Evidence map›Paper›PMID 28394285›Full record

ReviewJournal of clinical medicine2017

Diagnosis and Treatment of von Willebrand Disease and Rare Bleeding Disorders.

Giancarlo Castaman, Silvia Linari

Open access · goldAbstract readReview
In one paragraph

Review in Journal of clinical medicine, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.

0numbers the graph read from it
0cells of the map it votes in
34citing papers in PubMed
6.6field-weighted citation impact, top 3% 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

34 citing papers in PubMed, 88 citations in OpenAlex.

  1. Observational
  2. Seventh Åland Island Meeting on von Willebrand Disease.Haemophilia : the official journal of the World Federation of Hemophilia · 2026
    Article
  3. Von Willebrand disease: A century of progress.Research and practice in thrombosis and haemostasis · 2026
    Review
  4. Article
  5. Article
  6. Article
  7. Article
  8. Rare inherited coagulation disorders: no longer orphan and neglected.Research and practice in thrombosis and haemostasis · 2024
    Review
  9. Article
  10. Article
  11. Review
  12. Review
  13. Article
  14. Article
  15. Review
  16. Article
  17. Article
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  19. Review
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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

2 authors at 1 institution in 1 country.

Giancarlo CastamanCenter for Bleeding Disorders and Coagulation, Department of Oncology, Careggi University Hospital, Largo Brambilla 3, 50134 Florence, Italy. giancarlo.castaman@unifi.it.
Silvia LinariCenter for Bleeding Disorders and Coagulation, Department of Oncology, Careggi University Hospital, Largo Brambilla 3, 50134 Florence, Italy. linaris@aou-careggi.toscana.it.
Azienda Ospedaliero-Universitaria Careggi · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal dominant trait. The diagnosis may be difficult, based on a bleeding history and different diagnostic assays, which evaluate the pleiotropic functions of VWF. Different treatment options are available for optimal management of bleeding and their prevention, and long-term outcomes are generally good. RBDs are autosomal recessive disorders caused by a deficiency of any other clotting factor, apart from factor XII, and cover roughly 5% of all bleeding disorders. The prevalence of the severe forms can range from 1 case in 500,000 up to 1 in 2-3 million, according to the defect. Diagnosis is based on bleeding history, coagulation screening tests and specific factor assays. A crucial problem in RBDs diagnosis is represented by the non-linear relationship between clinical bleeding severity and residual clotting levels; genetic diagnosis may help in understanding the phenotype. Replacement therapies are differently available for patients with RBDs, allowing the successful treatment of the vast majority of bleeding symptoms.

Indexed as

bleedingclotting factor deficiencydesmopressininherited disorderon-demandprophylaxisreplacement therapyvon Willebrand factor

Identifiers

PMID28394285
PMCPMC5406777
OpenAlexW2606660244

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.