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By Fatih Kelesoglu ...
Tomohiro Koga, Kiyoshi Migita, Atsushi Kawakami
Familial Mediterranean fever (FMF) is the most common autoinflammatory hereditary disease characterized by self-limited attacks of fever and serositis. Although colchicine is the gold standard treatment for the attacks ∼10% of cases of FMF are resistant or intolerant to effective doses of colchicine. In such cases, however, there are increasing numbers of case reports or clinical trials treated by biologic agents which directly target the proinflammatory cytokines. Anti-interleukin-1 (IL-1) treatment has proven beneficial in improving the inflammation in terms of clinical manifestations and laboratory findings in clinical trials...
September 2016: Modern Rheumatology
Adem Kucuk, Mehmet Fatih Erol, Soner Senel, Emir Eroler, Havvanur Alparslan Yumun, Ali Ugur Uslu, Asiye Mukaddes Erol, Deniz Tihan, Ugur Duman, Tevfik Kucukkartallar, Yalcin Solak
BACKGROUND/AIMS: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by attacks of fever and diffuse abdominal pain. The primary concern with this presentation is to distinguish it from acute appendicitis promptly. Thus, we aimed to evaluate the role of neutrophil lymphocyte ratio (NLR) to leverage the differential diagnosis of acute FMF attack with histologically proven appendicitis. METHODS: Twenty-three patients with histologically confirmed acute appendicitis and 88 patients with acute attack of FMF were included in the study...
March 2016: Korean Journal of Internal Medicine
Burak Erer, Erkan Demirkaya, Seza Ozen, Tilmann Kallinich
The most dreaded complication of familial Mediterranean fever (FMF) is amyloidosis; controversy exists as to what acute phase reactant (APR) should be monitored in these patients. To analyze the best acute phase reactant for FMF follow-up to help guide physicians to decide on what APR parameter to use, we also attempted to define the best APR in predicting the complications of FMF, specifically the development of amyloidosis. Systematic review based on a sensitive search to capture studies that: (1) included FMF patients; (2) measured serum amyloid A (SAA), CRP (C-reactive protein), proteinuria, or ESR (erythrocyte sedimentation rate); (3) amyloidosis were the outcome measure; (4) sensitivity, specificity, predictive value, and other performance parameters could be calculated; and (5) had a longitudinal design...
April 2016: Rheumatology International
Seza Ozen, Erkan Demirkaya, Burak Erer, Avi Livneh, Eldad Ben-Chetrit, Gabriella Giancane, Huri Ozdogan, Illana Abu, Marco Gattorno, Philip N Hawkins, Sezin Yuce, Tilmann Kallinich, Yelda Bilginer, Daniel Kastner, Loreto Carmona
Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, but many rheumatologists are not well acquainted with its management. The objective of this report is to produce evidence-based recommendations to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF. A multidisciplinary panel, including rheumatologists, internists, paediatricians, a nurse, a methodologist and a patient representative, was assembled. Panellists came from the Eastern Mediterranean area, Europe and North America...
April 2016: Annals of the Rheumatic Diseases
Erkan Demirkaya, Cengizhan Acikel, Philip Hashkes, Marco Gattorno, Ahmet Gul, Huri Ozdogan, Turker Turker, Omer Karadag, Avi Livneh, Eldad Ben-Chetrit, Seza Ozen
OBJECTIVE: To develop widely accepted international severity score for children and adult patients with familial Mediterranean fever (FMF) that can be easily applied, in research and clinical practice. METHODS: Candidate severity criteria were suggested by several FMF expert physicians. After three rounds of Delphi survey, the candidate criteria, defined by the survey, were discussed by experts in a consensus meeting. Each expert brought data of clinical manifestations, laboratory findings and physician's global assessments (PGAs) of minimum 20 patients from their centres...
June 2016: Annals of the Rheumatic Diseases
Farhad Salehzadeh, Afshin Fathi
INTRODUCTION: Familial Mediterranean fever (FMF) is the most common auto-inflammatory disease with monogenic (MEditerranean FeVer -MEFV- gene) inherited pattern. It mainly affects ethnic groups living along the eastern Mediterranean Sea: Turks, Sephardic Jews, Armenians, and Arabs [1]. Today FMF is not rare disease in other Mediterranean ethnicities, such as Greeks, Italians, and Iranians. CASE REPORT: Here we report a child with complex allele mutations E148Q/V726A/R761H, whilst, whose mother showed E148Q/V726A and his father had R761H/wt in analysis...
August 2015: Medical Archives
Eldad Ben-Chetrit, Hasmik Hayrapetyan, Anna Yegiazaryan, Gohar Shahsuvaryan, Tamara Sarkisian
No abstract text is available yet for this article.
November 2015: Clinical and Experimental Rheumatology
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