儿童原发性肾病综合征合并颅内静脉血栓4例病例报告

目的 通过对原发性肾病综合征合并颅内静脉血栓患儿行临床分析，探讨儿童NS合并颅内静脉血栓早期诊断和治疗的可行方案。方法 纳入2012年1月至2015年9月在上海市儿童医院住院的原发性肾病综合征且经头颅CT和（或）MRI确诊的颅内静脉血栓患儿，对其临床症状、实验室指标、影像学检查结果、疗效及预后进行分析。结果 4例原发性肾病综合征合并颅内静脉血栓患儿进入分析，均为男性，年龄5岁4个月至11岁4个月，出现颅内静脉血栓时间距原发性肾病综合征起病时间为1个月至7年余。4例颅内静脉血栓发病时均有神经精神系统症状，查体均未见神经系统阳性体征。3例在颅内静脉血栓发病期间D-二聚体、纤维蛋白原降解产物（FDP）均升高，抗凝血酶Ⅲ（AT-Ⅲ）下降；确诊后D-二聚体、FDP较前继续升高； 4例血清白蛋白明显降低，总胆固醇明显升高。4例出现颅内静脉血栓临床症状当日或次日均行头颅MRI增强+MRV+MRA检查，3例为左侧乙状静脉窦血栓，1例为脑栓塞。明确颅内静脉血栓诊断后，3例予尿激酶溶栓，低分子肝素钙和双嘧达莫抗凝治疗；1例脑栓塞患儿予对症和抗凝治疗；4例症状均明显改善。3例出院后6~12个月随访头颅MRI增强+MRV显示颅内异常信号均有不同程度吸收。结论 儿童原发性肾病综合征合并 颅内静脉血栓易发生于左侧乙状静脉窦。在原发性肾病综合征病程中如出现神经精神系统症状时，应及时行头颅MRI相关序列检查，有助于颅内静脉血栓早期诊断；早期积极溶栓治疗预后良好。

Primary nephrotic syndrome with cerebral venous thrombosis in children: four cases report

Objective To get a better understanding and to explore a proper criteria for early diagnosis and treatment of nephrotic syndrome(NS)with cerebral venous thrombosis (CVT) in children from clinical analysis. Methods From Jan. 2012 to Sep. 2015, data of children of NS with CVT who had been diagnosed by brain CT scan and head MRI in Shanghai Children's Hospital were enrolled. Clinical symptom features, laboratory data and radiographs findings, treatment effect and prognosis of all cases were collected and analyzed.Results Four cases of NS patients with CVT were included in the analysis. Four cases were all male. The age range was from 5 years 4 months to 11 years 4 months. When they had CVT, the onset of NS was 1 months to 7 years later. Clinical manifestations of all patients were different, and they were mainly manifested as neurological symptoms. And they all had no positive signs of nervous system. D-dimer and FDP of 3 cases abnormally elevated significantly, and AT-Ⅲ dropped. After these children had been diagnosed, D-dimer and FDP increased further. Serum albumin levels of 4 cases obviously decreased, and total cholesterol obviously increased. The patients with clinical symptoms were requested to do head enhanced MRI+ MRV+MRA on the same day or the second day, and 3 cases were confirmed with the left sigmoid sinus thrombosis, 1 case was cerebral embolism. 3cases were treated immediately with urokinase thrombolysis, low molecular weight heparin calcium and dipyridamole anticoagulant after confirmed diagnosis. One child with cerebral embolism had symptomatic treatment and anticoagulation therapy. Patient's symptoms were rapidly relieved. Follow-up with head enhanced MRI+ MRV of 3 cases showed that intracranial abnormal signals had been absorbed with different degree.Conclusion NS with CVT in children was easy to occur in the left sigmoid sinus thrombosis. When suspected abnormal nervous-mental system symptom appeared during NS course, timely head MRI related examination may be helpful to make early diagnosis of CVT. Prompt thrombolysis therapy had a favourable prognosis.