PMID: 3766902Jan 1, 1986Paper

Serum and urinary carnitine and organic acids in Reye syndrome and Reye-like syndrome

Brain & Development
T SugimotoY Sakane

Abstract

Free and acyl-carnitine in serum and urine, and urinary organic acids were measured in 6 patients with Reye syndrome and Reye-like syndrome. The free and total carnitine concentrations were significantly reduced in serum during the acute phases of the diseases. Thus, the ratio of acylcarnitine to free carnitine was significantly increased. Urinary excretion of acylcarnitine was greatly increased, and the acylcarnitine to total carnitine ratio was therefore greater than in controls. The urinary organic acids comprised large amounts of lactic acid, dicarboxylic acids and ketone bodies. It is suggested that carnitine deficiency is induced as more carnitine is consumed to buffer the increased amount of toxic acyl-CoA compounds metabolized from free fatty acids and the many organic acids. These results indicate that administration of L-carnitine should generally be considered in patients with Reye syndrome and Reye-like syndrome.

References

Nov 1, 1978·Annals of Neurology·J H WillnerS DiMauro
Jul 1, 1985·Neurology·D A StumpfC Angelini
Jan 1, 1983·Brain & Development·T SugimotoT Matsumura
May 16, 1984·Clinica Chimica Acta; International Journal of Clinical Chemistry·S Di DonatoG Uziel
Jun 1, 1984·The Journal of Clinical Investigation·C R RoeC L Hoppel

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Citations

May 1, 1988·European Journal of Pediatrics·E Schmidt-SommerfeldD Penn
Jan 1, 1990·Biochemical Pharmacology·I RozasS Rodriguez-Segade
Jan 1, 1995·Pharmacology & Therapeutics·B Fromenty, D Pessayre
Jan 1, 1995·Biomedicine & Pharmacotherapy = Biomédecine & Pharmacothérapie·A Michalak, I A Qureshi
Aug 1, 1990·Acta Paediatrica Japonica; Overseas Edition·C S ChiN Lin
Mar 1, 1987·Epilepsia·T SugimotoY Kobayashi

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