Familial Mediterranean fever: current perspectives

Hafize Emine Sönmez,* Ezgi Deniz Batu,* Seza ÖzenDepartment of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey *These authors contributed equally to this workAbstract: Familial Mediterranean fever (FMF) is the most frequent monogenic a...

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Autores principales:	Sönmez HE, Batu ED, Özen S
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2016
Materias:	familial Meditarranean fever recommendation child Pathology RB1-214 Therapeutics. Pharmacology RM1-950
Acceso en línea:	https://doaj.org/article/a3cca0b24e6f4dbc873fe5549e188dd3
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spelling	oai:doaj.org-article:a3cca0b24e6f4dbc873fe5549e188dd32021-12-02T09:18:32ZFamilial Mediterranean fever: current perspectives1178-7031https://doaj.org/article/a3cca0b24e6f4dbc873fe5549e188dd32016-03-01T00:00:00Zhttps://www.dovepress.com/familial-mediterranean-fever-current-perspectives-peer-reviewed-article-JIRhttps://doaj.org/toc/1178-7031Hafize Emine Sönmez,* Ezgi Deniz Batu,* Seza ÖzenDepartment of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey *These authors contributed equally to this workAbstract: Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disease, and it is characterized by recurrent attacks of fever and polyserositis. The disease is associated with mutations in the MEFV gene encoding pyrin, which causes exaggerated inflammatory response through uncontrolled production of interleukin 1. The major long-term complication of FMF is amyloidosis. Colchicine remains the principle therapy, and the aim of treatment is to prevent acute attacks and the consequences of chronic inflammation. With the evolution in the concepts about the etiopathogenesis and genetics of the disease, we have understood that FMF is more complicated than an ordinary autosomal recessive monogenic disorder. Recently, recommendation sets have been generated for interpretation of genetic testing and genetic diagnosis of FMF. Here, we have reviewed the current perspectives in FMF in light of recent recommendations.Keywords: familial Meditarranean fever, recommendation, childSönmez HEBatu EDÖzen SDove Medical Pressarticlefamilial Meditarranean feverrecommendationchildPathologyRB1-214Therapeutics. PharmacologyRM1-950ENJournal of Inflammation Research, Vol 2016, Iss Issue 1, Pp 13-20 (2016)
institution	DOAJ
collection	DOAJ
language	EN
topic	familial Meditarranean fever recommendation child Pathology RB1-214 Therapeutics. Pharmacology RM1-950
spellingShingle	familial Meditarranean fever recommendation child Pathology RB1-214 Therapeutics. Pharmacology RM1-950 Sönmez HE Batu ED Özen S Familial Mediterranean fever: current perspectives
description	Hafize Emine Sönmez,* Ezgi Deniz Batu,* Seza ÖzenDepartment of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey *These authors contributed equally to this workAbstract: Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disease, and it is characterized by recurrent attacks of fever and polyserositis. The disease is associated with mutations in the MEFV gene encoding pyrin, which causes exaggerated inflammatory response through uncontrolled production of interleukin 1. The major long-term complication of FMF is amyloidosis. Colchicine remains the principle therapy, and the aim of treatment is to prevent acute attacks and the consequences of chronic inflammation. With the evolution in the concepts about the etiopathogenesis and genetics of the disease, we have understood that FMF is more complicated than an ordinary autosomal recessive monogenic disorder. Recently, recommendation sets have been generated for interpretation of genetic testing and genetic diagnosis of FMF. Here, we have reviewed the current perspectives in FMF in light of recent recommendations.Keywords: familial Meditarranean fever, recommendation, child
format	article
author	Sönmez HE Batu ED Özen S
author_facet	Sönmez HE Batu ED Özen S
author_sort	Sönmez HE
title	Familial Mediterranean fever: current perspectives
title_short	Familial Mediterranean fever: current perspectives
title_full	Familial Mediterranean fever: current perspectives
title_fullStr	Familial Mediterranean fever: current perspectives
title_full_unstemmed	Familial Mediterranean fever: current perspectives
title_sort	familial mediterranean fever: current perspectives
publisher	Dove Medical Press
publishDate	2016
url	https://doaj.org/article/a3cca0b24e6f4dbc873fe5549e188dd3
work_keys_str_mv	AT soumlnmezhe familialmediterraneanfevercurrentperspectives AT batued familialmediterraneanfevercurrentperspectives AT oumlzens familialmediterraneanfevercurrentperspectives
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Familial Mediterranean fever: current perspectives

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