Evidence map›Paper›PMID 30101073›Full record

ArticleMolecular genetics and metabolism reports2018

Early feeding practices in infants with phenylketonuria across 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

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Article in Molecular genetics and metabolism reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed
–field-weighted citation impact
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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

17 citing papers in PubMed.

  1. Review
  2. Review
  3. Observational
  4. Observational
  5. Article
  6. Article
  7. Article
  8. Review
  9. Article
  10. Review
  11. Review
  12. Article
  13. PKU dietary handbook to accompany PKU guidelines.Orphanet journal of rare diseases · 2020
    Review
  14. Article
  15. Article
  16. Article
  17. Article
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.

A PintoBirmingham Women's and Children's Hospital, Birmingham, UK.
S AdamsRoyal Victoria Infirmary, Newcastle, UK.
K AhringDepartment of PKU, Kennedy Centre, Copenhagen University Hospital, Glostrup, Denmark.
H AllenSheffield Children's NHS Foundation Trust, UK.
M F AlmeidaCentro de Genética Médica, Centro Hospitalar 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 ArslanDokuz Eylul University Faculty of Medicine, Division of Pediatric Metabolism and Nutrition, Izmır, Turkey.
M AssounCentre de référence des maladies héréditaires du métabolisme, Hôpital Necker enfants Malades, Paris, France.
Y Atik AltınokPediatric Metabolism Department, Ege University Medical Faculty, Izmir, Turkey.
D Barrio-CarrerasUnidad de Enfermedades Mitocondriales-Metabolicas Hereditarias. Servicio de Pediatría, Hospital 12 de Octubre, Madrid, Spain.
A Belanger QuintanaUnidad de Enfermedades Metabolicas, Servicio de Pediatria, Hospital Ramon y Cajal Madrid, Spain.
S M BernabeiChildren's Hospital Bambino Gesù, Division of Artificial Nutrition, Rome, Italy.
C BontempsCHRU Clocheville Tours, France.
F BoyleNational Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Ireland.
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 FrançoisHôpital Universitaire Robert-Debré, Centre de référence des maladies héréditaires du métabolisme, 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 ÖzelHacettepe University, İhsan Doğramacı Children's Hospital, 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 KoçUniversity of Health Sciences, Ankara Child's Health and Diseases Hematology Oncology Training and Research Hospital, Turkey.
I L KokUMC Utrecht, Wilhelmina Children's Hospital, Netherlands.
T Kozanoğluİstanbul University İstanbul Faculty of Medicine, Turkey.
B KumruGaziantep Cengiz Gökçek Maternity and Children's Hospital, Turkey.
F LangUniversity Hospital Mainz, Villa metabolica, Germany.
K LangNinewells Hospital, Dundee, UK.
I LiegeoisCHRU Nancy Hospital D'enfants, France.
A LiguoriChildren's Hospital Bambino Gesù, Division of Artificial Nutrition, Rome, Italy.
R LiljeOslo University Hospital, Norway.
O ĻubinaChildren's Clinical University Hospital, Riga, Latvia.
P Manta-VogliInborn Errors of Metabolism Department, Institute of Child Health, 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 MeyerMedical School Hannover, Clinic for Paediatric Kidney- Liver and Metabolic Diseases, Germany.
S MexiaCentro Hospitalar Lisboa Norte - H. Sta Maria - Unidade de Doenças Metabólicas, Portugal.
C NicolRoyal Victoria Infirmary, Newcastle, UK.
U OchUniversity Hospital Muenster, Center for Pediatrics, Metabolic Department, 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, No. 6 of the 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, Estonia.
M RobertHôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium.
L RobertsonRoyal Hospital for Children Edinburgh, UK.
J C RochaCentro de Genética Médica, Centro Hospitalar 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 SchulpisInborn Errors of Metabolism Department, Institute of Child Health, Athens, Greece.
J Serrano-NietoHRU Málaga, Spain.
A SkarpalezouInstitute of Child Health, "A. Sophia" Children's Hospital, Athens, Greece.
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 StraczekClinic of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age Pomeranian Medica University, Poland.
M GiżewskaClinic 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, 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.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundIn 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.

methodsWe sent a cross sectional, survey monkey® 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.

resultsNinety-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 ( 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.

Indexed as

BreastfeedingIMD, Inherited Metabolic DisordersInfant practicesPhe-free infant formulaPhenylalaninePhenylketonuriaPhe, PhenylalaninePKU, Phenylketonuria

Identifiers

PMID30101073
PMCPMC6082991

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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.