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家族性地中海热——腹痛的一个并非不常见的病因。

Familial Mediterranean fever--a not so unusual cause of abdominal pain.

作者信息

Simon Anna, van der Meer Jos W M, Drenth Joost P H

机构信息

Department of Medicine, Division of General Internal Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands.

出版信息

Best Pract Res Clin Gastroenterol. 2005 Apr;19(2):199-213. doi: 10.1016/j.bpg.2004.11.009.

Abstract

Familial Mediterranean fever is a hereditary syndrome characterised by recurrent episodes of fever and serositis, resulting in pain in the abdomen, chest, joints and muscles. It is primarily diagnosed in people of Jewish, Arabic, Turkish or Armenian ancestry and is caused by mutations in the gene encoding for pyrin. Abdominal FMF attacks resemble the clinical presentation of 'acute abdomen', with severe abdominal pain and rigidity, but in FMF symptoms always resolve spontaneously. It is important to distinguish these regular pain episodes from small bowel obstruction due to adhesions to prevent life-threatening bowel strangulation. In most cases, colchicine will prevent new painful attacks. This seminar also discusses other causes of abdominal pain in FMF patients.

摘要

家族性地中海热是一种遗传性综合征,其特征为发热和浆膜炎反复发作,导致腹部、胸部、关节和肌肉疼痛。该病主要在犹太、阿拉伯、土耳其或亚美尼亚血统的人群中被诊断出来,由编码吡喃素的基因突变引起。腹部家族性地中海热发作类似于“急腹症”的临床表现,伴有严重腹痛和腹肌紧张,但在家族性地中海热中症状总会自行缓解。将这些规律性疼痛发作与因粘连导致的小肠梗阻区分开来很重要,以防止危及生命的肠绞窄。在大多数情况下,秋水仙碱可预防新的疼痛发作。本次研讨会还讨论了家族性地中海热患者腹痛的其他原因。

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