Evidence map›Paper›PMID 36381287›Full record

ArticleResearch and practice in thrombosis and haemostasis2022

Ristocetin dependent cofactor activity in von Willebrand disease diagnosis: Limitations of relying on a single measure.

Pamela A Christopherson, Sandra L Haberichter, Veronica H Flood, Ursula O Sicking, Thomas C Abshire, Robert R Montgomery, Zimmerman Program Investigators

Open access · goldAbstract read
In one paragraph

Article in Research and practice in thrombosis and haemostasis, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
1.9field-weighted citation impact, top 12% 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

7 citing papers in PubMed, 13 citations in OpenAlex.

  1. Review
  2. Article
  3. Challenges and considerations of genetic testing in von Willebrand disease.Research and practice in thrombosis and haemostasis · 2025
    Review
  4. Article
  5. Review
  6. Article
  7. Diagnostic Performance of Von Willebrand Activity Versus Ristocetin Cofactor Tests in the Primary Evaluation of Von Willebrand Disease in Colombia.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis
    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

7 authors at 2 institutions in 1 country.

Pamela A ChristophersonVersiti Blood Research Institute Milwaukee Wisconsin USA.ORCID https://orcid.org/0000-0001-6758-9815
Sandra L HaberichterVersiti Blood Research Institute Milwaukee Wisconsin USA.ORCID https://orcid.org/0000-0002-5545-8014
Veronica H FloodVersiti Blood Research Institute Milwaukee Wisconsin USA.ORCID https://orcid.org/0000-0001-8998-6838
Ursula O SickingVersiti Blood Research Institute Milwaukee Wisconsin USA.
Thomas C AbshireVersiti Blood Research Institute Milwaukee Wisconsin USA.ORCID https://orcid.org/0000-0002-2386-7358
Robert R MontgomeryVersiti Blood Research Institute Milwaukee Wisconsin USA.ORCID https://orcid.org/0000-0003-2285-4077
Zimmerman Program Investigators
Versiti Blood Center of Wisconsin · USChildren's Hospital of Wisconsin · US

Funding

Zimmerman Program for the Molecular and Clinical Biology of VWDP01HL081588 · NHLBI · VERSITI WISCONSIN, INC. · PI MONTGOMERY, ROBERT R · 2005 to 2016
$19.3M
Zimmerman Program on the Biology of VWDP01HL144457 · NHLBI · VERSITI WISCONSIN, INC. · PI MONTGOMERY, ROBERT R · 2019 to 2023
$13.3M
Comparative Effectiveness in the Diagnosis of VWDR01HL112614 · NHLBI · VERSITI WISCONSIN, INC. · PI ABSHIRE, THOMAS CALVIN, MONTGOMERY, ROBERT R · 2013 to 2018
$4.1M
Mechanism of Type 4 Collagen Interactions with Von Willebrand FactorR01HL126810 · NHLBI · MEDICAL COLLEGE OF WISCONSIN · PI FLOOD, VERONICA H · 2016 to 2020
$1.9M
VWF - Mechanisms of RegulationR01HL136430 · NHLBI · VERSITI WISCONSIN, INC. · PI HABERICHTER, SANDRA · 2018 to 2021
$1.7M
NHLBI NIH HHS P01 HL081588NHLBI NIH HHS P01 HL144457NHLBI NIH HHS R01 HL112614NHLBI NIH HHS R01 HL126810NHLBI NIH HHS R01 HL136430
6 · The paper itself

Abstract

Background: Von Willebrand disease (VWD) is a common inherited bleeding disorder, however the diagnosis can be complicated by a subjective bleeding history and issues with some current von Willebrand factor (VWF) laboratory assays. Objectives: In the Zimmerman Program, we sought to determine how often a type 1 diagnosis was based on a single low VWF ristocetin cofactor (VWF:RCo) level resulting from the common genetic variant p.D1472H or an isolated assay issue, if that low value was corroborated by the VWF glycoprotein-IbM (VWF:GPIbM) assay, and if retesting confirmed original levels. Methods: New patients being evaluated for bleeding were consented. Analysis included Results: A total of 18% of VWD subjects had a low local VWF:RCo, but normal VWF:Ag and normal central testing including VWF:GPIbM. Seventy percent of the low VWF:RCo cohort had no pathogenic Conclusions: The diagnosis of VWD based on a single low VWF:RCo but normal VWF:Ag, was often attributed to p.D1472H or variability in VWF:RCo that was eliminated with VWF:GPIbM. Our study suggests that using VWF:RCo alone for diagnostic purposes may be insufficient while repeat VWF:RCo or VWF:GPIbM testing can be valuable in establishing a VWD diagnosis.

Indexed as

clinical laboratory testsgenetic polymorphismRistocetin cofactorvon Willebrand diseasevon Willebrand factor

Identifiers

PMID36381287
PMCPMC9637542
OpenAlexW4302004264

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
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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.