Dietary treatment of PKU using a low-phenylalanine peptide milk.

Abstract

AbstractA method of preparation of low phenylalanine (Phe) peptide fomiula for phenyketonuria (PKU) and the long term therapeutic effects of the milk in 6 PKU infants and one adult female patient aged 24 who wanted to become pregnant, are described. Low phenylalanine peptides (LPP) were prepared from whey protein. After hydrolysis by pronase (actinase), the hydrdysate was passed through a charcoal column to remove aromatic amino acids, and the free amino acids and salts were removed by passing through RD membranes to obtain LPP. Together with vegetable oils, lactose, dextrin, vitamins and minerals, as well as a small amount of amino acid mixture, low phenylalanine milk was madexs by freeze‐drying UP. The product (LPP milk), which does not have the bad taste and odor peculiar to amino acids, was administered to seven patients with PKU for one to three years. There were no differences between the LPP formula and conventional formulae collsisting of amino acids formula (AA formula), in reducing serum Phe levels in PKU patients. There were no adverse effects in patients who received LPP formula. UP formula can be used not only as milk, but also as an ingredient to make more palatable foods of low Phe content.