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Metabolic Effects of Carnitine Medication in a Patient with Multiple Acyl-CoA Dehydrogenation Deficiency

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Inherited Disorders of Vitamins and Cofactors

Abstract

In 1982 we described a patient with riboflavin responsive multiple acyl-CoA dehydrogenation deficiency (Gregersen et al., 1982). Free carnitine concentration in plasma was 6 μmol l-1 (control 26–74 μmol l-1) and esterified carnitine was 32μmoll-1 (control 17–32 mol l-1). It was suggested that the low carnitine was caused by a sequestration of free carnitine as short and medium chain acylcarnitines.

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References

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G. M. Addison K. Bartlett R. A. Harkness R. J. Pollitt

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© 1985 SSIEM and MTP Press Limited

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Gregersen, N., Christensen, M.F., Kølvraa, S. (1985). Metabolic Effects of Carnitine Medication in a Patient with Multiple Acyl-CoA Dehydrogenation Deficiency. In: Addison, G.M., Bartlett, K., Harkness, R.A., Pollitt, R.J. (eds) Inherited Disorders of Vitamins and Cofactors. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-8019-1_42

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  • DOI: https://doi.org/10.1007/978-94-011-8019-1_42

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-011-8021-4

  • Online ISBN: 978-94-011-8019-1

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