Webb K L, Sargent P, Burke J R
Mater Misericordiae Children's Hospital, Brisbane, Queensland, Australia.
J Paediatr Child Health. 1993 Jun;29(3):188-91. doi: 10.1111/j.1440-1754.1993.tb00484.x.
Five children with multiple relapsing steroid-dependent nephrotic syndrome were treated with continuous cyclosporin for periods ranging from 18 to 48 months. Renal biopsy showed mild mesangial proliferation in three of the children and minimal change in two. All children previously had been treated with cyclophosphamide. Cyclosporin was started during remission at 5 mg/kg per day. If a relapse occurred the dose was increased until a trough blood level of 100-250 ng/mL (HPLC) was achieved. In the initial 12 months of treatment, the mean number of relapses decreased from 6.4 +/- 0.54 (s.d.) per annum to 1.6 +/- 1.3 per annum (P < 0.01). Cyclosporin was effective in maintaining long-term remission in four of the five patients. Side effects included hypertrichosis (5) and gum hyperplasia (1). The mean creatinine clearance decreased from 126 +/- 16 to 97 +/- 22 mL/min per 1.73 m2 (P = NS). A renal biopsy in all five patients after 12 months therapy showed no nephrotoxicity. A further biopsy in one patient after 4 years therapy showed interstitial fibrosis. Cyclosporin should be considered in children with steroid-dependent nephrotic syndrome who show signs of steroid toxicity and have only a short remission period after cyclophosphamide. Serial renal biopsies are recommended if prolonged therapy is used.
对5例患有多次复发性类固醇依赖型肾病综合征的儿童进行了持续环孢素治疗，治疗时间为18至48个月。肾活检显示，其中3例儿童有轻度系膜增生，2例为微小病变。所有儿童此前均接受过环磷酰胺治疗。环孢素在缓解期开始使用，剂量为每日5mg/kg。如果复发，剂量增加直至血药谷浓度达到100 - 250ng/mL（高效液相色谱法）。在治疗的最初12个月，每年复发的平均次数从6.4±0.54（标准差）降至1.6±1.3（P<0.01）。5例患者中有4例通过环孢素有效维持了长期缓解。副作用包括多毛症（5例）和牙龈增生（1例）。平均肌酐清除率从每1.73m² 126±16降至97±22 mL/分钟（P=无显著性差异）。12个月治疗后对所有5例患者进行的肾活检均未显示肾毒性。1例患者在治疗4年后进行的进一步活检显示有间质纤维化。对于有类固醇毒性迹象且在环磷酰胺治疗后缓解期较短的类固醇依赖型肾病综合征儿童，应考虑使用环孢素。如果采用长期治疗，建议进行系列肾活检。
