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Case Reports
. 2010 Jul;203(1):e10-1.
doi: 10.1016/j.ajog.2010.03.032. Epub 2010 May 14.

Carbamyl phosphate synthetase deficiency and postpartum hyperammonemia

Yasushi Kotani  1 , Mitsuru Shiota, Masahiko Umemoto, Mitsuhiro Tsuritani, Hiroshi Hoshiai
Affiliations
Case Reports

Carbamyl phosphate synthetase deficiency and postpartum hyperammonemia

Yasushi Kotani et al. Am J Obstet Gynecol. 2010 Jul.

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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