Kenet Gili, Waldman Dalia, Lubetsky Aharon, Kornbrut Nurit, Khalil Abdalla, Koren Ariel, Wolach Baruch, Fattal Aviva, Kapelushnik Joseph, Tamary Hannah, Yacobovitch Joanne, Raveh Eyal, Revel-Vilk Shoshana, Toren Amos, Brenner Benjamin
The Israeli National Hemophilia Center, Sheba Medical Center, Tel-Hashomer, 52621, Israel.
Thromb Haemost. 2004 Oct;92(4):713-8. doi: 10.1160/TH04-03-0182.
The etiology and pathophysiology of cerebral sinus venous thrombosis (CSVT) in the paediatric population is still poorly understood, and the role of thrombophilic risk factors remains to be elucidated. In our multi-center case-controlled study we studied 46 patients with CSVT diagnosed from April 1996 to December 2003, consecutively referred for thrombophilia work-up. The results of thrombophilia screen were compared to 112 healthy paediatric controls. Anticoagulant therapy was applied according to treating physicians' decisions, and all cases were prospectively followed for a median of 4.1 years. Of 46 children, 8 had CSVT diagnosed in the neonatal period and therefore were analyzed separately. The prevalence of single thrombophilia markers and combinations of thrombophilic risk factors were similar among cases and controls. Among children with CSVT co-morbid systemic illness was present in most patients at diagnosis. Seven out of 8 children with idiopathic CSVT had thrombophilic risk factors as compared to 31/38 patients with co-morbid conditions. Anticoagulation was initiated in most patients, 11/46 patients continued therapy for a total of one year or more post event. Neither clinical presentation nor initial treatment decisions were affected by presence of thrombophilic risk factors in our study group. Thrombophilia prevalence was not increased in children with CSVT as compared to controls, however thrombophilia was more frequent among children with idiopathic CSVT. Thus, those selected patients would benefit most from thrombophilia work-up, required for long-term therapy considerations.
小儿人群中脑静脉窦血栓形成（CSVT）的病因和病理生理学仍未得到充分了解，血栓形成倾向危险因素的作用仍有待阐明。在我们的多中心病例对照研究中，我们研究了1996年4月至2003年12月期间连续转诊进行血栓形成倾向检查的46例CSVT患者。将血栓形成倾向筛查结果与112名健康小儿对照进行比较。根据治疗医生的决定应用抗凝治疗，并对所有病例进行前瞻性随访，中位随访时间为4.1年。46名儿童中，有8名在新生儿期被诊断为CSVT，因此单独进行分析。病例组和对照组中单一血栓形成倾向标志物及血栓形成倾向危险因素组合的患病率相似。在CSVT患儿中，大多数患者在诊断时伴有全身性疾病。8例特发性CSVT患儿中有7例存在血栓形成倾向危险因素，而38例合并疾病的患者中有31例存在该危险因素。大多数患者开始抗凝治疗，46例患者中有11例在事件发生后持续治疗共一年或更长时间。在我们的研究组中，血栓形成倾向危险因素的存在既不影响临床表现，也不影响初始治疗决策。与对照组相比，CSVT患儿的血栓形成倾向患病率并未增加，然而，特发性CSVT患儿中血栓形成倾向更为常见。因此，那些选定的患者将从血栓形成倾向检查中获益最大，这对于长期治疗考虑是必要的。
