This book has been replaced by the online service HowMuchPhe.org, and smaller, pocket books are available at howmuchphe.org/books. The Low Protein Food List for PKU has been an indispensible resource for everyone involved in the treatment of phenylketonuria (PKU) since 1995. This third edition contains over 6,000 entries, the most extensive listing yet of foods that are potentially suitable for the diet, nearly double the number found in the previous edition. It includes many new foods found at health-oriented grocery stores, expanding choices for the diet. The book provides information on phenylalanine (phe), protein, and calorie content of foods based on serving portions, both in common measures and gram weights, in an easily searchable format. The handy "mg phe/gm food" column allows users to compare phenylalanine density of foods and make appropriate food choices for individual diet needs. People using an "exchange" system for counting phe will find exchanges are calculated for each food portion as well. The front section of the book also contains valuable information and tips for managing the PKU diet.

Much more than a cookbook, Low Protein Cookery for Phenylketonuria (PKU) is a practical and easy-to-use guide for those who must maintain a protein-restricted diet for treatment of PKU or similar inherited diseases of protein metabolism. It contains hundreds of helpful suggestions for managing the diet. This third edition of Low Protein Cookery for PKU appears exactly twenty years after the original 1977 publication and includes the 450-plus recipes and the hints from the 1988 second edition that have been used and enjoyed by families for nearly a decade. The major new feature of the third edition is entirely new nutrient calculations. The available food supply has changed significantly in the past fifteen years, and nutrient information is much better now. The nutrient calculations in this edition of the cookbook are based on the updated 1995 Low Protein Food List for PKU compiled by the author, which is the most widely used food list for the PKU diet in the United States. Some of the changes in nutrient values are subtle, others more significant; all reflect the best information currently available. Low Protein Cookery for PKU offers recipes that appeal to a wide range of ages, suit a wide range of individual diet requirements, and facilitate integration of the diet into normal family eating routines. Many of the recipes are suitable for the entire family; others include instructions for adapting the recipe to suit the needs of family members not on the diet, or are accompanied by recipes for the preparation of similar non-diet items. The recipes provide gram weights when appropriate, for greater accuracy in preparing the recipes and in maintaining the diet.

A food list for use in treatment of phenylketonuria and other metabolic diseases requiring a low protein diet. It contains over 6,000 entries, providing phenylalanine, protein and calories for each food.

Phenylketonuria (pronounced fee-nill-key-toe-NURR-ee-uh) or PKU is a rare, inherited metabolic disorder. Each year, approximately 1 in 14,000 newborn babies are diagnosed with PKU. If you have PKU, your body cannot break down, or metabolize, the essential amino acid, phenylalanine (Phe). Amino acids are the building blocks of protein. The primary treatment for PKU is a special metabolic formula and the restriction of foods high in Phe (protein), such as meats, cheeses, milk, bread and pasta. Life-long dietary compliance is a critical component for proper growth and development in children and quality of life in adults. We hope Penny The Penguin can help your child on this "diet for life" journey.

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.

Dietary reference values (DRVs) for energy are based on estimating the total energy expenditure (TEE) for groups of people. TEE provides a measure of the energy requirement at energy balance i.e. when energy intake matches energy expenditure. The methodology to measure TEE - the doubly labelled water (DLW) method - has advanced and as a result, the evidence base on TEE in a wide variety of population groups has expanded considerably. With the high levels of overweight and obesity currently seen in the UK and the wealth of new data now available, it was considered timely for the Scientific Advisory Committee on Nutrition (SACN) to review recommendations for the UK population. This report details the evidence and approaches SACN have considered in order to update the DRVs for energy. SACN chose a prescriptive approach to estimating energy reference values; suitable reference body weight ranges consistent with long-term good health were used to calculate energy reference values. Thus, basal metabolic rate (BMR) values were predicted using healthy reference body weights. Using this approach, if overweight groups consume the amount of energy recommended for healthy weight groups, they are likely to lose weight, whereas underweight sections of the population should gain weight towards the healthy body weight range. SACN has derived new energy reference values. For most population groups, except for infants and young children, the values have increased. DRVs should be used to assess the energy requirements for large groups of people and populations, but should not be applied to individuals due to the large variation in physical activity and energy expenditure observed between people.