Chinese Medical E-ournals Database

Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition) ›› 2019, Vol. 15 ›› Issue (06): 612 -617. doi: 10.3877/cma.j.issn.1673-5250.2019.06.002

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Current research status of familial Mediterranean fever

Xue Li1, Min Shu1,(), Jianjun Deng1, Yinyue Li1   

  1. 1. Department of Pediatrics, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
  • Received:2019-06-02 Revised:2019-10-17 Published:2019-12-01
  • Corresponding author: Min Shu
  • About author:
    Corresponding author: Shu Min, Email:
  • Supported by:
    Scientific Research Project of Science and Technology Department of Sichuan Province(2017JY275)

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder (AID) associated with mutations in MEVF gene. FMF patients are characterized by recurrent self-limited fever and multiple hydrohymenitis, such as peritonitis, pleurisy, pericarditis, arthritis, etc.. FMF is common in the Arab, Turkish, Jewish and Iranian Mediterranean races, but it has been gradually found in other races in recent years. For example, in the past few years, FMF patients have been found in adults and children in Chinese. There are many diseases causing fever. For children with recurrent periodic fever, besides considering endocrine diseases and immune diseases, FMF should also be considered. The current effective drug for FMF is colchicine. At present, most of the studies on FMF are aimed at adult patients, including diagnostic criteria, treatment and so on. This article will summarize diagnostic criteria and doses of therapeutic drugs for children with FMF, and elaborate the pathogenesis, clinical manifestation, classification and general laboratory examinations of FMF, the types of gene mutation and its detection, diagnostic criteria and treatment results in the latest research progresses, aiming to provide a basis for early diagnosis and early treatment of this disease.

表1 儿童家族性地中海热的新诊断标准中的主要临床症状及其体征
表2 不同年龄家族性地中海热患者接受秋水仙碱治疗的剂量(±s)
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