Rong Liping, Chen Lizhi, Dong Zhi, Zhuang Hongjie, Lin Zhilang, Mo Ying, Jiang Xiaoyun
Department of Pediatrics, The Children Kidney Center, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Department of Radiology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Front Pediatr. 2020 Dec 23;8:607776. doi: 10.3389/fped.2020.607776. eCollection 2020.
To analyze the clinical characteristics of nephrotic syndrome (NS) with complications of cerebral sinovenous thrombosis (CSVT) in children. Clinical, radiographic, laboratory, and treatment data obtained from 10 confirmed cases of NS with complications of CSVT were analyzed. All patients were followed up for at least 18 months. CSVT was diagnosed by cerebral computed tomography (CT) and/or magnetic resonance imaging (MRI) with or without magnetic resonance venography (MRV) of the cerebral vessels. Among 10 cases reported, 4 were steroid-sensitive NS with frequent relapse, 5 were steroid-resistant (three of them had renal biopsies showing two minimal change disease and one IgA nephropathy), and 1 was steroid-sensitive with one relapse. Common clinical manifestations were headache or ophthalmodynia complicated by vomiting, dizziness, convulsion, and coma. Neuropathologic signs were positive in some cases. Papilledema appeared in only one case with winding of vein. Cerebrospinal fluid was examined in three cases with elevated pressure but normal cytological and biochemical results. D dimer and fibrinogen levels were elevated while prothrombin time and activated partial thromboplastin time were shortened. Five out of seven cases who had performed cranial CT were suspicious for cerebral thrombosis. Nine cases had cranial MRI with abnormal signs in seven cases. All of the cases received MRV, confirming the diagnosis of CVST. Clinical manifestations of NS with CSVT are not specific but varied. Therefore, CSVT should be considered once nervous manifestations present. MRV is a better method in the diagnosis of CSVT.
分析儿童肾病综合征(NS)合并脑静脉窦血栓形成(CSVT)的临床特征。对10例确诊为NS合并CSVT的病例的临床、影像学、实验室及治疗资料进行分析。所有患者均随访至少18个月。CSVT通过头颅计算机断层扫描(CT)和/或磁共振成像(MRI)诊断,必要时联合脑血管磁共振静脉血管造影(MRV)。在报告的10例病例中,4例为频繁复发的激素敏感型NS,5例为激素抵抗型(其中3例肾活检显示为微小病变肾病,1例为IgA肾病),1例为复发1次的激素敏感型。常见临床表现为头痛或眼痛伴呕吐、头晕、惊厥和昏迷。部分病例出现神经病理征阳性。仅1例出现视乳头水肿伴静脉迂曲。3例进行了脑脊液检查,压力升高但细胞及生化结果正常。D-二聚体和纤维蛋白原水平升高,而凝血酶原时间和活化部分凝血活酶时间缩短。7例进行头颅CT检查的病例中有5例怀疑有脑血栓形成。9例进行头颅MRI检查,7例有异常表现。所有病例均接受MRV检查,确诊为CVST。NS合并CSVT的临床表现不具有特异性且多样。因此,一旦出现神经症状应考虑CSVT。MRV是诊断CSVT的较好方法。