【摘要】 目的探讨肾病综合征(NS)合并颅内静脉窦血栓(CVST)致失明患儿的临床表现、治疗方案及其预后。方法分析1例NS合并CVST患儿的临床特点及诊治经过,并复习和总结国内外有关儿童CVST治疗相关研究的最新进展。结果患儿在NS标准化抗凝治疗过程中突然出现抽搐等癫发作症状,并有复视、双眼视力下降、失明,头颅磁共振及脑血管造影检查(MRV)示CVST形成,肝素化抗凝治疗效果不佳,后予尿激酶溶栓治疗1个月,复查MRV示颅内血栓已完全溶解。目前国际上针对CVST患儿已采取标准化抗凝治疗方案,并随时监测凝血功能如活化部分凝血活酶时间(APTT)、国际标准化比值(INR)等预防出血风险。结论临床考虑CVST的患儿需早期及时完善神经影像学检查,在排除出血风险后应立即予以抗凝或溶栓治疗,并按疗程使用3~6个月。更多还原

【Abstract】 Objectives To explore the clinical manifestations, treatment and prognosis of a case of blindness caused by nephrotic syndrome with cerebral venous sinus thrombosis(CVST). Methods The clinical manifestations, diagnosis and treatment of a case of NS with CVST were analyzed. The latest domestic and foreign reseach progresses in treatment for CVST in children were reviewed. Results Epilepsy suddenly appeared with diplopia, binocular vision loss and blindness in anticoagulant therapy for the child with NS. Brain magnetic resonance venography(MRV) suggested CVST. MRV reexamined showed that the intracranial thrombosis was completely dissolved after urokinase thrombolysis for one month followed by ineffective heparin anticoagulation. At present, international standards of anticoagulant therapy have been adopted in the treatment for CVST patients. Coagulation function(e.g.APTT) and international standardization ratio were monitored in order to prevent bleeding. Conclusions It is better to perform neural imaging examination early in suspected CVST patients. Anticoagulation and thrombolytic therapy should be given immediately once the risk of bleeding was excluded and used for 3-6 months.更多还原