Waldo F B, Benfield M R, Kohaut E C
University of Alabama at Birmingham, Department of Pediatrics, Children's Hospital, 35233, USA.
Pediatr Nephrol. 1998 Jun;12(5):397-400. doi: 10.1007/s004670050473.
Patients with steroid-resistant focal and segmental glomerulosclerosis (FSGS) have a poor prognosis but may benefit from high-dose methylprednisolone or cyclosporine A therapy. Ten patients were treated with a protocol of methylprednisolone infusions for 8 weeks followed by a combination of cyclosporine A and alternate-day prednisone for maintenance of remission for 2 weeks. Eight of ten patients remitted the nephrotic syndrome within 8 weeks of beginning treatment. One patient remitted edema but remained proteinuric, and one did not respond. After observation for 12-24 months, seven patients maintained remission with normal glomerular filtration rate. One non-responder had renal insufficiency and one patient had secondary non-response and end-stage renal disease. No patients developed hypertension. One patient had the diagnosis of Hodgkin disease made after 10 months of therapy. Follow-up renal biopsy in four patients showed no evidence of progressive interstitial disease. There were no other major side effects. Steroid-resistant FSGS may be successfully treated with the described protocol. Additional studies will be needed to determine if this approach prevents progression of renal disease.
患有激素抵抗性局灶节段性肾小球硬化（FSGS）的患者预后较差，但可能从大剂量甲基强的松龙或环孢素A治疗中获益。10例患者接受了甲基强的松龙输注方案治疗8周，随后联合环孢素A和隔日泼尼松维持缓解2周。10例患者中有8例在开始治疗的8周内肾病综合征缓解。1例患者水肿缓解但仍有蛋白尿，1例无反应。观察12 - 24个月后，7例患者维持缓解且肾小球滤过率正常。1例无反应者出现肾功能不全，1例患者出现继发性无反应并发展为终末期肾病。无患者发生高血压。1例患者在治疗10个月后被诊断为霍奇金病。4例患者的随访肾活检未显示进行性间质疾病的证据。无其他主要副作用。激素抵抗性FSGS可用所述方案成功治疗。需要进一步研究以确定这种方法是否能预防肾病进展。
