Thesis

Special formula for use in amino acid disorders

Disturbances i.n amino acid metabolism with subsequent plasma elevations that can lead to mental retardation can be effectively controlled by dietary regulation. Appropriate formula for management of amino acid disorders is manufactured at Children's Hospital of Los Angeles. This thesis describes specific formulations designed for patients with maple syrup urine disease, propionicacidemia, methylmalonicaciduria, and homocystinuria. A description of dry amino acid supplements for use in managing patients with these metabolic disorders also is provided. Overall nutritional management of children on special semi-synthetic formulas is discussed with examples of nutritional evaluational forms designed to collect requisite information.

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