以Addison病为首发表现的POEMS综合征的临诊应对  

Approach to the patient with POEMS syndrome first manifested as Addison's disease

作　　者：卢琳 王霖霞 邢小平 张聪丽[2] 李剑[2] Lu Lin;Wang Linxia;Xing Xiaoping;Zhang Congli;Li Jian(Department of Endocrinology,Key Laboratory of Endocrinology of National Health Commission,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院,北京协和医院内分泌科,国家卫生健康委员会内分泌重点实验室,100730 [2]北京协和医院血液科

出　　处：《中华内分泌代谢杂志》2018年第11期955-959,共5页Chinese Journal of Endocrinology and Metabolism

基　　金：中国医学科学院医学与健康科技创新工程项目(2017-12M-1-011).

摘　　要：本文报道1例以皮肤变黑、浮肿为主诉的原发性肾上腺皮质功能减退(Addison病)中年男性患者,同时合并高促性腺激素性性腺功能减退、甲状旁腺功能减退和亚临床甲状腺功能减退,此外患者还有皮肤增厚、多毛和多浆膜腔积液。经检查发现M蛋白和VEGF水平升高,进一步明确诊断为POEMS综合征。其肾上腺CT提示肾上腺正常。对于成年起病的多内分泌腺体功能异常,除了考虑自身免疫性多腺体综合(APS)以外,也需要考虑到POEMS综合征的可能性。A middle aged male presented with darkened skin and edema of lower extremities was reported.He was diagnosed as primary adrenocortical insufficiency (Addison's disease )accompanied with hypergonadotropic hypogonadism,hypoparathyroidism,and subclinical hypothyroidism after endocrinological workup.In addition,the patient also had thickened skin,hirsutism,and polyserositis.The diagnosis of POEMS syndrome was confirmed by elevated M protein and VEGF level.The image of CT showed normal adrenals.Besides the autoimmune polyglandular syndrome (APS),the possibility of POEMS syndrome in an adult patient with multiple endocrine dysfunction should be considered.

关 键 词：艾迪生病 POEMS综合征 多内分泌腺疾病 自身免疫性 

分 类 号：R586.1[医药卫生—内分泌]