PubMed ID:
22614108
Public Release Type:
Journal
Publication Year: 2012
Affiliation: Hospital for Sick Children, Toronto, Canada. binita.kamath@sickkids.ca
DOI:
https://doi.org/10.1097/MPG.0b013e31825eff61
Authors:
Piccoli DA,
Childhood Liver Disease Research and Education Network,
Sokol RJ,
Magee JC,
Kamath BM
Studies:
A Prospective Database of Infants With Cholestasis
,
Longitudinal Study of Genetic Causes of Intrahepatic Cholestasis
Alagille syndrome (ALGS) is an inherited multisystem disorder in which pancreatic insufficiency (PI) has been regarded a minor but important clinical manifestation. As part of a multicenter prospective study, 42 patients with ALGS underwent fecal elastase (FE) measurement to screen for exocrine PI. FE measurements were normal (>200 μg/g) in 40 (95%) and indeterminate (100-200 μg/g) in 2 (5%). As FE is the most reliable screen for PI, these data suggest that PI is not a prevalent problem in ALGS.