Affiliations 

  • 1 W S Lee, MRCP. Department of Paediatrics, University of Malaya Medical Centre, 50603, Kuala Lumpur
  • 2 L C S Lum, MRCP. Department of Paediatrics, University of Malaya Medical Centre, 50603, Kuala Lumpur
  • 3 F Harun, MRCP. Department of Paediatrics, University of Malaya Medical Centre, 50603, Kuala Lumpur
Med J Malaysia, 2003 Jun;58(2):279-81.
PMID: 14569750

Abstract

A six-week-old male infant was admitted for investigation of cholestasis and pale stools. He became lethargic and apnoeic with prolonged seizures after a percutaneous liver biopsy. Subsequent investigations showed conjugated hyperbilirubinaemia, elevated liver enzymes, and hypoglycaemia. The radinuclide hepatobiliary scintigraphy was non-excretory. After an operative cholangiogram, the infant developed Addisonian-like crisis with bradycardia, hypotension, respiratory distress, metabolic acidosis, hypoglycaemia, hyponatraemia, and hyperkalaemia. Blood investigations confirmed congenital hypopituitarism. Hormone replacement therapy with L-thyroxine and cortisone acetate resulted in dissolution of jaundice and the reduction of the liver size.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.