Intestinal Enterokinase Deficiency: A Newly-recognized Disorder of Protein Digestion

Abstract

Enterokinase is an intestinal enzyme necessary for the activation of pancreatic proteolytic zymogens. Two children with absence of enterokinase activity in the duodenal juice and intestinal mucosa are described. Both had diarrhoea from birth and failed to thrive; one developed hypoproteinaemic oedema. Oral administration of pancreatic extract resulted in prompt control of symptoms and acceleration of growth. A simple screening test for enterokinase deficiency is described.