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[儿童血栓形成倾向与血栓形成——来自三级中心脑静脉窦血栓形成登记处的经验教训]

[Thrombophilia and thrombosis in children--lessons from cerebral sinus vein thrombosis registry of a tertiary center].

作者信息

Waldman Dalia, Manashku Shay, Strauss Tzipora, Goldstein Gal, Ben-Zeev Bruria, Kenet Gilli

机构信息

The Children Hematology Institute and The Thrombosis and Hemostasis Unit, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, and Sackler Faculty of Medicine, Tel Aviv University

出版信息

Harefuah. 2010 May;149(5):270-3, 337.

PMID:20929064
Abstract

BACKGROUND

Pediatric thrombosis is a rare event, and its pathophysiology is often associated with the presence of thrombophilic risk factors as welt as acute comorbid conditions. Sinus venous thrombosis [SVT] in childhood is a serious disease with reported annual incidence of 0.67 per 100,000 children. The etiology and pathophysiology of SVT in the pediatric population is stilt poorty understood, and the role of thrombophilic risk factors remains to be elucidated.

METHODS

In our single tertiary center registry the authors documented all new acute cases of radiologically confirmed SVT from 1996 to 2008. Children were consecutively referred for thrombophilia work-up. Anticoagulant therapy was applied according to treating physicians' decision and all cases were prospectively followed for a median of 5 years.

RESULTS

Our patient group included 15 males and 8 females whose median age was 2.9 years. Comorbid systemic illness was present in patients at diagnosis. Thrombophilia results were similar in comparison to pediatric controls previously collected at the said medical center. Short term anticoagulation was immediately initiated in most cases, and neurologic outcome was good. Neither clinical presentation nor acute-phase treatment decisions were affected by the presence of thrombophilic risk factors in the specified study group. Nevertheless, since SVT is a rare multifactorial disorder, imaging studies are recommended in sick "high-risk" children for better diagnosis and early initiation of anticoagulant treatment. Despite the presence of comorbid conditions in most cases, thrombophilia work-up is recommended to determine treatment length and assess potential further future risks.

摘要

背景

儿童血栓形成是一种罕见事件,其病理生理学通常与血栓形成倾向风险因素以及急性合并症有关。儿童窦静脉血栓形成(SVT)是一种严重疾病,报告的年发病率为每10万名儿童中有0.67例。儿科人群中SVT的病因和病理生理学仍了解甚少,血栓形成倾向风险因素的作用仍有待阐明。

方法

在我们的单一三级中心登记处,作者记录了1996年至2008年所有经放射学证实的SVT新急性病例。儿童被连续转诊进行血栓形成倾向检查。根据治疗医生的决定应用抗凝治疗,所有病例均进行前瞻性随访,中位随访时间为5年。

结果

我们的患者组包括15名男性和8名女性,中位年龄为2.9岁。诊断时患者存在合并全身性疾病。与该医学中心先前收集的儿科对照相比,血栓形成倾向检查结果相似。大多数病例立即开始短期抗凝治疗,神经学结果良好。在指定研究组中,血栓形成倾向风险因素的存在既不影响临床表现也不影响急性期治疗决策。然而,由于SVT是一种罕见的多因素疾病,建议对患病的“高危”儿童进行影像学检查,以更好地诊断并尽早开始抗凝治疗。尽管大多数病例存在合并症,但建议进行血栓形成倾向检查以确定治疗时长并评估未来潜在的进一步风险。

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