Intestinal Enterokinase Deficiency Workup

Updated: Nov 28, 2017
  • Author: Agostino Nocerino, MD, PhD; Chief Editor: Carmen Cuffari, MD  more...
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Workup

Laboratory Studies

Determine proteolytic activity in patients with intestinal enterokinase deficiency before and after activation with exogenous enterokinase in a duodenal juice sample collected during a pancreozymin-secretin test.

Perform column chromatography of duodenal juice before and after activation with exogenous enterokinase.

Trypsin activity in duodenal fluid is low or absent; activity returns after the addition of enterokinase. Lipase and amylase activity levels are within reference ranges.

Assay enterokinase activity in duodenal juice and intestinal mucosa.

Enterokinase activity in duodenal fluid and mucosal homogenate is less than 10% of age-matched control values.

The human proenteropeptidase complementary DNA (cDNA) has been cloned, and the gene has been mapped. [8]