Carbamyl phosphate synthetase deficiency and postpartum hyperammonemia.

Kotani, Yasushi; Shiota, Mitsuru; Umemoto, Masahiko; Tsuritani, Mitsuhiro; Hoshiai, Hiroshi · Am J Obstet Gynecol · 2010

case_report · Level V

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Abstract

Carbamyl phosphate synthetase (CPS) is an enzyme that converts ammonia to carbamyl phosphate in the urea cycle. CPS deficiency is a genetic disorder that causes hyperammonemia because of enzyme activity deficiency. It is primarily diagnosed in neonates and infants and has a poor prognosis. We report an adult woman with CPS deficiency who developed hyperammonemia postpartum.

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