title: Pancreatic insufficiency is not a prevalent problem in Alagille syndrome.
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.
authors.affiliation: Hospital for Sick Children, Toronto, Canada. binita.kamath@sickkids.ca
authors.forename: Binita M
authors.initials: BM
authors.lastname: Kamath
authors.forename: David A
authors.initials: DA
authors.lastname: Piccoli
authors.forename: John C
authors.initials: JC
authors.lastname: Magee
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Sokol
mesh_terms: D000293:Adolescent; D000328:Adult; D016738:Alagille Syndrome / Q000150:complications* / Q000378:metabolism; D015415:Biomarkers / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D010188:Exocrine Pancreatic Insufficiency / Q000453:epidemiology / Q000209:etiology* / Q000378:metabolism; D005243:Feces / Q000737:chemistry*; D006801:Humans; D010196:Pancreatic Elastase / Q000378:metabolism*; D015995:Prevalence; D011446:Prospective Studies; D055815:Young Adult
chemical_list: D015415:Biomarkers; D010196:Pancreatic Elastase