[Congenital hypopituitarism and giant cell hepatitis in a three month old girl].
METHODS
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.


CONCLUSIONS
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.
Reiterer
E E
EE
Universitätsklinik für Kinder- und Jugendheilkunde Graz, Osterreich, Germany. ElkeReiterer@kfunigraz.ac.at
Zenz
W
W
Deutsch
J
J
Preisegger
K H
KH
Simbrunner
J
J
Borkenstein
M H
MH
D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities / Q000473:pathology; D001656:Biliary Atresia / Q000175:diagnosis / Q000473:pathology; D001706:Biopsy; D003937:Diagnosis, Differential; D004652:Empty Sella Syndrome / Q000151:congenital / Q000175:diagnosis / Q000473:pathology; D005260:Female; D015726:Giant Cells / Q000473:pathology; D006505:Hepatitis / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D007018:Hypopituitarism / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D007223:Infant; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D008279:Magnetic Resonance Imaging; D010902:Pituitary Gland / Q000002:abnormalities / Q000473:pathology