Metabolic Disorders

Food Lists for Nutritional Management of Metabolic Disorders

Applications

Booklets which provide a reference guide for nutritional management of rare genetic diseases.

Technical Summary

A majority of inherited metabolic disorders affect children and adults worldwide. Many metabolic disorders require special dietary therapy and monitoring by a team of physicians, nurses, genetic counsellors, social workers, and dietitians for improved health and longevity. A relatively common diet-affected metabolic disorder is called PKU or phenylketonuria. Other disorders include Maple Syrup Urine Disease (MSUD) and Urea Cycle Disorders (UCD). These conditions involve deficiency in an enzyme that breaks down an essential amino acid or acids to a non-essential amino acid or other compound. This means that the essential amino acid tends to build up in the body, causing a variety of problems. The disorders can be treated and managed by limiting the intake of the amino acid or other compound through nutritional management.

The booklets contain food lists and were developed to assist patients and clinicians in making appropriate dietary choices to manage the metabolic disorders. Foods are classified according to the amino acid or other compound they contain which allows the patient or clinician to easily determine and manage dietary intake. Metabolic diseases, specifically PKU and MSUD, are serious disorders with life-threatening consequences unless addressed promptly. However, they are both manageable through dietary treatment. These booklets help to provide appropriate nutritional management to patients with these rare genetic disorders.