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Arginine therapy in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes

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dc.contributor.author Ikawa M.
dc.contributor.author Povalko N.
dc.contributor.author Koga Y.
dc.date.accessioned 2021-02-25T06:40:06Z
dc.date.available 2021-02-25T06:40:06Z
dc.date.issued 2020
dc.identifier.issn 1363-1950
dc.identifier.uri https://dspace.kpfu.ru/xmlui/handle/net/160950
dc.description.abstract © 2020 Lippincott Williams and Wilkins. All rights reserved. Purpose of reviewWe would like to inform clinicians that the systematic administration of oral and intravenous l-Arginine is therapeutically beneficial and clinically useful for patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), when they maintain plasma arginine concentration at least 168 μmol/l.Recent findingsMELAS is associated with endothelial dysfunction by decreased plasma l-Arginine, nitric oxide (NO), and cyclic guanosine monophosphate. Endothelial dysfunction is also evident using flow-mediated vasodilation measurement by high-resolution Doppler echocardiography in the forearm artery in patients with MELAS. l-Arginine is known to be an important precursor of NO to normalize the endothelial function in MELAS. In our clinical trial followed by 7 years follow-up study, the systematic administration of l-Arginine to patients with MELAS significantly improved the survival curve of patients compared with natural history. Maintaining plasma arginine concentration at least 168 μmol/l may prevent the ictuses through the putative pathophysiologic mechanism and optimal normalization of endothelial dysfunction.SummaryNeither death nor bedriddenness occurred during the 2-year clinical trials, and the latter did not develop during the 7-year follow-up despite the progressively neurodegenerative and eventually life-Threatening nature of MELAS. Therapeutic regimen of l-Arginine on MELAS may be beneficial and clinically useful for patient care with MELAS.
dc.relation.ispartofseries Current Opinion in Clinical Nutrition and Metabolic Care
dc.subject and stroke-like episodes (MELAS)
dc.subject encephalopathy
dc.subject endothelial dysfunction
dc.subject L-Arginine
dc.subject lactic acidosis
dc.subject mitochondrial myopathy
dc.subject stroke-like episodes
dc.subject therapeutic regimen
dc.title Arginine therapy in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes
dc.type Review
dc.relation.ispartofseries-issue 1
dc.relation.ispartofseries-volume 23
dc.collection Публикации сотрудников КФУ
dc.relation.startpage 17
dc.source.id SCOPUS13631950-2020-23-1-SID85075960779

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  • Публикации сотрудников КФУ Scopus [24551]
    Коллекция содержит публикации сотрудников Казанского федерального (до 2010 года Казанского государственного) университета, проиндексированные в БД Scopus, начиная с 1970г.

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