Reiterer E E, Zenz W, Deutsch J, Preisegger K H, Simbrunner J, Borkenstein M H
Universitätsklinik für Kinder- und Jugendheilkunde Graz, Osterreich, Germany.
Klin Padiatr. 2002 May-Jun;214(3):136-9. doi: 10.1055/s-2002-30150.
A three month old girl, with recurrent hypoglycemia and neonatal cholestasis, is reported. A metabolic disease could be excluded. The liver biopsy revealed giant cell hepatitis and intrahepatic bile duct hypoplasia. ACTH, Cortisol and hGH measured during hypoglycemia were low. Magnetic tomography (MR) of the brain showed an "empty sella". After beginning a replacement therapy with hydrocortisone, growth hormone and thyroxine there was no further episode of hypoglycemia. Transaminases and bilirubin levels normalized. The girl is in good condition, growth and development are normal.
Hypoglycemia is often the first sign in childrens with neonatal hypopituitarism. The association of liver disease and hypopituitarism has been documented in a few reports. The pathophysiological mechanism leading to the liver dysfunction is not well understood. The prognosis of neonatal hypopituitarism as well as the concomitant liver disease is good under sufficient replacement therapy.
本文报道了一名3个月大的女童，患有复发性低血糖症和新生儿胆汁淤积症。可排除代谢性疾病。肝脏活检显示巨细胞性肝炎和肝内胆管发育不全。低血糖发作时测得的促肾上腺皮质激素（ACTH）、皮质醇和生长激素（hGH）水平较低。脑部磁共振断层扫描（MR）显示“空蝶鞍”。在开始使用氢化可的松、生长激素和甲状腺素进行替代治疗后，未再出现低血糖发作。转氨酶和胆红素水平恢复正常。女童状况良好，生长发育正常。
低血糖往往是新生儿垂体功能减退患儿的首个症状。少数报告记录了肝病与垂体功能减退之间的关联。导致肝功能障碍的病理生理机制尚不清楚。在充分的替代治疗下，新生儿垂体功能减退以及并发的肝病预后良好。
