Ellaway C J, Silinik M, Cowell C T, Gaskin K J, Kamath K R, Dorney S, Donaghue K C
Ray Williams Institute of Paediatric Endocrinology, Diabetes and Metabolism, Royal Alexandra Hospital for Children, Camperdown, New South Wales, Australia.
J Paediatr Child Health. 1995 Feb;31(1):51-3. doi: 10.1111/j.1440-1754.1995.tb02914.x.
The prevalence of cholestatic jaundice as a presenting feature of congenital hypopituitarism is assessed.
A retrospective case record analysis of the presenting features in all patients diagnosed as having congenital hypopituitarism between 1973-93.
Seven of the 20 patients with congenital hypopituitarism presented with cholestatic jaundice as the major initial manifestation of the disorder. Liver biopsy findings in three revealed intracellular bile pigment accumulation and variable giant cell formation.
Cholestatic jaundice was the major manifestation of congenital hypopituitarism in 35% of patients presenting in the neonatal or early infancy period.
评估胆汁淤积性黄疸作为先天性垂体功能减退症的一种表现特征的患病率。
对1973年至1993年间所有被诊断为先天性垂体功能减退症患者的临床表现进行回顾性病例记录分析。
20例先天性垂体功能减退症患者中有7例以胆汁淤积性黄疸作为该疾病的主要初始表现。3例患者的肝脏活检结果显示细胞内胆汁色素积聚和不同程度的巨细胞形成。
在新生儿期或婴儿早期就诊的患者中，35%的先天性垂体功能减退症患者以胆汁淤积性黄疸为主要表现。
