Tetrahydrobiopterin therapy vs phenylalanine-restricted diet: Impact on growth in PKU

Treatment of phenylketonuria based upon strict vegetarian diets, with very low phenylalanine intake and supplemented by phenylalanine-free formula, has proven to be effective in preventing the development of long-term neurological sequelae due to phenylalanine accumulation. On the other hand, such d...

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Veröffentlicht in:	Molecular genetics and metabolism 2013-08, Vol.109 (4), p.331-338
Hauptverfasser:	Aldámiz-Echevarría, Luis, Bueno, María A., Couce, María L., Lage, Sergio, Dalmau, Jaime, Vitoria, Isidro, Andrade, Fernando, Llarena, Marta, Blasco, Javier, Alcalde, Carlos, Gil, David, García, María C., González-Lamuño, Domingo, Ruiz, Mónica, Ruiz, María A., González, David, Sánchez-Valverde, Felix
Format:	Artikel
Sprache:	eng
Schlagworte:	Biopterins - administration & dosage Biopterins - analogs & derivatives Body Composition - drug effects Body Height - drug effects Body Mass Index Body Weight - drug effects Diet Follow-Up Studies Growth Humans Phenylalanine - administration & dosage Phenylalanine - metabolism Phenylketonuria Phenylketonurias - diet therapy Phenylketonurias - pathology Tetrahydrobiopterin
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