Anthropomorphic measurements and nutritional biomarkers after 5 years of BH4 treatment in phenylketonuria patients
The objective of this study was to assess the long-term effects of BH4 treatment on anthropomorphic measurements and nutritional biomarkers in phenylketonuria patients. A retrospective cohort study was conducted using data from 21 BH4-treated (mean age ± SD: 13.12 ± 9.18 years) and 21 birth date- an...
Saved in:
Published in | Molecular genetics and metabolism Vol. 124; no. 4; pp. 238 - 242 |
---|---|
Main Authors | , , , , , |
Format | Journal Article |
Language | English |
Published |
Elsevier Inc
01.08.2018
|
Subjects | |
Online Access | Get full text |
Cover
Loading…
Summary: | The objective of this study was to assess the long-term effects of BH4 treatment on anthropomorphic measurements and nutritional biomarkers in phenylketonuria patients.
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.
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).
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. |
---|---|
ISSN: | 1096-7192 1096-7206 |
DOI: | 10.1016/j.ymgme.2018.06.009 |