Effects of short term increase of phenylalanine levels on neuropsychological functions and well-being in adults with phenylketonuria: the *diet for life* study
- Conditions
- phenylketonuriaPKU1002742410021605
- Registration Number
- NL-OMON30968
- Lead Sponsor
- Academisch Medisch Centrum
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Pending
- Sex
- Not specified
- Target Recruitment
- 20
Inclusion Criteria
Patients with PKU aged 18 and older who have been detected by newborn screening and have been continuously treated with a protein restricted diet and supplementation of aminoacids.
Exclusion Criteria
Poor dietary adherence with a mean phe value above 1100 umol/l in the year prior to the start of the study
Pregnancy or the wish to conceive within 3 months after the end of the study
Study & Design
- Study Type
- Interventional
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method <p>What is the neuropsychological deficit profile of adults with treated PKU?<br /><br>Does supplementation of Phe to plasma levels comparable to levels observed in<br /><br>adult patients who fully discontinued their diet, influence neurocognitive<br /><br>functioning or wellbeing?</p><br>
- Secondary Outcome Measures
Name Time Method <p>Which neurophysiological mechanisms are responsible for neuropsychological<br /><br>dysfunction in adults with treated PKU? Can network disruption be demonstrated?<br /><br>Does supplementation of Phe to plasma levels comparable to levels observed in<br /><br>adult patients who fully discontinued their diet influence network disruption?<br /><br><br /><br><br /><br>This part of the study will be conducted by the department of neurology in the<br /><br>VUMC. This study will be submitted to the MEC of the VUMC. </p><br>