Familial Mediterranean fever-associated diseases in children

dc.contributor.authorOzcakar, Z. B.
dc.contributor.authorCakar, N.
dc.contributor.authorUncu, N.
dc.contributor.authorCelikel, B. A.
dc.contributor.authorYalcinkaya, F.
dc.date.accessioned2020-06-25T18:23:01Z
dc.date.available2020-06-25T18:23:01Z
dc.date.issued2017
dc.departmentKırıkkale Üniversitesi
dc.descriptionOzcakar, Zeynep/0000-0002-6376-9189; Cakar, Nilgun/0000-0002-1853-0101
dc.description.abstractBackground: MEditerranean FeVer (MEFV) gene encodes for the pyrin protein and a mutated pyrin is associated with a prolonged or augmented inflammation. Hence, various diseases were reported to be associated with familial Mediterranean fever (FMF) or carriers of MEFV mutations. However, systematic evaluation of all associated diseases in children with FMF has not been done previously. Aim: The aim of this study was to investigate the frequency and type of FMF-associated diseases in children. Design and Methods: Files of FMF patients who had been seen in two reference hospitals in Ankara, in the last two years, were retrospectively evaluated. Patients with FMF and concomitant diseases were included to the study. Results: Among 600 FMF patients, 77 were found to have a concomitant disease (12.8%). Thirty patients (5%) had vasculitis; 21 (3.5%) had juvenile idiopathic artritis (JIA); 7 (1.16%) had inflammatory bowel disease (IBD) and 19 had other diseases including 5 patients with isolated sacroiliitis. Overall, 13 (2.17%) patients had sacroiliitis in our cohort. The most frequent mutation was M694V/M694V (44%) and 81% of the patients had at least one M694V mutation. Majority of the patients (74%) developed associated diseases while they were not receiving colchicine therapy. Conclusions: Certain inflammatory diseases including vasculitis, chronic arthritis and IBD were more frequently detected in patients with FMF during childhood. M694V mutation is a susceptibility factor for associated diseases. In countries where FMF is prevalent, clinicians dealing with FMF and other inflammatory diseases should be aware of these associations.en_US
dc.identifier.citationZ.B. Özçakar and others, Familial Mediterranean fever-associated diseases in children, QJM: An International Journal of Medicine, Volume 110, Issue 5, May 2017, Pages 287–290.en_US
dc.identifier.doi10.1093/qjmed/hcw230
dc.identifier.endpage290en_US
dc.identifier.issn1460-2725
dc.identifier.issn1460-2393
dc.identifier.issue5en_US
dc.identifier.pmid28040706
dc.identifier.scopus2-s2.0-85020262386
dc.identifier.scopusqualityQ1
dc.identifier.startpage287en_US
dc.identifier.urihttps://doi.org/10.1093/qjmed/hcw230
dc.identifier.urihttps://hdl.handle.net/20.500.12587/6985
dc.identifier.volume110en_US
dc.identifier.wosWOS:000402854700006
dc.identifier.wosqualityQ1
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherOxford Univ Pressen_US
dc.relation.ispartofQjm-An International Journal Of Medicine
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleFamilial Mediterranean fever-associated diseases in childrenen_US
dc.typeArticle

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