Kurekci A Emin, Gokce Hafize, Akar Nejat
Departments of Pediatric Hematology, Ankara University, Ankara, Turkey.
Pediatr Int. 2003 Apr;45(2):159-62. doi: 10.1046/j.1442-200x.2003.01683.x.
A number of coagulation defects have been implicated as risk factors in thrombo-embolic disease. Of these, high levels of clotting factor VIII have been shown to be associated with a five- to six-fold increased risk of thrombosis, compared to levels < 100 IU/dL in adults. The objective of this study was to investigate the prevalence of elevated plasma levels of factor VIII in a pediatric population with thrombo-embolism (TE).
Forty-two children (17 female, 25 male) with TE and 165 healthy controls without familial history of thrombosis or stroke were included in the present study. Doppler ultrasonography with or without angiography, computed tomography, magnetic resonance imaging or echocardiography was utilized to establish the diagnosis. One-stage clotting assay with factor VIII-deficient plasma for measurement of factor VIII and immunoturbidometric assay for von Willebrand factor (vWF) levels were utilized. All measurements were performed in duplicate. Plasma levels of factor VIII were assessed in parents of nine patients to establish whether high levels of factor VIII were genetically determined.
The median age at onset of TE was 7 years (range 0-17 years). Among patients with TE compared to controls, the prevalence of high factor VIII levels was 59.5% versus 12.1% (odds ratio 10.6, 95% CI: 4.9-23.1). The prevalence of high factor VIII levels was detected in at least one of nine families.
The data in the present study provide evidence that elevated plasma factor VIII levels are associated with increased risk of thrombosis in children: thus, plasma concentration of factor VIII should be measured in all children with TE.
多种凝血缺陷被认为是血栓栓塞性疾病的危险因素。其中，与成人凝血因子 VIII 水平<100 IU/dL 相比，高水平的凝血因子 VIII 已被证明与血栓形成风险增加五至六倍有关。本研究的目的是调查血栓栓塞（TE）儿科人群中血浆凝血因子 VIII 水平升高的患病率。
本研究纳入了 42 名患有 TE 的儿童（17 名女性，25 名男性）和 165 名无血栓形成或中风家族史的健康对照。采用多普勒超声检查（有或无血管造影）、计算机断层扫描、磁共振成像或超声心动图来确诊。采用用缺乏凝血因子 VIII 的血浆进行的一期凝血测定法来测量凝血因子 VIII，并采用免疫比浊法测定血管性血友病因子（vWF）水平。所有测量均重复进行。对 9 名患者的父母进行了凝血因子 VIII 血浆水平评估，以确定高水平的凝血因子 VIII 是否由基因决定。
TE 发病的中位年龄为 7 岁（范围 0 - 17 岁）。与对照组相比，TE 患者中凝血因子 VIII 水平高的患病率为 59.5%，而对照组为 12.1%（优势比 10.6，95%置信区间：4.9 - 23.1）。在 9 个家族中至少有一个家族检测到凝血因子 VIII 水平高的患病率。
本研究数据提供了证据，表明血浆凝血因子 VIII 水平升高与儿童血栓形成风险增加有关：因此，所有 TE 儿童均应检测血浆凝血因子 VIII 浓度。
