Pediatric thromboembolism: a national survey in Japan
Pediatric thromboembolism: a national survey in Japan
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DOI：
10.1007/s12185-016-2079-y
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发表时间：
2017-01-01
影响因子：
2.1
通讯作者：
Shima, Midori
中科院分区：
文献类型：
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作者：
Ishiguro, Akira;Ezinne, Chibueze Chioma;Shima, Midori
在儿科三级护理医院中，血栓栓塞症的发生率正在上升。儿童血栓形成的发病率在不同国家不同，在日本也是未知的。我们向520名儿科主任和629名专家发送了一份调查问卷，询问2006至2010年间出现症状性血栓栓塞症的患者的详细情况。在280例符合条件的病例中，报告了先天性血栓形成和其他情况。先天性血栓形成症(n=54，19.3%)包括蛋白C缺陷(27例)、S蛋白缺陷(9例)和抗凝血酶缺陷(7例)。没有人有因子V Leiden或凝血酶原G20210A的突变，这两种突变在高加索人中都很常见。血栓形成的非先天性原因包括先天性心脏病、中心静脉导管的使用、肾病综合征、抗磷脂综合征以及使用或不使用L-天冬酰胺酶的恶性肿瘤。有先天性血栓形成倾向的患者发生血栓栓塞症的年龄明显更早(中位数1.0岁对5.0岁，p=0.014)，血缘关系(35.2vs.1.8%，p<；0.001)和血栓后综合征(29.6vs.13.3%，p=0.007)的发生率较高。与没有先天性血栓形成倾向的儿童相比，有先天性血栓形成倾向的儿童血栓栓塞症的复发频率更高(50.0%比13.7%，p<；0.001)，并与更多的后遗症相关(61.1vs.37.2%，p=0.009)。这项全国性调查首次对患有症状性血栓栓塞症的日本儿童进行了全面研究。严重的复发和后遗症需要优化的标准。
Thromboembolism is being detected at increasing rates in pediatric tertiary care hospitals. The incidence of pediatric thrombophilia differs across countries, and is unknown in Japan. We sent a survey to 520 pediatric department heads and 629 specialists, requesting details on patients who developed symptomatic thromboembolism between 2006 and 2010. Of 280 eligible cases, congenital thrombophilia and other conditions were reported. Congenital thrombophilia (n = 54, 19.3 %) comprised defects in protein C (27), protein S (9), and antithrombin (7). None had mutations in factor V Leiden or prothrombin G20210A, both of which are frequent in Caucasians. Non-congenital causes of thrombophilia included congenital heart disease, the use of central venous catheters, nephrotic syndrome, antiphospholipid syndrome, and malignancy with or without use of L-asparaginase. Patients with congenital thrombophilia developed thromboembolism at a significantly younger age (median 1.0 vs. 5.0 years, p = 0.014), had a higher frequency of consanguinity (35.2 vs. 1.8 %, p < 0.001) and post-thrombotic syndromes (29.6 vs. 13.3 %, p = 0.007) than those who did not. Thromboembolism in children with congenital thrombophilia recurred more frequently (50.0 vs. 13.7 %, p < 0.001) and was associated with more sequelae (61.1 vs. 37.2 %, p = 0.009) than in children without congenital thrombophilia. This nationwide survey provides the first comprehensive study of Japanese children with symptomatic thromboembolism. Significant recurrence and sequelae require optimized standards.