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Renal handling of carnitine in children with carnitine deficiency and hyperammonemia associated with valproate therapy



Renal handling of carnitine in children with carnitine deficiency and hyperammonemia associated with valproate therapy



Journal of Pediatrics 109(1): 131-134



Free and acylcarnitine in serum and urine samples were measured in five patients with hyperammonemia associated with anticonvulsant therapy including sodium valproate, of whom three had a Reye-like syndrome. All had considerable reduction in serum free carnitine and slight increase of acylcarnitine concentrations, suggesting increased conversion of free to acylcarnitine by valproate administration. Urinary excretion of both free and acylcarnitine was increased, accompanied by depressed reabsorption of free carnitine and decreased acylcarnitine/free carnitine clearance ratio. These results indicate a decreased threshold for free carnitine. The combination of these several factors may be responsible for carnitine deficiency in patients with hyperammonemia taking valproate.

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Accession: 006310170

Download citation: RISBibTeXText

PMID: 3088240

DOI: 10.1016/s0022-3476(86)80592-3


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