Quantitation of bleeding symptoms in children with von Willebrand disease: use of a standardized pediatric bleeding questionnaire.
BACKGROUND
Excessive bruising and mucocutaneous bleeding are frequent presenting symptoms in childhood. A detailed bleeding history can distinguish children who may have an inherited bleeding disorder from those who are normal. There is a lack of standardization of such history taking in pediatric practise.


OBJECTIVE
To assess the performance of a Pediatric Bleeding Questionnaire (PBQ), an adaptation of a standardized adult bleeding questionnaire and score that includes pediatric-specific bleeding symptoms, in a cohort of children with von Willebrand disease (VWD).


METHODS
Bleeding scores were determined by interview, for children with a previous diagnosis of VWD and a control group of unaffected siblings.


RESULTS
Bleeding scores were obtained for 100 children with VWD, median age 10.9 years (range, 0.8-17.8 years), and 21 unaffected siblings. Median bleeding score in children with VWD was 7.0 (range, 0-29) and in the control group was 0 (range, -1-2). Bleeding score varied within and between each VWD type: definite type 1, n = 40, median, 9.0 (range, 2-18); possible type 1, n = 38, median, 2.0 (0-15); type 2, n = 6, median, 14.0 (3-17); and type 3, n = 16, median, 12.0 (4-29). Bleeding scores in affected children correlated with age (Spearman's correlation coefficient, 0.35; P = 0.0004). The most frequent clinically significant bleeding symptoms were surgical bleeding, bleeding after tooth extraction and menorrhagia. Post-circumcision bleeding, cephalohematoma, macroscopic hematuria and umbilical stump bleeding were clinically significant in 32% (of circumcised males), 4%, 4% and 3% of children, respectively.


CONCLUSIONS
The PBQ provides a standardized quantitation of bleeding severity in children with VWD.
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.
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Biss
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Blanchette
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Clark
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Bowman
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Wakefield
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Lillicrap
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James
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D002648:Child; D006470:Hemorrhage; D006801:Humans; D011795:Surveys and Questionnaires; D014842:von Willebrand Diseases / Q000503:physiopathology*