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      Improved metabolic control in tetrahydrobiopterin (BH4), responsive phenylketonuria with sapropterin administered in two divided doses vs. a single daily dose.

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          Abstract

          Phenylketonuria (PKU) often requires a lifelong phenylalanine (Phe)-restricted diet. Introduction of 6R-tetrahydrobiopterin (BH4) has made a huge difference in the diets of patients with PKU. BH4 is the co-factor of the enzyme phenylalanine hydroxylase (PAH) and improves PAH activity and, thus, Phe tolerance in the diet. A limited number of published studies suggest a pharmacodynamic profile of BH4 more suitable to be administered in divided daily doses.

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          Author and article information

          Journal
          J Pediatr Endocrinol Metab
          Journal of pediatric endocrinology & metabolism : JPEM
          Walter de Gruyter GmbH
          2191-0251
          0334-018X
          Jul 26 2017
          : 30
          : 7
          Affiliations
          [1 ] .
          Article
          /j/jpem.ahead-of-print/jpem-2016-0461/jpem-2016-0461.xml
          10.1515/jpem-2016-0461
          28593914
          1c33614f-da16-4fed-8cda-481656134c6b
          History

          phenylketonuria,divided daily doses,BH4
          phenylketonuria, divided daily doses, BH4

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