Intestinal Enterokinase Deficiency in Pediatrics

Abstract: Congenital enteropeptidase deficiency (CEP), also known as enterokinase deficiency. CEP is an uncommon autosomal recessive genetic disorder mostly characterized by severe chronic diarrhoea after delivery, hypoproteinemia, and failure to grow. Enteropeptidase activity anticipated play a significant role in protein digestion. For growth appropriate development newborns with congenital lack of the enzyme, pancreatic enzyme replacement treatment or amino acid combination must be given. Only 13 cases insufficiency have been recorded since it was originally 1969. Couples should informed that prenatal screening option EKD has favourable prognosis. However, if patient born, parents aware feeding issue offer proper exocrine secretion medication. One research found all patients had diagnosed 25 years ago. Even when pancreatic-enzyme stopped, they appeared lead regular lives adults, free gastrointestinal issues normal body weight. This can explained fact trypsin, once liberated from its precursor, encourages additional trypsinogen activation positive-feedback manner. Better pharmaceutical preparations such enteric-coated minimicrospheres delayed-release capsules are used for better results maintains prevents breakdown stomach acidity. review aims summarise current knowledge pathophysiology, causes, Intestinal Enterokinase Deficiency Pediatrics