long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

a fatty acid oxidation disorder; patients may experience episodes of acute hypoketotic hypoglycemia (similar to that found in MCAD deficiency), cardiomyopathy, muscle weakness, and liver abnormalities.

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LCHAD deficiency

References in periodicals archive ?

Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency

Mito-conundrum: unraveling environmental effects on mitochondria

(35.) PRZYREMBEL, H; JAKOBS, C; IJLST, L; DE KLERK, J.B.C.; WANDERS, R.J.A; Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. J.

Patologia molecular de los errores hereditarios de la [beta]-oxidacion mitochondrial de los acidos grasos: alcances en el diagnostico y tratamiento

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatr Res 1991;29:406-11.

Metabolic, nutritional, iatrogenic, and artifactual sources of urinary organic acids: a comprehensive table

Acylcarnitines in fibroblasts of patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and other fatty acid oxidation disorders.

Accumulation of free 3-hydroxy fatty acids in the culture media of fibroblasts from patients deficient in long-chain L-3-hydroxyacyl-CoA dehydrogenase: a useful diagnostic aid

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528C mutation.

Improved stable isotope dilution-gas chromatography-mass spectrometry method for serum or plasma free 3-hydroxy-fatty acids and its utility for the study of disorders of mitochondrial fatty acid [beta]-oxidation

The long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Prog Clin Biol Res 1990;321:503-10.

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a severe fatty acid oxidation disorder.

Stability of long-chain and short-chain 3-hydroxyacyl-CoA dehyd rogenase activity in postmortem liver