Plasma zinc, copper, and erythrocyte superoxide dismutase in children with phelylketonuria

Plasma zinc, copper, and erythrocyte superoxide dismutase in children with phelylketonuria

Autor Fisberg, R. M. Google Scholar
Da Silva-Femandes, M. E. Google Scholar
Fisberg, M. Google Scholar
Schmidt, B. J. Google Scholar
Instituição Universidade Federal de São Paulo (UNIFESP)
Resumo Children with phenylketonuria (PKU) are treated with semisynthetic diets restricted in phenylalanine (PHE). the formulae must supply those trace elements and vitamins that are usually supplied by whole protein foods. We studied the effects of phenylalaline restricted diets in 42 children with PKU (P) and 31 normal (N) children, aged 1-12 y, divided into two groups (below and above 7 y). Plasma zinc and copper were analyzed by means of atomic spectrophotometry, and superoxide dismutase (CuZnSOD) activity was measured in erythrocytes, through NET inhibition and its profile, as determined by isoelectric focalization. Plasma zinc of PKU children greater than or equal to 7 years old was significantly lower than that in the control group (17 mu mol/L versus 20 mu mol/L) but still within the normal range; in children <7 years no substantial differences were found between the two groups. Plasma copper was not statistically different between PKU and normal children. Qualitative activity of CuZnSOD presented the same electrophoretic profile in both normal and PKU. Quantitative activity was not different in both P (1210 U/g Hb <7 versus 1328 U/g hemoglobin (Hb) greater than or equal to 7) and N (1675 U/g Hb <7 versus 1367 U/g Hb greater than or equal to 7). We concluded that children with PKU presented normal mean levels of zinc and copper, with preserved function, measured by enzyme activity. (C) Elsevier Science Inc. 1999.
Assunto phenylketonuria
superoxide dismutase
trace elements
artificial diets
Idioma Inglês
Data 1999-06-01
Publicado em Nutrition. New York: Elsevier B.V., v. 15, n. 6, p. 449-452, 1999.
ISSN 0899-9007 (Sherpa/Romeo, fator de impacto)
Editor Elsevier B.V.
Extensão 449-452
Direito de acesso Acesso restrito
Tipo Artigo
Web of Science WOS:000080758300008

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