Evidence map›Paper›PMID 30705824›Full record

ArticleMolecular genetics and metabolism reports2019

Weaning practices in phenylketonuria vary between health professionals in Europe.

A Pinto, S Adams, K Ahring, H Allen, M F Almeida, D Garcia-Arenas, N Arslan, M Assoun, Y Atik Altınok, D Barrio-Carreras and 107 more

Registry-linked trialOpen access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Molecular genetics and metabolism reports, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05497050 (The Effect of Nursing Empowerment Program Applied to Families of PKU Children, Coping Attitudes of Families and Phenylalanine Levels of Children), which is not on this map. Cited by 9 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
9citing papers in PubMed, 1 pooled it
1.3field-weighted citation impact, top 22% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT05497050 nacompletednot on this mapstarted 2021, after this paper: background citation

The Effect of Nursing Empowerment Program Applied to Families of PKU Children, Coping Attitudes of Families and Phenylalanine Levels of Children

TypeinterventionalSponsorCukurova UniversityRan2021 to 2023Enrolled36ConditionsPhenylketonuriasArmsFamily education
3 · Its place in the literature

Who cites it

9 citing papers in PubMed, 1 synthesis or guideline pooled it, 17 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Review
  4. Observational
  5. Article
  6. Article
  7. Article
  8. Article
  9. PKU dietary handbook to accompany PKU guidelines.Orphanet journal of rare diseases · 2020
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

117 authors at 20 institutions in 19 countries.

A PintoBirmingham Women's and Children's Hospital, Birmingham, UK.
S AdamsRoyal Victoria Infirmary, Newcastle, UK.
K AhringDepartment of PKU, Kennedy Centre, Department of Paediatrics and Adolescents Medicine, Copenhagen University Hospital, Glostrup, Denmark.
H AllenSheffield Children's NHS Foundation Trust, UK.
M F AlmeidaCentro de Genética Médica, Centro Hospitalar Universitário do Porto (CHP), Porto, Portugal.
D Garcia-ArenasCongenital and Metabolic Disease Unit, Gastroenterology, Hepatology and Pediatric Nutrition Unit, Sant Joan de Déu Hospital, Barcelona, Spain.
N ArslanDivision of Pediatric Metabolism and Nutrition, Dokuz Eylul University Faculty of Medicine, Izmır, Turkey.
M AssounHôpital Necker enfants Malades, Centre de référence des maladies héréditaires du métabolisme, Paris, France.
Y Atik AltınokPediatric Metabolism Department, Ege University Medical Faculty, Izmir, Turkey.
D Barrio-CarrerasServicio de Pediatria, Unidad de Enfermedades Mitocondriales-Metabolicas Hereditarias, Hospital 12 de Octubre, Madrid, Spain.
A Belanger QuintanaServicio de Pediatria, Hospital Ramon y Cajal Madrid, Unidad de Enfermedades Metabolicas, Spain.
S M BernabeiDivision of Artificial Nutrition, Children's Hospital Bambino Gesù, Rome, Italy.
C BontempsCHRU Clocheville Tours, France.
F BoyleNational Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Italy.
G BruniMeyer Children's hospital, Florence, Italy.
M Bueno-DelgadoChildren's Hospital Virgen del Rocío, Seville, Spain.
G CaineMid Yorks NHS Trust, UK.
R CarvalhoHospital Divino Espírito Santo, Ponta Delgada, Portugal.
A ChrobotChildren Voievodship Hospital, Bydgoszcz, Poland.
K ChyżInstitute of Mother and Child, Warsaw, Poland.
B CochraneRoyal Hospital for Children, Glasgow, UK.
C CorreiaCHLC- Hospital Dona Estefânia, Lisboa, Portugal.
K CorthoutsUniversity hospital Leuven, Belgium.
A DalyBirmingham Women's and Children's Hospital, Birmingham, UK.
S De LeoDepartment of Human Neuroscience, Sapienza University of Rome - Policlinico Umberto I of Rome, Italy.
A DesloovereUniversity hospital Ghent, Belgium.
A De MeyerCenter of Metabolic Diseases, University Hospital, Antwerp, Belgium.
A De TheuxIPG (Institut de Pathologie et de Genetique), Charleroi, Belgium.
B DidyczUniversity Children's Hospital, Cracow, Poland.
M E DijsselhofAMC Amsterdam/Emma Children's Hospital, Netherlands.
K DokoupilDr. von Hauner Children's Hospital of the University of Munich, Germany.
J DrabikUniversity Clinical Center in Gdansk, Poland.
C DunlopRoyal Hospital for Children Edinburgh, UK.
W Eberle-PellothUniversitäts-Kinderklinik Würzburg, Germany.
K EftringQueen Silivia's Children's Hospital Gothenburg, Sweden.
J EkengrenQueen Silivia's Children's Hospital Gothenburg, Sweden.
I ErrekaldeHospital Universitario de Cruces, Vizcaya, Spain.
S EvansBirmingham Women's and Children's Hospital, Birmingham, UK.
A FoucartCliniques universitaires Saint-Luc, Belgium.
L FokkemaUMC Utrecht Wilhelmina Children's Hospital, Netherlands.
L Françoiscentre de référence des maladies héréditaires du métabolisme, Hôpital Universitaire Robert-Debré, Paris, France.
M FrenchUniversity Hospitals of Leicester NHS Trust, UK.
E ForssellKarolinska University Hospital, Stockholm, Sweden.
C GingellNottingham Children's Hospital, UK.
C GonçalvesHospital Central do Funchal, Portugal.
H Gökmen Özelİhsan Doğramacı Children's Hospital, Hacettepe University, Turkey.
A GrimsleyRoyal Belfast Hospital for Sick Children, Northern Ireland, UK.
G GugelmoDepartment of Pediatrics, Inherited Metabolic Diseases Unit, University Hospital of Verona, Italy.
E GyüreAlbert Szent-Györgyi Clinical Centre, Hungary.
C HellerKinder- und Jugendklinik Erlangen, Germany.
R HenslerKlinikum Stuttgart Olgahospital, Germany.
I JardimCentro Hospitalar Lisboa Norte - H. Sta Maria - Unidade de Doenças Metabólicas, Portugal.
C JoostUniversity Children's Hospital, University Medical Center Hamburg Eppendorf, Germany.
M Jörg-StrellerUniversitätsklinik Innsbruck department für Kinder- und Jugendheilkunde, Austria.
C JouaultCHU, Angers, France.
A JungCharite, Virchow Klinikum Berlin, Germany.
M KantheSkane University Hospital, Sweden.
N KoçChild's Health and Diseases Hematology Oncology Training and Research Hospital, University of Health Sciences, Ankara, Turkey.
I L KokUMC Utrecht Wilhelmina Children's Hospital, Netherlands.
T Kozanoğluİstanbul University İstanbul Faculty of Medicine, Turkey.
B KumruCengiz Gökçek Maternity and Children's Hospital, Gaziantep, Turkey.
F LangUniversity Hospital Mainz, Villa metabolica, Germany.
K LangNinewells Hospital, Dundee, Scotland, UK.
I LiegeoisCHRU, Hospital D'enfants, Nancy, France.
A LiguoriDivision of Artificial Nutrition, Children's Hospital Bambino Gesù, Rome, Italy.
R LiljeOslo University Hospital, Norway.
O ĻubinaChildren's Clinical University Hospital, Riga, Latvia.
P Manta-VogliAgia Sophia Childrens' Hospital, Athens, Greece.
D MayrUniversitätsklinik für Jugend und Kinderheilkunde, Müllner Hauptstr, Salzburg, Austria.
C MenesesHospital de Santo Espírito da Ilha Terceira, EPER, Portugal.
C NewbyBristol Royal Hospital for Children, UK.
U MeyerClinic for Paediatric Kidney-, Liver and Metabolic Diseases, Medical School Hannover, Germany.
S MexiaCentro Hospitalar Lisboa Norte - H. Sta Maria - Unidade de Doenças Metabólicas, Portugal.
C NicolRoyal Victoria Infirmary, Newcastle, UK.
U OchMetabolic Department, University Hospital Muenster, Center for Pediatrics, Germany.
S M OlivasCongenital and Metabolic Disease Unit, Gastroenterology, Hepatology and Pediatric Nutrition Unit, Sant Joan de Déu Hospital, Barcelona, Spain.
C Pedrón-GinerHospital Infantil Universitario Niño Jesús, Madrid, Spain.
R PereiraNorfolk & Norwich hospital, UK.
K Plutowska-HoffmannThe Independent Public Clinical Hospital, Medical University of Silesia in Katowice John Paul II Upper Silesian Child Health Centre, Poland.
J PurvesRoyal Hospital for Children Edinburgh, UK.
A Re DionigiDepartment of Pediatrics, San Paolo Hospital, ASST Santi Paolo e Carlo, University of Milan, Italy.
K ReinsonTartu University Hospital, United Laboratories, Department of Genetics, Italy.
M RobertHôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium.
L RobertsonRoyal Hospital for Children Edinburgh, UK.
J C RochaCentro de Genética Médica, Centro Hospitalar Universitário do Porto (CHP), Porto, Portugal.
C RohdeHospital for Children and Adolescents, Department of Women and Child Health, University Hospitals, University of Leipzig, Germany.
S Rosenbaum-FabianDepartment of General Pediatrics, Adolescent Medicine and Neonatology, Medical Center - University of Freiburg, Faculty of Medicine, Freiburg, Germany.
A RossiDivision of Inherited Metabolic Diseases, Reference Centre Expanded Newborn Screening, Department of Woman's and Child's Health, University Hospital of Padua, Italy.
M RuizHospital Universitario Nuestra Señora de Candelaria, Tenerife, Spain.
J SaligovaChildren's Faculty Hospital, Kosice, Slovakia.
A Gutiérrez-SánchezCongenital and Metabolic Disease Unit, Gastroenterology, Hepatology and Pediatric Nutrition Unit, Sant Joan de Déu Hospital, Barcelona, Spain.
A SchluneDepartment of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital Duesseldorf, Heinrich Heine University, Düsseldorf, Germany.
K SchulpisAgia Sophia Childrens' Hospital, Athens, Greece.
J Serrano-NietoHRU, Málaga, Spain.
A SkarpalezouInstitute of Child Health, "A. Sophia" Children's Hospital, Athens.
R SkeathGreat Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
A SlabbertEvelina Children's Hospital, Guy's & St. Thomas' NHS Foundation Trust, London, UK.
K StraczekDepartment of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age Pomeranian Medica University, Poland.
M GiżewskaDepartment of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age Pomeranian Medica University, Poland.
A TerryAlder Hey Children's NHS Foundation Trust, Liverpool, UK.
R ThomRoyal Belfast Hospital for Sick Children, Northern Ireland, UK.
A TookeNottingham Children's Hospital, UK.
J TuokkolaClinical Nutrition Unit, Internal Medicine and Rehabilitation and Pediatric Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
E van DamUniversity of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Dietetics, Groningen, the Netherlands.
T A M van den HurkUMC Utrecht Wilhelmina Children's Hospital, Netherlands.
E M C van der PloegMaastricht University Medical Centre, Maastricht, Netherlands.
K Vande KerckhoveUniversity hospital Leuven, Belgium.
M Van DriesscheUniversity hospital Ghent, Belgium.
A M J van WegbergDepartment of Gastroenterology and Hepatology - Dietetics, Radboud University Medical Centre, Nijmegen, Netherlands.
K van WykManchester University NHS Foundation Trust, UK.
C VasconcelosCentro Hospitalar São João, Porto, Portugal.
V Velez GarcíaUnit of Nutrition and Metabolopathies, Hospital La Fe, Valencia, Spain.
J WildgooseBradford Teaching Hospitals St Lukes, UK.
T WinklerKlinik für Kinder- und Jugendmedizin, Carl-Thiem-Klinikum gGmbH Cottbus, Germany.
J ŻółkowskaInstitute of Mother and Child, Warsaw, Poland.
J ZuvadelliDepartment of Pediatrics, San Paolo Hospital, ASST Santi Paolo e Carlo, University of Milan, Italy.
A MacDonaldBirmingham Women's and Children's Hospital, Birmingham, UK.
Polish Mother’s Memorial Hospital Research Institute · PLHospital Universitario Virgen del Rocío · ESUniversity of Bydgoszcz · PLMid Yorkshire Hospitals NHS Trust · GBHospital do Divino Espírito Santo · PTWilhelmina Children's Hospital · NLCentre Hospitalier Universitaire de Tours · FRHospital Sant Joan de Déu Barcelona · ESAntwerp University Hospital · BEBambino Gesù Children's Hospital · ITCentro Hospitalar Lisboa Norte · PTCharité - Universitätsmedizin Berlin · DECliniques Universitaires Saint-Luc · BEDrottning Silvias barn- och ungdomssjukhus · SEGhent University Hospital · BEHospital Dr. Nélio Mendonça · PTHospital Universitario Ramón y Cajal · ESKU Leuven · BERadboud University Medical Center · NLRoyal Hospital for Children · GB

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundIn 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.

methodsA 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.

resultsWeaning started at 17-26 weeks in 85% (

conclusionsWeaning 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.

Indexed as

InfantPhe-free infant formulaPhenylalaninePhenylketonuriaWeaning

Identifiers

PMID30705824
PMCPMC6349955
OpenAlexW2900666564

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.