded CT-radiologists graded the cases in active/inactive disease on the basis of nine morphological criteria each. A third radiologist rated the MRI-cases as active/inactive, depending on the signal-intensities of lung tissues. Results: The pneumologist classified 14 patients into group A and 7 patients into group B. Using CT, 6 cases were classified as active, 15 cases as inactive disease. With MRI 12 cases were classified as active and 9 cases as inactive. In the complete group of 21 patients MRI decisions and CT decisions respectively were false positive/false negative/correct in 2/4/15 respectively 0/8/13 cases. Correct diagnoses were obtained in 72% (MRI) respectively 62% (CT). In the subgroup of 12 cases including HRCT, MRI respectively CT were false positive/false negative/correct in 2/1/9 respectively 0/5/7 cases. Correct diagnoses were obtained in 75% (MRI) respectively 58% (CT). Conclusion: Highfield MRI of the lung is feasible and performed slightly better compared to CT in the

A clinical approach to Lyme disease.

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Nadelman, R B; Wormser, G P

1990-05-01

Lyme disease (also known as Lyme borreliosis) is an emerging, newly described infectious disease with diverse clinical manifestations. The disease is caused by the spirochetal agent Borrelia burgdorferi, which is transmitted to humans by the bite of certain species of Ixodes ticks harboring the organism. The most readily identifiable clinical feature is the distinctive skin lesion, erythema migrans. If recently infected patients go untreated, approximately 15% will develop neurologic conditions (most commonly facial nerve palsy), 8% will develop myocarditis (typically with heart block), and 60% will develop migratory mono- or pauci-articular arthritis. Diagnosis depends on clinical suspicion, recognition of the characteristic signs and symptoms, and appropriate testing for antibody to B. burgdorferi. Serology for Lyme disease, although in need of better standardization, is most useful in diagnosing patients with manifestations of Lyme disease other than erythema migrans. All manifestations of Lyme disease are potentially treatable with either a beta-lactam antibiotic (for instance penicillin, amoxicillin, or ceftriaxone) or a tetracycline preparation. However, the optimal antimicrobial regimen, including choice of drug, drug dose, route of administration, and length of therapy, is unknown. Other important areas for future research include Ixodes biology and control, improved laboratory tests for diagnosis and for assessing response to therapy, and vaccine development.

Radiologic evaluation of nonalcoholic fatty liver disease

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Lee, Seung Soo; Park, Seong Ho

2014-01-01

Nonalcoholic fatty liver disease (NAFLD) is a frequent cause of chronic liver diseases, ranging from simple steatosis to nonalcoholic steatohepatitis (NASH)-related liver cirrhosis. Although liver biopsy is still the gold standard for the diagnosis of NAFLD, especially for the diagnosis of NASH, imaging methods have been increasingly accepted as noninvasive alternatives to liver biopsy. Ultrasonography is a well-established and cost-effective imaging technique for the diagnosis of hepatic steatosis, especially for screening a large population at risk of NAFLD. Ultrasonography has a reasonable accuracy in detecting moderate-to-severe hepatic steatosis although it is less accurate for detecting mild hepatic steatosis, operator-dependent, and rather qualitative. Computed tomography is not appropriate for general population assessment of hepatic steatosis given its inaccuracy in detecting mild hepatic steatosis and potential radiation hazard. However, computed tomography may be effective in specific clinical situations, such as evaluation of donor candidates for hepatic transplantation. Magnetic resonance spectroscopy and magnetic resonance imaging are now regarded as the most accurate practical methods of measuring liver fat in clinical practice, especially for longitudinal follow-up of patients with NAFLD. Ultrasound elastography and magnetic resonance elastography are increasingly used to evaluate the degree of liver fibrosis in patients with NAFLD and to differentiate NASH from simple steatosis. This article will review current imaging methods used to evaluate hepatic steatosis, including the diagnostic accuracy, limitations, and practical applicability of each method. It will also briefly describe the potential role of elastography techniques in the evaluation of patients with NAFLD. PMID:24966609

Common dental and periodontal diseases: evaluation and management.

Science.gov (United States)

Laudenbach, Joel M; Simon, Ziv

2014-11-01

Physicians may encounter patients with dental and periodontal diseases in the context of outpatient medical practice. It is important for physicians to be aware of common dental and periodontal conditions and be able to assess for the presence and severity of these diseases. This article reviews common dental and periodontal conditions, their cardinal signs and symptoms, outpatient-setting assessment techniques, as well as common methods of treatment. Physicians detecting gross abnormalities on clinical examination should refer the patient to a dentist for further evaluation and management. Copyright © 2014 Elsevier Inc. All rights reserved.

Kikuchi-Fujimoto disease: Clinical and laboratory characteristics and outcome

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P S Rakesh

2014-01-01

Full Text Available Introduction: Kikuchi-Fujimoto disease is an uncommon disorder with worldwide distribution, characterized by fever and benign enlargement of the lymph nodes, primarily affecting young adults. Awareness about this disorder may help prevent misdiagnosis and inappropriate investigations and treatment. The objective of the study was to evaluate the clinical and laboratory characteristics of histopathologically confirmed cases of Kikuchi′s disease from a tertiary care center in southern India. Materials and Methods: Retrospective analysis of all adult patients with histopathologically confirmed Kikuchi′s disease from January 2007 to December 2011 in a 2700-bed teaching hospital in South India was done. The clinical and laboratory characteristics and outcome were analyzed. Results: There were 22 histopathologically confirmed cases of Kikuchi′s disease over the 5-year period of this study. The mean age of the subjects′ was 29.7 years (SD 8.11 and majority were women (Male: female- 1:3.4. Apart from enlarged cervical lymph nodes, prolonged fever was the most common presenting complaint (77.3%. The major laboratory features included anemia (54.5%, increased erythrocyte sedimentation rate (31.8%, elevated alanine aminotransferase (27.2% and elevated lactate dehydrogenase (LDH (31.8%. Conclusion: Even though rare, Kikuchi′s disease should be considered in the differential diagnosis of young individuals, especially women, presenting with lymphadenopathy and prolonged fever. Establishing the diagnosis histopathologically is essential to avoid inappropriate investigations and therapy.

Clinical manifestations and management of Gaucher disease.

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Linari, Silvia; Castaman, Giancarlo

2015-01-01

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.

Postoperative MRI evaluation of anorectal malformations with clinical correlation

International Nuclear Information System (INIS)

Fukuya, T.; Honda, H.; Kubota, M.; Hayashi, T.; Kawashima, A.; Tateshi, Y.; Shono, T.; Suita, S.; Masuda, K.

1993-01-01

Sixteen postoperative patients with anorectal malformation were evaluated by MRI, and the results compared with the clinical assessment. Patients were classified into three groups - good (group 1, n = 10), fair (group 2, n = 3) and poor (group 3, n = 3) - on the basis of Kelly's clinical score of incontinence. The degree of development of the puborectalis and external sphincter muscles and the levator hammock was evaluated on MRI in comparison with patients without anorectal disease. The proportions of fair or poor development of the muscles were 37% in group 1,22% in group 2 and 67% in group 3. Although fair or poor development of the muscles was seen more frequently in group 3, there was no statistically significant difference between groups. However, poorly developed muscles were seen only in patients with fair or poor clinical scores. The difference in the anorectal angle measured on sagittal MRI images between patients in group 1 and groups 2 or 3 was significant. Our study indicates that MRI evaluation based solely on muscle development can be misleading, and measurement of the anorectal angle should be included in the MRI evaluation. (orig.)

Clinical and computerized evaluation in study of temporo-mandibular joint intracapsular disease.

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Ciavarella, D; Mastrovincenzo, M; Sabatucci, A; Parziale, V; Granatelli, F; Violante, F; Bossù, M; Lo Muzio, L; Chimenti, C

2010-03-01

In this work authors show a diagnostic criteria in study of TMID: neuro occlusal clinical evaluation (NOE), T-Scan 2 system and surface electromyography (sEMG). Nine patients 25-30 years old with TMID problem and 9 healthy group control have been selected and examined. On each patients it has been performed NOE, T-Scan and sEMG test. NOE has been calculated on each patient photos lateral mandibular excursion angle called: masticatory functional angle (AFM). T-Scan System is a computerized occlusal analyzer that provide in-depth understanding of the overall balance of the occlusion. At the same time of T-Scan record sEMG tests, in resting position and in maximum clench, have been performed. In healthy control there were no AFM difference. In no healthy group there were difference between the two AFM greater than 6 degrees. T-Scan COF showed how in healthy group control there was never a difference of COF greater than 5%. In no healthy group the difference were greater than 5% Pjoint sound than the balance side P<0.001. sEMG show how in TMID patients maximum masseter activation is always lower than maximum masseter activation of healthy subjects P<0.001. Neuro occlusal clinical evaluation (NOE) in TMID patients is supported by instrumental evaluation.

Evaluation of Wet Cupping Therapy: Systematic Review of Randomized Clinical Trials.

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Al Bedah, Abdullah M N; Khalil, Mohamed K M; Posadzki, Paul; Sohaibani, Imen; Aboushanab, Tamer Shaaban; AlQaed, Meshari; Ali, Gazzaffi I M

2016-10-01

Wet cupping is a widely used traditional therapy in many countries, which justifies a continuous scientific evaluation of its efficacy and safety. To perform a systematic review to critically evaluate and update the available evidence of wet cupping in traditional and complementary medicine. Ten electronic databases were searched from their inceptions to February 2016. Included studies were randomized clinical trials (RCTs) that evaluated wet cupping against any type of control interventions in patients with any clinical condition, as well as healthy individuals. Cochrane risk of bias tool was used to appraise the included RCTs. Fourteen RCTs met the eligibility criteria. The included studies evaluated the following clinical conditions: nonspecific low back pain (NSLBP), hypertension, brachialgia, carpal tunnel syndrome (CTS), chronic neck pain, metabolic syndrome, migraine headaches, oxygen saturation in smokers with chronic obstructive pulmonary disease (COPD), and oral and genital ulcers due to Behçet disease. Two RCTs evaluated physiologic and biochemical parameters of healthy individuals. Overall, 9 RCTs favored wet cupping over various control interventions in NSLBP (n = 2), hypertension (n = 1), brachialgia (n = 1), CTS (n = 1), chronic neck pain (n = 2), oxygen saturation in smokers with COPD (n = 1), and oral and genital ulcers due to Behçet disease (n = 1). Five RCTs showed no statistically significant between-group differences: NSLBP (n = 1), metabolic syndrome (n = 1), migraine headaches (n = 1), and physiologic and biochemical parameters of healthy individuals (n = 2). Included RCTs had a variable risk of bias across all domains and suffered methodologic limitations. There is a promising evidence in favor of the use of wet cupping for musculoskeletal pain, specifically NSLBP, neck pain, CTS, and brachialgia. Better-quality trials are needed to generate solid evidence and firmly inform policy makers.

Laterality of repetitive finger movement performance and clinical features of Parkinson's disease.

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Stegemöller, Elizabeth; Zaman, Andrew; MacKinnon, Colum D; Tillman, Mark D; Hass, Chris J; Okun, Michael S

2016-10-01

Impairments in acoustically cued repetitive finger movement often emerge at rates near to and above 2Hz in persons with Parkinson's Disease (PD) in which some patients move faster (hastening) and others move slower (bradykinetic). The clinical features impacting this differential performance of repetitive finger movement remain unknown. The purpose of this study was to compare repetitive finger movement performance between the more and less affected side, and the difference in clinical ratings among performance groups. Forty-one participants diagnosed with idiopathic PD completed an acoustically cued repetitive finger movement task while "on" medication. Eighteen participants moved faster, 10 moved slower, and 13 were able to maintain the appropriate rate at rates above 2Hz. Clinical measures of laterality, disease severity, and the UPDRS were obtained. There were no significant differences between the more and less affected sides regardless of performance group. Comparison of disease severity, tremor, and rigidity among performance groups revealed no significant differences. Comparison of posture and postural instability scores revealed that the participants that demonstrated hastening had worse posture and postural instability scores. Consideration of movement rate during the clinical evaluation of repetitive finger movement may provide additional insight into varying disease features in persons with PD. Copyright © 2016 Elsevier B.V. All rights reserved.

[Clinical stages of patients with Alzheimer disease treated in specialist clinics in Spain. The EACE study].

Science.gov (United States)

Alom Poveda, J; Baquero, M; González-Adalid Guerreiro, M

2013-10-01

The diagnostic paradigm of Alzheimer disease (AD) is changing; there is a trend toward diagnosing the disease in its early stages, even before the complete syndrome of dementia is apparent. The clinical stage at which AD is usually diagnosed in our area is unknown. Therefore, the purpose of this study is to describe the clinical stages of AD patients at time of diagnosis. Multicentre, observational and cross-sectional study. Patients with probable AD according to NINCDS-ARDRA criteria, attended in specialist clinics in Spain, were included in the study. We recorded the symptom onset to evaluation and symptom onset to diagnosis intervals and clinical status of AD (based on MMSE, NPI questionnaire, and CDR scale). Participants in this study included 437 specialists representing all of Spain's autonomous communities and a total of 1,707 patients, of whom 1,694 were included in the analysis. Mean MMSE score was 17.6±4.8 (95% CI:17.4-17.9). Moderate cognitive impairment (MMSE between 10 and 20) was detected in 64% of the patients, and severe cognitive impairment (MMSEde Neurología. Published by Elsevier Espana. All rights reserved.

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

Science.gov (United States)

Kallenberg, K.; Summers, D. M.; Romero, C.; Taratuto, A.; Heinemann, U.; Breithaupt, M.; Varges, D.; Meissner, B.; Ladogana, A.; Schuur, M.; Haik, S.; Collins, S. J.; Jansen, Gerard H.; Stokin, G. B.; Pimentel, J.; Hewer, E.; Collie, D.; Smith, P.; Roberts, H.; Brandel, J. P.; van Duijn, C.; Pocchiari, M.; Begue, C.; Cras, P.; Will, R. G.; Sanchez-Juan, P.

2009-01-01

Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt–Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Patients with sporadic Creutzfeldt–Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt–Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt–Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt–Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic

Clinical utility of ustekinumab in Crohn’s disease

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Kotze PG

2018-02-01

Full Text Available Paulo Gustavo Kotze,1,2 Christopher Ma,1 Abdulelah Almutairdi,1,3 Remo Panaccione1 1Inflammatory Bowel Disease Unit, Division of Gastroenterology and Hepatology, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada; 2Inflammatory Bowel Disease Outpatient Clinics, Colorectal Surgery Unit, Catholic University of Paraná, Curitiba, Brazil; 3Department of Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia Abstract: The introduction of anti-tumor necrosis factor (TNF therapy marked an important milestone in the management of moderate-to-severe Crohn’s disease (CD. However, there remains a pressing demand for alternative therapeutic options for patients with primary nonresponse, secondary loss of response, or intolerable side effects to conventional treatment and TNF antagonists. Ustekinumab (UST is a fully human IgG1κ monoclonal antibody that inhibits the p40 subunit shared by the proinflammatory cytokines, the interleukin (IL-12 and -23. This blockade leads to dampening of the inflammatory cascade and differentiation of inflammatory T cells. The clinical development program for UST in CD includes dose finding Phase II (Crohn’s Evaluation of Response to Ustekinumab Anti-Interleukin-12/23 for Induction [CERTIFI] and the pivotal Phase III (UNITI trials that demonstrated both the clinical efficacy and safety in anti-TNF-naive and anti-TNF-exposed patients. Real-world evidence has further defined the role of UST in CD management. In this review, we discuss the mechanism of action of UST, describe the results of the randomized controlled trials with this agent, and review the real-world efficacy and safety data from observational cohorts. Finally, we identify areas of future research in the IL-12/23 inflammatory pathway and discuss the positioning of this novel therapeutic option in CD treatment algorithms. Keywords: ustekinumab, Crohn’s disease, interleukin

Moyamoya Disease Clinical Course and Severity in Childhood

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Ayse Kacar Bayram

2016-01-01

Full Text Available Aim: Moyamoya disease (MMD is a rare, progressive and oclusive cerebrovascular disorder, predominantly affecting the terminal segment of the internal carotid arteries (ICA and its main branches. The purpose of this study is to evaluate the clinical course and severity of MMD in pediatric patients. Material and Method: We examined 5 consecutive pediatric patients with MMD, focusing on clinical and radiological features, the therapy and outcome over the 58-month follow-up period. Results: The study population consisted of 3 boys and 2 girls. The mean age at diagnosis of patients was 7.2 ± 3.4 years (age range: 3-10 years. The mean duration of follow-up was 30.4 ± 17.4 months (follow-up interval: 12-58 months. Neurological findings at presentation included: motor deficit in 4 patients (80.0%, epileptic seizures in 2 patients (40.0%, movement disorders in 3 patients (60.0%, and headache in 1 patients (20.0%. There was areas of infarction on brain MRI in all patients. Angiographic findings included: internal carotid artery stenosis in all patients, anterior cerebral artery stenosis in 3 patients, middle cerebral artery stenosis in 3 patients, posterior cerebral artery stenosis in 2 patients, and vertebral artery stenosis in 1 patient. Enoxaparine therapy was started to all patients. Subdural hematoma developed in 1 patient during follow-up. Cerebral infarctions recurred despite medical treatment in 4 patients. Discussion: Although this disease is rare, it is an important cause of pediatric stroke. MMD shows different clinical course and disease severity in childhood. Early diagnosis and appropriate treatment are crucial.

Clinical Practice Guidelines for Rare Diseases: The Orphanet Database.

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Sonia Pavan

Full Text Available Clinical practice guidelines (CPGs for rare diseases (RDs are scarce, may be difficult to identify through Internet searches and may vary in quality depending on the source and methodology used. In order to contribute to the improvement of the diagnosis, treatment and care of patients, Orphanet (www.orpha.net has set up a procedure for the selection, quality evaluation and dissemination of CPGs, with the aim to provide easy access to relevant, accurate and specific recommendations for the management of RDs. This article provides an analysis of selected CPGs by medical domain coverage, prevalence of diseases, languages and type of producer, and addresses the variability in CPG quality and availability. CPGs are identified via bibliographic databases, websites of research networks, expert centres or medical societies. They are assessed according to quality criteria derived from the Appraisal of Guidelines, REsearch and Evaluation (AGREE II Instrument. Only open access CPGs and documents for which permission from the copyright holders has been obtained are disseminated on the Orphanet website. From January 2012 to July 2015, 277 CPGs were disseminated, representing coverage of 1,122 groups of diseases, diseases or subtypes in the Orphanet database. No language restriction is applied, and so far 10 languages are represented, with a predominance of CPGs in English, French and German (92% of all CPGs. A large proportion of diseases with identified CPGs belong to rare oncologic, neurologic, hematologic diseases or developmental anomalies. The Orphanet project on CPG collection, evaluation and dissemination is a continuous process, with regular addition of new guidelines, and updates. CPGs meeting the quality criteria are integrated to the Orphanet database of rare diseases, together with other types of textual information and the appropriate services for patients, researchers and healthcare professionals in 40 countries.

Clinical-physiologic correlates of Alzheimer's disease and frontal lobe dementia

International Nuclear Information System (INIS)

Jagust, W.J.; Reed, B.R.; Seab, J.P.; Kramer, J.H.; Budinger, T.F.

1989-01-01

Thirty patients with degenerative dementia underwent clinical evaluation, neuropsychological testing, and single photon emission computed tomography (SPECT) with the blood flow tracer [ 123 I]-N-isopropyl-p-iodoamphetamine. Five of these patients were clinically and psychologically different from the others, demonstrating predominant behavioral disturbances with relative preservation of memory function. These five patients, who were felt to have a frontal lobe dementia (FLD), showed SPECT perfusion patterns which differed from the remaining 25 patients, who were diagnosed as having Alzheimer's disease (AD), and from 16 healthy control subjects. The FLD patients showed diminished perfusion in orbitofrontal, dorsolateral frontal, and temporal cortex relative to controls, while the AD patients showed lower perfusion in temporal and parietal cortex than controls. The FLD patients also showed hypoperfusion in both frontal cortical regions relative to AD patients. The pattern of performance on neuropsychological testing paralleled these differences in regional perfusion. These results suggest that clinical evaluation and physiological imaging may enable the differentiation of groups of degenerative dementia patients during life

Guidelines for the field evaluation of desert tortoise health and disease

Science.gov (United States)

Berry, Kristin H.; Christopher, Mary M.

2001-01-01

Field evaluation of free-ranging wildlife requires the systematic documentation of a variety of environmental conditions and individual parameters of health and disease, particularly in the case of rare or endangered species. In addition, defined criteria are needed for the humane salvage of ill or dying animals. The purpose of this paper is to describe, in detail, the preparation, procedures, and protocols we developed and tested for the field evaluation of wild desert tortoises (Gopherus agassizii). These guidelines describe: preparations for the field, including developing familiarity with tortoise behavior and ecology, and preparation of standardized data sheets; journal notes to document background data on weather conditions, temperature, rainfall, locality, and historic and recent human activities; procedures to prevent the spread of disease and parasites; data sheets for live tortoises to record tortoise identification, location, sex, body measurements and activity; health profile forms for documenting and grading physical abnormalities of tortoise posture and movements, general condition (e.g., lethargy, cachexia), external parasites, and clinical abnormalities associated with shell and upper respiratory diseases; permanent photographic records for the retrospective analysis of progression and regression of upper respiratory and eye diseases, analysis of shell lesions and evaluation of growth and age; and indications and methods for salvaging ill or dying tortoises for necropsy evaluation. These guidelines, tested on 5,000 to 20,000 tortoises over a 10 to 27 yr period, were designed to maximize acquisition of data for demographic, ecological, health and disease research projects; to reduce handling and stress of individual animals; to avoid spread of infectious disease; to promote high quality and consistent data sets; and to reduce the duration and number of field trips. The field methods are adapted for desert tortoise life cycle, behavior, anatomy

Radiological evaluation of swallowing and clinical patterns of systemic sclerosis

International Nuclear Information System (INIS)

Montesi, A.; Pesaresi, A.; Cavalli, M.L.; Serri, L.; Salmistraro, D.; Candela, M.; Gabrielli, A.

1990-01-01

Fifty-one patients with systemic sclerosis (scleroderma) were studied by means of videofluoroscopy in order to evaluate the abnormalities in the oral-pharyngeal and esophageal phases of deglutition and to correlate the radiological patterns with the clinical features of the disease. Thirteen patients (25.5%) exhibited swallowing disorders such as oral leakage, retention, penetration, mild or moderate aspiration and abnormal upper esophageal sphincter behavior. These dysfunctions were more evident in patients with esophageal motility abnormalities. A normal radiological pattern in the esophagus was not associated with swallowing alterations. Remarkably, patients with oral-pharyngeal disorders had a higher incidence of lung diseases. Forty-five patients (88%) exhibited disorders of the esophageal phase of deglutition, such as mild or severe motility abnormalities or hiatal hernia, gastro-esophageal reflux, reflux esophagitis, and stricture. Radiological findings in the esophagus can be abnormal in the early stages of the disease. On the other hand, the radiological pattern of esophageal motility can be occasionally negative in advanced or extensive disease. This indicates a discrepancy between clinical symptoms and radiological picture of the esophagus. The radiological examination of the oral-pharyngeal and esophageal phases of deglutition is important in patients with scleroderma in order to evaluate visceral involvement, motility disorders, and risk of aspiration. Such radiological information can be useful in preventing esophagitis and pulmonary complications

The clinical evaluation of CT and radionuclide examination in renal diseases

International Nuclear Information System (INIS)

Kutani, Wataru; Ishida, Hirofumi; Shirakawa, Shigetoshi; Shintaku, Takao; Funaki, Ryo

1980-01-01

One hundred and twelve cases of renal diseases were studied by computed tomography (CT) using EMI 5005/12. Of them, 60 were examined by both CT and renal scintigraphy, and comparatively evaluated. The CT units were checked before and after the contrast enhancement. Renal scintigrams were obtained by gamma cameras (PHO/GAMMA HP 6406, PHO/GAMMA LFOV) using 99 M Tc-DMSA. CT was especially useful in diagnosing the renal cysts and the hydronephrosis. Cysts in other organs (liver, spleen and pancreas) were simultaneously ascertained in polycystic diseases. CT was not helpful in diagnosing nephritis and diabetic nephropathy. Floating kidney and horse-shoe kidney were difficult to diagnose with CT. The renal scintigram was the reflection of the renal function, and was relatively more useful than CT in diagnosing horse-shoe kidney, floating kidney and nephritis, while it was not useful for non-functioning kidneys. (author)

Ankle tenosynovitis in rheumatoid arthritis: clinical and ultrasonographic evaluation

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Ana Luiza Naves Pereira

2016-11-01

Full Text Available Objective: To investigate ankle tenosynovitis in rheumatoid arthritis patients, regarding its presence, the kind of tendon involved and the concordance between clinical and ultrasound findings. Methods: Twenty patients with rheumatoid arthritis and pain or swollen ankle joint were evaluated. Tendon involvement was evaluated with ultrasound imaging. The Health Assessment Questionnaire (HAQ was performed for disability evaluation. Age, sex, disease duration, and vocational activity levels were also obtained. The statistical analysis included Fisher’s exact test. The significance level was 0.05. Results: Tenosynovitis was found in 13 of 20 (65.0% patients in 19 joints, in which 6 were bilaterally (46.1% and unilateral in 7 (53.8%. Tibialis posterior tenosynovitis was seen in nine (45.0% patients, Achilles tenosynovitis in seven (35.0%, tibialis anterior tenosynovitis in three (15.0%, and peroneal tenosynovitis in three (15.0% patients. We found concordance between symptomatic ankle and ultrasonographic findings in 92.3% of the patients with tenosynovitis. Association between severe HAQ with tendon involvement was not found (p>0.05. Disease duration was not associated with tenosynovitis. Patients were predominantly older, female, with mean age around 50.8 years. The long disease duration of patients presented a mean of 11.4 years and, most of them, with no vocational activity (65.0%. Conclusions: The results indicate that ankle tenosynovitis is very common in rheumatoid arthritis patients, both unilateral and bilateral. Tibialis posterior was the most common tendon involvement found. Finally, we found concordance between the clinical and ultrasound findings in almost all rheumatoid arthritis patients with ankle tenosynovitis.

Clinical availability of cholescintigraphy in evaluating diffuse liver parenchymal diseases

International Nuclear Information System (INIS)

Itoh, Hisao; Shimono, Reiko; Hamamoto, Ken; Ohshima, Kanji; Akamatsu, Koichi

1988-01-01

Technetium-99m N-pyridoxyl-5-methyltryptophan (PMT) cholescintigraphy has been performed in 46 consecutive patients with diffuse liver parenchymal diseases, including acute hepatitis (9), chronic hepatitis (17), and liver cirrhosis (20), and 18 controls. Blood clearance rate, liver uptake rate, liver excretion rate, and half time (T1/2) were determined from cardiac and hepatic time-activity curves. Regarding the four parameters, there were statistically significant differences between the control group and the groups of acute hepatitis and liver cirrhosis. Both blood clearance rate and liver uptake rate were well correlated with ICG-k values (r = 0.874 and r = 0.791, respectively). Liver excretion rate was most highly correlated with total serum bilirubin levels (r = 0.763), followed by ICG-k values. T1/2 was well correlated as well with total serum bilirubin levels. During the process where liver excretory ability was lowered in association with elevated serum bilirubin levels, threshold values for liver excretion rate appeared to be established. Cholescintigraphy may be of value in evaluating the pathophysiology of diffuse liver parenchymal diseases in that it is capable of quantitatively determining excretory function of hepatic cells. (Namekawa, K.)

Clinical Characteristics of Fungal Sensitization in Children with Allergic Respiratory Diseases

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Pınar Uysal

2016-08-01

Full Text Available Objective: The aim of the study was to evaluate the prevelance of fungal sensitization among school-aged children with allergic respiratory diseases who attended our outpatient clinic and to evaluate its clinical impact on disease severity. Materials and Methods: Children with allergic symptoms during mould season, who attended our outpatient clinic between January 2014 and August 2015, were evaluated for allergic respiratory diseases. Skin prick testing with fungal and other commercial standardized solutions of aeroallergens was performed in all children. Spirometry was performed in children with asthma. Serum total immunoglobulin E (IgE and aeroallergen specific IgE (sIgE levels were measured. Results: A total of 112 children were included in the study. The prevelance of fungal sensitization was 6.4%. Alternaria alterna was the most common fungal allergen in both mono and polysensitized groups (p=0.002, p=0.004, respectively. Alternaria alterna sensitization was significantly higher in patients with persistent allergic rhinitis compared to those with intermittant allergic rhinitis (p=0.002. The patients with mild asthma were mostly monosensitized (p=0.003, but cases with severe asthma (SA were polysensitized (p=0.007. In polysensitized cases, Alternaria alterna and Cladosporium spp. coexistance was the most common combination compared to other fungal combinations (p<0.001. The sensitivity rate of sIgE was found to be 88%. In spirometric analysis, forced expiratory volume in 1 second (FEV1 and FEV1/forced vital capacity values were lower in polysensitized children with asthma and in children with asthma coexisting allergic rhinitis compared to children with allergic rhinitis only (p=0.004, p=0.001, respectively. Conclusion: The most common fungal allergen was Alternaria alterna in children with mono or polysensitization. Polysensitization with fungal allergens was closely associated with SA and lower spirometric parameters.

Clinical presentation of adult coeliac disease.

LENUS (Irish Health Repository)

Tajuddin, T

2012-02-01

The mode of presentation of coeliac disease has been changing to more atypical or silent disease. Few studies described the clinical presentation of adult coeliac disease in Ireland in recent years. We retrospectively collected the clinical data for all patients who had a diagnosis of coeliac disease made in our centre between January 07 and December 08. Forty seven adults, predominantly females (n = 30), had a confirmed diagnosis of coeliac disease made during the study period. In our patient cohort, the presenting symptom was diarrhoea in 19 (40%) patients, while 16 patients (34%) did not have any G.I. symptoms, 10 (21%) presented with anaemia. Females presented at a significantly younger age compared to males, with median ages at diagnosis of 44.5 and 57 years, respectively (p = 0.04). Females also presented more commonly with non G.I. symptoms (p = 0.07). The reasons behind this gender difference need further study.

Dopaminreceptorscintigraphy in Parkinson's disease - Clinical correlation

International Nuclear Information System (INIS)

Riklund Aahlstroem, K.E.; Hietala, S.-O.; Johansson, F.

2002-01-01

Parkinson's disease is a severe, progressive neuro degenerative disorder which is characterised by a degeneration of the dopamine containing cells and loss of dopamine transporters (DA) in substantia nigra. Earlier 123 I-β-CIT SPECT studies have demonstrated this loss of DA content in Parkinson's disease. Recently a new radioligand 123 I-FP-CIT, with faster kinetics than b-CIT became available for imaging of the DA transporter. The applicability of this radioligand was tested in a large clinical material with early and advanced Parkinson's disease using a one day protocol. 123 I-FP-CIT uptake was decreased in patients with Parkinson's disease and this was seen three hours after injection of the radioligand. In the Parkinson's disease group the uptake in the putamen was reduced more than in the caudate nucleus. Specific to non-specific striatal uptake ratios correlated with the Hoehn and Yahr stage. It appeared that 123 I-FP-CIT SPECT allows a significant discrimination between patients with Parkinson's disease and other movement disorders. The scintigraphic observations were correlated to clinical findings. The results will be presented and discussed

Retinal Diseases Caused by Mutations in Genes Not Specifically Associated with the Clinical Diagnosis.

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Xia Wang

Full Text Available When seeking a confirmed molecular diagnosis in the research setting, patients with one descriptive diagnosis of retinal disease could carry pathogenic variants in genes not specifically associated with that description. However, this event has not been evaluated systematically in clinical diagnostic laboratories that validate fully all target genes to minimize false negatives/positives.We performed targeted next-generation sequencing analysis on 207 ocular disease-related genes for 42 patients whose DNA had been tested negative for disease-specific panels of genes known to be associated with retinitis pigmentosa, Leber congenital amaurosis, or exudative vitreoretinopathy.Pathogenic variants, including single nucleotide variations and copy number variations, were identified in 9 patients, including 6 with variants in syndromic retinal disease genes and 3 whose molecular diagnosis could not be distinguished easily from their submitted clinical diagnosis, accounting for 21% (9/42 of the unsolved cases.Our study underscores the clinical and genetic heterogeneity of retinal disorders and provides valuable reference to estimate the fraction of clinical samples whose retinal disorders could be explained by genes not specifically associated with the corresponding clinical diagnosis. Our data suggest that sequencing a larger set of retinal disorder related genes can increase the molecular diagnostic yield, especially for clinically hard-to-distinguish cases.

Development and reliability of a multi-modality scoring system for evaluation of disease progression in pre-clinical models of osteoarthritis: celecoxib may possess disease-modifying properties.

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Panahifar, A; Jaremko, J L; Tessier, A G; Lambert, R G; Maksymowych, W P; Fallone, B G; Doschak, M R

2014-10-01

We sought to develop a comprehensive scoring system for evaluation of pre-clinical models of osteoarthritis (OA) progression, and use this to evaluate two different classes of drugs for management of OA. Post-traumatic OA (PTOA) was surgically induced in skeletally mature rats. Rats were randomly divided in three groups receiving either glucosamine (high dose of 192 mg/kg) or celecoxib (clinical dose) or no treatment. Disease progression was monitored utilizing micro-magnetic resonance imaging (MRI), micro-computed tomography (CT) and histology. Pertinent features such as osteophytes, subchondral sclerosis, joint effusion, bone marrow lesion (BML), cysts, loose bodies and cartilage abnormalities were included in designing a sensitive multi-modality based scoring system, termed the rat arthritis knee scoring system (RAKSS). Overall, an inter-observer correlation coefficient (ICC) of greater than 0.750 was achieved for each scored feature. None of the treatments prevented cartilage loss, synovitis, joint effusion, or sclerosis. However, celecoxib significantly reduced osteophyte development compared to placebo. Although signs of inflammation such as synovitis and joint effusion were readily identified at 4 weeks post-operation, we did not detect any BML. We report the development of a sensitive and reliable multi-modality scoring system, the RAKSS, for evaluation of OA severity in pre-clinical animal models. Using this scoring system, we found that celecoxib prevented enlargement of osteophytes in this animal model of PTOA, and thus it may be useful in preventing OA progression. However, it did not show any chondroprotective effect using the recommended dose. In contrast, high dose glucosamine had no measurable effects. Copyright © 2014 Osteoarthritis Research Society International. Published by Elsevier Ltd. All rights reserved.

Lyme disease: clinical diagnosis and treatment

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Hatchette, TF; Davis, I; Johnston, BL

2014-01-01

Background Lyme disease is an emerging zoonotic infection in Canada. As the Ixodes tick expands its range, more Canadians will be exposed to Borrelia burgdorferi, the bacterium that causes Lyme disease. Objective To review the clinical diagnosis and treatment of Lyme disease for front-line clinicians. Methods A literature search using PubMed and restricted to articles published in English between 1977 and 2014. Results Individuals in Lyme-endemic areas are at greatest risk, but not all tick bites transmit Lyme disease. The diagnosis is predominantly clinical. Patients with Lyme disease may present with early disease that is characterized by a “bull’s eye rash”, fever and myalgias or with early disseminated disease that can manifest with arthralgias, cardiac conduction abnormalities or neurologic symptoms. Late Lyme disease in North America typically manifests with oligoarticular arthritis but can present with a subacute encephalopathy. Antibiotic treatment is effective against Lyme disease and works best when given early in the infection. Prophylaxis with doxycyline may be indicated in certain circumstances. While a minority of patients may have persistent symptoms, evidence does not demonstrate that prolonged courses of antibiotics improve outcome. Conclusion Clinicians need to be aware of the signs and symptoms of Lyme disease. Knowing the regions where Borrelia infection is endemic in North America is important for recognizing patients at risk and informing the need for treatment. PMID:29769842

Evaluation of cardiology consultations sought from the anaesthesia clinic

International Nuclear Information System (INIS)

Minai, F.N.; Kamal, R.S.

2004-01-01

Objective: To evaluate the criteria for cardiology referrals and to assess the perioperative relevance of the cardiology advice given in patients evaluated for non-cardiac surgery. Materials and Methods: A review of case files of 70 patients, scheduled for non-cardiac surgery, who were referred for cardiology consultations from the Anaesthesia Clinic at AKUH during the study period. The clinical criteria for seeking cardiology advice, the cardiology advice given, its influence on patient management, as well as number of adverse cardiac events in the perioperative period were documented. Results: A history of hypertension, ischemic heart disease, and ECG abnormalities were the major criteria for seeking opinion on cardiac status. Cardiology advice frequently resulted in the ordering of extensive cardiac investigations. Among the patients identified for further tests by the cardiologists, 75% had no evidence of ischemic heart disease or myocardial dysfunction; none of them were monitored invasively intraoperatively or had adverse cardiac events in the perioperative period requiring intensive care or prolonged hospital stay. Conclusion: No definite criteria or pattern for referrals was identified. Most of the referrals did not fall within the AHA guidelines. Cardiology advice given had very little influence on the perioperative management. (author)

[Clinical effectiveness and economical evaluation of preventive vaccination].

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Vaz Carneiro, António; Belo, Ana Isabel; Gouveia, Miguel; Costa, João; Borges, Margarida

2011-01-01

The value of mass vaccination as a preventive measure for infectious diseases is one of the most important advances of modern Medicine. The impact on incidence of several infectious diseases, until recently responsible for significant morbidity and mortality at world level, is well proved in a series of high quality epidemiological studies. In this scientific review we aimed firstly to briefly resume the history of mass vaccination and its scientists, responsible for synthesis and marketing of these drugs. In second place we present a group of a few disease preventable by vaccines as well as the Portuguese National Vaccination Plan and its benefits. In third place we identified groups of subjects in which a well structured vaccination plan is particularly important, as well as the correspondent diseases to be covered by vaccination. Fourthly, we discussed the ethical considerations of vaccination, and its tensions between subject autonomy and society advantages in com pulsive programs. Fifthly, we analyzed clinical effectiveness of vaccines through the concept of herd immunity, clinical evaluation of immune response to vaccines and some examples of systematic reviews on three relevant diseases (influenza, meningococcal and pneumococcal infections). In sixth place we discussed vaccine safety presenting monitoring methods of vaccination risks, as well as discussing the public myths concerning vaccines. Finally we present a economic analysis of preventive vaccination with a review of some published literature on specific diseases. We conclude that mass vaccination is a efficacious preventive measure, as well as a economic rational choice, and that this public health intervention should be a pillar of a modern preventive system.

Clinical evaluation of FMPSPGR sequence of the brain MR imaging

International Nuclear Information System (INIS)

Takahashi, Mitsuyuki; Hasegawa, Makoto; Mori, Naohiko; Yamanoguchi, Minoru; Matsubara, Tadashi

1998-01-01

In order to apply the FMPSPGR (fast multi planar spoiled GRASS) method to diagnose brain diseases, authors obtained the optimal condition for imaging by the phantom experiments and examined the clinical usefulness. Six kinds of the phantom, which were 4 of diluted Gd solution with different concentrations, olive oil and physiological saline solution were used. From the phantom experiments, TR/TE/FR=300/3.3/90 degrees was the optimal condition. The evaluation of the clinical images was performed on the same section by the ST method and the FMPSPGR method. Fifteen patients (9 men and 6 women, aged from 17 to 80 years) suspected of brain diseases were examined, including 8 of cerebral infarction, 1 of pontine infarction, 1 of brain contusion, 1 of intracerebral bleeding and 4 of brain tumors. Four cases of brain tumor were evaluated on the contrast imaging and the others were on the plain imaging. In the plain imaging, the FMPSPGR method was better than the SE method on the low signal region in the T1 weighted imaging. Furthermore, in the contrast imaging, it could give more clear images of the lesion in anterior cranial pit by suppressing artifacts of blood flow. The present results indicate that the FMPSPGR method is useful to diagnose brain diseases. (K.H.)

Randomized, controlled clinical study to evaluate efficacy of novel indigenously designed controlled release flurbiprofen gel system for management of periodontal diseases

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Neeraj C Deshpande

2013-01-01

Full Text Available Background: This randomized, controlled clinical study was planned to evaluate the use of anti-inflammatory drug flurbiprofen in the form of locally delivered controlled release gel in the treatment of periodontal disease. Materials and Methods: The flurbiprofen gel was indigenously prepared in the concentration of 0.3%. The 30 patients with localized periodontal pockets measuring ≥5 mm were randomly divided into three groups. The groups received flurbiprofen gel, flurbiprofen gel after prophylaxis, and placebo gel after oral prophylaxis, respectively. The clinical parameters for plaque and gingival inflammation were evaluated at baseline, 7 th day, and 14 th day. Results: The results of the study suggested the statistically significant ( P < 0.05 improvement in the gingival status of the patients with the use of flurbiprofen gel as an adjunct to scaling and root planing as compared to oral prophylaxis or gel alone. Conclusion: The data demonstrated that the additional use of local drug delivery of flurbiprofen through gel media enhances the positive effects of scaling and root planing and helps in faster resolution of the inflammation.

Symptoms and biomarkers associated with celiac disease: evaluation of a population-based screening program in adults.

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Kårhus, Line L; Thuesen, Betina H; Rumessen, Jüri J; Linneberg, Allan

2016-11-01

To identify possible early predictors (symptoms and biomarkers) of celiac disease, compare symptoms before and after screening, and evaluate the diagnostic efficacy of serologic screening for celiac disease in an adult Danish population. This cross-sectional population-based study was based on the 5-year follow-up of the Health2006 cohort, where 2297 individuals were screened for celiac disease; 56 were antibody positive and thus invited to clinical evaluation. Eight were diagnosed with biopsy-verified celiac disease. A follow-up questionnaire was sent to antibody-positive individuals 19 months after the clinical evaluation to obtain information on their symptoms and their experience with participation in the screening. Before screening, participants subsequently diagnosed with celiac disease did not differ from the rest of the population with respect to symptoms, but had significantly lower total cholesterol. Tissue transglutaminase IgA antibodies with a cut-off of 10 U/ml had a positive predictive value of 88%. The majority of participants were satisfied with their participation in the screening program. Individuals with celiac disease were generally satisfied with having been diagnosed and 71% felt better on a gluten-free diet. There were no differences in the prevalence of symptoms between participants with and without screening-detected celiac disease, confirming that risk stratification in a general population by symptoms is difficult. The majority of participants diagnosed with celiac disease felt better on a gluten-free diet despite not reporting abdominal symptoms before diagnosis and participants in the clinical evaluation were generally satisfied with participation in the screening program.

Integrating utilization-focused evaluation with business process modeling for clinical research improvement.

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Kagan, Jonathan M; Rosas, Scott; Trochim, William M K

2010-10-01

New discoveries in basic science are creating extraordinary opportunities to design novel biomedical preventions and therapeutics for human disease. But the clinical evaluation of these new interventions is, in many instances, being hindered by a variety of legal, regulatory, policy and operational factors, few of which enhance research quality, the safety of study participants or research ethics. With the goal of helping increase the efficiency and effectiveness of clinical research, we have examined how the integration of utilization-focused evaluation with elements of business process modeling can reveal opportunities for systematic improvements in clinical research. Using data from the NIH global HIV/AIDS clinical trials networks, we analyzed the absolute and relative times required to traverse defined phases associated with specific activities within the clinical protocol lifecycle. Using simple median duration and Kaplan-Meyer survival analysis, we show how such time-based analyses can provide a rationale for the prioritization of research process analysis and re-engineering, as well as a means for statistically assessing the impact of policy modifications, resource utilization, re-engineered processes and best practices. Successfully applied, this approach can help researchers be more efficient in capitalizing on new science to speed the development of improved interventions for human disease.

Pelvic insufficiency fractures associated with radiation atrophy: clinical recognition and diagnostic evaluation

International Nuclear Information System (INIS)

Mumber, M.P.; Greven, K.M.; Haygood, T.M.

1997-01-01

Pelvic bone injuries are infrequent complications of radiotherapy. However, insufficiency fractures in irradiated pelvic bones may be underdetected, particularly in postmenopausal women. We describe the clinical presentation, radiologic evaluation, and course of disease in three patients with postradiation pelvic insufficiency fractures. Differential diagnosis included metastatic disease, tumor recurrence, and second malignancy. Recognition of radiographic features may prevent unnecessary, possibly morbid treatments. (orig.). With 6 figs

Evaluation [corrected] of the "Iceberg Phenomenon" in Johne's disease through mathematical modelling.

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Gesham Magombedze

Full Text Available Johne's disease (JD is a chronic, enteric disease in ruminants caused by Mycobacterium avium subsp. paratuberculosis (MAP. Disease progression follows four distinct stages: silent, subclinical, clinical and advanced. Available diagnostic tests have poor sensitivity and cannot detect early stages of the infection; as a result, only animals in the clinical and advanced stages, which represent the tip of the 'iceberg', are identified through testing. The Iceberg Phenomenon is then applied to provide estimates for JD prevalence. For one animal in the advanced stage, it is assumed that there are one to two in the clinical stage, four to eight in the subclinical stage, and ten to fourteen in the silent stage. These ratios, however, are based on little evidence. To evaluate the ratios, we developed a deterministic ordinary differential equation model of JD transmission and disease progression dynamics. When duration periods associated with the natural course of the disease progression are used, the above ratios do not hold. The ratios used to estimate JD prevalence need to be further investigated.

Neuro degenerative diseases: clinical concerns

International Nuclear Information System (INIS)

Ibanez, V.

2005-01-01

Idiopathic Parkinson's disease (PD) and Alzheimer's disease (AD) are the main neuro-degenerative diseases (NDDs) seen clinically. They share some common clinical symptoms and neuro-pathological findings. The increase of life expectancy in the developed countries will inevitably contribute to enhance the prevalence of these diseases. Behavioral disorders, common in NDDs, will produce major care management challenges. Idiopathic Parkinson's disease corresponds to a histopathological diagnosis, based on the observation of a de-pigmentation and a neuronal loss in the substantia nigra, as well as on the presence of intra-neuronal inclusion bodies. AD is insidious with slowly progressive dementia in which the decline in memory constitutes the main complaint. The diagnosis of definite AD requires the presence of clinical criteria as well as the histopathological confirmation of brain lesions. The two main lesions are the presence of senile plaques and neuro-fibrillary tangles. Positron emission tomography (PET) explores cerebral metabolism and neurotransmitter kinetics in NDDs using principally [ 18 F]-deoxyglucose and [ 18 F]-dopa. Nigrostriatal dopaminergic function is altered in PD, as evidenced by the low uptake of [ 18 F]-dopa in the posterior putamen as compared to anterior putamen and caudate nucleus. In contrast, [ 18 F]-dopa uptake is equally depressed in all striatal structures in progressive supra-nuclear palsy. Regional glucose metabolism at rest is preserved in elderly once cerebral atrophy is taken into account. On the contrary, glucose metabolism is globally reduced in AD, with marked decrease in the parietal and temporal regions. PET has proved to be useful to study in vivo neurochemical processes in patients suffering from NDDs. The potential of this approach is still largely unexploited, and depends on new ligand production to establish early diagnosis and treatment follow-up. (author)

HIV-associated salivary gland disease--clinical or imaging diagnosis?

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da Silva Rath, Inês Beatriz; Beltrame, Ana Paula C A; Carvalho, Aroldo P; Schaeffer, Marcela B; Almeida, Izabel C S

2015-07-01

This work aimed at studying the salivary gland disease (SGD) as it relates to associated factors, such as persistent generalised lymphadenopathy (PGL), lymphocytic interstitial pneumonia (LIP), clinical and immunological features of AIDS, and salivary flow rate and pH, as well as at exploring the relationship between the clinical diagnosis and the imaging diagnosis by ultrasound (US) examination of the parotid glands. Information regarding the observation of parotid gland enlargement, PGL, LIP, and clinical and immunological features of AIDS was gathered from medical records, and a saliva sample for unstimulated salivary flow rate and pH measurement was collected from 142 children aged 3 through 10 years treated at the Department of Infectious Diseases of Joana de Gusmão Children's Hospital, Florianópolis, SC, Brazil. High-resolution ultrasonography was performed in 58 children. Pearson's chi-square test and t-test were used to evaluate the association between the variables. A significant association was found between SGD and LIP. Ultrasound revealed a 50% higher incidence of SGD that was not reported in the patients' records. US examination proved to be essential for the correct diagnosis and monitoring of the progression of HIV/SGD. © 2014 BSPD, IAPD and John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Patterns of clinical presentation of adult coeliac disease in a rural setting

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D'Souza Charles

2006-09-01

Full Text Available Abstract Background In recent years there has been increasing recognition that the pattern of presentation of coeliac disease may be changing. The classic sprue syndrome with diarrhoea and weight loss may be less common than the more subtle presentations of coeliac disease such as an isolated iron deficiency anaemia. As a result, the diagnosis of this treatable condition is often delayed or missed. Recent serologic screening tests allow non-invasive screening to identify most patients with the disease and can be applied in patients with even subtle symptoms indicative of coeliac disease. Both benign and malignant complications of coeliac disease can be avoided by early diagnosis and a strict compliance with a gluten free diet. Aim The aim of this study is to evaluate the trends in clinical presentation of patients diagnosed with adult coeliac disease. In addition, we studied the biochemical and serological features and the prevalence of associated conditions in patients with adult coeliac disease. Methods This is an observational, retrospective, cross-sectional review of the medical notes of 32 adult patients attending the specialist coeliac clinic in a district general hospital. Results Anaemia was the most common mode of presentation accounting for 66% of patients. Less than half of the patients had any of the classical symptoms of coeliac disease and 25% had none of the classical symptoms at presentation. Anti-gliadin antibodies, anti-endomysial antibody and anti-tissue transglutaminase showed 75%, 68% and 90% sensitivity respectively. In combination, serology results were 100% sensitive as screening tests for adult coeliac disease. Fifty nine percent patients had either osteoporosis or osteopenia. There were no malignant complications observed during the follow up of our patients. Conclusion Most adults with coeliac disease have a sub clinical form of the disease and iron deficiency anaemia may be its sole presenting symptom. Only a minority

Wilson's disease: cranial MRI observations and clinical correlation

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Sinha, S.; Taly, A.B.; Prashanth, L.K.; Venugopal, K.S.; Arunodaya, G.R.; Swamy, H.S. [National Institute of Mental Health and Neurosciences (NIMHANS), Department of Neurology, Bangalore (India); Ravishankar, S.; Vasudev, M.K. [National Institute of Mental Health and Neurosciences (NIMHANS), Department of Neuroimaging and Interventional Radiology, Bangalore (India)

2006-09-15

Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson's disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD. MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3{+-}8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted. The mean duration of illness and treatment were 8.3{+-}10.8 years and 7.5{+-}7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), ''Face of giant panda'' sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P<0.001) but did not correlate with the duration of illness. MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease. (orig.)

The clinical experience of interventional embolization in treatment of graves disease

International Nuclear Information System (INIS)

Di Zhenhai; Wang Xiaochuan; Liu Longtu; Wang Xiuhua; Wang Zubin

2002-01-01

Objective: To evaluate the method and result of thyroid artery embolization as a new therapy for Graves disease. Methods: Ten patients with Graves disease underwent selective thyroid artery embolization. Totally 25 thyroid arteries were embolized with PVA microspheres. The indications to this therapy were as following: Graves disease with recurrent clinical symptoms or with leucopenia during the period of treating with administration of antithyroid drugs or recurrence after subtotal thyroidectomy. Results: Serum level of thyroid hormones dropped significantly [median FT 3 from 20.90 pmol/L (13.36-50.92 pmol/L) to 7.81 pmol/L (3.67-35.3 pmol/L), median FT 4 from 57.9 pmol/L (30.96-57.9 pmol/L) to 28.13 pmol/L (20.44-39.60 pmol/L), (P < 0.005)] and then followed-up for 5-8 months. The symptoms of hyperthyroidism were controlled in 7 patients and the remaining 3 cases were treated with lower dosage of antithyroid drug therapy. None serious complications were found. Conclusions: thyroid artery embolization represents a promising new method for treating Graves disease with safety and good clinical results. Further investigation would be required to assess its long-term effect

The usefulness of CT scanning in clinical observation on the patients with Alzheimer's disease

International Nuclear Information System (INIS)

Golebiowski, M.; Barcikowska, M.; Pfeffer-Baczuk, A.; Luczywek, E.

1994-01-01

On the basis of brain CT studies of 150 patients of the clinic for persons with Alzheimer's disease the diagnostic utility of measurement of hippocampal fissure and craniocerebral ratios was assessed. In analyzed group hippocampal fissure measurements greater than 4 mm occurred only in patients with symptoms of Alzheimer's type dementia. The measurement showed 90% sensitivity and 70% specificity as the prognostic factor of clinical course, especially at the first part of the disease. The evaluation of the hippocampal fissure in conjunction with detailed analysis of CT picture of the atrophic brain allowed for precise final diagnosis. (author)

Autobiographical memory compromise in Alzheimer's disease: a cognitive and clinical overview.

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El Haj, Mohamad; Roche, Jean; Gallouj, Karim; Gandolphe, Marie-Charlotte

2017-12-01

Autobiographical memory refers to memory for personal information. The compromise of autobiographical memory in Alzheimer's disease (AD) results in a loss of knowledge about events and facts that defined the patients' life, and consequently, degradation of their self-knowledge and sense of identity. The compromise of autobiographical memory in AD can be attributed to a diminished subjective experience of memory and a diminished sense of the self. Our review provides a comprehensive overview of cognitive and clinical processes that may be involved in difficulties to retrieve autobiographical memories in Alzheimer's disease. Our review also proposes a theoretical model according to which, the diminished ability to retrieve contextual information and the overgenerality of recall result in a diminished subjective experience of past and future thinking. Besides its theoretical contribution, our review proposes clinical applicability for evaluation and rehabilitation of autobiographical memory in AD.

Clinical evaluation of 99Tcm-MIBI myocardial perfusion imaging for the detection of coronary artery disease in patients with metabolic syndrome

International Nuclear Information System (INIS)

Tian Yueqin; Wei Hongxing; Guo Xinhua; Guo Feng; He Zuoxiang

2008-01-01

Objective: Metabolic syndrome is a combination of medical disorders that consist of a collection of independent factors at risk of developing coronary artery disease. The purpose of this study was to evaluate the value of 99 Tc m -methoxyisobutylisonitrile (MIBI) myocardial perfusion imaging for the diagnosis of coronary artery disease in patients with metabolic syndrome. Methods: A total of 251 patients [mean age (59 ± 10) years, 179 men, 72 women] were included in this study. All patients underwent exercise and rest 99 Tc m -MIBI myocardial perfusion imaging and coronary angiography. Results: Of the 163 patients with significant coronary artery stenosis, 116 showed abnormal 99 Tc m -MIBI myocardial perfusion imaging; and among the 88 patients with normal coronary angiography, 82 showed normal myocardial perfusion imaging. The sensitivity, specificity and accuracy of 99 Tc m -MIBI myocardial perfusion imaging for coronary artery disease detection were 71% (116/163), 93% (82/88) and 79% (198/251), respectively. The positive and negative predictive values were 95% (116/122) and 64% (82/129), respectively. Conclusion: 99 Tc m -MIBI myocardial perfusion imaging has important clinical value for detecting coronary artery disease in patients with metabolic syndrome. (authors)

Clinical outcome of Crohn's disease according to the Vienna classification: disease location is a useful predictor of disease course

NARCIS (Netherlands)

Oostenbrug, Liekele E.; van Dullemen, Hendrik M.; te Meerman, Gerard J.; Jansen, Peter L. M.; Kleibeuker, Jan H.

2006-01-01

OBJECTIVES: Crohn's disease (CD) is a complex genetic disease with multiple clinical patterns. Clinical classifications may help to identify subgroups of patients that have a distinct pattern of disease, and they are also a prerequisite for the conduction of genetic and therapeutic studies. The aim

Clinical outcome of Crohn's disease according to the Vienna classification : disease location is a useful predictor of disease course

NARCIS (Netherlands)

Oostenbrug, Liekele E.; van Dullemen, Hendrik M.; te Meerman, Gerard J.; Jansen, Peter L. M.; Kleibeuker, Jan H.

Objectives Crohn's disease (CD) is a complex genetic disease with multiple clinical patterns. Clinical classifications may help to identify subgroups of patients that have a distinct pattern of disease, and they are also a prerequisite for the conduction of genetic and therapeutic studies. The aim

Addison's disease due to adrenal tuberculosis: Contrast-enhanced CT features and clinical duration correlation

International Nuclear Information System (INIS)

Guo Yingkun; Yang Zhigang; Li Yuan; Ma Ensen; Deng Yuping; Min Pengqiu; Yin Longlin; Hu Jian; Zhang Xiaochun; Chen Tianwu

2007-01-01

Purpose: To describe CT morphology of untreated adrenal tuberculosis during the different stages of the natural history of the disease and to evaluate the diagnostic implications of CT features. Materials and methods: We retrospectively evaluated CT features in 42 patients with documented adrenal tuberculosis for the location, size, morphology, and enhancement patterns shown on CT images. The clinical duration were correlated with the CT features. Results: Of the 42 patients with untreated adrenal tuberculosis, bilaterally enlarged adrenal glands were revealed in 38 cases (91%), unilaterally enlarged in 3 cases (7%), and normal size in 1 case (2%). Of the 41 cases (98%) with enlargement, mass-like enlargement was seen in 20 cases (49%) and enlargement with preserved contours in 21 cases (51%). Peripheral rim enhancement presented in 22 cases (52%) on contrast-enhanced CT. Non-enhanced CT scan revealed calcification in 21 cases (50%). As the duration of Addison's disease increased, the presence of calcification and contour preservation increased concomitantly (p < 0.001), whereas peripheral rim enhancement and mass-like enlargement decreased concomitantly on CT images (p < 0.001). Conclusion: CT may be helpful in diagnosing adrenal tuberculosis when clinically suspected, and CT features are correlated to the clinical duration of Addison's disease

Information systems for administration, clinical documentation and quality assurance in an Austrian disease management programme.

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Beck, Peter; Truskaller, Thomas; Rakovac, Ivo; Bruner, Fritz; Zanettin, Dominik; Pieber, Thomas R

2009-01-01

5.9% of the Austrian population is affected by diabetes mellitus. Disease Management is a structured treatment approach that is suitable for application to the diabetes mellitus area and often is supported by information technology. This article describes the information systems developed and implemented in the Austrian disease management programme for type 2 diabetes. Several workflows for administration as well as for clinical documentation have been implemented utilizing the Austrian e-Health infrastructure. De-identified clinical data is available for creating feedback reports for providers and programme evaluation.

Parkinsonian syndroms: Clinical phenotype, differential diagnosis and disease progression

International Nuclear Information System (INIS)

Storch, A.

2002-01-01

Parkinsonian syndromes include idiopathic Parkinson's disease (IPD), other neurodegenerative diseases with parkinsonism, the so-called atypical parkinsonian syndromes, and symptomatic parkinsonian syndromes, such as Wilson's disease. IPD is the most frequent disease with parkinsonism as the main clinical feature and is responsible for approx. 80% of all parkinsonian syndromes. Atypical parkinsonian syndromes are the most important differential diagnoses of IPD. The two most frequent types are multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). For clinical diagnosis it is essential to take a careful medical history and to examine the patients physically in regular intervals. However, various clinico-pathological studies have shown that approx. 25% of patients with clinical diagnosis of IPD may have other causes of parkinsonism. Selected technical investigations, in particular functional imaging of the central dopaminergic system using PET or SPECT, may help to make clinical diagnosis more secure. This paper reviews the clinical features and diagnostic findings in diseases with parkinsonism and summarises the difficulties in establishing early and differential diagnoses. (orig.) [de

[Alzheimer's disease cerebro-spinal fluid biomarkers: A clinical research tool sometimes useful in daily clinical practice of memory clinics for the diagnosis of complex cases].

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Magnin, E; Dumurgier, J; Bouaziz-Amar, E; Bombois, S; Wallon, D; Gabelle, A; Lehmann, S; Blanc, F; Bousiges, O; Hannequin, D; Jung, B; Miguet-Alfonsi, C; Quillard, M; Pasquier, F; Peoc'h, K; Laplanche, J-L; Hugon, J; Paquet, C

2017-04-01

The role of biomarkers in clinical research was recently highlighted in the new criteria for the diagnosis of Alzheimer's disease. Cerebro-spinal fluid (CSF) biomarkers (total Tau protein, threonine 181 phosphorylated Tau protein and amyloid Aβ1-42 peptide) are associated with cerebral neuropathological lesions observed in Alzheimer's disease (neuronal death, neurofibrillary tangle with abnormal Tau deposits and amyloid plaque). Aβ1-40 amyloid peptide dosage helps to interpret Aβ1-42 results. As suggested in the latest international criteria and the French HAS (Haute Autorité de santé) recommendations, using theses CSF biomarkers should not be systematic but sometimes could be performed to improve confidence about the diagnostic of Alzheimer's disease in young subjects or in complex clinical situations. Future biomarkers actually in development will additionally help in diagnostic process (differential diagnosis) and in prognostic evaluation of neurodegenerative diseases. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Huperzine A for Alzheimer's disease: a systematic review and meta-analysis of randomized clinical trials.

Directory of Open Access Journals (Sweden)

Guoyan Yang

Full Text Available Huperzine A is a Chinese herb extract used for Alzheimer's disease. We conducted this review to evaluate the beneficial and harmful effect of Huperzine A for treatment of Alzheimer's disease.We searched for randomized clinical trials (RCTs of Huperzine A for Alzheimer's disease in PubMed, Cochrane Library, and four major Chinese electronic databases from their inception to June 2013. We performed meta-analyses using RevMan 5.1 software. (Protocol ID: CRD42012003249.20 RCTs including 1823 participants were included. The methodological quality of most included trials had a high risk of bias. Compared with placebo, Huperzine A showed a significant beneficial effect on the improvement of cognitive function as measured by Mini-Mental State Examination (MMSE at 8 weeks, 12 weeks and 16 weeks, and by Hastgawa Dementia Scale (HDS and Wechsler Memory Scale (WMS at 8 weeks and 12 weeks. Activities of daily living favored Huperzine A as measured by Activities of Daily Living Scale (ADL at 6 weeks, 12 weeks and 16 weeks. One trial found Huperzine A improved global clinical assessment as measured by Clinical Dementia Rating Scale (CDR. One trial demonstrated no significant change in cognitive function as measured by Alzheimer's disease Assessment Scale-Cognitive Subscale (ADAS-Cog and activity of daily living as measured by Alzheimer's disease Cooperative Study Activities of Daily Living Inventory (ADCS-ADL in Huperzine A group. Trials comparing Huperzine A with no treatment, psychotherapy and conventional medicine demonstrated similar findings. No trial evaluated quality of life. No trial reported severe adverse events of Huperzine A.Huperzine A appears to have beneficial effects on improvement of cognitive function, daily living activity, and global clinical assessment in participants with Alzheimer's disease. However, the findings should be interpreted with caution due to the poor methodological quality of the included trials.

Clinical and laboratory signs associated to serious dengue disease in hospitalized children

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Sheila Moura Pone

Full Text Available Abstract Objective: To evaluate the validity of clinical and laboratory signs to serious dengue disease in hospitalized children. Methods: Retrospective cohort of children (19 and negative likelihood ratio <0.6. Pleural effusion and abdominal distension had higher sensitivity (82.6%. History of bleeding (epistaxis, gingival or gastrointestinal bleeding and severe hemorrhage (pulmonary or gastrointestinal bleeding in physical examination were more frequent in serious dengue disease (p < 0.01, but with poor accuracy (positive likelihood ratio = 1.89 and 3.89; negative likelihood ratio = 0.53 and 0.60, respectively. Serum albumin was lower in serious dengue forms (p < 0.01. Despite statistical significance (p < 0.05, both groups presented thrombocytopenia. Platelets count, hematocrit, and hemoglobin parameters had area under the curve <0.5. Conclusions: Lethargy, abdominal distension, pleural effusion, and hypoalbuminemia were the best clinical and laboratorial markers of serious dengue disease in hospitalized children, while bleeding, severe hemorrhage, hemoconcentration and thrombocytopenia did not reach adequate diagnostic accuracy. In pediatric referral hospitals, the absence of hemoconcentration does not imply absence of plasma leakage, particularly in children with previous fluid replacement. These findings may contribute to the clinical management of dengue in children at referral hospitals.

Trichinella infection and clinical disease

DEFF Research Database (Denmark)

Clausen, M R; Meyer, C N; Krantz, T

1996-01-01

Trichinellosis is caused by ingestion of insufficiently cooked meat contaminated with infective larvae of Trichinella species. The clinical course is highly variable, ranging from no apparent infection to severe and even fatal disease. We report two illustrative cases of trichinellosis. Returning....... Life-threatening cardiopulmonary, renal and central nervous system complications developed. The patient recovered after several months. Her husband, who also ate the pork, did not have clinical symptoms, but an increased eosinophil count and a single larva in a muscle biopsy confirmed infection....... The epidemiology, clinical manifestations, diagnosis, treatment and prevention of trichinellosis are reviewed....

The evaluation of clinical usefulness of transbrochoscopic lung biopsy in undefined interstitial lung diseases: a retrospective study.

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Han, Qian; Luo, Qun; Chen, Xiaobo; Xie, Jiaxing; Wu, Lulu; Chen, Rongchang

2017-03-01

Previous studies mostly focused on the diagnostic accuracy of transbronchoscopic lung biopsy (TBLB) in the diagnosis of interstitial lung diseases (ILDs). We aimed to explore the clinical usefulness of TBLB results in the diagnostic procedure of undefined ILDs. The retrospective analysis included patients undergoing TBLB for the diagnosis of undefined ILDs from January 2007 to December 2010. The clinically useful TBLB was defined as that lead to a specific histopathological diagnosis or that was consistent with the working diagnosis based on existing clinical and radiological data. A total of 664 patients were included in the study. TBLB failed to obtain lung parenchyma in 155 cases (23.3%). TBLB was considered clinically helpful in 202 procedures (30.4%), including 114 cases that provided definitive histopathological diagnoses and 88 cases that were consistent with working diagnoses. Among 202 cases of clinically useful TBLBs, the majority were diagnosed as pulmonary alveolar proteinosis (PAP) (67 cases, 33.2%), connective tissue disease-related ILDs (CTD-ILDs) (65, 32.2%) and idiopathic pulmonary fibrosis (IPF) (33, 16.3%). Although TBLB could provide definitive histopathological diagnoses in all cases diagnosed as PAP, only few cases of IPF (7, 21.2% of IPF diagnoses) and CTD-ILDs (9, 13.8% of CTD-ILD diagnoses) could be identified by TBLBs. The clinical usefulness of TBLB, in conjunction with thorough clinical and radiological data, in the diagnosis of ILDs may be varied depending on different subtypes. The use of histopathological analysis and the type of biopsy employed should therefore be considered on a case-by-case basis. © 2015 John Wiley & Sons Ltd.

New scoring methodology improves the sensitivity of the Alzheimer's Disease Assessment Scale-Cognitive subscale (ADAS-Cog) in clinical trials.

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Verma, Nishant; Beretvas, S Natasha; Pascual, Belen; Masdeu, Joseph C; Markey, Mia K

2015-11-12

As currently used, the Alzheimer's Disease Assessment Scale-Cognitive subscale (ADAS-Cog) has low sensitivity for measuring Alzheimer's disease progression in clinical trials. A major reason behind the low sensitivity is its sub-optimal scoring methodology, which can be improved to obtain better sensitivity. Using item response theory, we developed a new scoring methodology (ADAS-CogIRT) for the ADAS-Cog, which addresses several major limitations of the current scoring methodology. The sensitivity of the ADAS-CogIRT methodology was evaluated using clinical trial simulations as well as a negative clinical trial, which had shown an evidence of a treatment effect. The ADAS-Cog was found to measure impairment in three cognitive domains of memory, language, and praxis. The ADAS-CogIRT methodology required significantly fewer patients and shorter trial durations as compared to the current scoring methodology when both were evaluated in simulated clinical trials. When validated on data from a real clinical trial, the ADAS-CogIRT methodology had higher sensitivity than the current scoring methodology in detecting the treatment effect. The proposed scoring methodology significantly improves the sensitivity of the ADAS-Cog in measuring progression of cognitive impairment in clinical trials focused in the mild-to-moderate Alzheimer's disease stage. This provides a boost to the efficiency of clinical trials requiring fewer patients and shorter durations for investigating disease-modifying treatments.

Harnessing Cerebrospinal Fluid Biomarkers in Clinical Trials for Treating Alzheimer's and Parkinson's Diseases: Potential and Challenges.

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Kim, Dana; Kim, Young Sam; Shin, Dong Wun; Park, Chang Shin; Kang, Ju Hee

2016-10-01

No disease-modifying therapies (DMT) for neurodegenerative diseases (NDs) have been established, particularly for Alzheimer's disease (AD) and Parkinson's disease (PD). It is unclear why candidate drugs that successfully demonstrate therapeutic effects in animal models fail to show disease-modifying effects in clinical trials. To overcome this hurdle, patients with homogeneous pathologies should be detected as early as possible. The early detection of AD patients using sufficiently tested biomarkers could demonstrate the potential usefulness of combining biomarkers with clinical measures as a diagnostic tool. Cerebrospinal fluid (CSF) biomarkers for NDs are being incorporated in clinical trials designed with the aim of detecting patients earlier, evaluating target engagement, collecting homogeneous patients, facilitating prevention trials, and testing the potential of surrogate markers relative to clinical measures. In this review we summarize the latest information on CSF biomarkers in NDs, particularly AD and PD, and their use in clinical trials. The large number of issues related to CSF biomarker measurements and applications has resulted in relatively few clinical trials on CSF biomarkers being conducted. However, the available CSF biomarker data obtained in clinical trials support the advantages of incorporating CSF biomarkers in clinical trials, even though the data have mostly been obtained in AD trials. We describe the current issues with and ongoing efforts for the use of CSF biomarkers in clinical trials and the plans to harness CSF biomarkers for the development of DMT and clinical routines. This effort requires nationwide, global, and multidisciplinary efforts in academia, industry, and regulatory agencies to facilitate a new era.

Periodontal disease in pregnant patients with rheumatic valvular disease: clinical and microbiological study.

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Avila, Walkiria Samuel; Timerman, Lilia; Romito, Giuseppe Alexandre; Marcelino, Sílvia Linard; Neves, Itamara Lúcia Itagiba; Zugaib, Marcelo; Grinberg, Max

2011-04-01

The periodontal disease during pregnancy of women with rheumatic valve disease imply infective endocarditis risks and higher rate of preterm birth and low birth weight. To study the periodontal disease rate of women with rheumatic valve disease during pregnancy. We studied 140 pregnant women who included 70 patients with rheumatic valve disease and 70 healthy women. The periodontal examination included: 1) periodontal clinical exam regard the follow variables: a) probing depth; b) gingival margin; c) clinical attachment level; d) bleeding on probing; e) plaque index and f) gingival index; and 2) microbiological test was performed in samples serum and gingival crevicular fluid and considered positive controls to Porphyromonas gingivalis, Tannerella forsithia e Aggregobacter actinomycetemcomitans. Age and parity were similar between groups; as single or combined the mitral valve disease was prevalent among the rheumatic valve lesion in 45 (32.1%) e 20 (28.5%) cases, respectively. Among the periodontal variables gingival margin (p=0.01) and plaque index (p=0.04) were different between groups. The periodontal disease was identified in 20 (14,3%) pregnant women, seven (10%) of them were patients with valve rheumatic disease and the remain 13 (18,6%) were healthy women, its percentual was not different between groups (p=0,147). Microbiological analyses of oral samples showed higher percentual of P. gingivalis in healthy pregnant women (p=0.004). The clinical and microbiological study during pregnancy showed comparable incidence of periodontal disease between women with rheumatic valve disease and healthy women.

Clinical and Histopathological Evaluation of Cutaneous Pythiosis in Donkeys (Equusasinus

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José Alberto Cardona Álvarez

2013-06-01

Full Text Available The purpose of this study was to evaluate and characterize the clinical and histopathological aspects of cutaneous pythiosis in donkeys (Equus asinus in the Department of Cordoba, Colombia. A descriptive, non-probability study was conducted on domesticated animals. Nine donkeys clinically and histopathologically diagnosed with cutaneous pythiosis were analyzed. After describing the cases, a serious, crater-shaped granulomatous ulcer was observed, with necrotic tissue, a tumor appearance, abundant fibrinous-bloody exudation and yellowish-white caseous material known as kunkers. The lesions were found in the members, ventral abdomen and chest of the animals. Histopathologically, with the hematoxylin-eosin staining, severe pyogranulomatous dermatitis was observed, with abundant eosinophilic and neutrophilic inflammatory infiltrate; with the Grocott staining, hyphae with septa were found, partially branching into a right angle and brown in color. The definitive diagnosis of the disease was based on the clinical features, the differential diagnosis and the histopathological findings. The diagnosis of cutaneous pythiosis was conclusive. This study becomes the first report of this disease in donkeys (Equus asinus in the department of Córdoba and in Colombia.

CDKD: a clinical database of kidney diseases

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Singh Sanjay

2012-04-01

Full Text Available Abstract 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.

Criterion Validation Testing of Clinical Metrology Instruments for Measuring Degenerative Joint Disease Associated Mobility Impairment in Cats.

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Gruen, Margaret E; Griffith, Emily H; Thomson, Andrea E; Simpson, Wendy; Lascelles, B Duncan X

2015-01-01

Degenerative joint disease and associated pain are common in cats, particularly in older cats. There is a need for treatment options, however evaluation of putative therapies is limited by a lack of suitable, validated outcome measures that can be used in the target population of client owned cats. The objectives of this study were to evaluate low-dose daily meloxicam for the treatment of pain associated with degenerative joint disease in cats, and further validate two clinical metrology instruments, the Feline Musculoskeletal Pain Index (FMPI) and the Client Specific Outcome Measures (CSOM). Sixty-six client owned cats with degenerative joint disease and owner-reported impairments in mobility were screened and enrolled into a double-masked, placebo-controlled, randomized clinical trial. Following a run-in baseline period, cats were given either placebo or meloxicam for 21 days, then in a masked washout, cats were all given placebo for 21 days. Subsequently, cats were given the opposite treatment, placebo or meloxicam, for 21 days. Cats wore activity monitors throughout the study, owners completed clinical metrology instruments following each period. Activity counts were increased in cats during treatment with daily meloxicam (pdegenerative joint disease.

Clinical characteristics of sleep disorders in patients with Parkinson's disease.

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Mao, Zhi-Juan; Liu, Chan-Chan; Ji, Su-Qiong; Yang, Qing-Mei; Ye, Hong-Xiang; Han, Hai-Yan; Xue, Zheng

2017-02-01

In order to investigate the sleep quality and influencing factors in patients with Parkinson's disease (PD), 201 PD patients were enrolled and underwent extensive clinical evaluations. Subjective sleep evaluation was assessed using the Pittsburgh Sleep Quality Index (PSQI), and the Epworth Sleepiness Scale (ESS). It was found that poor sleep quality (77.11%) and excessive daytime sleepiness (32.34%) were commonly seen in PD patients and positively correlated with disease severity. Then 70 out of the 201 PD patients and 70 age- and sex-matched controls underwent a polysomnographic recording. The parameters were compared between PD group and control group and the influencing factors of sleep in PD patients were analyzed. The results showed that sleep efficiency (SE) was significantly decreased (Psleep latency (SL) and the arousal index (AI) were increased (Psleep time (TST) were positively correlated with the Hoehn and Yahr (H&Y) stage. There was significant difference in the extent of hypopnea and hypoxemia between the PD group and the control group (Psleep quality and a high prevalence of sleep disorder, which may be correlated with the disease severity. Respiratory function and oxygen supply are also affected to a certain degree in PD patients.

SMARTHealth India: Development and Field Evaluation of a Mobile Clinical Decision Support System for Cardiovascular Diseases in Rural India.

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Praveen, Devarsetty; Patel, Anushka; Raghu, Arvind; Clifford, Gari D; Maulik, Pallab K; Mohammad Abdul, Ameer; Mogulluru, Kishor; Tarassenko, Lionel; MacMahon, Stephen; Peiris, David

2014-12-08

Cardiovascular disease (CVD) is the major cause of premature death and disability in India and yet few people at risk of CVD are able to access best practice health care. Mobile health (mHealth) is a promising solution, but very few mHealth interventions have been subjected to robust evaluation in India. The objectives were to develop a multifaceted, mobile clinical decision support system (CDSS) for CVD management and evaluate it for use by public nonphysician health care workers (NPHWs) and physicians in a rural Indian setting. Plain language clinical rules were developed based on standard guidelines and programmed into a computer tablet app. The algorithm was validated and field-tested in 11 villages in Andhra Pradesh, involving 11 NPHWs and 3 primary health center (PHC) physicians. A mixed method evaluation was conducted comprising clinical and survey data and in-depth patient and staff interviews to understand barriers and enablers to the use of the system. Then this was thematically analyzed using NVivo 10. During validation of the algorithm, there was an initial agreement for 70% of the 42 calculated variables between the CDSS and SPSS software outputs. Discrepancies were identified and amendments were made until perfect agreement was achieved. During field testing, NPHWs and PHC physicians used the CDSS to screen 227 and 65 adults, respectively. The NPHWs identified 39% (88/227) of patients for referral with 78% (69/88) of these having a definite indication for blood pressure (BP)-lowering medication. However, only 35% (24/69) attended a clinic within 1 month of referral, with 42% (10/24) of these reporting continuing medications at 3-month follow-up. Physicians identified and recommended 17% (11/65) of patients for BP-lowering medications. Qualitative interviews identified 3 interrelated interview themes: (1) the CDSS had potential to change prevailing health care models, (2) task-shifting to NPHWs was the central driver of change, and (3) despite high

Social Cognition Dysfunctions in Neurodegenerative Diseases: Neuroanatomical Correlates and Clinical Implications

Science.gov (United States)

Santamaría-García, Hernando; Santangelo, Gabriella

2018-01-01

Social cognitive function, involved in the perception, processing, and interpretation of social information, has been shown to be crucial for successful communication and interpersonal relationships, thereby significantly impacting mental health, well-being, and quality of life. In this regard, assessment of social cognition, mainly focusing on four key domains, such as theory of mind (ToM), emotional empathy, and social perception and behavior, has been increasingly evaluated in clinical settings, given the potential implications of impairments of these skills for therapeutic decision-making. With regard to neurodegenerative diseases (NDs), most disorders, characterized by variable disease phenotypes and progression, although similar for the unfavorable prognosis, are associated to impairments of social cognitive function, with consequent negative effects on patients' management. Specifically, in some NDs these deficits may represent core diagnostic criteria, such as for behavioral variant frontotemporal dementia (bvFTD), or may emerge during the disease course as critical aspects, such as for Parkinson's and Alzheimer's diseases. On this background, we aimed to revise the most updated evidence on the neurobiological hypotheses derived from network-based approaches, clinical manifestations, and assessment tools of social cognitive dysfunctions in NDs, also prospecting potential benefits on patients' well-being, quality of life, and outcome derived from potential therapeutic perspectives of these deficits. PMID:29854017

Social Cognition Dysfunctions in Neurodegenerative Diseases: Neuroanatomical Correlates and Clinical Implications

Directory of Open Access Journals (Sweden)

Foteini Christidi

2018-01-01

Full Text Available Social cognitive function, involved in the perception, processing, and interpretation of social information, has been shown to be crucial for successful communication and interpersonal relationships, thereby significantly impacting mental health, well-being, and quality of life. In this regard, assessment of social cognition, mainly focusing on four key domains, such as theory of mind (ToM, emotional empathy, and social perception and behavior, has been increasingly evaluated in clinical settings, given the potential implications of impairments of these skills for therapeutic decision-making. With regard to neurodegenerative diseases (NDs, most disorders, characterized by variable disease phenotypes and progression, although similar for the unfavorable prognosis, are associated to impairments of social cognitive function, with consequent negative effects on patients’ management. Specifically, in some NDs these deficits may represent core diagnostic criteria, such as for behavioral variant frontotemporal dementia (bvFTD, or may emerge during the disease course as critical aspects, such as for Parkinson’s and Alzheimer’s diseases. On this background, we aimed to revise the most updated evidence on the neurobiological hypotheses derived from network-based approaches, clinical manifestations, and assessment tools of social cognitive dysfunctions in NDs, also prospecting potential benefits on patients’ well-being, quality of life, and outcome derived from potential therapeutic perspectives of these deficits.

Male breast disease: clinical, mammographic, and ultrasonographic features

International Nuclear Information System (INIS)

Guenhan-Bilgen, Isil; Bozkaya, Halil; Uestuen, Esin Emin; Memis, Aysenur

2002-01-01

Purpose: To describe and quantitate the radiological (mammographic and ultrasonographic) characteristics of male breast disease and to report the clinical and pathological findings. Materials and methods: Two-hundred-thirty-six male patients with different male breast diseases, diagnosed at our institution between January 1990 and July 2001, were retrospectively evaluated. The history, physical examination, mammographic and ultrasonographic findings were analyzed. Results: The spectrum of the disease in 236 male patients were gynecomastia (n=206), primary breast carcinoma (n=14), fat necrosis (n=5), lipoma (n=3), subareolar abscess (n=2), epidermal inclusion cyst (n=1), sebaceous cyst (n=1), hematoma (n=1), myeloma (n=1), and metastatic carcinoma (n=2). The distribution of patterns of gynecomastia were; 34% (n=71) nodular, 35% (n=73) dendritic and 31% (n=62) diffuse glandular. Gynecomastia was unilateral in 55% (n=113) and bilateral in 45% (n=93) of the patients. Male breast cancer presented as a mass without microcalcifications in 86% (n=12) and with microcalcifications in 7% (n=1) of patients. The mass was obscured by gynecomastia, partially in two, totally in one patient. The location of the mass was retroareolar in 46% (n=6) and eccentric to the nipple in 54% (n=7) of patients. On ultrasonography (US), the contours were well-circumscribed in 20% (n=3) and irregular in 80% (n=12) of the masses. Conclusion: Male breast has a wide spectrum of diseases, some of which have characteristic radiological appearances that can be correlated with their pathologic diagnosis. In the evaluation of the male breast, mammography and US are essential and should be performed along with physical examination

Male breast disease: clinical, mammographic, and ultrasonographic features

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Guenhan-Bilgen, Isil E-mail: isilbilgen@hotmail.com; Bozkaya, Halil; Uestuen, Esin Emin; Memis, Aysenur

2002-09-01

Purpose: To describe and quantitate the radiological (mammographic and ultrasonographic) characteristics of male breast disease and to report the clinical and pathological findings. Materials and methods: Two-hundred-thirty-six male patients with different male breast diseases, diagnosed at our institution between January 1990 and July 2001, were retrospectively evaluated. The history, physical examination, mammographic and ultrasonographic findings were analyzed. Results: The spectrum of the disease in 236 male patients were gynecomastia (n=206), primary breast carcinoma (n=14), fat necrosis (n=5), lipoma (n=3), subareolar abscess (n=2), epidermal inclusion cyst (n=1), sebaceous cyst (n=1), hematoma (n=1), myeloma (n=1), and metastatic carcinoma (n=2). The distribution of patterns of gynecomastia were; 34% (n=71) nodular, 35% (n=73) dendritic and 31% (n=62) diffuse glandular. Gynecomastia was unilateral in 55% (n=113) and bilateral in 45% (n=93) of the patients. Male breast cancer presented as a mass without microcalcifications in 86% (n=12) and with microcalcifications in 7% (n=1) of patients. The mass was obscured by gynecomastia, partially in two, totally in one patient. The location of the mass was retroareolar in 46% (n=6) and eccentric to the nipple in 54% (n=7) of patients. On ultrasonography (US), the contours were well-circumscribed in 20% (n=3) and irregular in 80% (n=12) of the masses. Conclusion: Male breast has a wide spectrum of diseases, some of which have characteristic radiological appearances that can be correlated with their pathologic diagnosis. In the evaluation of the male breast, mammography and US are essential and should be performed along with physical examination.

Effects of curcumin consumption on human chronic diseases: A narrative review of the most recent clinical data.

Science.gov (United States)

Mantzorou, Maria; Pavlidou, Eleni; Vasios, George; Tsagalioti, Eftychia; Giaginis, Constantinos

2018-06-01

Numerous clinical trials have investigated the potential beneficial effects of curcumin supplementation against several human chronic diseases. Up to now, it has been claimed that curcumin consumption may exert beneficial effects against several chronic diseases by promoting human health and preventing diseases. In this aspect, the present review aims to critically collect and in-depth summarize the most recent, well-designed clinical studies evaluating the potential beneficial effects of curcumin consumption on human health promotion and disease prevention. According to recent and well-designed clinical studies, curcumin consumption may benefit against obesity, metabolic syndrome, and diabetes. Moreover, curcumin consumption seems to exert a positive effect on people suffering from various types of cancer, fatty liver disease, depression, arthritis, skin diseases, gut inflammation, and symptoms of premenstrual syndrome. Due to the strong heterogeneity among the clinical studies concerning the exact effective curcumin dose and formulation, as well as the recommended treatment duration for each chronic disease, no precise and definitive conclusions could be drawn. Further large-scale prospective studies are strongly recommended, being well-designed as far as follow-up times, dosage, formulation, and duration of curcumin supplementation are concerned. Moreover, potential confounders in each specific chronic disease should carefully be taken into account in future studies. Copyright © 2018 John Wiley & Sons, Ltd.

Clinical and epidemiological profile of patients with valvular heart disease admitted to the emergency department

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Moraes, Ricardo Casalino Sanches de [Instituto do Coração, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Katz, Marcelo [Hospital Israelita Albert Einstein, São Paulo, SP (Brazil); Tarasoutchi, Flávio [Instituto do Coração, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil)

2014-07-01

To evaluate the clinical and epidemiological profile of patients with valvular heart disease who arrived decompensated at the emergency department of a university hospital in Brazil. A descriptive analysis of clinical and echocardiographic data of 174 patients with severe valvular disease, who were clinically decompensated and went to the emergency department of a tertiary cardiology hospital, in the State of São Paulo, in 2009. The mean age of participants was 56±17 years and 54% were female. The main cause of valve disease was rheumatic in 60%, followed by 15% of degenerative aortic disease and mitral valve prolapse in 13%. Mitral regurgitation (27.5%) was the most common isolated valve disease, followed by aortic stenosis (23%), aortic regurgitation (13%) and mitral stenosis (11%). In echocardiographic data, the mean left atrial diameter was 48±12mm, 38±12mm for the left ventricular systolic diameter, and 54±12mm for the diastolic diameter; the mean ejection fraction was 56±13%, and the mean pulmonary artery pressure was 53±16mmHg. Approximately half of patients (44%) presented atrial fibrillation, and over one third of them (37%) had already undergone another cardiac surgery. Despite increased comorbidities and age-dependent risk factors commonly described in patients with valvular heart disease, the clinical profile of patients arriving at the emergency department represented a cohort of rheumatic patients in more advanced stages of disease. These patients require priority care in high complexity specialized hospitals.

Clinical and epidemiological profile of patients with valvular heart disease admitted to the emergency department

International Nuclear Information System (INIS)

Moraes, Ricardo Casalino Sanches de; Katz, Marcelo; Tarasoutchi, Flávio

2014-01-01

To evaluate the clinical and epidemiological profile of patients with valvular heart disease who arrived decompensated at the emergency department of a university hospital in Brazil. A descriptive analysis of clinical and echocardiographic data of 174 patients with severe valvular disease, who were clinically decompensated and went to the emergency department of a tertiary cardiology hospital, in the State of São Paulo, in 2009. The mean age of participants was 56±17 years and 54% were female. The main cause of valve disease was rheumatic in 60%, followed by 15% of degenerative aortic disease and mitral valve prolapse in 13%. Mitral regurgitation (27.5%) was the most common isolated valve disease, followed by aortic stenosis (23%), aortic regurgitation (13%) and mitral stenosis (11%). In echocardiographic data, the mean left atrial diameter was 48±12mm, 38±12mm for the left ventricular systolic diameter, and 54±12mm for the diastolic diameter; the mean ejection fraction was 56±13%, and the mean pulmonary artery pressure was 53±16mmHg. Approximately half of patients (44%) presented atrial fibrillation, and over one third of them (37%) had already undergone another cardiac surgery. Despite increased comorbidities and age-dependent risk factors commonly described in patients with valvular heart disease, the clinical profile of patients arriving at the emergency department represented a cohort of rheumatic patients in more advanced stages of disease. These patients require priority care in high complexity specialized hospitals

IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

OpenAIRE

N. V. Fedorova; A. V. Nikitina

2015-01-01

Objective – a description of clinical cases of impulsive­compulsive disorders in Parkinson,s disease. The first clinical case. Patient N., 75 years old, suffering for 15 years from Parkinson,s disease, akinetic­rigid form of the disease, stage 4 by Hyun–Yar. Since 2009, he received levodopa/carbidopa 250/25 mg 5 times/day (daily dose of 1250 mg of levodopa); pramipexole 3.5 mg per day (daily dose 3.5 mg), amantadine sulfate 100 mg 5 times/day (daily dose 500 mg). While taking antiparkinsonian...

Clinical Characteristics and Associated Systemic Diseases in Patients With Esophageal "Absent Contractility"-A Clinical Algorithm.

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Laique, Sobia; Singh, Tavankit; Dornblaser, David; Gadre, Abhishek; Rangan, Vikram; Fass, Ronnie; Kirby, Donald; Chatterjee, Soumya; Gabbard, Scott

2018-01-19

This study was carried out to assess the clinical characteristics and associated systemic diseases seen in patients diagnosed with absent contractility as per the Chicago Classification version 3.0, allowing us to propose a diagnostic algorithm for their etiologic testing. The Chicago Classification version 3.0 has redefined major and minor esophageal motility disorders using high-resolution esophageal manometry. There is a dearth of publications based on research on absent contractility, which historically has been associated with myopathic processes such as systemic sclerosis (SSc). We conducted a retrospective, multicenter study. Data of patients diagnosed with absent contractility were pooled from Cleveland Clinic, Cleveland, OH (January 2006 to July 2016) and Metrohealth Medical Center, Cleveland, OH (July 2014 to July 2016) and included: age, gender, associated medical conditions, surgical history, medications, and specific antibody testing. A total of 207 patients, including 57 male individuals and 150 female individuals, with mean age of 56.1 and 60.0 years, respectively, were included. Disease distribution was as follows: SSc (diffuse or limited cutaneous) 132, overlap syndromes 7, systemic lupus erythematosus17, Sjögren syndrome 4, polymyositis 3, and dermatomyositis 3. Various other etiologies including gastroesophageal reflux disease, postradiation esophagitis, neuromuscular disorders, and surgical complications were seen in the remaining cohort. Most practitioners use the term "absent contractility" interchangeably with "scleroderma esophagus"; however, only 63% of patients with absent contractility had SSc. Overall, 20% had another systemic autoimmune rheumatologic disease and 16% had a nonrheumatologic etiology for absent contractility. Therefore, alternate diagnosis must be sought in these patients. We propose an algorithm for their etiologic evaluation.

Task shifting to clinical officer-led echocardiography screening for detecting rheumatic heart disease in Malawi, Africa.

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Sims Sanyahumbi, Amy; Sable, Craig A; Karlsten, Melissa; Hosseinipour, Mina C; Kazembe, Peter N; Minard, Charles G; Penny, Daniel J

2017-08-01

Echocardiographic screening for rheumatic heart disease in asymptomatic children may result in early diagnosis and prevent progression. Physician-led screening is not feasible in Malawi. Task shifting to mid-level providers such as clinical officers may enable more widespread screening. Hypothesis With short-course training, clinical officers can accurately screen for rheumatic heart disease using focussed echocardiography. A total of eight clinical officers completed three half-days of didactics and 2 days of hands-on echocardiography training. Clinical officers were evaluated by performing screening echocardiograms on 20 children with known rheumatic heart disease status. They indicated whether children should be referred for follow-up. Referral was indicated if mitral regurgitation measured more than 1.5 cm or there was any measurable aortic regurgitation. The κ statistic was calculated to measure referral agreement with a paediatric cardiologist. Sensitivity and specificity were estimated using a generalised linear mixed model, and were calculated on the basis of World Heart Federation diagnostic criteria. The mean κ statistic comparing clinical officer referrals with the paediatric cardiologist was 0.72 (95% confidence interval: 0.62, 0.82). The κ value ranged from a minimum of 0.57 to a maximum of 0.90. For rheumatic heart disease diagnosis, sensitivity was 0.91 (95% confidence interval: 0.86, 0.95) and specificity was 0.65 (95% confidence interval: 0.57, 0.72). There was substantial agreement between clinical officers and paediatric cardiologists on whether to refer. Clinical officers had a high sensitivity in detecting rheumatic heart disease. With short-course training, clinical officer-led echo screening for rheumatic heart disease is a viable alternative to physician-led screening in resource-limited settings.

Radiological evaluation of esophageal motility and gastroesophageal reflux disease

International Nuclear Information System (INIS)

Schima, W.; Pokieser, P.; Schober, E.

1995-01-01

Radiological evaluation of esophageal motility and the lower esophageal sphincter has gained increased attention in recent years. Videofluoroscopic investigation of esophageal motor function is superior to static film radiography, as repeated analysis of the videotaped recordings is possible. With emphasis on radiological techniques, normal esophagel physiology and motility and a variety of esophageal motor disorders are discussed in this review paper. Radiological evaluation of gastroesophageal reflux and reflux esophagitis is described. Clinical and radiological findings in esophageal motility disorders and gastroesophageal reflux disease and the radiological efficacy compared to that of manometry and pH-metry are discussed. (orig.) [de

Criteria for evaluating response and outcome in clinical trials for children with juvenile myelomonocytic leukemia.

LENUS (Irish Health Repository)

Niemeyer, Charlotte M

2015-01-01

Juvenile myelomonocytic leukemia is a rare myeloproliferative disease in young children. While hematopoietic stem cell transplantation remains the only curative therapeutic option for most patients, children with juvenile myelomonocytic leukemia increasingly receive novel agents in phase I-II clinical trials as pre-transplant therapy or therapy for relapse after transplantation. However, response criteria or definitions of outcome for standardized evaluation of treatment effect in patients with juvenile myelomonocytic leukemia are currently lacking. Here we propose criteria to evaluate the response to the non-transplant therapy and definitions of remission status after hematopoietic stem cell transplantation. For the evaluation of non-transplant therapy, we defined 6 clinical variables (white blood cell count, platelet count, hematopoietic precursors and blasts in peripheral blood, bone marrow blast percentage, spleen size and extramedullary disease) and 3 genetic variables (cytogenetic, molecular and chimerism response) which serve to describe the heterogeneous picture of response to therapy in each individual case. It is hoped that these criteria will facilitate the comparison of results between clinical trials in juvenile myelomonocytic leukemia.

Clinical validation of the CHRONIOUS wearable system in patients with chronic disease.

Science.gov (United States)

Bellos, Christos; Papadopoulos, Athanassios; Rosso, Roberto; Fotiadis, Dimitrios I

2013-01-01

The CHRONIOUS system defines a powerful and easy to use framework which has been designed to provide services to clinicians and their patients suffering from chronic diseases. The system is composed of a wearable shirt that integrate several body sensors, a portable smart device and a central sub-system that is responsible for the long term storage of the collected patient's data. A multi-parametric expert system is developed for the analysis of the collected data using intelligent algorithms and complex techniques. Apart for the vital signals, dietary habits, drug intake, activity data, environmental and biochemical parameters are recorded. The CHRONIOUS platform is validated through clinical trials in several medical centers and patient's home environments recruiting patients suffering from Chronic Obstructive pulmonary disease (COPD) and Chronic Kidney Disease (CKD) diseases. The clinical trials contribute in improving the system's accuracy, while Pulmonologists and Nephrologists experts utilized the CHRONIOUS platform to evaluate its efficiency and performance. The results of the utilization of the system were very encouraging. The CHRONIOUS system has been proven to be a well-validated real-time patient monitoring and supervision platform, providing a useful tool for the clinician and the patient that would contribute to the more effective management of chronic diseases.

Studies on bile acid and bilirubin in liver diseases Part 2. Clinical significance of serum bilirubin sulfate in various liver diseases

OpenAIRE

石川, 泰祐

1980-01-01

The clinical significance of serum bilirubin sulfate, one of the direct bilirubin, was evaluated in various liver diseases with over 2 mg/dl of serum bilirubin concentration. The diagnosis included 25 cases of acute hepatitis, 8 cases of chronic hepatitis, 8 cases of liver cirrhosis and 16 cases of liver cirrhosis with hepatoma. Bilirubin sulfate was fractioned by Yonei's solvent partition method. The clinical significance of bilirubin sulfate was assessed by comparison of bilirubin sulfate w...

Characteristics of randomised trials on diseases in the digestive system registered in ClinicalTrials.gov: a retrospective analysis

DEFF Research Database (Denmark)

Wildt, Signe; Krag, Aleksander; Gluud, Liselotte

2011-01-01

Objectives To evaluate the adequacy of reporting of protocols for randomised trials on diseases of the digestive system registered in http://ClinicalTrials.gov and the consistency between primary outcomes, secondary outcomes and sample size specified in http://ClinicalTrials.gov and published...

Infectious Disease Clinical Research Program (IDCRP)

Data.gov (United States)

Federal Laboratory Consortium — Our mission is to conduct infectious disease clinical research of importance to the military through a unique, adaptive, and collaborative network, to inform health...

Clinical application of multi-shot diffusion EPI in neurological disease

International Nuclear Information System (INIS)

Ishihara, Tetsuya; Hirata, Koichi; Kubo, Jin; Yamazaki, Kaoru; Sato, Toshihiko

1998-01-01

Using the multi-shot EPI method we investigated the clinical application of diffusion weighted imaging (DWI) in the diagnosis of neurological disease. The multi-shot method provided better susceptibility artifact-free DWI than the single-shot method particularly in the region of the posterior cranial fossa. DWI using the multi-shot EPI method readily shows the pyramidal tract extending from the internal capsule to the brainstems which is inaccessible by the conventional single-shot EPI method, and providing three-dimensional and distinct images of pyramidal tract changes in amyotrophic lateral sclerosis or cerebral infarction with pyramidal tract disturbance. Our findings suggest that the use of DWI with the multi-shot EPI method would provide a technique for the easy diagnosis and evaluation of various neurological diseases. (author)

Clinical application of multi-shot diffusion EPI in neurological disease

Energy Technology Data Exchange (ETDEWEB)

Ishihara, Tetsuya; Hirata, Koichi; Kubo, Jin; Yamazaki, Kaoru [Dokkyo Univ., Mibu, Tochigi (Japan). School of Medicine; Sato, Toshihiko

1998-05-01

Using the multi-shot EPI method we investigated the clinical application of diffusion weighted imaging (DWI) in the diagnosis of neurological disease. The multi-shot method provided better susceptibility artifact-free DWI than the single-shot method particularly in the region of the posterior cranial fossa. DWI using the multi-shot EPI method readily shows the pyramidal tract extending from the internal capsule to the brainstems which is inaccessible by the conventional single-shot EPI method, and providing three-dimensional and distinct images of pyramidal tract changes in amyotrophic lateral sclerosis or cerebral infarction with pyramidal tract disturbance. Our findings suggest that the use of DWI with the multi-shot EPI method would provide a technique for the easy diagnosis and evaluation of various neurological diseases. (author)

Clinical utility of FDG-PET in amyotrophic lateral sclerosis and Huntington's disease.

Science.gov (United States)

Agosta, Federica; Altomare, Daniele; Festari, Cristina; Orini, Stefania; Gandolfo, Federica; Boccardi, Marina; Arbizu, Javier; Bouwman, Femke; Drzezga, Alexander; Nestor, Peter; Nobili, Flavio; Walker, Zuzana; Pagani, Marco

2018-05-01

To evaluate the incremental value of FDG-PET over clinical tests in: (i) diagnosis of amyotrophic lateral sclerosis (ALS); (ii) picking early signs of neurodegeneration in patients with a genetic risk of Huntington's disease (HD); and detecting metabolic changes related to cognitive impairment in (iii) ALS and (iv) HD patients. Four comprehensive literature searches were conducted using the PICO model to extract evidence from relevant studies. An expert panel then voted using the Delphi method on these four diagnostic scenarios. The availability of evidence was good for FDG-PET utility to support the diagnosis of ALS, poor for identifying presymptomatic subjects carrying HD mutation who will convert to HD, and lacking for identifying cognitive-related metabolic changes in both ALS and HD. After the Delphi consensual procedure, the panel did not support the clinical use of FDG-PET for any of the four scenarios. Relative to other neurodegenerative diseases, the clinical use of FDG-PET in ALS and HD is still in its infancy. Once validated by disease-control studies, FDG-PET might represent a potentially useful biomarker for ALS diagnosis. FDG-PET is presently not justified as a routine investigation to predict conversion to HD, nor to detect evidence of brain dysfunction justifying cognitive decline in ALS and HD.

DNA-abzymes in autoimmune diseases in clinic and experiment

Directory of Open Access Journals (Sweden)

T E Naumova

2003-01-01

Full Text Available DNA-abzymes enzymes in autoimmune diseases in clinic and experiment T.E. Naumova, O.M. Durova, A.G. Gabibov, Z.S. Alekberova, S. V. Suchkov DNA-hydrolyzing autoantibodies (AAB or DNA-abzymes can be found in autoimmune diseases in clinic and experiment. Technology of serum express screening for presence of DNA abzymes is described. Comparative study of DNA-hydrolising activity in patients with different forms of systemic and organ-specific autoimmune diseases was performed. Blood of clinically healthy donors was usually free of IgG DNA-abzymes. DNA-abzymes were most often revealed in patients with systemic lupus erythematosus (SLE and rheumatoid arthritis (RA less often in patients with organ-specific forms of autoimmune disturbances. The results of the study confirm the hypothesis of autoimmune origin of IgG DNA abzymes and demonstrate the possibility to use them in clinical practice for monitoring to disease activity in SLE and RA.

Evaluation of vitamin B 12 deficiency in various clinical condition

International Nuclear Information System (INIS)

Baig, J.A.; Alam, J.M.; Kazmi, T.; Waseem, S.; Hussain, A.; Arif, S.; Shaheen, R.; Sultana, I.

2010-01-01

Low levels of vitamin B 12 have been associated with several clinical conditions. However no single symptom or group of symptoms can be made responsible. Reported causes of deficiency among older population are hematologic or neurological, followed by gastrointestinal and possibly vascular symptoms. The present prospective observational study was, hence, initiated to evaluate the underlying clinical condition or symptoms associated with vitamin B12 deficiency. The study was prospective observational and carried out on 121 patients (males, n=63 and females, n=58) for the period from January 1, 2004 to January 24, 2007. Age ranges were between 16 - 70 years, and categorized as > 60 yrs and < 60 years. All blood parameters were analyzed by standardized methods on automated analyzers. The deficiency was found to be more prevalent in males and increased from 52.06% to 58.10% in individuals with vitamin B12 <150 pg/ml. Mal nourishment was noted among the most subjects and weakness and anemia were frequent clinical findings (35.55%, n=43, 14%, n=51). Other clinical conditions were neuropsychiatric. Whereas less frequent findings were paraesthesia and gastrointestinal symptoms. Hypertension was more prevalent in vitamin B12 deficient individuals followed by diabetes, dementia, stroke, ischemic heart disease and Parkinson's disease. (author)

Methodologic Guide for Evaluating Clinical Performance and Effect of Artificial Intelligence Technology for Medical Diagnosis and Prediction.

Science.gov (United States)

Park, Seong Ho; Han, Kyunghwa

2018-03-01

The use of artificial intelligence in medicine is currently an issue of great interest, especially with regard to the diagnostic or predictive analysis of medical images. Adoption of an artificial intelligence tool in clinical practice requires careful confirmation of its clinical utility. Herein, the authors explain key methodology points involved in a clinical evaluation of artificial intelligence technology for use in medicine, especially high-dimensional or overparameterized diagnostic or predictive models in which artificial deep neural networks are used, mainly from the standpoints of clinical epidemiology and biostatistics. First, statistical methods for assessing the discrimination and calibration performances of a diagnostic or predictive model are summarized. Next, the effects of disease manifestation spectrum and disease prevalence on the performance results are explained, followed by a discussion of the difference between evaluating the performance with use of internal and external datasets, the importance of using an adequate external dataset obtained from a well-defined clinical cohort to avoid overestimating the clinical performance as a result of overfitting in high-dimensional or overparameterized classification model and spectrum bias, and the essentials for achieving a more robust clinical evaluation. Finally, the authors review the role of clinical trials and observational outcome studies for ultimate clinical verification of diagnostic or predictive artificial intelligence tools through patient outcomes, beyond performance metrics, and how to design such studies. © RSNA, 2018.

Phenotype and Clinical Course of Inflammatory Bowel Disease with Co-Existent Celiac Disease.

Science.gov (United States)

Tse, Chung Sang; Deepak, Parakkal; De La Fuente, Jaime; Bledsoe, Adam C; Larson, Joseph J; Murray, Joseph A; Papadakis, Konstantinos A

2018-05-07

Inflammatory bowel diseases, principally Crohn's disease and ulcerative colitis, and celiac disease are among the most common immune-mediated gastrointestinal diseases. We aim to elucidate the clinical course and outcomes of patients with concomitant inflammatory bowel disease and celiac disease, a unique population that remains scarcely studied to date. A retrospective matched case-control study of adults with coexistent inflammatory bowel disease and celiac disease was performed at a tertiary referral institution in North America. Logistic regression and Kaplan-Meier curves compared disease characteristics and clinical outcomes of the two groups. A total of 342 inflammatory bowel disease patients were included in this study, of which 114 had coexistent celiac disease and 228 did not. Patients with coexistent inflammatory bowel disease and celiac disease had higher rates of primary sclerosing cholangitis (19.3% vs 5.7%; odds ratio, 4.4; 95% confidence interval, 2.1-9.4; pceliac disease (10.5% vs 3.5%; odds ratio 3.2; 95% confidence interval 1.3-8.2; p=0.01), compared to patients without concomitant celiac disease. Patients with inflammatory bowel disease with concomitant celiac disease have unique phenotypic features compared to non-celiac inflammatory bowel disease, with higher risks for colitis-related hospitalizations, extensive colitis, and primary sclerosing cholangitis. Increased recognition of coexistent IBD and celiac disease can prompt clinicians to investigate for concomitant disease sooner, particularly in patients with seemingly refractory disease.

Clinical Evaluation of Brain Perfusion SPECT with Brodmann Areas Mapping in Early Diagnosis of Alzheimer's Disease.

Science.gov (United States)

Valotassiou, Varvara; Papatriantafyllou, John; Sifakis, Nikolaos; Tzavara, Chara; Tsougos, Ioannis; Psimadas, Dimitrios; Fezoulidis, Ioannis; Kapsalaki, Eftychia; Hadjigeorgiou, George; Georgoulias, Panagiotis

2015-01-01

Early diagnosis of Alzheimer's disease (AD) based on clinical criteria alone may be problematic, while current and future treatments should be administered earlier in order to be more effective. Thus, various disease biomarkers could be used for early detection of AD. We evaluated brain perfusion with 99mTc-HMPAO single photon emission computed tomography (SPECT) and Brodmann areas (BAs) mapping in mild AD using an automated software (NeuroGam) for the semi-quantitative evaluation of perfusion in BAs and the comparison with the software's normal database. We studied 34 consecutive patients with mild AD: 9 men, 25 women, mean age 70.9 ± 8.1 years, mean Mini-Mental State Examination 22.6 ± 2.5. BAs 25L, 25R, 38L, 38R, 28L, 28R, 36L, and 36R had the lower mean perfusion values, while BAs 31L, 31R, 19R, 18L, 18R, 17L, and 17R had the higher mean values. Compared with healthy subjects of the same age, perfusion values in BAs 25L, 25R, 28R, 28L, 36L, and 36R had the greatest deviations from the healthy sample, while the lowest deviations were found in BAs 32L, 32R, 19R, 24L, 17L, 17R, 18L, and 18R. A percentage of ≥94% of patients had perfusion values more than -2SDs below the mean of healthy subjects in BAs 38R, 38L, 36L, 36R, 23L, 23R, 22L, 44L, 28L, 28R, 25L, and 25R. The corresponding proportion was less than 38% for BAs 11L, 19R, 32L, 32R, 18L, 18R, 24L, and 17R. In conclusion, brain SPECT studies with automated perfusion mapping could be useful as an ancillary tool in daily practice, revealing perfusion impairments in early AD.

The objective evaluation of obstructive pulmonary diseases with spirometry

Directory of Open Access Journals (Sweden)

Özkaya S

2016-08-01

Full Text Available Sevket Ozkaya,1 Adem Dirican,2 Tibel Tuna3 1Department of Pulmonary Medicine, Faculty of Medicine, Bahçes¸ehir University, Istanbul, 2Department of Pulmonary Medicine, Samsun Medical Park Hospital, 3Department of Pulmonary Medicine, Samsun Chest Diseases and Thoracic Surgery Hospital, Samsun, Turkey Abstract: Airway obstruction is variable in asthma, while it is progressive and persistent in chronic bronchitis and emphysema. However, some of the patients presenting with symptoms of chronic airway diseases have clinical features of both asthma and COPD. The group with “Asthma–COPD Overlap Syndrome” (ACOS phenotype was characterized by definitely irreversible airway obstruction accompanied by symptoms and signs of reversibility. In this study, we aimed to classify obstructive airway diseases by clinical, radiological, and pulmonary function tests. Patients at Samsun Medical Park Hospital Chest Diseases outpatient clinic were evaluated between January 2013 and April 2016, and a total of 235 patients were included in this study. Mean age of the patients was 55.3±14.5 (15–88 years, and the male/female ratio was 45/190. The baseline pulmonary function test results of the patients were as follows: mean forced vital capacity (FVC values 2,825±1,108 (710–6,870 mL and 74.3±22.4 (24–155%, forced expiratory volume in 1 second (FEV1 values 1,789±774 (480–4,810 mL and 58.1±20.0 (20–130%, FEV1/FVC values 62.5±6.8 (39–70%. Reversibility criteria following bronchodilator treatment were present in 107 (45.5% patients. We specified five subgroups for patients according to their clinical, radiological, and pulmonary test findings, namely Group 1 (asthma, Group 2 (ACOS, Group 3 (chronic bronchitis, and Group 4 (emphysema. Additionally, a group of patients who had clinical and spirometric features of both asthma and chronic bronchitis in association with underlying emphysema (emphysema with chronic bronchitis and emphysema with asthma

Evaluation of Clinical Interest of Anti-Aquaporin-4 Autoantibody Followup in Neuromyelitis Optica

Directory of Open Access Journals (Sweden)

Jean-Baptiste Chanson

2013-01-01

Full Text Available Neuromyelitis optica (NMO is an autoimmune disease in which a specific biomarker named NMO-IgG and directed against aquaporin-4 (AQP4 has been found. A correlation between disease activity and anti-AQP4 antibody (Ab serum concentration or complement-mediated cytotoxicity has been reported, but the usefulness of longitudinal evaluation of these parameters remains to be evaluated in actual clinical practice. Thirty serum samples from 10 NMO patients positive for NMO-IgG were collected from 2006 to 2011. Anti-AQP4 Ab serum concentration and complement-mediated cytotoxicity were measured by flow cytometry using two quantitative cell-based assays (CBA and compared with clinical parameters. We found a strong correlation between serum anti-AQP4 Ab concentration and complement-mediated cytotoxicity (P<0.0001. Nevertheless, neither relapse nor worsening of impairment level was closely associated with a significant increase in serum Ab concentration or cytotoxicity. These results suggest that complement-mediated serum cytotoxicity assessment does not provide extra insight compared to anti-AQP4 Ab serum concentration. Furthermore, none of these parameters appears closely related to disease activity and/or severity. Therefore, in clinical practice, serum anti-AQP4 reactivity seems not helpful as a predictive biomarker in the followup of NMO patients as a means of predicting the onset of a relapse and adapting the treatment accordingly.

Radiographic Evaluation of Valvular Heart Disease With Computed Tomography and Magnetic Resonance Correlation.

Science.gov (United States)

Lempel, Jason K; Bolen, Michael A; Renapurkar, Rahul D; Azok, Joseph T; White, Charles S

2016-09-01

Valvular heart disease is a group of complex entities with varying etiologies and clinical presentations. There are a number of imaging tools available to supplement clinical evaluation of suspected valvular heart disease, with echocardiography being the most common and clinically established, and more recent emergence of computed tomography and magnetic resonance imaging as additional supportive techniques. Yet even with these newer and more sophisticated modalities, chest radiography remains one of the earliest and most common diagnostic examinations performed during the triage of patients with suspected cardiac dysfunction. Recognizing the anatomic and pathologic features of cardiac radiography including the heart's adaptation to varying hemodynamic changes can provide clues to the radiologist regarding the underlying etiology. In this article, we will elucidate several principles relating to chamber modifications in response to pressure and volume overload as well as radiographic appearances associated with pulmonary fluid status and cardiac dysfunction. We will also present a pattern approach to optimize analysis of the chest radiograph for valvular heart disease, which will help guide the radiologist down a differential diagnostic pathway and create a more meaningful clinical report.

Evaluating a mobile application for improving clinical laboratory test ordering and diagnosis.

Science.gov (United States)

Meyer, Ashley N D; Thompson, Pamela J; Khanna, Arushi; Desai, Samir; Mathews, Benji K; Yousef, Elham; Kusnoor, Anita V; Singh, Hardeep

2018-04-20

Mobile applications for improving diagnostic decision making often lack clinical evaluation. We evaluated if a mobile application improves generalist physicians' appropriate laboratory test ordering and diagnosis decisions and assessed if physicians perceive it as useful for learning. In an experimental, vignette study, physicians diagnosed 8 patient vignettes with normal prothrombin times (PT) and abnormal partial thromboplastin times (PTT). Physicians made test ordering and diagnosis decisions for 4 vignettes using each resource: a mobile app, PTT Advisor, developed by the Centers for Disease Control and Prevention (CDC)'s Clinical Laboratory Integration into Healthcare Collaborative (CLIHC); and usual clinical decision support. Then, physicians answered questions regarding their perceptions of the app's usefulness for diagnostic decision making and learning using a modified Kirkpatrick Training Evaluation Framework. Data from 368 vignettes solved by 46 physicians at 7 US health care institutions show advantages for using PTT Advisor over usual clinical decision support on test ordering and diagnostic decision accuracy (82.6 vs 70.2% correct; P < .001), confidence in decisions (7.5 vs 6.3 out of 10; P < .001), and vignette completion time (3:02 vs 3:53 min.; P = .06). Physicians reported positive perceptions of the app's potential for improved clinical decision making, and recommended it be used to address broader diagnostic challenges. A mobile app, PTT Advisor, may contribute to better test ordering and diagnosis, serve as a learning tool for diagnostic evaluation of certain clinical disorders, and improve patient outcomes. Similar methods could be useful for evaluating apps aimed at improving testing and diagnosis for other conditions.

Hematologic manifestations of Crohn's disease: two clinical cases

Directory of Open Access Journals (Sweden)

O. V. Taratina

2017-01-01

Full Text Available Inflammatory bowel diseases (IBD are commonly associated with extraintestinal manifestations, hematological disorders being the most special among them. In some cases, they dominate the clinical picture masking the intestinal manifestations of the underlying disease. Aplastic anemia is an extremely rare extraintestinal IBD manifestation. There are only two clinical cases of aplastic anemia associated with ulcerative colitis and non with Crohn's disease reported in the literature. Combination of Crohn's disease and В₁₂-deficient anemia is more prevalent, but is seen usually only after more than 20 cm of the ileus has been resected. The first clinical case presented in this paper is a  combination of severe fistula-forming Crohn's disease with a constriction in the terminal part of the ileus and profound pancytopenia as an outcome of aplastic anemia. This profound pancytopenia is associated with an extremely high risk of life-threatening complications both of surgical treatment, as well as of several chemotherapeutic agents, which made the management of this patient difficult. The second clinical case demonstrates the manifestation of Crohn's disease as ileocolitis starting from the symptoms of cobalamin deficiency: severe В₁₂-deficient anemia, funicular myelosis and sensory ataxia, with blunted intestinal symptoms. This made the initial diagnosis and timely treatment difficult. Replacement therapy with cobalamin injections and treatment with glucocorticoids and antibacterials led to endoscopically confirmed remission of Crohn's disease and normalization of hematological parameters, with persistent polyneuropathy. Thus, management of patients with Crohn's disease should be multidisciplinary. In the case of anemia, leucopenia and/or thrombocytopenia in IBD patients it is necessary to exclude potential myelodysplasia and bone marrow aplasia. In the event of megaloblastic anemia and/or progressive polyneuropathy one should bear in mind

From Appearance of Adrenal Autoantibodies to Clinical Symptoms of Addison's Disease: Natural History.

Science.gov (United States)

Betterle, Corrado; Garelli, Silvia; Presotto, Fabio; Furmaniak, Jadwiga

2016-01-01

Recent progress in the immunopathology field has greatly improved our understanding of the natural history of autoimmune diseases, particularly of Addison's disease. Addison's disease is known to be a chronic illness characterized by adrenocortical gland insufficiency that develops following a long and mainly asymptomatic period, characterized by the presence of circulating autoantibodies directed to adrenal cortex antigens. In this chapter we describe the groups of subjects at risk of developing Addison's disease, together with the diagnostic tests considered the most appropriate for evaluating adrenal function: determination of basal plasma adrenocorticotropic hormone (ACTH) levels, plasma renin activity, plasma aldosterone and cortisol levels, and cortisol levels after intravenous stimulation with ACTH (ACTH test). The employment of specific clinical, immunological and functional criteria in the subjects with autoantibodies to the adrenal cortex allows identifying those at risk of developing overt disease. The independent risk factors for the progression to adrenal failure have also been identified and they contribute to different risks of developing clinical Addison's disease. Based on the risk level, the subjects should be monitored over time to observe early signs of adrenal dysfunction, and start substitutive treatment as soon as possible. For patients presenting with high risk, prevention strategies and trials might be available. © 2016 S. Karger AG, Basel.

The Clinical Evaluation of Parkinson's Tremor

NARCIS (Netherlands)

Zach, H.; Dirkx, M.; Bloem, B.R.; Helmich, R.C.

2015-01-01

Parkinson's disease harbours many different tremors that differ in distribution, frequency, and context in which they occur. A good clinical tremor assessment is important for weighing up possible differential diagnoses of Parkinson's disease, but also to measure the severity of the tremor as a

Sarcopenia in Alcoholic Liver Disease: Clinical and Molecular Advances.

Science.gov (United States)

Dasarathy, Jaividhya; McCullough, Arthur J; Dasarathy, Srinivasan

2017-08-01

Despite advances in treatment of alcohol use disorders that focus on increasing abstinence and reducing recidivism, alcoholic liver disease (ALD) is projected to be the major cause of cirrhosis and its complications. Malnutrition is recognized as the most frequent complication in ALD, and despite the high clinical significance, there are no effective therapies to reverse malnutrition in ALD. Malnutrition is a relatively imprecise term, and sarcopenia or skeletal muscle loss, the major component of malnutrition, is primarily responsible for the adverse clinical consequences in patients with liver disease. It is, therefore, critical to define the specific abnormality (sarcopenia) rather than malnutrition in ALD, so that therapies targeting sarcopenia can be developed. Skeletal muscle mass is maintained by a balance between protein synthesis and proteolysis. Both direct effects of ethanol (EtOH) and its metabolites on the skeletal muscle and the consequences of liver disease result in disturbed proteostasis (protein homeostasis) and consequent sarcopenia. Once cirrhosis develops in patients with ALD, abstinence is unlikely to be effective in completely reversing sarcopenia, as other contributors including hyperammonemia, hormonal, and cytokine abnormalities aggravate sarcopenia and maintain a state of anabolic resistance initiated by EtOH. Cirrhosis is also a state of accelerated starvation, with increased gluconeogenesis that requires amino acid diversion from signaling and substrate functions. Novel therapeutic options are being recognized that are likely to supplant the current "deficiency replacement" approach and instead focus on specific molecular perturbations, given the increasing availability of small molecules that can target specific signaling components. Myostatin antagonists, leucine supplementation, and mitochondrial protective agents are currently in various stages of evaluation in preclinical studies to prevent and reverse sarcopenia, in cirrhosis in

Integrating Transgenic Vector Manipulation with Clinical Interventions to Manage Vector-Borne Diseases.

Directory of Open Access Journals (Sweden)

Kenichi W Okamoto

2016-03-01

Full Text Available Many vector-borne diseases lack effective vaccines and medications, and the limitations of traditional vector control have inspired novel approaches based on using genetic engineering to manipulate vector populations and thereby reduce transmission. Yet both the short- and long-term epidemiological effects of these transgenic strategies are highly uncertain. If neither vaccines, medications, nor transgenic strategies can by themselves suffice for managing vector-borne diseases, integrating these approaches becomes key. Here we develop a framework to evaluate how clinical interventions (i.e., vaccination and medication can be integrated with transgenic vector manipulation strategies to prevent disease invasion and reduce disease incidence. We show that the ability of clinical interventions to accelerate disease suppression can depend on the nature of the transgenic manipulation deployed (e.g., whether vector population reduction or replacement is attempted. We find that making a specific, individual strategy highly effective may not be necessary for attaining public-health objectives, provided suitable combinations can be adopted. However, we show how combining only partially effective antimicrobial drugs or vaccination with transgenic vector manipulations that merely temporarily lower vector competence can amplify disease resurgence following transient suppression. Thus, transgenic vector manipulation that cannot be sustained can have adverse consequences-consequences which ineffective clinical interventions can at best only mitigate, and at worst temporarily exacerbate. This result, which arises from differences between the time scale on which the interventions affect disease dynamics and the time scale of host population dynamics, highlights the importance of accounting for the potential delay in the effects of deploying public health strategies on long-term disease incidence. We find that for systems at the disease-endemic equilibrium, even

Clinical evaluation of chitotriosidase enzyme activity in Gaucher and Niemann Pick A/B diseases: A retrospective study from India.

Science.gov (United States)

Kadali, Srilatha; Kolusu, Anusha; Sunkara, Satish; Gummadi, Maheshwar Reddy; Undamatla, Jayanthi

2016-06-01

Plasma chitotriosidase originates from activated macrophages and is reported to be elevated in many Lysosomal Storage Disorders. Measurement of this enzyme activity has been an available tool for monitoring therapy of Gaucher disease. The degree of elevation of chitotriosidase is useful for differential diagnosis of Gaucher disease and Niemann Pick A/B. However the potential utility of this chitotriosidase assay depends on the frequency of deficient chitotriosidase activity in a particular population. We therefore aim to study the clinical utility of this assay Gaucher and Niemann Pick A/B diseases in the backdrop of chitotriosidase deficiency in our population. The study comprises 173 patients with clinical suspicion of either Gaucher disease (n=108) or Niemann Pick A/B (n=65) and 92 healthy controls. The plasma samples of controls, Gaucher disease, and Niemann Pick A/B showed chitotriosidase deficiency of 12%, 25% and 27% respectively. The degree of elevation of chitotriosidase in Gaucher disease and Niemann Pick A/B patients is 40-326 (11,325.7±6395.4nmol/h/ml) and 7-22 folds (1192.5±463.0nmol/h/ml) respectively. In view of these findings of distinguishable fold elevation of chitotriosidase in Gaucher disease or Niemann Pick A/B, it can be a potential surrogate differential diagnostic marker for these groups of diseases, except in the patients in whom this enzyme is deficient. Copyright © 2016 Elsevier B.V. All rights reserved.

Clinical relevance of precore mutations of hepatitis B virus in chronic liver disease

Directory of Open Access Journals (Sweden)

Chaloska-Ivanova Viktorija

2014-07-01

Full Text Available Introduction: Hepatitis B is one of the most frequent etiological factors for chronic liver diseases worldwide. Recent studies have suggested the important role of the genetic diversity of the virus on natural course of hepatitis B. Hepatitis B e-antigen negative type of chronic hepatitis is associated with mutations in the precore region and basic core promoter of hepatitis B viral genome. Aim of study was to identify precore mutations in viral genome of patients with chronic hepatitis B and to evaluate clinical patterns of liver disease related to this type of hepatitis B. Methods: Sixty seven patients with hepatitis B were included in the study. In order to evaluate the clinical patterns of chronic liver disease related to hepatitis B viral infection, biochemical and virological investigations were done, as well as a quantification of serum viral load. All patients underwent liver biopsy and semiquantification of necroinflammation and/or fibrosis according to Knodell scoring was done. In the group of e antigen-negative patients, molecular analysis was performed in order to identify presence of mutations in precore region of the virus. Results: Study group was divided in 25 HBeAg-positive and 42 HBeAg-negative subjects. Al anin-aminotransferase activity and level of viral load were higher in HBeAg-positive (p < 0.05, but average age and histology activity index were significantly higher in the HBeAg-negative patients (p < 0.01. Precore mutants were found in 38 of 42 patients with HBeAg-negative hepatitis (90%. Fibrosis was found in 30/38 cases with mutations. Discussion: Mutations in precore region of HBV in HBeAg-negative patients were more prevalent in older age and were associated with higher rate of fibrosis in liver tissue, meaning more advanced stage of the disease. This could be a consequence of longer duration of HBV infection or more severe clinical course of the disease. Conclusion: Our results suggest that precore mutations are

Clinical and imaging diagnosis of IgG4-related disease in the head and neck

International Nuclear Information System (INIS)

Yu Changliang; Liu Bin; Yu Yongqiang

2013-01-01

IgG4-related disease in the head and neck is a newly recognized multi-organ system disease characterized by elevated serum IgG4, infiltration of numerous IgG4-positive plasma cells, tissue fibrosis, and dramatic response to corticosteroid treatment. IgG4-related disease of the head and neck has some relative characteristics on CT and MRI, which can provide valuable information for the diagnosis and differential diagnosis, and are helpful for the clinical treatment, evaluation of therapeutic effects and prediction of prognosis. (authors)

The clinical relevance of plasma CD147/basigin in biopsy-proven kidney diseases.

Science.gov (United States)

Mori, Yoshiko; Masuda, Tomohiro; Kosugi, Tomoki; Yoshioka, Tomoki; Hori, Mayuko; Nagaya, Hiroshi; Maeda, Kayaho; Sato, Yuka; Kojima, Hiroshi; Kato, Noritoshi; Ishimoto, Takuji; Katsuno, Takayuki; Yuzawa, Yukio; Kadomatsu, Kenji; Maruyama, Shoichi

2017-12-12

Precise understanding of kidney disease activity is needed to design therapeutic strategies. CD147/basigin is involved in the pathogenesis of acute kidney injury and renal fibrosis through inflammatory cell infiltration. The present study examined the clinical relevance of CD147 in biopsy-proven kidney diseases that lead to the progression of chronic kidney disease. Kidney biopsy specimens and plasma and urine samples were obtained from patients with kidney diseases, including IgA nephropathy (IgAN), Henoch-Schönlein purpura nephritis (HSPN), diabetic kidney disease (DKD), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (MN), who underwent renal biopsy between 2011 and 2014. Plasma and urinary CD147 levels were measured and evaluated for their ability to reflect histological features. Disease activity of IgAN tissues was evaluated according to the Oxford classification and the Japanese histological grading system. In biopsy tissues, CD147 induction was detected in injured lesions representing renal inflammation. Plasma CD147 values correlated with eGFR in patients with inflammation-related kidney diseases such as IgAN, HSPN, and DKD. Particularly in IgAN patients, plasma CD147 levels were correlated with injured regions comprising more than 50% of glomeruli or with tubular atrophy/interstitial injury in biopsy tissues. Proteinuria showed a closer correlation with urinary values of CD147 and L-FABP. Of note, plasma and urinary CD147 levels showed a strong correlation with eGFR or proteinuria, respectively, only in DKD patients. Evaluation of plasma and urinary CD147 levels might provide key insights for the understanding of the activity of various kidney diseases.

Clinical Application of 18F-FDG PET in Alzheimer's Disease

International Nuclear Information System (INIS)

Ryu, Young Hoon

2008-01-01

PET of the cerebral metabolic rate of glucose is increasingly used to support the clinical diagnosis in the examination of patients with suspected major neurodegenerative disorders, such as Alzheimer's disease. 18 F-FDG PET has been reported to have high diagnostic performance, especially, very high sensitivity in the diagnosis and clinical assessment of therapeutic efficacy. According to clinical research data hitherto, 18 F-FDG PET is expected to be an effective diagnostic tool in early and differential diagnosis of Alzheimer's disease. Since 2004, Medicare covers 18 F-FDG PET scans for the differential diagnosis of fronto-temporal dementia (FTD) and Alzheimer's disease (AD) under specific requirements; or, its use in a CMS approved practical clinical trial focused on the utility of 18 F-FDG PET in the diagnosis or treatment of dementing neurodegenerative diseases

Neurophysiological evaluation of patients with degenerative diseases of the cervical spine

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Ilić Tihomir V.

2011-01-01

Full Text Available Bacground/Aim. Diagnostic protocol for patients with degenerative diseases of the cervical spine demands, in parallel with neuroimaging methods, functional evaluation through neurophysiological methods (somatosensitive and motor evoked potentials and electromyoneurography aiming to evaluate possible subclinical affection of spinal medula resulting in neurological signs of long tract abnormalities. Considering diversities of clinical outcomes for these patients, complex diagnostic evaluation provides a prognosis of the disease progression. Methods. The study included 21 patients (48.24 ± 11.01 years of age with clinical presentation of cervical spondylarthropathy, without neuroradiological signs of myelopathy. For each patient, in addition to conventional neurophysiological tests (somatisensory evoked potentials - SSEP, motor evoked potentials - MEP, electromyoneurography - EMG, nerve conduction studies, we calculated central motor conduction time (CMCTF, as well the same parameter in relation to a different position of the head (maximal anteflexion and retroflexion, so-called dynamic tests. Results. Abnormalities of the peripheral motor neurone by conventional EMNeG was established in 2/3 of the patients, correponding to the findings of root condution time. Prolonged conventional CVMPF were found in 29% of the patients, comparing to 43% CVMPF abnormalities found with the dynamic tests. In addition, the SSEP findings were abnormal in 38% of the patients with degenerative diseases of the cervical spine. Conclusion. An extended neurophysiological protocol of testing corticospinal functions, including dynamic tests of central and periheral motor neurons are relevant for detection of subclinical forms of cervical spondylothic myelopathy, even at early stages. In addition to the conventional neurophysiological tests, we found usefull to include the dynamic motor tests and root conduction time measurement in diagnostic evaluation.

Fluorophotometry as a diagnostic tool for the evaluation of dry eye disease

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Fan Vincent C

2006-05-01

Full Text Available Abstract Background Dry eye disease is a common debilitating ocular disease. Current diagnostic tests used in dry eye disease are often neither sensitive nor reproducible, making it difficult to accurately diagnose and determine end points for clinical trials, or evaluate the usefulness of different medications in the treatment of dry eye disease. The recently developed fluorophotometer can objectively detect changes in the corneal epithelium by quantitatively measuring its barrier function or permeability. The purpose of the study is to investigate the use of corneal fluorescein penetration measured by the fluorophotometer as a diagnostic tool in the evaluation of dry eye patients. Methods Dry eye patients (16 eyes, who presented with a chief complaint of ocular irritation corresponding with dry eye, low Schirmer's one test ( Results Ten minutes after fluorescein installition, patients with dry eye disease averaged a five-fold increase in corneal tissue fluorescein concentration (mean = 375.26 ± 202.67 ng/ml compared with that of normal subjects (mean = 128.19 ± 85.84 ng/ml. Sixty minutes after dye installation, patients with dry eye disease still revealed higher corneal tissue fluorescein concentration (mean = 112.87 ± 52.83 ng/ml compared with that of controls (mean = 40.64 ± 7.96 ng/ml, averaging a three-fold increase. Conclusion Patients with dry eye disease demonstrated an increased corneal permeability and a slower rate of elimination to topically administered fluorescein when measured by the fluorophotometer. This suggests that fluorophotometry may serve as a valuable quantitative and objective tool for the diagnosis of dry eye disease, and in following patients' response to new treatment modalities. Fluorophotometry may serve as an objective non-invasive tool for end-point analysis in clinical trials of new treatments for dry eye disease.

Evaluating impact of clinical guidelines using a realist evaluation framework.

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Reddy, Sandeep; Wakerman, John; Westhorp, Gill; Herring, Sally

2015-12-01

The Remote Primary Health Care Manuals (RPHCM) project team manages the development and publication of clinical protocols and procedures for primary care clinicians practicing in remote Australia. The Central Australian Rural Practitioners Association Standard Treatment Manual, the flagship manual of the RPHCM suite, has been evaluated for accessibility and acceptability in remote clinics three times in its 20-year history. These evaluations did not consider a theory-based framework or a programme theory, resulting in some limitations with the evaluation findings. With the RPHCM having an aim of enabling evidence-based practice in remote clinics and anecdotally reported to do so, testing this empirically for the full suite is vital for both stakeholders and future editions of the RPHCM. The project team utilized a realist evaluation framework to assess how, why and for what the RPHCM were being used by remote practitioners. A theory regarding the circumstances in which the manuals have and have not enabled evidence-based practice in the remote clinical context was tested. The project assessed this theory for all the manuals in the RPHCM suite, across government and aboriginal community-controlled clinics, in three regions of Australia. Implementing a realist evaluation framework to generate robust findings in this context has required innovation in the evaluation design and adaptation by researchers. This article captures the RPHCM team's experience in designing this evaluation. © 2015 John Wiley & Sons, Ltd.

Quality Assessment of Clinical Practice Guidelines for Respiratory Diseases in China: A Systematic Appraisal.

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Jiang, Mei; Liao, Li-Yue; Liu, Xiao-Qing; He, Wei-Qun; Guan, Wei-Jie; Chen, Hao; Li, Yi-Min

2015-09-01

There has been a significant increase in the publication of clinical practice guidelines (CPGs) for respiratory diseases in China. However, little is known about the quality and potential impacts of these CPGs. Our objective was to critically evaluate the quality of Chinese CPGs for respiratory diseases that were published in peer-reviewed medical journals. A systematic search of scientific literature published between 1979 and 2013 was undertaken to identify and select CPGs that were related to respiratory diseases. Four Chinese databases (the Chinese Biomedical Literature database [CBM], the China National Knowledge Infrastructure [CNKI], the VIP database, and the WANFANG database) were used. The quality of eligible guidelines was assessed independently by four reviewers using the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument. The overall agreement among reviewers was evaluated using an intraclass correlation coefficient. A total of 109 guidelines published in 27 medical journals from 1979 to 2013 were evaluated. The overall agreement among reviewers was considered good (intraclass correlation coefficient, 0.838; 95% CI, 0.812-0.862). The scores of the six AGREE domains were low: 57.3% for scope and purpose (range, 4.2%-80.5%), 23.8% for stakeholder involvement (range, 2.8%-54.2%), 7.7% for rigor of development (range, 0%-27.1%), 59.8% for clarity and presentation (range, 22.2%-80.6%), 10.9% for applicability (range, 0%-22.9%), and 0.6% for editorial independence (range, 0%-16.7%). Scores for all guidelines were below 60%, and only three guidelines (2.8%) were recommended for clinical practice with modifications. The quality of the guidelines was low, and stakeholder involvement, rigor of development, applicability, and editorial independence should be considered in the future development of CPGs for respiratory diseases in China.

Ultrasound and computer tomography in the evaluation of focal liver disease

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Bartolozzi, C; Ciatti, S; Lucarelli, E; Villari, N; de Dominicis, R [Florence Univ. (Italy). Ist. di Radiologia

1981-01-01

The results of ultrasound and computer tomography in 150 patients with possible focal liver disease were compared. Ultrasound was more sensitive than CT in detecting lesions but similar information on the nature of the lesion was obtained with both methods. CT provided more complete information than US on the extent of the lesion. Thus, CT and US are supplementary in the evaluation of focal liver disease. Because of the higher sensitivity US should be the first examination, followed, if clinically indicated, by CT for confirmation and possible further information regarding the extent of the lesion.

Motor outcome measures in Huntington disease clinical trials.

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Reilmann, Ralf; Schubert, Robin

2017-01-01

Deficits in motor function are a hallmark of Huntington disease (HD). The Unified Huntington's Disease Rating Scale Total Motor Score (UHDRS-TMS) is a categoric clinical rating scale assessing multiple domains of motor disability in HD. The UHDRS-TMS or subsets of its items have served as primary or secondary endpoints in numerous clinical trials. In spite of a well-established video-based annual online certification system, intra- and interrater variability, subjective error, and rater-induced placebo effects remain a concern. In addition, the UHDRS-TMS was designed to primarily assess motor symptoms in manifest HD. Recently, advancement of technology resulted in the introduction of the objective Q-Motor (i.e., Quantitative-Motor) assessments in biomarker studies and clinical trials in HD. Q-Motor measures detected motor signs in blinded cross-sectional and longitudinal analyses of manifest, prodromal, and premanifest HD cohorts up to two decades before clinical diagnosis. In a multicenter clinical trial in HD, Q-Motor measures were more sensitive than the UHDRS-TMS and exhibited no placebo effects. Thus, Q-Motor measures are currently explored in several multicenter trials targeting both symptomatic and disease-modifying mechanisms. They may supplement the UHDRS-TMS, increase the sensitivity and reliability in proof-of-concept studies, and open the door for phenotype assessments in clinical trials in prodromal and premanifest HD. Copyright © 2017 Elsevier B.V. All rights reserved.

Electrophysiological Evaluation of Oropharyngeal Dysphagia in Parkinson’s Disease

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Ertekin, Cumhur

2014-01-01

Parkinson’s disease (PD) is a chronic, neurodegenerative movement disorder that typically affects elderly patients. Swallowing disorders are highly prevalent in PD and can have grave consequences, including pneumonia, malnutrition, dehydration and mortality. Neurogenic dysphagia in PD can manifest with both overt clinical symptoms or silent dysphagia. Regardless, early diagnosis and objective follow-up of dysphagia in PD is crucial for timely and appropriate care for these patients. In this review, we provide a comprehensive summary of the electrophysiological methods that can be used to objectively evaluate dysphagia in PD. We discuss the electrophysiological abnormalities that can be observed in PD, their clinical correlates and the pathophysiology underlying these findings. PMID:25360228

[Clinical trials in dermatology. Evaluation of the tolerability and efficacy of a topical anti-acne].

Science.gov (United States)

Verschoore, M; Poncet, M; Schaefer, H; Revuz, J; De Prost, Y; Guillaume, J C; Ortonne, J P; Czernielewski, J

1991-01-01

Acne is a frequent dermatologic disease of the teenagers. Methodology of antiacne preparations clinical trials has evolved recently, leading to better comprehension of acne treatment on acne lesions. The main rules for good clinical evaluation of acne treatments are: an objective counting of each individual lesions on a defined area (face, back), a global acne assessment, a therapy duration from 1 to 3 months or more, a skin safety evaluation for erythema, desquamation, dryness, itching, burning and oiliness with a 0 to 3 scoring system.

IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

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N. V. Fedorova

2015-01-01

Full Text Available Objective – a description of clinical cases of impulsive­compulsive disorders in Parkinson,s disease. The first clinical case. Patient N., 75 years old, suffering for 15 years from Parkinson,s disease, akinetic­rigid form of the disease, stage 4 by Hyun–Yar. Since 2009, he received levodopa/carbidopa 250/25 mg 5 times/day (daily dose of 1250 mg of levodopa; pramipexole 3.5 mg per day (daily dose 3.5 mg, amantadine sulfate 100 mg 5 times/day (daily dose 500 mg. While taking antiparkinsonian drugs the patient developed behavioral disorders such as dopamine disregulatory syndrome combined with punding, hypersexuality and compulsive shopping accompanied by visual hallucinations. The total equivalent dose of levodopa was 1600 mg per day. The second clinical case. Patient R., 52 years old, suffers from Parkinson,s disease about 5 years, a mixed form. She complained of slowness of movement, tremor in her left hand, sleep disturbances, poor mood. The clinic was appointed piribedil 50 mg 3 times per day. Despite the fact that the patient took only one of dopaminergic drugs in a therapeutic daily dose, she developed impulsive­compulsive disorder as hyper­ sexuality, compulsive shopping and binge eating. Results. In the first clinical case for correction of behavioral disorders in patients with Parkinson,s disease levodopa/carbidopa dose was reduced to 750 mg per day (3/4 Tab. 4 times a day; added to levodopa/benserazide dispersible 100 mg morning and levodopa/benserazide 100 mg before sleep (total dose of levodopa of 950 mg per day. Amantadine sulfate and pramipexole were canceled. It was added to the therapy of atypical neuroleptic clozapine dose 6,25 mg overnight. After 3 months marked improvement, regressed visual hallucina­ tions, improved family relationships, background mood became more stable. The patient continue to sing karaoke, but this hobby has be­ come less intrusive. In the second clinical event correction impulsive

Alzheimer's disease: Cerebrovascular dysfunction, oxidative stress, and advanced clinical therapies

NARCIS (Netherlands)

Marlatt, M.W.; Lucassen, P.J.; Perry, G.; Smith, M.A.; Zhu, X.

2008-01-01

Many lines of independent research have provided convergent evidence regarding oxidative stress, cerebrovascular disease, dementia, and Alzheimer's disease (AD). Clinical studies spurred by these findings engage basic and clinical communities with tangible results regarding molecular targets and

Advanced and controlled drug delivery systems in clinical disease management

NARCIS (Netherlands)

Brouwers, JRBJ

1996-01-01

Advanced and controlled drug delivery systems are important for clinical disease management. In this review the most important new systems which have reached clinical application are highlighted. Microbiologically controlled drug delivery is important for gastrointestinal diseases like ulcerative

Clinical evaluation of cardiovascular disease by gated-MRI (magnetic resonance imaging) in the operating field of 0.35 and 1.5 Tesla

International Nuclear Information System (INIS)

Nishimura, Tsunehiko; Naito, Hiroaki; Yamada, Yukinori; Kozuka, Takahiro

1985-01-01

To evaluate the clinical usefulness of magnetic resonance imaging (MRI) in the cardiovascular disease, 21 patients were examined using 0.35 and 1.5 Tesla superconductive type (Magnetom, Siemens). In our study, all patients were performed using ECG-gated MRI. Therefore, the cardiac chambers were discriminated clearly from the myocardial wall compared to non-gated MRI. Gated-MRI was performed in 6 normal persons in the operating field at 0.35 and 1.5 Tesla. The image of the latter showed superior than that of the former because of high S/N ratio. In myocardial infarction, infarct area was demonstrated as the wall thinning in 4 of 5 patients. Hypertrophic cardiomyopathy showed thickened left ventricle associated with its narrowed cavity in 7 patients. In the remaining such as congenital and valvular heart disease, global and regional cardiac morphology were assessed noninvasively by gated MRI. In addition, gated MRI was also applied to the diagnosis of peripheral vascular diseases. In dissecting aneurysm, double channels with an intimal flap in the aorta were clearly visualized. And in the aortitis syndrome, aortic dilatation and stenosis were also assessed noninvasively. In conclusion, gated MRI in diagnosing various abnormalities of cardiovascular disease was confirmed. (author)

Hunter disease eClinic: interactive, computer-assisted, problem-based approach to independent learning about a rare genetic disease

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Moldovan Laura

2010-10-01

Full Text Available Abstract Background Computer-based teaching (CBT is a well-known educational device, but it has never been applied systematically to the teaching of a complex, rare, genetic disease, such as Hunter disease (MPS II. Aim To develop interactive teaching software functioning as a virtual clinic for the management of MPS II. Implementation and Results The Hunter disease eClinic, a self-training, user-friendly educational software program, available at the Lysosomal Storage Research Group (http://www.lysosomalstorageresearch.ca, was developed using the Adobe Flash multimedia platform. It was designed to function both to provide a realistic, interactive virtual clinic and instantaneous access to supporting literature on Hunter disease. The Hunter disease eClinic consists of an eBook and an eClinic. The eClinic is the interactive virtual clinic component of the software. Within an environment resembling a real clinic, the trainee is instructed to perform a medical history, to examine the patient, and to order appropriate investigation. The program provides clinical data derived from the management of actual patients with Hunter disease. The eBook provides instantaneous, electronic access to a vast collection of reference information to provide detailed background clinical and basic science, including relevant biochemistry, physiology, and genetics. In the eClinic, the trainee is presented with quizzes designed to provide immediate feedback on both trainee effectiveness and efficiency. User feedback on the merits of the program was collected at several seminars and formal clinical rounds at several medical centres, primarily in Canada. In addition, online usage statistics were documented for a 2-year period. Feedback was consistently positive and confirmed the practical benefit of the program. The online English-language version is accessed daily by users from all over the world; a Japanese translation of the program is also available. Conclusions The

Hunter disease eClinic: interactive, computer-assisted, problem-based approach to independent learning about a rare genetic disease.

Science.gov (United States)

Al-Jasmi, Fatma; Moldovan, Laura; Clarke, Joe T R

2010-10-25

Computer-based teaching (CBT) is a well-known educational device, but it has never been applied systematically to the teaching of a complex, rare, genetic disease, such as Hunter disease (MPS II). To develop interactive teaching software functioning as a virtual clinic for the management of MPS II. The Hunter disease eClinic, a self-training, user-friendly educational software program, available at the Lysosomal Storage Research Group (http://www.lysosomalstorageresearch.ca), was developed using the Adobe Flash multimedia platform. It was designed to function both to provide a realistic, interactive virtual clinic and instantaneous access to supporting literature on Hunter disease. The Hunter disease eClinic consists of an eBook and an eClinic. The eClinic is the interactive virtual clinic component of the software. Within an environment resembling a real clinic, the trainee is instructed to perform a medical history, to examine the patient, and to order appropriate investigation. The program provides clinical data derived from the management of actual patients with Hunter disease. The eBook provides instantaneous, electronic access to a vast collection of reference information to provide detailed background clinical and basic science, including relevant biochemistry, physiology, and genetics. In the eClinic, the trainee is presented with quizzes designed to provide immediate feedback on both trainee effectiveness and efficiency. User feedback on the merits of the program was collected at several seminars and formal clinical rounds at several medical centres, primarily in Canada. In addition, online usage statistics were documented for a 2-year period. Feedback was consistently positive and confirmed the practical benefit of the program. The online English-language version is accessed daily by users from all over the world; a Japanese translation of the program is also available. The Hunter disease eClinic employs a CBT model providing the trainee with realistic

Refractory coeliac disease in a country with a high prevalence of clinically-diagnosed coeliac disease.

Science.gov (United States)

Ilus, T; Kaukinen, K; Virta, L J; Huhtala, H; Mäki, M; Kurppa, K; Heikkinen, M; Heikura, M; Hirsi, E; Jantunen, K; Moilanen, V; Nielsen, C; Puhto, M; Pölkki, H; Vihriälä, I; Collin, P

2014-02-01

Refractory coeliac disease (RCD) is thought to be a rare disorder, but the accurate prevalence is unknown. We aimed to identify the prevalence of and the risk factors for developing RCD in a Finnish population where the clinical detection rate of coeliac disease is high. The study involved 11 hospital districts in Finland where the number of treated RCD patients (n = 44), clinically diagnosed coeliac disease patients (n = 12 243) and adult inhabitants (n = 1.7 million) was known. Clinical characteristics at diagnosis of coeliac disease between the RCD patients and patients with uncomplicated disease were compared. The prevalence of RCD was 0.31% among diagnosed coeliac disease patients and 0.002% in the general population. Of the enrolled 44 RCD patients, 68% had type I and 23% type II; in 9% the type was undetermined. Comparing 886 patients with uncomplicated coeliac disease with these 44 patients that developed RCD later in life, the latter were significantly older (median 56 vs 44 years, P coeliac disease. Patients with evolving RCD had more severe symptoms at the diagnosis of coeliac disease, including weight loss in 36% (vs. 16%, P = 0.001) and diarrhoea in 54% (vs. 38%, P = 0.050). Refractory coeliac disease is very rare in the general population. Patients of male gender, older age, severe symptoms or seronegativity at the diagnosis of coeliac disease are at risk of future refractory coeliac disease and should be followed up carefully. © 2014 John Wiley & Sons Ltd.

Clinical trials and late-stage drug development for Alzheimer’s disease: an appraisal from 1984 to 2014

Science.gov (United States)

Schneider, Lon S.; Mangialasche, Francesca; Andreasen, Niels; Feldman, Howard; Giacobini, Ezio; Jones, Roy; Mantua, Valentina; Mecocci, Patrizia; Pani, Luca; Winblad, Bengt; Kivipelto, Miia

2014-01-01

The modern era of drug development for Alzheimer’s disease began with the proposal of the cholinergic hypothesis of memory impairment and the 1984 research criteria for Alzheimer’s disease. Since then, despite the evaluation of numerous potential treatments in clinical trials, only four cholinesterase inhibitors and memantine have shown sufficient safety and efficacy to allow marketing approval at an international level. Although this is probably because the other drugs tested were ineffective, inadequate clinical development methods have also been blamed for the failures. Here we review the development of treatments for Alzheimer’s disease during the past 30 years, considering the drugs, potential targets, late-stage clinical trials, development methods, emerging use of biomarkers and evolution of regulatory considerations in order to summarize advances and anticipate future developments. We have considered late-stage Alzheimer’s disease drug development from 1984 to 2013, including individual clinical trials, systematic and qualitative reviews, meta-analyses, methods, commentaries, position papers and guidelines. We then review the evolution of drugs in late clinical development, methods, biomarkers and regulatory issues. Although a range of small molecules and biological products against many targets have been investigated in clinical trials, the predominant drug targets have been the cholinergic system and the amyloid cascade. Trial methods have evolved incrementally: inclusion criteria have largely remained focused on mild to moderate Alzheimer’s disease criteria, recently extending to early or prodromal Alzheimer disease or ‘mild cognitive impairment due to Alzheimer’s disease’, for drugs considered to be disease modifying. The duration of trials has remained at 6 to 12 months for drugs intended to improve symptoms; 18- to 24-month trials have been established for drugs expected to attenuate clinical course. Cognitive performance, activities

To evaluate the differences of risk factors in patients with lower extremity venous disease

International Nuclear Information System (INIS)

Sadikoglu, G.; Ozcakir, A.; Ercan, I.; Ozkaya, G.; Sadikoglu, Y.M.

2007-01-01

To determine whether there is difference between risk factors in patients diagnosed to have clinically documented lower extremity venous disease after confirming the diagnosis radiologically by ultrasonographic and venographic evaluation. This study was performed from January 2002 to January 2005 in Bursa, the fourth biggest city of Turkey situated in the west of country in the Marmara region. The study center is a private imaging center working in conjunction with the Department of Health, which performs diagnostic and therapeutic vascular protocols in the region. Five hundred and fifty-three cases with clinically and radiologically documented diagnoses were evaluated with Multi-Variate Statistical Package 3.13 for the presence of pre-defined clusters of 14 different variables. Other statistical analyses were performed by the Statistical Package for Social sciences, version 13.0. Three different clusters were defined. The variables used to define the clusters were: age, gender, educational level, presence of smoking, amount of smoking (pack/per year), disease symptoms, presence of heart disease and radiologically documented diagnosis. Chronic venous insufficiency and varicose veins are venous system diseases that are most commonly present in association with more than one concomitant risk factors. (author)

Clinical spectrum of Castleman disease-associated neuropathy.

Science.gov (United States)

Naddaf, Elie; Dispenzieri, Angela; Mandrekar, Jay; Mauermann, Michelle L

2016-12-06

To define the peripheral neuropathy phenotypes associated with Castleman disease. We conducted a retrospective chart review for patients with biopsy-proven Castleman disease evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy were identified and divided into 2 groups: those with Castleman disease without POEMS syndrome (CD-PN) and those with Castleman disease with POEMS syndrome (CD-POEMS). We used a cohort of patients with POEMS as controls. Clinical, electrodiagnostic, and laboratory characteristics were collected and compared among patient subgroups. There were 7 patients with CD-PN, 20 with CD-POEMS, and 122 with POEMS. Patients with CD-PN had the mildest neuropathy characterized by predominant sensory symptoms with no pain and mild distal sensory deficits (median Neuropathy Impairment Score of 7 points). Although both patients with CD-POEMS and patients with POEMS had a severe sensory and motor neuropathy, patients with CD-POEMS were less affected (median Neuropathy Impairment Score of 33 and 66 points, respectively). The degree of severity was also reflected on electrodiagnostic testing in which patients with CD-PN demonstrated a mild degree of axonal loss, followed by patients with CD-POEMS and then those with POEMS. Demyelinating features, defined by European Federation of Neurologic Societies/Peripheral Nerve Society criteria, were present in 43% of the CD-PN, 78% of the CD-POEMS, and 86% of the POEMS group. There is a spectrum of demyelinating peripheral neuropathies associated with Castleman disease. CD-PN is sensory predominant and is the mildest phenotype, whereas CD-POEMS is a more severe sensory and motor neuropathy. Compared to the POEMS cohort, those with CD-POEMS neuropathy have a similar but less severe phenotype. Whether these patients respond differently to treatment deserves further study. © 2016 American Academy of Neurology.

Nosocomial Legionnaires’ Disease: Clinical and Radiographic Patterns

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Thomas J Marrie

1992-01-01

Full Text Available From 1981 to 1991, 55 patients (33 males, 22 females, mean age 58.6 years with nosocomial Legionnaires’ disease were studied. The mortality rate was 64%. One-half of the patients developed nosocomial Legionnaires’ disease within three weeks of admission. A surprising clinical feature was the low rate of findings of consolidation on physical examination, despite the fact that 52% of patients had this finding on chest radiograph. More than one-half of patients had pre-existing lung disease, rendering a radiographic diagnosis of pneumonia due to Legionella pneumophila impossible in 16% of cases despite microbiological confirmation. Nineteen per cent of patients who had blood cultures done had a pathogen other than L pneumophila isolated, suggesting dual infection in at least some of the patients. When the clinical and radiographic findings were combined it was noted that 40% of patients had one of three patterns suggestive of nosocomial Legionnaires’ disease: rapidly progressive pneumonia, lobar opacity and multiple peripheral opacities. However, in 60% of patients there were no distinctive features.

Ebola Virus Disease Candidate Vaccines Under Evaluation in Clinical Trials

Science.gov (United States)

2016-06-02

evidence that oral vaccines fail in populations with disturbed microbiota, poor nutrition , and high intestinal inflammation [102-104]. Additionally...countermeasure development against Ebola virus disease becoming a global public- health priority. This review summarizes the status quo of candidate...members of the mononegaviral family Filoviridae) cause two diseases recognized by the World Health Organization (WHO): Ebola virus disease (EVD) can be

Clinical significance of adrenal computed tomography in Addison's disease

International Nuclear Information System (INIS)

Sun, Zhong-Hua; Nomura, Kaoru; Toraya, Shohzoh; Ujihara, Makoto; Horiba, Nobuo; Suda, Toshihiro; Tsushima, Toshio; Demura, Hiroshi; Kono, Atsushi

1992-01-01

Adrenal computed tomographic (CT) scanning was conducted in twelve patients with Addison's disease during the clinical course. In tuberculous Addison's disease (n=8), three of four patients examined during the first two years after disease onset had bilaterally enlarged adrenals, while one of four had a unilaterally enlarged one. At least one adrenal gland was enlarged after onset in all six patients examined during the first four years. Thereafter, the adrenal glands was atrophied bilaterally, in contrast to adrenal glands in idiopathic Addison's disease which was atrophied bilaterally from disease onset (n=2). Adrenal calcification was a less sensitive clue in tracing pathogenesis, i.e., adrenal calcification was observed in five of eight patients with tuberculous Addison's disease, but not idiopathic patients. Thus, adrenal CT scanning could show the etiology of Addison's disease (infection or autoimmunity) and the phase of Addison's disease secondary to tuberculosis, which may be clinically important for initiating antituberculous treatment. (author)

Economic evaluations of pharmacogenetic approaches in infectious diseases: a review of current approaches and evaluation of critical aspects affecting their quality

Directory of Open Access Journals (Sweden)

Paolo Meoni

2013-11-01

Full Text Available Pharmacogenetics holds great potential for improving the effectiveness of treatment modalities in infectious diseases by taking into account the genetic determinants of both the host and infectious agents’ individuality. Better utilization of resources and improved therapeutic efficiency are the expected outcomes of personalized medicine using pharmacogenetic and pharmacogenomics information made available by technological advances. However, there has been growing concern in the clinical community regarding the evaluation of the true benefits of these approaches. This perception is partly due to the limited number and perceived poor quality of economic evaluations in this field, and initiatives aimed at harmonizing and communicating strategies improving the quality of these studies and their acceptance by the clinical community are greatly needed. This paper reviews current literature of economic evaluations of pharmacogenetics interventions guiding pharmacotherapy in infectious diseases. PubMed and the NHS Centre for Reviews and Dissemination databases were searched using a combination of five broad research terms related to pharmacogenetic approaches, and papers relative to economic evaluations of pharmacogenetic interventions in infectious diseases retained for further analysis. Using these criteria, a total of 14 papers were included in this review. The area of economic evaluation of pharmacogenetic interventions in infectious diseases remains understudied and would benefit from greater harmonization. The main weaknesses of evaluations reviewed in this paper seem to be represented by poor evidence of pharmacogenetic marker validation, inconsistencies in the selection of costs and utility included in the economic models and the choice of sensitivity analysis. All these factors limit the overall transparency of the studies, greater acceptance of their results and applicability to diverse and possibly resourcelimited environments where these

Clinical image quality evaluation for panoramic radiography in Korean dental clinics

International Nuclear Information System (INIS)

Choi, Bo Ram; Choi, Da Hye; Huh, Kyung Hoe; Yi, Won Jin; Heo, Min Suk; Choi, Soon Chul; Bae, Kwang Hak; Lee, Sam Sun

2012-01-01

The purpose of this study was to investigate the level of clinical image quality of panoramic radiographs and to analyze the parameters that influence the overall image quality. Korean dental clinics were asked to provide three randomly selected panoramic radiographs. An oral and maxillofacial radiology specialist evaluated those images using our self-developed Clinical Image Quality Evaluation Chart. Three evaluators classified the overall image quality of the panoramic radiographs and evaluated the causes of imaging errors. A total of 297 panoramic radiographs were collected from 99 dental hospitals and clinics. The mean of the scores according to the Clinical Image Quality Evaluation Chart was 79.9. In the classification of the overall image quality, 17 images were deemed 'optimal for obtaining diagnostic information,' 153 were 'adequate for diagnosis,' 109 were 'poor but diagnosable,' and nine were 'unrecognizable and too poor for diagnosis'. The results of the analysis of the causes of the errors in all the images are as follows: 139 errors in the positioning, 135 in the processing, 50 from the radiographic unit, and 13 due to anatomic abnormality. Panoramic radiographs taken at local dental clinics generally have a normal or higher-level image quality. Principal factors affecting image quality were positioning of the patient and image density, sharpness, and contrast. Therefore, when images are taken, the patient position should be adjusted with great care. Also, standardizing objective criteria of image density, sharpness, and contrast is required to evaluate image quality effectively.

Hotspots in clinical management of severe liver diseases

Directory of Open Access Journals (Sweden)

LYU Jiayu

2017-09-01

Full Text Available Severe liver diseases such as liver failure and acute decompensated cirrhosis have critical conditions and high mortality rates, and the prognosis of such patients is closely associated with early warning, timely dynamic assessment, and comprehensive and effective therapy. The patients require a series of effective clinical management measures for elimination of causative factors, organ support, and prevention and treatment of complications. Medical treatment-artificial liver-liver transplantation is an important modality for severe liver diseases. Granulocyte colony-stimulating factor, stem cell therapy, and bioartificial liver have a promising future, while there are still controversies over non-selective β-blocker. This article reviews the hotspots in the clinical management of severe liver diseases.

Evaluating critical thinking in clinical practice.

Science.gov (United States)

Oermann, M H

1997-01-01

Although much has been written about measurement instruments for evaluating critical thinking in nursing, this article describes clinical evaluation strategies for critical thinking. Five methods are discussed: 1) observation of students in practice; 2) questions for critical thinking, including Socratic questioning; 3) conferences; 4) problem-solving strategies; and 5) written assignments. These methods provide a means of evaluating students' critical thinking within the context of clinical practice.

Controlled clinical trial of cannabidiol in Huntington's disease.

Science.gov (United States)

Consroe, P; Laguna, J; Allender, J; Snider, S; Stern, L; Sandyk, R; Kennedy, K; Schram, K

1991-11-01

Based on encouraging preliminary findings, cannabidiol (CBD), a major nonpsychotropic constituent of Cannabis, was evaluated for symptomatic efficacy and safety in 15 neuroleptic-free patients with Huntington's Disease (HD). The effects of oral CBD (10 mg/kg/day for 6 weeks) and placebo (sesame oil for 6 weeks) were ascertained weekly under a double-blind, randomized cross-over design. A comparison of the effects of CBD and placebo on chorea severity and other therapeutic outcome variables, and on a Cannabis side effect inventory, clinical lab tests and other safety outcome variables, indicated no significant (p greater than 0.05) or clinically important differences. Correspondingly, plasma levels of CBD were assayed by GC/MS, and the weekly levels (mean range of 5.9 to 11.2 ng/ml) did not differ significantly over the 6 weeks of CBD administration. In summary, CBD, at an average daily dose of about 700 mg/day for 6 weeks, was neither symptomatically effective nor toxic, relative to placebo, in neuroleptic-free patients with HD.

The LIBERTY study: Design of a prospective, observational, multicenter trial to evaluate the acute and long-term clinical and economic outcomes of real-world endovascular device interventions in treating peripheral artery disease.

Science.gov (United States)

Adams, George L; Mustapha, Jihad; Gray, William; Hargus, Nick J; Martinsen, Brad J; Ansel, Gary; Jaff, Michael R

2016-04-01

Most peripheral artery disease (PAD) clinical device trials are supported by commercial manufacturers and designed for regulatory device approval, with extensive inclusion/exclusion criteria to support homogeneous patient populations. High-risk patients with advanced disease, including critical limb ischemia (CLI), are often excluded leading to difficulty in translating trial results into real-world clinical practice. As a result, physicians have no direct guidance regarding the use of endovascular devices. There is a need for objectively assessed studies to evaluate clinical, functional, and economic outcomes in PAD patient populations. LIBERTY is a prospective, observational, multicenter study sponsored by Cardiovascular Systems Inc (St Paul, MN) to evaluate procedural and long-term clinical and economic outcomes of endovascular device interventions in patients with symptomatic lower extremity PAD. Approximately 1,200 patients will be enrolled and followed up to 5 years: 500 patients in the "Claudicant Rutherford 2-3" arm, 600 in the "CLI Rutherford 4-5" arm, and 100 in the "CLI Rutherford 6" arm. The study will use 4 core laboratories for independent analysis and will evaluate the following: procedural and lesion success, rates of major adverse events, duplex ultrasound interpretations, wound status, quality of life, 6-minute walk test, and economic analysis. The LIBERTY Patient Risk Score(s) will be developed as a clinical predictor of outcomes to provide guidance for interventions in this patient population. LIBERTY will investigate real-world PAD patients treated with endovascular revascularization with rigorous study guidelines and independent oversight of outcomes. This study will provide observational, all-comer patient clinical data to guide future endovascular therapy. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

[Thinking about vertigo effectiveness evaluation methods in clinical research of Chinese medicine].

Science.gov (United States)

Liu, Hong-mei; Li, Tao

2014-10-01

Vertigo is a kind of patients' subjective feelings. The severity of vertigo is closely related to many factors. But we are short of a well accepted quantitative evaluation method capable of accurately and comprehensively evaluating vertigo in clinics. Reducing the onset of vertigo, enhancing the re- covery of equilibrium function, and improving the quality of life of vertigo patients should be taken as the focus of evaluating therapeutic effects. As for establishing a Chinese medical effectiveness evaluation system for vertigo, we believe we should distinguish different "diseases". We could roughly identify it as systemic vertigo and non-systemic vertigo. For systemic vertigo, the efficacy of vertigo could be comprehensively evaluated by UCLA vertigo questionnaire or dizziness handicap inventory combined with equilibrium function testing indices. But for non-systemic vertigo, the efficacy of vertigo could be comprehensively evaluated by taking UCLA vertigo questionnaire or dizziness handicap inventory as main efficacy indices. Secondly, we should analyze different reasons for vertigo, choose symptoms and signs in line with vertigo features as well as with Chinese medical theories, and formulate corresponding syndrome effectiveness standards according to different diseases. We should not simply take syndrome diagnosis standards as efficacy evaluation standards.

MRI evaluation of soft tissue hydatid disease

International Nuclear Information System (INIS)

Garcia-Diez, A.I.; Ros Mendoza, L.H.; Villacampa, V.M.; Cozar, M.; Fuertes, M.I.

2000-01-01

Infestation in soft tissue by Echinococcus granulosus is not a common disease, and its diagnosis is based on clinical, laboratory data and radiological findings. The aim of our retrospective study is to give an overview of the different signs and patterns shown by MRI that can be useful in characterizing soft tissue hydatid disease. The MRI images obtained in seven patients with soft tissue and subcutaneous hydatidosis were reviewed. Typical signs of hydatidosis were multivesicular lesions with or without hypointense peripheral ring (''rim sign''). Related to the presence and absence, respectively, of viable scolices in the microscopic exam, daughter cysts were presented either as high signal intensity or low signal intensity on T2-weighted images. Low-intensity detached layers within the cyst and peripheral enhancement with gadolinium-DTPA were also presented. Atypical signs were presented in an infected muscular cyst, a subcutaneous unilocular cyst and several unilocular cysts. Knowledge of the different patterns in MRI of soft tissue hydatid disease can be useful in diagnosing this entity. We observed that the ''rim sign'' is not as common as in other locations, and in addition, MRI seems to be of assistance when evaluating the vitality of the cysts. (orig.)

Clinical relevance of surgical site infection as defined by the criteria of the Centers for Disease Control and Prevention

DEFF Research Database (Denmark)

Henriksen, N A; Meyhoff, C S; Wetterslev, J

2010-01-01

Surgical site infection (SSI) is a common complication after abdominal surgery and the Centers for Disease Control and Prevention (CDC) criteria are commonly used for diagnosis and surveillance. The aim of this study was to evaluate whether SSI diagnosed according to CDC is clinically relevant (C...... a suitable standard definition for monitoring and identifying SSI, even if some cases of less clinically significant superficial SSI are included.......Surgical site infection (SSI) is a common complication after abdominal surgery and the Centers for Disease Control and Prevention (CDC) criteria are commonly used for diagnosis and surveillance. The aim of this study was to evaluate whether SSI diagnosed according to CDC is clinically relevant...... hospital stay or referral to an intensive care unit for SSI. The rate of CRSSI was 38 of 54 (70%) in patients with CDC-diagnosed SSI and none in patients without a CDC-diagnosed SSI. Sixty-one percent of the CDC-diagnosed SSIs were superficial, of which 48% were considered clinically relevant...

Meningococcal disease, a clinical and epidemiological review.

Science.gov (United States)

Batista, Rodrigo Siqueira; Gomes, Andréia Patrícia; Dutra Gazineo, Jorge Luiz; Balbino Miguel, Paulo Sérgio; Santana, Luiz Alberto; Oliveira, Lisa; Geller, Mauro

2017-11-01

Meningococcal disease is the acute infection caused by Neisseria meningitidis, which has humans as the only natural host. The disease is widespread around the globe and is known for its epidemical potential and high rates of lethality and morbidity. The highest number of cases of the disease is registered in the semi-arid regions of sub-Saharan Africa. In Brazil, it is endemic with occasional outbreaks, epidemics and sporadic cases occurring throughout the year, especially in the winter. The major epidemics of the disease occurred in Brazil in the 70's caused by serogroups A and C. Serogroups B, C and Y represent the majority of cases in Europe, the Americas and Australia. However, there has been a growing increase in serogroup W in some areas. The pathogen transmission happens for respiratory route (droplets) and clinically can lead to meningitis and sepsis (meningococcemia). The treatment is made with antimicrobial and supportive care. For successful prevention, we have some measures like vaccination, chemoprophylaxis and droplets' precautions. In this review, we have described and clarify clinical features of the disease caused by N. meningitidis regarding its relevance for healthcare professionals. Copyright © 2017 Hainan Medical University. Production and hosting by Elsevier B.V. All rights reserved.

Quantitative evaluation of capillaroscopic microvascular changes in patients with established coronary heart disease.

Science.gov (United States)

Sanchez-Garcia, M Esther; Ramirez-Lara, Irene; Gomez-Delgado, Francisco; Yubero-Serrano, Elena M; Leon-Acuña, Ana; Marin, Carmen; Alcala-Diaz, Juan F; Camargo, Antonio; Lopez-Moreno, Javier; Perez-Martinez, Pablo; Tinahones, Francisco José; Ordovas, Jose M; Caballero, Javier; Blanco-Molina, Angeles; Lopez-Miranda, Jose; Delgado-Lista, Javier

2018-02-23

Microcirculation disturbances have been associated to most of the cardiovascular risk factors as well as to multiple inflammatory diseases. However, whether these abnormalities are specifically augmented in patients with coronary heart disease is still unknown. We aimed to evaluate if there is a relationship between the presence of coronary heart disease and the existence of functional and structural capillary abnormalities evaluated in the cutaneous microcirculation by videocapillaroscopy. Two matched samples of 30 participants with and without coronary heart disease but with similar clinical and anthropometric characteristics were evaluated by videocapillaroscopy at the dorsal skin of the third finger of the non-dominant hand. We calculated basal capillary density as well as capillary density after a period of arterial and venous occlusion in order to evaluate functionality and maximum capillary density. We also measured capillary recruitment. Microvascular capillary density at rest was significantly lower in patients suffering from coronary heart disease than in controls. This fact was also found after dynamic tests (arterial and venous occlusion), suggesting functional impairments. Capillary recruitment of the samples was not different in our sample. In our study, patients with coronary heart disease exhibit functional and structural microvascular disturbances. Although this is a very preliminary study, these findings open the door for further studying the microvascular functionality in coronary patients and how it relates to the response to treatment and/or the prognosis of the disease. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

The clinical application of lymphoscintigraphy for the diagnosis in hematological diseases

International Nuclear Information System (INIS)

Zhu Jun; Zhu Ruisen; Zhu Jifang; Jin Changqing; Yu Jianfang

2000-01-01

Results of lymphoscintigraphy in 78 patients with clinically suspected malignant lymphoma and leukemia were reported and its clinical value for in diagnosis of hematological diseases were evaluated. Confirmed by pathological examination, 30 cases were diagnosed as malignant lymphoma and 24 cases non-malignant lymphoma. In malignant lymphoma, the sensitivity of lymphoscintigraphy was 83.3% and the specificity 62.5%, where the sensitivity of CT and ultrasound, were 83.3%, 66.7% and 22.2% respectively. Confirmed by bone marrow biopsy, leukemia was found in 9 cases and non-leukemia in 15. In leukemia, the sensitivity of lymphoscintigraphy was 88.9% and specificity 53.3%. Whereas the sensitivity of CT, was 50%. Therefore, the lymphoscintigraphy have comparatively high sensitivity for the diagnosis of malignant lymphoma and leukemia

Clinical image quality evaluation for panoramic radiography in Korean dental clinics

Energy Technology Data Exchange (ETDEWEB)

Choi, Bo Ram; Choi, Da Hye; Huh, Kyung Hoe; Yi, Won Jin; Heo, Min Suk; Choi, Soon Chul; Bae, Kwang Hak; Lee, Sam Sun [School of Dentistry, Seoul National University, Seoul (Korea, Republic of)

2012-09-15

The purpose of this study was to investigate the level of clinical image quality of panoramic radiographs and to analyze the parameters that influence the overall image quality. Korean dental clinics were asked to provide three randomly selected panoramic radiographs. An oral and maxillofacial radiology specialist evaluated those images using our self-developed Clinical Image Quality Evaluation Chart. Three evaluators classified the overall image quality of the panoramic radiographs and evaluated the causes of imaging errors. A total of 297 panoramic radiographs were collected from 99 dental hospitals and clinics. The mean of the scores according to the Clinical Image Quality Evaluation Chart was 79.9. In the classification of the overall image quality, 17 images were deemed 'optimal for obtaining diagnostic information,' 153 were 'adequate for diagnosis,' 109 were 'poor but diagnosable,' and nine were 'unrecognizable and too poor for diagnosis'. The results of the analysis of the causes of the errors in all the images are as follows: 139 errors in the positioning, 135 in the processing, 50 from the radiographic unit, and 13 due to anatomic abnormality. Panoramic radiographs taken at local dental clinics generally have a normal or higher-level image quality. Principal factors affecting image quality were positioning of the patient and image density, sharpness, and contrast. Therefore, when images are taken, the patient position should be adjusted with great care. Also, standardizing objective criteria of image density, sharpness, and contrast is required to evaluate image quality effectively.

Restless legs syndrome in Parkinson's disease: clinical characteristics and biochemical correlations

Directory of Open Access Journals (Sweden)

Tiago Machado Guerreiro

2010-12-01

Full Text Available Restless legs syndrome (RLS is a neurological disorder that responds to dopaminergic drugs, indicating a common pathophysiology with Parkinson's disease (PD. The prevalence of RLS was estimated in a group of PD patients and its clinical and biochemical characteristics were analysed. Forty-eight patients with PD were evaluated into two groups, with and without RLS. Clinical characteristics assessed in both groups were age, gender, duration of PD, Hoehn and Yahr, and Schwab and England scales. Laboratory variables such as hemoglobin, s-iron, s-ferritin and creatinine were obtained. The prevalence of RLS was 18.75%. No significant differences regarding clinical variables and biochemical parameters were observed. The high prevalence of RLS found in PD patients suggests the concept of a common etiological link and it seems that secondary causes did not play a central role in the pathophysiology of RLS in this group of parkinsonian patients.

Imaging evaluation of non-alcoholic fatty liver disease: focused on quantification

Science.gov (United States)

2017-01-01

Non-alcoholic fatty liver disease (NAFLD) has been an emerging major health problem, and the most common cause of chronic liver disease in Western countries. Traditionally, liver biopsy has been gold standard method for quantification of hepatic steatosis. However, its invasive nature with potential complication as well as measurement variability are major problem. Thus, various imaging studies have been used for evaluation of hepatic steatosis. Ultrasonography provides fairly good accuracy to detect moderate-to-severe degree hepatic steatosis, but limited accuracy for mild steatosis. Operator-dependency and subjective/qualitative nature of examination are another major drawbacks of ultrasonography. Computed tomography can be considered as an unsuitable imaging modality for evaluation of NAFLD due to potential risk of radiation exposure and limited accuracy in detecting mild steatosis. Both magnetic resonance spectroscopy and magnetic resonance imaging using chemical shift technique provide highly accurate and reproducible diagnostic performance for evaluating NAFLD, and therefore, have been used in many clinical trials as a non-invasive reference of standard method. PMID:28994271

Symptomatic and asymptomatic apical periodontitis associated with red complex bacteria: clinical and microbiological evaluation.

Science.gov (United States)

Buonavoglia, Alessio; Latronico, Francesca; Pirani, Chiara; Greco, Maria Fiorella; Corrente, Marialaura; Prati, Carlo

2013-01-01

In this study, the association of red complex (RC) bacteria that include Treponema denticola, Tannerella forsythia and Porphyromonas gingivalis with acute, exacerbated or chronic apical periodontitis was evaluated. Seventy-one patients with periapical disease were evaluated by clinical examination and microbiological samples obtained from the root canals were analyzed by a polymerase chain reaction assay. Twenty-one (29.6%) samples were positive for RC bacteria, with T. denticola, T. forsythia and P. gingivalis being detected in 14 (19.7%), 10 (14.1%) and 6 (8.5%) samples, respectively. RC bacteria were mainly associated with acute apical periodontitis (29.2%) and phoenix abscess (63.2%), while they were only sporadically detected (7.1%) in patients with chronic apical periodontitis. Generally, RC bacteria were associated with pain and a higher frequency of intracanalar/intrasulcular pus drainage. Involvement of RC bacteria in symptomatic periapical disease should be suspected in the presence of particularly severe clinical pain and pus drainage.

Mediastinal lymph node enlargement in patients with valvular heart disease: CT evaluation and clinical correlation

International Nuclear Information System (INIS)

Park, Hye Ju; Jung, Jung Im; Ahn, Myeong Im; Han, Dae Hee; Park, Seog Hee

2016-01-01

To evaluate the presence, size and location of enlarged mediastinal lymph nodes (LNs) in patients with valvular heart disease (VHD) using computed tomography scans in correlation with ejection fraction (EF). We retrospectively evaluated 30 patients with VHD, without pre-existing diseases that could cause lymphadenopathy (LAP). The presence, size, and location of LNs greater than 1 cm in short axis diameter were evaluated. The location of mediastinal LNs was recorded according to the International Association for the Study of Lung Cancer. Furthermore, we evaluated the presence of pulmonary edema, pleural effusion, and other thoracic abnormalities and evaluated EF of the heart on transthoracic echocardiography. Sixteen patients (53%) had at least 1 enlarged mediastinal LN. The most frequent locations were lower paratracheal (4R, n = 8/4L, n = 6), subcarinal (7, n = 5) and right upper paratracheal (2R, n = 4) regions. The frequency of mediastinal LAP was higher in patients with aortic regurgitation (2 of 2, 100%) followed by mitral regurgitation (8 of 11, 73%); it was also high in patients with pulmonary edema (80%), pleural effusion (81%), or both (77%), as compared to patients without pulmonary edema or pleural effusion (17%) (p = 0.001). Ten of 30 patients showed an abnormal EF of < 55%; among them, 8 had mediastinal LAP. However, the relationship between EF and LAP was not statistically significant (p = 0.058). Mediastinal LN enlargement is common in patients with VHD, especially in cases of pulmonary edema and pleural effusion. Enlarged mediastinal LNs were frequently observed with abnormal EF, however, the relationship between EF and mediastinal LAP was not statistically significant.

Mediastinal lymph node enlargement in patients with valvular heart disease: CT evaluation and clinical correlation

Energy Technology Data Exchange (ETDEWEB)

Park, Hye Ju; Jung, Jung Im; Ahn, Myeong Im; Han, Dae Hee; Park, Seog Hee [Dept. of Radiology, Seoul St. Mary' s Hospital, College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

2016-04-15

To evaluate the presence, size and location of enlarged mediastinal lymph nodes (LNs) in patients with valvular heart disease (VHD) using computed tomography scans in correlation with ejection fraction (EF). We retrospectively evaluated 30 patients with VHD, without pre-existing diseases that could cause lymphadenopathy (LAP). The presence, size, and location of LNs greater than 1 cm in short axis diameter were evaluated. The location of mediastinal LNs was recorded according to the International Association for the Study of Lung Cancer. Furthermore, we evaluated the presence of pulmonary edema, pleural effusion, and other thoracic abnormalities and evaluated EF of the heart on transthoracic echocardiography. Sixteen patients (53%) had at least 1 enlarged mediastinal LN. The most frequent locations were lower paratracheal (4R, n = 8/4L, n = 6), subcarinal (7, n = 5) and right upper paratracheal (2R, n = 4) regions. The frequency of mediastinal LAP was higher in patients with aortic regurgitation (2 of 2, 100%) followed by mitral regurgitation (8 of 11, 73%); it was also high in patients with pulmonary edema (80%), pleural effusion (81%), or both (77%), as compared to patients without pulmonary edema or pleural effusion (17%) (p = 0.001). Ten of 30 patients showed an abnormal EF of < 55%; among them, 8 had mediastinal LAP. However, the relationship between EF and LAP was not statistically significant (p = 0.058). Mediastinal LN enlargement is common in patients with VHD, especially in cases of pulmonary edema and pleural effusion. Enlarged mediastinal LNs were frequently observed with abnormal EF, however, the relationship between EF and mediastinal LAP was not statistically significant.

Cost-Effectiveness of Clinical Pharmacy Education on Infection Management Among Patients with Chronic Kidney Disease in an Indonesian Hospital

OpenAIRE

Nasution, Azizah; Sulaiman, SA. Syed; Shafie, A.A

2016-01-01

Objectives This study evaluated the clinical and economic impacts of clinical pharmacy education (CPE) on infection management among patients with chronic kidney disease (CKD) stages 4 and 5 in Haji Adam Malik Hospital, Indonesia. Methods A quasi-experimental economic evaluation comparing CPE impact on 6-month CKD mortality was conducted on the basis of payer perspective. The experimental group (n = 63) received care by health care providers who were given CPE on drug-related probl...

Huntington's disease: a clinical review

Directory of Open Access Journals (Sweden)

Roos Raymund AC

2010-12-01

Full Text Available Abstract Huntington disease (HD is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school (Juvenile Huntington's disease; JHD. The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded. Patients experience psychiatric symptoms and cognitive decline. HD is an autosomal dominant inherited disease caused by an elongated CAG repeat (36 repeats or more on the short arm of chromosome 4p16.3 in the Huntingtine gene. The longer the CAG repeat, the earlier the onset of disease. In cases of JHD the repeat often exceeds 55. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD, and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Differential diagnoses include other causes of chorea including general internal disorders or iatrogenic disorders. Phenocopies (clinically diagnosed cases of HD without the genetic mutation are observed. Prenatal diagnosis is possible by chorionic villus sampling or amniocentesis. Preimplantation diagnosis with in vitro fertilization is offered in several countries. There is no cure. Management should be multidisciplinary and is based on treating symptoms with a view to improving quality of life. Chorea is treated with dopamine receptor blocking or depleting agents. Medication and non-medical care for depression and aggressive behavior may be required. The progression of the disease leads to a complete dependency in daily life, which

Podometry: a critical evaluation of its use in Hansen's disease

Directory of Open Access Journals (Sweden)

Júlia Maria D'Andréa Greve

Full Text Available The aim of this paper is to evaluate the effectiveness of podometry to measure the pressure developed over the feet and the value of these findings to help in the prevention of the plantar neuropathic ulcer in patients bearing Hansen's disease. We evaluated 13 patients with impaired plantar pain and touch sensations and 17 normal patients. All the patients were submitted to static evaluation using the podometer. The system employed was the "Midcapteur" commercial podometer composed of a platform for acquisition of analogic data capable of registering the segmental pressures applied to the feet. These data are read by a 386 IBM/PC compatible computer that registers the graphic patterns obtained from the pressures developed and also calculates the modes of pressure distribution in the four quadrants of the foot. These data obtained by means of static podometry were compared to the clinical evaluation of pain and touch sensation of the feet in hansenian patients. The results demonstrate that podometery is an efficient method for evaluating the pressure in impaired feet in Hansen's disease and is a progression of neuropathic ulcer; it is sensitive in the identification of the spots of increased pressure in anesthetic and anomalous areas, asymmetries and in correlating the presence of ulcers with increased pressure.

Infertility etiologies are genetically and clinically linked with other diseases in single meta-diseases.

Science.gov (United States)

Tarín, Juan J; García-Pérez, Miguel A; Hamatani, Toshio; Cano, Antonio

2015-04-15

The present review aims to ascertain whether different infertility etiologies share particular genes and/or molecular pathways with other pathologies and are associated with distinct and particular risks of later-life morbidity and mortality. In order to reach this aim, we use two different sources of information: (1) a public web server named DiseaseConnect ( http://disease-connect.org ) focused on the analysis of common genes and molecular mechanisms shared by diseases by integrating comprehensive omics and literature data; and (2) a literature search directed to find clinical comorbid relationships of infertility etiologies with only those diseases appearing after infertility is manifested. This literature search is performed because DiseaseConnect web server does not discriminate between pathologies emerging before, concomitantly or after infertility is manifested. Data show that different infertility etiologies not only share particular genes and/or molecular pathways with other pathologies but they have distinct clinical relationships with other diseases appearing after infertility is manifested. In particular, (1) testicular and high-grade prostate cancer in male infertility; (2) non-fatal stroke and endometrial cancer, and likely non-fatal coronary heart disease and ovarian cancer in polycystic ovary syndrome; (3) osteoporosis, psychosexual dysfunction, mood disorders and dementia in premature ovarian failure; (4) breast and ovarian cancer in carriers of BRCA1/2 mutations in diminished ovarian reserve; (5) clear cell and endometrioid histologic subtypes of invasive ovarian cancer, and likely low-grade serous invasive ovarian cancer, melanoma and non-Hodgkin lymphoma in endometriosis; and (6) endometrial and ovarian cancer in idiopathic infertility. The present data endorse the principle that the occurrence of a disease (in our case infertility) is non-random in the population and suggest that different infertility etiologies are genetically and clinically

A systematic literature review of evidence-based clinical practice for rare diseases

DEFF Research Database (Denmark)

Rath, Ana; Salamon, Valérie; Peixoto, Sandra

2017-01-01

diseases comprise the difficulty to recruit participants because of rarity, scattering of patients, limited knowledge on natural history of diseases, difficulties to achieve accurate diagnosis and identify patients in health information systems, and difficulties choosing clinically relevant outcomes....... CONCLUSIONS: Evidence-based clinical practice for rare diseases should start by collecting clinical data in databases and registries; defining measurable patient-centred outcomes; and selecting appropriate study designs adapted to small study populations. Rare diseases constitute one of the most paradigmatic...

Morgellons disease: Analysis of a population with clinically confirmed microscopic subcutaneous fibers of unknown etiology.

Science.gov (United States)

Savely, Virginia R; Stricker, Raphael B

2010-05-13

Morgellons disease is a controversial illness in which patients complain of stinging, burning, and biting sensations under the skin. Unusual subcutaneous fibers are the unique objective finding. The etiology of Morgellons disease is unknown, and diagnostic criteria have yet to be established. Our goal was to identify prevalent symptoms in patients with clinically confirmed subcutaneous fibers in order to develop a case definition for Morgellons disease. Patients with subcutaneous fibers observed on physical examination (designated as the fiber group) were evaluated using a data extraction tool that measured clinical and demographic characteristics. The prevalence of symptoms common to the fiber group was then compared with the prevalence of these symptoms in patients with Lyme disease and no complaints of skin fibers. The fiber group consisted of 122 patients. Significant findings in this group were an association with tick-borne diseases and hypothyroidism, high numbers from two states (Texas and California), high prevalence in middle-aged Caucasian women, and an increased prevalence of smoking and substance abuse. Although depression was noted in 29% of the fiber patients, pre-existing delusional disease was not reported. After adjusting for nonspecific symptoms, the most common symptoms reported in the fiber group were: crawling sensations under the skin; spontaneously appearing, slow-healing lesions; hyperpigmented scars when lesions heal; intense pruritus; seed-like objects, black specks, or "fuzz balls" in lesions or on intact skin; fine, thread-like fibers of varying colors in lesions and intact skin; lesions containing thick, tough, translucent fibers that are highly resistant to extraction; and a sensation of something trying to penetrate the skin from the inside out. This study of the largest clinical cohort reported to date provides the basis for an accurate and clinically useful case definition for Morgellons disease.

[Lyme disease--clinical manifestations and treatment].

Science.gov (United States)

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.

Assessing the activity of perianal Crohn's disease: comparison of clinical indices and computer-assisted anal ultrasound.

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Losco, Alessandra; Viganò, Chiara; Conte, Dario; Cesana, Bruno Mario; Basilisco, Guido

2009-05-01

Assessing perianal disease activity is important for the treatment and prognosis of Crohn's disease (CD) patients, but the diagnostic accuracy of the activity indices has not yet been established. The aim of this study was to determine the accuracy and agreement of the Fistula Drainage Assessment (FDA), Perianal Disease Activity Index (PDAI), and computer-assisted anal ultrasound imaging (AUS). Sixty-two consecutive patients with CD and perianal fistulae underwent clinical, FDA, PDAI, and AUS evaluation. Perianal disease was considered active in the presence of visible fistula drainage and/or signs of local inflammation (induration and pain at digital compression) upon clinical examination. The AUS images were analyzed by calculating the mean gray-scale tone of the lesion. The PDAI and gray-scale tone values discriminating active and inactive perianal disease were defined using receiver operating characteristics statistics. Perianal disease was active in 46 patients. The accuracy of the FDA was 87% (confidence interval [CI]: 76%-94%). A PDAI of >4 and a mean gray-scale tone value of 117 maximized sensitivity and specificity; their diagnostic accuracy was, respectively, 87% (CI: 76%-94%) and 81% (CI: 69%-90%). The agreement of the 3 evaluations was fair to moderate. The addition of AUS to the PDAI or FDA increased their diagnostic accuracy to respectively 95% and 98%. The diagnostic accuracy of the FDA, PDAI, and computer-assisted AUS imaging was good in assessing perianal disease activity in patients with CD. The agreement between the techniques was fair to moderate. Overall accuracy can be increased by combining the FDA or PDAI with AUS.

Skin denervation and its clinical significance in late-stage chronic kidney disease.

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Chao, Chi-Chao; Wu, Vin-Cent; Tan, Chun-Hsiang; Wang, Yi-Mei; Tseng, Ming-Tsung; Wu, Pei-Chen; Lin, Yea-Huey; Lin, Whei-Min; Wu, Kwan-Dun; Hsieh, Sung-Tsang

2011-02-01

To investigate the skin innervation and its clinical significance in late-stage chronic kidney disease (CKD). Case series. National Taiwan University Hospital, Taipei, Taiwan. Forty consecutive nondiabetic patients with late-stage CKD (14 female and 26 male; mean [SD] age, 60.7 [12.3] years), including 2 cases with stage 3 CKD, 6 with stage 4 CKD, and 32 with stage 5 CKD, ie, end-stage kidney disease. Clinical evaluation of neurological deficits, nerve conduction study, autonomic function tests, and a 3-mm-diameter skin biopsy specimen taken from the distal leg. Quantitation of epidermal innervation, parameters of nerve conduction study, R-R interval variability, and sympathetic skin response. Clinically, 21 patients (52.5%) were symptomatic with paresthesia over the limbs or autonomic symptoms. The intraepidermal nerve fiber (IENF) density was markedly reduced in patients with CKD compared with age- and sex-matched controls (mean [SD], 2.8 [2.0] vs 8.6 [2.8] fibers/mm; P Skin denervation was observed in 27 patients (67.5%). Fifteen patients (37.5%) had abnormalities on nerve conduction studies, and 29 patients (72.5%) had abnormal results on autonomic function tests. By analysis with multiple regression models, the IENF density was negatively correlated with the duration of renal disease (P = .02). Additionally, the R-R interval variability at rest was linearly correlated with the IENF density (P = .02) and the absence of sympathetic skin responses at the soles was associated with reduced IENF density (P = .03). Small-fiber sensory and autonomic neuropathies constitute the major form of neuropathy in late-stage CKD. Furthermore, skin denervation was associated with the duration of renal disease.

Clinical investigation in Wilson's disease

International Nuclear Information System (INIS)

Mizutani, Naoki; Maehara, Mitsuo; Negoro, Tamiko; Watanabe, Kazuyoshi

1983-01-01

Wilson's disease of cerebral type with a chief complaint of tremor occurred in 3 brothers and sisters of a certain family line. Treatment with D-penicillamine produced remarkable clinical improvement as shown by the disappearance of tremor and Kayser-Fleischer ring. Cranial CT scans made before the treatment revealed abnormal findings such as low density areas in the (bilateral) thalamus and the lenticular nucleus, atrophy of the cerebral cortex, and enlargement of the ventricles. After the treatment, cranial CT revealed that the low density areas in the thalamus and the lenticular nucleus disappeared corresponding to the clinical improvement. However, the atrophy of the cerebral cortex and the enlargement of the ventricles were not ameliorated. (Ueda, J.)

Evaluation of thyroid eye disease: quality-of-life questionnaire (TED-QOL) in Korean patients.

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Son, Byeong Jae; Lee, Sang Yeul; Yoon, Jin Sook

2014-04-01

To assess impaired quality of life (QOL) of Korean patients with thyroid eye disease (TED) using the TED-QOL questionnaire, to evaluate the adaptability of the questionnaire, and to assess the correlation between TED-QOL and scales of disease severity. Prospective, cross-sectional study. Total of 90 consecutive adult patients with TED and Graves' disease were included in this study. TED-QOL was translated into Korean and administered to the patients. The results were compared with clinical severity scores (clinical activity score, VISA (vision loss (optic neuropathy); inflammation; strabismus/motility; appearance/exposure) classification, modified NOSPECS (no signs or symptoms; only signs; soft tissue; proptosis; extraocular muscle; cornea; sight loss) score, Gorman diplopia scale, and European Group of Graves' Orbitopathy Classification). Clinical scores indicating inflammation and strabismus in patients with TED were positively correlated with overall and visual function-related QOL (Spearman coefficient 0.21-0.38, p < 0.05). Clinical scores associated with appearance were positively correlated with appearance-related QOL (Spearman coefficient 0.26-0.27, p < 0.05). In multivariate analysis, age, soft-tissue inflammation, motility disorder of modified NOSPECS, and motility disorder of VISA classification had positive correlation with overall and function-related QOL. Sex, soft-tissue inflammation, proptosis of modified NOSPECS, and appearance of VISA classification had correlation with appearance-related QOL. In addition, validity of TED-QOL was proved sufficient based on the outcomes of patient interviews and correlation between the subscales of TED-QOL. TED-QOL showed significant correlations with various objective clinical parameters of TED. TED-QOL was a simple and useful tool for rapid evaluation of QOL in daily outpatient clinics, which could be readily translated into different languages to be widely applicable to various populations. Copyright © 2014

Pharmacogenetics in Neurodegenerative Diseases: Implications for Clinical Trials.

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Tortelli, Rosanna; Seripa, Davide; Panza, Francesco; Solfrizzi, Vincenzo; Logroscino, Giancarlo

2016-01-01

Pharmacogenetics has become extremely important over the last 20 years for identifying individuals more likely to be responsive to pharmacological interventions. The role of genetic background as a predictor of drug response is a young and mostly unexplored field in neurodegenerative diseases. Mendelian mutations in neurodegenerative diseases have been used as models for early diagnosis and intervention. On the other hand, genetic polymorphisms or risk factors for late-onset Alzheimer's disease (AD) or other neurodegenerative diseases, probably influencing drug response, are hardly taken into account in randomized clinical trial (RCT) design. The same is true for genetic variants in cytochrome P450 (CYP), the principal enzymes influencing drug metabolism. A better characterization of individual genetic background may optimize clinical trial design and personal drug response. This chapter describes the state of the art about the impact of genetic factors in RCTs on neurodegenerative disease, with AD, frontotemporal dementia, Parkinson's disease, amyotrophic lateral sclerosis, and Huntington's disease as examples. Furthermore, a brief description of the genetic bases of drug response focusing on neurodegenerative diseases will be conducted. The role of pharmacogenetics in RCTs for neurodegenerative diseases is still a young, unexplored, and promising field. Genetic tools allow increased sophistication in patient profiling and treatment optimization. Pharmaceutical companies are aware of the value of collecting genetic data during their RCTs. Pharmacogenetic research is bidirectional with RCTs: efficacy data are correlated with genetic polymorphisms, which in turn define subjects for treatment stratification. © 2016 S. Karger AG, Basel.

The impact of Medicaid-linked reimbursements on revenues of public sexually transmitted disease clinics.

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Downey, Lois; Lafferty, William E; Krekeler, Barbara

2002-02-01

Public sexually transmitted disease (STD) clinics faced with decreased tax revenue and increased costs must evaluate alternative revenue sources. To report one public STD clinic's Medicaid-linked revenue and discuss the association between system characteristics and reimbursement potential. This was a cross-sectional study of 4208 patients visiting the clinic for new problems during a 6-month period. Of 458 Medicaid-enrolled patients, only 55% acknowledged enrollment at the time of visit. The clinic captured revenue for many of the remaining 45% through a centralized public health information/billing system, which submitted retroactive STD clinic claims when patients self-reported Medicaid enrollment at later visits to other public health clinics. These belated self-reports also contributed to Medicaid administrative-match reimbursements. An estimated $100,000 (31% of the clinic's direct reimbursements for service) would have been lost in 2000, had detection of Medicaid enrollment been based exclusively on patients' self-reports at STD clinic visits.

Morgellons disease: Analysis of a population with clinically confirmed microscopic subcutaneous fibers of unknown etiology

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Virginia R Savely

2010-05-01

Full Text Available Virginia R Savely1, Raphael B Stricker21TBD Medical Associates, San Francisco, CA, USA; 2International Lyme and Associated Diseases Society, Bethesda, MD, USABackground: Morgellons disease is a controversial illness in which patients complain of stinging, burning, and biting sensations under the skin. Unusual subcutaneous fibers are the unique objective finding. The etiology of Morgellons disease is unknown, and diagnostic criteria have yet to be established. Our goal was to identify prevalent symptoms in patients with clinically confirmed subcutaneous fibers in order to develop a case definition for Morgellons disease.Methods: Patients with subcutaneous fibers observed on physical examination (designated as the fiber group were evaluated using a data extraction tool that measured clinical and demographic characteristics. The prevalence of symptoms common to the fiber group was then compared with the prevalence of these symptoms in patients with Lyme disease and no complaints of skin fibers.Results: The fiber group consisted of 122 patients. Significant findings in this group were an association with tick-borne diseases and hypothyroidism, high numbers from two states (Texas and California, high prevalence in middle-aged Caucasian women, and an increased prevalence of smoking and substance abuse. Although depression was noted in 29% of the fiber patients, pre-existing delusional disease was not reported. After adjusting for nonspecific symptoms, the most common symptoms reported in the fiber group were: crawling sensations under the skin; spontaneously appearing, slow-healing lesions; hyperpigmented scars when lesions heal; intense pruritus; seed-like objects, black specks, or “fuzz balls” in lesions or on intact skin; fine, thread-like fibers of varying colors in lesions and intact skin; lesions containing thick, tough, translucent fibers that are highly resistant to extraction; and a sensation of something trying to penetrate the skin from

The hidden Niemann-Pick type C patient: clinical niches for a rare inherited metabolic disease.

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Hendriksz, Christian J; Anheim, Mathieu; Bauer, Peter; Bonnot, Olivier; Chakrapani, Anupam; Corvol, Jean-Christophe; de Koning, Tom J; Degtyareva, Anna; Dionisi-Vici, Carlo; Doss, Sarah; Duning, Thomas; Giunti, Paola; Iodice, Rosa; Johnston, Tracy; Kelly, Dierdre; Klünemann, Hans-Hermann; Lorenzl, Stefan; Padovani, Alessandro; Pocovi, Miguel; Synofzik, Matthis; Terblanche, Alta; Then Bergh, Florian; Topçu, Meral; Tranchant, Christine; Walterfang, Mark; Velten, Christian; Kolb, Stefan A

2017-05-01

Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to under-recognition or misdiagnosis across a wide range of medical fields. New screening and diagnostic methods provide an opportunity to improve detection of unrecognized cases in clinical sub-populations associated with a higher risk of NP-C. Patients in these at-risk groups ("clinical niches") have symptoms that are potentially related to NP-C, but go unrecognized due to other, more prevalent clinical features, and lack of awareness regarding underlying metabolic causes. Twelve potential clinical niches identified by clinical experts were evaluated based on a comprehensive, non-systematic review of literature published to date. Relevant publications were identified by targeted literature searches of EMBASE and PubMed using key search terms specific to each niche. Articles published in English or other European languages up to 2016 were included. Several niches were found to be relevant based on available data: movement disorders (early-onset ataxia and dystonia), organic psychosis, early-onset cholestasis/(hepato)splenomegaly, cases with relevant antenatal findings or fetal abnormalities, and patients affected by family history, consanguinity, and endogamy. Potentially relevant niches requiring further supportive data included: early-onset cognitive decline, frontotemporal dementia, parkinsonism, and chronic inflammatory CNS disease. There was relatively weak evidence to suggest amyotrophic lateral sclerosis or progressive supranuclear gaze palsy as potential niches. Several clinical niches have been identified that harbor patients at increased risk of NP-C.

Infections Disease in the Dental Clinical Nigerian Dentists ...

African Journals Online (AJOL)

The dental clinic is a potential source of transmission of infectious disease due to the invasive nature of many dental procedures. Infectious disease therefore constitutes serious occupational hazards to all oral health care workers. This descriptive study aims to assess the knowledge of dentists on common transmissible ...

Resistive index for kidney evaluation in normal and diseased cats.

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Tipisca, Vlad; Murino, Carla; Cortese, Laura; Mennonna, Giuseppina; Auletta, Luigi; Vulpe, Vasile; Meomartino, Leonardo

2016-06-01

The objectives were to determine the resistive index (RI) in normal cats and in cats with various renal diseases, and to evaluate the effect of age on RI. The subjects were cats that had ultrasonography (US) of the urinary tract and RI measurement at our centre between January 2003 and April 2014. Based on clinical evaluation, biochemical and haematological tests, urinalysis and US, the cats were classified as healthy or diseased. RI measurements were made from the interlobar or arcuate arteries. Data were analysed for differences between the right and the left kidney, the two sexes, different age groups in healthy cats, and between healthy and diseased cats. A total of 116 cats (68 males, 48 females) were included: 24 healthy and 92 diseased. In the healthy cats, RI (mean ± SD) differed significantly (P = 0.02) between the right kidney (0.54 ± 0.07) and the left kidney (0.59 ± 0.08). For the left kidney, RI was significantly higher in cats with chronic kidney disease (0.73 ± 0.12) and acute kidney injury (0.72 ± 0.08) (P = 0.0008). For the right kidney, RI was significantly higher in cats with chronic kidney disease (0.72 ± 0.11), acute kidney injury (0.74 ± 0.08), polycystic kidney disease (0.77 ± 0.11) and renal tumour (0.74 ± 0.001) (P cats, useful in the differential diagnosis of diffuse renal diseases. While it does not change with the age of the cat, ultrasonographers should be aware that RI may differ between the two kidneys. © ISFM and AAFP 2015.

Evaluation of Clinical and Pathological Response after Two Cycles ...

African Journals Online (AJOL)

Results: The clinical response rate was 83%; 11 patients (11.2%) had a complete clinical remission (cCR); 71 had a partial remission (72.4%); 13 had stable disease (13.3%), and 3 had progressive disease (3.1%). Seven patients had complete pathological response. Conclusion: Neoadjuvant chemotherapy can achieve a ...

Clinical neurorestorative progress in Parkinson's disease

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Chen L

2015-06-01

Full Text Available Lin Chen,1,2 Hongyun Huang,3–5 Wei-Ming Duan,6 Gengsheng Mao3 1Department of Neurosurgery, Yuquan Hospital, Tsinghua University, 2Department of Neurosurgery, Medical Center, Tsinghua University, 3Department of Neurosurgery, General Hospital of Chinese People's Armed Police Forces, 4Center of Cell Research, Beijing Rehabilitation Hospital of Capital Medical University, 5Beijing Hongtianji Neuroscience Academy, 6Department of Anatomy, Capital Medical University, Beijing, People's Republic of China Abstract: Parkinson’s disease (PD is one of the common neurodegenerative diseases. Besides the symptomatic therapies, the increasing numbers of neurorestorative therapies have shown the potential therapeutic value of reversing the neurodegenerative process and improving the patient's quality of life. Currrently available novel clinical neurorestorative strategies include pharmacological managements (glial cell-line derived neurotrophic factor, selegiline, recombinant human erythropoietin, neuromodulation intervention (deep brain stimulation, repetitive transcranial magnetic stimulation, transcranial direct current stimulation, tissue and cell transplantation (fetal ventral mesencephalic tissue, sympathetic neurons, carotid body cells, bone marrow stromal cells, retinal pigment epithelium cells, gene therapy, and neurorehabilitative therapy. Herein, we briefly review the progress in this field and describe the neurorestorative mechanisms of the above-mentioned therapies for PD. Keywords: Parkinson’s disease, clinical study, neurorestorative treatment, cell transplantation, neuromodulation

Clinical, genetic, and neuroimaging features of Early Onset Alzheimer Disease: the challenges of diagnosis and treatment.

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Alberici, Antonella; Benussi, Alberto; Premi, Enrico; Borroni, Barbara; Padovani, Alessandro

2014-01-01

Early Onset Alzheimer Disease (EOAD) is a rare condition, frequently associated with genetic causes. The dissemination of genetic testing along with biomarker determinations have prompted a wider recognition of EOAD in experienced clinical settings. However, despite the great efforts in establishing the contribution of causative genes to EOAD, atypical disease presentation and clinical features still makes its diagnosis and treatment a challenge for the clinicians. This review aims to provide an extensive evaluation of literature data on EOAD, in order to improve understanding and knowledge of EOAD, underscore its significant impact on patients and their caregivers and influence public policies. This would be crucial to define the urgency of evidence-based treatment approaches.

The clinical significance of brain microbleeds in patients with Alzheimer′s disease: Preliminary study

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Jae-Hyeok Heo

2016-01-01

Full Text Available Background: Microbleeds (MBs are observed frequently in Alzheimer′s disease (AD and suggested to play a crucial role in the pathophysiology, but their clinical significance remains unclear. Materials and Methods: The study recruited 100 patients with AD who were diagnosed at the memory clinic in Seoul Medical Center in 2014. For each patient, baseline characteristics, neuropsychological tests, cerebrovascular risk factors, medial temporal lobe atrophy (MTLA, and severity of small vessel disease (SVD according to the existence of MBs were evaluated. Results: The prevalence of MBs in patients with AD was 33%. The percentage of male gender, the severity of SVD and MTLA were significantly increased in MB(+ group. The MB(+ group showed more severe MTLA and SVD than MB(− group. Conclusions: These results suggested that MBs might reflect the burden of amyloid and ischemic vascular pathology.

NON-COMMUNICABLE DISEASE PROGRAM IN AMPANGAN HEALTH CLINIC

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MASTURA I

2010-01-01

Full Text Available Non-communicable diseases (NCDs represent among the most common and debilitating conditions seen in primary care. Patients’ care will often involves multiple providers and follow-up requires persistence by patients and clinicians alike, therefore ideal outcomes are often difficult to achieve. The need for better disease management policies and practice is growing. This is due to the changing demographic profile of the population, the increasing cost of managing people in acute care hospitals and the availability of new technologies and services. All these changes enable a different care paradigm which is more cost effective and provides people with chronic conditions an improved quality of life. Management of the NCDs therefore offers an excellent opportunity to practice chronic disease management - a systems approach designed to ensure excellent care. The NCD team has developed a comprehensive approach to chronic disease care. We would like to describe the NCD Program in Ampangan Health Clinic which represents many typical government health clinics in Malaysia and the processes by which it was developed. Included are specific examples of the tools and how they can be used by individual clinicians incaring for patients. The integration of Chronic Disease Management Services into health care systems is the direction being undertaken to tackle the burden of chronic disease. Disease management supports the shift in healthcare from an emphasis on managing the acute episode to managing the entire disease course, highlighting both prevention and maintenance of wellbeing for patients with chronic diseases. Disease management promotes better integration and coordination of care across all aspects of the health sector.

Building clinical networks: a developmental evaluation framework.

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Carswell, Peter; Manning, Benjamin; Long, Janet; Braithwaite, Jeffrey

2014-05-01

Clinical networks have been designed as a cross-organisational mechanism to plan and deliver health services. With recent concerns about the effectiveness of these structures, it is timely to consider an evidence-informed approach for how they can be developed and evaluated. To document an evaluation framework for clinical networks by drawing on the network evaluation literature and a 5-year study of clinical networks. We searched literature in three domains: network evaluation, factors that aid or inhibit network development, and on robust methods to measure network characteristics. This material was used to build a framework required for effective developmental evaluation. The framework's architecture identifies three stages of clinical network development; partner selection, network design and network management. Within each stage is evidence about factors that act as facilitators and barriers to network growth. These factors can be used to measure progress via appropriate methods and tools. The framework can provide for network growth and support informed decisions about progress. For the first time in one place a framework incorporating rigorous methods and tools can identify factors known to affect the development of clinical networks. The target user group is internal stakeholders who need to conduct developmental evaluation to inform key decisions along their network's developmental pathway.

The use of intravenous digital subtraction angiography in evaluating patients with complex congenital heart disease

International Nuclear Information System (INIS)

Moodie, D.S.

1986-01-01

The author previously described his experience in 450 patients with congenital heart disease using intravenous digital subtraction angiography (DSA) to define cardiac anatomy. He has been impressed by the utility of DSA in the evaluation of patients with congenital heart disease. It is now an integral part of his clinical practice to perform intravenous DSA studies both pre- and postoperatively on an inpatient as well as outpatient basis. This chapter details his DSA experience with complex forms of congenital heart disease

MRI evaluation of soft tissue hydatid disease

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Garcia-Diez, A.I.; Ros Mendoza, L.H.; Villacampa, V.M.; Cozar, M.; Fuertes, M.I. [Dept. of Radiology, Hospital Miguel Servet, Zaragoza (Spain)

2000-03-01

Infestation in soft tissue by Echinococcus granulosus is not a common disease, and its diagnosis is based on clinical, laboratory data and radiological findings. The aim of our retrospective study is to give an overview of the different signs and patterns shown by MRI that can be useful in characterizing soft tissue hydatid disease. The MRI images obtained in seven patients with soft tissue and subcutaneous hydatidosis were reviewed. Typical signs of hydatidosis were multivesicular lesions with or without hypointense peripheral ring (''rim sign''). Related to the presence and absence, respectively, of viable scolices in the microscopic exam, daughter cysts were presented either as high signal intensity or low signal intensity on T2-weighted images. Low-intensity detached layers within the cyst and peripheral enhancement with gadolinium-DTPA were also presented. Atypical signs were presented in an infected muscular cyst, a subcutaneous unilocular cyst and several unilocular cysts. Knowledge of the different patterns in MRI of soft tissue hydatid disease can be useful in diagnosing this entity. We observed that the ''rim sign'' is not as common as in other locations, and in addition, MRI seems to be of assistance when evaluating the vitality of the cysts. (orig.)

Transition clinic attendance is associated with improved beliefs and attitudes toward medicine in patients with inflammatory bowel disease

OpenAIRE

Fu, Nancy; Jacobson, Kevan; Round, Andrew; Evans, Kathi; Qian, Hong; Bressler, Brian

2017-01-01

AIM To evaluated the differences in knowledge, adherence, attitudes, and beliefs about medicine in adolescents with inflammatory bowel disease (IBD) attending transition clinics. METHODS We prospectively enrolled patients from July 2012 to June 2013. All adolescents who attended a tertiary-centre-based dedicated IBD transition clinic were invited to participate. Adolescent controls were recruited from university-affiliated gastroenterology offices. Participants completed questionnaires about ...

Study on clinical symptoms in canine cardiac diseases

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F. Karlette Anne

Full Text Available Cardiac diseases in canines are an extensively studied phenomenon all over the world but meagre information has been reported in India. Certain problems, including historical, physical, and laboratory abnormalities, are associated with cardiovascular or pulmonary disease. In India however, the recognition of canine cardiac diseases has been delayed, and ignored on account of lack of awareness and knowledge by the owner and inadequate diagnostic facility to a field veterinarian. Considering the above facts, the present study was undertaken in Gujarat to survey the prevalence of common cardiac diseases in hospital population of dogs along with the clinical symptoms which often goes undetected due to lack of proper diagnostic techniques to be implied and the most forms of heart disease may be present for many years before any evidence of failure develops. In the present study most of the clinical cases of cardiac diseases were presented with a history of nocturnal coughing (seven cases; 2.55%, exercise intolerance (five cases; 1.82%, partial or complete anorexia (five cases; 1.82%, swelling in abdominal area (four cases; 1.45%, dullness and depression (two cases; 0.72%, cachexia and hepatojugular pulsation (one case each; 0.36% each at times. [Vet World 2009; 2(8.000: 307-309

Alzheimer biomarkers and clinical Alzheimer disease were not associated with increased cerebrovascular disease in a memory clinic population.

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Spies, Petra E; Verbeek, Marcel M; Sjogren, Magnus J C; de Leeuw, Frank-Erik; Claassen, Jurgen A H R

2014-01-01

Preclinical and post-mortem studies suggest that Alzheimer disease (AD) causes cerebrovascular dysfunction, and therefore may enhance susceptibility to cerebrovascular disease (CVD). The objective of this study was to investigate this association in a memory clinic population. The AD biomarkers CSF amyloid β42, amyloid β40 and APOE-ε4 status have all been linked to increased CVD risk in AD, and therefore the first aim of this study was to analyze the association between these biomarkers and CVD. In 92 memory clinic patients the cross-sectional association between AD biomarkersand the severity of CVD was investigated with linear regression analysis. Additionally, we studied whether AD biomarkers modified the relation between vascular risk factors and CVD. CVD was assessed on MRI through a visual rating scale.Analyses were adjusted for age. The second aim of this study was to investigate the association between clinical AD and CVD, where 'clinical AD' was defined as follows: impairment in episodic memory, hippocampal atrophy and an aberrant concentration of cerebrospinal fluid (CSF) biomarkers. 47 of the 92 patients had AD. No association between CSF amyloid β42, amyloid β40 or APOE-ε4 status and CVD severity was found, nor did these AD biomarkers modify the relation between vascular risk factors and CVD. Clinical AD was not associated with CVD severity (p=0.83). Patients with more vascular risk factors had more CVD, but this relationship was not convincingly modified by AD (p=0.06). In this memory clinic population, CVD in patients with AD was related to vascular risk factors and age, comparable to patients without AD. Therefore, in our study, the preclinical and post-mortem evidence that AD would predispose to CVD could not be translated clinically. Further work, including replication of this work in a different and larger sample, is warranted.

Cerebrovascular disease in newborn infants: report of three cases with clinical follow-up and brain SPECT imaging

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Moura-Ribeiro, Maria Valeriana L. de; Ciasca, Sylvia Maria; Vale-Cavalcanti, Mariza; Etchebehere, Elba C.S.C.; Camargo, Edwaldo E. [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas

1999-07-01

The clinical and neurological findings of three neonates with the diagnosis of cerebrovascular disease are reported. The neuropsychological evaluation disclosed impairment of fine motor function, coordination, language, perception and behavioral disturbances. Brain SPECT imaging revealed perfusional deficits in the three cases. (author)

Cerebrovascular disease in newborn infants: report of three cases with clinical follow-up and brain SPECT imaging

International Nuclear Information System (INIS)

Moura-Ribeiro, Maria Valeriana L. de; Ciasca, Sylvia Maria; Vale-Cavalcanti, Mariza; Etchebehere, Elba C.S.C.; Camargo, Edwaldo E.

1999-01-01

The clinical and neurological findings of three neonates with the diagnosis of cerebrovascular disease are reported. The neuropsychological evaluation disclosed impairment of fine motor function, coordination, language, perception and behavioral disturbances. Brain SPECT imaging revealed perfusional deficits in the three cases. (author)

Pre-Clinical Tests of an Integrated CMOS Biomolecular Sensor for Cardiac Diseases Diagnosis.

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Lee, Jen-Kuang; Wang, I-Shun; Huang, Chi-Hsien; Chen, Yih-Fan; Huang, Nien-Tsu; Lin, Chih-Ting

2017-11-26

Coronary artery disease and its related complications pose great threats to human health. In this work, we aim to clinically evaluate a CMOS field-effect biomolecular sensor for cardiac biomarkers, cardiac-specific troponin-I (cTnI), N -terminal prohormone brain natriuretic peptide (NT-proBNP), and interleukin-6 (IL-6). The CMOS biosensor is implemented via a standard commercialized 0.35 μm CMOS process. To validate the sensing characteristics, in buffer conditions, the developed CMOS biosensor has identified the detection limits of IL-6, cTnI, and NT-proBNP as being 45 pM, 32 pM, and 32 pM, respectively. In clinical serum conditions, furthermore, the developed CMOS biosensor performs a good correlation with an enzyme-linked immuno-sorbent assay (ELISA) obtained from a hospital central laboratory. Based on this work, the CMOS field-effect biosensor poses good potential for accomplishing the needs of a point-of-care testing (POCT) system for heart disease diagnosis.

The influence of smoking on clinical periodontal disease

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Ina Hendiani

2009-07-01

Full Text Available Periodontal disease has very complex and multi factor etiology. Plaque bacteria is the main cause of periodontal disease and another risk factor that also plays a role is a smoking habit. Cigarette product such as nicotine can influence the development of periodontal disease that can directly and systemically damage the function of PMN cell. The research was conducted by taking a clinical examination on the smoking influence that covers the number of cigarettes and the period of smoking, and kind of cigarette to the worse of periodontal disease, and by measuring the epithelial attachment loss and the bleeding index. The research was conducted to 152 male aged 20-45 years old, comprised 80 smokers and 72 nonsmokers at the Clinic of Faculty of Dentistry Universitas Indonesia, Jakarta. The result of the research showed that smoking gave influence on the worse of the periodontal disease. There was a profound relationship between the smoking period and the number of cigarettes consumed everyday indicated by the epithelial attachment loss. Smoking did not enhance gingival bleeding. The relationship between kinds of cigarette and the gingival bleeding score and the epithelial attachment loss did not show a significant bleeding.

Clinical uses of melatonin: evaluation of human trials.

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Sánchez-Barceló, E J; Mediavilla, M D; Tan, D X; Reiter, R J

2010-01-01

During the last 20 years, numerous clinical trials have examined the therapeutic usefulness of melatonin in different fields of medicine. The objective of this article is to review, in depth, the science regarding clinical trials performed to date. The efficacy of melatonin has been assessed as a treatment of ocular diseases, blood diseases, gastrointestinal tract diseases, cardiovascular diseases, diabetes, rheumatoid arthritis, fibromyalgia, chronic fatigue syndrome, infectious diseases, neurological diseases, sleep disturbances, aging and depression. Melatonin has been also used as a complementary treatment in anaesthesia, hemodialysis, in vitro fertilization and neonatal care. The conclusion of the current review is that the use of melatonin as an adjuvant therapy seems to be well funded for macular degeneration, glaucoma, protection of the gastric mucosa, irritable bowel syndrome, arterial hypertension, diabetes, side effects of chemotherapy and radiation in cancer patients or hemodialysis in patients with renal insufficiency and, especially, for sleep disorders of circadian etiology (jet lag, delayed sleep phase syndrome, sleep deterioration associated with aging, etc.) as well as in those related with neurological degenerative diseases (Alzheimer, etc.,) or Smith-Magenis syndrome. The utility of melatonin in anesthetic procedures has been also confirmed. More clinical studies are required to clarify whether, as the preliminary data suggest, melatonin is useful for treatment of fibromyalgia, chronic fatigue syndrome, infectious diseases, neoplasias or neonatal care. Preliminary data regarding the utility of melatonin in the treatment of ulcerative colitis, Crohn's disease, rheumatoid arthritis are either ambiguous or negative. Although in a few cases melatonin seems to aggravate some conditions, the vast majority of studies document the very low toxicity of melatonin over a wide range of doses.

Fluid biomarkers in clinical trials of Alzheimer’s disease therapeutics

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Aaron eRitter

2015-08-01

Full Text Available With the demographic shift of the global population towards longer life expectancy, the number of people living with Alzheimer’s disease (AD has rapidly expanded and is projected to triple by the year 2050. Current treatments provide symptomatic relief but do not affect the underlying pathology of the disease. Therapies that prevent or slow the progression of the disease are urgently needed to avoid this growing public health emergency. Insights gained from decades of research have begun to unlock the pathophysiology of this complex disease and have provided targets for disease modifying therapies. In the last decade, few therapeutic agents designed to modify the underlying disease process have progressed to clinical trials and none have been brought to market. With the focus on disease modification, biomarkers promise to play an increasingly important role in clinical trials. Six biomarkers have now been included in diagnostic criteria for AD and are regularly incorporated into clinical trials. Three biomarkers are neuroimaging measures—hippocampal atrophy measured by magnetic resonance imaging (MRI, amyloid uptake as measured by Pittsburg compound B positron emission tomography (PiB PET, and decreased fluorodeoxyglucose (18F uptake as measured by positron emission tomography (FDG PET—and three are sampled from fluid sources—cerebrospinal fluid (CSF levels

Colour Doppler evaluation of extracranial carotid artery in patients presenting with features of cerebrovascular disease: A clinical and radiological correlation

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Sanjeev Sehrawat

2012-01-01

Full Text Available Aim: To evaluate the morphological and hemodynamic changes that take place in carotid arteries by colour Doppler in patients presenting with features of stroke. Background and Objectives: Cerebrovascular accidents constitute a major cause of adult mortality. The principal indication for cerebrovascular Doppler examination is stroke prevention. Colour Doppler sonography is a sensitive method for detection of atherosclerotic plaque and provides considerable information about the extent and severity of plaque as well as the resulting diminution of arterial lumen. The main strengths of sonography of carotid arteries are patient comfort, lack of risk and accuracy in detecting carotid stenosis. Material and Methods: A prospective study of Colour Doppler in carotid arteries was carried out for 12 months from 1 st July 2009 to 1 st July 2010. The study was carried out on 40 individuals, suspected of cerebrovascular insufficiency and having one or the other risk factors for cerebrovascular disease. A detailed clinical history, CNS examination findings and evidence of hypertension, diabetes mellitus, hyperlipidemia and ischemic heart disease were noted. Carotid Doppler evaluation was done by using Siemens Antares Ultrasound system. The data gathered were grey scale and Doppler findings of common carotid artery, internal carotid artery and external carotid arteries. Doppler findings were correlated with clinical features and risk factors. Results: In our study of 40 patients, the commonest lesion found was the atherosclerotic plaque. Highest incidence of plaque was seen in males 41% in the age group of 60-70 years and in females 37% in age group of 70-80 years. Cigarette smoking was the most common risk factor (60% associated with stroke/ Transient Ischaemic Attacks (TIA. Hemiparesis was the most common presenting symptom (35% among the symptomatic cases. Atheromatous plaque was most commonly found in the right carotid system (60%. Most common site for

Clinical benefit of fixed-dose dual bronchodilation with glycopyrronium and indacaterol once daily in patients with chronic obstructive pulmonary disease

DEFF Research Database (Denmark)

Ulrik, Charlotte Suppli

2014-01-01

BACKGROUND AND AIM: Long-acting bronchodilators are the preferred option for maintenance therapy of patients with chronic obstructive pulmonary disease (COPD). The aim of this review is to provide an overview of the clinical studies evaluating the clinical efficacy of the once-daily fixed-dose du...... for chronic Obstructive Lung Disease [GOLD] spirometric criteria). Furthermore, a very recent study has shown that fixed-dose indacaterol/glycopyrronium improves exercise endurance time compared with placebo, although no significant difference was observed between fixed-dose indacaterol...

Recent clinical trials in valvular heart disease.

Science.gov (United States)

Kiss, Daniel; Anwaruddin, Saif

2017-07-01

With widespread adoption of transcatheter aortic valve replacement, there has been a change in the approach to management of valvular heart disease. New interest has taken hold in transcatheter therapies for valvular heart disease, as well as research into pathophysiology and progression of disease. Additionally, several key trials have further refined our understanding of surgical management of valvular heart disease. This review will elucidate recent clinical trial data leading to changes in practice. There have been several landmark trials expanding the indications for transcatheter aortic valve replacement. Additionally, although still early, trials are beginning to demonstrate the feasibility and safety of transcatheter mitral valves. Options for transcatheter management of right-sided valvular disease continue to evolve, and these are areas of active investigation. The emergence of novel therapies for valvular heart disease has expanded the management options available, allowing physicians to better individualize treatment of patients with valvular heart disease. This review will focus on the recent (within 2 years) trials in this field of interest.

Considerations on Intervention Goal and Efficacy Evaluation of Traditional Chinese Medicine in the Treatment of Neurological Diseases

Institute of Scientific and Technical Information of China (English)

XIE Ren-ming; DU Bao-xin; HUANG Yan; ZHOU Dao-you; WANG yong-yan; HUANG pei-xin

2007-01-01

In the last several years, traditional Chinese medicine (TCM) has made much progress in the treatment of neurological diseases. The living space of TCM in neurological diseases lies in refractory diseases, aging and chronic diseases caused by multiple factors as well as sub-health state and chronic fatigue state. The effect model of TCM mainly consists of whole effect, self-organization,self-stable model, holographic effect and butterfly effect. The effective point of TCM in neurological diseases lies mainly in end-points and health-related events. Moreover, TCM has advantages in the evaluation of symptoms, syndrome and quality of life (QOL). Some key indexes should be included when evaluating the efficacy of TCM in neurological diseases. Meanwhile, the advantages of TCM such as end-points, health-related events and QOL should be highlighted. Multi-subject researching methods could be adopted to make a comprehensive evaluation of subjective and objective indexes.The clinical evidence on the TCM efficacy evaluation may come from RCTs, and other types of designs can also be considered.

Clinical education and clinical evaluation of respiratory therapy students.

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Cullen, Deborah L

2005-09-01

Different blends of knowledge, decision making, problem solving,professional behaviors, values, and technical skills are necessary in the changing health care environments in which respiratory therapists practice. Frequently, novice students are expected to perform quickly and efficiently,and it may be forgotten that students are still learning and mastering the foundation pieces of practice. Clinical educators take on the responsibility of student development in addition to overseeing patient care. Normally,these volunteer instructors are role models for respiratory therapy students. The characteristic of initiative when demonstrated by a beginning student is attractive to the clinical instructor, promotes sharing of experiences, and may evolve into a mentor-protege relationship. Some clinical instructors may be underprepared to teach and are uncomfortable with student evaluation. Respiratory therapy facilities in conjunction with academic institutions may consider sponsoring ongoing programs for clinical teachers. Teaching and learning in the clinical environment is more than demonstration of skills and knowledge. Furthermore, it can be debated whether the memorization of facts or of the steps of a skill is more valuable than competency in problem solving, clinical reasoning, or information retrieval. New knowledge is built within a context and is further integrated when grounded by experience. Development of "prediction in practice" or the anticipation of the next necessary actions may be worth integrating into the instructional toolbox. Intuition has been defined as an "understanding without a rationale". This definition separates intuition from rational decision making and presents intuition as a type of innate ability. Reflection when guided by clinical instructors can help deepen critical thinking, as will Socratic questioning on a regular basis. Most clinical staff can agree on the performance of an incompetent student, but discrimination of the levels of

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

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

2017-02-01

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

Current clinical research of immunoglobulin G4-related orbital disease

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Yang Wang

2016-05-01

Full Text Available Immunoglobulin G4-related disease(IgG4-related diseasehas received lots of attention in medical community as a recently recognized fibro-inflammatory condition. It is characterized by infiltration of IgG4-immunopositive plasmacytes and concentration of elevated serum IgG4. IgG4-related disease shows organ enlargement or nodular/hyperplastic lesions in various organs including the pancreas, hepatobiliary tract and orbit, which is called IgG4-related orbital disease. The diagnostic criteria for IgG4-related disease and IgG4-related orbital disease has recently been established, which is based on clinical, imaging and histopathologic features of the orbital lesions. Besides, attention should be drawn to the differentiation from other diseases. The treatment is empirical including corticosteroids, immunosuppressive drugs, radiotherapy, and rituximab. This article reviews clinical progression of IgG4-related orbital disease.

Norrie disease: extraocular clinical manifestations in 56 patients.

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

2012-08-01

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

Clinical status, quality of life, and work productivity in Crohn's disease patients after one year of treatment with adalimumab.

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Saro, Cristina; Ceballos, Daniel; Muñoz, Fernando; de la Coba, Cristóbal; Aguilar, María Dolores; Lázaro, Pablo; García-Sánchez, Valle; Hernández, Mariola; Barrio, Jesús; de Francisco, Ruth; Fernández, Luis I; Barreiro-de Acosta, Manuel

2017-02-01

Clinical trials have shown the efficacy of adalimumab in Crohn's disease, but the outcome in regular practice remains unknown. The aim of the study was to examine clinical status, quality of life, and work productivity of Crohn's disease patients receiving adalimumab for one year in the context of usual clinical practice. This was a prospective, observational study with a one-year follow-up. After baseline, Crohn's disease patients were evaluated at 1, 3, 6, 9, and 12 months after starting treatment with adalimumab. Outcome variables included: clinical status (measured with CDAI), quality of life (measured with EuroQoL-5D and IBDQ), and work productivity (measured with WPAI questionnaire). These outcome variables were compared using the Student's t test or Wilcoxon test for paired comparison data according to the data distribution. Statistical significance was set at two-sided p work productivity impact decreased from 53% to 24% (p quality of life and work productivity.

Trust and memory: organizational strategies, institutional conditions and trust negotiations in specialty clinics for Alzheimer's disease.

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Beard, Renée L

2008-03-01

Clinicians aim to establish trust during medical encounters because, without it, health consumers may not seek medical care, consider their diagnoses legitimate, or adhere to treatment regimens. This paper examines the identification and treatment of memory loss within two specialty clinics to understand how cultural dynamics, such as organizational ethos and work practices, influence the social fabric of cognitive evaluations. Ethnographic data suggest important historical and cultural differences in the approaches to Alzheimer's disease (AD). Organizational routines, however, support a common goal, that of moving individuals from "potential patients" to patients, and ultimately research subjects, through establishing trust. Although the processes through which trust is potentially achieved, or the social conditions of trust, were similar at the sites, the object of trust was different. Whereas one clinic encouraged trust in collective medical expertise, the other focused on trust in specific clinicians. These conditions affect the clinical consequences of trust, particularly how and when the diagnosis is delivered, use of the AD label and other terminology, and the level of standardization. The individual consequences include perceptions of patients and depictions of the prognosis. Whether cognitive impairment is viewed as a scientific puzzle to be solved or is seen as a chronic illness significantly shapes the organizational processes of clinical evaluation. Alzheimer's disease, as a cultural object, is a particularly salient exemplar of the clinical negotiation of ambiguous diagnostic categorizations and the unpredictable patient in daily biomedical practice.

Clinical evaluation of patients with patellofemoral disorders.

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Post, W R

1999-01-01

Accurate clinical evaluation of patients with patellofemoral disorders is the cornerstone of effective treatment. This article defines how a careful history and physical examination can direct strategies for nonoperative and operative management. A critical analysis of traditional methods of evaluation and a streamlined rational approach to clinical evaluation is presented. Key questions and important physical findings that affect treatment decisions are emphasized.

ACG clinical guidelines: diagnosis and management of celiac disease.

Science.gov (United States)

Rubio-Tapia, Alberto; Hill, Ivor D; Kelly, Ciarán P; Calderwood, Audrey H; Murray, Joseph A

2013-05-01

This guideline presents recommendations for the diagnosis and management of patients with celiac disease. Celiac disease is an immune-based reaction to dietary gluten (storage protein for wheat, barley, and rye) that primarily affects the small intestine in those with a genetic predisposition and resolves with exclusion of gluten from the diet. There has been a substantial increase in the prevalence of celiac disease over the last 50 years and an increase in the rate of diagnosis in the last 10 years. Celiac disease can present with many symptoms, including typical gastrointestinal symptoms (e.g., diarrhea, steatorrhea, weight loss, bloating, flatulence, abdominal pain) and also non-gastrointestinal abnormalities (e.g., abnormal liver function tests, iron deficiency anemia, bone disease, skin disorders, and many other protean manifestations). Indeed, many individuals with celiac disease may have no symptoms at all. Celiac disease is usually detected by serologic testing of celiac-specific antibodies. The diagnosis is confirmed by duodenal mucosal biopsies. Both serology and biopsy should be performed on a gluten-containing diet. The treatment for celiac disease is primarily a gluten-free diet (GFD), which requires significant patient education, motivation, and follow-up. Non-responsive celiac disease occurs frequently, particularly in those diagnosed in adulthood. Persistent or recurring symptoms should lead to a review of the patient's original diagnosis to exclude alternative diagnoses, a review of the GFD to ensure there is no obvious gluten contamination, and serologic testing to confirm adherence with the GFD. In addition, evaluation for disorders associated with celiac disease that could cause persistent symptoms, such as microscopic colitis, pancreatic exocrine dysfunction, and complications of celiac disease, such as enteropathy-associated lymphoma or refractory celiac disease, should be entertained. Newer therapeutic modalities are being studied in

Autoinflammatory diseases in adults. Clinical characteristics and prognostic implications.

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González García, A; Patier de la Peña, J L; Ortego Centeno, N

2017-03-01

Autoinflammatory diseases are clinical conditions with inflammatory manifestations that present in a periodic or persistent manner and are caused by acquired or hereditary disorders of the innate immune response. In general, these diseases are more common in childhood, but cases have been reported in adults and are therefore important for all specialists. There are few references on these diseases in adults due to their low prevalence and underdiagnosis. The aim of this study is to review the scientific literature on these disorders to systematise their clinical, prognostic and treatment response characteristics in adults. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

Bilingualism delays clinical manifestation of Alzheimer's disease

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Woumans, Evy; Santens, Patrick; Sieben, Anne; Versijpt, Jan; Stevens, Michaël; Duyck, Wouter

2015-01-01

The current study investigated the effects of bilingualism on the clinical manifestation of Alzheimer's disease (AD) in a European sample of patients. We assessed all incoming AD patients in two university hospitals within a specified timeframe. Sixty-nine monolinguals and 65 bilinguals diagnosed with probable AD were compared for time of clinical AD manifestation and diagnosis. The influence of other potentially interacting variables was also examined. Results indicated a significant delay f...

Clinical pharmacy activities in chronic kidney disease and end-stage renal disease patients: a systematic literature review

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Stemer Gunar

2011-07-01

Full Text Available Abstract Background Chronic kidney disease (CKD and end-stage renal disease (ESRD represent worldwide health problems with an epidemic extent. Therefore, attention must be given to the optimisation of patient care, as gaps in the care of CKD and ESRD patients are well documented. As part of a multidisciplinary patient care strategy, clinical pharmacy services have led to improvements in patient care. The purpose of this study was to summarise the available evidence regarding the role and impact of clinical pharmacy services for these patient populations. Methods A literature search was conducted using the Medline, Embase and International Pharmaceutical Abstracts databases to identify relevant studies on the impact of clinical pharmacists on CKD and ESRD patients, regarding disease-oriented and patient-oriented outcomes, and clinical pharmacist interventions on drug-related problems. Results Among a total of 21 studies, only four (19% were controlled trials. The majority of studies were descriptive (67% and before-after studies (14%. Interventions comprised general clinical pharmacy services with a focus on detecting, resolving and preventing drug-related problems, clinical pharmacy services with a focus on disease management, or clinical pharmacy services with a focus on patient education in order to increase medication knowledge. Anaemia was the most common comorbidity managed by clinical pharmacists, and their involvement led to significant improvement in investigated disease-oriented outcomes, for example, haemoglobin levels. Only four of the studies (including three controlled trials presented data on patient-oriented outcomes, for example, quality of life and length of hospitalisation. Studies investigating the number and type of clinical pharmacist interventions and physician acceptance rates reported a mean acceptance rate of 79%. The most common reported drug-related problems were incorrect dosing, the need for additional

miRNAs in inflammatory skin diseases and their clinical implications

DEFF Research Database (Denmark)

Løvendorf, Marianne B; Skov, Lone

2015-01-01

biological processes. The clinical implications of miRNAs are intriguing, both from a diagnostic and a therapeutic perspective. Accordingly, there is emerging evidence for the clinical potential of miRNAs as both biomarkers and possible therapeutic targets in skin diseases. Future studies will hopefully...... incomplete; however, it is known that miRNAs are implicated in various cellular processes of both normal and diseased skin. Some miRNAs appear to be consistently deregulated in several different inflammatory skin diseases, including psoriasis and atopic dermatitis, indicating a common role in fundamental...

METHODOLOGICAL APPROACHES TO EXPERT EVALUATION OF PRECLINICAL AND CLINICAL TRIALS OF HUMAN IMMUNOGLOBULIN PRODUCTS

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V. B. Ivanov

2017-01-01

Full Text Available The article considers the experience of Russian and leading foreign regulatory agencies in organisation and conduction of preclinical and clinical trials of human immunoglobulin products. The authors suggest a classification of human immunoglobulins and provide updated information on authorization of these products in Russia. The article summarizes methodological approaches, basic scientific principles and criteria relating to expert evaluation of preclinical and clinical trials of blood products. The authors further define the expert body’s requirements for data on preclinical and clinical trials of human normal immuniglobulins and human specific immunoglobulins for the prevention and/or treatment of infectious and non-infectious diseases which are submitted as part of applications for marketing authorization or marketing authorization variation. The article suggests programs of preclinical and clinical trials for human normal immunoglobulins and human specific immunoglobulins for the prevention and/or treatment of infectious and non-infectious diseases that are aligned with the Russian legislation and Eurasian Economic Union’s regulations on medicines circulation, and have been elaborated with respect to the guidelines of the European Medicines Agency.

The use of Phyllanthus niruri L. as an immunomodulator for the treatment of infectious diseases in clinical settings

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Raymond Rubianto Tjandrawinata

2017-03-01

Full Text Available Phyllanthus niruri L. (Euphorbiaceae (P. niruri has traditionally been used in many tropical countries to treat various ailments, such as kidney stones, chronic liver diseases, diabetes and viral infections. The versatile ethnomedicinal usage of the herb is tightly associated with its multiple pharmacological properties such as immunomodulator, anti-viral, antibacterial, diuretic, anti-hyperglycemia and hepatoprotector. The scope of this review is limited only to the clinical evidences demonstrating benefits of the plant P. niruri with its immunomodulatory properties, for the treatment of various infectious diseases. These evidences are expected to provide the plant a more significant place in the current clinical settings, particularly in the management of infectious diseases. P. niruri as an immunomodulator has scientifically been studied and evaluated in various clinical trials for the treatment of chronic hepatitis B, pulmonary tuberculosis, vaginitis, as well as varicella-zoster infection. In such diseases, the effective immune system is crucial to the treatment success and eradication of the pathogens. In those clinical studies, P. niruri has been proven for its capacity to modulate and activate the immune system. In fact, there are numerous in vitro and animal studies reporting potential benefits of the immunomodulatory properties of P. niruri, and numbers of randomized controlled clinical studies have been published to date. In the light of the scarcity of research to discover new, more effective and safe anti-infection chemical entities, that is also complicated with the growing threat from the new generations of drug resistant-pathogens, the utilization of nature-derived immunomodulatory agents, either alone or combined with the currently available antibiotics or antivirals, is undoubtedly promising and of clinical importance. Most of the studies on P. niruri warrant its potential benefits in various infectious diseases, and are

A clinical decision support system algorithm for intravenous to oral antibiotic switch therapy: validity, clinical relevance and usefulness in a three-step evaluation study.

Science.gov (United States)

Akhloufi, H; Hulscher, M; van der Hoeven, C P; Prins, J M; van der Sijs, H; Melles, D C; Verbon, A

2018-04-26

To evaluate a clinical decision support system (CDSS) based on consensus-based intravenous to oral switch criteria, which identifies intravenous to oral switch candidates. A three-step evaluation study of a stand-alone CDSS with electronic health record interoperability was performed at the Erasmus University Medical Centre in the Netherlands. During the first step, we performed a technical validation. During the second step, we determined the sensitivity, specificity, negative predictive value and positive predictive value in a retrospective cohort of all hospitalized adult patients starting at least one therapeutic antibacterial drug between 1 and 16 May 2013. ICU, paediatric and psychiatric wards were excluded. During the last step the clinical relevance and usefulness was prospectively assessed by reports to infectious disease specialists. An alert was considered clinically relevant if antibiotics could be discontinued or switched to oral therapy at the time of the alert. During the first step, one technical error was found. The second step yielded a positive predictive value of 76.6% and a negative predictive value of 99.1%. The third step showed that alerts were clinically relevant in 53.5% of patients. For 43.4% it had already been decided to discontinue or switch the intravenous antibiotics by the treating physician. In 10.1%, the alert resulted in advice to change antibiotic policy and was considered useful. This prospective cohort study shows that the alerts were clinically relevant in >50% (n = 449) and useful in 10% (n = 85). The CDSS needs to be evaluated in hospitals with varying activity of infectious disease consultancy services as this probably influences usefulness.

Phase 2 clinical study of 123I-IBF, a dopamine D2 receptor imaging agent, to evaluate clinical efficacy and safety in Parkinson's disease and Parkinson syndromes

International Nuclear Information System (INIS)

Torizuka, Kanji; Mizuno, Yoshikuni; Kubo, Atsushi

1999-01-01

A Phase 2 multicenter trial of 123 I-IBF, (S)-5-iodo-7-N-[(1-ethyl-2-pyrrolidinyl)methyl] carboxamido-2,3-dihydrobenzofuran, was conducted to evaluate its clinical efficacy and safety in 158 patients with Parkinson's disease (PD) or Parkinson syndromes (PS). SPECT data were acquired at two hours (2H-SPECT), after intravenous injection of 123 I-IBF (167 MBq). Additional SPECT scan at three hours (3H-SPECT) and dynamic SPECT scan at most until one hour were performed when possible. No severe side effects due to 123 I-IBF injection were observed. The sensitivity, specificity and accuracy for discriminating PS from PD using the striatal specific binding count-to-frontal cortex count ratio (St/Fc-1) in 3H-SPECT were 72.7%, 81.0% and 78.1% in 64 clinically definite cases (i.e., typical cases), respectively. The putamen-to-caudate ratios were significantly lower in striatonigral degeneration compared with those in PD. The contralateral-to-ipsilateral ratios against the symptomatic side of tremor and/or rigidity in the patients with PD (Hoehn and Yahr I) were significantly higher than the left-to-right ratios in the normal controls. St/Fc-1 in 3H-SPECT was significantly lower in the patients showing a poor response to levodopa than in those showing a good response. The dopamine D 2 receptor binding potential (k 3 /k 4 ), obtained by dynamic SPECT based on compartment model analysis, correlated well with the striatal specific binding count-to-occipital cortex count ratio. These results suggest that 123 I-IBF is a promising agent for differential diagnosis and pathophysiological evaluation of PD and PS. (author)

Periodontal disease in diabetic patients - clinical and histopathological aspects.

Science.gov (United States)

Corlan Puşcu, Dorina; Ciuluvică, Radu Constantin; Anghel, Andreea; Mălăescu, Gheorghe Dan; Ciursaş, Adina Nicoleta; Popa, Gabriel Valeriu; Agop Forna, Doriana; Busuioc, Cristina Jana; Siloşi, Izabela

2016-01-01

Periodontal disease is one of the most frequent diseases affecting people all over the world. The relation between periodontal disease and diabetes mellitus raised the interest both of dentists and doctors treating metabolic diseases, as the two conditions influence one another. In our study, we analyzed a number of 75 patients with diabetes mellitus and periodontal disease that presented to the medical consultory for conditions of the dental maxillary system. The clinical study showed that periodontal disease and diabetes may affect young adults as well, still this pathological association more frequently appears after the age of 50. The disease was identified especially in the women living in urban area. The clinical examination of the dental maxillary system identified the presence of gingival ulcerations, dental calculus, gingival bleeding, radicular leftovers with anfractuous margins, fixed prostheses with an inappropriate cervical adjustment. Of the systemic diseases associated to periodontal disease and diabetes mellitus, there was observed that 66.66% of the patients also suffered from cardiovascular diseases (high blood pressure, ischemic cardiopathy, heart failure), and 37.33% suffered from obesity. The histopathological and immunohistochemical tests highlighted the presence of an inflammatory chronic, intense reaction, mainly formed of lymphocytes, plasmocytes, macrophages and granulocytes, heterogeneously disseminated and alteration of the structure of marginal and superficial periodontium. The inflammatory reaction in the patients with periodontal disease and diabetes was more intense than in the patients with periodontal disease without diabetes.

Clinic Attendance of Youth With Sickle Cell Disease on Hydroxyurea Treatment.

Science.gov (United States)

Ingerski, Lisa M; Arnold, Trisha L; Banks, Gabrielle; Porter, Jerlym S; Wang, Winfred C

2017-07-01

The objective of this study is to describe rates of clinic attendance of youth with sickle cell disease prescribed hydroxyurea and examine potential demographic and medical factors related to consistent clinic attendance. Participants included 148 youth diagnosed with sickle cell disease and prescribed hydroxyurea during a single calendar year. Clinic attendance and potential demographic and medical factors related to attendance were extracted via systematic retrospective medical chart review. Youth attended 90.3% of scheduled appointments and 85.1% of youth attended at least 80% of scheduled clinic appointments during the study window. Adjusting for other factors, multivariate analysis revealed families with fewer children in the household, families with private insurance, youth experiencing fever, and youth not experiencing pain during the calendar year were more likely to consistently attend clinic visits. Adherence to clinic appointments is critical to optimizing health outcomes for youth with sickle cell disease and integral for adequate monitoring of youth prescribed hydroxyurea, in particular. Findings may aid providers in appropriately identifying possible barriers to clinic attendance to develop attendance promotion interventions.

Clinical features and prevalence of gastroesophageal reflux disease in infants attending a pediatric gastroenterology reference service.

Science.gov (United States)

Koda, Yu Kar Ling; Ozaki, Marcos J; Murasca, Kelly; Vidolin, Eliana

2010-01-01

In infants, it is not always easy to distinguish between pathological and physiological gastroesophageal reflux based only on clinical criteria. In Brazil, studies about gastroesophageal reflux disease in infants are few and are even rare those that used prolonged esophageal pH monitoring for its evaluation. To describe the clinical features of gastroesophageal reflux disease and to determine its prevalence in infants with gastroesophageal reflux attending a tertiary Pediatric Gastroenterology Service and submitted to esophageal pH monitoring for investigation. Descriptive study in 307 infants in whom esophageal pH monitoring (Mark III Digitrapper, Synectics Medical AB, Sweden) was performed during the period December, 1998-December, 2008. The clinical features studied were age group (1-12 months and 13-24 months), and clinical manifestations that motivated the indication of pH monitoring. One hundred twenty-four (40.4%) were female and 183 (59.6%) male with mean age 12.2 +/- 6.2 months (1-23 months). The prevalence of gastroesophageal reflux disease was 18.2% (56/307). One hundred forty-eight (48.2%) were 1-12 months old and 159 (51.8%), 13-24 months. No significant difference was found between the prevalence of these two age groups (P = 0.3006). Gastroesophageal reflux disease was more frequent in those with digestive manifestations (24.2%), crisis of cyanosis/apnea (23.8%) and mixed manifestations (21.5%). Respiratory manifestations were the most frequent indication (39.1%) of pH monitoring. However, the prevalence of gastroesophageal reflux disease was lower (12.5%) in this group compared with in those with digestive manifestations (P = 0.0574), crisis of cyanosis/apnea (P = 0.0882) and mixed manifestations (P = 0.1377). All infants that presented clinical manifestations as crisis of cyanosis/apnea and abnormal pH-metry were Service, the prevalence of gastroesophageal reflux disease associated with acid reflux in infants revealed elevated. Infants with crisis

Effectiveness of nurse-led cardiac clinics in adult patients with a diagnosis of coronary heart disease.

Science.gov (United States)

Page, Tamara; Lockwood, Craig; Conroy-Hiller, Tiffany

2005-02-01

Background  Coronary heart disease is the major cause of illness and death in Western countries and this is likely to increase as the average age of the population rises. Consumers with established coronary heart disease are at the highest risk of experiencing further coronary events. Lifestyle measures can contribute significantly to a reduction in cardiovascular mortality in established coronary heart disease. Improved management of cardiac risk factors by providing education and referrals as required has been suggested as one way of maintaining quality care in patients with established coronary heart disease. There is a need to ascertain whether or not nurse-led clinics would be an effective adjunct for patients with coronary heart disease to supplement general practitioner advice and care. Objectives  The objective of this review was to present the best available evidence related to nurse-led cardiac clinics. Inclusion criteria  This review considered any randomised controlled trials that evaluated cardiac nurse-led clinics. In the absence of randomised controlled trials, other research designs such as non-randomised controlled trials and before and after studies were considered for inclusion. Participants were adults (18 years and older) with new or existing coronary heart disease. The interventions of interest to the review included education, assessment, consultation, referral and administrative structures. Outcomes measured included adverse event rates, readmissions, admissions, clinical and cost effectiveness, consumer satisfaction and compliance with therapy. Results  Based on the search terms used, 80 papers were initially identified and reviewed for inclusion; full reports of 24 of these papers were retrieved. There were no papers included that addressed cost effectiveness or adverse events; and none addressed the outcome of referrals. A critical appraisal of the 24 remaining papers identified a total of six randomised controlled trials that

Clinical evaluation of patients with spinal cord infarction in mashhad, iran.

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Ghandehari, Kavian; Gerami Sarabi, Mohammad Reza; Maarufi, Parham

2010-10-26

Background. Spinal Cord Infarction (SCI) is a rare and disabling disease. This hospital-based study was conducted for clinical evaluation of SCI patients in east of Iran. Methods. Consecutive SCI patients admitted in Ghaem hospital,Mashhad during 2006-2010 were enrolled in a prospective clinical study. Diagnosis of SCI was made by neurologists and radiologists. Demographic features, clinical syndrome, and Magnetic Resonance Imaging (MRI) findings were recorded. All of the patients underwent a standard battery of diagnostic investigations. All of the patients suspected to SCI had MRI of spinal cord at the symptomatic level of cord with a 0.5 Tesla generation, Philips NT Intra, Netherland equipment. An equal number of patients with Brain Infarction (BI) were randomly selected from our stroke registry data bank. Etiology and degree of disability were compared between these groups of patients. Results. Fourteen SCI patients (9 females, 5 males) with mean age 38.8 ± SD: 19.9 years were evaluated. Miscellaneous causes consisted 50% of etiologies in patients with SCI. Uncertain etiology, atherosclerosis, and cardioembolisms consisted 35.7%, 7.1%, and 7.1% of SCI causes, respectively. Distribution of etiologies was significantly different between SCI and BI patients, X(2) = 12.94, df = 3, P = .003. Difference in mean disability score at acute phase of stroke was not significant between two studied groups, z = 1.54, P = .057. Difference in mean changes of disability score at 90 days postevent was significant in two groups of patients, z = 2.65, P = .019. Conclusion. SCI is a rare disease with poor recovery. Distribution of etiologies of SCI patients is quite different than of BI patients.

Clinical Evaluation of Patients with Spinal Cord Infarction in Mashhad, Iran

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Kavian Ghandehari

2010-01-01

Full Text Available Background. Spinal Cord Infarction (SCI is a rare and disabling disease. This hospital-based study was conducted for clinical evaluation of SCI patients in east of Iran. Methods. Consecutive SCI patients admitted in Ghaem hospital,Mashhad during 2006–2010 were enrolled in a prospective clinical study. Diagnosis of SCI was made by neurologists and radiologists. Demographic features, clinical syndrome, and Magnetic Resonance Imaging (MRI findings were recorded. All of the patients underwent a standard battery of diagnostic investigations. All of the patients suspected to SCI had MRI of spinal cord at the symptomatic level of cord with a 0.5 Tesla generation, Philips NT Intra, Netherland equipment. An equal number of patients with Brain Infarction (BI were randomly selected from our stroke registry data bank. Etiology and degree of disability were compared between these groups of patients. Results. Fourteen SCI patients (9 females, 5 males with mean age 38.8±SD: 19.9 years were evaluated. Miscellaneous causes consisted 50% of etiologies in patients with SCI. Uncertain etiology, atherosclerosis, and cardioembolisms consisted 35.7%, 7.1%, and 7.1% of SCI causes, respectively. Distribution of etiologies was significantly different between SCI and BI patients, X2=12.94, df=3, P=.003. Difference in mean disability score at acute phase of stroke was not significant between two studied groups, z=1.54, P=.057. Difference in mean changes of disability score at 90 days postevent was significant in two groups of patients, z=2.65, P=.019. Conclusion. SCI is a rare disease with poor recovery. Distribution of etiologies of SCI patients is quite different than of BI patients.

Evaluation of Clinical and Laboratory Data in Patients with Recurrent Infections and Suspected Immunodeficiency

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Hamid Ahanchian

2014-09-01

Full Text Available Background: Frequent infections is among the most frequent clinical dilemmas for primary care physicians. Immunodeficiency disorders are a heterogeneous group of illnesses that predispose patients to the recurrent infections, autoimmunity and malignancies. The aim of this study was to evaluate the clinical and laboratory data collected for the final diagnosis of patients referred with recurrent infections and suspected immunodeficiency to a local immunodeficiency clinic.   Methods: This epidemiological study was carried out between April 2010 and September 2012 at the Immunodeficiency Clinic of Mashhad. All patients with clinical manifestations of immunodeficiency who were referred to our clinic during this period of time were included in this study. 41 patients aged from 10 months to 51 years, were evaluated. Results: Forty one patients, aged between 10 months and 51 years were evaluated. Eleven patients had a primary immunodeficiency, four cases had a secondary immunodeficiency, in three patients an underlying structural disease were found, eight patients were predisposed to recurrent infections as a result of allergies and finally, fifteen cases were found to be normal individuals.   Discussion: Most patients with recurrent infection have a normal immune system. Allergic disorders are the most common predisposing factor to recurrent infection. However, as immunodeficiency disorders are potentially serious, early diagnosis can improve the quality of life and outcome and prevent severe sequels in future.

Calcium pyrophosphate deposition disease: clinical manifestations

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M.A. Cimmino

2012-01-01

Full Text Available Calcium pyrophosphate deposition (CPPD disease is an arthropathy caused by calcium pyrophosphate dihydrate (CPP crystal deposits in articular tissues, most commonly fibrocartilage and hyaline cartilage. According to EULAR, four different clinical presentations can be observed: 1 asymptomatic CPPD; 2 osteoarthritis (OA with CPPD; 3 acute CPP crystal arthritis; 4 chronic CPP inflammatory crystal arthritis. Acute CPP crystal arthritis is characterized by sudden onset of pain, swelling and tenderness with overlying erythema, usually in a large joint, most often the knee, wrist, shoulder, and hip. Occasionally, ligaments, tendons, bursae, bone and the spine can be involved. CPPD of the atlanto-occipital joint (crowned dens syndrome can cause periodic acute cervico-occipital pain with fever, neck stiffness and laboratory inflammatory syndrome. Chronic inflammatory arthritis is characterized by joint swelling, morning stiffness, pain, and high ESR and CRP. The relationship between OA and CPPD is still unclear. The main problem is whether such crystals are directly involved in the pathogenesis of OA or if they are the result of joint degeneration. Diagnosis is based on evaluation of history and clinical features, conventional radiology, and synovial fluid examination. Non-polarized light microscopy should be used initially to screen for CPPD crystals based upon their characteristic morphology, and compensated polarized light microscopy, showing the crystals to be weakly positive birefringent, is recommended for definitive identification, although this last pattern only occurs in about 20% of samples. The main goals of CPPD therapy are control of the acute or chronic inflammatory reaction and prevention of further episodes.

Clinical evaluation of rituximab treatment for neuromyelitis optica.

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Fernández-Megía, M J; Casanova-Estruch, B; Pérez-Miralles, F; Ruiz-Ramos, J; Alcalá-Vicente, C; Poveda-Andrés, J L

2015-10-01

Neuromyelitis optica is an inflammatory and usually relapsing demyelinating autoimmune disease of the central nervous system that targets the optic nerves and spinal cord. Rituximab has been used for different neurological diseases that are probably immune-mediated or involving humoural immunity. The objective of this study is to evaluate the efficacy and safety of rituximab as treatment for neuromyelitis optica in a tertiary hospital. Retrospective study of patients with neuromyelitis optica treated with rituximab 1000mg on days 1 and 15, repeated every 6 to 8 months. We recorded EDSS score, relapse rate, overall condition, CD19+ count, presence of anti-NMO antibodies, and possible adverse reactions. Six patients were treated; all were women with a median age of 46 years (range, 38-58). Anti-NMO antibodies were detected in 3 patients (50%). Baseline EDSS was 4 (range 2.0-5.5). Two patients had previously been treated with an immunomodulatory drug. Median time from the first rituximab infusion to first relapse was 3.7 years (range 1.7-6.9). Two patients had infusion reactions after the first dose of rituximab. Four patients remained relapse-free and their EDSS score did not progress during rituximab treatment, one patient showed no clinical improvement, and one patient could not be evaluated. Rituximab can be considered an attractive therapeutic alternative for patients with neuromyelitis optica as there are no approved treatments for this disease. Further studies with rituximab are needed to establish the role of this drug in treating neuromyelitis optica. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.

The clinical usefulness of NP-59 scintigraphy in adrenal cortical diseases

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Kim, Duk Kyu

1997-01-01

131 I-6-β-iodomethyl-19-norcholesterol (NP-59) has an advantage to assess adrenal dysfunction caused by adrenal cortical disorders. The aim of this study is to evaluate the clinical usefulness of NP-59 scintigraphy in each adrenal disease. Ten patients who did eleven NP-59 adrenal scintigraphies at Dong-A University Hospital from March 1990 to December 1996 were selected as the subject. Among the subject there were 5 cases of Cushing's syndrome, 2 cases of incidentaloma, 1 case of metastatic adrenal tumor, liver cirrhosis with hirsutism and hypertension respectively. Among 5 cases of Cushing's syndrome, there were 2 cases of Cushing's disease, 2 cases of adrenal adenoma and 1 case of adrenal carcinoma. There are no disagreement between clinical diagnosis and scan finding in Cushing's syndrome. In 2 incidentaloma cases, even though one is interpretated as a functioning tumor, both of 2 cases could avoid unnecessary biopsy according to scintigraphy result. One case of hirsutism, clinically adrenal originated, revealed the normal scintigraphic finding after dexamethasone suppression scan. It could suggest that the etiology of hirsutism was extra-adrenal origin. One case of hypertension took the study to exclude the possibility of primary aldosteronism. Normal suppression scan finding revealed that primary aldosteronism did not exist in this case. In conclusion, NP-59 scintigraphy was very useful in diagnosis and differential diagnosis of Cushing's syndrome and it could avoid unnecessary biopsy in the incidental adrenal tumor

Chronic Disease Management in Family Practice: Clinical Note.

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1998-03-01

disease management in the family practice selling. This paper discusses chronic disease management in the family practice selling....Chronic disease management is the process of evaluating and treating a medical condition or disease state which can not be readily cured so as to...minimize it’s negative impact on the individual. Examples of chronic disease management include the treatment of hypertension, diabetes, osteoporosis

Comparing Clinical Profiles in Alzheimer's Disease and Parkinson's Disease Dementia

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Martin R. Farlow

2013-09-01

Full Text Available Background: Greater understanding of differences in baseline impairment and disease progression in patients with Alzheimer's disease (AD and Parkinson's disease dementia (PDD may improve the interpretation of drug effects and the design of future studies. Methods: This was a retrospective analysis of three randomized, double-blind rivastigmine databases (one in PDD, two in AD. Impairment on the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog, Alzheimer's Disease Cooperative Study-Activities of Daily Living (ADCS-ADL scale, 10-item Neuropsychiatric Inventory (NPI-10 and the ADCS-Clinical Global Impression of Change (CGIC was compared [standardized difference (Cohen's d, similar if Results: Patients with AD or PDD had similar levels of impairment on the ADAS-cog and NPI-10. Scores on the ADCS-ADL scale (standardized difference = 0.47 and the ADAS-cog memory domain (total, 0.33; items, 0.10-0.58 were higher in AD; PDD patients were more impaired in the language (0.23 and praxis (0.34 domains. AD patients receiving placebo showed greater deterioration on the ADAS-cog (0.14 and improvement on the NPI-10 (0.11 compared with patients with PDD. Conclusion: Differing patterns of impairment occur in AD and PDD.

Comparing clinical profiles in Alzheimer's disease and Parkinson's disease dementia.

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Farlow, Martin R; Schmitt, Frederick; Aarsland, Dag; Grossberg, George T; Somogyi, Monique; Meng, Xiangyi

2013-01-01

Greater understanding of differences in baseline impairment and disease progression in patients with Alzheimer's disease (AD) and Parkinson's disease dementia (PDD) may improve the interpretation of drug effects and the design of future studies. This was a retrospective analysis of three randomized, double-blind rivastigmine databases (one in PDD, two in AD). Impairment on the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog), Alzheimer's Disease Cooperative Study-Activities of Daily Living (ADCS-ADL) scale, 10-item Neuropsychiatric Inventory (NPI-10) and the ADCS-Clinical Global Impression of Change (CGIC) was compared [standardized difference (Cohen's d), similar if <0.1]. Patients with AD or PDD had similar levels of impairment on the ADAS-cog and NPI-10. Scores on the ADCS-ADL scale (standardized difference = 0.47) and the ADAS-cog memory domain (total, 0.33; items, 0.10-0.58) were higher in AD; PDD patients were more impaired in the language (0.23) and praxis (0.34) domains. AD patients receiving placebo showed greater deterioration on the ADAS-cog (0.14) and improvement on the NPI-10 (0.11) compared with patients with PDD. Differing patterns of impairment occur in AD and PDD.

High rate of smoking in female patients with Mondor's disease in an outpatient clinic in Japan

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Okumura T

2012-09-01

Full Text Available Toshikatsu Okumura,1 Masumi Ohhira,1 Tsukasa Nozu21Department of General Medicine, 2Department of Regional Medicine and Education, Asahikawa Medical University, Asahikawa, Hokkaido, JapanPurpose: Little is known about the epidemiology of Mondor's disease. The aim of this study was to analyze the clinical features of Mondor's disease in an outpatient clinic where primary care physicians are working in Japan, to better understand the epidemiological characteristics of the disease.Patients and methods: The data for consecutive outpatients who were new visitors to the Department of General Medicine in the teaching hospital (Asahikawa Medical University Hospital at Asahikawa Medical University, Asahikawa, Hokkaido, Japan, between April 2004 and March 2012 were analyzed. Parameters such as age, sex, diagnosis, and clinical presentation were investigated.Results: During the 8-year period covered in this study, six (0.07% out of 8767 patients were diagnosed as having Mondor's disease. All of these patients with Mondor's disease were female, and the mean age was 41 plus or minus 12 years; the overall rate of Mondor's disease in all female patients involved in this study was 0.12%. The patients complained of pain and a cord-like structure in the anterolateral thoracoabdominal wall. The painful mass had persisted for 1–4 weeks before presenting at the Department of General Medicine and it disappeared within a couple of weeks. Current smoking was significantly higher in the patients with Mondor's disease than in the age-matched female patients without Mondor's disease who were also evaluated in this study.Conclusion: These results suggest that a high rate of smoking in middle-aged females may be a characteristic feature of Mondor's disease. These epidemiological data may be useful in detection of the disease in the primary care setting in Japan.Keywords: primary care, epidemiology, current smoking, women

[Clinical and therapeutical considerations regarding the diagnosis and treatment in meconial disease].

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Sabetay, C; Ciobanu, O; Zavate, A; Ciucă, M; Malos, A; Marin-Diu, B

2010-01-01

The meconium disease represents a problem of great impact over the vital prognosis of the newborn, the mortality of this disease being rated in various studies between 30 and 50%. The meconial disease that reunites the meconial ileus and the meconial peritonitis is often associated with cystic fibrosis a disease of general impact over the entire organism increasing the gravity and influencing the vital prognosis of these patients. The authors' purpose is to evaluate the results obtained after the treatment of the meconial disease in the Pediatric Surgery Clinic of the Emergency Hospital of Craiova over a period of 10 years. The study has been made on a number of 16 cases of meconial ileus, plug and peritonitis analising the difficulty of the diagnostic process, the preoperative care as well as the possibilities of surgical treatment. The surgical treatment is composed of multiple procedures including simple suture of the bowel defect and segmentary bowel resections with primary anastomosis and/or secondary anastomosis after ileostoma. The mortality on the studied cases was of 37.5% despite recent progresses achieved in postoperative care of these type of patients.

Clinical application of dynamic digital subtraction angiography in cerebrovascular ischemic diseases

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Hirata, Yoshifumi; Nonaka, Nobuhito; Matsukado, Yasuhiko; Takahashi, Mutsumasa

1987-09-01

Dynamic intravenous digital subtraction angiography (IV-DSA) was performed in 37 patients with cerebrovascular ischemic diseases. The time density curve of IV-DSA was analysed, and peak time, mean transit time and mode of transit time were obtained in each patient. On the basis of these values, cerebral perfusion was classified into low, normal and high perfusion patterns. Normal perfusion pattern was noted in 40% of patients with transient ischemic attack (TIA) and 7 % of patients with cerebral infarction. Low perfusion pattern was observed in 60 % of patients with TIA and 87 % of patients with cerebral infarction. High perfusion pattern was encountered only in 7 % of patients with cerebral infarction. In ischemic patients with moyamoya disease, extremely prolonged cerebral circulation time was evidenced by the presence of a flat or uphill type of the time density curve. This finding well correlated with decreased cerebral blood flow on single photon emission tomography. These findings suggest that the analysis of dynamic DSA is very important and useful in the clinical evaluation of patients with cerebrovascular ischemic diseases.

The Affordability of Providing Sexually Transmitted Disease Services at a Safety-net Clinic.

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Dean, Lorraine T; Montgomery, Madeline C; Raifman, Julia; Nunn, Amy; Bertrand, Thomas; Almonte, Alexi; Chan, Philip A

2018-04-01

Sexually transmitted diseases continue to increase in the U.S. There is a growing need for financially viable models to ensure the longevity of safety-net sexually transmitted disease clinics, which provide testing and treatment to high-risk populations. This micro-costing analysis estimated the number of visits required to balance cost and revenue of a sexually transmitted disease clinic in a Medicaid expansion state. In 2017, actual and projected cost and revenues were estimated from the Rhode Island sexually transmitted disease clinic in 2015. Projected revenues for a hypothetical clinic offering a standard set of sexually transmitted disease services were based on Medicaid; private ("commercial") insurance; and institutional ("list price") reimbursement rates. The number of visits needed to cover clinic costs at each rate was assessed. Total operating cost for 2,153 clinic visits was estimated at $255,769, or $119 per visit. Laboratory testing and salaries each accounted for 44% of operating costs, medications for treatment 7%, supplies 5%, and 28% of visits used insurance. For a standard clinic offering a basic set of sexually transmitted disease services to break even, a projected 73% of visits need to be covered at the Medicaid rate, 38% at private rate, or 11% at institutional rate. Sexually transmitted disease clinics may be financially viable when a majority of visits are billed at a Medicaid rate; however, mixed private/public models may be needed if not all visits are billed. In this manner, sexually transmitted disease clinics can be solvent even if not all visits are billed to insurance, thus ensuring access to uninsured or underinsured patients. Copyright © 2018 American Journal of Preventive Medicine. Published by Elsevier Inc. All rights reserved.

Reflective journaling for clinical judgment development and evaluation.

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Lasater, Kathie; Nielsen, Ann

2009-01-01

Reflective journaling is a strategy used often in clinical education to gain insight into students' clinical thinking; however, studies indicate that students may benefit from guided reflections. Numerous tools have been used to structure student reflection with varying results. This article describes the outcomes from using the Guide for Reflection based on Tanner's Clinical Judgment Model. The Lasater Clinical Judgment Rubric, created from the Model, is used to evaluate development of clinical judgment and provides language to communicate about clinical thinking with students. Senior immersion course competencies, also developed with language from Tanner's Clinical Judgment Model,offer a comprehensive package that fosters students' clinical judgment development, faculty-student communication about clinical judgment, and evaluation of students' clinical thinking.

Ultrasound sonoelastography in the evaluation of thyroiditis and autoimmune thyroid disease.

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Ruchała, Marek; Szmyt, Krzysztof; Sławek, Sylwia; Zybek, Ariadna; Szczepanek-Parulska, Ewelina

2014-01-01

Sonoelastography (USE) is a constantly evolving imaging technique used for the noninvasive and objective estimation of tissue stiffness. Several USE methods have been developed, including Quasi-Static or Strain Elastography and Shear Wave Elastography. The utility of USE has been demonstrated in differentiating between malignant and benign thyroid lesions. Recently, USE has been applied in the evaluation of thyroiditis and autoimmune thyroid disease (AITD).Thyroid inflammatory illnesses constitute a diverse group of diseases and may manifest various symptoms. These conditions may share some parallel clinical, biochemical, and ultrasonographic features, which can lead to diagnostic difficulties. USE may be an additional tool, supporting other methods in the diagnosis and treatment monitoring of thyroid diseases, other than thyroid nodular disease.The aim of this article was to analyse and summarise the available literature on the applicability of different elastographic techniques in the diagnosis, differentiation and monitoring of various types of thyroiditis and AITD. Advantages and limitations of this technique are also discussed.

Evaluation of a model for induction of periodontal disease in dogs

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Rodrigo V. Sepúlveda

2014-06-01

Full Text Available There are several methods for inducing periodontal disease in animal models, being the bone defect one of the most reported. This study aimed to evaluate this model, through clinical, radiographic, tomographic and histological analyzes, thus providing standardized data for future regenerative works. Twelve dogs were subjected to the induction protocol. In a first surgical procedure, a mucoperiosteal flap was made on the buccal aspect of the right third and fourth premolars and a defect was produced exposing the furcation and mesial and distal roots, with dimensions: 5mm coronoapical, 5mm mesiodistal, and 3mm buccolingual. Periodontal ligament and cementum were curetted and the defect was filled with molding polyester, which was removed after 21 days on new surgical procedure. Clinical and radiographic examinations were performed after the two surgeries and before the collection of parts for dental tomography and histological analysis. All animals showed grade II furcation exposure in both teeth. Clinical attachment level increased after induction. Defect size did not change for coronoapical and buccolingual measurements, while mesiodistal size was significantly higher than at the time of defect production. Radiographic analysis showed decreased radiopacity and discontinuity of lamina dura in every tooth in the furcation area. The horizontal progression of the disease was evident in micro-computed tomography and defect content in the histological analysis. Therefore, it is concluded that this method promotes the induction of periodontal disease in dogs in a standardized way, thus being a good model for future work.

Clinical Utility of Additional Measurement of Total Lung Capacity in Diagnosing Obstructive Lung Disease in Subjects With Restrictive Pattern of Spirometry.

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Lee, Hyun; Chang, Boksoon; Kim, Kyunga; Song, Won Jun; Chon, Hae Ri; Kang, Hyung Koo; Kim, Jung Soo; Jeong, Byeong-Ho; Oh, Yeon-Mok; Koh, Won-Jung; Park, Hye Yun

2016-04-01

Total lung capacity (TLC), forced expiratory flow between 25 and 75% (FEF25-75%), peak expiratory flow (PEF), or post-bronchodilator volume response is recommended to detect obstructive abnormalities in the lung. The present study was performed to evaluate the usefulness of these pulmonary function test (PFT) parameters to diagnose obstructive lung disease in subjects with a restrictive pattern of spirometry. A retrospective study was conducted in 64 subjects with a restrictive pattern of spirometry (normal FEV1/FVC and low FVC) out of 3,030 patients who underwent all pre- and post-bronchodilator spirometry and lung volume measurement between April 2008 and December 2010. After subjects were clinically classified into those with obstructive lung disease, restrictive lung disease, and mixed lung disease, the agreements between the clinical diagnosis and PFT classification according to TLC, FEF(25-75%), PEF, and post-bronchodilator response criteria were compared. Of 64 subjects, 18 (28.1%) were classified with obstructive lung disease, 39 (60.9%) had restrictive lung disease, 1 (1.6%) had mixed lung disease, and 6 (9.4%) had no clinical lung disease. Among the 58 subjects with clinical lung disease, 22 (37.9%), 37 (63.8%), 33 (56.9%), and 3 (5.2%) were classified as having obstructive pattern based on TLC, FEF25-75%, PEF, and post-bronchodilator response criteria, respectively. The kappa coefficients for the agreement between the clinical classification and PFT classification using TLC, FEF25-75%, PEF, and post-bronchodilator response criteria in 58 subjects were 0.59, 0.18, 0.17, and spirometry, when obstructive lung disease is clinically suspected. Copyright © 2016 by Daedalus Enterprises.

Evaluating the quality of life in children with gastroesophagel reflux disease

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I.M. Nesinа

2017-08-01

Full Text Available Background. The purpose of the study was the assessment of the quality of life in adolescents with gastroesophageal reflux disease (GERD during the treatment. Materials and methods. 49 children aged 13–17 years with gastroesophageal reflux disease were examined. The comparison group consisted of 40 apparently healthy children of the same age. The effectiveness of therapy was evaluated during the treatment and in catamnesis, three months later. Quality of life was assessed using the PedsQL 4.0 inventory adapted for children aged 13 to 18 years, and GSRS — a specialized questionnaire for patients with digestive system diseases. Results. It was found that during the exacerbation of GERD, PedsQL 4.0 scores for all scales of the quality of life, especially psychophysiological features in children, reduced significantly by parent’s estimations. According to the GSRS questionnaire, a positive clinical effect was observed after the treatment, but the analysis of the PedsQL 4.0 inventory scales showed that the rates remained significantly lower compared to the control group. Only after 3 months, there was a positive trend toward increasing the number of points according to all the criteria of the PedsQL 4.0 inventory, which described improvements in the quality of teenager’s lives. Conclusions. The study of the quality of life parameters in the period of GERD clinical remission is a necessary component of treatment and the main criterion for assessing the state in adolescents. It allow to determine the clinical effectiveness and time of the main course of therapy, as well as to predict further course of the disease.

Developments in statistical evaluation of clinical trials

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Oud, Johan; Ghidey, Wendimagegn

2014-01-01

This book describes various ways of approaching and interpreting the data produced by clinical trial studies, with a special emphasis on the essential role that biostatistics plays in clinical trials. Over the past few decades the role of statistics in the evaluation and interpretation of clinical data has become of paramount importance. As a result the standards of clinical study design, conduct and interpretation have undergone substantial improvement. The book includes 18 carefully reviewed chapters on recent developments in clinical trials and their statistical evaluation, with each chapter providing one or more examples involving typical data sets, enabling readers to apply the proposed procedures. The chapters employ a uniform style to enhance comparability between the approaches.

Contrast-enhanced MRI compared with the physical examination in the evaluation of disease activity in juvenile idiopathic arthritis

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Hemke, Robert; Maas, Mario; Veenendaal, Mira van; Kuijpers, Taco W.; Dolman, Koert M.; Rossum, Marion A.J. van; Berg, J.M. van den

2014-01-01

To assess the value of magnetic resonance imaging (MRI) in discriminating between active and inactive juvenile idiopathic arthritis (JIA) patients and to compare physical examination outcomes with MRI outcomes in the assessment of disease status in JIA patients. Consecutive JIA patients with knee involvement were prospectively studied using an open-bore MRI. Imaging findings from 146 JIA patients were analysed (59.6 % female; mean age, 12.9 years). Patients were classified as clinically active or inactive. MRI features were evaluated using the JAMRIS system, comprising validated scores for synovial hypertrophy, bone marrow oedema, cartilage lesions and bone erosions. Inter-reader reliability was good for all MRI features (intra-class correlation coefficient [ICC] = 0.87-0.94). No differences were found between the two groups regarding MRI scores of bone marrow oedema, cartilage lesions or bone erosions. Synovial hypertrophy scores differed significantly between groups (P = 0.016). Nonetheless, synovial hypertrophy was also present in 14 JIA patients (35.9 %) with clinically inactive disease. Of JIA patients considered clinically active, 48.6 % showed no signs of MRI-based synovitis. MRI can discriminate between clinically active and inactive JIA patients. However, physical examination is neither very sensitive nor specific in evaluating JIA disease activity compared with MRI. Subclinical synovitis was present in >35 % of presumed clinically inactive patients. (orig.)

[Clinical and morphological variants of diverticular disease in colon].

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Levchenko, S V; Lazebnik, L B; Potapova, V B; Rogozina, V A

2013-01-01

Our own results of two-stage research are presented in the article. The first stage contains the retrospective analysis of 3682 X-ray examining of large bowel which were conducted in 2002-2004 to define the structure of colon disease and to determine gender differences. The second stage is prospective research which took place from 2003 to 2012 and 486 patients with diverticular disease were regularly observed. Following parameters were estimated: dynamics of complaints, life quality, clinical symptoms. Multiple X-ray and endoscopic examining were done with estimation of quantity and size of diverticula, changes of colon mucosa, comparison of X-ray and endoscopic methods in prognosis of complications. Two basic clinical morphological variants of diverticular disease (DD) of colon are made out as a result of our research. There are IBD-like and DD with ischemic component. The variants differ by pain characteristics, presence of accompanying diseases, life quality parameters and description of colon mucosa morphological research. We suppose that different ethiopathogenetic factors of development of both variants mentioned above influence the disease prognosis and selection of treatment.

Heterogeneous pattern of bone disease in adult type 1 Gaucher disease: clinical and pathological correlates.

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van Dussen, L; Lips, P; van Essen, H W; Hollak, C E M; Bravenboer, N

2014-09-01

Gaucher disease (GD) is a lysosomal storage disorder characterized by accumulation of glucosylceramide in macrophages, so-called Gaucher cells, as a result of a deficiency of the lysosomal enzyme glucocerebrosidase. Bone complications are an important cause of morbidity of GD and are thought to result from imbalance in bone remodeling. Bone manifestations among GD patients demonstrate a large variation including increased osteoclastic bone resorption, low bone formation and osteonecrosis. The purpose of the current case series is to describe the histological features observed in undecalcified bone samples, obtained from three GD patients, and evaluate the relationship with clinical features in these patients. Bone fragments were obtained from three adult type 1 GD patients with variable degrees of bone disease during orthopedic surgery. Specimens were embedded without prior decalcification in methylmethacrylate and prepared for histology according to standardized laboratory procedures. Histology revealed a heterogeneous pattern of bone involvement. High cellularity of bone marrow, abundant presence of Gaucher cells (GCs) and high turnover were observed in a patient with a history of multiple bone complications, while minimal bone turnover and few GCs were detected in the mildest affected patient in this series. An intermediate picture with relatively low bone turnover and a substantial amount of Gaucher cells was demonstrated in the third, moderately affected patient. No gross abnormalities in three biochemical markers of bone turnover (osteocalcin, N-terminal propeptide of type 1 procollagen and type 1 collagen C-terminal telopeptide) were noted. Plastic embedding and subsequent Goldner and TRAP staining offered a unique possibility to study bone histological findings in GD. Our data show that bone manifestations in GD may vary both clinically as well as histologically and bone disease in GD will likely require a personalized approach. Copyright © 2014 Elsevier

Genetics of liver disease: From pathophysiology to clinical practice.

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Karlsen, Tom H; Lammert, Frank; Thompson, Richard J

2015-04-01

Paralleling the first 30 years of the Journal of Hepatology we have witnessed huge advances in our understanding of liver disease and physiology. Genetic advances have played no small part in that. Initial studies in the 1970s and 1980s identified the strong major histocompatibility complex associations in autoimmune liver diseases. During the 1990 s, developments in genomic technologies drove the identification of genes responsible for Mendelian liver diseases. Over the last decade, genome-wide association studies have allowed for the dissection of the genetic susceptibility to complex liver disorders, in which also environmental co-factors play important roles. Findings have allowed the identification and elaboration of pathophysiological processes, have indicated the need for reclassification of liver diseases and have already pointed to new disease treatments. In the immediate future genetics will allow further stratification of liver diseases and contribute to personalized medicine. Challenges exist with regard to clinical implementation of rapidly developing technologies and interpretation of the wealth of accumulating genetic data. The historical perspective of genetics in liver diseases illustrates the opportunities for future research and clinical care of our patients. Copyright © 2015 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Pilonidal sinus disease - Etiological factors, pathogenesis and clinical features

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Kazim Duman

2016-12-01

Full Text Available and lsquo;Pilonidal sinus' disease, which is most commonly seen in reproductive populations, such as young adults - mostly in males who are in their twenties - is actually a controversial disease in that there is no consensus on its many facets. It is sometimes seen as an infected abscess draining from an opening or a lesion extending to the perineum. It may also present as a draining fistula opening to skin. In terms of etiological factors, various theories (main theories being congenital and acquired have been established since it was first described, no universal understanding achieved. A long and significant post-operative care period with different lengths of recovery depending on the type of operation are quite prevalent with regards to recurrence and complication status. In order to prevent recurrence and improve the quality of life, etiological and predisposing factors as well as clinical features of sacrococcygeal pilonidal disease should be well known, a detailed differential diagnosis should be made, and a suitable and timely intervention should be performed. It was aimed here to explain the etiological factors, pathogenesis and clinical features of the disease that may present with various clinical symptoms. [Arch Clin Exp Surg 2016; 5(4.000: 228-232

Automated interpretable computational biology in the clinic: a framework to predict disease severity and stratify patients from clinical data

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Soumya Banerjee

2017-10-01

Full Text Available We outline an automated computational and machine learning framework that predicts disease severity and stratifies patients. We apply our framework to available clinical data. Our algorithm automatically generates insights and predicts disease severity with minimal operator intervention. The computational framework presented here can be used to stratify patients, predict disease severity and propose novel biomarkers for disease. Insights from machine learning algorithms coupled with clinical data may help guide therapy, personalize treatment and help clinicians understand the change in disease over time. Computational techniques like these can be used in translational medicine in close collaboration with clinicians and healthcare providers. Our models are also interpretable, allowing clinicians with minimal machine learning experience to engage in model building. This work is a step towards automated machine learning in the clinic.

Clinical presentation of late haemorrhagic disease of newborn

International Nuclear Information System (INIS)

Majeed, R.; Memon, Y.; Majeed, F.

2008-01-01

To observe the clinical presentation of late haemorrhagic disease of the newborn (LHDNB), and clinical improvement after the administration of vitamin K/sub 1/. This is a prospective descriptive study. All the children older than seven days who presented with bleeding were admitted in pediatrics ward of Isra University Hyderabad from April 2006 to April 2007 were included. Data collection was done by means of detailed proforma. Analysis was done on SPSS version 11. Thirty five cases were included. Commonest site of bleeding was subcutaneous followed by oral and injection site. Mean age of late haemorrhagic disease of newborn was 109 days and minimum age of presentation was 28 days. Common clinical presentations were irritability, convulsions, poor reflexes and poor feeding. Mostly recovery was within 24 hours after vit K. Late HDN results in severe hemorrhage especially hemorrhage in the central nervous system. Administration of Vitamin K (1mg, 1M) at birth can present these severe complications. (author)

Mammography and ultrasound in the evaluation of male breast disease

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Munoz Carrasco, Rafaela; Alvarez Benito, Marina; Raya Povedano, Jose Luis [Hospital Universitario ' Reina Sofia' , Radiology Department, Cordoba (Spain); Munoz Gomariz, Elisa [Hospital Universitario ' Reina Sofia' , Support Unit for Research, Cordoba (Spain); Martinez Paredes, Maria [University of Cordoba, Radiology and Physical Medicine Area, Cordoba (Spain)

2010-12-15

To assess clinical variables that may be useful in differentiating gynaecomastia from carcinoma and to analyse the contribution of mammography and ultrasound to the evaluation of male breast disease. All men who underwent mammography and/or ultrasound between 1993 and 2006 in our hospital were retrospectively evaluated. Clinical characteristics in patients with gynaecomastia and those with carcinoma were compared. Radiological findings were classified according to the BI-RADS (Breast Imaging Reporting and Data System) criteria. The diagnostic performance of physical examination, mammography and ultrasound was determined and compared. A total of 628 patients with 518 mammograms and 423 ultrasounds were reviewed. The final diagnoses were: 19 carcinomas, 526 gynaecomastias, 84 other benign conditions and 25 normal. There were statistically significant differences in age, bilateral involvement, clinical presentation and physical examination between patients with carcinoma and those with gynaecomastia. The diagnostic performance of physical examination was lower than that of mammography and ultrasound (p < 0.05 for specificity). Mammography was the most sensitive (94.7%) and ultrasound the most specific (95.3%) for detection of malignancy (p > 0.05). We propose an algorithm for the use of mammography and ultrasound in men. Mammography and ultrasound, with a negative predictive value close to 100%, make it possible to avoid very many unnecessary surgical procedures in men. (orig.)

Clinical heterogeneity in newly diagnosed Parkinson's disease

NARCIS (Netherlands)

Post, Bart; Speelman, Johannes D.; de Haan, Rob J.

2008-01-01

OBJECTIVE: To determine clinical heterogeneity in newly diagnosed Parkinson's disease using cluster analysis and to describe the subgroups in terms of impairment, disability, perceived quality of life, and use of dopaminergic therapy. METHODS: We conducted a k-means cluster analysis in a prospective

Evaluation Apprehension and Impression Management in Clinical Medical Education.

Science.gov (United States)

McGaghie, William C

2018-05-01

Historically, clinical medical education has relied on subjective evaluations of students and residents to judge their clinical competence. The uncertainty associated with these subjective clinical evaluations has produced evaluation apprehension among learners and attempts to manage one's professional persona (impression management) among peers and supervisors. Such behavior has been documented from antiquity through the Middle Ages to the present, including in two new qualitative studies in this issue of Academic Medicine on the social psychology of clinical medical education. New approaches to medical education, including competency-based education, mastery learning, and assessment methods that unite evaluation and education, are slowly changing the culture of clinical medical education. The author of this Invited Commentary argues that this shift will bring greater transparency and accountability to clinical medical education and gradually reduce evaluation apprehension and the impression management motives it produces.

CLINICAL PROFILE OF PATTERN OF DYSLIPIDAEMIA AND ISCHAEMIC HEART DISEASE IN TYPE II DIABETES MELLITUS PATIENTS

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Atul Vijayrao Rajkondawar

2017-07-01

Full Text Available BACKGROUND The present research was undertaken to study the pattern of dyslipidaemia and varied clinical manifestations of ischaemic heart disease, its risk factors in type 2 diabetes mellitus patients. Diabetes Mellitus (DM has become major public health problem in India. It is not only increasing in morbidity and mortality, but also decreases the quality of life. Also, disease and its complications are causing heavy economic burden for patients suffering from it.1,2 Diabetes is endemic globally with increasing prevalence in both developing and developed countries. Diabetes is a major cause of mortality, but several studies indicate that it is likely underreported as a cause of death. A recent estimate suggested that diabetes was the fifth leading cause of death worldwide and was responsible for almost 3 million deaths annually (1.7-5.2% of all deaths. MATERIALS AND METHODS A total of 100 patients attending the outpatient department or admitted to medical wards, ICU of tertiary care centre and fulfilling the inclusion criteria were evaluated clinically. A baseline Electrocardiogram (ECG was taken in all cases irrespective of clinical evidence of cardiac involvement. Patients with normal ECG pattern were further evaluated by Treadmill Testing (TMT or stress test for subclinical cardiac involvement. Risk factor evaluation was done in all cases. RESULTS Prevalence of IHD was found to be 41% with a male predominance (1.067:1. Evaluation of risk factors has shown its strong association with IHD. Incidence of IHD was high when low HDL (P 25 had negatively significant association with IHD in type 2 diabetics (P=0.072. Smoking was not statistically associated (P=0.577 and in male alcoholics, IHD had positive association with alcohol (P=0.193. CONCLUSION The current study points out that there exists an increased incidence of ischaemic heart disease in diabetics with few, but not all risk factors contributing to it. Early detection, optimal glycaemic

Idiopathic Canalicular Inflammatory Disease: New Disease Description of Clinical Patterns, Investigations, Management, and Outcomes.

Science.gov (United States)

Ali, Mohammad Javed

2018-01-25

The objective of this perspective is to present a separate disease description of "idiopathic canalicular inflammatory disease" and outline the diagnostic criteria and early experiences with its investigations and management. Retrospective case series of 44 canaliculi of 22 eyes of 11 patients presenting at a tertiary care Dacryology service over a period of 2 years with typical clinical patterns of inflammatory canaliculitis and its outcomes were studied. All the patients underwent microbiological work-up with culture and sensitivity, dacryoendoscopy imaging, serial Fourier domain ocular coherence tomography, and collagen vascular profiles. Stages in the evolution of the disease were studied. All patients were treated initially with topical steroids followed by punctal dilatation and placement of mini-monoka stents. Five patients in addition had a small biopsy from the inflamed portion of the vertical canaliculus. Stents were extubated at 6 weeks. Forty-four canaliculi were diagnosed to have idiopathic canalicular inflammatory disease during the study period. There was a female preponderance (81.8%, 9/11) and the mean age at presentation was 57 years. All patients presented with unilateral epiphora without any discharge, pain, or swelling. Collagen vascular profiles and screening for autoimmune diseases were negative. Clinical picture ranged from stages 1 to 5, consisting of edema, progressive centripetal vascularization, pouting of vascularized mucosa, membrane formation, and progressive scarring. The presentation begins in 1 eye and usually involves the other eye at a mean of 6 months. Ocular coherence tomography and dacryoendoscopy were of adjunctive value in the diagnosis. Histopathological examination was suggestive of a chronic inflammation. All patients had relentless progression to end-stage disease, although delayed significantly by steroids and monoka intubation. Idiopathic canalicular inflammatory disease has a distinct and typical clinical behavior and

Evaluating clinical librarian services: a systematic review.

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Brettle, Alison; Maden-Jenkins, Michelle; Anderson, Lucy; McNally, Rosalind; Pratchett, Tracey; Tancock, Jenny; Thornton, Debra; Webb, Anne

2011-03-01

  Previous systematic reviews have indicated limited evidence and poor quality evaluations of clinical librarian (CL) services. Rigorous evaluations should demonstrate the value of CL services, but guidance is needed before this can be achieved.   To undertake a systematic review which examines models of CL services, quality, methods and perspectives of clinical librarian service evaluations.   Systematic review methodology and synthesis of evidence, undertaken collaboratively by a group of 8 librarians to develop research and critical appraisal skills.   There are four clear models of clinical library service provision. Clinical librarians are effective in saving health professionals time, providing relevant, useful information and high quality services. Clinical librarians have a positive effect on clinical decision making by contributing to better informed decisions, diagnosis and choice of drug or therapy. The quality of CL studies is improving, but more work is needed on reducing bias and providing evidence of specific impacts on patient care. The Critical Incident Technique as part of a mixed method approach appears to offer a useful approach to demonstrating impact.   This systematic review provides practical guidance regarding the evaluation of CL services. It also provides updated evidence regarding the effectiveness and impact of CL services. The approach used was successful in developing research and critical appraisal skills in a group of librarians. © 2010 The authors. Health Information and Libraries Journal © 2010 Health Libraries Group.

The Infectious Diseases Society of America emerging infections network: bridging the gap between clinical infectious diseases and public health.

Science.gov (United States)

Pillai, Satish K; Beekmann, Susan E; Santibanez, Scott; Polgreen, Philip M

2014-04-01

In 1995, the Centers for Disease Control and Prevention granted a Cooperative Agreement Program award to the Infectious Diseases Society of America to develop a provider-based emerging infections sentinel network, the Emerging Infections Network (EIN). Over the past 17 years, the EIN has evolved into a flexible, nationwide network with membership representing a broad cross-section of infectious disease physicians. The EIN has an active electronic mail conference (listserv) that facilitates communication among infectious disease providers and the public health community, and also sends members periodic queries (short surveys on infectious disease topics) that have addressed numerous topics relevant to both clinical infectious diseases and public health practice. The article reviews how the various functions of EIN contribute to clinical care and public health, identifies opportunities to further link clinical medicine and public health, and describes future directions for the EIN.

Bronchoscopic examinations for evaluating chest abnormal shadows associated with hematological disease

International Nuclear Information System (INIS)

Nakayama, Masayuki; Bando, Masashi; Kobayashi, Akira; Yamasawa, Hideaki; Ohno, Shoji; Sugiyama, Yukihiko

2006-01-01

Hematological diseases cause various respiratory complications, but their differentiation only by blood tests and chest radiology is often difficult. To clarify the characteristics of respiratory complications associated with hematological diseases and the diagnostic usefulness of bronchoscopic examinations for these complications, we clinically evaluated mainly underlying diseases, chest radiological findings, and bronchoscopic findings in 31 patients in whom we performed bronchoscopy for chest abnormal shadows associated with hematological disease during the past 13-year period. Among hematological disease, leukemia was most frequently observed, followed by malignant lymphoma and myelodysplastic syndrome. The most frequently observed chest CT findings were localized consolidation and diffuse Ground-glass opacity. Bronchoscopic examinations provided a definitive diagnosis in 20 patients (64.5%), and the most frequent diagnosis was pulmonary invasion by neoplastic cells (7 patients). Pulmonary invasion by neoplastic cells showed various images, and transbronchial lung biopsy : TBLB was useful for definitive diagnosis. After consideration of the general condition of patients and the risk of complications, bronchoscopy including TBLB should be performed when possible. (author)

Clinical outcomes for young people with screening-detected and clinically-diagnosed rheumatic heart disease in Fiji.

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Engelman, Daniel; Mataika, Reapi L; Ah Kee, Maureen; Donath, Susan; Parks, Tom; Colquhoun, Samantha M; Carapetis, Jonathan R; Kado, Joseph H; Steer, Andrew C

2017-08-01

Echocardiographic screening is under consideration as a disease control strategy for rheumatic heart disease (RHD). However, clinical outcomes of young people with screening-detected RHD are unknown. We aimed to describe the outcomes for a cohort with screening-detected RHD, in comparison to patients with clinically-diagnosed RHD. A retrospective cohort study included all young people with screening-detected RHD in the Central Division of Fiji in the primary cohort. Screen-negative and clinically-diagnosed comparison groups were matched 1:1 to the primary cohort. Data were collected on mortality, clinical complications and healthcare utilisation from the electronic and paper health records and existing databases. Seventy participants were included in each group. Demographic characteristics of the groups were similar (median age 11years, 69% female, median follow-up 7years). There were nine (12.9%) RHD-related deaths in the clinically-diagnosed group and one (1.4%) in the screening-detected group (Incident Rate Ratio: 9.6, 95% CI 1.3-420.6). Complications of RHD were observed in 39 (55.7%) clinically-diagnosed cases, four (20%) screening-detected cases and one (1.4%) screen-negative case. There were significant differences in the cumulative complication curves of the groups (pFiji. The prognosis of clinically-diagnosed RHD remains poor, with very high mortality and complication rates. Further studies in other settings will inform RHD screening policy. Comprehensive control strategies are required for disease prevention. Copyright © 2017 Elsevier B.V. All rights reserved.

Adenosine stress cardiovascular magnetic resonance with variable-density spiral pulse sequences accurately detects coronary artery disease: initial clinical evaluation.

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Salerno, Michael; Taylor, Angela; Yang, Yang; Kuruvilla, Sujith; Ragosta, Michael; Meyer, Craig H; Kramer, Christopher M

2014-07-01

Adenosine stress cardiovascular magnetic resonance perfusion imaging can be limited by motion-induced dark-rim artifacts, which may be mistaken for true perfusion abnormalities. A high-resolution variable-density spiral pulse sequence with a novel density compensation strategy has been shown to reduce dark-rim artifacts in first-pass perfusion imaging. We aimed to assess the clinical performance of adenosine stress cardiovascular magnetic resonance using this new perfusion sequence to detect obstructive coronary artery disease. Cardiovascular magnetic resonance perfusion imaging was performed during adenosine stress (140 μg/kg per minute) and at rest on a Siemens 1.5-T Avanto scanner in 41 subjects with chest pain scheduled for coronary angiography. Perfusion images were acquired during injection of 0.1 mmol/kg Gadolinium-diethylenetriaminepentacetate at 3 short-axis locations using a saturation recovery interleaved variable-density spiral pulse sequence. Significant stenosis was defined as >50% by quantitative coronary angiography. Two blinded reviewers evaluated the perfusion images for the presence of adenosine-induced perfusion abnormalities and assessed image quality using a 5-point scale (1 [poor] to 5 [excellent]). The prevalence of obstructive coronary artery disease by quantitative coronary angiography was 68%. The average sensitivity, specificity, and accuracy were 89%, 85%, and 88%, respectively, with a positive predictive value and negative predictive value of 93% and 79%, respectively. The average image quality score was 4.4±0.7, with only 1 study with more than mild dark-rim artifacts. There was good inter-reader reliability with a κ statistic of 0.67. Spiral adenosine stress cardiovascular magnetic resonance results in high diagnostic accuracy for the detection of obstructive coronary artery disease with excellent image quality and minimal dark-rim artifacts. © 2014 American Heart Association, Inc.

Clinical evaluation of stress thallium spect in ischemic heart disease

International Nuclear Information System (INIS)

Sui, Osamu; Kimura, Nazuna; Soeki; Takeshi; Takeichi, Naoki; Shinohara, Hisanori; Tamura, Yoshiyuki; Fukuda, Nobuo

1997-01-01

Thallium SPECT was performed in patients with significant coronary artery stenosis, 67 cases were after maximal exercise and 74 cases were during coronary vasodilation induced by ATP (adenosine triphosphate) infusion. In patients suspected of angina pectoris, the sensitivity, specificity and predictive accuracy for detection of coronary artery disease (CAD) were 88%, 78% and 82% for exercise SPECT, and 100%, 72% and 84% for ATP SPECT studies, respectively. In patients with old myocardial infarction, these were 73%, 100% and 88% for exercise SPECT and 71%, 100% and 81% for ATP SPECT. These were 75%, 49% and 60% for treadmill exercise test in the patient group including both angina and myocardial infarction. For detection of diseased vessels, the diagnostic accuracy for left anterior descending artery and right coronary artery lesions was almost equal for ATP and exercise SPECT study, but ATP SPECT study was more sensitive than exercise SPECT study in detection of left circumflex artery lesions. ATP as well as exercise SPECT studies occasionally gave false positive results in patients with single-vessel disease. ATP as well as exercise SPECT studies underestimated the severity of multi-vessel disease. In general, the results of ATP SPECT imaging were highly concordant with the results of exercise SPECT imaging. ATP stress thallium SPECT imaging provided a safe and highly accurate diagnostic tool for detection of CAD. (author)

Recommendations for cerebrospinal fluid Alzheimer's disease biomarkers in the diagnostic evaluation of mild cognitive impairment

DEFF Research Database (Denmark)

Herukka, Sanna-Kaisa; Simonsen, Anja Hviid; Andreasen, Niels

2017-01-01

) patient counseling. The working group recommended using CSF AD biomarkers in the diagnostic workup of MCI patients, after prebiomarker counseling, as an add-on to clinical evaluation to predict functional decline or conversion to AD dementia and to guide disease management. Because of insufficient...... evidence, it was uncertain whether CSF AD biomarkers outperform imaging biomarkers. Furthermore, the working group provided recommendations for interpretation of ambiguous CSF biomarker results and for pre- and post-biomarker counseling....... impairment (MCI). The recommendations were developed by a multidisciplinary working group and based on the available evidence and consensus from focused group discussions for 1) prediction of clinical progression to Alzheimer's disease (AD) dementia, 2) cost-effectiveness, 3) interpretation of results, and 4...

[Advanced Parkinson's disease: clinical characteristics and treatment (part 1)].

Science.gov (United States)

Kulisevsky, J; Luquin, M R; Arbelo, J M; Burguera, J A; Carrillo, F; Castro, A; Chacón, J; García-Ruiz, P J; Lezcano, E; Mir, P; Martinez-Castrillo, J C; Martínez-Torres, I; Puente, V; Sesar, A; Valldeoriola-Serra, F; Yañez, R

2013-10-01

A large percentage of patients with Parkinson's disease (PD) develop motor fluctuations, dyskinesias, and severe non-motor symptoms within 3 to 5 years of starting dopaminergic therapy, and these motor complications are refractory to treatment. Several authors refer to this stage of the disease as advanced Parkinson's disease. To define the clinical manifestations of advanced PD and the risk factors for reaching this stage of the disease. This consensus document has been prepared by using an exhaustive literature search and by discussion of the contents by an expert group on movement disorders of the Sociedad Española de Neurología (Spanish Neurology Society), coordinated by two of the authors (JK and MRL). Severe motor fluctuations and dyskinesias, axial motor symptoms resistant to levodopa, and cognitive decline are the main signs in the clinical phenotype of advanced PD. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

Semantic Modeling for Exposomics with Exploratory Evaluation in Clinical Context

Directory of Open Access Journals (Sweden)

Jung-wei Fan

2017-01-01

Full Text Available Exposome is a critical dimension in the precision medicine paradigm. Effective representation of exposomics knowledge is instrumental to melding nongenetic factors into data analytics for clinical research. There is still limited work in (1 modeling exposome entities and relations with proper integration to mainstream ontologies and (2 systematically studying their presence in clinical context. Through selected ontological relations, we developed a template-driven approach to identifying exposome concepts from the Unified Medical Language System (UMLS. The derived concepts were evaluated in terms of literature coverage and the ability to assist in annotating clinical text. The generated semantic model represents rich domain knowledge about exposure events (454 pairs of relations between exposure and outcome. Additionally, a list of 5667 disorder concepts with microbial etiology was created for inferred pathogen exposures. The model consistently covered about 90% of PubMed literature on exposure-induced iatrogenic diseases over 10 years (2001–2010. The model contributed to the efficiency of exposome annotation in clinical text by filtering out 78% of irrelevant machine annotations. Analysis into 50 annotated discharge summaries helped advance our understanding of the exposome information in clinical text. This pilot study demonstrated feasibility of semiautomatically developing a useful semantic resource for exposomics.

Clinical evaluation of total IgE in tears of patients with allergic conjunctivitis disease using a novel application of the immunochromatography method.

Science.gov (United States)

Inada, Noriko; Shoji, Jun; Kato, Hiroshi; Kiely, Surayah; Mulyanto; Sawa, Mitsuru

2009-12-01

The determination of total IgE in tears is useful as a diagnostic tool in allergic conjunctivitis disease (ACD). We evaluated the efficacy of this diagnostic tool for ACD, which is a clinically applicable novel immunochromagraphic method to determine total IgE in tears. The subjects comprised 4 groups: 15 patients with vernal keratoconjunctivitis (VKC group), 8 patients with atopic keratoconjunctivitis (AKC group), 18 patients with allergic conjunctivitis (AC group), and 7 normal healthy volunteers as a control (control group). Tears were sampled using filter paper, and the total IgE in tears was determined by immunochromatography assay. Semiquantitative determination was carried out by examining the intensity of the colored line using an immunochromatoreader (IgE index). The relationship between IgE indices in tears and total IgE levels in serum or between IgE indices and the clinical scores of ACD was examined. The positive ratio obtained by this novel application of the immunochromatography assay was 38 of the 41 in the patients with ACD and none in the 7 controls. IgE indices for the VKC group, AKC group and AC group were 27.5 +/- 15.6, 19.8 +/- 15.8, and 4.0 +/- 3.1 (mean +/- SD), respectively. IgE indices in tears showed significant correlation with both total IgE levels in serum (P tears is a useful clinical tool to investigate ACD.

Rethinking dry eye disease: a perspective on clinical implications.

Science.gov (United States)

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

Clinical Syndromes Associated with Cardiovascular Diseases: A Review

Directory of Open Access Journals (Sweden)

Xing Sheng Yang, MD, PhD, FACC, FAHA

2017-02-01

Full Text Available In clinical practice, a variety of syndromes are associated with cardiovascular disease and have characteristic findings. Most of them are an autosomal dominant genetic disorder and have different types of cardiovascular abnormalities, including electrocardiographic conduction defects, arrhythmias, cardiomyopathy, vascular and valvular diseases, cardiac septal defects, and pulmonary problems. There is a growing need for physicians to pay more attention to these syndromes.

Clinical perspective of coronary computed tomographic angiography in diagnosis of coronary artery disease

International Nuclear Information System (INIS)

Chang, Hyuk-Jae; Chung, Namsik

2011-01-01

Since a 4-detector row coronary computed tomographic angiography (CCTA) was launched in 1998, CCTA has experienced rapid improvement of imaging qualities with the ongoing evolution of computed tomography (CT) technology. The diagnostic accuracy of CCTA to detect coronary artery stenosis is well established, whereas improvements are still needed to reduce the overestimation of coronary artery disease (CAD) and assess plaque composition. CCTA has been used to evaluate CAD in various clinical settings. For example, CCTA could be an efficient initial triage tool at emergency departments for patients with acute chest pain with low-to-intermediate risk because of its high negative predictive value. In patients with suspected CAD, CCTA could be a cost-effective alternative to myocardial perfusion imaging and exercise electrocardiogram for the initial coronary evaluation of patients with intermediate pre-test likelihood suspected CAD. However, in asymptomatic populations, there is a lack of studies that show an improved prognostic power of CCTA over other modalities. Therefore, the clinical use of CCTA to detect CAD for purposes of risk stratification in asymptomatic individuals should be discouraged. As CT technology evolves, CCTA will provide better quality coronary imaging and non-coronary information with lower radiation exposure. Future studies should cover these ongoing technical improvements and evaluate the prognostic power of CCTA in various clinical settings of CAD in large, well-designed, randomized trials. (author)

Occupational Lung Disease: Clinical-Pathological-Radiological Correlation

International Nuclear Information System (INIS)

Carrillo Bayona, Jorge Alberto; Rivera Bernal, Aura Lucia; Ojeda Paulina; Paez Garcia, Diana Sofia

2008-01-01

People are exposed to hundreds of substances daily, some of which may induce pulmonary injury. Occupational Lung Disease diagnosis requires 4 elements: Exposure to the harmful agent, adequate latency between exposure and beginning of the symptoms, syndrome with post-exposure abnormalities, and exclusion of other conditions which may otherwise explain signs and symptoms. Several occupational lung disease classifications based on structural or functional injury, type of agent, or both have been proposed. Generally, 5 groups are considered: Pneumoconiosis, hypersensitivity pneumonitis, toxic fumes exposure, asthma, and occupational lung infections. Conventional radiographs and in specific situations, CT, are crucial elements for the diagnosis of Occupational Lung Disease. In the patient with respiratory symptoms and altered imaging studies, the possibility of Occupational Lung Disease should be considered. Radiologist should be familiar the variety of substances that cause these entities and their radiological features. In this article Occupational Lung diseases are reviewed, including diagnostic criteria, classification, physiopathology, clinical and radiological manifestations as well as their corresponding histopathological features.

Evaluation of aortic regurgitation in congenital heart disease: value of MR imaging in comparison to echocardiography

Energy Technology Data Exchange (ETDEWEB)

Ley, Sebastian [German Cancer Research Centre (DKFZ), Department of Radiology (E010), Heidelberg (Germany); University Hospital, Department of Pediatric Radiology, Heidelberg (Germany); Eichhorn, Joachim; Ulmer, Herbert [University Hospital, Department of Pediatric Cardiology, Heidelberg (Germany); Ley-Zaporozhan, Julia [German Cancer Research Centre (DKFZ), Department of Radiology (E010), Heidelberg (Germany); University Hospital Mainz, Department of Radiology, Mainz (Germany); Schenk, Jens-Peter [University Hospital, Department of Pediatric Radiology, Heidelberg (Germany); Kauczor, Hans-Ulrich [German Cancer Research Centre (DKFZ), Department of Radiology (E010), Heidelberg (Germany); Arnold, Raoul [University Hospital, Department of Pediatric Cardiology, Heidelberg (Germany); University Hospital, Department of Pediatric Cardiology, Freiburg (Germany)

2007-05-15

Evaluation of the severity and the follow-up of aortic insufficiency (AI) are important tasks in paediatric cardiology. Assessment is based on clinical and echocardiographic (ECHO) findings such as the configuration of the valve and the regurgitation fraction (RF). The goal of this study was to evaluate MRI compared to ECHO for determination of clinical severity, valve morphology and RF. Thirty patients (age 3-27 years) with mild-to-severe AI were evaluated by clinical examination, ECHO (2-D and Doppler), and MRI at 1.5 T (2-D true-FISP cine short axis, phase-contrast flow in the ascending aorta). Both methods identified 13 bicuspid and 17 tricuspid valves. Good correlations between ECHO and cine MRI were found for ventricular mass, stroke volume, and ejection fraction. A good linear correlation was found for the RF determined by ECHO and phase-contrast MRI (r = 0.7). The RF was 6% in mild AI, 17% in moderate AI, and 30% in severe AI. The different severity groups showed significantly different RF and it was possible to discriminate between clinical severity grades (P = 0.01). ECHO and MRI showed good agreement in evaluating morphology and function of the left ventricle. The clinical severity of the disease can be evaluated correctly using MRI. (orig.)

Evaluation of aortic regurgitation in congenital heart disease: value of MR imaging in comparison to echocardiography

International Nuclear Information System (INIS)

Ley, Sebastian; Eichhorn, Joachim; Ulmer, Herbert; Ley-Zaporozhan, Julia; Schenk, Jens-Peter; Kauczor, Hans-Ulrich; Arnold, Raoul

2007-01-01

Evaluation of the severity and the follow-up of aortic insufficiency (AI) are important tasks in paediatric cardiology. Assessment is based on clinical and echocardiographic (ECHO) findings such as the configuration of the valve and the regurgitation fraction (RF). The goal of this study was to evaluate MRI compared to ECHO for determination of clinical severity, valve morphology and RF. Thirty patients (age 3-27 years) with mild-to-severe AI were evaluated by clinical examination, ECHO (2-D and Doppler), and MRI at 1.5 T (2-D true-FISP cine short axis, phase-contrast flow in the ascending aorta). Both methods identified 13 bicuspid and 17 tricuspid valves. Good correlations between ECHO and cine MRI were found for ventricular mass, stroke volume, and ejection fraction. A good linear correlation was found for the RF determined by ECHO and phase-contrast MRI (r = 0.7). The RF was 6% in mild AI, 17% in moderate AI, and 30% in severe AI. The different severity groups showed significantly different RF and it was possible to discriminate between clinical severity grades (P 0.01). ECHO and MRI showed good agreement in evaluating morphology and function of the left ventricle. The clinical severity of the disease can be evaluated correctly using MRI. (orig.)

Professional challenges and opportunities in clinical microbiology and infectious diseases in Europe.

Science.gov (United States)

Read, Robert C; Cornaglia, Giuseppe; Kahlmeter, Gunnar

2011-05-01

The two closely linked specialties of clinical microbiology and infectious diseases face important challenges. We report the consensus of clinical microbiologists and infectious disease physicians assembled by the European Society for Clinical Microbiology and Infectious Diseases. Both specialties have different training requirements in different European countries and are not universally recognised as professions. The specialties are rapidly evolving as they adapt to the changing demands within hospital practice, including the need to deal with emerging infections, rapidly increasing internationalisation, and immigration. Clinical microbiology needs to develop and master technological advances such as laboratory automation and an avalanche of new methods for rapid diagnostics. Simultaneously, the pressure for concentration, amalgamation, and out-sourcing of laboratory services is ever-increasing. Infectious disease physicians have to meet the professional challenge of subspecialisation and the continual need to find new niches for their skills. Despite these challenges, each of these specialties continues to thrive in Europe and will enjoy important opportunities over the next few years. The recently formed European Centre for Disease Prevention and Control in Stockholm, Sweden, will increase demands in areas of surveillance of infectious diseases and antimicrobial resistance on both specialties. Copyright © 2011 Elsevier Ltd. All rights reserved.

DIAGNOSTIC ASPECTS OF PAGET’S DISEASE OF BONE IN CLINICAL PRACTICE

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I. B. Bashkova

2017-01-01

Full Text Available Paget’s disease of bone (PDB is a chronic localized skeletal disease that belongs to a group of metabolic osteopathies and is characterized by impaired bone remodeling to form foci of increased bone resorption followed by replacement with an excessive amount of defective, less durable bone that is prone to deformities and pathologic fractures. The course of PDB shows three stages: rarefaction, compaction, and coarse-trabecular remodeling – each of which is characterized by certain clinical, biochemical, and radiological manifestations. The majority of the clinical manifestations of the disease are associated with skeletal injury. The disease is characterized by the appearance of bone and joint pain in case of secondary osteoarthritis, bone deformities, pathological fractures, hearing loss due to damage to the skull bones, etc. In many patients, the disease is asymptomatic and detected incidentally after finding a high serum alkaline phosphatase activity or during bone X-ray for any pathological processes, but it can be diagnosed fairly late in the development of complications, as shown in the clinical examples. A combination of clinical, biochemical, morphological data and radiological findings allows for a diagnosis. The use of bisphosphonates is the method of choice for the treatment of PDB. 

[Clinical application evaluation of Guidelines for Diagnosis and Treatment of Common Diseases of Otolaryngology in Traditional Chinese Medicine].

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Liu, Yu-Qi; Liu, Meng-Yu; Li, Chun; Shi, Nan-Nan; Wang, Yue-Xi; Wang, Li-Ying; Zhao, Xue-Yao; Kou, Shuang; Han, Xue-Jie; Wang, Yan-Ping

2017-09-01

This study is to assess the Guidelines for Diagnosis and Treatment of Common Diseases of Otolaryngology in Traditional Chinese Medicine in clinical application and provide evidence for further guideline revision. The assessment was divided into applicability assessment and practicability assessment. The applicability assessment based on questionnaire survey and the traditional Chinese medicine (TCM) practitioners were asked to independently fill the Questionnaire for Applicability Assessment on the Guidelines for Diagnosis and Treatment in Traditional Chinese Medicine. The practicability assessment was based on prospective case investigation and analysis method and the TCM practitioners-in-charge filled the Case Investigation Questionnaire for Practicability Assessment on the Guidelines for Diagnosis and Treatment in Traditional Chinese Medicine. The data were analyzed in descriptive statistics. 151 questionnaires were investigated for applicability assessment and 1 016 patients were included for practicability assessment. The results showed that 88.74% of them were familiar with the guidelines and 45.70% used them. The guidelines quality and related items were similar in applicability assessment and practicability assessment, and scored highly as more than 85.00% except the "recuperating and prevention". The results suggested that the quality of Guidelines for Diagnosis and Treatment of Common Diseases of Otolaryngology in Traditional Chinese Medicine was high and could better guide the clinical practice. The "recuperating and prevention" part should be improved and the evidence data should be included in future guideline revision, so that the clinical utilization rate could be increased. Copyright© by the Chinese Pharmaceutical Association.

[Clinical overview of auto-inflammatory diseases].

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Georgin-Lavialle, S; Rodrigues, F; Hentgen, V; Fayand, A; Quartier, P; Bader-Meunier, B; Bachmeyer, C; Savey, L; Louvrier, C; Sarrabay, G; Melki, I; Belot, A; Koné-Paut, I; Grateau, G

2018-04-01

Monogenic auto-inflammatory diseases are characterized by genetic abnormalities coding for proteins involved in innate immunity. They were initially described in mirror with auto-immune diseases because of the absence of circulating autoantibodies. Their main feature is the presence of peripheral blood inflammation in crisis without infection. The best-known auto-inflammatory diseases are mediated by interleukines that consisted in the 4 following diseases familial Mediterranean fever, cryopyrinopathies, TNFRSF1A-related intermittent fever, and mevalonate kinase deficiency. Since 10 years, many other diseases have been discovered, especially thanks to the progress in genetics. In this review, we propose the actual panorama of the main known auto-inflammatory diseases. Some of them are recurrent fevers with crisis and remission; some others evaluate more chronically; some are associated with immunodeficiency. From a physiopathological point of view, we can separate diseases mediated by interleukine-1 and diseases mediated by interferon. Then some polygenic inflammatory diseases will be shortly described: Still disease, Schnitzler syndrome, aseptic abscesses syndrome. The diagnosis of auto-inflammatory disease is largely based on anamnesis, the presence of peripheral inflammation during attacks and genetic analysis, which are more and more performant. Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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

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Chang Mo Moon

Full Text Available 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.

Clinical Prediction Models for Cardiovascular Disease: Tufts Predictive Analytics and Comparative Effectiveness Clinical Prediction Model Database.

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Wessler, Benjamin S; Lai Yh, Lana; Kramer, Whitney; Cangelosi, Michael; Raman, Gowri; Lutz, Jennifer S; Kent, David M

2015-07-01

Clinical prediction models (CPMs) estimate the probability of clinical outcomes and hold the potential to improve decision making and individualize care. For patients with cardiovascular disease, there are numerous CPMs available although the extent of this literature is not well described. We conducted a systematic review for articles containing CPMs for cardiovascular disease published between January 1990 and May 2012. Cardiovascular disease includes coronary heart disease, heart failure, arrhythmias, stroke, venous thromboembolism, and peripheral vascular disease. We created a novel database and characterized CPMs based on the stage of development, population under study, performance, covariates, and predicted outcomes. There are 796 models included in this database. The number of CPMs published each year is increasing steadily over time. Seven hundred seventeen (90%) are de novo CPMs, 21 (3%) are CPM recalibrations, and 58 (7%) are CPM adaptations. This database contains CPMs for 31 index conditions, including 215 CPMs for patients with coronary artery disease, 168 CPMs for population samples, and 79 models for patients with heart failure. There are 77 distinct index/outcome pairings. Of the de novo models in this database, 450 (63%) report a c-statistic and 259 (36%) report some information on calibration. There is an abundance of CPMs available for a wide assortment of cardiovascular disease conditions, with substantial redundancy in the literature. The comparative performance of these models, the consistency of effects and risk estimates across models and the actual and potential clinical impact of this body of literature is poorly understood. © 2015 American Heart Association, Inc.

Alzheimer´s disease: a clinical-practice-oriented review

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Luísa eAlves

2012-04-01

Full Text Available Investigation in the field of Alzheimer´s disease (AD, the commonest cause of dementia, has been very active in recent years and it may be difficult for the clinician to keep up with all the innovations and to be aware of the implications they have in clinical practice. The authors, thus, reviewed recent literature on the theme in order to provide the clinician with an updated overview, intended to support decision-making on aspects of diagnosis and management. This article begins to focus on the concept of AD and on its pathogenesis. Afterwards, epidemiology and non-genetic risk factors are approached. Genetics, including genetic risk factors and guidelines for genetic testing, are mentioned next. Recommendations for diagnosis of AD, including recently proposed criteria, are then reviewed. Data on the variants of AD is presented. First approach to the patient is dealt with next, followed by neuropsychological evaluation. Biomarkers, namely MRI, SPECT, FDG PET, PiB PET, CSF tau and Aβ analysis, as well as available data on their diagnostic accuracy, are also discussed. Factors predicting rate of disease progression are briefly mentioned. Finally, non-pharmacological and pharmacological treatments, including established and emerging drugs, are addressed.

Graves' disease: cost-effectiveness of clinical and radioiodine treatments

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Cruz junior, Antonio F.; Takahashi, Miriam H.; Albino, Claudio C.

2005-01-01

Full text: In this study, we set out to evaluate the costs and effectiveness of the two most used therapies in Graves' disease: antithyroid drugs (ATD) and radioiodine (RAI). Twenty-tree patients, 7 men and 16 women, with a mean age of 35.4 years, treated with ATD and 35 patients, 5 men and 30 women, mean age of 39.4 years, treated with RAI were studied. After 2 years receiving ATD, 21 patients achieved euthyroidism and 2 remained hyperthyroid. In the RAI group, 21 patients presented hypothyroidism and 13 became euthyroid. To calculate the costs of each therapy, we analysed the number of visits during this period, the laboratory data and the drugs needed, such as tiamazol and/or thyroxine. The group treated only with ATD needed a higher number of visits and laboratory measurements, with the mean total cost of U$ 791.65, while the RAI group spent a mean amount of U$ 366.44. Therefore, the costs of the RAI treatment were 53,7 % lower than clinical therapy with ATD. Conclusion: The present study demonstrates that RAI treatment has a lower cost than ATD, being very effective in controlling the hyperthyroidism of Graves' disease. (author)

Bandit strategies evaluated in the context of clinical trials in rare life-threatening diseases.

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Villar, Sofía S

2018-04-01

In a rare life-threatening disease setting the number of patients in the trial is a high proportion of all patients with the condition (if not all of them). Further, this number is usually not enough to guarantee the required statistical power to detect a treatment effect of a meaningful size. In such a context, the idea of prioritizing patient benefit over hypothesis testing as the goal of the trial can lead to a trial design that produces useful information to guide treatment, even if it does not do so with the standard levels of statistical confidence. The idealised model to consider such an optimal design of a clinical trial is known as a classic multi-armed bandit problem with a finite patient horizon and a patient benefit objective function. Such a design maximises patient benefit by balancing the learning and earning goals as data accumulates and given the patient horizon. On the other hand, optimally solving such a model has a very high computational cost (many times prohibitive) and more importantly, a cumbersome implementation, even for populations as small as a hundred patients. Several computationally feasible heuristic rules to address this problem have been proposed over the last 40 years in the literature. In this article we study a novel heuristic approach to solve it based on the reformulation of the problem as a Restless bandit problem and the derivation of its corresponding Whittle index rule. Such rule was recently proposed in the context of a clinical trial in Villar et al (2015). We perform extensive computational studies to compare through both exact value calculations and simulated values the performance of this rule, other index rules and simpler heuristics previously proposed in the literature. Our results suggest that for the two and three-armed case and a patient horizon less or equal than a hundred patients, all index rules are a priori practically identical in terms of the expected proportion of success attained when all arms start