Isolated co-lipase deficiency in two brothers.
Two normally developed Assyrian brothers with isolated pancreatic co-lipase deficiency are described. They presented at the age of 5-6 years with loose stools. They had steatorrhoea, and analysis of exocrine pancreatic enzymes in the small intestine showed co-lipase deficiency, while amylase, chymotrypsin, trypsin and lipase were normal. Intraduodenal infusion of purified co-lipase improved fat digestion measured by the triolein breath test. Their steatorrhoea diminished on treatment with enteric-coated pancreatic enzymes.
D001647:Bile Acids and Salts / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D003089:Colipases / Q000172:deficiency*; D006801:Humans; D007408:Intestinal Absorption; D007413:Intestinal Mucosa / Q000378:metabolism; D008297:Male; D008661:Metabolism, Inborn Errors / Q000235:genetics* / Q000378:metabolism; D010179:Pancreas / Q000201:enzymology; D011488:Protein Deficiency
D001647:Bile Acids and Salts; D003089:Colipases