Evidence map›Paper›PMID 23349392›Full record

ArticleBlood2013

VWF propeptide and ratios between VWF, VWF propeptide, and FVIII in the characterization of type 1 von Willebrand disease.

Jeroen Eikenboom, Augusto B Federici, Richard J Dirven, Giancarlo Castaman, Francesco Rodeghiero, Ulrich Budde, Reinhard Schneppenheim, Javier Batlle, Maria Teresa Canciani, Jenny Goudemand and 3 more

Registry-linked trialOpen access · bronzeAbstract read
In one paragraph

Article in Blood, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02869074 (Molecular and Clinical Profile of Von Willebrand Disease), which is not on this map. Cited by 55 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
55citing papers in PubMed, 1 pooled it
7.8field-weighted citation impact, top 2% 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.

NCT02869074 completednot on this mapstarted 2017, after this paper: background citation

Molecular and Clinical Profile of Von Willebrand Disease (VWD) in Spain (PCM-EVW-ES). Recruitment Extension, Further Data Analysis, Improvement of Registry Platform, Diagnosis and Management of VWD Application Development

Typeobservational_patient_registrySponsorSpanish Society of Thrombosis and HaemostasisRan2017 to 2021Enrolled790ConditionsVon Willebrand DiseaseArmsVWF gene analysis
3 · Its place in the literature

Who cites it

55 citing papers in PubMed, 1 synthesis or guideline pooled it, 110 citations in OpenAlex.

  1. Pooled it
  2. Trial
  3. Review
  4. Article
  5. Review
  6. Review
  7. Review
  8. Article
  9. Review
  10. Article
  11. Sexuality and bleeding in von Willebrand disease.Research and practice in thrombosis and haemostasis · 2025
    Article
  12. Application of genetic testing for the diagnosis of von Willebrand disease.Journal of thrombosis and haemostasis : JTH · 2024
    Review
  13. von Willebrand disease.Nature reviews. Disease primers · 2024
    Review
  14. Article
  15. Article
  16. Article
  17. Article
  18. Article
  19. Article
  20. 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

13 authors at 7 institutions in 5 countries.

Jeroen EikenboomEinthoven Laboratory for Experimental Vascular Medicine, Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands. h.c.j.eikenboom@LUMC.nl
Augusto B Federici
Richard J Dirven
Giancarlo Castaman
Francesco Rodeghiero
Ulrich Budde
Reinhard Schneppenheim
Javier Batlle
Maria Teresa Canciani
Jenny Goudemand
Ian Peake
Anne Goodeve
MCMDM-1VWD Study Group
Leiden University Medical Center · NLOspedale San Bortolo · ITUniversity of Sheffield · GBFondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico · ITUniversité de Lille · FRUniversity Medical Center Hamburg-Eppendorf · DEUniversity of Milan · IT

Funding

Transgenic/Knockout CoreP01HL044612 · NHLBI · VERSITI WISCONSIN, INC. · PI NEWMAN, PETER J · 1990 to 2015
$33.4M
Zimmerman Program for the Molecular and Clinical Biology of VWDP01HL081588 · NHLBI · VERSITI WISCONSIN, INC. · PI MONTGOMERY, ROBERT R · 2005 to 2016
$19.3M
NHLBI NIH HHS P01 HL044612NHLBI NIH HHS P01 HL081588
6 · The paper itself

Abstract

During posttranslational modifications of von Willebrand factor (VWF), the VWF propeptide (VWFpp) is cleaved. The ratio between VWFpp and VWF antigen (VWF:Ag) and the ratio between factor VIII (FVIII:C) and VWF:Ag may be used to assess synthesis and clearance of VWF. We analyzed the contribution of VWFpp and ratios of VWFpp/VWF:Ag and FVIII:C/VWF:Ag in the pathophysiological characterization of type 1 von Willebrand disease (VWD) in the Molecular and Clinical Markers for the Diagnosis and Management of Type 1 VWD (MCMDM-1VWD) study. The VWFpp/VWF:Ag and FVIII:C/VWF:Ag ratios were increased among patients compared with unaffected family members and healthy controls. The VWFpp/VWF:Ag ratio was higher in individuals heterozygous for missense mutations than in those heterozygous for null alleles. In contrast, the FVIII:C/VWF:Ag ratio was highest among heterozygotes for VWF null alleles. The ratios of VWFpp/VWF:Ag and FVIII:C/VWF:Ag indicate that the pathophysiological mechanisms of type 1 VWD include reduced production and accelerated clearance of VWF, but that often a combination of both mechanisms is implicated.

Indexed as

Case-Control StudiesCohort StudiesFactor VIIIFamilyGenetic Carrier ScreeningGenetic LinkageHumansMutationProtein MultimerizationProtein PrecursorsProtein Processing, Post-Translationalvon Willebrand Disease, Type 1von Willebrand FactorFactor VIIIProtein Precursorsvon Willebrand Factor

Identifiers

PMID23349392
PMCPMC4191434
OpenAlexW2087850171

What OpenQuestion holds

Textmetadata
Read underepoch 390

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.