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多腺体自身免疫综合征(PGA):三例报告及文献综述

Polyglandular autoimmune (PGA) syndromes: report of three cases and review of the literature.

作者信息

Bunnag P, Rajatanavin R

机构信息

Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

出版信息

J Med Assoc Thai. 1994 Jun;77(6):327-33.

PMID:7869020
Abstract

Polyglandular autoimmune (PGA) syndrome is caused by autoimmune process in multiple endocrine glands. This usually results in endocrine gland hypofunction, except for the thyroid gland, in which both hyper or hypofunction may occur. The syndrome can be classified into two types, type I and type II, each with distinct clinical characteristics. We report three cases of PGA syndromes. The first patient had type I PGA syndrome, characterized by hypoparathyrodism, primary adrenal insufficiency and primary ovarian failure. She also had chronic mucocutaneous candidiasis, which is the distinct feature in this syndrome. The second patient had type II PGA syndrome, with primary adrenal insufficiency, Hashimoto's thyroiditis and primary ovarian failure. She also had widespread vitilgo. The last patient also had type II PGA syndrome. She had insulin dependent diabetes mellitus, Graves' disease and alopecia areata.

摘要

多腺体自身免疫(PGA)综合征是由多个内分泌腺的自身免疫过程引起的。这通常会导致内分泌腺功能减退,但甲状腺除外,甲状腺可能出现功能亢进或减退。该综合征可分为I型和II型,每种类型都有独特的临床特征。我们报告了3例PGA综合征病例。首例患者患有I型PGA综合征,其特征为甲状旁腺功能减退、原发性肾上腺皮质功能不全和原发性卵巢功能衰竭。她还患有慢性黏膜皮肤念珠菌病,这是该综合征的独特特征。第二例患者患有II型PGA综合征,伴有原发性肾上腺皮质功能不全、桥本甲状腺炎和原发性卵巢功能衰竭。她还患有广泛的白癜风。最后一例患者也患有II型PGA综合征。她患有胰岛素依赖型糖尿病、格雷夫斯病和斑秃。

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