Clinical and Biomolecular Studies on Thrombophilia in Children.

儿童血栓形成倾向的临床和生物分子研究。

基本信息

批准号：
10470212
负责人：
YOSHIOKA Akira
金额：
$ 6.85万
依托单位：
Nara Medical University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (B)
财政年份：
1998
资助国家：
日本
起止时间：
1998 至 2000
项目状态：
已结题

项目摘要

In order to know the patients' number and its clinical conditions, we conducted a nation-wide survey of thrombophilia in children using questionnaire. We sent the first questionnaire to 1, 319 medical doctors and got 806 answers (61.1 %). They are as follows; congenital antithrombin(AT) deficiency 137 families/190 cases (15 children), protein C (PC) deficiency 146/182(27), protein S (PS) deficiency 62/84(16), heparin cofactor-Iiciency (HC-II2/3(0), abnormal plasminogenemia 65/65+α (7), abnormal fibrinogenemia 47/57 (13), others 16/20 (4), and totally 475/601 (82). We got answers of 36 cases (43.9 %) from 82 cases by the 2nd questionnaire. It was divided into 2 groups, positive history of thromboembolic events in childhood, 21 cases (AT 4, PC10, PS7) and negative history of those in childhood, 15 cases (AT3, PC4, PS8). Sex ratio of theformergroup was M11: F10. Age at onset of the events was ranged between newborn period and adlescence. Main initial events of thromboembolism were deep vein thrombosis in lower extremity (DVT), pulmonary thromboembolism (PTE), brain embolism and etc. Laboratory examinations showed medium low levels of activity and antigen of AT, PC and PS (Type I, heterozygote) in the majority of the cases and extremely low levels of those of PC and PS in only 3 cases, gene analysis was performed in 8 cases. Several kinds of antithrombotic agents were used at the in itial events of thromboembolism in these cases and warfarin was mostly applied for prophylaxis treatment after establishment of each diagnosis. Prothrombin time-INR and/or Thrombotest were used as a monitor to evalute good hemostatic control. It is suggested that careful and continuous observation is most important in children with thrombophilia. Other clinical and biomolecular studies on thrombophilia in children were also performed.

为了了解患者的人数及其临床状况，我们在全国范围内对使用问卷调查的儿童进行了全国性的血小板调查。我们将第一份问卷发送给了1、319位医生，并得到了806个答案（61.1％）。它们如下；先天性抗凝血酶（AT）缺乏137个家庭/190例（15个儿童），蛋白C（PC）缺乏146/182（27），蛋白质S（PS）缺乏症62/84（16），肝素cofactor-iisial，HC-II2/3（HC-II2/3（HC-II2/3）（HC-III2/3（0），绝对静脉基因基因基因65/65/65/65/65/65/65/65+al（7）（13），16/20（4）和475/601（82）。 M11：F10。 PC和PS仅在3例中，在8例中进行了基因分析。在这些情况下，在倾斜事件中使用了几种类型的抗血栓药物，而在建立每个诊断后，华法林主要用于预防治疗。凝血酶原时间INR和/或血栓级用作评估良好的止血控制的监测器。建议在血栓上的儿童中仔细而连续的观察最重要。还进行了其他关于儿童血小板性的临床和生物分子研究。