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. 2015 Dec 22:10:162.
doi: 10.1186/s13023-015-0378-7.

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

Maria João Pena  1 , Manuela Ferreira Almeida  2   3 , Esther van Dam  4 , Kirsten Ahring  5 , Amaya Bélanger-Quintana  6 , Katharina Dokoupil  7 , Hulya Gokmen-Ozel  8 , Anna Maria Lammardo  9 , Anita MacDonald  10 , Martine Robert  11 , Júlio César Rocha  12   13   14
Affiliations

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

Maria João Pena et al. Orphanet J Rare Dis. .

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.

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