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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1998-12-17
pubmed:abstractText
Abnormal oxidative stress was observed in some inborn errors of metabolism owing to the accumulation of toxic metabolites leading to excessive free radical production and to the influence of restricted diets on the antioxidant status. Erythrocyte antioxidant enzymes activities and tocopherol concentrations were measured in a group of phenylketonuric (n = 42) and mild-hyperphenylalaninemic (n = 28) patients compared with 45 age-matched controls. We also determined plasma selenium levels in these groups. We also evaluated the possible relationship between antioxidant status and neuropsychological disorders. Erythrocyte glutathione peroxidase (GSH-Px) activity was significantly lower (P < 0.001) in both phenylketonuric and mild-hyperphenylalaninemic patients compared with the control group, but no differences were observed between the two groups of patients. Neuropsychological disturbances were more frequent in the group of PKU patients with low GSH-Px activity than in PKU patients with normal GSH-Px. Low GSH-Px activity might be explained in phenylketonuria as a result of a selenium deficiency caused by a poor selenium intake or absorption, but not in mild hyperphenylalaninemic patients with free diet. Selenium levels were normal in both groups of patients, so low glutathione peroxidase activity in both phenylketonuric and hyperphenylalaninemic groups might be influenced by other factors, such as the consequences of an unbalanced amino acid profile, common to both conditions.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0009-8981
pubmed:author
pubmed:issnType
Print
pubmed:day
10
pubmed:volume
276
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1-9
pubmed:dateRevised
2005-11-17
pubmed:meshHeading
pubmed-meshheading:9760015-Adolescent, pubmed-meshheading:9760015-Amino Acid Metabolism, Inborn Errors, pubmed-meshheading:9760015-Antioxidants, pubmed-meshheading:9760015-Catalase, pubmed-meshheading:9760015-Child, pubmed-meshheading:9760015-Child, Preschool, pubmed-meshheading:9760015-Electroencephalography, pubmed-meshheading:9760015-Erythrocytes, pubmed-meshheading:9760015-Female, pubmed-meshheading:9760015-Glutathione Peroxidase, pubmed-meshheading:9760015-Glutathione Reductase, pubmed-meshheading:9760015-Humans, pubmed-meshheading:9760015-Infant, pubmed-meshheading:9760015-Intelligence Tests, pubmed-meshheading:9760015-Male, pubmed-meshheading:9760015-Phenylalanine, pubmed-meshheading:9760015-Phenylketonurias, pubmed-meshheading:9760015-Superoxide Dismutase, pubmed-meshheading:9760015-Vitamin E
pubmed:year
1998
pubmed:articleTitle
Antioxidant status in hyperphenylalaninemia.
pubmed:affiliation
Laboratori, Hospital Universitari Sant Joan de Déu, Barcelona, Spain.
pubmed:publicationType
Journal Article