Protein substitutes for phenylketonuria in Europe: access and nutritional composition.
Protein substitutes (PS) are an essential component in the dietary management of phenylketonuria (PKU). PS are available as phenylalanine-free amino-acid mixtures (AAM), glycomacropeptide-based PS (GMP) and large neutral amino acids (LNAA). There is a lack of information regarding their availability in different countries and comparison of their nutritional composition is limited. The objectives of this study were to identify the number of PS available in different European countries and Turkey and to compare their nutritional composition.



Members of the European Nutritionist Expert Panel on PKU (ENEP) (Portugal, Spain, Belgium, Italy, Germany, Netherlands, United Kingdom, Denmark and Turkey) provided data on PS available in each country. The nutritional composition of PS available in Portugal was analyzed.



The number of PS available in each country varied from 30 (Turkey) to 105 (Germany), with a median of 64. GMP was available only in Portugal, whereas LNAA was an option in Portugal, Italy, Turkey and Denmark. Some PS were designed for weaning. Many PS did not contain added fat and fiber. GMP contained the highest carbohydrate (CHO) and energy content as well as higher LNAA content compared with AAM. Only one AAM contained added fructo-oligosaccharides and galacto-oligosaccharides. AAM designed for the first year of life had the highest CHO, fat and LNAA contribution. Liquid AAM had lower CHO and fat contents compared with powdered AAM, but contained higher LNAA.



There was widely dissimilar numbers of PS available in different countries. Nutritional composition of different PS was variable and should be considered before prescription.
Centro de Genética Médica, Centro Hospitalar do Porto (CHP), Porto, Portugal.
M J
MJ
Pena
Centro de Genética Médica, Centro Hospitalar do Porto (CHP), Porto, Portugal.|Unit for Multidisciplinary Research in Biomedicine (UMIB), Instituto de Ciências Biomédicas Abel Salazar (ICBAS), University of Porto, Porto, Portugal.
M F
MF
de Almeida
University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Groningen, The Netherlands.
E
E
van Dam
Department of PKU, Kennedy Centre, Copenhagen University Hospital, Glostrup, Denmark.
K
K
Ahring
Unidad Enfermedades Metabolicas Servicio de Pediatria Hospital Ramon y Cajal, Madrid, Spain.
A
A
Bélanger-Quintana
Department of Metabolism and Nutrition, Dr von Hauner Children's Hospital, University of Munich, Munich, Germany.
K
K
Dokoupil
Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey.
H
H
Gokmen-Ozel
Department of Pediatrics, San Paolo Hospital University of Milan, Milan, Italy.
A M
AM
Lammardo
The Children's Hospital, Birmingham, UK.
A
A
MacDonald
Nutrition and Metabolism Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium.
M
M
Robert
Centro de Genética Médica, Centro Hospitalar do Porto (CHP), Porto, Portugal.|Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Porto, Portugal.|Center for Health Technology and Services Research (CINTESIS), Porto, Portugal.
J C
JC
Rocha
D000596:Amino Acids / Q000032:analysis / Q000627:therapeutic use*; D021542:Amino Acids, Neutral / Q000032:analysis / Q000627:therapeutic use; D002364:Caseins / Q000737:chemistry / Q000627:therapeutic use; D004044:Dietary Proteins / Q000737:chemistry / Q000627:therapeutic use*; D005060:Europe; D005526:Food, Formulated / Q000032:analysis / Q000600:supply & distribution*; D006801:Humans; D010446:Peptide Fragments / Q000737:chemistry / Q000627:therapeutic use; D010649:Phenylalanine; D010661:Phenylketonurias / Q000178:diet therapy*; D014421:Turkey
D000596:Amino Acids; D021542:Amino Acids, Neutral; D002364:Caseins; D004044:Dietary Proteins; D010446:Peptide Fragments; C065989:caseinomacropeptide; D010649:Phenylalanine