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 radinuc...

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Bibliographic Details
Main Authors: Lee, Way Seah, Lum, L.C., Harun, F.
Format: Article
Language:English
Published: Malaysian Medical Association 2003
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Online Access:http://eprints.um.edu.my/10921/1/Addisonian-like_crisis_in_congenital_hypopituitarism.pdf
http://eprints.um.edu.my/10921/
http://www.e-mjm.org/2003/v58n2/Congenital_Hypopituitarism.pdf
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Institution: Universiti Malaya
Language: English
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Summary: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.