Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH(4)).

@article{Thiele2013NutritionalCA,
  title={Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH(4)).},
  author={Alexandra Thiele and Johannes F. W. Weigel and B. Ziesch and Carmen Rohde and Ulrike M{\"u}tze and Uta Ceglarek and Jean Paul Thiery and Andreas Stefan Mueller and Wielan Kiess and Skadi Beblo},
  journal={JIMD reports},
  year={2013},
  volume={9},
  pages={31-40}
}
BACKGROUND Since 2008 patients with BH(4)-sensitive phenylketonuria can be treated with sapropterin dihydrochloride (Kuvan®) in addition to the classic phenylalanine (Phe) restricted diet. The aim of this study was to evaluate the nutritional changes and micronutrient supply in patients with phenylketonuria (PKU) under therapy with tetrahydrobiopterin (BH(4)). SUBJECTS AND METHODS 19 children with PKU (4-18 years) and potential BH(4)-sensitivity were included, 14 completed the study protocol… CONTINUE READING