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阿拉伯人中的家族性地中海热(复发性遗传性多浆膜炎)——175例患者的研究及文献综述

Familial Mediterranean fever (recurrent hereditary polyserositis) in Arabs--a study of 175 patients and review of the literature.

作者信息

Barakat M H, Karnik A M, Majeed H W, el-Sobki N I, Fenech F F

出版信息

Q J Med. 1986 Sep;60(233):837-47.

PMID:3306755
Abstract

Recurrent hereditary polyserositis (RHP) or familial Mediterranean fever (FMF) is a chronic inherited illness of obscure aetiology. The disease is characterised by paroxysmal attacks of fever, peritonitis, pleuritis or arthritis, and predominantly affects Sephardic Jews, Arabs, Turks and Armenians. In this study, we report our 11-year experience of 175 Arab patients with this disease. As with other ethnic groups, the most common manifestation (93.7 per cent) was peritonitis. Arthritis (33.7 per cent) and pleurisy (32 per cent) were next in frequency. Adult patients in this series unlike those in other ethnic groups, rarely presented with arthritis. Similarly rare were amyloidosis, rashes, splenomegaly, hepatomegaly or lymphadenopathy. The aetiology of this disease is not clear but we suspect that abnormalities in catecholamine metabolism may be a factor in the pathogenesis.

摘要

复发性遗传性多浆膜炎(RHP)或家族性地中海热(FMF)是一种病因不明的慢性遗传性疾病。该病的特征为发热、腹膜炎、胸膜炎或关节炎的阵发性发作,主要影响西班牙裔犹太人、阿拉伯人、土耳其人和亚美尼亚人。在本研究中,我们报告了175例患此病的阿拉伯患者的11年治疗经验。与其他种族群体一样,最常见的表现(93.7%)是腹膜炎。关节炎(33.7%)和胸膜炎(32%)的发生率次之。该系列中的成年患者与其他种族群体的患者不同,很少出现关节炎。同样罕见的是淀粉样变性、皮疹、脾肿大、肝肿大或淋巴结病。该病的病因尚不清楚,但我们怀疑儿茶酚胺代谢异常可能是发病机制中的一个因素。

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