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Exocrine pancreatic function in children with progressive familial intrahepatic cholestasis type 2

Jaroslaw Walkowiak1, Irena Jankowska, Joanna Pawlowska

  • 1Department of Gastroenterology and Metabolism, Poznan University of Medical Sciences, Poznan, Poland. jarwalk@am.poznan.pl

Journal of Pediatric Gastroenterology and Nutrition
|April 28, 2006
PubMed

Insights

Progressive familial intrahepatic cholestasis type 2 (PFIC-2) patients have normal pancreatic exocrine function. Steatorrhea in PFIC-2 is not caused by pancreatic insufficiency, according to fecal tests in children.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Digestive Physiology

Background:

  • Progressive familial intrahepatic cholestasis type 2 (PFIC-2) is characterized by severe steatorrhea.
  • The role of pancreatic exocrine function in PFIC-2-associated steatorrhea remains uninvestigated.

Purpose of the Study:

  • To evaluate pancreatic exocrine function in children diagnosed with PFIC-2.
  • To determine if pancreatic insufficiency contributes to steatorrhea in PFIC-2.

Main Methods:

  • Standard fecal tests were employed to assess pancreatic function.
  • Fecal lipase, elastase-1 concentrations, and chymotrypsin activities were measured.
  • PFIC-2 patients with and without steatorrhea were compared to healthy controls.

Main Results:

  • Normal fecal lipase concentrations ruled out isolated lipase deficiency.
  • No significant differences in fecal elastase-1 or chymotrypsin were observed between PFIC-2 subgroups and controls.
  • These findings indicate preserved pancreatic exocrine secretion in PFIC-2.

Conclusions:

  • Pancreatic exocrine function is normal in children with PFIC-2.
  • Steatorrhea in PFIC-2 is not attributable to pancreatic insufficiency.

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