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

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...

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Bibliographic Details
Published in:Nutrition (Burbank, Los Angeles County, Calif.) Los Angeles County, Calif.), 1999-06, Vol.15 (6), p.449-452
Main Authors: Fisberg, Regina Mara, da Silva-Femandes, Maria Eugênia, Fisberg, Mauro, Schmidt, Benjamin José
Format: Article
Language:English
Subjects:
Aminoacid disorders
artificial diets
Biological and medical sciences
Child
Child, Preschool
copper
Copper - blood
Errors of metabolism
Erythrocytes - enzymology
Female
Humans
Infant
Male
Medical sciences
Metabolic diseases
Phenylalanine - administration & dosage
phenylketonuria
Phenylketonurias - blood
Phenylketonurias - diet therapy
Phenylketonurias - enzymology
Reference Values
superoxide dismutase
Superoxide Dismutase - blood
trace elements
zinc
Zinc - blood
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Summary: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 NBT inhibition and its profile, as determined by isoelectric focalization. Plasma zinc of PKU children ≥7 years old was significantly lower than that in the control group (17 μmol/L versus 20 μmol/L) but still within the normal range; in children
ISSN:0899-9007
1873-1244
DOI:10.1016/S0899-9007(99)00082-9