BH4 therapy impacts the nutrition status and intake in children with phenylketonuria: 2-year follow-up

@article{Singh2010BH4TI,
  title={BH4 therapy impacts the nutrition status and intake in children with phenylketonuria: 2-year follow-up},
  author={Rani H. Singh and Meghan E. Quirk and Teresa D. Douglas and Mary C. Brauchla},
  journal={Journal of Inherited Metabolic Disease},
  year={2010},
  volume={33},
  pages={689-695}
}
The impact of tetrahydrobiopterin (BH4) treatment on phenylalanine tolerance, medical-food consumption, and nutrition status in patients with phenylketonuria (PKU) was investigated. Six children (5–12 years) with well-controlled PKU, responding to a BH4 dose of 20 mg/kg per day, were assessed for 24 months. Mean dietary phenylalanine tolerance increased from 421 ± 128 to 1470 ± 455 mg/day. Height Z scores significantly improved from 0.25 ± 0.99 at baseline to 0.53 ± 1.16 at 24 months (p for… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 18 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 12 references

Recommendations for personalized dietary adjustments based on patient response to tetrahydrobiopterin (BH4) in phenylketonuria

  • RH Singh, E Jurecki, R Rohr
  • Top Clin Nutr
  • 2008
1 Excerpt

Long - term treatment of patients with mild and classical phenylketonuria by tetrahydrobiopterin

  • JH Walter, FJ White, SK Hall
  • Mol Genet Metab
  • 2005

Similar Papers

Loading similar papers…