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Weaning practices in phenylketonuria vary between health professionals in Europe

2018; Elsevier BV; Volume: 18; Linguagem: Inglês

10.1016/j.ymgmr.2018.11.003

2214-4269

Alex Pinto, Sandra Adams, Kirsten Ahring, Heather Allen, Manuela Ferreira de Almeida, Dolores García-Arenas, Nur Arslan, Murielle Assoun, Yasemin Atik Altınok, D. Barrio‐Carreras, Alicia Quintana, S. Bernabei, C. Bontemps, F. Boyle, Giulia Innocenti Bruni, María Amor Bueno-Delgado, G. Caine, R. Carvalho, Agnieszka Chrobot, Katarzyna Chyż, Barbara Cochrane, Carla Correia, K. Corthouts, Anne Daly, Sabrina Leo, An Desloovere, A. De Meyer, A. De Theux, Bożena Didycz, M.E. Dijsselhof, Katharina Dokoupil, Józef Drabik, Carolyn Dunlop, W. Eberle-Pelloth, K. Eftring, J. Ekengren, I. Errekalde, Sharon Evans, A. Foucart, L. Fokkema, Luís Acao Francois, Moira French, E. Forssell, C. Gingell, C. Gonçalves, Hülya Gökmen Özel, Anne Grimsley, Giorgia Gugelmo, E. Gyüre, C. Heller, R. Hensler, I. Jardim, C. Joost, Monika Jörg‐Streller, C. Jouault, Alexandra Jung, M. Kanthe, Nevra Koç, Irene L. Kok, Tuğba Kozanoğlu, Burcu Kumru, Frauke Lang, Karen A. Lang, I. Liegeois, A. Liguori, R. Lilje, Olga Ļubina, Penelope D. Manta-Vogli, Doris Mayr, Clarice Franco Meneses, Camille Newby, U. Meyer, S. Mexia, Claire Nicol, Ulrike Och, S.M. Olivas, Consuelo Pedrón‐Giner, Renata Junqueira Pereira, K. Plutowska-Hoffmann, Janet Purves, Alice Re Dionigi, Karit Reinson, Marianne Robert, Louise Robertson, Júlio César Rocha, Carmen Rohde, Stefanie Rosenbaum‐Fabian, Alfio Rossi, M. Ruiz, Jana Šaligová, A. Gutiérrez-Sánchez, Andrea Schlune, Kleopatra H. Schulpis, Juliana Serrano-Nieto, Astrinia Skarpalezou, Rachel Skeath, A. Slabbert, Kamilla Strączek, Maria Giżewska, Allyson Terry, Ruth Thom, Alison Tooke, Jetta Tuokkola, Esther van Dam, T.A.M. van den Hurk, E.M.C. van der Ploeg, K. Vande Kerckhove, Marleen Van den Driessche, Annemiek M. J. van Wegberg, K. van Wyk, Carla Maria Lins de Vasconcelos, V. Velez García, J. Wildgoose, Tina Winkler, Joanna Żółkowska, Juri Zuvadelli, Anita MacDonald,

Mitochondrial Function and Pathology

In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe. A cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis. Weaning started at 17–26 weeks in 85% (n = 81/95) of centres, >26 weeks in 12% (n = 11/95) and < 17 weeks in 3% (n = 3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% (n = 48/95) of centres introduced Phe containing foods at 17–26 weeks and 48% (n = 46/95) at >26 weeks. First solids were mainly low Phe vegetables (59%, n = 56/95) and fruit (34%, n = 32/95). A Phe exchange system to allocate dietary Phe was used by 52% (n = 49/95) of centres predominantly from Northern and Southern Europe and 48% (n = 46/95) calculated most Phe containing food sources (all centres in Eastern Europe and the majority from Germany and Austria). Some centres used a combination of both methods. A second stage Phe-free L-amino acid supplement containing a higher protein equivalent was introduced by 41% (n = 39/95) of centres at infant age 26–36 weeks (mainly from Germany, Austria, Northern and Eastern Europe) and 37% (n = 35/95) at infant age > 1y mainly from Southern Europe. 53% (n = 50/95) of centres recommended a second stage Phe-free L-amino acid supplement in a spoonable or semi-solid form. Weaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.