Open AccessPancreatic Insufficiency Is Not a Prevalent Problem in Alagille Syndrome
Author(s) -
Kamath Binita M.,
Piccoli David A.,
Magee John C.,
Sokol Ronald J.
Publication year - 2012
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0b013e31825eff61
Subject(s) - alagille syndrome , medicine , exocrine pancreatic insufficiency , gastroenterology , pancreas , 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.