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Protein substitutes for phenylketonuria in Europe: access and nutritional composition
M J Pena1, M F de Almeida1,2, E van Dam3
1Centro de Genética Médica, Centro Hospitalar do Porto (CHP), Porto, Portugal.
European Journal of Clinical Nutrition
|April 28, 2016
Summary
Protein substitutes (PS) for phenylketonuria (PKU) vary widely in availability and nutritional content across European countries and Turkey. Careful consideration of these factors is crucial for effective dietary management.
Area of Science:
- Nutritional Science
- Metabolic Disorders
- Public Health Nutrition
Background:
- Protein substitutes (PS) are vital for managing phenylketonuria (PKU).
- Available PS types include amino acid mixtures (AAM), glycomacropeptide (GMP)-based PS, and large neutral amino acids (LNAA).
- Limited data exists on PS availability and nutritional composition across different countries.
Purpose of the Study:
- To determine the number of PS available in various European countries and Turkey.
- To compare the nutritional composition of available PS.
Main Methods:
- Data on PS availability was collected from the European Nutritionist Expert Panel on PKU (ENEP) members across nine countries and Turkey.
- Nutritional composition analysis was performed on PS available in Portugal.
Main Results:
- The number of available PS varied significantly, ranging from 30 in Turkey to 105 in Germany (median 64).
- Glycomacropeptide-based PS were only available in Portugal, while LNAA options existed in Portugal, Italy, Turkey, and Denmark.
- Nutritional profiles differed, with GMP having higher carbohydrate and energy content than AAM. Infant-specific AAM and liquid AAM showed distinct nutritional contributions.
Conclusions:
- Significant disparities exist in the availability of protein substitutes across different countries.
- The variable nutritional composition of protein substitutes necessitates careful consideration prior to prescription for phenylketonuria management.
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