Symptomatic hyperammonemia caused by a congenital portosystemic shunt.

Kitagawa, S; Gleason, W A; Northrup, H; Middlebrook, M R; Ueberschar, E · J Pediatr · 1992

case_report · Level V

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Abstract

A child with trisomy 21 had altered mental status and hyperammonemia at presentation and was found to have a congenital portosystemic shunt as a result of a congenital abnormality of the portal venous system. Anomalies of the portal venous system leading to portosystemic shunting, although they are infrequent, should be considered in the differential diagnosis of hyperammonemia.

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