Hirano Daishi, Nishizaki Naoto, Kanai Hiroaki, Hara Satoshi, Ohtomo Yoshiyuki, Umino Daisuke, Fujinaga Shuichiro
Division of Nephrology, Saitama Children's Medical Center, Saitama, Japan.
Nihon Jinzo Gakkai Shi. 2010;52(8):1029-36.
We retrospectively analyzed the long-term outcome of 82 children (SRNS group, 10; SDNS group, 35; IRNS group, 37) who were initially treated with the ISKDC regimen at the Saitama Children's Medical Center. The ISKDC regimen consisted of PSL 60 mg/m2/day for 4 weeks, followed by 40 mg/m2 on alternate days for another 4 weeks. The aims of our study were to identify factors at onset that could predict the relapse pattern after using the initial ISKDC regimen, and to assess the prognosis and renal histology after long-term CsA therapy in 31 children. All of six asymptomatic children without edema and identified by chance proteinuria on a urinary screening program had an extremely favorable clinical course. Initial remission time of 9 or more days and the time interval from the initial therapy to the first relapse were significant predictors of steroid dependency. The sensitivity and specificity of these findings were 100% and 90%, respectively, with a positive predictive value of 95% and a negative predictive value of 100%. In addition, after the introduction of CsA therapy, termination of steroid therapy was achieved in 56% of patients with SRNS, and 64% of SDNS, respectively. However, after CsA therapy was tapered or stopped, most patients (21/20: 95%) developed relapses of NS. Of these, 76% (16/21) returned to SDNS, resulting in the reintroduction of CsA. Ten of 22 patients taking CsA (mean duration 31.3 months) had chronic nephrotoxicity. In conclusion, the initial ISKDC regimen is useful for the early prediction of whether or not the patient will develop SDNS. When pediatric nephrologists introduce CsA therapy in children with SDNS, an alternative strategy after long-term use of the agent should be considered.
我们回顾性分析了82例儿童（SRNS组10例、SDNS组35例、IRNS组37例）的长期预后，这些儿童最初在埼玉儿童医疗中心接受ISKDC方案治疗。ISKDC方案包括泼尼松龙60mg/m²/天，共4周，随后隔天40mg/m²，再持续4周。我们研究的目的是确定起病时能够预测使用初始ISKDC方案后复发模式的因素，并评估31例儿童长期使用环孢素A（CsA）治疗后的预后及肾脏组织学情况。在尿液筛查项目中偶然发现蛋白尿且无水肿的6例无症状儿童，其临床病程均极为良好。初始缓解时间达9天或更长以及从初始治疗至首次复发的时间间隔是激素依赖的显著预测因素。这些发现的敏感性和特异性分别为100%和90%，阳性预测值为95%，阴性预测值为100%。此外，在引入CsA治疗后，SRNS患者和SDNS患者中分别有56%和64%实现了激素治疗的终止。然而，在CsA治疗减量或停药后，大多数患者（21/20：95%）出现了肾病综合征复发。其中，76%（16/21）复发为SDNS，导致再次引入CsA。22例服用CsA的患者（平均疗程31.3个月）中有10例出现慢性肾毒性。总之，初始ISKDC方案有助于早期预测患者是否会发展为SDNS。当儿科肾脏病学家对SDNS儿童引入CsA治疗时，应考虑长期使用该药物后的替代策略。
