Literature DB >> 3088240

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

I Matsuda, Y Ohtani, N Ninomiya.   

Abstract

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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Year:  1986        PMID: 3088240     DOI: 10.1016/s0022-3476(86)80592-3

Source DB:  PubMed          Journal:  J Pediatr        ISSN: 0022-3476            Impact factor:   4.406


  8 in total

Review 1.  Delineating the role of alterations in lipid metabolism to the pathogenesis of inherited skeletal and cardiac muscle disorders: Thematic Review Series: Genetics of Human Lipid Diseases.

Authors:  Harjot K Saini-Chohan; Ryan W Mitchell; Frédéric M Vaz; Teresa Zelinski; Grant M Hatch
Journal:  J Lipid Res       Date:  2011-11-07       Impact factor: 5.922

2.  Secondary renal Fanconi syndrome caused by valproate therapy.

Authors:  Toru Watanabe; Hideto Yoshikawa; Sawako Yamazaki; Yuki Abe; Tokinari Abe
Journal:  Pediatr Nephrol       Date:  2005-03-23       Impact factor: 3.714

Review 3.  Valproate-induced hyperammonemic encephalopathy.

Authors:  Alberto Verrotti; Daniela Trotta; Guido Morgese; Francesco Chiarelli
Journal:  Metab Brain Dis       Date:  2002-12       Impact factor: 3.584

4.  Heterozygotes for plasmalemmal carnitine transporter defect are at increased risk for valproic acid-associated impairment of carnitine uptake in cultured human skin fibroblasts.

Authors:  I Tein; S DiMauro; Z W Xie; D C De Vivo
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

5.  Short-term effects of administration of anticonvulsant drugs on free carnitine and acylcarnitine in mouse serum and tissues.

Authors:  M F Camiña; I Rozas; M Gómez; J M Paz; C Alonso; S Rodriguez-Segade
Journal:  Br J Pharmacol       Date:  1991-05       Impact factor: 8.739

6.  A case of carbamylphosphate synthetase-I deficiency associated with secondary carnitine deficiency--L-carnitine treatment of CPS-I deficiency.

Authors:  T Mori; A Tsuchiyama; K Nagai; M Nagao; K Oyanagi; S Tsugawa
Journal:  Eur J Pediatr       Date:  1990-01       Impact factor: 3.183

7.  Plasma and hepatic carnitine and coenzyme A pools in a patient with fatal, valproate induced hepatotoxicity.

Authors:  S Krähenbühl; G Mang; H Kupferschmidt; P J Meier; M Krause
Journal:  Gut       Date:  1995-07       Impact factor: 23.059

8.  Hyperammonemic encephalopathy caused by carnitine deficiency.

Authors:  Berkeley N Limketkai; Stephen D Zucker
Journal:  J Gen Intern Med       Date:  2007-12-13       Impact factor: 5.128

  8 in total

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