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Efficacy of mycophenolate treatment in adults with steroid-dependent/frequently relapsing idiopathic nephrotic syndrome

机译:麦考酚酯治疗成人类固醇依赖/反复发作的特发性肾病综合征的疗效

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Background This study assessed the efficacy of therapy with mycophenolate (MF) and reduced doses of steroids in adults with steroid-dependent/frequently relapsing idiopathic nephrotic syndrome (SD/FR-INS). Methods Twenty-nine nephrotic patients (including 16 males and 13 females; mean age: 40 years, range: 18–74) were treated. Starting doses of MF were 2000?mg/day for mofetil MF (1500?mg/day in one patient) or 1440?mg/day for sodium MF. The initial prednisone (PDN) dose was 10?mg/day in 14 patients, 5?mg/day in two patients and no steroids in one patient. In the remaining 12 patients, moderate initial doses of PDN were administered (mean: 23.7?mg/day, range: 15–40), tapering to 10?mg/day after 1?month. Results Nephrotic syndrome remission was achieved in 27/29 cases (93.1%) (25 complete, 2 partial). Two patients showed resistance to the prescribed schedule. The first cycle of MF therapy was concluded in 20 patients after a mean (range) of 16.9?months (12–49). Maintenance of remission was observed in 11 of these 20 cases (55%) after a mean follow-up of 32.8?months (12–108). In nine patients with nephrotic syndrome relapse after tapering of MF (MF dependency), the same MF-PDN schedule was restarted, leading again to remission in all nine. The remaining seven MF-sensitive patients are still receiving their first therapeutic cycle. To date, the mean time under therapy in the 27 MF-sensitive patients is 38?months (4–216). Regarding complications, only minor digestive disorders and a slight decrease in blood haemoglobin levels were observed in a few patients. Conclusions MF plus reduced doses of PDN is an effective and well-tolerated therapy for adult SD/FR-INS. Though MF dependence is observed, its low toxicity could allow long periods of therapy if it is required to maintain nephrotic syndrome remission.
机译:背景本研究评估了麦考酚酸酯(MF)和降低类固醇依赖剂量对成年激素依赖型/复发性特发性肾病综合征(SD / FR-INS)成年人的疗效。方法治疗29例肾病患者(男16例,女13例;平均年龄:40岁,范围:18-74)。 Mofetil MF的MF起始剂量为2000?mg /天(一名患者为1500?mg /天)或MF钠为1440?mg /天。泼尼松初始剂量(PDN)在14例患者中为10?mg /天,在2例患者中为5?mg /天,在1例患者中无类固醇。在其余12例患者中,给予中等剂量的PDN初始剂量(平均:23.7?mg /天,范围:15-40),在1个月后逐渐减少至10?mg /天。结果肾病综合征缓解率达到27/29例,占93.1%(25例,部分2例)。两名患者对处方表显示有抵抗力。 MF治疗的第一个周期在平均(范围)为16.9个月(12-49)之后结束了20例患者。在平均随访32.8个月(12-108个月)后,在这20例病例中有11例(55%)观察到维持缓解。在9名MF逐渐减少(MF依赖性)后复发的肾病综合征患者中,重新开始了相同的MF-PDN时间表,导致所有9名患者再次缓解。其余7名对MF敏感的患者仍在接受第一个治疗周期。迄今为止,在27位对MF敏感的患者中,平均接受治疗的时间为38个月(4–216)。关于并发症,在少数患者中仅观察到轻微的消化系统疾病和血液中血红蛋白水平的轻微下降。结论MF加降低剂量的PDN是成人SD / FR-INS的有效且耐受良好的疗法。尽管观察到MF依赖性,但是如果需要维持肾病综合征缓解,其低毒性可以允许长期治疗。

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