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Six-year follow up of phenylalanine intakes and plasma phenylalanine concentrations
U Wendel1, K Ullrich, H Schmidt
1Heinrich Heine Universität Düsseldorf, Zentrum für Kinderheilkunde, Federal Republic of Germany.
Insights
This study evaluated the dietary phenylalanine (Phe) intake for children with phenylketonuria (PKU). Controlled Phe intake is crucial for managing PKU and ensuring healthy growth in treated patients.
Area of Science:
- Biochemistry
- Pediatric Nutrition
- Metabolic Disorders
Background:
- Phenylketonuria (PKU) is a genetic disorder requiring lifelong dietary management.
- Maintaining controlled plasma phenylalanine levels is essential for neurodevelopment in PKU patients.
- Understanding optimal phenylalanine intake is critical for growth in treated children.
Purpose of the Study:
- To assess the daily phenylalanine (Phe) intake in normally growing, treated pediatric PKU patients aged 1-6 years.
- To determine the relationship between Phe intake and plasma Phe levels in this cohort.
- To establish reference ranges for Phe intake necessary for optimal growth and metabolic control.
Main Methods:
- Longitudinal evaluation of daily phenylalanine intake in grams per kilogram of body weight.
- Monitoring of protein intake, which varied from 2.26 g/kg/day at 6 months to 1.81 g/kg/day at 72 months.
- Measurement of plasma phenylalanine levels to ensure they remained around a target of 6.0 mg/dL.
Main Results:
- Mean phenylalanine intake decreased from 34 mg/kg/day at 6 months to 15 mg/kg/day at 72 months.
- This declining Phe intake was necessary to maintain target plasma Phe levels.
- The study confirms the need for adjusted Phe intake as children with PKU grow.
Conclusions:
- Dietary management of PKU requires careful adjustment of phenylalanine intake throughout childhood.
- Controlled Phe intake is achievable and essential for healthy growth and metabolic control in treated PKU patients.
- These findings provide valuable data for nutritional guidelines in pediatric PKU management.
Abstract:
The daily Phe intakes of normally growing 1- to 6-year-old treated PKU patients were evaluated. The children received protein in amounts that varied from 2.26 +/- 0.47 g/kg body weight per day (mean +/- SD) at the age of 6 to 1.81 +/- 0.35 at the age of 72 months. Mean Phe intakes declining from 34 +/- 7 at the age of 6 months to 15 +/- 5 mg/kg body weight per day at the age of 72 months were required to maintain mean median plasma Phe levels around 6.0 mg/dl.