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Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile

2015Journal article Open access
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dc.contributor.authorPena, M.
dc.contributor.authorAlmeida, M.
dc.contributor.authorvan Dam, E.
dc.contributor.authorAhring, K.
dc.contributor.authorBélanger-Quintana, A.
dc.contributor.authorDokoupil, K.
dc.contributor.authorGokmen-Ozel, H.
dc.contributor.authorLammardo, A.
dc.contributor.authorMacDonald, A.
dc.contributor.authorRobert, M.
dc.contributor.authorRocha, J.
dc.date.accessioned2016-07-26T12:26:25Z
dc.date.available2016-07-26T12:26:25Z
dc.date.issued2015
dc.description.abstractBackground 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.pt_PT
dc.identifier.citationOrphanet J Rare Dis. 2015;10:162pt_PT
dc.identifier.doi10.1186/s13023-015-0378-7pt_PT
dc.identifier.issn1750-1172
dc.identifier.urihttp://hdl.handle.net/10400.16/1973
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherBioMed Centralpt_PT
dc.relation.publisherversionhttp://ojrd.biomedcentral.com/articles/10.1186/s13023-015-0378-7pt_PT
dc.subjectDiet, Protein-Restrictedpt_PT
dc.subjectEuropept_PT
dc.subjectFood Labelingpt_PT
dc.subjectHumanspt_PT
dc.subjectPhenylketonuriaspt_PT
dc.subjectNutritive Valuept_PT
dc.titleSpecial low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profilept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceEnglandpt_PT
oaire.citation.issue1pt_PT
oaire.citation.startPage162pt_PT
oaire.citation.titleOrphanet Journal of Rare Diseasespt_PT
oaire.citation.volume10pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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