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.

Modeling plasticity and dysplasia of pancreatic ductal organoids derived from human pluripotent stem cells

“…The ductal compartment of the pancreas is the origin of various diseases. These range from common diseases, such as pancreatic ductal adenocarcinoma (PDAC) (Ferreira et al, 2017;Kopp et al, 2018), to rare diseases, such as the Alagille syndrome, which is characterized by ductal malfunction (Golson et al, 2009;Gliwicz et al, 2016). In vitro disease models for PDAC are of major clinical relevance (Boj et al, 2015;Frappart et al, 2020;Moreira et al, 2017), not least due to its dismal prognosis and recent predictions that PDAC will rank as the second most common cause of cancer-related deaths in the Western world by 2030 (Rahib et al, 2014).…”