[Malnutrition in congenital deficiency of enterokynase (author's transl)]

A case of Congenital deficiency of Enterokynase is presented. The main clinical findings are: Chronic diarrhea, malnutrition and edema. Diagnosis was made by determination of tripsine activity in duodenal juice before and later to confront it to Enterokynase. The patient was treated with pancreatic supplements and protein hidrolysate with favourable results.