Nutrition support of inherited disorders of amino acid metabolism: Part 1

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Nutrition support of patients with inherited disorders of amino acid metabolism requires thorough knowledge of normal growth and development, normal and abnormal metabolism, nutrient requirements at various stages of the life cycle, and effects fo enzyme deficiencies on nutrient requirements. Requirements of patients are modified from normal nutrient requirements, not only by the enzyme defect, but also by the elemental nature of medical foods. Elemental medical foods require care in preparation to prevent the Maillard Reaction, which decreases bioavailability of amino acids and sugars. Frequent monitoring of nutrition status and fine tuning of the nutrition support prescription enhance normal growth and development.

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