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家族性地中海热所致的肾、胃及甲状腺淀粉样变性

Renal, gastric and thyroidal amyloidosis due to familial Mediterranean fever.

作者信息

Kavukçu S, Türkmen M, Eroğlu Y, Canda T, Yörükoğlu K, Iğci E, Büyükgebiz A

机构信息

Department of Pediatrics, Faculty of Medicine, Dokuz Eylül University, Izmir, Turkey.

出版信息

Pediatr Nephrol. 1997 Apr;11(2):210-2. doi: 10.1007/s004670050263.

Abstract

Chronic renal failure developed in a 10-year-old girl due to renal amyloidosis secondary to familial Mediterranean fever (FMF). During management of the chronic renal failure by hemodialysis and of FMF with colchicine, goiter and hypothroidism were observed. Thyroid fine-needle aspiration and gastric endoscopical biopsies, performed when recurrent abdominal pain could not be controlled, revealed amyloid deposits in both thyroid and gastric tissues. After 6 months' therapy with colchicine and levothyroxine, there was no significant change in the thyroid volume. This is the first case in which gastric amyloidosis secondary to FMF in childhood has been demonstrated. Patients with amyloidosis secondary to FMF who have thyroid enlargement and unexplained gastrointestinal symptoms despite adequate therapy should be evaluated with imaging studies and biopsy examinations.

摘要

一名10岁女孩因家族性地中海热(FMF)继发肾淀粉样变性而发展为慢性肾衰竭。在通过血液透析治疗慢性肾衰竭以及用秋水仙碱治疗FMF的过程中,观察到甲状腺肿和甲状腺功能减退。在反复出现的腹痛无法控制时进行的甲状腺细针穿刺和胃内镜活检显示,甲状腺和胃组织中均有淀粉样沉积物。在用秋水仙碱和左甲状腺素治疗6个月后,甲状腺体积没有明显变化。这是第一例证实儿童期FMF继发胃淀粉样变性的病例。对于FMF继发淀粉样变性且尽管进行了充分治疗仍有甲状腺肿大和无法解释的胃肠道症状的患者,应通过影像学检查和活检进行评估。

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