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Nutrition Management of Inherited Metabolic Diseases - Lessons from Metabolic University (Paperback, 2nd ed. 2022)
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Nutrition Management of Inherited Metabolic Diseases - Lessons from Metabolic University (Paperback, 2nd ed. 2022)
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This text presents a compilation of topics that have been taught at
Metabolic University (MU), an interactive, didactic educational
program that has trained over 600 metabolic
dietitians/nutritionists, physicians, nurses and genetic
counselors. This book was created in 2014 for the metabolic
community. The 1st edition contains only subject matter covered at
Metabolic University; therefore, it is not a comprehensive treatise
on Inherited Metabolic Disorders (IMD) but rather a text on the
most frequently encountered challenges in IMD nutrition. Each
chapter in the book highlights principles of nutrition management,
how to initiate a diet, and biomarkers to monitor the diet.
Recognizing that there are variations in practice, this book
addresses that the key to management lies in the understanding how
the inactivity of an enzyme in a metabolic pathway determines which
components of the diet must be restricted and which must be
supplemented as well as the monitoring of appropriate biomarkers to
make diet adjustments and ensure the goals of therapy are met The
2nd edition is an updated and more extensive version covering the
nutrition management of IMD, and 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 also
addressed. In addition, current medical management therapies is
included.
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