Ozbek Namik, Alioglu Bulent, Avci Zekai, Malbora Baris, Onay Ozge, Ozyurek Emel, Atac Fatma Belgin
Department of Pediatric Hematology, Baskent University Faculty of Medicine, Ankara, Turkey.
Pediatr Hematol Oncol. 2009 Jan;26(1):11-29. doi: 10.1080/08880010802423969.
This study was conducted to analyze the incidence of and risk for thrombosis in thrombotic children monitored in the Department of Pediatric Hematology of our hospital at the time of diagnosis, in addition to the clinical characteristics of those patients. The clinical and laboratory findings of 122 patients diagnosed with thrombosis from 1997 to 2006 were retrospectively analyzed. The incidence of thrombosis was 88.6/10,000 hospital admissions. The authors found that 31.1% of the patients studied had a thrombosis in more than 1 region. The incidence of thrombosis by anatomic site was as follows: 42 thromboses in the peripheral arterial system, 39 in an intracardiac region, 38 in the abdominal venous system, 36 in the deep peripheral venous system, and 28 in the cerebral vascular system. The mean age of the patients at the time of diagnosis was 4.9 years. Of the patients studied, 10.7% were neonates, 35.3% were infants younger than 1 year, and 48.4% were younger than 2 years. Most of the patients had a congenital cardiac disease and spontaneous thrombosis, and 66.1% had at least 1 acquired risk factor, the most common of which were having undergone surgery (42%) or wearing a central venous catheter (39%). A hereditary factor for the development of thrombosis was present in 54% of the patients. The most frequently observed hereditary risk factor was the MTHFR 677C-T mutation, and the second most common was the factor V Leiden mutation. Thrombosis should be considered a systemic disorder, and thrombotic patients should be evaluated with appropriate methods. Acquired and hereditary risk factors should be analyzed systematically in thrombotic patients.
本研究旨在分析我院儿科血液科在血栓形成患儿诊断时的血栓发生率及风险,以及这些患者的临床特征。回顾性分析了1997年至2006年期间诊断为血栓形成的122例患者的临床和实验室检查结果。血栓形成的发生率为88.6/10000次住院。作者发现,31.1%的研究患者在一个以上区域发生血栓形成。按解剖部位划分的血栓形成发生率如下:外周动脉系统42例血栓形成,心内区域39例,腹部静脉系统38例,外周深静脉系统36例,脑血管系统28例。患者诊断时的平均年龄为4.9岁。在研究患者中,10.7%为新生儿,35.3%为1岁以下婴儿,48.4%为2岁以下儿童。大多数患者患有先天性心脏病并发生自发性血栓形成,66.1%的患者至少有1个获得性危险因素,其中最常见的是接受过手术(42%)或使用中心静脉导管(39%)。54%的患者存在血栓形成的遗传因素。最常观察到的遗传危险因素是MTHFR 677C-T突变,第二常见的是因子V莱顿突变。应将血栓形成视为一种全身性疾病,对血栓形成患者应采用适当方法进行评估。应对血栓形成患者的获得性和遗传危险因素进行系统分析。