POLYUNSATURATED FATTY-ACID STATUS IN PATIENTS WITH PHENYLKETONURIA

To determine whether the n-6 and n-3 polyunsaturated fatty acid levels can be affected by the PKU diet, fatty acid composition was studied i n PKU subjects and controls together with their lipid intake. The stud y population consisted of 40 PKU patients treated with a phenylalanine -restricted diet. Controls were 50 children on whom preoperative tests had been carried out for minor surgery. Methyl esters were obtained f rom plasma and red blood-cells and separated by gas chromatography. PK U patients showed lower docosahexaenoic acid levels (22:6 n-3) (p < 0. 001) and higher n-6 series levels in plasma (18:2 n-6) (p<0.001) and i n red blood-cells (20:4 n-6) (p<0.001) than the control population. Si nce docosahexaenoic and arachidonic acids are fundamental structural c omponents for normal brain development, it seems necessary to consider a dietary adjustment in these patients.