Pediatric Hemophilia Center, Dr von Hauner's Children's Hospital, University of Munich, Munich, Germany.
J Thromb Haemost. 2012 Jul;10(7):1335-41. doi: 10.1111/j.1538-7836.2012.04775.x.
Diagnosing mild bleeding disorders (BDs) in children is difficult. Bleeding scores (BSs) have been proposed for obtaining standardized quantitative histories.
To compare the Canadian pediatric bleeding questionnaire (PBQ) with the new ISTH bleeding assessment tool (ISTH BAT) for the determination of BS in a routine pediatric outpatient setting.
One hundred children with a suspected BD were enrolled in this cross-sectional study. Bleeding scores were calculated for all children and their natural parents. For all children, extensive laboratory investigations were performed.
Based on laboratory tests, 56 children were diagnosed as having no BD, 11 were diagnosed with possible VWD, 12 with VWD 1, 11 with VWD 2, five with possible platelet defects, and five with mild factor deficiencies. Both questionnaires were able to discriminate between no BD and VWD (P = 0.0001), but the area under the receiver characteristics curve to detect any mild BD was only 0.76. Despite the inherited nature of the BD, a family score did not increase the ability to discriminate between no BD and VWD (P = 0.2052). There was no significant difference between the two tools used (P = 0.3253) or simple qualitative criteria, such as yes/no questions regarding bleeding (P = 0.3477).
The two tools translated into German did not differ substantially. Both were able to discriminate between no BD and a possible BD with acceptable accuracy. A BS of < 2 makes a BD unlikely. Simple qualitative criteria were similar; however, to allow comparison of studies and follow-up in patients over time, we recommend the ISTH BAT.
儿童轻度出血性疾病（BD）的诊断较为困难。出血评分（BS）已被提出，以获得标准化的定量病史。
在常规儿科门诊环境中，比较加拿大儿科出血问卷（PBQ）与新的 ISTH 出血评估工具（ISTH BAT）用于确定 BS 的能力。
本横断面研究纳入了 100 名疑似 BD 的儿童。为所有儿童及其亲生父母计算了出血评分。对所有儿童均进行了广泛的实验室检查。
根据实验室检查结果，56 名儿童被诊断为无 BD，11 名儿童被诊断为可能的 VWD，12 名儿童被诊断为 VWD 1，11 名儿童被诊断为 VWD 2，5 名儿童可能存在血小板缺陷，5 名儿童存在轻度因子缺乏。两种问卷均能区分无 BD 和 VWD（P = 0.0001），但用于检测任何轻度 BD 的受试者工作特征曲线下面积仅为 0.76。尽管 BD 具有遗传性，但家族评分并不能提高区分无 BD 和 VWD 的能力（P = 0.2052）。两种工具（P = 0.3253）或简单的定性标准（例如，关于出血的是/否问题，P = 0.3477）之间无显著差异。
两种翻译成德语的工具在本质上没有显著差异。两者均能以可接受的准确度区分无 BD 和可能的 BD。BS<2 提示 BD 不太可能。简单的定性标准相似；但是，为了允许在研究之间进行比较，并随着时间的推移对患者进行随访，我们推荐使用 ISTH BAT。
