Severe Pancreatic Dysfunction But Compensated Nutritional Status in Monogenic Pancreatic Disease Caused by Carboxyl-Ester Lipase Mutations

Abstract

Pancreatic exocrine dysfunction in CEL-MODY is associated with severely reduced acinar and moderately reduced ductal dysfunction. Despite severely reduced exocrine pancreatic function, CEL-MODY patients revealed only minor signs of malnutrition.