Prescribed intake of micronutrients and fatty acids in patients with phenylketonuria, and effects on blood micronutrient and fatty acid levels, bone mass and bone turnover.
Completed
- Conditions
- PhenylketonuriaPKU1002742410037008
- Registration Number
- NL-OMON38467
- Lead Sponsor
- Academisch Medisch Centrum
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Completed
- Sex
- Not specified
- Target Recruitment
- 80
Inclusion Criteria
This study includes patients with PKU, age of 1 year and older, diagnosed by newborn screening and treated with a protein restricted diet with supplementation of an amino acid mixture, recruited from three academic metabolic centers in the Netherlands. Patients treated with sapropterin are also included, with or without dietary treatment.
Exclusion Criteria
Significant changes in the type or amount of amino acid supplements used, within one month before the inclusion date.
Pregnancy.
Study & Design
- Study Type
- Observational invasive
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method <p>- To asses the prescribed micronutrient and fatty acid intake of patients with<br /><br>PKU treated with a protein restricted diet with supplementation of amino acids<br /><br>in a mixture with vitamins and minerals, or sapropterin.<br /><br>- To correlate the prescribed intake of micronutrients and fatty acids from the<br /><br>diet supplemented with an amino acid mixture, or sapropterin, to blood levels<br /><br>of micronutrients and fatty acids.</p><br>
- Secondary Outcome Measures
Name Time Method <p>- To assess the BMD and bone turnover in patients with PKU treated with a<br /><br>protein restricted diet with supplementation of amino acids in a mixture with<br /><br>vitamins and minerals, or sapropterin.<br /><br>- To correlate the prescribed intake of micronutrients and fatty acids from a<br /><br>protein restricted diet supplemented with an amino acid mixture, or<br /><br>sapropterin, to BMD and bone turnover.<br /><br>- To correlate blood levels of micronutrients and fatty acids in patients with<br /><br>phenylketonuria to BMD and bone turnover.</p><br>