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土耳其患有血管性血友病和血小板功能障碍儿童的儿科出血问卷评估

Evaluation of Pediatric Bleeding Questionnaire in Turkish Children With Von Willebrand Disease and Platelet Function Disorders.

作者信息

Belen Burcu, Kocak Ulker, Isik Melek, Keskin Ebru Yilmaz, Oner Nergiz, Sal Ertan, Kaya Zuhre, Yenicesu Idil, Gursel Turkiz

机构信息

Department of Pediatric Hematology and Oncology, Gaziantep Childrens' Hospital, Gaziantep, Turkey

Department of Pediatric Hematology, Gazi University Medical School, Ankara, Turkey.

出版信息

Clin Appl Thromb Hemost. 2015 Sep;21(6):565-9. doi: 10.1177/1076029614522546. Epub 2014 Feb 20.

Abstract

The diagnosis of mild bleeding disorders is not easy as most of the "healthy" individuals also report bleeding symptoms. In order to get a precise bleeding history, Pediatric Bleeding Questionnaire (PBQ) has been developed. In our study, Turkish children diagnosed with Von Willebrand disease (VWD), platelet function defect (PFD), and healthy children without any symptoms (control group 1) and healthy children with symptoms but found hemostatically normal (control group 2) were analyzed with PBQ. The cut off level for "positive bleeding score" was found to be ≥2 (area under the curve [AUC]: 0.785, 95% confidence interval [CI]: 0.718-0.852). The sensitivity, specificity, positive predictive value, and negative predictive value of PBQ to define VWD versus control group 1 was 100%, 97.4%, 96.4%, and 100%; VWD versus control group 2 was 100%, 53.1%, 64.3%, and 100%; PFD versus control group 1 was 93.3%, 53.1%, 73.7%, and 85%; and PFD versus control group 2 was 93.3%, 53.1%, 73.7%, and 85%, respectively.

摘要

轻度出血性疾病的诊断并不容易,因为大多数“健康”个体也报告有出血症状。为了获得准确的出血病史,已经开发了儿童出血问卷(PBQ)。在我们的研究中,对诊断为血管性血友病(VWD)、血小板功能缺陷(PFD)的土耳其儿童,以及无症状的健康儿童(对照组1)和有症状但止血功能正常的健康儿童(对照组2)进行了PBQ分析。发现“阳性出血评分”的截断水平为≥2(曲线下面积[AUC]:0.785,95%置信区间[CI]:0.718 - 0.852)。PBQ用于定义VWD与对照组1相比的敏感性、特异性、阳性预测值和阴性预测值分别为100%、97.4%、96.4%和100%;VWD与对照组2相比分别为100%、53.1%、64.3%和100%;PFD与对照组1相比分别为93.3%、53.1%、73.7%和85%;PFD与对照组2相比分别为93.3%、53.1%、73.7%和85%。

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