1. Early Feeding Practices in Infants with Phenylketonuria Across Europe
- Author
-
Karit Reinson, F. Lang, M.E. Dijsselhof, J. Żółkowska, K. Dokoupil, T.A.M. van den Hurk, W. Eberle-Pelloth, Anita MacDonald, Carolyn Dunlop, María A. Ruiz, D. Barrio-Carreras, T. Kozanoğlu, K. Vande Kerckhove, I. Jardim, Andrea Schlune, L. François, J. Wildgoose, C. Correia, A. Re Dionigi, A. De Theux, Bozena Didycz, S. De Leo, A. Skarpalezou, P. Manta-Vogli, K. Straczek, K. Chyż, A. Chrobot, H. Gokmen Ozel, Clara Vasconcelos, Maria Gizewska, Alex Pinto, Karen Corthouts, V. Velez García, M. Jörg-Streller, A. Belanger Quintana, C. Meneses, Barbara Cochrane, M.F. Almeida, K. Schulpis, C. Pedrón-Giner, R. Lilje, A. Grimsley, A.M.J. van Wegberg, T. Winkler, R. Hensler, Júlio César Rocha, G. Bruni, Louise Robertson, K. Plutowska-Hoffmann, M. Bueno-Delgado, N. Koç, Anne Daly, L. Fokkema, R. Pereira, K. Ahring, D. Garcia-Arenas, Andreas Jung, Martine Robert, S.M. Olivas, J. Serrano-Nieto, J. Saligova, S.M. Bernabei, Ulrike Och, E. Forssell, Jetta Tuokkola, R. Thom, I. Liegeois, J. Ekengren, C. Jouault, A. Gutiérrez-Sánchez, K. Lang, Camille Newby, Nur Arslan, U. Meyer, C. Joost, Moira French, C. Bontemps, H. Allen, M. Kanthe, Juri Zuvadelli, E. van Dam, A. Foucart, M. Van Driessche, I.L. Kok, A. De Meyer, J. Drabik, Carmen Rohde, Rachel Skeath, Sharon Evans, An Desloovere, C. Gingell, E.M.C. van der Ploeg, D. Mayr, E. Gyüre, Y. Atik Altınok, B. Kumru, O. Ļubina, A. Slabbert, Stefanie Rosenbaum-Fabian, G. Gugelmo, Claire Nicol, G. Caine, I. Errekalde, A. Liguori, Sandra Adams, A. Rossi, A. Tooke, R. Carvalho, J. Purves, C. Heller, M. Assoun, Carolina Gonçalves, K. Eftring, F. Boyle, A. Terry, S. Mexia, K. van Wyk, Ege Üniversitesi, HUS Children and Adolescents, Clinicum, University of Helsinki, HUS Internal Medicine and Rehabilitation, Children's Hospital, MUMC+: TPZ Dietetiek (9), RS: FHML non-thematic output, and UCL - (SLuc) Unité d'endocrinologie pédiatrique
- Subjects
0301 basic medicine ,Pediatrics ,PKU, Phenylketonuria ,Breastfeeding ,lnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4] ,IMD, Inherited Metabolic Disorders ,PROTEIN ,030105 genetics & heredity ,Phe-Free Infant Formula ,Endocrinology ,IMD ,Standard infant formula ,HDE PED ,Medicine ,Phenylketonuria ,lcsh:QH301-705.5 ,2. Zero hunger ,lcsh:R5-920 ,1184 Genetics, developmental biology, physiology ,Inherited Metabolic Disorders ,3. Good health ,PKU ,GROWTH ,lcsh:Medicine (General) ,DIETARY-MANAGEMENT ,Research Paper ,Phe ,medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,Phe, Phenylalanine ,Phenylalanine ,Breast milk ,03 medical and health sciences ,Phe-free infant formula ,Infant practices ,Genetics ,Weaning ,Molecular Biology ,Newborn screening ,Infant Practices ,business.industry ,Dietary management ,nutritional and metabolic diseases ,lcsh:Biology (General) ,Infant formula ,Human medicine ,business ,Breast feeding - Abstract
WOS: 000442229500021, PubMed ID: 30101073, 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 (R) 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 < 17 weeks in 3%. Discussion: This is the largest European survey completed on PKU infant feeding practices. It is evident that practices varied widely across Europe, and the practicalities of infant feeding in PKU received little focus in the PKU European Guidelines (2017). There are few reports comparing different feeding techniques with blood Phe control, Phe fluctuations and growth. Controlled prospective studies are necessary to assess how different infant feeding practices may influence longer term feeding development., Vitaflo, We thank Vitaflo for supporting the publication cost of this paper.
- Published
- 2018