肾病综合征的诊疗.ppt

减药方法举例（隔日法）阶段剂量时间诱导缓解1mg/kg?d或60mg/d8~12周减药阶段60mg/双日50mg/单日6~8天6~8天6~8天6~8天6~8天6~8天60mg/双日40mg/单日60mg/双日30mg/单日60mg/双日20mg/单日60mg/双日10mg/单日60mg/双日0mg/单日50mg/双日或隔日40mg隔日30mg隔日20mg隔日8~10天8~10天10~20天20~30天5~15mg隔日维持阶段6~12月难治性肾病综合征甲基强的松龙冲击剂量500~1000mgivgtt×3天口服强的松维持疗程1年治疗结果激素敏感(12周内NS缓解)激素依赖(无诱因，激素减量过程中复发)激素抵抗(激素治疗无效)肾上腺皮质激素副作用柯兴氏表现三大代谢紊乱（糖、脂肪、盐）胃肠道反应、消化道出血感染（细菌、病毒、霉菌）生长延缓骨质疏松精神、神经系统症状白内障动脉硬化肾上腺分泌不足（三）主要治疗：抑制免疫与炎症2、细胞毒药物环磷酰胺（CTX）瘤可宁（苯丁酸氮芥，CB1248）硫唑嘌呤（AZA）环孢素A（CysA）霉酚酸酯（MMF）FK5061、病理（1）光镜系膜细胞和系膜基质弥漫增生系膜细胞插入到基底膜和内皮细胞之间，形成“双轨状”（2）免疫荧光系膜区、毛细血管壁IgG、C3颗粒样沉积（3）电镜系膜区、内皮下电子致密物沉积I（三）系膜毛细血管性

（膜增生型，Mesangiocapillaryglomerulonephritis,MPGN）系膜毛细血管型－光镜Thissilverstaindemonstratesadoublecontourtomanybasementmembranes,orthetram-trackingthatischaracteristicofmembranoproliferativeglomerulonephritis(MPGN)typeIthatresultsfrombasementmembranereduplication.系膜毛细血管型－免疫荧光（IgG）Thisismembranoproliferativeglomerulonephritis(MPGN).ThosecasesthatareidiopathicandprimaryaredividedintotypesIandIIbypathologicfindings.Asseenhere,theglomerulushasincreasedoverallcellularity,mainlyincreasedmesangialcellularity.SomecasesofMPGNaresecondarytounderlyingconditionssuchasautoimmunediseases,infections,andmalignancies,amongothers.Thiselectronmicrographdemonstratesamesangialcellatthelowerleftthatisinterposingitscytoplasmatthearrowintothebasementmembrane,leadingtosplittingandreduplicationofbasementmembranethatispiledupabovethemesangialcytoplasminthismicrograph.ThisisMPGNtypeI.ThesecharacteristicEMchangesoccurwhenthemesangialcell(whichhasamacrophage-likefunction)goesaftersubendothelialimmunedeposits,butmakesamessofthebasementmembraneintheprocess.Theelectronmicrographabovedemonstratesdensedepositsinthebasementmembranety