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家族性地中海热中的淀粉样甲状腺肿:三例患者报告及文献综述

Amyloid goitre in familial Mediterranean fever: report on three patients and review of the literature.

作者信息

Altiparmak M R, Pamuk O N, Pamuk G E, Apaydin S, Ataman R, Serdengeçti K

机构信息

Cerrrahpaşa Medical Faculty, University of Istanbul, Istanbul, Turkey.

出版信息

Clin Rheumatol. 2002 Nov;21(6):497-500. doi: 10.1007/s100670200122.

Abstract

Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients.

摘要

家族性地中海热(FMF)是一种遗传性疾病,其最具威胁性的并发症是系统性淀粉样变性。在大多数继发于FMF的系统性淀粉样变性患者中,甲状腺可能无明显症状受累。然而,临床上可检测到的甲状腺肿大相当罕见,迄今为止,仅有9例继发于FMF淀粉样沉积的甲状腺肿大病例被报道。在我们中心定期随访的1100例FMF患者中,仅3例(0.27%)可检测到因淀粉样物质积聚导致的甲状腺肿大。在本报告中,我们总结了这些患者的临床和实验室特征。所有3例患者甲状腺功能均正常。1例患者因压迫症状接受了全甲状腺切除术,另外2例则出于美观目的接受了手术。在FMF高发国家,如土耳其,对于长期患有FMF的患者,应考虑甲状腺继发性淀粉样变性。

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