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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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Publicat a:Mol Genet Metab Rep
Autors principals: Thiele, Alena G., Rohde, Carmen, Mütze, Ulrike, Arelin, Maria, Ceglarek, Uta, Thiery, Joachim, Baerwald, Christoph, Kiess, Wieland, Beblo, Skadi
Format: Artigo
Idioma:Inglês
Publicat: Elsevier 2015
Matèries:
SI:Therapy
Accés en línia: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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