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Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile
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 av...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4688958/ https://www.ncbi.nlm.nih.gov/pubmed/26693706 http://dx.doi.org/10.1186/s13023-015-0378-7 |
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author | Pena, Maria João Almeida, Manuela Ferreira van Dam, Esther Ahring, Kirsten Bélanger-Quintana, Amaya Dokoupil, Katharina Gokmen-Ozel, Hulya Lammardo, Anna Maria MacDonald, Anita Robert, Martine Rocha, Júlio César |
author_facet | Pena, Maria João Almeida, Manuela Ferreira van Dam, Esther Ahring, Kirsten Bélanger-Quintana, Amaya Dokoupil, Katharina Gokmen-Ozel, Hulya Lammardo, Anna Maria MacDonald, Anita Robert, Martine Rocha, Júlio César |
author_sort | Pena, Maria João |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-4688958 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-46889582015-12-24 Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile Pena, Maria João Almeida, Manuela Ferreira van Dam, Esther Ahring, Kirsten Bélanger-Quintana, Amaya Dokoupil, Katharina Gokmen-Ozel, Hulya Lammardo, Anna Maria MacDonald, Anita Robert, Martine Rocha, Júlio César Orphanet J Rare Dis Research 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. BioMed Central 2015-12-22 /pmc/articles/PMC4688958/ /pubmed/26693706 http://dx.doi.org/10.1186/s13023-015-0378-7 Text en © Pena et al. 2015 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Pena, Maria João Almeida, Manuela Ferreira van Dam, Esther Ahring, Kirsten Bélanger-Quintana, Amaya Dokoupil, Katharina Gokmen-Ozel, Hulya Lammardo, Anna Maria MacDonald, Anita Robert, Martine Rocha, Júlio César Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile |
title | Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile |
title_full | Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile |
title_fullStr | Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile |
title_full_unstemmed | Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile |
title_short | Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile |
title_sort | special low protein foods for phenylketonuria: availability in europe and an examination of their nutritional profile |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4688958/ https://www.ncbi.nlm.nih.gov/pubmed/26693706 http://dx.doi.org/10.1186/s13023-015-0378-7 |
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