Early Feeding Practices in Infants with Phenylketonuria Across Europe

Autor: 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
Přispěvatelé: 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, UCL - (SLuc) Unité d'endocrinologie pédiatrique
Jazyk: angličtina
Rok vydání: 2018
Předmět:
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
Zdroj: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Agência para a Sociedade do Conhecimento (UMIC)-FCT-Sociedade da Informação
instacron:RCAAP
Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports, 16, pp. 82-89
Molecular Genetics and Metabolism Reports, 16, 82. Elsevier BV
Repositorio Institucional de la Consejería de Sanidad de la Comunidad de Madrid
Consejería de Sanidad de la Comunidad de Madrid
Molecular Genetics and Metabolism Reports, 16, 82-89. Elsevier Science
Molecular Genetics and Metabolism Reports, Vol. 16, no. ?, p. 82-89 (2018)
Molecular Genetics and Metabolism Reports, 16, 82-89
Molecular Genetics And Metabolism Reports
r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
instname
Molecular genetics and metabolism reports, 16, 82-89. ELSEVIER SCIENCE BV
Molecular Genetics and Metabolism Reports, Vol 16, Iss, Pp 82-89 (2018)
ISSN: 2214-4269
Popis: 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.
Databáze: OpenAIRE
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