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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Detalhes bibliográficos
Publicado no:Mol Genet Metab Rep
Main Authors: Thiele, Alena G., Rohde, Carmen, Mütze, Ulrike, Arelin, Maria, Ceglarek, Uta, Thiery, Joachim, Baerwald, Christoph, Kiess, Wieland, Beblo, Skadi
Formato: Artigo
Idioma:Inglês
Publicado em: Elsevier 2015
Assuntos:
SI:Therapy
Acesso em linha: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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