儿童肾病综合征并发血栓8例分析

Retrospective investigation of 8 children with nephrotic syndrome complicated with thromboembolism

目的探讨儿童肾病综合征(NS)并发血栓的种类和特点。方法回顾性分析北京大学第一医院儿科1996年7月至2011年5月NS并发血栓患儿的临床资料,对血栓发生年龄、部位、症状、血清学指标和短期预后进行总结。结果 8例NS并发血栓患儿进入分析,其中男7例,女1例,发生血栓的平均年龄为10.6岁。患儿以头痛、呕吐、抽搐、腹痛、血便、肉眼血尿、咯血或胸闷等症状就诊。静脉血栓6例(75%),见于门静脉、肠系膜上静脉、肺栓塞、肾静脉、下腔静脉、颅内静脉,其中肺栓塞3例,肾静脉血栓2例,颅内静脉血栓2例;动脉血栓2例,左胫前动脉和颈内动脉血栓各1例。3例颅内血栓通过头颅MR I和MRV或MRA确诊,其余部位血栓分别通过血管超声、肺灌注显像和CT检查确诊。7例患儿血清白蛋白<20 g.L-1,8例患儿血浆纤维蛋白原>4 g.L-1,7例患儿血D-二聚体增高。8例患儿确诊后均予以肝素、尿激酶、华法林或双嘧达莫等抗凝及溶栓治疗,2例患儿经内科治疗无效分别行截肢和肠切除术。近期随访血栓或相关症状消失或改善。结论 NS患儿肺栓塞发生情况可能并不少于肾静脉血栓。对于10岁左右男性,伴显著低白蛋白血症、高纤维蛋白原血症和血D-二聚体增高者可能更需警惕血栓,应尽早行影像学检查。

Objective To summarize the occurrence,the site and the risk of thromboembolism complication in children with nephrotic syndrome（NS）.Methods The data of children with NS complicated with thromboembolism admitted in Peking University First Hospital from July 1996 to May of 2011 were analyzed restropectively.Gender,the onset age of thromboembolism,symptom,site,short-term prognosis,serum albumin,fibrinogen and D-dimmer were investigated.Results There were 8 NS children complicated with thromboembolism with an average age of 10.6 years old including 7 boys and 1 girl.All children had symptoms such as headache,vomitting,convulsion,abdominal pain,bloody stool,gross hematuria,hemoptysis and chest pain.There were 6 children（75%） with venous thromboembolism including portal vein,mesenteric vein,pulmonary embolism,renal vein,inferior vena cava and cerebral venous sinus thromboembolism.There were 3 children with pulmonary embolism,2 with renal vein thrombus and 2 with intracranial venous sinus thrombosis.Two children had arterial thromboembolism including lower limb arterial thrombus in 1 child and internal carotid arterial thrombus in 1 child.All the children were diagnosed by imaging techniques such as ultrasound,CT,MRI,MRA,MRV and lung perfusion.Seven children had low serum albumin below 20 g·L-1.All children had elevated serum fibrinogen above 4 g·L-1.Seven children had elevation of serum D-dimmer.All children received anticoagulants,2 showed poor response and underwent amputation surgery and bowel resection,respectively.Conclusions In children with NS,the occurrence of pulmonary embolism was not rarer than renal vein thrombosis.Male teenagers with hypoalbuminemia,hyperfibrinogemia and elevation of serum D-dimmer should be paid more attention to the complications of thromboembolism.