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Nutrition Studies in Long-Chain Fatty Acid Oxidation Disorders: Diet Composition and Monitoring

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Nutrition Management of Inherited Metabolic Diseases

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Abstract

In patients with long-chain fatty acid oxidation disorders (LCFAOD):

  • Energy requirements may differ from that of the normal population.

  • Diets high in lean sources of protein appear to increase lean body mass and decrease liver lipid content.

  • Plasma acylcarnitine profiles improve when dietary long-chain fat is restricted and MCT is supplemented.

  • MCT improves exercise tolerance.

  • Low-fat diets may cause deficiencies of essential fatty acids, DHA, and/or fat-soluble vitamins.

  • DHA supplementation improves retinal function in patients with LCHAD.

  • Lowering potentially toxic metabolites, the hydroxyacylcarnitines, in plasma slows the progression of retinopathy.

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Authors and Affiliations

  1. Department of Molecular and Medical Genetics, Oregon Health and Science University, 3180 SW Sam Jackson Park Road, Mail code L103, Portland, OR, 97239, USA

    Melanie Boyd Gillingham PhD, RD

Authors
  1. Melanie Boyd Gillingham PhD, RD
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Correspondence to Melanie Boyd Gillingham PhD, RD .

Editor information

Editors and Affiliations

  1. Clinical Genetics and Metabolism, Children’s Hospital Colorado, University of Colorado Denver – Anschutz Medical Campus, Aurora, Colorado, USA

    Laurie E. Bernstein

  2. Division of Genetics and Genomics, Boston Children’s Hospital, Boston, Massachusetts, USA

    Fran Rohr

  3. Clinical Genetics and Metabolism, University of Colorado Denver, Children’s Hospital Colorado, Aurora, Colorado, USA

    Joanna R. Helm

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Gillingham, M.B. (2015). Nutrition Studies in Long-Chain Fatty Acid Oxidation Disorders: Diet Composition and Monitoring. In: Bernstein, L., Rohr, F., Helm, J. (eds) Nutrition Management of Inherited Metabolic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-14621-8_23

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  • DOI: https://doi.org/10.1007/978-3-319-14621-8_23

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-14620-1

  • Online ISBN: 978-3-319-14621-8

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