Pancreatic insufficiency is not a prevalent problem in Alagille syndrome

J Pediatr Gastroenterol Nutr. 2012 Nov;55(5):612-4. doi: 10.1097/MPG.0b013e31825eff61.

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.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Adult
  • Alagille Syndrome / complications*
  • Alagille Syndrome / metabolism
  • Biomarkers / metabolism
  • Child
  • Child, Preschool
  • Exocrine Pancreatic Insufficiency / epidemiology
  • Exocrine Pancreatic Insufficiency / etiology*
  • Exocrine Pancreatic Insufficiency / metabolism
  • Feces / chemistry*
  • Humans
  • Pancreatic Elastase / metabolism*
  • Prevalence
  • Prospective Studies
  • Young Adult

Substances

  • Biomarkers
  • Pancreatic Elastase

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