Quantitation of bleeding symptoms in children with von Willebrand disease: use of a standardized pediatric bleeding questionnaire

Summary.  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. Objectives: 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). Patients/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.