HEPATIC HILAR AREA: THE PLATE SYSTEM

 The plate system consists of bile ducts and blood vessels
surrounded by a sheath that is continuous with Glisson’s
capsule, intrahepatically, and the hepatoduodenal
ligament, extrahepatically. This system also contains a
large number of lymphatics, nerves, and a small vascular
network. 
Couinaud  states that the bile ducts and
hepatic artery are located within the plate system, but
that the portal vein is covered with a separate sheath
of loose connective tissue, and that is why the plate
containing the extrahepatic bile duct and hepatic artery
can be easily separated from the portal vein. Three
plates are found in the hilar area: the hilar plate, the
cystic plate, and the umbilical plate

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A young boy presents with failure to thrive. Biochemical analysis of a duodenal aspirate after a meal reveals a deficiency of enteropeptidase. The levels of the foll digestive enzyme would be affected:

a. Amylase.
b. Pepsin.
c. Lactose.
d. Trypsin.


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Enterokinase deficiency

Congenital deficiency of this small intestine enzyme has been reported in a few children, resulting in the complete absence of pancreatic proteolytic activity; enterokinase is an essential activator of pancreatic trypsinogens.

The presentation is of infants that are ill from very early in life with severe diarrhoea and failure to thrive. Hypoproteinaemia is common in this condition and this may cause oedema. The tryptic activity in duodenal juice is missing while lipase and amylase are normal. In vitro the activity of the juice can be restored by the addition of enterokinase. The major result of this condition is malabsorption of protein although steatorrhoea has been reported.

The supplementation of the diet with pancreatic enzyme replacements restores normal digestive function.