دورية أكاديمية
أعرض في EDS

Neurocognitive evidence for revision of treatment targets and guidelines for phenylketonuria.

التفاصيل البيبلوغرافية
العنوان: Neurocognitive evidence for revision of treatment targets and guidelines for phenylketonuria.
المؤلفون: Jahja R; Division of Metabolic Diseases, University Medical Center Groningen, Beatrix Children's Hospital, University of Groningen, Groningen, The Netherlands. Electronic address: jahjar@umcg.nl., Huijbregts SC; Department of Clinical Child and Adolescent Studies; Leiden Institute for Brain and Cognition Leiden University, Leiden, The Netherlands., de Sonneville LM; Department of Clinical Child and Adolescent Studies; Leiden Institute for Brain and Cognition Leiden University, Leiden, The Netherlands., van der Meere JJ; Department of Developmental and Clinical Neuropsychology, University of Groningen, Groningen, The Netherlands., van Spronsen FJ; Division of Metabolic Diseases, University Medical Center Groningen, Beatrix Children's Hospital, University of Groningen, Groningen, The Netherlands.
المصدر: The Journal of pediatrics [J Pediatr] 2014 Apr; Vol. 164 (4), pp. 895-899.e2. Date of Electronic Publication: 2014 Jan 30.
نوع المنشور: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Mosby Country of Publication: United States NLM ID: 0375410 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1097-6833 (Electronic) Linking ISSN: 00223476 NLM ISO Abbreviation: J Pediatr Subsets: MEDLINE
أسماء مطبوعة: Original Publication: St. Louis, MO : Mosby
مواضيع طبية MeSH: Phenylketonurias/*drug therapy , Phenylketonurias/*physiopathology, Adolescent ; Child ; Female ; Humans ; Male ; Neuropsychological Tests ; Phenylalanine/blood ; Phenylketonurias/blood ; Practice Guidelines as Topic ; Tyrosine/blood
مستخلص: Objectives: To compare the neurocognitive outcomes of patients with phenylketonuria (PKU) to determine whether decreasing phenylalanine (Phe) levels to <240 is preferable to the use of 360 μmol/L as an upper-target Phe level. An additional aim was to establish the influence of biochemical indices other than Phe on neurocognitive outcomes.
Study Design: Patients with PKU (n = 63; mean age 10.8 ± 2.3 years) and healthy controls (n = 73; mean age 10.9 ± 2.2 years) performed computerized tasks measuring neurocognitive functions (inhibitory control, cognitive flexibility, and motor control). Lifetime and concurrent blood Phe levels, Phe-to-tyrosine ratio (Phe:Tyr), and Phe variations were examined in relation to neurocognitive outcomes using nonparametric tests and regression analyses.
Results: Patients with PKU with Phe levels ≤240 μmol/L and healthy controls performed equally well. Patients with Phe levels between 240 and 360 μmol/L and ≥360 μmol/L performed more poorly than did controls across tasks. Patients with Phe levels ≤240 μmol/L performed significantly better than patients with levels between 240 and 360 μmol/L on tasks measuring inhibitory control and cognitive flexibility. Absolute Phe levels and Phe variation were the best predictors of motor control, whereas Phe:Tyr were the best predictors of inhibitory control.
Conclusions: The results of this study suggest that upper Phe targets should be lowered to optimize neurocognitive outcomes. Moreover, Phe variation and Phe:Tyr appear to be of additional value in treatment monitoring.
(Copyright © 2014 Mosby, Inc. All rights reserved.)
المشرفين على المادة: 42HK56048U (Tyrosine)
47E5O17Y3R (Phenylalanine)
تواريخ الأحداث: Date Created: 20140204 Date Completed: 20140512 Latest Revision: 20220321
رمز التحديث: 20231215
DOI: 10.1016/j.jpeds.2013.12.015
PMID: 24485821
قاعدة البيانات: MEDLINE
الوصف
تدمد:1097-6833
DOI:10.1016/j.jpeds.2013.12.015