Familial mediterranean fever / 中华实用儿科临床杂志

ABSTRACT

Familial mediterranean fever (FMF) is a hereditary inflammatory disorder,which caused by mutations in MEFV gene located on short arm of Chromosome 16 p13.3.The majority of patients onset before the age of 10 years,which is characterized clinically by the episodes of inflammatory reaction and serositis,including fever,peritonitis,synovitis and pleurisy.AA-amyloidosis with kidney failure is the most important complication.The children patients were diagnosed mainly on clinical evaluation and the criteria developed by Yal(c)inkaya.The ultimate goal of treatment in FMF is to obtain complete control of unprovoked attacks and minimise subclinical inflammation in between attacks.The only agent that decreases the frequency and severity of the episodes and the development of amyloidosis is colchicine,which should be startcd as soon as a clinical diagnosis is made,alternative biological treatments such as IL-1 receptor antagonist are indicated in patients with non-responders or resistant to colchicine.