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

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

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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