Cholestatic jaundice and congenital hypopituitarism.
OBJECTIVE
The prevalence of cholestatic jaundice as a presenting feature of congenital hypopituitarism is assessed.


METHODS
A retrospective case record analysis of the presenting features in all patients diagnosed as having congenital hypopituitarism between 1973-93.


RESULTS
Seven of the 20 patients with congenital hypopituitarism presented with cholestatic jaundice as the major initial manifestation of the disorder. Liver biopsy findings in three revealed intracellular bile pigment accumulation and variable giant cell formation.


CONCLUSIONS
Cholestatic jaundice was the major manifestation of congenital hypopituitarism in 35% of patients presenting in the neonatal or early infancy period.
Ellaway
C J
CJ
Ray Williams Institute of Paediatric Endocrinology, Diabetes and Metabolism, Royal Alexandra Hospital for Children, Camperdown, New South Wales, Australia.
Silinik
M
M
Cowell
C T
CT
Gaskin
K J
KJ
Kamath
K R
KR
Dorney
S
S
Donaghue
K C
KC
D017668:Age of Onset; D001654:Bile Pigments; D001706:Biopsy; D002779:Cholestasis / Q000209:etiology* / Q000628:therapy; D005260:Female; D015726:Giant Cells; D006801:Humans; D007018:Hypopituitarism / Q000150:complications / Q000151:congenital* / Q000175:diagnosis; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000150:complications; D008297:Male; D009517:New South Wales / Q000453:epidemiology; D015995:Prevalence; D012189:Retrospective Studies
D001654:Bile Pigments