Long-Term Follow-Up of Cognition and Mental Health in Adult Phenylketonuria: A PKU-COBESO Study
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Le résumé fourni par la source
Cognitive and mental health problems in individuals with the inherited metabolic disorder phenylketonuria (PKU) have often been associated with metabolic control and its history. For the present study executive functioning (EF) was assessed in 21 PKU patients during childhood (T1, mean age 10.4 years, SD = 2.0) and again in adulthood (T2, mean age 25.8 years, SD = 2.3). At T2 additional assessments of EF in daily life and mental health were performed. Childhood (i.e. 0-12 years) blood phenylalanine was significantly related to cognitive flexibility, executive motor control, EF in daily life and mental health in adulthood (i.e. at T2). Patients with a greater increase in phenylalanine levels after the age of 12 performed more poorly on EF-tasks at T2. Group-based analyses showed that patients with phenylalanine <360 µmol/L in childhood and phenylalanine ≥360 µmol/L from age 13 onwards (n = 11) had better cognitive flexibility and executive motor control than those who had phenylalanine ≥360 µmol/L throughout life (n = 7), supporting the notion that phenylalanine should be below the recommended upper treatment target of 360 µmol/L during childhood for better outcome in adulthood. Despite some results indicating additional influence of phenylalanine levels between 13 and 17 years of age, evidence for a continued influence of phenylalanine levels after childhood on adult outcomes was largely lacking. This may be explained by the fact that the patients in the present study had relatively low phenylalanine levels during childhood (mean: 330 µmol/L, range: 219-581 µmol/L) and thereafter (mean Index of Dietary Control at T2: 464 µmol/L, range: 276-743 µmol/L), which may have buffered against transitory periods of poor metabolic control during adolescence and early adulthood.
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Long-Term Follow-Up of Cognition and Mental Health in Adult Phenylketonuria: A PKU-COBESO Study
- Date Crossref
- 03/08/2017
- Éditeur
- Springer Science and Business Media LLC
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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University Medical Center Groningen pays non établi dans la noticeÉtablissement de santé
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University of Groningen pays non établi dans la noticeUniversité ou école supérieure
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Leiden University Department of Clinical Child and Adolescent Studies & Leiden Institute for Brain and Cognition pays non établi dans la noticeUniversité ou école supérieure
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Amsterdam UMC Location University of Amsterdam pays non établi dans la noticeÉtablissement de santé
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Maastricht University Medical Centre pays non établi dans la noticeOrganisme public
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University Medical Center Utrecht Wilhelmina Children's Hospital pays non établi dans la noticeÉtablissement de santé
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Wilhelmina Children's Hospital pays non établi dans la noticeÉtablissement de santé
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Radboud University Nijmegen pays non établi dans la noticeUniversité ou école supérieure
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Radboud University Medical Center pays non établi dans la noticeOrganisme public
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Erasmus MC pays non établi dans la noticeÉtablissement de santé
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Academic Medical Center pays non établi dans la noticeÉtablissement de santé
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University Hospital Maastricht and Laboratory Genetic Metabolic Diseases Department of Internal Medicine pays non établi dans la noticeUniversité ou école supérieure
University Medical Center Groningen, University of Groningen et Department of Clinical Child and Adolescent Studies & Leiden Institute for Brain and Cognition — Leiden University, avec 9 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.