Addisonian-like crisis in congenital hypopituitarism and cholestatic jaundice.
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.
Lee
W S
WS
Department of Paediatrics, University of Malaya Medical Centre, 50603, Kuala Lumpur.
Lum
L C
LC
Harun
F
F
D000224:Addison Disease / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D002779:Cholestasis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007018:Hypopituitarism / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D007223:Infant; D008297:Male