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dc.contributor.authorYalçınkaya, Fatoş
dc.contributor.authorTurkish FMF Study Group.
dc.date.accessioned2020-03-24T08:40:37Z
dc.date.available2020-03-24T08:40:37Z
dc.date.issued2005-01-01
dc.identifier.citationTunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, Tutar E, Ozen S, Topaloglu R, Yilmaz E, Arici M, Bakkaloglu A, Besbas N, Akpolat T, Dinc A, Erken E; Turkish FMF Study Group.Medicine (Baltimore). Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. 2005;84(1):1-11. DOI: 10.1097/01.md.0000152370.84628.0ctr_TR
dc.identifier.urihttps://doi.org/10.1097/01.md.0000152370.84628.0ctr_TR
dc.identifier.urihttp://hdl.handle.net/20.500.12575/70755
dc.description.abstractFamilial Mediterranean fever (FMF) is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly non-Ashkenazi Jews, Armenians, Turks, and Arabs. Since a large proportion of all the FMF patients in the world live in Turkey, the Turkish FMF Study Group (FMF-TR) was founded to develop a patient registry database and analyze demographic, clinical, and genetic features. The cohort was composed of 2838 patients (mean age, 23.0 +/- 13.33 yr; range, 2-87 yr), with a male:female ratio of 1.2:1. There was a mean period of 6.9 +/- 7.65 years from disease onset to diagnosis; the period was about 2 years shorter for each decade since 1981. Ninety-four percent of patients were living in the central-western parts of the country; however, their familial origins (70% from the central-eastern and Black Sea regions) reflected not only the ongoing east to west migration, but also the historical roots of FMF in Turkey. Patients' clinical features included peritonitis (93.7%), fever (92.5%), arthritis (47.4%), pleuritis (31.2%), myalgia (39.6%), and erysipelas-like erythema (20.9%). Arthritis, arthralgia, myalgia, and erysipelas-like erythema were significantly more frequent (p < 0.001) among patients with disease onset before the age of 18 years. Genetic analysis of 1090 patients revealed that M694V was the most frequent mutation (51.4%), followed by M680I (14.4%) and V726A (8.6%). Patients with the M694V/M694V genotype were found to have an earlier age of onset and higher frequencies of arthritis and arthralgia compared with the other groups (both p < 0.001). In contrast to other reported studies, there was no correlation between amyloidosis and M694V homozygosity in this cohort. However, amyloidosis was still remarkably frequent in our patients (12.9%), and it was prevalent (27.8%) even among the 18 patients with a disease onset after age 40 years. Twenty-two patients (0.8%) had nonamyloid glomerular diseases. The high prevalence of vasculitides (0.9% for polyarteritis nodosa and 2.7% for Henoch-Schonlein purpura) and high frequency of pericarditis (1.4%) were striking findings in the cohort. Phenotype II cases (those patients with amyloidosis as the presenting or only manifestation of disease) were rare (0.3% or less). There was a high rate of a past diagnosis of acute rheumatic fever, which suggested a possible misdiagnosis in children with FMF presenting with recurrent arthritis. To our knowledge, this is the largest series of patients with FMF reported from 1 country. We describe the features of the disease in the Turkish population and show that amyloidosis is still a substantial problem.tr_TR
dc.language.isoentr_TR
dc.publisherWolters Kluwertr_TR
dc.relation.isversionof10.1097/01.md.0000152370.84628.0ctr_TR
dc.subjectFamilial Mediterranean fevertr_TR
dc.subjectTurkeytr_TR
dc.titleFamilial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study.tr_TR
dc.typeArticletr_TR
dc.relation.journalMedicinetr_TR
dc.contributor.departmentTıp Fakültesitr_TR
dc.contributor.authorID0000-0002-6467-7470tr_TR
dc.identifier.volume84tr_TR
dc.identifier.issue1tr_TR
dc.identifier.startpage1tr_TR
dc.identifier.endpage11tr_TR
dc.relation.publicationcategoryMakale - Uluslararası - Editör Denetimli Dergitr_TR
dc.identifier.issn/e-issn1536-5964


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