Maximal daily dose of pancreatic enzyme replacement therapy in infants with cystic fibrosis: a reconsideration

Abstract

The current recommendations for dosing of pancreatic enzyme replacement therapy (PERT) in infants with cystic fibrosis (CF) were made using a limited evidence base. The per meal recommended dose was extrapolated from dosing guidelines for older patients into a maximal daily dose for infants. We discuss why this maximal daily dose recommendation may be insufficient for young infants with CF, although the optimal dose of PERT for infants with CF remains unknown.

Keywords: Cystic fibrosis; Infant nutrition; Pancreatic enzymes; Pancreatic insufficiency.