Weaning practices in phenylketonuria vary between health professionals in Europe

Pinto A, Adams S, Ahring K, Allen H, Almeida MF, Garcia-Arenas D, Arslan N, Assoun M, Atik Altınok Y, Barrio-Carreras D, Belanger Quintana A, Bernabei SM, Bontemps C, Boyle F, Bruni G, Bueno-Delgado M, Caine G, Carvalho R, Chrobot A, Chyż K, Cochrane B, Correia C, Corthouts K, Daly A, De Leo S, Desloovere A, De Meyer A, De Theux A, Didycz B, Dijsselhof ME, Dokoupil K, Drabik J, Dunlop C, Eberle-Pelloth W, Eftring K, Ekengren J, Errekalde I, Evans S, Foucart A, Fokkema L, François L, French M, Forssell E, Gingell C, Gonçalves C, Gökmen Özel H, Grimsley A, Gugelmo G, Gyüre E, Heller C, Hensler R, Jardim I, Joost C, Jörg-Streller M, Jouault C, Jung A, Kanthe M, Koç N, Kok IL, Kozanoğlu T, Kumru B, Lang F, Lang K, Liegeois I, Liguori A, Lilje R, Ļubina O, Manta-Vogli P, Mayr D, Meneses C, Newby C, Meyer U, Mexia S, Nicol C, Och U, Olivas SM, Pedrón-Giner C, Pereira R, Plutowska-Hoffmann K, Purves J, Re Dionigi A, Reinson K, Robert M, Robertson L, Rocha JC, Rohde C, Rosenbaum-Fabian S, Rossi A, Ruiz M, Saligova J, Gutiérrez-Sánchez A, Schlune A, Schulpis K, Serrano-Nieto J, Skarpalezou A, Skeath R, Slabbert A, Straczek K, Giżewska M, Terry A, Thom R, Tooke A, Tuokkola J, van Dam E, van den Hurk TA, van der Ploeg EM, Vande Kerckhove K, Van Driessche M, van Wegberg AM, van Wyk K, Vasconcelos C, Velez García V, Wildgoose J, Winkler T, Żółkowska J, Zuvadelli J, MacDonald A (2019)

Publication Type: Journal article

Publication year: 2019

Journal

Book Volume: 18

Pages Range: 39-44

DOI: 10.1016/j.ymgmr.2018.11.003

Abstract

Background: 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. Methods: 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. Results: 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. Conclusions: 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.

Involved external institutions

Hospital Sant Joan de Déu Barcelona Spain (ES) Birmingham Women's and Children's NHS Foundation Trust United Kingdom (GB) University Hospital Leuven (UZ) / Universitaire ziekenhuizen Leuven Belgium (BE) Copenhagen University Hospital Denmark (DK) Hospital Network Antwerp / Ziekenhuis Netwerk Antwerpen (ZNA) Belgium (BE) Ospedale Pediatrico Bambino Gesu Italy (IT) Cliniques universitaires Saint-Luc (CHU St-Luc) Belgium (BE) Institute of Mother and Child / Instytut Matki I Dziecka Poland (PL) Sheffield Children's NHS Foundation Trust United Kingdom (GB) Dokuz Eylül Üniversitesi (DEU) Turkey (TR) Hospital Geral de Santo António Portugal (PT) Institute of Pathology and Genetics (IPG) / Institut de Génétique et de Pathologie Belgium (BE) Osakidetza Spain (ES) Academic Medical Centre / Academisch Medisch Centrum (AMC) Netherlands (NL) Drottning Silvias barnsjukhus Sweden (SE) Hospitales Universitarios Virgen Macarena y Virgen del Rocío Spain (ES) University Children’s Hospital / Uniwersytecki Szpital Dziecięcy Poland (PL) Ege University / Ege Üniversitesi Turkey (TR) Universitätsklinikum Würzburg Germany (DE) Royal Hospital for Sick Children United Kingdom (GB) University Medical Centre Utrecht (UMC Utrecht) Netherlands (NL) University Hospitals of Leicester NHS Trust United Kingdom (GB) Royal Belfast Hospital for Sick Children United Kingdom (GB) Klinik der Medizinischen Universität Innsbruck Austria (AT) “Aghia Sophia” Children’s Hospital / Νοσοκομείο Παίδων "Η Αγία Σοφία" Greece (GR) University of Silesia in Katowice / Uniwersytet Śląski w Katowicach Poland (PL) Alder Hey Children's Hospital United Kingdom (GB) Universitätsklinikum Leipzig Germany (DE) Children's Clinical University Hospital / Bērnu klīniskā universitātes slimnīca Latvia (LV) Universitätsklinikum Freiburg Germany (DE) Pomeranian Medical University / Pomorski Uniwersytet Medyczny w Szczecinie (PMU) Poland (PL) Hospital Dr. Nélio Mendonça / Hospital Central do Funchal Portugal (PT) Hospital Infantil Universitario Niño Jesús / Hospital del Niño Jesús Spain (ES) Hacettepe University Turkey (TR) Medizinische Hochschule Hannover (MHH) / Hannover Medical School Germany (DE) University Hospital of Verona / Azienda Ospedaliera Universitaria Integrata Verona (AOUI) Italy (IT) Royal Victoria Infirmary United Kingdom (GB) Cengiz Gökçek Maternity and Children's Hospital / Cengiz Gökçek Kadın Doğum ve Çocuk Hastalıkları Hastanesi Turkey (TR) University of Szeged / József Attila Tudományegyetem Szeged Hungary (HU) Hospital de Santo Espírito da Ilha Terceira Portugal (PT) Universitätsklinikum Münster Germany (DE) Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet Norway (NO) Skåne University Hospital / Skånes universitetssjukhus Sweden (SE) Universitätsklinikum Düsseldorf Germany (DE) Great Ormond Street Hospital (GOSH) United Kingdom (GB) Helsinki University Central Hospital (HUCH) / Helsingin seudun yliopistollinen keskussairaala (HYKS) Finland (FI) Hospital Universitario y Politécnico de La Fe Spain (ES) St. Luke's Hospital United Kingdom (GB) Manchester University NHS Foundation Trust (MFT) United Kingdom (GB) Maastricht University Netherlands (NL) University of Groningen / Rijksuniversiteit Groningen Netherlands (NL) Radboud University Nijmegen Medical Centre / Radboudumc of voluit Radboud Universitair Medisch Centrum (UMC) Netherlands (NL) Hospital de Dona Estefânia Portugal (PT) Royal Hospital for Children United Kingdom (GB) Centre Hospitalier Régional et Universitaire de Nancy (CHRU de Nancy / CHU de Nancy) France (FR) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) University Hospital Ghent Belgium (BE) Hospital Regional de Málaga Spain (ES) Hospital do Divino Espírito Santo Portugal (PT) Carl-Thiem-Klinikum Germany (DE) Evelina London Children's Hospital United Kingdom (GB) Hospital de São João / Saint John Hospital Portugal (PT) Hôpital Universitaire Robert-Debré France (FR) Università degli studi "La Sapienza" Italy (IT) Karolinska University Hospital / Karolinska Universitetssjukhuset Sweden (SE) Istanbul University / İstanbul Üniversitesi Turkey (TR) Pavol Jozef Šafárik University in Kosice / Univerzita Pavla Jozefa Šafárika v Košiciach Slovakia (SK) Nottingham University Hospitals United Kingdom (GB) University of Health Sciences Vientiane / ມະຫາວິທະຍາໄລ ວິທະຍາສາດ ສຸຂະພາບ Lao People's Democratic Republic (LA) Charité - Universitätsmedizin Berlin Germany (DE) Hospital Universitario Ramón y Cajal Spain (ES) Azienda Ospedaliera Universitaria Meyer Italy (IT) Bristol Royal Hospital for Children (BRHC) United Kingdom (GB) Temple Street Children's University Hospital / Children's Health Ireland (CHI) Ireland (IE) Università degli studi di Milano Italy (IT) Azienda Ospedale Università Padova / University Hospital of Padova Italy (IT) Tartu University Hospital Estonia (EE) Universitätsmedizin der Johannes Gutenberg-Universität Mainz Germany (DE) Hospital Universitario 12 de Octubre Spain (ES) Centre hospitalier universitaire (CHU) d'Angers France (FR) Hospital Universitario Nuestra Señora de Candelaria (HUNSC) Spain (ES) Klinikum Stuttgart Germany (DE) Queen Fabiola Childrens Hospital / L'Hôpital universitaire des enfants Reine Fabiola Belgium (BE) Norfolk and Norwich University Hospital United Kingdom (GB) Klinikum der Universität München (LMU Klinikum) Germany (DE) Scottish Huntington's Association United Kingdom (GB) Mid Yorkshire Teaching NHS Trust United Kingdom (GB) Centre Hospitalier Régional Universitaire de Tours France (FR) Hôpital Necker-Enfants malades France (FR)

How to cite

APA:

Pinto, A., Adams, S., Ahring, K., Allen, H., Almeida, M.F., Garcia-Arenas, D.,... MacDonald, A. (2019). Weaning practices in phenylketonuria vary between health professionals in Europe. Molecular Genetics and Metabolism Reports, 18, 39-44. https://doi.org/10.1016/j.ymgmr.2018.11.003

MLA:

Pinto, A., et al. "Weaning practices in phenylketonuria vary between health professionals in Europe." Molecular Genetics and Metabolism Reports 18 (2019): 39-44.

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