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[家族性地中海热中的滑膜炎]

[Synovitis in familial Mediterranean fever].

作者信息

Langer H E, Huth F, Behfar S, Zeidler H

出版信息

Dtsch Med Wochenschr. 1985 Nov 1;110(44):1695-700. doi: 10.1055/s-2008-1069072.

Abstract

Arthroscopy done on a 24-year-old turkish male with familial Mediterranean fever (FMF) and arthritis of the knee joint provided morphological data during the acute stage of FMF-arthritis. Main finding is a heavy granulocytic infiltration of the subsynovial stratum, similar to that seen in non-specific purulent inflammation, accompanied by marked ectasis and hyperaemia of the synovial vessels. In the microbiologically sterile synovial fluid cell counts and lactate values are found as in bacterial arthritis. The typical history and the characteristic course of the disease are indicators for the diagnosis. Serological, immunological and radiological findings are non-specific. The efficacy of prophylactic colchicine in symptomatic therapy could be verified but it is essential that the drug is taken regularly and that the patient is instructed accordingly. Investigation of other members of the family showed a high frequency of intermarriages and the presence of this autosomal-recessive inherited disease in three generations.

摘要

对一名患有家族性地中海热(FMF)和膝关节关节炎的24岁土耳其男性进行关节镜检查,提供了FMF-关节炎急性期的形态学数据。主要发现是滑膜下层有大量粒细胞浸润,类似于非特异性脓性炎症所见,同时伴有滑膜血管明显扩张和充血。在微生物学无菌的滑液中,细胞计数和乳酸值与细菌性关节炎相同。该疾病的典型病史和特征性病程是诊断的指标。血清学、免疫学和放射学检查结果均无特异性。预防性秋水仙碱在症状性治疗中的疗效得到了证实,但必须定期服药并相应地指导患者。对该家族其他成员的调查显示近亲结婚频率很高,且这种常染色体隐性遗传病在三代人中均有出现。

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