Donia Ahmed F, Amer Galal M, Ahmed Hassan A, Gazareen Sanaa H, Moustafa Fatma E, Shoeib Ahmed A, Ismail Amani M, Khamis Said, Sobh Mohamed A
Urology and Nephrology Center, Mansoura University, Mansoura, Egypt.
Pediatr Nephrol. 2002 May;17(5):355-8. doi: 10.1007/s00467-001-0813-y.
In children with minimal change nephrotic syndrome (MCNS), the steroid dependent group constitutes an especially difficult case for management. Patients in this group are prone to serious steroid side effects. Additionally, alkylating agents commonly fail to maintain remission and expose patients to more side effects. Therapy with the immunostimulant drug levamisole may therefore be another option in the attempt to maintain remission with minimal side effects. We prospectively treated 20 of our steroid dependent primary MCNS patients with levamisole. All patients were children, with an age range of 3-15 years; 16 were boys and 4 were girls. Remission was firstly induced by steroids, then levamisole was added in a dose of 2.5 mg/kg body weight on alternate days for 6 months. During this period we attempted to withdraw steroids completely and maintain patients on levamisole alone. We followed up our patients for the occurrence of relapse and side effects during this period and for a further 6 months after stopping levamisole. In 11 out of 20 children (55%), we successfully stopped steroids for more than 2 weeks. At the end of the 6-month treatment period (i.e. after 4 months of steroid discontinuation), ten patients (50%) were maintaining remission on levamisole alone. At the end of the 12-month study period (i.e. after 6 months of levamisole discontinuation), five patients (25%) were still in remission without any treatment for the previous 6 months. No significant side effects were reported during levamisole therapy. None of the patients developed neutropenia, but the leukocyte count showed a significant reduction in those who responded to levamisole treatment. We concluded that levamisole therapy for 6 months is a safe and perhaps effective therapy in a subset of children with steroid dependent MCNS to enable an otherwise infeasible withdrawal of steroids. This may be worth a trial before other types of more hazardous adjunctive therapies are considered.
在微小病变型肾病综合征(MCNS)患儿中,激素依赖组的治疗尤其棘手。该组患者易出现严重的激素副作用。此外,烷化剂通常无法维持缓解状态,且会使患者面临更多副作用。因此,使用免疫刺激药物左旋咪唑进行治疗可能是另一种以最小副作用维持缓解的选择。我们对20例激素依赖型原发性MCNS患者进行了左旋咪唑的前瞻性治疗。所有患者均为儿童,年龄在3至15岁之间;16例为男孩,4例为女孩。首先用激素诱导缓解,然后以2.5毫克/千克体重的剂量隔天添加左旋咪唑,持续6个月。在此期间,我们试图完全停用激素,仅用左旋咪唑维持治疗。我们对患者在此期间及停用左旋咪唑后的另外6个月进行随访,观察复发和副作用情况。20名儿童中有11名(55%)成功停用激素超过2周。在6个月治疗期结束时(即停用激素4个月后),10名患者(50%)仅用左旋咪唑维持缓解。在12个月研究期结束时(即停用左旋咪唑6个月后),5名患者(25%)在过去6个月未接受任何治疗的情况下仍处于缓解状态。左旋咪唑治疗期间未报告明显副作用。所有患者均未发生中性粒细胞减少,但对左旋咪唑治疗有反应的患者白细胞计数显著降低。我们得出结论,对于一部分激素依赖型MCNS患儿,6个月的左旋咪唑治疗是一种安全且可能有效的治疗方法,能够实现原本不可行的激素撤停。在考虑其他更具危险性的辅助治疗类型之前,这可能值得一试。