Article
Clinical and nutritional evaluation of phenylketonuric patients on tetrahydrobiopterin monotherapy.
Molecular genetics and metabolism - 1 Dec 2005
Lambruschini Nilo, Pérez-Dueñas Belén, Vilaseca Maria Antonia, Mas Anna, Artuch Rafael, Gassió Rosa, Gómez Lilian, Gutiérrez Alejandra, Campistol Jaume
Abstract excerpt
The clinical, nutritional, and neuropsychological data of 11 mild/moderate PKU patients after one year of treatment with BH4 are evaluated. BH4 monotherapy was introduced at 5 mg/kg/day in 14 PKU patients. In 11/14 patients, Phe tolerance increased significantly from 356+/-172 to 1546+/-192 mg/day (p=0.004), and special PKU formula was gradually reduced until complete removal. In them, mean plasma Phe...
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