Evidence map›Paper›PMID 30770394›Full record

ArticleBlood2019

Increased galactose expression and enhanced clearance in patients with low von Willebrand factor.

Sonia Aguila, Michelle Lavin, Niall Dalton, Sean Patmore, Alain Chion, George D Trahan, Kenneth L Jones, Catriona Keenan, Teresa M Brophy, Niamh M O'Connell and 9 more

Registry-linked trialOpen access · bronzeAbstract read
In one paragraph

Article in Blood, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03167320 (Low Von Willebrand in Ireland Cohort Study), which is not on this map. Cited by 23 papers.

0numbers the graph read from it
0cells of the map it votes in
23citing papers in PubMed
4.1field-weighted citation impact, top 6% 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.

NCT03167320 unknown statusnot on this map

Low Von Willebrand in Ireland Cohort Study

TypeobservationalSponsorSt. James's Hospital, IrelandRan2014 to 2022Enrolled400ConditionsVon Willebrand Factor, Deficiency
3 · Its place in the literature

Who cites it

23 citing papers in PubMed, 48 citations in OpenAlex.

  1. Trial
  2. Review
  3. Article
  4. Article
  5. Review
  6. Challenges and considerations of genetic testing in von Willebrand disease.Research and practice in thrombosis and haemostasis · 2025
    Review
  7. Low von Willebrand factor-unraveling an enigma wrapped in a conundrum.Journal of thrombosis and haemostasis : JTH · 2024
    Review
  8. Article
  9. Article
  10. An Updated Review on Glycoprotein IIb/IIIa Inhibitors as Antiplatelet Agents: Basic and Clinical Perspectives.High blood pressure & cardiovascular prevention : the official journal of the Italian Society of Hypertension · 2023
    Review
  11. Article
  12. Article
  13. Article
  14. Article
  15. Article
  16. ADAMTS13 regulation of VWF multimer distribution in severe COVID-19.Journal of thrombosis and haemostasis : JTH · 2021
    Article
  17. New developments in von Willebrand disease.British journal of haematology · 2020
    Review
  18. Article
  19. Review
  20. Observational
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

19 authors at 4 institutions in 3 countries.

Sonia AguilaHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
Michelle LavinHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.ORCID 0000-0003-2999-4216
Niall DaltonHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
Sean PatmoreHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
Alain ChionHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
George D TrahanDepartment of Pediatrics and Human Genetics and Genomics Program, University of Colorado, Denver, Anschutz Medical Campus, Aurora, CO; and.
Kenneth L JonesDepartment of Pediatrics and Human Genetics and Genomics Program, University of Colorado, Denver, Anschutz Medical Campus, Aurora, CO; and.
Catriona KeenanNational Coagulation Centre, St James's Hospital, Dublin, Ireland.
Teresa M BrophyNational Coagulation Centre, St James's Hospital, Dublin, Ireland.
Niamh M O'ConnellNational Coagulation Centre, St James's Hospital, Dublin, Ireland.
Kevin RyanNational Coagulation Centre, St James's Hospital, Dublin, Ireland.
Mary ByrneNational Coagulation Centre, St James's Hospital, Dublin, Ireland.
Margaret NolanNational Coagulation Centre, St James's Hospital, Dublin, Ireland.
Anjali PatelHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
Roger J S PrestonHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
Paula JamesDepartment of Medicine, Queen's University, Kingston, ON, Canada.
Jorge Di PaolaDepartment of Pediatrics and Human Genetics and Genomics Program, University of Colorado, Denver, Anschutz Medical Campus, Aurora, CO; and.
Jamie M O'SullivanNational Coagulation Centre, St James's Hospital, Dublin, Ireland.
James S O'DonnellHaemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.
Royal College of Surgeons in Ireland · IESt. James's Hospital · IEUniversity of Colorado Anschutz Medical Campus · USQueen's University · CA

Funding

Zimmerman Program on the Biology of VWDP01HL144457 · NHLBI · VERSITI WISCONSIN, INC. · PI MONTGOMERY, ROBERT R · 2019 to 2023
$13.3M
NHLBI NIH HHS P01 HL144457
6 · The paper itself

Abstract

Glycan determinants on von Willebrand factor (VWF) play critical roles in regulating its susceptibility to proteolysis and clearance. Abnormal glycosylation has been shown to cause von Willebrand disease (VWD) in a number of different mouse models. However, because of the significant technical challenges associated with accurate assessment of VWF glycan composition, the importance of carbohydrates in human VWD pathogenesis remains largely unexplored. To address this, we developed a novel lectin-binding panel to enable human VWF glycan characterization. This methodology was then used to study glycan expression in a cohort of 110 patients with low VWF compared with O blood group-matched healthy controls. Interestingly, significant interindividual heterogeneity in VWF glycan expression was seen in the healthy control population. This variation included terminal sialylation and ABO(H) blood group expression on VWF. Importantly, we also observed evidence of aberrant glycosylation in a subgroup of patients with low VWF. In particular, terminal α(2-6)-linked sialylation was reduced in patients with low VWF, with a secondary increase in galactose (Gal) exposure. Furthermore, an inverse correlation between Gal exposure and estimated VWF half-life was observed in those patients with enhanced VWF clearance. Together, these findings support the hypothesis that loss of terminal sialylation contributes to the pathophysiology underpinning low VWF in at least a subgroup of patients by promoting enhanced clearance. In addition, alterations in VWF carbohydrate expression are likely to contribute to quantitative and qualitative variations in VWF levels in the normal population. This trial was registered at www.clinicaltrials.gov as #NCT03167320.

Indexed as

ABO Blood-Group SystemCase-Control StudiesGalactoseGlycosylationHumansMetabolic Clearance RateN-Acetylneuraminic AcidPolysaccharidesvon Willebrand FactorABO Blood-Group SystemGalactoseN-Acetylneuraminic AcidPolysaccharidesvon Willebrand Factor

Identifiers

PMID30770394
PMCPMC13127295
OpenAlexW2912442487

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.