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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Faculty of Pharmacy, Department of Analytical Chemistry, Hacettepe University, Ankara 06230, Türkiye.
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Instituto de Biomedicina de Valencia, IBV-CSIC, Valencia, Spain.
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Department of Paediatrics I, Medical University of Innsbruck, Innsbruck, Austria.
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Department of Genetic and Metabolism,Children's Hospital,Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.
Objectives: To investigate genotype-phenotype characteristics and long-term prognosis of neonatal carbamoyl phosphate synthetase 1 (CPS1) deficiency among children through newborn screening in Zhejiang province.
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Ethyl Carbamate in Fermented Food Products: Sources of Appearance, Hazards and Methods for Reducing Its Content.
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Institute of Bioengineering, Research Center of Biotechnology of the Russian Academy of Sciences, 119071 Moscow, Russia.
Ethyl carbamate, the ethyl ester of carbamic acid, has been identified in fermented foods and alcoholic beverages. Since ethyl carbamate is a probable human carcinogen, reduction of its content is important for food safety and human health. In alcoholic beverages, ethyl carbamate is mostly formed from the reaction of ethanol with urea, citrulline and carbamyl phosphate during fermentation and storage.
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