Nutrition Management of Inherited Metabolic Diseases: Lessons from Metabolic University

The book "Metabolic University" is a compilation of topics taught at Metabolic University, an educational program for metabolic dietitians/nutritionists, physicians, nurses, and genetic counselors. It covers nutrition management of Inherited Metabolic Disorders (IMD), including phenylketonuria, aminoacidopathies, organic acidemias, urea cycle disorders, fatty acid oxidation disorders, galactosemia, and glycogen storage diseases. The 2nd edition is an updated and extensive version, covering laboratory evaluations, biochemical testing, monitoring, newborn screening, pregnancy, and transplantation. It also includes current medical management therapies.

Format: Paperback / softback
Length: 428 pages
Publication date: 16 June 2022
Publisher: Springer Nature Switzerland AG

Metabolic University (MU), an interactive and didactic educational program, has trained over 600 metabolic dietitians/nutritionists, physicians, nurses, and genetic counselors. This comprehensive book, first published in 2014, serves as a valuable resource for the metabolic community. The first edition focuses solely on the subject matter covered at MU, making it a concise guide rather than a comprehensive treatise on Inherited Metabolic Disorders (IMD). However, it addresses the most frequently encountered challenges in IMD nutrition.

Each chapter in the book emphasizes the principles of nutrition management, guides the initiation of diets, and highlights biomarkers for monitoring progress. Recognizing that practices may vary, the book acknowledges that understanding the inactivity of an enzyme in a metabolic pathway is crucial for determining dietary restrictions and supplementation requirements. It also emphasizes the importance of monitoring appropriate biomarkers to make diet adjustments and ensure therapy goals are met.

The second edition, an updated and expanded version, covers the nutrition management of a wide range of IMD, including phenylketonuria and other aminoacidopathies, organic acidemias, urea cycle disorders, fatty acid oxidation disorders, galactosemia, and glycogen storage diseases. Guidance is provided on laboratory evaluations and biochemical testing, as well as monitoring. Special attention is given to newborn screening for IMD, nutrition management during pregnancy and transplantation, and current medical management therapies.

This book is a valuable tool for healthcare professionals, researchers, and students interested in the field of metabolic disorders. It provides a comprehensive and practical approach to nutrition management, enabling practitioners to deliver optimal care to individuals with inherited metabolic disorders.

Weight: 982g
Dimension: 254 x 178 (mm)
ISBN-13: 9783030945091
Edition number: 2nd ed. 2022