Academic Journal
Early feeding practices in infants with phenylketonuria across Europe
Title: | Early feeding practices in infants with phenylketonuria across Europe |
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Authors: | A. Pinto, S. Adams, K. Ahring, H. Allen, M.F. Almeida, D. Garcia-Arenas, N. Arslan, M. Assoun, Y. Atik Altınok, D. Barrio-Carreras, A. Belanger Quintana, S.M. Bernabei, C. Bontemps, F. Boyle, G. Bruni, M. Bueno-Delgado, G. Caine, R. Carvalho, A. Chrobot, K. Chyż, B. Cochrane, C. Correia, K. Corthouts, A. Daly, S. De Leo, A. Desloovere, A. De Meyer, A. De Theux, B. Didycz, M.E. Dijsselhof, K. Dokoupil, J. Drabik, C. Dunlop, W. Eberle-Pelloth, K. Eftring, J. Ekengren, I. Errekalde, S. Evans, A. Foucart, L. Fokkema, L. François, M. French, E. Forssell, C. Gingell, C. Gonçalves, H. Gökmen Özel, A. Grimsley, G. Gugelmo, E. Gyüre, C. Heller, R. Hensler, I. Jardim, C. Joost, M. Jörg-Streller, C. Jouault, A. Jung, M. Kanthe, N. Koç, I.L. Kok, T. Kozanoğlu, B. Kumru, F. Lang, K. Lang, I. Liegeois, A. Liguori, R. Lilje, O. Ļubina, P. Manta-Vogli, D. Mayr, C. Meneses, C. Newby, U. Meyer, S. Mexia, C. Nicol, U. Och, S.M. Olivas, C. Pedrón-Giner, R. Pereira, K. Plutowska-Hoffmann, J. Purves, A. Re Dionigi, K. Reinson, M. Robert, L. Robertson, J.C. Rocha, C. Rohde, S. Rosenbaum-Fabian, A. Rossi, M. Ruiz, J. Saligova, A. Gutiérrez-Sánchez, A. Schlune, K. Schulpis, J. Serrano-Nieto, A. Skarpalezou, R. Skeath, A. Slabbert, K. Straczek, M. Giżewska, A. Terry, R. Thom, A. Tooke, J. Tuokkola, E. van Dam, T.A.M. van den Hurk, E.M.C. van der Ploeg, K. Vande Kerckhove, M. Van Driessche, A.M.J. van Wegberg, K. van Wyk, C. Vasconcelos, V. Velez García, J. Wildgoose, T. Winkler, J. Żółkowska, J. Zuvadelli, A. MacDonald |
Source: | Molecular Genetics and Metabolism Reports, Vol 16, Iss , Pp 82-89 (2018) |
Publisher Information: | Elsevier, 2018. |
Publication Year: | 2018 |
Collection: | LCC:Medicine (General) LCC:Biology (General) |
Subject Terms: | Medicine (General), R5-920, Biology (General), QH301-705.5 |
More Details: | Background: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe. Methods: We sent a cross sectional, survey monkey® questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions. Results: Ninety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months.53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, ≥26 weeks in 12% and |
Document Type: | article |
File Description: | electronic resource |
Language: | English |
ISSN: | 2214-4269 |
Relation: | http://www.sciencedirect.com/science/article/pii/S2214426918300831; https://doaj.org/toc/2214-4269 |
DOI: | 10.1016/j.ymgmr.2018.07.008 |
Access URL: | https://doaj.org/article/6dd963fe6ace47018cab1ed1c53da85c |
Accession Number: | edsdoj.6dd963fe6ace47018cab1ed1c53da85c |
Database: | Directory of Open Access Journals |
ISSN: | 22144269 |
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DOI: | 10.1016/j.ymgmr.2018.07.008 |
Published in: | Molecular Genetics and Metabolism Reports |
Language: | English |