title: Exocrine pancreatic function in children with Alagille syndrome.
abstract: Alagille syndrome (AGS) is often associated with symptoms of maldigestion, such as steatorrhea, hypotrophy and growth retardation. Exocrine pancreatic insufficiency was proposed as the underlying cause. We aimed to assess the exocrine pancreatic function with the use of different methods in AGS patients. Concentrations of fecal elastase-1 (FE1) and fecal lipase (FL) activities were measured in 33 children with AGS. The C-mixed triglyceride breath test (MTBT) in a subgroup comprising 15 patients. In all patients studied, FE1 concentrations and FL activities were normal. Abnormal MTBT results were documented in 4 (26.7%) patients. The FE1 and FL levels in MTBT-positive and MTBT-negative children did not differ. The results of this research do not confirm the presence of exocrine pancreatic dysfunction in AGS patients. Routine screening for exocrine pancreatic insufficiency of this group of patients is not necessary.
authors.affiliation: The Children's Memorial Health Institute, Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Warsaw, Poland.
authors.forename: Dorota
authors.initials: D
authors.lastname: Gliwicz
authors.affiliation: The Children's Memorial Health Institute, Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Warsaw, Poland.
authors.forename: Irena
authors.initials: I
authors.lastname: Jankowska
authors.affiliation: The Children's Memorial Health Institute, Department of Biochemistry and Experimental Medicine, Warsaw, Poland.
authors.forename: Aldona
authors.initials: A
authors.lastname: Wierzbicka
authors.affiliation: Poznan University of Medical Sciences, Department of Pediatric Gastroenterology and Metabolic Diseases, Poznań, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Miśkiewicz-Chotnicka
authors.affiliation: Poznan University of Medical Sciences, Department of Pediatric Gastroenterology and Metabolic Diseases, Poznań, Poland.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Lisowska
authors.affiliation: Poznan University of Medical Sciences, Department of Pediatric Gastroenterology and Metabolic Diseases, Poznań, Poland.
authors.forename: Jarosław
authors.initials: J
authors.lastname: Walkowiak
mesh_terms: D000293:Adolescent; D016738:Alagille Syndrome / Q000150:complications* / Q000503:physiopathology; D001944:Breath Tests; D002648:Child; D002675:Child, Preschool; D004063:Digestion; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications* / Q000503:physiopathology; D005243:Feces; D006801:Humans; D007223:Infant; D008049:Lipase / Q000378:metabolism*; D009154:Mutation; D010179:Pancreas / Q000201:enzymology*; D010196:Pancreatic Elastase / Q000378:metabolism*; D045602:Steatorrhea; D014280:Triglycerides / Q000378:metabolism; D055815:Young Adult
chemical_list: D014280:Triglycerides; D008049:Lipase; D010196:Pancreatic Elastase