Publication

Anthropomorphic measurements and nutritional biomarkers after 5 years of BH4 treatment in phenylketonuria patients

Evers, R. A. F., van Wegberg, A. M. J., van Dam, E., de Vries, M. C., Janssen, M. C. H. & van Spronsen, F. J., Aug-2018, In : Molecular Genetics and Metabolism. 124, 4, p. 238-242 5 p.

Research output: Contribution to journalArticleAcademicpeer-review

Copy link to clipboard

Documents

  • Anthropomorphic measurements and nutritional biomarkers after 5 years of BH4 treatment in phenylketonuria patients

    Final publisher's version, 418 KB, PDF document

DOI

Objective: The objective of this study was to assess the long-term effects of BH4 treatment on anthropomorphic measurements and nutritional biomarkers in phenylketonuria patients.

Study design: A retrospective cohort study was conducted using data from 21 BH4-treated (mean age +/- SD: 13.12 +/- 9.18 years) and 21 birth date- and gender-matched phenylketonuria patients. Study parameters were age- and gender-adjusted SD scores of anthropomorphic measurements, various nutritional biomarkers, and prescribed vitamin/mineral supplements from baseline and after 5 years of BH4 treatment. Parametric and non parametric statistical analyses were performed for continuous data, and McNemar's and Chi-squared tests were used for dichotomous outcomes.

Results: Prescribed natural protein intake increased following BH4 treatment (p <0.001), while amino acid supplement intake decreased (p <0.001). Weight SDS had increased significantly after 5 years of BH4 treatment. Serum concentration of cholesterol, hemoglobin, phosphate, and MMA had changed significantly after 5 years of BH4 treatment as well. These changes in nutritional biomarkers are however probably age-related, as no differences compared to the control group were seen. A tendency to an increase in calcium supplementation was observed in BH4-treated patients (p = 0.063).

Conclusions: The dietary changes following BH4 treatment had little effect on anthropomorphic measurements and nutritional biomarkers when compared to a control group, although dietary follow-up of BH4-treated patients is still warranted.

Original languageEnglish
Pages (from-to)238-242
Number of pages5
JournalMolecular Genetics and Metabolism
Volume124
Issue number4
Publication statusPublished - Aug-2018

    Keywords

  • Phenylketonuria, Tetrahydrobiopterin, Diet, Nutrition, Anthropomorphic measurements, TETRAHYDROBIOPTERIN BH4, CHILDREN, THERAPY, AGE

ID: 75805431