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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.

SUBMITTER: Gliwicz D 

PROVIDER: S-EPMC5066201 | biostudies-literature | 2016 Oct

REPOSITORIES: biostudies-literature

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Exocrine pancreatic function in children with Alagille syndrome.

Gliwicz Dorota D   Jankowska Irena I   Wierzbicka Aldona A   Miśkiewicz-Chotnicka Anna A   Lisowska Aleksandra A   Walkowiak Jarosław J  

Scientific reports 20161017


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.  ...[more]

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