fatty acid oxidation disorder


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fatty acid oxidation disorder

n. trastorno de oxidación de ácidos grasos, trastorno metabólico heredado de oxidación de ácidos grasos por el cual estos no pueden actuar sobre las grasas para transformarlas en energía.
References in periodicals archive ?
14) Although MCT-supplemented diet has been reported to prevent the development of cardiomyopathy and skeletal myopathy in fatty acid oxidation disorders, a recent study in VLCAD knock-out mice showed that long term MCT supplementation resulted in steatohepatitis and a phenotype like the metabolic syndrome, (15) and this could also be true in humans.
However, the predictive value of tandem MS metabolic screening using postmortem blood is high for the fatty acid oxidation disorders identified (4).
Sudden and unexpected neonatal death: a protocol for the postmortem diagnosis of fatty acid oxidation disorders.
Fatty acid oxidation disorders and sudden death: diagnosis of inborn errors of metabolism in sudden death cases by acylcarnitine analysis of postmortem bile.
Fatty acid oxidation disorders are a group of autosomal recessive genetic disorders characterized by metabolic deficiencies in which the body is unable to break down fatty acids into energy.
Current issues regarding treatment of mitochondrial fatty acid oxidation disorders.
By now, it should be well known that this approach is inappropriate for several fatty acid oxidation disorders, in particular very long-chain acyl-CoA dehydrogenase deficiency (3, 4).
13: Mechanisms of toxicity in fatty acid oxidation disorders
The 119 patients suffered from three main categories of IEM, namely; amino acids and urea cycle defects, organic acid disorders, and fatty acid oxidation disorders.
Biochemical diagnosis of fatty acid oxidation disorders by metabolite analysis of post mortem liver.
4) The TMS could identify about 30 different metabolic diseases belonging to aminoacidopathies, urea cycle disorders, organic acidemia, and fatty acid oxidation disorders by analyzing amino acids and acylcarnitine levels on DBS sample.
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