Nutrition Management of Inherited Metabolic Diseases

Nutrition Management of Inherited Metabolic Diseases

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This up-to-date reference on the nutrition management of inherited metabolic diseases (IMD) covers a wide range of these disorders, including phenylketonuria and other aminoacidopathies, organic acidemias, urea cycle disorders, fatty acid oxidation disorders, galactosemia and glycogen storage diseases. Guidance is also provided on laboratory evaluations and biochemical testing and monitoring. Topics such as newborn screening for IMD, as well as nutrition management during pregnancy and transplantation, are addressed. The book is based on 7 years of lectures delivered through Metabolic University a€“ an interactive, didactic program designed to provide training to dietitians who work with individuals with IMD. This book provides the basic information required to manage nutrition care and is a resource for clinicians new to this complex field.Table 21.1 Recommendations for total and rate of weight gain during pregnancy based on prepregnancy BMI [26] Total ... when poor energy intake is common, during any intercurrent illness, with prolonged delivery, and in the postpartum period. ... Weight loss should be avoided since this can cause protein catabolism and elevated amino acid concentrations ... Rates of weight gain in 2nd and 3rd aTo calculate BMI, go to Energy = EERnonpregnant +anbsp;...

Title:Nutrition Management of Inherited Metabolic Diseases
Author:Laurie E. Bernstein, Fran Rohr, Joanna R Helm
Publisher:Springer - 2015-07-14


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