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Zinc and Copper Status of Treated Children with Phenylketonuria

Division of Medical Genetics, Departments of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, University of Colorado Health Sciences Center, Denver, Colorado

Paul M. Fernhoff, M.D.

Division of Medical Genetics, Departments of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, University of Colorado Health Sciences Center, Denver, Colorado

Hope S. Warshaw, M.M.Sc.

Division of Medical Genetics, Departments of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, University of Colorado Health Sciences Center, Denver, Colorado

K. Michael Hambidge, M.R.C.P.

Division of Medical Genetics, Departments of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, University of Colorado Health Sciences Center, Denver, Colorado

Arlene Ernest, M.A.

Division of Medical Genetics, Departments of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, University of Colorado Health Sciences Center, Denver, Colorado

Edward R.B. Mccabe, PH.D., M.D.

Division of Medical Genetics, Departments of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, University of Colorado Health Sciences Center, Denver, Colorado

Louis J. Elsas, II, M.D.

Division of Medical Genetics, Departments of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, University of Colorado Health Sciences Center, Denver, Colorado

Children with phenylketonuria (PKU) are treated with semi-synthetic diets restricted in phenylalanine. Low or phenylalanine-free formulae provide the majority of protein and energy in the diet while phenylalanine requirements are met by low-protein natural foods. Because of the restriction of natural protein sources in this diet, the study assessed the zinc and copper nutriture of treated children with PKU and correlated linear growth with zinc status. The plasma zinc of the PKU population was 66.6 ± 3.3 µg/dl (mean ± SEM). The hair zinc was 70.2 ± 11.5 µg/g (mean ± SEM). The mean plasma and hair zinc of the PKU population were significantly different (p < 0.05) when compared to normal values of 84.2 ± 2.9 µg/dl and 130.7 ± 8.3 µg/g (mean ± SEM), respectively. The dietary zinc intake of 10 PKU patients was 8.56 ± 2.68 mg/day (mean ± SD). No significant differences (p < 0.123) were found when the mean zinc intake was compared with recommended dietary allowances for age of 10 mg/day. No significant correlations were found when plasma and hair zinc were plotted with height percentiles. Plasma copper of the PKU subjects (87.6 ± 6.6 µg/ dl, mean ± SEM) was significantly less than that of normal young children (121.5 ± 3.1 µg/dl, mean ± SEM) despite a copper intake of 1.45 ± 0.35 mg/day (mean ± SD).

Journal of Parenteral and Enteral Nutrition, Vol. 5, No. 5, 406-409 (1981)
DOI: 10.1177/0148607181005005406


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				E. R. B. McCabe, A. M. Nord, A. Ernest, and L. McCabe Evaluation of a Phenylalanine-Free Product for Treatment of Phenylketonuria Arch Pediatr Adolesc Med, December 1, 1987; 141(12): 1327 - 1329. [Abstract] [PDF]

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