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静脉血栓栓塞症患儿的血栓前状态异常

Prothrombotic abnormalities in children with venous thromboembolism.

作者信息

Bonduel M, Hepner M, Sciuccati G, Torres A F, Pieroni G, Frontroth J P

机构信息

Hematology-Oncology Department, Hospital de Pediatría, Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina.

出版信息

J Pediatr Hematol Oncol. 2000 Jan-Feb;22(1):66-72. doi: 10.1097/00043426-200001000-00013.

Abstract

PURPOSE

The aim of this study was to determine the frequency of acquired or inherited prothrombotic disorders in a pediatric population with venous thromboembolism (VTE).

PATIENTS AND METHODS

From May 1992 to April 1998, 56 consecutive children with VTE were prospectively studied at a single center.

RESULTS

The median age was 8.4 years (range, 0.1-18 years). There was a male predominance. Fifty (89%) children had thrombosis in the lower venous system. Risk factors were detected in 54 (96%) children. Twenty-one (38%) thrombotic episodes were related to central venous lines. Family history of thrombosis was positive in 13 (23%) patients. In 26 (46%) patients, a prothrombotic disorder was detected. Nine of them had inherited disorders (protein C deficiency, 5 patients; protein S deficiency, 3 patients; Factor V Leiden mutation, 1 patient), and 13 children had acquired disorders (antiphospholipid antibodies, 5 patients; antithrombin deficiency, 8 patients). The remaining four showed combined abnormalities (Factor V Leiden mutation associated with inherited protein S deficiency, 1 patient; acquired antithrombin deficiency, 2 patients and inherited antithrombin deficiency, 1 patient).

CONCLUSIONS

In the series, a high percentage of prothrombotic disorders was detected; thus, a complete hemostatic evaluation should be performed in all of the children with VTE whether the patients have one or more risk factors.

摘要

目的

本研究旨在确定患有静脉血栓栓塞症(VTE)的儿科人群中获得性或遗传性血栓前状态疾病的发生率。

患者与方法

从1992年5月至1998年4月,在单一中心对56例连续的VTE患儿进行前瞻性研究。

结果

中位年龄为8.4岁(范围0.1 - 18岁)。男性居多。50例(89%)患儿的血栓形成于下肢静脉系统。54例(96%)患儿检测到危险因素。21例(38%)血栓形成事件与中心静脉置管有关。13例(23%)患者有血栓形成家族史。26例(46%)患者检测到血栓前状态疾病。其中9例患有遗传性疾病(蛋白C缺乏症5例;蛋白S缺乏症3例;凝血因子V莱顿突变1例),13例儿童患有获得性疾病(抗磷脂抗体5例;抗凝血酶缺乏症8例)。其余4例表现为合并异常(凝血因子V莱顿突变合并遗传性蛋白S缺乏症1例;获得性抗凝血酶缺乏症2例和遗传性抗凝血酶缺乏症1例)。

结论

在该系列研究中,检测到高比例的血栓前状态疾病;因此,对于所有VTE患儿,无论其有无一个或多个危险因素,均应进行全面的止血评估。

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