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Nutr Clin Pract. 2015 Aug;30(4):502-10. doi: 10.1177/0884533615586201. Epub 2015 Jun 16.

Inherited Metabolic Disorders: Aspects of Chronic Nutrition Management.

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Department of Molecular and Human Genetics, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas
Department of Food and Nutrition, Texas Children's Hospital, Houston, Texas.
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas.


The introduction of newborn screening and the development of new therapies have led to an expanding population of patients with inherited metabolic disorders, and these patients are now entering adulthood. Dietary therapy is the mainstay of treatment for many of these disorders, and thus, trained metabolic dietitians are critical members of the multidisciplinary team required for management of such patients. The main goals of dietary therapy in inborn errors of metabolism are the maintenance of normal growth and development while limiting offending metabolites and providing deficient products. Typically, the offending metabolite is either significantly reduced or removed completely from the diet and then reintroduced in small quantities until blood levels are within the normal range. Such treatment is required in infancy, childhood, and adulthood and requires careful monitoring of micronutrient and macronutrient intake throughout the life span. The goal of this review is to highlight the basic principles of chronic nutrition management of the inborn errors of protein, carbohydrate, and fat metabolism.


diet therapy; inborn errors of metabolism; metabolic diseases; metabolic disorder; nutrition therapy

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