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J Pediatr Gastroenterol Nutr. 2006 Apr;42(4):416-8.

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

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  • 1Department of Gastroenterology and Metabolism, Poznan University of Medical Sciences, Poznan, Poland.


In progressive familial intrahepatic cholestasis type 2 (PFIC-2), severe steatorrhea is often documented. However, pancreatic exocrine secretion has not yet been studied. In 14 children with PFIC-2, pancreatic function was assessed using standard fecal tests. Normal fecal lipase concentrations excluded isolated lipase deficiency. No differences in fecal elastase-1 concentrations and chymotrypsin activities were detected between PFIC-2 patients with or without steatorrhea, nor between these patients and healthy subjects. In conclusion, pancreatic exocrine function in patients with PFIC-2 is normal. Steatorrhea observed in those patients is not related to pancreatic insufficiency.

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