Treatment of carnitine deficiency
@article{Winter2004TreatmentOC, title={Treatment of carnitine deficiency}, author={Susan C. Winter}, journal={Journal of Inherited Metabolic Disease}, year={2004}, volume={26}, pages={171-180} }
Summary: Carnitine deficiency is a secondary complication of many inborn errors of metabolism. Pharmacological treatment with carnitine not only corrects the deficiency, it facilitates removal of accumulating toxic acyl intermediates and the generation of mitochondrial free coenzyme A (CoA). The United States Food and Drug Administration (US FDA) approved the use of carnitine for the treatment of inborn errors of metabolism in 1992. This approval was based on retrospective chart analysis of 90…
55 Citations
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Efficacy of levocarnitine treatment in children with very low free carnitine level differs according to pathogenesis of diseases, and the highest efficacy was observed in primary systemic carnitines deficiency.
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The lack of evidence both on the effectiveness and safety of carnitine supplementation and on the necessary dose and frequency to be prescribed means that the current prescribing practice should continue to be observed and monitored with care until further evidence is available.
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The purpose of this review is to summarize the role of carnitine in human nutrition and disease and highlight the major areas of research in this field.
Role of carnitine in disease
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- 2010
Clinical application of carnitine holds much promise in a range of neural disorders such as Alzheimer's disease, hepatic encephalopathy and other painful neuropathies and topical application in dry eye offers osmoprotection and modulates immune and inflammatory responses.
Reviewof carnitine in disease
- Medicine, Biology
- 2010
Clinical application of carnitine holds much promise in a range of neural disorders such as Alzheimer's disease, hepatic encephalopathy and other painful neuropathies and topical application in dry eye offers osmoprotection and modulates immune and inflammatory responses.
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Current knowledge of carnitine biology in health and disease is presented, including its established function as a carrier of activated fatty acids and activates acetate across the inner mitochondrial membrane.
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- 2005
The aim of this thesis work was to develop analytical tools for the determination of carnitine and acylcarnitines in biological fluids, and one developed assay was utilized for the follow up of a clinical study.
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This is the best known primary carnitine deficiency, first described by Engel and Angelhi* and so far recognized in about 30 patients. It is characterized by a pronounced weakness of muscles, coupled…
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It is apparent from the foregoing discussion that carnitine plays an essential role in human intermediary metabolism, but precisely the role of this amino acid in the biochemical and physiological events that participate in the pathogenesis of each disease must be determined.
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