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

Orphanet Journal of Rare Diseases

Júlio César Rocha⁴  Martine Robert⁸  Anita MacDonald⁵  Anna Maria Lammardo²  Hulya Gokmen-Ozel^1,11  Katharina Dokoupil⁹  Amaya Bélanger-Quintana⁶  Kirsten Ahring³  Esther van Dam⁷  Manuela Ferreira Almeida^1,10  Maria João Pena¹ 

[1] Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Praça Pedro Nunes, 88, Porto, 4099-028, Portugal;Department of Pediatrics, San Paolo Hospital University of Milan, Milan, Italy;Department of PKU, Kennedy Centre, Glostrup, Denmark;Center for Health Technology and Services Research (CINTESIS), Porto, Portugal;The Children’s Hospital, Birmingham, UK;Unidad Enfermedades Metabolicas Servicio de Pediatria Hospital Ramon y Cajal, Madrid, Spain;Beatrix Children’s Hospital, University of Groningen, University Medical Center’ Groningen Netherlands, Groningen, Netherlands;Nutrition and Metabolism Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium;Department of Metabolism and Nutrition, Dr. von Hauner Children’s Hospital, University of Munich, Munich, Germany;Unit for Multidisciplinary Research in Biomedicine, Abel Salazar Institute of Biomedical Sciences, University of Porto-UMIB/ICBAS/UP, Porto, Portugal;Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey

关键词: Europe;    Nutritional composition;    Availability;    Special low protein foods;    Phenylketonuria;   

Others  :  1235012
DOI  :  10.1186/s13023-015-0378-7

 received in 2015-10-12, accepted in 2015-12-14,  发布年份 2015

【 摘 要 】

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.

【 授权许可】

   
2015 Pena et al.

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