Effect of Special Low-Protein Foods Consumption in the Dietary Pattern and Biochemical Profile of Patients with Inborn Errors of Protein Metabolism: Application of a Database of Special Low-Protein Foods
Dolores Garcia-Arenas,
Blanca Barrau-Martinez,
Arnau Gonzalez-Rodriguez,
Rafael Llorach,
Jaume Campistol-Plana,
Angeles García-Cazorla,
Aida Ormazabal,
Mireia Urpi-Sarda
Affiliations
Dolores Garcia-Arenas
Nutrition, Food Science and Gastronomy Department, Xarxa d’Innovació Alimentària (XIA), Faculty of Pharmacy and Food Science, Food Science and Nutrition Torribera Campus, University of Barcelona, Av. Prat de la Riba 171, 08921 Santa Coloma de Gramenet, Barcelona, Spain
Blanca Barrau-Martinez
Nutrition, Food Science and Gastronomy Department, Xarxa d’Innovació Alimentària (XIA), Faculty of Pharmacy and Food Science, Food Science and Nutrition Torribera Campus, University of Barcelona, Av. Prat de la Riba 171, 08921 Santa Coloma de Gramenet, Barcelona, Spain
Arnau Gonzalez-Rodriguez
Nutrition, Food Science and Gastronomy Department, Xarxa d’Innovació Alimentària (XIA), Faculty of Pharmacy and Food Science, Food Science and Nutrition Torribera Campus, University of Barcelona, Av. Prat de la Riba 171, 08921 Santa Coloma de Gramenet, Barcelona, Spain
Rafael Llorach
Nutrition, Food Science and Gastronomy Department, Xarxa d’Innovació Alimentària (XIA), Faculty of Pharmacy and Food Science, Food Science and Nutrition Torribera Campus, University of Barcelona, Av. Prat de la Riba 171, 08921 Santa Coloma de Gramenet, Barcelona, Spain
Jaume Campistol-Plana
Inborn Errors of Metabolism Unit, Sant Joan de Déu Hospital, Passeig Sant Joan de Déu 2, 08950 Esplugues de Llobregat, Barcelona, Spain
Angeles García-Cazorla
Inborn Errors of Metabolism Unit, Sant Joan de Déu Hospital, Passeig Sant Joan de Déu 2, 08950 Esplugues de Llobregat, Barcelona, Spain
Aida Ormazabal
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III, 28029 Madrid, Spain
Mireia Urpi-Sarda
Nutrition, Food Science and Gastronomy Department, Xarxa d’Innovació Alimentària (XIA), Faculty of Pharmacy and Food Science, Food Science and Nutrition Torribera Campus, University of Barcelona, Av. Prat de la Riba 171, 08921 Santa Coloma de Gramenet, Barcelona, Spain
In inborn errors of intermediate protein metabolism (IEM), the effect of special low-protein foods (SLPFs) on dietary intake has been scarcely studied. The aim of this study was to compare the nutritional profile of SLPFs with usual foods and to assess whether their intake determines the dietary pattern and affects the plasma biochemical profile in children with IEMs with different protein restrictions. A database with the nutritional composition of 250 SLPFs was created. A total of 59 children with IEMs were included in this cross-sectional observational study. The greatest significant differences in macronutrient composition were observed between dairy, meat, fish, and egg SLPFs and regular foods. After stratifying subjects by SLPFs, the participants with the highest intake (>32%) had a higher total energy intake and lower intake of natural protein than those in the lowest tertile (p 5%) showed a higher intake of sugars, total and saturated fats, and higher plasma levels of total and low-density lipoprotein cholesterol than those in the first tertile (p < 0.05). The variability in the nutritional composition of SLPFs highlights the need for up-to-date databases which would greatly assist in optimizing individualized recommendations for children with IEMs and protein restrictions.