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Protein Requirements in Inherited Metabolic Diseases

  • Chapter
Nutrition Management of Inherited Metabolic Diseases

Abstract

  • Protein is a critical part of the diet in individuals with inherited metabolic diseases (IMD).

  • Current Dietary Reference Intakes may underestimate protein needs for individuals with IMD.

  • Low “whole” protein diets without the use of amino acid-based medical foods may not contain sufficient protein for majority of individuals with an inherited metabolic disease.

  • Distributing protein intake throughout the day facilitates anabolism.

  • Protein requirements for catch-up growth should be calculated as adjusted weight for age and not actual weight.

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  1. Medical and Scientific Affairs, Nutricia North America, 9900 Belward Campus Drive, Suite 100, Rockville, MD, 20850, USA

    Steven Yannicelli PhD, RD

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  1. Steven Yannicelli PhD, RD
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Correspondence to Steven Yannicelli PhD, RD .

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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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Yannicelli, S. (2015). Protein Requirements in Inherited Metabolic Diseases. 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_7

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  • DOI: https://doi.org/10.1007/978-3-319-14621-8_7

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