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  • Title: Carbamyl phosphate synthetase-1 deficiency discovered after valproic acid-induced coma.
    Author: Verbiest HB, Straver JS, Colombo JP, van der Vijver JC, van Woerkom TC.
    Journal: Acta Neurol Scand; 1992 Sep; 86(3):275-9. PubMed ID: 1414247.
    Abstract:
    Valproic acid induced coma is presented in an adult patient without a history of metabolic disease. Liver biopsy revealed a reduction in activity of carbamyl phosphate synthetase-I, an enzyme obligated for transformation of ammonia to urea in the urea cycle. After recovery CT scan follow-up showed marked cerebral atrophy which did not exist prior to the state of coma. Risk factors are discussed.
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