Phadke K, Ballal S, Maiya V
Department of Pediatrics, Manipal Hospital, Bangalore.
Indian Pediatr. 1998 Feb;35(2):111-6.
To analyze the use of Cyclosporine (CyA) in nephrotic syndrome.
Thirty five children of mean age of 5.9 years with steroid dependent (n = 26) or steroid resistant (n = 9) primary nephrotic syndrome with normal renal functions and who received CyA were studied. CyA was used at a dosage of 6-7 mg/kg/day orally in two divided doses. The mean duration of therapy was 9.6 weeks. All received a minimum of 8 weeks of CyA therapy. In a few who received longer therapy, the dose was reduced to 4 mg/kg/day. All patients were monitored serially for hepatotoxicity and nephrotoxicity. The nephrotic state was evaluated serially with biochemical tests and followed up for a mean period of 2.55 years.
Thirty one patients completed the study. The response to therapy was categorized into 5 groups-no response (4 patients), good response (4 patients), partial response (4 patients), cyclosporine dependence (16 patients), and infrequent relapsers (3 patients). Good response was defined as complete remission lasting for at least one year after cessation of therapy. Patients who showed partial response had reduction in quantitative proteinuria and needed less diuretics. Sixteen patients went into complete remission while on therapy but relapsed within 3 months of discontinuation (CyA dependence). The response to CyA correlated more with steroid-responsiveness than with the underlying histopathology. The drug was well tolerated.
In steroid-dependent or steroid-resistant nephrotic children with normal renal functions, CyA therapy may be considered as one of the possible therapeutic options. Our results suggest that a longer duration of CyA therapy may possibly be indicated in these cases.
分析环孢素(CyA)在肾病综合征中的应用。
研究了35例平均年龄5.9岁、患有激素依赖型(n = 26)或激素抵抗型(n = 9)原发性肾病综合征且肾功能正常并接受CyA治疗的儿童。CyA口服剂量为6 - 7mg/kg/天,分两次服用。平均治疗持续时间为9.6周。所有患者至少接受8周的CyA治疗。少数接受更长疗程治疗的患者,剂量减至4mg/kg/天。对所有患者连续监测肝毒性和肾毒性。通过生化检测对肾病状态进行连续评估,并平均随访2.55年。
31例患者完成研究。治疗反应分为5组——无反应(4例患者)、良好反应(4例患者)、部分反应(4例患者)、环孢素依赖(16例患者)和偶发复发(3例患者)。良好反应定义为治疗停止后完全缓解持续至少一年。表现出部分反应的患者定量蛋白尿减少,所需利尿剂减少。16例患者在治疗期间进入完全缓解,但在停药后3个月内复发(环孢素依赖)。CyA的反应与激素反应性的相关性大于与潜在组织病理学的相关性。该药物耐受性良好。
对于肾功能正常的激素依赖型或激素抵抗型肾病儿童,CyA治疗可被视为可能的治疗选择之一。我们的结果表明,在这些病例中可能需要更长疗程的CyA治疗。