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The Diet for Life study.

Recruiting
Conditions
1. PKU
<br />2. Phenylalanine
<br />3. Diet
<br />4. Neuropsychological function
<br />5. Wellbeing.
Registration Number
NL-OMON23483
Lead Sponsor
AMC
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Recruiting
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
NameTimeMethod
The neuropsychological deficit profile of adults with treated PKU. <br /><br>1. What are the relative strengths and weaknesses in the neurocognitive functioning of adults with treated PKU?;<br /><br>2. Does supplementation of Phe to plasma levels comparable to levels observed in adult patients who fully discontinued their diet, influence neurocognitive functioning, and/or other disease parameters?<br>
Secondary Outcome Measures
NameTimeMethod
The neurophysiological mechanisms responsible for neuropsychological dysfunction in adults with treated PKU.<br /><br>1. Can abnormal functional networks be demonstrated in adults with treated PKU?;<br /> <br>2. Is network disruption more severe following the intervention-related increase in phenylalanine level?;<br /><br>3. Is there a correlation between the nature and severity of neuropsychological dysfunction in adults with treated PKU and the nature and severity of network disruption in these subjects?<br>
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