title: Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile.
abstract: BACKGROUND
  Special low protein foods (SLPF) are essential in the nutritional management of patients with phenylketonuria (PKU). The study objectives were to: 1) identify the number of SLPF available for use in eight European countries and Turkey and 2) analyse the nutritional composition of SLPF available in one of these countries.
  
  
  METHODS
  European Nutritionist Expert Panel on PKU (ENEP) members (Portugal, Spain, Belgium, Italy, Germany, Netherlands, UK, Denmark and Turkey) provided data on SPLF available in each country. The nutritional composition of Portuguese SLPF was compared with regular food products.
  
  
  RESULTS
  The number of different SLPF available in each country varied widely with a median of 107 [ranging from 73 (Portugal) and 256 (Italy)]. Food analysis of SLPF available from a single country (Portugal) indicated that the mean phenylalanine content was higher in low protein baby cereals (mean 48 mg/100 g) and chocolate/energy bars/jelly (mean 41 mg/100 g). The energy content of different foods from a sub-group of SLPF (cookies) varied widely between 23 and 96 kcal/cookie. Low protein bread had a high fat content [mean 5.8 g/100 g (range 3.7 to 10)] compared with 1.6 g/100 g in regular bread. Seven of the 12 SLPF sub-groups (58 %) did not declare any vitamin content, and only 4 (33 %) identified a limited number of minerals.
  
  
  CONCLUSIONS
  Whilst equal and free access to all SLPF is desirable, the widely variable nutritional composition requires careful nutritional knowledge of all products when prescribed for individual patients with PKU. There is a need for more specific nutritional standards for special low protein foods.
authors.affiliation: Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Praça Pedro Nunes, 88, 4099-028, Porto, Portugal. mariajoao0502@gmail.com.
authors.forename: Maria João
authors.initials: MJ
authors.lastname: Pena
authors.affiliation: Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Praça Pedro Nunes, 88, 4099-028, Porto, Portugal. manuela.almeida@chporto.min-saude.pt.|Unit for Multidisciplinary Research in Biomedicine, Abel Salazar Institute of Biomedical Sciences, University of Porto-UMIB/ICBAS/UP, Porto, Portugal. manuela.almeida@chporto.min-saude.pt.
authors.forename: Manuela Ferreira
authors.initials: MF
authors.lastname: Almeida
authors.affiliation: Beatrix Children's Hospital, University of Groningen, University Medical Center' Groningen Netherlands, Groningen, Netherlands. e.van.dam@umcg.nl.
authors.forename: Esther
authors.initials: E
authors.lastname: van Dam
authors.affiliation: Department of PKU, Kennedy Centre, Glostrup, Denmark. kirstenahring@hotmail.com.
authors.forename: Kirsten
authors.initials: K
authors.lastname: Ahring
authors.affiliation: Unidad Enfermedades Metabolicas Servicio de Pediatria Hospital Ramon y Cajal, Madrid, Spain. amaya.belanger@salud.madrid.org.
authors.forename: Amaya
authors.initials: A
authors.lastname: Bélanger-Quintana
authors.affiliation: Department of Metabolism and Nutrition, Dr. von Hauner Children's Hospital, University of Munich, Munich, Germany. kathi.dokupil@med.uni-muenchen.de.
authors.forename: Katharina
authors.initials: K
authors.lastname: Dokoupil
authors.affiliation: Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey. hgokmen@hacettepe.edu.tr.
authors.forename: Hulya
authors.initials: H
authors.lastname: Gokmen-Ozel
authors.affiliation: Department of Pediatrics, San Paolo Hospital University of Milan, Milan, Italy. annamaria.lammardo@tin.it.
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Lammardo
authors.affiliation: The Children's Hospital, Birmingham, UK. Anita.Macdonald@bch.nhs.uk.
authors.forename: Anita
authors.initials: A
authors.lastname: MacDonald
authors.affiliation: Nutrition and Metabolism Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium. martine.robert@huderf.be.
authors.forename: Martine
authors.initials: M
authors.lastname: Robert
authors.affiliation: Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Praça Pedro Nunes, 88, 4099-028, Porto, Portugal. julio.rocha@chporto.min-saude.pt.|Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Porto, Portugal. julio.rocha@chporto.min-saude.pt.|Center for Health Technology and Services Research (CINTESIS), Porto, Portugal. julio.rocha@chporto.min-saude.pt.
authors.forename: Júlio César
authors.initials: JC
authors.lastname: Rocha
mesh_terms: D018753:Diet, Protein-Restricted / Q000592:standards*; D005060:Europe; D005515:Food Labeling / Q000592:standards; D006801:Humans; D009753:Nutritive Value; D010661:Phenylketonurias / Q000178:diet therapy*