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Pancreatic insufficiency is not a prevalent problem in Alagille syndrome
Binita M Kamath1, David A Piccoli, John C Magee
1Hospital for Sick Children, Toronto, Canada. binita.kamath@sickkids.ca
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.
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