Adequacy of Nutritional Therapy

Carefully executed diet therapy for individuals with PKU is widely considered to be safe as well as efficacious in preventing mental and neurological impairment. However, it cannot be assumed that largely synthetic diets supplemented with individual vitamins, minerals, and trace elements will confer the same benefits as diets composed of whole foods. Synthetic diets may have an inherent inability to supply all essential nutrients. In addition, patients who are noncompliant or partially compliant with their intake of medical food are at increased nutritional risk. Formerly treated patients who are 'off diet' tend to select high-carbohydrate diets and continue their habit of avoiding high-protein foods such as meat, milk, and eggs. Micronutrients previously supplied by the medical food, such as vitamin B12, zinc, and iron, may not be replaced in adequate amounts on such a self-selected diet.

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