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Case Reports
. 1992 Sep;86(3):275-9.
doi: 10.1111/j.1600-0404.1992.tb05085.x.

Carbamyl phosphate synthetase-1 deficiency discovered after valproic acid-induced coma

H B Verbiest  1 , J S Straver, J P Colombo, J C van der Vijver, T C van Woerkom
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Case Reports

Carbamyl phosphate synthetase-1 deficiency discovered after valproic acid-induced coma

H B Verbiest et al. Acta Neurol Scand. 1992 Sep.

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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