The effect of slow releasing carbohydrates on the variability of plasma amino acid concentrations in PKU-patients in childhood.

Conditions: aangeboren stofwisselingsziekte
Phenylalanine hydroxylase deficiency
10037008

Registration Number: NL-OMON31050

Lead Sponsor: niversitair Medisch Centrum Groningen

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Pending

Sex: Not specified

Target Recruitment: 6

Inclusion Criteria

They are in good metabolic control, defined as a blood Phe concentration measurement within the target range of 120-360 µmol/l (Dutch PKU Advice Committee) one month prior to the study period. Apart from the PKU the patients are in a healthy state during the study periods. Their growth figures are within +/- 2 SD of the national growth charts. Patients take their amino acid supplementation divided into three portions each day. Participants / parents or caretakers are capable of routinely home skin puncturing without problems.

Exclusion Criteria

Blood Phe concentrations frequently beyond the target range, bad compliance in intake of the amino acid mixture, growth retardation > -2SD, obesity > +2SD, insufficient knowledge of the Dutch language of the caretaker, not taking blood samples at home

Study & Design

Study Type: Interventional

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Differences in blood Phe concentrations (and other amino acids?) between early<br /><br>morning (A) and end of the day (B) in the two study periods under the<br /><br>different conditions (without intervention, with SRC and with SRC) will be<br /><br>compared.</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Relationship between the severity of the disease defined as the individual Phe<br /><br>tolerance at 5 years of age, and the resulting mean and SD of blood Phe<br /><br>concentrations of each period will be tested with univariate linear regression<br /><br>analysis (ANOVA). </p><br>