Trace element status of PKU children ingesting an elemental diet.

Abstract

We assessed the trace metal status of 10 children (3.5 to 13.3 yr) with phenylketonuria (PKU) who were successively treated for at least 6 months with a semi-synthetic formula restricted in phenylalanine, then for 6 months with a reformulated formula. The reformulated product contained higher concentrations of magnesium (Mg), potassium (K), phosphorus (P), selenium (Se), and zinc (Zn) and lower concentrations of calcium (Ca), sodium (Na), and unsaturated fats. Diet records, blood samples, and urine specimens were collected from each subject. Mean intakes of Mg, K, and Zn increased significantly when subjects were switched to the reformulated product. The subject's mean intakes of Se (for all age groups) and Zn (children 11 to 14 yr old) were below 67% of recommended intakes while they were ingesting the original formula. The mean trace metal concentrations of urine, blood, and serum did not differ significantly during the two periods. Mean serum Zn, Se, and Cu concentrations of the subjects were less than those reported for normal children. Significant negative correlations were obtained between serum Zn concentrations and the molar ratio of dietary (Ca + P):Zn, and the molar ratio of dietary Fe:Zn. No significant differences in mean urinary Cu and Zn excretion (mg/g creatinine) were observed in subjects after they were changed to the reformulated product. Individuals whose primary source of energy and protein is derived from semi-synthetic elemental diets are especially at risk for multiple trace element deficiencies.