Evidence map›Paper›PMID 29021961›Full record

ArticleMolecular genetics and metabolism reports2017

Dietary practices in propionic acidemia: A European survey.

A Daly, A Pinto, S Evans, M F Almeida, M Assoun, A Belanger-Quintana, S M Bernabei, S Bollhalder, D Cassiman, H Champion and 66 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Molecular genetics and metabolism reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the 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
–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

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

  1. Guideline
  2. Review
  3. Review
  4. Article
  5. Article
  6. Long term follow-up of the dietary intake in propionic acidemia.Molecular genetics and metabolism reports · 2021
    Article
  7. Article
  8. Early feeding practices in infants with phenylketonuria across Europe.Molecular genetics and metabolism reports · 2018
    Article
  9. 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

76 authors.

A DalyBirmingham Women's and Children's Hospital, Birmingham, UK.
A PintoBirmingham Women's and Children's Hospital, Birmingham, UK.
S EvansBirmingham Women's and Children's Hospital, Birmingham, UK.
M F AlmeidaCentro de Genética Médica, Centro Hospitalar do Porto - CHP, Porto, Portugal.
M AssounCentre de référence des maladies héréditaires du métabolisme, Hôpital Necker Enfants Malades, Paris, France.
A Belanger-QuintanaUnidad de Enfermedades Metabolicas, Servicio de Pediatria, Hospital Ramon y Cajal Madrid, Spain.
S M BernabeiChildren Hospital Bambino Gesù, Division of Artificial Nutrition, Rome, Italy.
S BollhalderUniverisity Hospital Zurich, Switzerland.
D CassimanMetabolic Center, University Hospitals Leuven and KU Leuven, Belgium.
H ChampionAddenbrooke's Hospital, Cambridge, UK.
H ChanEvelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
J DalmauUnit of Nutrition and Metabolopathies, Hospital La Fe, Valencia, Spain.
F de BoerUniversity of Groningen, University Medical Center Groningen, Netherlands.
C de LaetHôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium.
A de MeyerCenter of Metabolic Diseases, University Hospital, Antwerp, Belgium.
A DesloovereUniversity Hospital Ghent, Belgium.
A DianinDepartment of Pediatrics, Regional Centre for Newborn Screening, Diagnosis and Treatment of Inherited Metabolic Diseases and Congenital Endocrine Diseases, University Hospital of Verona, Italy.
M DixonGreat Ormond Street Hospital for Children NHS FoundationTrust, London, UK.
K DokoupilDr. von Hauner Children's Hospital, Munich, Germany.
S DuboisCentre de référence des maladies héréditaires du métabolisme, Hôpital Necker Enfants Malades, Paris, France.
F EyskensCenter of Metabolic Diseases, University Hospital, Antwerp, Belgium.
A FariaHospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, EPE, Portugal.
I FasanDivision of Inherited Metabolic Diseases, Department of Pediatrics, University Hospital of Padova, Italy.
E FavreReference center for Inborn Errors of Metabolism, Department of Pediatrics, Children's University Hospital, Nancy, France.
F FeilletReference center for Inborn Errors of Metabolism, Department of Pediatrics, Children's University Hospital, Nancy, France.
A FeketeMetabolic Centre of Vienna, Austria.
G GalloChildren Hospital Bambino Gesù, Division of Artificial Nutrition, Rome, Italy.
C GingellNottingham University Hospitals, UK.
J GribbenEvelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
K Kaalund HansenCharles Dent Metabolic Unit National Hospital for Neurology and Surgery, London, UK.
N M Ter HorstEmma Children's Hospital, AMC, Amsterdam, Netherlands.
C JankowskiBristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, UK.
R Janssen-RegelinkRadboud University Medical Center Nijmegen, Netherlands.
I JonesCenter of Metabolic Diseases, University Hospital, Antwerp, Belgium.
C JouaultCHU Angers, France.
G E KahrsHaukeland University Hospital, Bergen, Norway.
I L KokWilhelmina Children's Hospital, University Medical Centre Utrecht, Netherlands.
A KowalikInstitute of Mother & Child, Warsaw, Poland.
C LaguerreCentre de Compétence de L'Hôpital des Enfants de Toulouse, France.
S Le VergeCentre de référence des maladies héréditaires du métabolisme, Hôpital Necker Enfants Malades, Paris, France.
R LiljeOslo University Hospital, Norway.
C MaddalonUniversity Children's Hospital Zurich, Switzerland.
D MayrErnährungsmedizinische Beratung, Universitätsklinik für Kinder- und Jugendheilkunde, Salzburg, Austria.
U MeyerClinic of Paediatric Kidney, Liver- and Metabolic Diseases, Medical School Hannover, Germany.
A MiccicheEvelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
U OchUniversity Children's Hospital, Munster, Germany.
M RobertHôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium.
J C RochaCentro de Genética Médica, Centro Hospitalar do Porto - CHP, Porto, Portugal.
H RogozinskiBradford Teaching Hospital NHS Foundation Trust, UK.
C RohdeHospital of Children's & Adolescents, University of Leipzig, Germany.
K RossRoyal Aberdeen Children's Hospital, Scotland.
I SaruggiaCentre de Reference des Maladies Héréditaires du Métabolisme du Pr. B. Chabrol CHU Timone Enfant, Marseille, France.
A SchluneDepartment of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich Heine University, Moorenstr. 5, 40225 Düsseldorf, Germany.
K SingletonUniversity Hospital of Wales, Cardiff, UK.
E SjoqvistChildren's Hospital, University Hospital, Lund, Sweden.
R SkeathGreat Ormond Street Hospital for Children NHS FoundationTrust, London, UK.
L H StolenOslo University Hospital, Norway.
A TerryAlder Hey Children's Hospital NHS Foundation Trust Liverpool, UK.
C TimmerAcademisch Medisch Centrum, Amsterdam, Netherlands.
L TomlinsonUniversity Hospitals Birmingham NHS Foundation Trust, UK.
A TookeNottingham University Hospitals, UK.
K Vande KerckhoveMetabolic Center, University Hospitals Leuven and KU Leuven, Belgium.
E van DamUniversity of Groningen, University Medical Center Groningen, Netherlands.
T van den HurkWilhelmina Children's Hospital, University Medical Centre Utrecht, Netherlands.
L van der PloegMaastricht University Medical Centre + (MUMC +), Netherlands.
M van DriesscheUniversity Hospital Ghent, Belgium.
M van RijnUniversity of Groningen, University Medical Center Groningen, Netherlands.
A van WegbergRadboud University Medical Center Nijmegen, Netherlands.
C VasconcelosCentro Hospitalar São João - Unidade de Doenças Metabólicas, Porto, Portugal.
H VestergaardNational University Hospital, Copenhagen, Denmark.
I VitoriaUnit of Nutrition and Metabolopathies, Hospital La Fe, Valencia, Spain.
D WebsterBristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, UK.
F J WhiteCentral Manchester University Hospitals NHS Foundation Trust, Manchester, UK.
L WhiteSheffield Children's Hospital, UK.
H ZweersRadboud University Medical Center Nijmegen, Netherlands.
A MacDonaldBirmingham Women's and Children's Hospital, Birmingham, UK.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThe definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance.

aimTo describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines.

methodsThis was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014.

resultsInformation on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds.

conclusionsThere was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.

Indexed as

Natural proteinPrecursor-free amino acidsPropionic acidemiaProtein restricted diet

Identifiers

PMID29021961
PMCPMC5633157

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

None linked

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.