缓解:有高质量的证据表明,他克莫司或环孢素(环孢菌素)联合皮质类固醇对皮质类固醇耐药型肾病综合征患者诱导缓解有相似疗效。[25]Bagga A, Mantan M. Nephrotic syndrome in children. Indian J Med Res. 2005;122:13-28.http://www.icmr.nic.in/ijmr/2005/july/0701.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/16106086?tool=bestpractice.com[26]Colquitt JL, Kirby J, Green C, et al. The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic corticosteroid-resistant nephrotic syndrome: a systematic review. Health Technol Assess. 2007;11:iii-iv, ix-xi, 1-93.http://www.journalslibrary.nihr.ac.uk/hta/volume-11/issue-21http://www.ncbi.nlm.nih.gov/pubmed/17537341?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
缓解:有高质量的证据表明,他克莫司或环孢素(环孢菌素)联合皮质类固醇对皮质类固醇耐药型肾病综合征患者诱导缓解有相似疗效。[25]Bagga A, Mantan M. Nephrotic syndrome in children. Indian J Med Res. 2005;122:13-28.http://www.icmr.nic.in/ijmr/2005/july/0701.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/16106086?tool=bestpractice.com[26]Colquitt JL, Kirby J, Green C, et al. The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic corticosteroid-resistant nephrotic syndrome: a systematic review. Health Technol Assess. 2007;11:iii-iv, ix-xi, 1-93.http://www.journalslibrary.nihr.ac.uk/hta/volume-11/issue-21http://www.ncbi.nlm.nih.gov/pubmed/17537341?tool=bestpractice.com
肾病综合征的病因:对 521 例表现为肾病综合征而无系统性疾病并接受肾活检的儿童进行组织学统计分析如下:MCD 77%;膜性增生性肾小球肾炎 8%;局灶性节段性肾小球硬化症 7%;增生性肾小球肾炎、系膜增生、局灶性和球性肾小球硬化以及膜性肾小球肾病各 2%。[11]International Study of Kidney Disease in Children. Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int. 1978;13:159-165.http://www.ncbi.nlm.nih.gov/pubmed/713276?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
肾病综合征的病因:对 521 例表现为肾病综合征而无系统性疾病并接受肾活检的儿童进行组织学统计分析如下:MCD 77%;膜性增生性肾小球肾炎 8%;局灶性节段性肾小球硬化症 7%;增生性肾小球肾炎、系膜增生、局灶性和球性肾小球硬化以及膜性肾小球肾病各 2%。[11]International Study of Kidney Disease in Children. Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int. 1978;13:159-165.http://www.ncbi.nlm.nih.gov/pubmed/713276?tool=bestpractice.com
降低复发率:有中等质量的证据表明,较长疗程的泼尼松龙(泼尼松)治疗(60 mg/m2体表面积,每日口服,连服 6 周,然后 40 mg/m2体表面积,隔日口服,连服 6 周)对皮质类固醇敏感型肾病综合征的表现优于标准治疗方案(60 mg/m2体表面积,每日口服,连服 4 周,然后 40 mg/m2 体表面积,隔日口服,连服 4 周),因为它降低了随后的复发率,但没有增加严重皮质类固醇不良反应的风险。[24]Ehrich JH, Brodehl J. Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children. Arbeitsgemeinschaft fur Padiatrische Nephrologie. Eur J Pediatr. 1993;152:357-361.http://www.ncbi.nlm.nih.gov/pubmed/8482290?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
降低复发率:有中等质量的证据表明,较长疗程的泼尼松龙(泼尼松)治疗(60 mg/m2体表面积,每日口服,连服 6 周,然后 40 mg/m2体表面积,隔日口服,连服 6 周)对皮质类固醇敏感型肾病综合征的表现优于标准治疗方案(60 mg/m2体表面积,每日口服,连服 4 周,然后 40 mg/m2 体表面积,隔日口服,连服 4 周),因为它降低了随后的复发率,但没有增加严重皮质类固醇不良反应的风险。[24]Ehrich JH, Brodehl J. Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children. Arbeitsgemeinschaft fur Padiatrische Nephrologie. Eur J Pediatr. 1993;152:357-361.http://www.ncbi.nlm.nih.gov/pubmed/8482290?tool=bestpractice.com