The Role of Standardized Bleeding Score in Diagnosis of Pediatric Von Willebrand's Disease

Study Overview
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Brief Summary
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Von Willebrand's disease (VWD) is the most common inherited bleeding disorder. It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion. Typical patients present with mucocutaneous bleeding symptoms because of reduced circulating von Willebrand factor (VWF) In this study we will use the ISTH/SSC BLEEDING assessment tool as a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders

Detailed Description
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Von Willebrand's disease (VWD) is the most common inherited bleeding disorder. It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion. Typical patients present with mucocutaneous bleeding symptoms because of reduced circulating von Willebrand factor (VWF) . A predictive bleeding score could reveal individuals who may benefit from repetitive testing and those for whom repetitive testing is unlikely to be of benefit . In 2005, Rodeghiero, et al. developed a standardized questionnaire for the evaluation of haemorrhagic symptoms, referred to hereafter as the Vicenza score. Their retrospective analysis found that >3 haemorrhagic symptoms or bleeding scores of 3 in men and 5 in women was very specific (98.6%) for VWD, although less sensitive (69.1%)]. These investigators later reported in a larger population that higher bleeding scores were associated with increasing likelihood of VWD and bleeding after surgery or tooth extraction. Neither of these studies prospectively surveyed paediatric populations. As a result of their shorter life experience, children have fewer exposures to bleeding challenges such as dental extractions, surgeries, menarche and childbirth. In 2009, Bowman, et al. administered a modified Vicenza bleeding score to a naive primary care paediatric population and determined a bleeding score ≥2 was abnormal. Using this definition, their bleeding score had a high negative predictive value (0.99) and could accurately distinguish between children with and without VWD. In this study we will use the ISTH/SSC BLEEDING assessment tool as a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. For each specific bleeding symptom, the ISTH/SSC joint working group proposed minimal criteria in order to classify a symptom as significant and thus receive a score of 1 or more. Symptoms included in this questionnaire are epistaxis , cutaneous bleeding , minor cutaneous wound , oral cavity bleeding , hematemesis , melena and hematochezia , hematuria , tooth extraction , surgical bleeding , menorrhagia , postpartum bleeding , muscle hematomas or hemoarthrosis and CNS bleeding .

Official Title
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The Role of Standardized Bleeding Score in Diagnosis of Pediatric Von Willebrand's Disease

Conditions
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Von Willebrand's Diseased Children, Bleeding Questionnaire is Done

Intervention / Treatment
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* Diagnostic Test: Bleeding score ( questionnaire )


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: This study included children, aged 1 week-18 years, referred to Sohag university hospital for evaluation of bleeding symptoms, family history of a bleeding disorder and/or abnormal coagulation studies. Bleeding questionnaire is done during patients' clinic visit. Exclusion Criteria: Patients older than 18 years. Other inherited or acquired bleeding disorders. Patients on regular antiplatelets or anticoagulants. Inclusion Criteria: This study included children, aged 1 week-18 years, referred to Sohag university hospital for evaluation of bleeding symptoms, family history of a bleeding disorder and/or abnormal coagulation studies. Bleeding questionnaire is done during patients' clinic visit. Exclusion Criteria: Patients older than 18 years. Other inherited or acquired bleeding disorders. Patients on regular antiplatelets or anticoagulants.

Ages Eligible for Study
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Minimum Age: 1 Week
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Diagnostic Test: Bleeding score ( questionnaire )|Bleeding score is done for children diagnosed as von Willebrand's disease .|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Use bleeding score as diagnostic tool of von Willebrand's disease | The aim of the present study is to assess the diagnostic power of the bleeding score in identifying VWD in a prospective cohort of patients referred to a paediatric haematology clinic for evaluation of a bleeding disorder , so we can predict the next bleeding episode and minimize the complications | Within one year |