成人微小病变肾病医学课件.pptxVIP

KDIGO肾小球肾炎临床实践指南——微小病变肾病循证治疗肾小球上皮细胞损害IT细胞释放细胞因子破坏肾小球滤过屏障IITreg功能不良，抑制T效应细胞作用减弱发病机制III足突细胞蛋白CD80和Angptl4表达上调IV淋巴细胞核内NF-related kB上调 Jonathan Hogan et al. J Am Soc Nephrol 24***-***, 2013成人MCD的疾病特点MCD是儿童NS最常见的病因，在成人NS中占10%～15%成人MCD需要考虑继发性因素，主要包括霍奇金淋巴瘤、胸腺瘤、NSAID、锂制剂、类圆线虫类的感染、过敏症20-25%的成人MCD患者病程中发生急性肾损伤危险因素包括高龄、高血压、严重肾病综合征、潜在的动脉硬化仔细评估容量情况，建议持续激素治疗以及其他支持治疗，如进行肾脏替代大部分AKI后肾功能恢复，少数进入慢性肾功能不全 Eknoyan G, et al. Kidney inter, Suppl. 2012;2:139-274成人MCD的治疗需求研究提示，未治的MCD肾病综合征与高脂血症引起的动脉粥样硬化加速、感染及血栓栓塞事件明显相关MCD的治疗目标是获得缓解，即降低尿蛋白至0.3g/dEknoyan G, et al. Kidney inter, Suppl. 2012;2:139-274成人MCD对于激素治疗反应的定义　DefinitionComplete remission (CR)Reduction of proteinuria to 0.3 g/d or 300 mg/g (30 mg/mmol) urine creatinine and normal serum creatinine and serum albumin 3.5 g/dl (35 g/l) Partial remission (PR) Reduction of proteinuria to 0.3–3.5 g/d (300–3500 mg/g [30–350mg/mmol]) urine creatinine and stable serum creatinine (change in creatinine 25%) or Reduction of proteinuria to 0.3–3.5 g/d (300–3500 mg/g [30–350mg/mmol]) urine creatinine and a decrease50% from baseline, and stable serum creatinine (change in creatinine 25%) RelapseProteinuria 3.5 g/d or 3500 mg/g (350 mg/mmol) urine creatinine after complete remission has been obtainedFrequent relapse(FR)*two or more relapses within the first six months after a steroid-induced remission, or four or more within the first yearSteroid-dependent(SD)Two relapses during or within 2 weeks of completing steroid therapySteroid-resistant(SR)Persistence of proteinuria despite prednisone 1 mg/kg/d or 2 mg/kg every other day for 4 monthsAdapted from adult FSGS guidelines in KDIGO* No formal definition exists in adults; adapted from the definition for children with FR MCD from KDIGO.“不同于儿童患者，在成人MCD患者中缺乏良好设计的RCTs”——KDIGO MCD指南KDIGO 肾小球肾炎指南推荐成人初发MCD的治疗5.1.1：推荐糖皮质激素作为初发MCD肾病综合征患者的初始治疗（1C）5.1.2：建议泼尼松或泼尼松龙1mg/kg每日顿服（最大剂量80mg），或者2mg/kg隔日顿服（最大剂量120mg）（2C）5.1.3：建议起始的大剂量糖皮质激素至少维持4周（达到完全缓解的患者），但不超过16周（未达到完全缓解的患者）（2C）5.1.4：达到缓解后，建议糖皮质激素在6个月内缓慢减量（2D）5.1.5：对于使用糖皮质激素有相