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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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Detalles Bibliográficos
Autores principales:	Thiele, Alena G., Rohde, Carmen, Mütze, Ulrike, Arelin, Maria, Ceglarek, Uta, Thiery, Joachim, Baerwald, Christoph, Kiess, Wieland, Beblo, Skadi
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Elsevier 2015
Materias:	SI:Therapy
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4750587/ https://www.ncbi.nlm.nih.gov/pubmed/26937412 http://dx.doi.org/10.1016/j.ymgmr.2015.07.002

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