Use Of The Pediatric Bleeding Questionnaire As A Screening Tool For Von Willebrand Disease

Here is a link to the web page resource on the Pediatric Bleeding Questionnaire  (PBQ) from the World Federation Of Hemophilia.

The above resource has direct links to The Pediatric Bleeding Questionnaire PDF and to the Scoring Key PDF.

The above web page resource also has additional resources on the PDQ:

  • Description
  • Utility
  • Administration
  • Psychometrics
  • References

Here are links to and excerpts from Utility of a Paediatric Bleeding Questionnaire as a screening tool for von Willebrand disease in apparently healthy children [PubMed Abstract] [Full Text HTML] [Full Text PDF]. Haemophilia. 2015 Nov;21(6):806-11. doi: 10.1111/hae.12689. Epub 2015 May 16.

Here are some excerpts:


von Willebrand disease (VWD) is an inherited bleeding disorder caused by deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates the adhesion of
platelets at sites of vascular injury and also binds to and stabilizes blood coagulation factor VIII (FVIII) thereby prolonging its half-life in the circulation. Deficiency or defects in the function of VWF can cause bleeding by impairing platelet adhesion or reducing the concentration of FVIII in the plasma.

VWD is the most common bleeding disorder but its prevalence varies considerably among studies and depends largely on the case definition used. The prevalence has been estimated
in several countries on the basis of the number of symptomatic patients seen at specialized haemostasis centers, and ranges from roughly 23 to 110 per million population [1].

VWD is classified into three major categories: partial quantitative deficiency (type 1), qualitative deficiency (type 2), and total deficiency (type 3). Type 2 VWD is further subdivided further into four variants (2A, 2B, 2M, 2N) on the basis of the laboratory phenotype [2,3].

Three key features form the basis for diagnosis: a personal history of excessivemucocutaneous bleeding, the hallmark of the disease, a family history of the same, and abnormal VWF laboratory studies [4]. Most often, bleeding includes excessive and/or unexplained bruising, epistaxis, bleeding from the gums and from trivial wounds, and in females, menorrhagia and postpartum hemorrhage. Prolonged and excessive bleeding may
also occur after surgical and dental procedures.


The importance of a sound history cannot be overemphasized in the diagnosis of VWD. Bleeding manifestations are frequent among healthy children, hence raising the question of
whether history alone can be relied upon to provide accurate screening for VWD in children and in determining whom to refer for further investigations.

Bleeding assessment tools (BAT) have been developed and studied in a variety of clinical settings [5,6,9-12]. These tools are invaluable in the identification of symptomatic patients given a prevalence of 1 in 1000; however, a review of diagnosed cases reveals that far fewer patients have been diagnosed, and therefore, have access to appropriate treatment. This is
also likely to be true for children presenting to the pediatrician’s office with bleeding complaints.

Additional Resources:

(1) Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor [PubMed Abstract] [Full HTML] [Full Text PDF]. J Thromb Haemost. 2006 Oct;4(10):2103-14. Epub 2006 Aug 2.

The above article has been cited by over 100 PubMed Central articles.

(2) Closing the gap – detection of clinically relevant von Willebrand disease in emergency settings through an improved algorithm based on rotational Thromboelastometry [PubMed Abstract] [Full Text HTML] [Full Text PDF]. BMC Anesthesiol. 2019 Jan 10;19(1):10. doi: 10.1186/s12871-018-0672-8.

Here is the link to the references for this PMC article.

(3) Episode 6: Your guide to TEG, ROTEM and the Medical Betamax Vs VHS Battle  April 18, 2017 from The Surge: Surgery. Trauma. Critical Care.


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