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Article

[Tetrahydrobiopterin therapy for hyperphenylalaninemia due to phenylalanine hydroxylase deficiency. When and how?].

Anales de pediatria (Barcelona, Spain : 2003) - 1 Feb 2006

Baldellou Vázquez A, Salazar García-Blanco María I, Ruiz-Echarri Zalaya María P, Campos Calleja C, Ruiz Desviat L, Ugarte Pérez M

Abstract excerpt

INTRODUCTION: Some patients with hyperphenylalaninemia due to phenylalanine hydroxylase deficiency respond with a variable decrease in plasma phenylalanine levels after oral tetrahydrobiopterin (BH4) administration and are then able to tolerate higher dietary phenylalanine intake or even to discontinue a phenylalanine-restricted diet. BH4-sensitive patients are usually identified by means of a BH4 loading test,...

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PMID: 16527067
DOI: 10.1157/13084174

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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