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Publication Information

PubMed ID
Public Release Type
Journal
Publication Year
2012
Affiliation
Hospital for Sick Children, Toronto, Canada. binita.kamath@sickkids.ca
Authors
Childhood Liver Disease Research and Education Network, Kamath BM, Magee JC, Piccoli DA, Sokol RJ
Studies
Citation
Kamath BM, Piccoli DA, Magee JC, Sokol RJ, Childhood Liver Disease Research and Education Network. Pancreatic insufficiency is not a prevalent problem in Alagille syndrome. J Pediatr Gastroenterol Nutr 2012 Nov;55(5):612-4.

Abstract

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.