Konnataler Panhypopituitarismus und Riesenzellhepatitis bei einem 3 Monate alten Säugling

Autor: E. E. Reiterer, K.-H. Preisegger, J. Simbrunner, J. Deutsch, W. Zenz, M. H. Borkenstein
Rok vydání: 2002
Předmět:
Zdroj: Klinische Pädiatrie. 214:136-139
ISSN: 1439-3824
0300-8630
DOI: 10.1055/s-2002-30150
Popis: CASE REPORT 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. DISCUSSION 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.
Databáze: OpenAIRE