儿童自身免疫性多内分泌腺病综合征临床分析  

The Clinical Features of Autoimmune Polyendocrine Gland Syndrome in Children

作　　者：石琳[1] 马小花 徐玲玲[1] 梁娟[1] 马娟[2] 赵银霞[1] SHI Lin;MA Xiaohua;XU Lingling;LIANG Juan;MA Juan;ZHAO Yinxia(Department of Pediatrics,the General Hospital of Ningxia Medical University,Yinchuan 750004,China;Ningxia Medical University,Yinchuan 750004,China)

机构地区：[1]宁夏医科大学总医院儿科,银川750004 [2]宁夏医科大学,银川750004

出　　处：《宁夏医科大学学报》2021年第2期194-198,共5页Journal of Ningxia Medical University

基　　金：宁夏自然科学基金(NZ17168)。

摘　　要：目的探讨儿童自身免疫性多内分泌腺病综合征(APS)的临床特征。方法回顾性分析2例APS患儿的临床资料,并结合文献复习总结其临床特点。结果 1例患儿7年前出现免疫性血小板减少性紫癜,5年后出现甲状腺肿大伴有自身免疫性甲状腺病,治疗期间出现甲状腺危象治疗无效死亡。1例患儿家族有近亲结婚病史,主要表现为甲状腺弥漫性肿大,伴有明显消瘦,明确自身免疫性甲状腺病合并有1型糖尿病。结论 APS同一患者先后或同时出现2种及以上内分泌腺自身免疫疾病。各种自身免疫性疾病及多种内分泌疾病的发生间隔时间长,临床表现复杂多变。APS易出现甲亢危象、糖尿病酮症酸中毒等急危重症,病死率高。Objective To explore the clinical features of autoimmune polyendocrine gland syndrome(APS)in children. Methods The clinical characteristics of APS were summarized by analyzing clinical data of 2 cases of APS retrospectively and reviewing published literature. Results Of the 2 female cases,1 developed immune thrombocytopenic purpura 7 years ago,and goiter developed 5 years later,accompanied with autoimmune thyroid disease. The patient died of thyroid crisis during treatment. Case 2,who has a family history of consanguineous marriage,was mainly manifested as diffuse goiter with obvious weight loss. She was diagnosed as autoimmune thyroid disease,accompanied with type 1 diabetes. Conclusion Two or more endocrine gland autoimmune diseases often develop successively or simultaneously in the same patient with APS. The occurrence interval of autoimmune diseases and endocrine diseases remains long and clinical manifestations are complicated and changeable. The morbidity and mortality of hyperthyroidism crisis,diabetic ketoacidosis and other critical diseases is high in patient of APS.

关 键 词：自身免疫性多内分泌腺病综合征 自身免疫性 1型糖尿病 

分 类 号：R58[医药卫生—内分泌]