Congenital Pancreatic Lipase Deficiency

Abstract

The purpose of this communication is to record two families, each consisting of two children. The four children have presented an identical clinical picture associated with a congenital severe reduction of pancreatic lipase, and have responded to persistent treatment with lipase given as pancreatin. A brief reference was made to Case 1 in the Charles West Lecture, delivered before the Royal College of Physicians of London in 1955 (Sheldon, 1955). The families are not related to each other. The parents are not consanguineous, and both are healthy. Also recorded is a fifth case, concerning a child who experienced a temporary reduction of pan-creatic lipase production following varicella.