The challenge of long-term tetrahydrobiopterin (BH(4)) therapy in phenylketonuria: Effects on metabolic control, nutritional habits and nutrient supply

BACKGROUND AND AIMS: BH(4)-sensitive phenylketonuria (PKU) patients relax their phenylalanine (Phe) restricted diet due to increased Phe tolerance, while keeping dried blood Phe concentrations with in the therapeutic range. We aimed to investigate metabolic control, eating habits and nutrient supply...

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Bibliografski detalji
Izdano u:Mol Genet Metab Rep
Glavni autori: Thiele, Alena G., Rohde, Carmen, Mütze, Ulrike, Arelin, Maria, Ceglarek, Uta, Thiery, Joachim, Baerwald, Christoph, Kiess, Wieland, Beblo, Skadi
Format: Artigo
Jezik:Inglês
Izdano: Elsevier 2015
Teme:
SI:Therapy
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC4750587/
https://ncbi.nlm.nih.gov/pubmed/26937412
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgmr.2015.07.002
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