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Phenylketonuria, Oxidative Stress, and BH4

Phenylketonuria Clinical Trial

Official title:
The Ability of Kuvan® (Sapropterin Dihydrochloride) to Prevent Meal-induced Lipid Peroxidation and Endothelial Dysfunction in Patients With Phenylketonuria: a Pilot Study

Clinical Trial Summary

The purpose of this study is to see how tetrahydrobiopterin therapy (BH4; also known as sapropterin dihydrochloride or Kuvan) affects measures of oxidative stress and endothelial function in patients with Phenylketonuria (PKU).

Clinical Trial Description

Oxidative stress will be induced by having participants eat a meal in polyunsaturated fats. The investigators will see how participants respond to the meal challenge over the course of 6 hours.

- PKU participants who are currently on BH4 therapy will be evaluated while taking BH4 with the meal at a single study visit.

- PKU participants who did not responded to BH4 therapy will be evaluated twice. At the first study visit the investigators will see how you react to the meal challenge when you are not given BH4. You will be given BH4 for two weeks, then return for a second meal challenge study visit while taking BH4.

- Healthy controls will be evaluated after consuming the meal only (no BH4) at a single study visit. ;

Study Design

Allocation: Non-Randomized, Intervention Model: Parallel Assignment, Masking: Open Label

Related Conditions & MeSH terms

Clinical Trial Details
NCT number NCT01395394
Study type Interventional
Source Emory University
Contact
Status Terminated
Phase Phase 2
Start date June 2011
Completion date June 2013
View Details
See also
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