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[家族性地中海热]

[Familial Mediterranean fever].

作者信息

Rupieper C, Heller K

机构信息

Kinderklinik Marienhospitals Witten.

出版信息

Klin Padiatr. 1991 Nov-Dec;203(6):458-61. doi: 10.1055/s-2007-1025474.

Abstract

Familial Mediterranean Fever is a genetically determined disease occurring predominantly in Arabs, Armenians, Jews and very rarely in Turks. It is characterized by moderately elevated body temperature and by abdominal, pulmonary or arthritic complaints. These symptoms are recurrent appearing at short intervals and persisting for not more than two days. Renal amyloidosis may be a complication. Thus, treatment should be continued for life. The pathomechanism of the disease is not clear. Colchicine has been shown to give good symptomatic relief. Surprisingly, the prolonged use of this mitotic poison is virtually devoid of untoward side effects, even in pregnancy and childhood.

摘要

家族性地中海热是一种由基因决定的疾病,主要发生在阿拉伯人、亚美尼亚人、犹太人中,在土耳其人中极为罕见。其特征为体温中度升高,并伴有腹部、肺部或关节方面的不适。这些症状呈间歇性反复出现,持续时间不超过两天。肾淀粉样变性可能是一种并发症。因此,治疗应持续终身。该疾病的发病机制尚不清楚。秋水仙碱已被证明能有效缓解症状。令人惊讶的是,即使在孕期和儿童期,长期使用这种有丝分裂毒素实际上也几乎没有不良副作用。

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