L-CARNITINE SUPPLEMENTATION IN SYSTEMIC CARNITINE DEFICIENCY

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منابع مشابه

Combined therapy with PPARalpha agonist and L-carnitine rescues lipotoxic cardiomyopathy due to systemic carnitine deficiency.

OBJECTIVE Peroxisome proliferator-activated receptors (PPAR) are ligand-activated transcription factors that belong to the nuclear hormone receptor superfamily and are key regulators of fatty acid oxidation (FAO) in the heart. Systemic carnitine deficiency (SCD) causes disorders of FAO and induces hypertrophic cardiomyopathy with lipid accumulation. We hypothesized that activation of PPARalpha ...

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l-Carnitine Supplementation in Recovery after Exercise

Given its pivotal role in fatty acid oxidation and energy metabolism, l-carnitine has been investigated as ergogenic aid for enhancing exercise capacity in the healthy athletic population. Early research indicates its beneficial effects on acute physical performance, such as increased maximum oxygen consumption and higher power output. Later studies point to the positive impact of dietary suppl...

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MR in children with L-carnitine deficiency.

PURPOSE To describe the MR imaging findings in five children with proved L-carnitine deficiency. METHODS MR imaging studies (five without contrast, two with contrast) were obtained in five children (mean age, 9 years) who presented with stroke symptoms and who proved to have L-carnitine deficiency as established by serum levels. RESULTS In three of five patients, infarctions were confined t...

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Systemic primary carnitine deficiency with hypoglycemic encephalopathy

Acute hypoglycemia in children is not an uncommon disease that can be encountered in the Emergency Department. Most cases of childhood hypoglycemia are caused by ketotic hypoglycemia due to missed meals. Often, hypoketotic hypoglycemia can also occur, which suggests hyperinsulinemia or a defect in fatty acid oxidation. Carnitine is essential for long chain fatty acids transfer into mitochondria...

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In the first experiment (Exp1), three consecutive breeding rounds were performed by two groups of six pigeon couples in order to study the impact of L-carnitine supplementation (80 mg x d(-1)) of parent pigeons on zootechnical performance. Both in the second and third experiments (Exp2, Exp3), one breeding round was performed by two groups of six pigeon couples to reveal the biochemical backgro...

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ژورنال

عنوان ژورنال: Pediatric Research

سال: 1984

ISSN: 0031-3998,1530-0447

DOI: 10.1203/00006450-198404001-01226