Plasma Lipid Concentrations in 42 Treated Phenylketonuric Children

Abstract

A low phenylalanine diet is the only therapy for classic phenylketonuria (PKU) (Berry et al., 1967). This diet is based on a reduced intake of natural proteins replaced, to a great extent, by synthetic protein substitutes, such as very low phenylanine protein hydrolysates or phenyl-alanine-free amino acid mixtures. The remaining protein requirements are generally met by vegetable proteins with a reduced content of phenylalanine (Schurrle and Bickel, 1971; Giovannini et al., 1979). Such a diet can be defined as “non-atherogenic” as the reduction of animal proteins (meat, milk, eggs) involves a decreased intake of animal lipids, cholesterol, saturated fatty acids (with consequent increased plasma polyunsaturated/satur-ated fatty acids ratio), decreased plasma lysine/arginine ratio and increased intake of dietary fibres (Vahouny, 1982). All these factors have well-known effects on the control of plasma cholesterol, while the reduced plasma levels of sulphur amino acids seem to lower the presence of circulating mono- and disulphurated compounds.