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Nutrition Management of Fatty Acid Oxidation Disorders

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Nutrition Management of Inherited Metabolic Diseases

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Abstract

Long-Chain Fatty Acid Oxidation Disorders

  • Management of acute illness and the avoidance of prolonged fasting are important treatment strategies to minimize the reliance on fat as an energy source.

  • Nutrition management depends on the degree of disease severity; in the most severe forms, long-chain fat is limited to 10% of total energy intake.

  • Diets restricted in long-chain fat require monitoring of essential fatty acids and fat-soluble vitamins.

Medium-Chain Acyl-CoA Dehydrogenase Deficiency (MCAD)

  • Management of acute illness and the avoidance of prolonged fasting are important treatment strategies to minimize the reliance on fat as an energy source.

  • MCT should be avoided in MCAD, however, the restriction of other fats is not indicated, and a normal, healthy diet is recommended.

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Authors and Affiliations

  1. Met Ed Co, Boulder, CO, USA

    Fran Rohr

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  1. Fran Rohr
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Correspondence to Fran Rohr .

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Editors and Affiliations

  1. Children’s Hospital Colorado, University of Colorado Hospital, Aurora, CO, USA

    Laurie E. Bernstein

  2. Met Ed Co, Boulder, CO, USA

    Fran Rohr

  3. Molecular and Medical Genetics, Oregon Health & Science University, Portland, OR, USA

    Sandy van Calcar

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Rohr, F. (2022). Nutrition Management of Fatty Acid Oxidation Disorders. In: Bernstein, L.E., Rohr, F., van Calcar, S. (eds) Nutrition Management of Inherited Metabolic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-030-94510-7_23

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  • DOI: https://doi.org/10.1007/978-3-030-94510-7_23

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  • Print ISBN: 978-3-030-94509-1

  • Online ISBN: 978-3-030-94510-7

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