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Article

Response of phenylketonuria to tetrahydrobiopterin.

The Journal of nutrition - 1 Jun 2007

Michals-Matalon Kimberlee, Bhatia Gita, Guttler Flemming, Tyring Stephen K, Matalon Reuben

Abstract excerpt

A favorable response, indicated by decline of blood phenylalanine (Phe) in patients with phenylketonuria (PKU), to orally administered 6-R-L-erythro-5, 6, 7, 8-tetrahydrobiopterin (BH4) has been reported in many countries following the first publication in 1999. In this review, we describe the experience in the United States with PKU patients and their response to BH4. A significant response to BH4 is arbitrarily...

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PMID: 17513426
DOI: 10.1093/jn/137.6.1564S

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