Intestinal enterokinase deficiency. A newly-recognized disorder of protein digestion

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 oedem...

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Bibliographic Details
Published in:Archives of disease in childhood 1970-10, Vol.45 (243), p.651-655
Main Authors: Tarlow, M J, Hadorn, B, Arthurton, M W, Lloyd, J K
Format: Article
Language:English
Subjects:
Diarrhea, Infantile - complications
Duodenum - enzymology
Edema - etiology
Endopeptidases
Female
Growth
Humans
Infant Nutrition Disorders - complications
Infant, Newborn
Intestinal Mucosa - enzymology
Intestinal Secretions - enzymology
Metabolism, Inborn Errors - complications
Metabolism, Inborn Errors - diagnosis
Metabolism, Inborn Errors - drug therapy
Pancreatic Extracts - administration & dosage
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Description
Summary: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.
ISSN:0003-9888
1468-2044
DOI:10.1136/adc.45.243.651