Skalova S, Lukes A, Vanicek H, Klein T, Hak J, Dedek P, Ocenaskova E
Department of Paediatrics, Charles University in Prague, Medical Faculty Hradec Kralove, Czech Republic.
Bratisl Lek Listy. 2008;109(12):573-5.
An intracardiac thrombus is extremely rare in children with the nephrotic syndrome (NS).
To present a case report of a child with steroid resistant NS and intracardiac thrombus.
A 3.5-year-old boy with the first attack of steroid resistant NS was admitted to the hospital. A histological evaluation of the renal biopsy specimen revealed the minimal changes disease (MCD). There were no mutations in the podocin gene. The treatment with furosemide, albumin, prednisone, metylprednisolone, cyclophosphamide, enalapril and losartan was ineffective, as the intermittent oedema, proteinuria, hypoalbuminemia and hypercholesterolemia were still present. 8 weeks after the disease onset, the sinus tachycardia occurred and the echocardiography revealed a thrombus in the right ventricle, which had gradually proceeded to the pulmonary artery bifurcation. The thrombolysis with 40 mg of alteplase was initiated. Two hours after the alteplase application, the thrombus was not detectable. The mutational analysis of factors V, II and MFTHR genes were negative. The repeated echocardiography performed after 1 week, 2 and 6 months, respectively, revealed a normal cardiac function and morphology. The patient received prophylactic doses of fraxiparin for 3.5 months followed by warfarin. A remission of the nephrotic syndrome was achieved with high doses of cyclosporine A together with atorvastatin at 7 month after the disease onset.
The thromboembolism as a result of the hypercoagulation status is a serious complication of the nephrotic syndrome. The intracardiac localisation of thrombus is extremely rare (Fig. 2, Ref. 10). Full Text (Free, PDF) www.bmj.sk.
心内血栓在肾病综合征（NS）患儿中极为罕见。
报告一例患有激素抵抗型NS并伴有心内血栓的患儿病例。
一名3.5岁首次发作激素抵抗型NS的男孩入院。肾活检标本的组织学评估显示为微小病变病（MCD）。足突蛋白基因无突变。使用呋塞米、白蛋白、泼尼松、甲泼尼龙、环磷酰胺、依那普利和氯沙坦治疗均无效，因为仍存在间歇性水肿、蛋白尿、低白蛋白血症和高胆固醇血症。发病8周后，出现窦性心动过速，超声心动图显示右心室有血栓，该血栓已逐渐延伸至肺动脉分叉处。开始用40mg阿替普酶进行溶栓治疗。应用阿替普酶两小时后，血栓消失。凝血因子V、II和亚甲基四氢叶酸还原酶（MFTHR）基因的突变分析为阴性。分别在1周、2个月和6个月后进行的重复超声心动图检查显示心脏功能和形态正常。患者接受了3.5个月的预防剂量的磺达肝癸钠，随后使用华法林。发病7个月后，高剂量环孢素A联合阿托伐他汀使肾病综合征缓解。
高凝状态导致的血栓栓塞是肾病综合征的严重并发症。心内血栓定位极为罕见（图2，参考文献10）。全文（免费，PDF）www.bmj.sk 。
