Late Onset Ornithine Transcarbamylase Deficiency Accompanying Severe HyperammonemiaAfter Cesarean Section: Case Report

Birsen Dogu^1, , Nezir Yılmaz², Sabriye Ozcekic² and Hafize Oksuz²

¹Marash Life Hospital, Kahramanmaras, Turkey

²Kahramanmaras Sutcu Imam University Medical School Department of Anesthesiology and Reanimation Kahramanmaras, Turkey

Cite this paper

Birsen Dogu, Nezir Yılmaz, Sabriye Ozcekic and Hafize Oksuz. Late Onset Ornithine Transcarbamylase Deficiency Accompanying Severe HyperammonemiaAfter Cesarean Section: Case Report. American Journal of Medical Case Reports. 2015; 3(5):133-136. doi: 10.12691/AJMCR-3-5-4

Abstract

Hyperammonemia is one of the common complications of porto-systemic shunt or liver failure. In patients without liver failure or porto-systemic shunt, hyperammonemia can be caused by urea cycle disorders. Nitrogen excretion pathway or enzyme deficiency disorder can result in any hyperammonemia, coma, or death that may cause severe clinical encephalopathy presenting with neurological symptoms. The most common cause of genetic disorders in the urea cycle is ornithine carbamoyltransferase deficiency. In this article, 28-year-old female patient who had late-onset ornithine carbamoyltransferase deficiency with severe acute hyperammonemia resulting coma and death after cesarian section is described. In addition, treatment of hyperammonemia including sodium benzoate, arginine, and hemodialysis were discussed.

Keywords

ornithine carbamoyltransferase deficiency, urea cycle disorders, hyperammonemia, hemodialysis

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

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