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

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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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Most cited references 7

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Nutritional Management of Phenylketonuria

Erin MacLeod, Denise M Ney (2010)

Phenylketonuria (PKU) is caused by deficient activity of the enzyme phenylalanine hydroxylase, needed to convert the essential amino acid (AA) phenylalanine (phe) to tyrosine. In order to prevent neurological damage, lifelong adherence to a low-phe diet that is restricted in natural foods and requires ingestion of a phe-free AA formula to meet protein needs is required. The goal of nutritional management for those with PKU is to maintain plasma phe concentrations that support optimal growth, development, and mental functioning while providing a nutritionally complete diet. This paper reviews developing a lifelong dietary prescription for those with PKU, outcomes of nutritional management, compliance with the low-phe diet across the life cycle, and new options for nutritional management. An individualized dietary prescription is needed to meet nutrient requirements, and the adequacy of phe intake is monitored with assessment of blood phe levels. Elevated phe concentrations may occur due to illness, excessive or inadequate phe intake, or inadequate intake of AA formula. Although normal growth and development occurs with adherence to the low-phe diet, it is important to monitor vitamin, mineral and essential fatty acid status, especially in those who do not consume sufficient AA formula. Given the growing population of adults with PKU, further research is needed to understand the risks for developing osteoporosis and cardiovascular disease. There are promising new options to liberalize the diet and improve metabolic control such as tetrahydrobiopterin therapy or supplementation with large neutral AAs. Moreover, foods made with glycomacropeptide, an intact protein that contains minimal phe, improves the PKU diet by offering a palatable alternative to AA formula. In summary, continued efforts are needed to overcome the biggest challenge to living with PKU – lifelong adherence to the low-phe diet.

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Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome.

C Oel, Claudia T Guimaraes, George Rocha … (2012)

Little is known about the consequences of the special energy enriched diet used to treat patients with phenylketonuria (PKU) in terms of obesity and metabolic syndrome (MetSyn) development. To investigate the prevalence of overweight and obesity, and its consequences in terms of body composition and MetSyn in early treated patients with PKU compared to controls. A sample of 89 patients with PKU (3-30 y; 14.4±6.6 y) and 79 controls (3-47 y; 16.3±7.9 y) were studied. In the fasted state, anthropometric, body composition, blood pressure and analytical parameters [amino acids, glucose, insulin, total and HDL-cholesterol (HDL-c), triglycerides (TG), high sensitivity c-reactive protein and uric acid] were performed. Data on dietary intake was collected. BMI was classified using WHO criteria, while the definition from International Diabetes Federation (IDF) was used for MetSyn. Prevalence of overweight and obesity (32.6% vs. 24.1%; p=0.293), body fat percentage (22% vs. 23.1%, p=0.581) and central obesity (36.9% vs. 36.4%, p=0.999) were comparable to controls. Patients revealed a higher TG/HDL-c (p<0.001). The prevalence of MetSyn was 1.5% and 6.1% in patients and controls, respectively. Patients and not controls with central obesity revealed a further significant increase in TG/HDL-c compared with those without central obesity (p=0.023). Patients and controls were similar in terms of overweight and obesity, body composition and MetSyn. However, the dyslipidemia in patients with PKU in relation to overweight and obesity may help us trying to understand the course and the etiology of MetSyn not only in PKU but also in the general population. Copyright © 2012 Elsevier Inc. All rights reserved.

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Is overweight an issue in phenylketonuria?

Bruno C L M Rocha, Anita MacDonald, Friedrich Trefz (2012)

Dietary treatment may be associated with an increased risk of obesity in phenylketonuria (PKU). The earliest studies describe a tendency for overweight in PKU, but not all recent publications confirm this, although there are an increasing number of studies describing increased obesity in female patients with PKU. There is little data describing the metabolic consequences of obesity in PKU. It is difficult to interpret and compare published results due to variable patient age, differing dietary treatment approaches, poor treatment adherence, inconsistencies in metabolic control achieved, variable criteria used to classify overweight. There is also a lack of comparison with normal population data which is widely variable between countries. Generally in PKU it is unknown if obesity etiology is a result of the underlying condition, a treatment consequence, or an outcome of inadequate metabolic control. Differences in treatment strategies, target ranges for blood phenylalanine concentrations and severity of PKU can alter nutritional intakes and dietary experiences which ultimately modulate the course of overweight development. It is clear further investigation is required. Treating overweight and obesity in the general population is difficult and no studies have described the impact of obesity treatment strategies in PKU. However, the PKU management team has an important role in monitoring nutritional status and preventing overweight and obesity. It is important that PKU treatment attends to the general aspects of nutrition, feeding behavior and exercise in order to prevent the development of overweight in these individuals. © 2013.

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Author and article information

Contributors

Maria João Pena: mariajoao0502@gmail.com

Manuela Ferreira Almeida: manuela.almeida@chporto.min-saude.pt

Esther van Dam: e.van.dam@umcg.nl

Kirsten Ahring: kirstenahring@hotmail.com

Amaya Bélanger-Quintana: amaya.belanger@salud.madrid.org

Katharina Dokoupil: kathi.dokupil@med.uni-muenchen.de

Hulya Gokmen-Ozel: hgokmen@hacettepe.edu.tr

Anna Maria Lammardo: annamaria.lammardo@tin.it

Anita MacDonald: Anita.Macdonald@bch.nhs.uk

Martine Robert: martine.robert@huderf.be

Júlio César Rocha: +351 22 6070 339 , julio.rocha@chporto.min-saude.pt

Journal

Journal ID (nlm-ta): Orphanet J Rare Dis

Journal ID (iso-abbrev): Orphanet J Rare Dis

Title: Orphanet Journal of Rare Diseases

Publisher: BioMed Central (London )

ISSN (Electronic): 1750-1172

Publication date (Electronic): 22 December 2015

Publication date PMC-release: 22 December 2015

Publication date Collection: 2015

Volume: 10

Electronic Location Identifier: 162

Affiliations

[ ]Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Praça Pedro Nunes, 88, 4099-028 Porto, Portugal

[ ]Unit for Multidisciplinary Research in Biomedicine, Abel Salazar Institute of Biomedical Sciences, University of Porto-UMIB/ICBAS/UP, Porto, Portugal

[ ]Beatrix Children’s Hospital, University of Groningen, University Medical Center’ Groningen Netherlands, Groningen, Netherlands

[ ]Department of PKU, Kennedy Centre, Glostrup, Denmark

[ ]Unidad Enfermedades Metabolicas Servicio de Pediatria Hospital Ramon y Cajal, Madrid, Spain

[ ]Department of Metabolism and Nutrition, Dr. von Hauner Children’s Hospital, University of Munich, Munich, Germany

[ ]Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey

[ ]Department of Pediatrics, San Paolo Hospital University of Milan, Milan, Italy

[ ]The Children’s Hospital, Birmingham, UK

[ ]Nutrition and Metabolism Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium

[ ]Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Porto, Portugal

[ ]Center for Health Technology and Services Research (CINTESIS), Porto, Portugal

Article

Publisher ID: 378

DOI: 10.1186/s13023-015-0378-7

PMC ID: 4688958

PubMed ID: 26693706

SO-VID: 85a7bfbb-fd80-4a18-8aaf-272d3fc55d36

License:

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.

History

Date received : 12 October 2015

Date accepted : 14 December 2015

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ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: phenylketonuria,special low protein foods,availability,nutritional composition,europe

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ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: phenylketonuria, special low protein foods, availability, nutritional composition, europe

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

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Abstract

Background

Methods

Results

Conclusions

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Microbiology Society: Coronaviruses

Most cited references 7

Nutritional Management of Phenylketonuria

Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome.

Is overweight an issue in phenylketonuria?

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