Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.
J Thromb Haemost. 2010 May;8(5):950-6. doi: 10.1111/j.1538-7836.2010.03796.x. Epub 2010 Feb 2.
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.
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.
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.
The PBQ provides a standardized quantitation of bleeding severity in children with VWD.
过度瘀伤和黏膜出血是儿童常见的首发症状。详细的出血史可以区分可能患有遗传性出血性疾病的儿童和正常儿童。儿科实践中缺乏这种病史采集的标准化。
评估一种儿科出血问卷 (PBQ) 的性能，该问卷是一种标准化成人出血问卷的改编版，包括儿科特有的出血症状，并在患有血管性血友病 (VWD) 的儿童队列中进行评估。
患者/方法：通过访谈确定有先前 VWD 诊断的儿童和未受影响的兄弟姐妹对照组的出血评分。
共获得 100 名 VWD 患儿的出血评分，中位年龄 10.9 岁（范围，0.8-17.8 岁），21 名未受影响的兄弟姐妹。VWD 患儿的中位出血评分为 7.0（范围，0-29），对照组为 0（范围，-1-2）。VWD 各型之间的出血评分存在差异：明确 1 型，n = 40，中位数 9.0（范围，2-18）；可能 1 型，n = 38，中位数 2.0（0-15）；2 型，n = 6，中位数 14.0（3-17）；3 型，n = 16，中位数 12.0（4-29）。受影响儿童的出血评分与年龄相关（Spearman 相关系数，0.35；P = 0.0004）。最常见的临床显著出血症状是手术出血、拔牙后出血和月经过多。割礼后出血、头颅血肿、肉眼血尿和脐带残端出血在 32%（割礼男性）、4%、4%和 3%的儿童中具有临床意义。
PBQ 为 VWD 患儿的出血严重程度提供了标准化的定量评估。
