Mori Kazuetsu, Honda Masataka, Ikeda Masahiro
Department of Nephrology, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan.
Pediatr Nephrol. 2004 Nov;19(11):1232-6. doi: 10.1007/s00467-004-1584-z. Epub 2004 Aug 18.
Steroid-resistant nephrotic syndrome (SRNS) in children is intractable, and the optimal treatment regimen is not known. We investigated the efficacy of methylprednisolone pulse therapy (MPT) in SRNS patients. Using only MPT plus heparin, we have treated ten pediatric SRNS patients that were resistant to cyclophosphamide (CPM) or cyclosporin A (CsA) and predicted to have a very poor prognosis. Administration of 30 mg/kg per day methylprednisolone with heparin for 3 days was taken as one course, and this was given 14 times over 2 years. One patient discontinued MPT because of peritonitis. Of the remaining nine patients, complete remission was achieved by four patients, persistent mild proteinuria remained in three patients, and no effect was observed in two patients. All patients that had been diagnosed with minimal change (MC) pathology ( n=3) at the initial renal biopsy achieved complete remission. Observed adverse events were peritonitis in one patient and a transient decrease in pulse rate only during MPT in one patient. These results demonstrate that MPT with heparin can induce remission in children with SRNS, even when the patient is resistant to CPM and CsA.
儿童类固醇抵抗型肾病综合征（SRNS）难以治疗，目前尚无最佳治疗方案。我们研究了甲泼尼龙冲击疗法（MPT）对SRNS患者的疗效。仅使用MPT加肝素，我们治疗了10例对环磷酰胺（CPM）或环孢素A（CsA）耐药且预计预后很差的儿科SRNS患者。每天给予30mg/kg甲泼尼龙加肝素，持续3天作为一个疗程，在2年内进行了14次。1例患者因腹膜炎停用MPT。其余9例患者中，4例完全缓解，3例持续存在轻度蛋白尿，2例无效。所有在初次肾活检时被诊断为微小病变（MC）病理类型（n = 3）的患者均实现了完全缓解。观察到的不良事件为1例患者发生腹膜炎，1例患者仅在MPT期间出现脉搏率短暂下降。这些结果表明，即使患者对CPM和CsA耐药，MPT联合肝素也可诱导儿童SRNS缓解。
