Nutrition support of inborn errors of amino acid metabolism

Programs for nutrition support of patients with phenylketonuria, maternal phenylketonuria, branched chain ketoaciduria and vitamin B-6 non-responsive homocystinuria were written in BASIC. These programs plan diets to fill diet prescriptions using natural foods, available amino acid-free or restricted elemental products, milk or infant proprietary formulae and protein-free fat and carbohydrate sources. Emphasis is placed on satisfying the amino acid and protein prescriptions simultaneously. The final semisynthetic formula is evaluated for vitamins, minerals, renal solute load and renal net acid excretion. Fluid requirement is estimated. The paper describes how the conventional protocols are enhanced by computerization and details the requisite calculations.