Michele T M, Fleckenstein J, Sgrignoli A R, Thuluvath P J
Division of Gastroenterology, Johns Hopkins University Hospital, Baltimore, MD 21205.
Postgrad Med J. 1994 Feb;70(820):128-31. doi: 10.1136/pgmj.70.820.128.
Type I polyglandular autoimmune syndrome is characterized by the triad of hypoparathyroidism, Addison's disease and chronic mucocutaneous candidiasis. Chronic active hepatitis has been associated with this syndrome but its incidence and severity have not been well documented. We describe a sibship of two patients with type I polyglandular autoimmune syndrome who presented with autoimmune chronic active hepatitis. The first patient presented to us with advanced disease and died despite an emergent liver transplant, while the second patient responded to steroids. Autoimmune chronic active hepatitis, a major cause of mortality in this syndrome, can present without symptoms or physical signs of liver disease. We suggest periodic screening of liver enzymes in subjects with this syndrome.
I型多腺体自身免疫综合征的特征为甲状旁腺功能减退、艾迪生病和慢性黏膜皮肤念珠菌病三联征。慢性活动性肝炎与该综合征有关，但其发病率和严重程度尚无充分文献记载。我们描述了一对患有I型多腺体自身免疫综合征且出现自身免疫性慢性活动性肝炎的同胞患者。首例患者就诊时病情已很严重，尽管紧急进行了肝移植仍死亡，而第二例患者对类固醇治疗有反应。自身免疫性慢性活动性肝炎是该综合征的主要死亡原因，可在无肝病症状或体征的情况下出现。我们建议对患有该综合征的患者定期筛查肝酶。
