环磷酰胺冲击治疗难治性肾病综合征

难治性肾病综合征 (ＲＮＳ)是多病因、频复发、激素依赖和耐药及病情迁延的一种小儿常见病 ,临床治疗颇为棘手。我院自 1992年以来采用环磷酰胺 (ＣＴＸ)、强的松、保肾康联合治疗ＲＮＳ ,取得良好效果 ,现报告如下。资料与方法1　一般资料　共观察 43例 ,随机分为两组 :(1)治疗组 (加ＣＴＸ) 2 3例 ,男 15例 ,女 8例 ;年龄 <3岁 5例 ,3～ 7岁11例 ,7～ 14岁 7例。对照组 2 0例 ,男 13例 ,女 7例 ;年龄<3岁 3例 ,3～ 7岁 10例 ,7～ 14岁 7例。均为住院患儿 ,符合 1981年全国肾脏病学术会议修订的诊断标准与临床分型。两组病人中 ,单纯性肾病…     

活血化瘀方治疗难治性肾病综合征疗效观察

难治性肾病综合征（难治性ＮＳ）临床上治疗比较棘手，疗效差，复发率高。近年来，我们在常规用强的松和环磷酰胺（ＣＴＸ）的同时，应用叶任高教授的活血化瘀方，治疗难治性 ＮＳ４３例，取得了较好的疗效，现报道如下。 资料与方法 １ 临床资料 本组病人８６例，均为住院或门诊追踪的患者，随机分成２组。治疗组４３例，男２８例，女１５例；年龄１８岁～６０岁，病程８月～４８月；激素无效应８例，部分效应１７例，激素依赖７例，常复发１１例。对照组４３例，男２６例，女１７例；年龄１９岁～６０岁，病程８月～４６月；激素无效应７…     

霉酚酸酯治疗难治性肾病综合征疗效观察

为了观察霉酚酸酯治疗难治性肾病综合征的疗效和安全性，我们对16例难治性肾病综合征患者加用霉酚酸酯治疗，取得了较好的疗效，且副作用少，现报道如下。     

中西医结合治疗难治性肾病综合征疗效观察

自 1999年～ 2 0 0 0年我们对 14例难治性肾病综合征(ＮＳ)采用中西医结合治疗 ,疗效尚满意 ,现报告如下。资料与方法1　一般资料　 2 6例ＮＳ为住院患者 ,诊断符合文献标准 ,为原发性肾病综合征经首次激素治疗后完全缓解 ,但在1年内复发 4次或半年内复发 2次者。随机分为 2组 ,西医治疗组 (Ａ组 ) 12例 ,男 7例 ,女 5例 ;年龄 18岁～ 5 4岁 ;病程 6月～ 32月 ;重度水肿 (全身性水肿 ) 8例 ,中度水肿 (介于轻、重度水肿之间 ) 2例 ,轻度水肿 (晨起时颜面及眼睑水肿 ) 2例 ;实验室检查 :血胆固醇 6 .2 0～ 7.2 5ｍｍｏｌ/Ｌ ,血清白蛋白 16 .…     

双倍剂量雷公藤多甙治疗成人难治性原发性肾病综合征的疗效分析

自从1977年黎磊石等首次证实雷公藤对肾小球肾炎有减少蛋白尿、消水肿的作用以来,雷公藤制剂在肾科的应用日益广泛[1].随后胡伟新等首次报告应用双倍剂量雷公藤多甙(TⅡ)治疗原发性肾病综合征的疗效[2],有关雷公藤治疗肾病综合征量效关系的探讨此后并不多见.我们以双倍剂量雷公藤多甙治疗成人难治性原发性肾病综合征取得明显疗效,现总结报告如下.     

中西医结合治疗难治性肾病综合征疗效观察

难治性肾病综合征是指半年内2次复发或1年内3次复发者,对临床肾科医师而言颇感棘手,多年来笔者采用中药专方配合激素等治疗,大大提高了疗效,减少了复发.现报道如下.     

中西医结合治疗难治性肾病综合征的疗效观察

难治性肾病综合征不是一个独立性疾病,而是许多疾病过程中损伤了肾小球毛细血管滤过膜的通透性而发生的一组症候群.该病在临床治疗上比较棘手,疗效差,且复发率高.近年来我们采用中西医结合疗法,对31例难治性肾病综合征患者在常规激素治疗的基础上加用中药治疗,并进行对比观察现报告如下.     

多靶点治疗对难治性肾病综合征疗效观察

目的:探讨多靶点疗法对难治性肾病综合征(RNS)的疗效。方法:将40例RNS随机分两组,每组20例,治疗组采用多靶点疗法(泼尼松+MMF+FK506),对照组采用泼尼松+环磷酰胺治疗,两组均同时加服中药养阴益气、清利活血方,疗程12个月,观察疗效及相关生化指标和毒副反应。结果:治疗组完全缓解13例(65%),总有效率90%,对照组完全缓解6例(30%),总有效率70%,治疗组疗效显著高于对照组(P=0.027)。两组治疗后尿蛋白均比治疗前降低(P=0.000;P=0.018)、血清白蛋白均比治疗前增高(P=0.000;P=0.048),两组治疗后比较,治疗组尿蛋白低于对照组(P=0.017),血白蛋白高于对照组(P=0.000 4)。治疗组治疗后胆固醇、三酰甘油均显著降低(均P=0.000),两组本组治疗前后Scr均差异无统计学意义(P=0.214;P=0.88),两组治疗后Scr比较亦差异无统计学意义(P=0.310),两组均未发生严重的毒副反应。无因毒副反应退出治疗。结论:多靶点治疗RNS疗效显著,高于激素+环磷酰胺治疗,且毒副反应并不增加,不失为治疗RNS的一种有效且安全的治疗方案,值得临床推广应用。     

Mizoribine oral pulse therapy for steroid-dependent nephrotic syndrome

There have been reports of the use of mizoribine (MZB) oral pulse therapy for the treatment of systemic lupus erythematosus. We report its efficacy in a 9-year-old girl with steroid- and cyclosporine-dependent nephrotic syndrome (NS). The patient experienced relapses of NS when prednisolone was tapered to 20 mg/day after discontinuing cyclosporine due to biopsy proven toxicity. When methylprednisolone pulse therapy combined with prednisolone therapy (40 mg/day) failed to result in a complete remission after 3 weeks, oral MZB pulse therapy (total dose of 500 mg, 10 mg/kg per day in three divided daily doses twice a week) was given. This therapy was continued for 9 months and resulted in complete remission of the NS for 6 months despite the discontinuation of prednisolone. The serum concentration of MZB was above 2.5 g/ml for about 10 h (from 3 h after the first dose of MZB to 2 h after the final dose). Thus, our results suggest that this regimen may be effective for patients with steroid-dependent NS.     

Vincristine treatment in steroid-dependent nephrotic syndrome

Treatment of children with steroid-dependent nephrotic syndrome (SDNS) continues to be a challenge when relapses recur after treatment with cyclophosphamide and side effects or non-compliance make steroids and cyclosporin unsatisfactory. We treated 12 patients with intravenous vincristine for SDNS in a regime of 1–1.5 mg/m² weekly for 4 weeks then monthly for 4 months. Four of the 5 patients in relapse when commencing vincristine remitted within 2 doses. Comparing relapse frequency in the 12 months before and after vincristine, there was a reduction from 4 to 1.5 (p=0.004) relapses per year. Median sustained remission was 5 months, but 1 frequently relapsing patient remains in remission 4 years after vincristine. Vincristine was also successfully used in 1 or 2 doses at weekly intervals for subsequent relapses in 5 patients. Side effects were minimal in most cases. Abdominal pain occurred in 2 patients who commenced vincristine at 1.5 mg/m², but resolved when continued at 1 mg/m². We felt vincristine had a role in a subset of children with challenging SDNS administered as 1 mg/m² weekly for 4 weeks then 1.5 mg/m² monthly for 4 months. Vincristine allowed steroid- and cyclosporin-sparing, contributed to long-term remission in some patients, and was especially valuable in children with poor compliance with oral medication. Many patients expressed a preference for a few doses of vincristine rather than a standard course of oral prednisolone or cyclosporin.     

Clinical observations of mycophenolate mofetil therapy in refractory primary nephrotic syndrome

SUMMARY:   Mycophenolate mofetil (MMF) is an effective immunosuppressive agent in renal transplantation, and preliminary studies suggest that it may also be effective in the treatment of lupus nephritis. This study investigated the efficacy and safety of MMF therapy in patients with refractory primary nephrotic syndrome in a prospective multicentre clinical observation. Nineteen refractory nephrotic patients with minimal change disease or mesangial proliferative glomerulonephritis were enrolled in this study. Combined MMF and prednisone therapy was used for 6 months with an initial MMF dose of 1.0–2.0 g/day and a prednisone dose of 20–60 mg/day; both drugs were tapered gradually. It was found that all patients achieved clinical remission and 11 of 19 responded within 4 weeks, and 12 of 19 patients entered complete clinical remission. The prednisone dose in those patients who were previously steroid dependent could be successfully tapered. During follow up, three patients experienced transient increasing of proteinuria associated with infections and recovered without an adjustment of therapy. One patient was withdrawn from the study because of a fall in haemoglobin levels; other adverse effects did not necessitate withdrawal. Follow-up renal biopsies in two patients found no alteration in renal pathology. Mycophenolate mofetil is an effective and well-tolerated immunosuppressive agent for patients with refractory nephrotic syndrome.     

Oral mizoribine pulse therapy for patients with steroid-resistant and frequently relapsing steroid-dependent nephrotic syndrome.

BACKGROUND: We investigated the efficacy of oral mizoribine pulse therapy (mizoribine-pulse) for cyclosporin (CyA)-dependent, steroid-resistant nephrotic syndrome (SRNS) and frequently relapsing, steroid-dependent nephrotic syndrome (FR-SDNS). METHODS: One child with CyA-dependent SRNS and eight children with CyA-dependent FR-SDNS were treated with mizoribine-pulse (daily dose: 10 mg/kg; maximum total dose 500 mg). We compared clinical manifestations before and after mizoribine-pulse, and studied the changes in serum mizoribine concentration in each patient on days when mizoribine was administered. RESULTS: Four patients had no subsequent relapses (responders). Two of the four responders discontinued prednisolone and CyA, the other two discontinued CyA. Although each of the five other patients (non-responders) experienced single subsequent relapses, the dosages of prednisolone and CyA after mizoribine-pulse were decreased significantly compared with before mizoribine-pulse. The peak blood concentration of mizoribine in the responders was higher than in the non-responders (3.6+/-0.9 vs 1.8+/-0.4 microg/ml). CONCLUSIONS: Mizoribine-pulse may be effective for some patients with CyA-dependent SRNS and FR-SDNS.     

Vincristine in steroid-resistant nephrotic syndrome

The therapeutic response to vincristine was examined in seven children (aged 2–15 years) with corticosteroid-resistant (CR) nephrotic syndrome (NS) with focal and segmental glomerulosclerosis (FGS). Five were also resistant to cyclophosphamide. Vincristine was given weekly (1.5 mg/m² intravenously) for 8 weeks. Simultaneously, prednisone (60 mg/m² per day, orally) was given for 4 weeks and then gradually tapered. Two of these patients had a complete and stable remission; in five no benefit was observed. It was not possible to identify any characteristics to predict the response to vincristine. Although there is a case for trying vincristine therapy in CR NS with FGS, the results of this study are not encouraging and a better understanding of its action and indications is necessary.     

Remission of refractory minimal change nephrotic syndrome after basiliximab therapy

Minimal change nephrotic syndrome has been proposed to be a disorder of T cell dysfunction. It is hypothesized that a circulating factor(s) from activated T cells might alter glomerular permeability to protein. Some studies have provided evidence that up-regulation of interleukin-2 may be involved, not only in the pathophysiology of minimal change nephrotic syndrome, but also in steroid resistance. Basiliximab, an anti-interleukin-2 receptor antibody, is indicated for the prophylaxis of acute organ rejection in adults and children with kidney transplants. Clinical trials have shown that basiliximab is effective and well tolerated. We describe here a pediatric patient who continuously had massive proteinuria and hypoalbuminemia for 5 years, despite pulse therapy with methylprednisolone and cyclophosphamide and prolonged oral treatment with cyclosporine and mizoribine. He had experienced several disease- and treatment-associated complications, such as bacterial infections, indirect inguinal hernias, and cataracts. After he had been given a single dose of basiliximab, he achieved complete remission of proteinuria and then discontinued all immunosuppressant treatment.     

High-dose methylprednisolone therapy for nephrotic syndrome in Henoch-Schoenlein nephritis

Twelve patients with nephrotic syndrome (NS) in Henoch-Schoenlein (HS) nephritis were treated with a high dose of intravenous methylprednisolone (MP) on each of nine alternate days followed by oral prednisolone for 4 to 6 months. Renal biopsy was performed on 10 of the 12 patients. The glomerular change in 5 patients, which was accompanied by crescents and/or sclerosis, with NS and acute nephritic syndrome (ANS) at onset, was more severe than that of the other 5 patients with NS and hematuria at onset. The renal insufficiency or hypertension in these 5 patients with NS and ANS improved within 2 weeks on this MP therapy. After a mean follow-up period of 40.5 months, all patients except 2 revealed normal physical findings and renal function as well as urinary findings. Repeated biopsies in the 2 patients with NS and ANS at onset demonstrated an improved renal pathology in comparison with their initial biopsies. No severe side effects related to high-dose intravenous MP followed by oral prednisolone therapy were encountered in any of the patients. Our results suggest that high-dose intravenous MP therapy can lead to a favorable outcome in patients with NS in HS nephritis.     

High-dose mizoribine therapy for childhood-onset frequently relapsing steroid-dependent nephrotic syndrome with cyclosporin nephrotoxicity

Cyclosporin A (CsA) is an effective treatment for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal. We report 9 adolescent patients with childhood-onset FR-SDNS who had been treated with long-term CsA that resulted in moderate-to-severe CsA nephropathy (CsAN). They were treated with high-dose (mean: 10.1 mg/kg per day) mizoribine (MZR) in an attempt to allow weaning of CsA and/or steroid therapy, and reduce the frequency of relapses. Seven out of 9 patients were weaned off CsA by 1-year follow-up, although in the remaining 2 patients, MZR did not show any beneficial effects. Overall, this high-dose MZR therapy results in significant steroid sparing and reduction in relapse rates in our patients. Our experience shows that high-dose MZR therapy in patients with FR-SDNS who are also CsA-dependent appears to be effective in reducing CsA exposure as well as in decreasing the frequency of relapses.