Pancreatic Insufficiency Is Not a Prevalent Problem in Alagille Syndrome | Zendy

Kamath Binita M. | Zendy; Piccoli David A. | Zendy; Magee John C. | Zendy; Sokol Ronald J. | Zendy

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Pancreatic 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.

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