Use and design of low protein diets for children with inborn metabolic disorders

Low protein diets are used to treat infants and children with hyperammonemia due to urea cycle and other metabolic disorders as well as a number of amino and organic acidopathies. The incidence of these disorders is small and many are life-threatening. As a result, there is little in the literature on the dietary management of these patients. This paper draws on 10 years of clinical experience at the Hospital for Sick Children in Toronto, Ontario and presents a guide to the preparation of infant formulas providing levels of protein intake from 0.5 to 2.0 g per kg. Also described is a low protein equivalency system that is a useful guide for measuring both baby foods and table foods for affected children up to about six years of age. This dietary information is accompanied by a description of the disorders amenable to low protein diets, some of the adjunctive therapies employed and the nutritional concerns associated with severe restriction of protein.