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Endothelial dysfunction in MELAS improved by l-arginine supplementation

From the Department of Pediatrics and Child Health (Y.K., Y.A., N.J., S.Y., N.P., R.F., T.M.), Kurume University School of Medicine, Fukuoka, Japan; Research Centre for Medical Genetics RAMNS (N.P.), Moscow, Russia; and Department of Pediatrics (M.I.), Kitasato University School of Medicine, Kanagawa, Japan.

Address correspondence and reprint requests to Dr. Yasutoshi Koga, Department of Pediatrics and Child Health, Kurume University School of Medicine, 67 Asahi-Machi, Kurume City, Fukuoka 830-0011, Japan; e-mail: yasukoga{at}med.kurume-u.ac.jp

The authors evaluated endothelial function in patients with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke) by flow-mediated vasodilation (FMD) and found a significant decrease vs controls. Two years of supplementation with oral l-arginine, a nitric oxide precursor, significantly improved endothelial function to control levels and was harmonized with the normalized plasma levels of l-arginine in patients. l-Arginine therapy improved endothelial dysfunction and showed promise in treating strokelike episodes in MELAS.

Supported in part by grants 13670853 (Y.K.) and 16390308 (Y.K.) from the Ministry of Culture and Education, Japan; grants H14-006 (Y.K.) and H14-team (syouni)-005 (Y.K.) from Evidence-based Medicine, Ministry of Health, Labor and Welfare, Japan; a research grant (17A-10) for nervous and mental disorders from the Ministry of Health, Labor and Welfare, Japan; and Uehara Memorial Foundation. Y.A. is a recipient of Grant-in-Aid for Young Investigators 17790733 from the Ministry of Culture and Education in Japan.

Disclosure: The authors report no conflicts of interest.

Received July 8, 2005. Accepted in final form February 20, 2006.

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