+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

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.

Please choose payment method:

(PDF emailed within 0-6 h: $19.90)

Accession: 006310170

Download citation: RISBibTeXText

PMID: 3088240

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

Related references

Primary systemic carnitine deficiency 2. renal handling of carnitine. Neurology 31(7): 819-825, 1981

Renal handling of carnitine in secondary carnitine deficiency disorders. Pediatric Research 34(1): 89-97, 1993

The Effect of Carnitine Supplementation on Hyperammonemia and Carnitine Deficiency Treated with Valproic Acid in a Psychiatric Setting. Innovations in Clinical Neuroscience 12(9-10): 18-24, 2015

Carnitine acyl carnitine translocase deficiency; An important cause of hyperammonemia. Pediatric Research 45(4 Part 2): 135A, 1999

Effect of carnitine supplementation on valproate mediated transient hyperammonemia in children. Epilepsia 36(Suppl. 4): 125, 1995

Correlation between renal tubular dysfunction and serum carnitine deficiency in cases with valproate therapy. No to Hattatsu 41(5): 363-365, 2009

Carnitine supplementation for valproate-related hyperammonemia to maintain therapeutic valproate level. Journal of Clinical Psychopharmacology 31(5): 680-682, 2011

The risk of asymptomatic hyperammonemia in children with idiopathic epilepsy treated with valproate: relationship to blood carnitine status. Epilepsy Research 86(1): 32-41, 2009

Carnitine deficiency and hyperammonemia associated with valproic acid therapy. Journal of Pediatrics 101(5): 782-785, 1982

Valproate-associated hyperammonemia and DL-carnitine supplement. Kobe Journal of Medical Sciences 33(3): 81-87, 1987

Carnitine deficiency with valproate therapy. Journal of Pediatrics 103(1): 175-176, 1983

Effect of l carnitine on valproate induced hyperammonemia. Epilepsia 32(Suppl. 1): 79, 1991

Carnitine deficiency and hyperammonemia in children receiving valproic acid with and without other anticonvulsant drugs. International Journal of Clinical & Laboratory Research 29(1): 36-40, 1999

Diet- and valproate-induced transient hyperammonemia: effect of L-carnitine. Pediatric Neurology 16(4): 301-305, 1997

Carnitine Deficiency in Chinese Children with Epilepsy on Valproate Monotherapy. Indian Pediatrics 55(3): 222-224, 2018