Evidence map›Paper›PMID 28275552›Full record

ArticleMolecular genetics and metabolism reports2017

Dietary practices in isovaleric acidemia: A European survey.

A Pinto, A Daly, S Evans, M F Almeida, M Assoun, A Belanger-Quintana, S Bernabei, S Bollhalder, D Cassiman, H Champion and 65 more

Open access · goldAbstract 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 11 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed, 1 pooled it
2.1field-weighted citation impact, top 16% 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.

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

11 citing papers in PubMed, 1 synthesis or guideline pooled it, 26 citations in OpenAlex.

  1. Pooled it
  2. Article
  3. Review
  4. Article
  5. Article
  6. Article
  7. Review
  8. Organic acidurias in adults: late complications and management.Journal of inherited metabolic disease · 2018
    Review
  9. Aspects of Newborn Screening in Isovaleric Acidemia.International journal of neonatal screening · 2018
    Review
  10. Article
  11. Dietary practices in propionic acidemia: A European survey.Molecular genetics and metabolism reports · 2017
    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

75 authors at 20 institutions in 14 countries.

A PintoBirmingham Children's Hospital, Birmingham, UK.
A DalyBirmingham Children's Hospital, Birmingham, UK.
S EvansBirmingham Children's Hospital, Birmingham, UK.
M F AlmeidaCentro de Genética Médica, Centro Hospitalar do Porto - CHP, Porto, Portugal; Unit for Multidisciplinary Research in Biomedicine, Abel Salazar Institute of Biomedical Sciences, University of Porto-UMIB/ICBAS/UP, Porto, Portugal.
M AssounCentre de référence des maladies héréditaires du métabolisme, hôpital Necker enfants Malades, Paris.
A Belanger-QuintanaUnidad de Enfermedades Metabolicas, Servicio de Pediatria, Hospital Ramon y Cajal Madrid, Spain.
S BernabeiChildren's Hospital Bambino Gesù, Division of Metabolism, Rome, Italy.
S BollhalderUniversity 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 DianinPediatric Department, University Hospital of Borgo Roma Verona, Italy.
M DixonGreat Ormond Street Hospital for Children NHS Foundation Trust, 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.
F EyskensCenter of Metabolic Diseases, University Hospital, Antwerp, Belgium.
A FariaHospital Pediatrico, 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's Hospital Bambino Gesù, Division of Metabolism, 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 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, The 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.
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.
M RobertHôpital Universitaire des Enfants, Reine Fabiola, Bruxelles, Belgium.
J C RochaCentro de Genética Médica, Centro Hospitalar do Porto - CHP, Porto, Portugal; Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Portugal; Centre for Health Technology and Services Research (CINTESIS), 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.
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 Teeffelen-HeithoffUniversity Children's Hospital, Munster, Germany.
A van WegbergRadboud University Medical Center Nijmegen, The 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, The Netherlands.
A MacDonaldBirmingham Children's Hospital, Birmingham, UK.
Hôpital Necker-Enfants Malades · FRGhent University Hospital · BEBirmingham Children's Hospital · GBQueen Fabiola Children's University Hospital · BEAntwerp University Hospital · BEGreat Ormond Street Hospital for Children NHS Foundation Trust · GBGuy's and St Thomas' NHS Foundation Trust · GBNottingham University Hospitals NHS Trust · GBBambino Gesù Children's Hospital · ITKU Leuven · BERoyal Aberdeen Children's Hospital · GBUniversity Hospital of Wales · GBAcademic Medical Center · NLAddenbrooke's Hospital · GBAlder Hey Children's Hospital · GBBoston Children's Hospital · USBradford Teaching Hospitals NHS Foundation Trust · GBBristol Royal Hospital for Children · GBCentre for Health Technology and Services Research · PTCentre Hospitalier Universitaire d'Angers · FR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundIn Europe, dietary management of isovaleric acidemia (IVA) may vary widely. There is limited collective information about dietetic management.

aimTo describe European practice regarding the dietary management of IVA, prior to the availability of the E-IMD IVA guidelines (E-IMD 2014).

methodsA cross-sectional questionnaire was sent to all European dietitians who were either members of the Society for the Study of Inborn Errors of Metabolism Dietitians Group (SSIEM-DG) or whom had responded to previous questionnaires on dietetic practice (n = 53). The questionnaire comprised 27 questions about the dietary management of IVA.

resultsInformation on 140 patients with IVA from 39 centres was reported. 133 patients (38 centres) were given a protein restricted diet. Leucine-free amino acid supplements (LFAA) were routinely used to supplement protein intake in 58% of centres. The median total protein intake prescribed achieved the WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Centres that prescribed LFAA had lower natural protein intakes in most age groups except 1 to 10 y. In contrast, when centres were not using LFAA, the median natural protein intake met WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Enteral tube feeding was rarely prescribed.

conclusionsThis survey demonstrates wide differences in dietary practice in the management of IVA across European centres. It provides unique dietary data collectively representing European practices in IVA which can be used as a foundation to compare dietary management changes as a consequence of the first E-IMD IVA guidelines availability.

Indexed as

Isovaleric acidemiaLeucineLeucine free l-amino acidsNatural proteinProtein restricted diet

Identifiers

PMID28275552
PMCPMC5328917
OpenAlexW2593341713

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Registered trials

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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.