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No Impairment in Bone Turnover or Executive Functions in Well-Treated Preschoolers with Phenylketonuria—A Pilot Study

Authors :
Beatrice Hanusch
Michael Falkenstein
Stefan Volkenstein
Stefan Dazert
Thomas Lücke
Kathrin Sinningen
Source :
Nutrients, Vol 16, Iss 13, p 2072 (2024)
Publication Year :
2024
Publisher :
MDPI AG, 2024.

Abstract

Patients with phenylketonuria (PKU) present signs of impaired executive functioning and bone health in adolescence and adulthood, depending in part on the success of therapy in childhood. Therefore, nine children with well-treated PKU (4–7 years old, 22.2% ♀, seven with a full set of data, two included into partial analysis) and 18 age-, gender- and season-matched controls were analyzed for differences in executive functioning and bone parameters in plasma. Plasma was analyzed with commercially available kits. Cognitive performance in tonic alertness, visuo-spatial working memory, inhibitory control and task switching was assessed by a task battery presented on a touch screen. Regarding cognition, only the performance in incongruent conditions in inhibitory control was significantly better in children with PKU than in controls. No further differences in cognitive tests were detected. Furthermore, no significant difference in the bone turnover markers osteocalcin, undercarboxylated osteocalcin and CTX were detected between children with PKU and controls, while children with PKU had a significantly higher vitamin D concentration (69.44 ± 12.83 nmol/L vs. 41.87 ± 15.99 nmol/L, p < 0.001) and trended towards lower parathyroid hormone concentrations than controls (48.27 ± 15.16 pg/mL vs. 70.61 ± 30.53 pg/mL, p = 0.066). In this small group of well-treated preschoolers with PKU, no impairments in cognitive performance and bone turnover were observed, while vitamin D supplementation of amino acid supplements seems to be sufficient to achieve good vitamin D status.

Details

Language :
English
ISSN :
20726643
Volume :
16
Issue :
13
Database :
Directory of Open Access Journals
Journal :
Nutrients
Publication Type :
Academic Journal
Accession number :
edsdoj.57811f136fb94eab895b1cc553d2c2cb
Document Type :
article
Full Text :
https://doi.org/10.3390/nu16132072