来氟米特及雷公藤多甙联合泼尼松治疗难治性肾病综合征

难治性肾病综合征治疗方案的研究是肾脏疾病临床工作的重要内容。我院采用来氟米特及雷公藤多甙联合泼尼松治疗难治性肾病综合征，取得良好的效果，现报道如下。     

调理脾胃法治疗难治性肾病综合征50例临床分析

难治性肾病综合征是指激素抵抗、激素依赖或反复发作，且不能耐受激素副作用而难以继续用药的原发性肾病综合征。难治性肾病综合征在临床上极为常见，患者病程迁延，病情反复，是肾脏内科的难题之一。     

左旋咪唑对难治性肾病综合征疗效的影响

<正>肾病综合征(nephrotic syndrome,NS)以大量蛋白尿,低蛋白血症、高脂血症、水肿为特征的一种临床常见肾脏疾病。糖皮质激素常作为其首选药物,根据对激素的反应,分为激素敏感型、激素抵抗型和激素依赖型或频繁复发,后两者可称为难治性肾病综合征。1难治性肾病综合征诊断标准常规使用泼尼松1 mg·kg-1·d-1,8周后临床症状不缓解的称之为肾上腺皮质激素抵抗;肾上腺皮质激素依赖在最初     

刘宝厚教授治疗难治性肾病综合征的用药经验

难治性肾病综合征(RNS)是指经正规足量泼尼松疗程治疗无效或对激素依赖或反复发作且不能耐受激素副作用而难以继续服药的原发性肾病综合征.在临床上较常见,治疗棘手.刘宝厚教授是我国著名的中西医结合肾病专家,多年来采用"中西医双重诊断,中西药有机结合"的方法,对于难治性肾病综合征的治疗形成了一套行之有效的用药规律,兹就其临证经验简述如下.     

聂莉芳教授辨治难治性肾病综合征水肿的临证经验

难治性肾病综合征是指激素抵抗、激素依赖或反复发作,且不能耐受激素副作用而难以继续用药的原发性肾病综合征[1].难治性肾病综合征的水肿在临床极为常见,运用西医的扩容利尿法往往难以奏效,成因主要与病理类型、激素使用与撤减不规范、感染因素、高凝状态及高脂血症等多方面综合因素相关,致使病程迁延,是肾脏内科的难题之一.聂莉芳教授是我国著名的肾病专家,她运用中医辨证治疗难治性肾病综合征的水肿,有较好的疗效.兹将聂教授的学术思想特点和临证经验介绍如下.     

霉酚酸酯与抵克立得治疗难治性肾病临床观察

原发性难治性肾病综合征指标准8周大剂量糖皮质激素(泼尼松1 mg·kg-1·d-1 )治疗病情无缓解.临床常见两种类型的肾病综合征(NS),一类是激素依赖或频繁复发型(指每年复发4次或半年复发2次),另一类是激素抵抗型.后者其严重性是10年后50%进入终末期肾衰竭.肾病综合征治疗缓解率与肾存活率呈正相关.目前对难治性肾病综合征尚无统一治疗方案,其关键在于难治因素比较复杂,即使同一方案,对同一病理类型也疗效各异,常让临床医师棘手.以往多局限于糖皮质激素(GC)及免疫抑制应用的研究,往往不能取得满意疗效,作者应用霉酚酸酯与抵克立得治疗难治性肾病,取得较好疗效.     

霉酚酸酯治疗难治性原发性肾病综合征疗效观察

霉酚酸酯（MMF）治疗成人狼疮肾炎和难治性肾病已显示了良好的疗效和安全性’‘’。我们观察了MMF治疗小儿难治性原发性肾病综合征的疗效和毒副作用,旨在探索一种治疗小儿难治性肾病综合征的新方法。 一、材料和方法 1 对象:ZI例难治性原发性肾病综合征为1999年川月-2000年10月在我科住院的患儿,诊断依据1981年全国儿科肾脏病科研协作组制订的标准l‘]。;男13例,女8例,年龄2岁10个月-16岁,平均年龄7岁6个月;其中,<6岁12例,6-12岁7例,门-16岁2例;病程6个月-7年。所有病例外周血细胞计数和肝肾     

原发性难治性肾病综合征治疗中注意的问题

原发性难治性肾病综合征指标准8周大剂量糖皮质激素(泼尼松1mg·kg-1·d-1)治疗病情无缓解.临床常见两种类型的肾病综合征(NS),一类是激素依赖或频繁复发型(指每年复发4次或半年复发2次),另一类是激素抵抗型.后者其严重性是10年后50%进入终末期肾衰竭.肾病综合征治疗缓解率与肾存活率呈正相关.目前对难治性肾病综合征尚无统一治疗方案,其关键在于难治因素比较复杂,即使同一方案,对同一病理类型也疗效各异,常让临床医师棘手.以往多局限于糖皮质激素(GC)及免疫抑制应用的研究,往往不能取得满意疗效,目前认为其多种并发症可影响肾病综合征治疗疗效,重者可致命,故对此应十分重视.     

肾炎灵颗粒剂治疗难治性肾病综合征的临床研究

目的 :评价肾炎灵颗粒剂治疗难治性肾病综合征的临床疗效及毒副反应。方法 :口服肾炎灵颗粒剂治疗 6 5例难治性肾病综合征患者 4月～ 6月 ,观察患者症状、体征变化和血生化、尿蛋白等检查结果以及与雷公藤多甙片副反应的对比。并对患者进行 6月～ 48月的随访。结果 :肾炎灵颗粒剂对难治性肾病综合征近期总有效率为 78.46 % ,对肾功能有保护作用 ,随访复发率仅为 15 .4% ,其常见副反应明显减少。结论 :肾炎灵颗粒剂对难治性肾病综合征有较好的治疗作用 ,临床副反应明显低于雷公藤。     

中西医结合治疗特发性膜性肾病疗效观察

特发性膜性肾病（ IMN）是成人肾病综合征中最常见的原因之一,膜性肾病属难治性肾病,现代医学仍缺乏较为公认的行之有效的治疗方案［1］。为寻求更加有效、安全的治疗方法,我们从2007年1月~2012年1月,在临床实践中应用中西医结合的方法治疗30例,现报告如下。     

他克莫司治疗难治性肾病综合征的新进展

难治性肾病综合征约占原发性肾病综合征的30%～50%,包括激素依赖型、激素抵抗型和频复发型,具有病程长、易复发等特点。若得不到合理治疗可导致疾病难以缓解,并可能出现严重并发症,甚至进展为不可逆性慢性肾衰竭,极大影响患者生存质量,并威胁生命。目前,难治性肾病综合征患者多加用免疫抑制剂治疗,以糖皮质激素联合烷化剂较为经典,但其潜在的性腺毒性、骨髓抑制及致癌等不良反应限制了临床应用。近年,国内外应用新型免疫抑制剂他克莫司在治疗难治性肾病综合征中取得了较好疗效,本文就他克莫司治疗难治性肾病综合征的疗效及安全性方面的新进展做一综述。     

肾病综合征1009例病理类型及流行病学分析

目的探讨肾活组织病理检查（下称肾活检）在肾病综合征诊治中的意义。方法选择近10年诊断为肾病综合征患者1009例，分析肾活组织穿刺检查术后的病理结果。结果原发性肾小球肾炎占71．9％，病理类型以非IgA系膜增生性肾炎、IgA肾病及膜性肾病多见；继发性肾病综合征占28．1％，其中居前3位的是狼疮性肾炎、过敏性紫癜性肾炎和乙肝病毒相关性肾炎。结论肾活检对肾病综合征的诊断、治疗及评估预后有重要意义。     

Congenital nephrotic syndrome

Congentital nephrotic syndrome (CNS) is an uncommon disorder. Several different diseases may cause the syndrome. These may be inherited, sporadic, acquired or part of a general malformation syndrome. The problems associated with nephrotic syndrome in early infancy are divided into three parts: diagnosis, treatment and prenatal diagnosis. Accurate diagnosis is essential for the treatment, genetic counselling and prenatal diagnosis. The ultimate curative treatment of CNS is renal transplantation. The supportive treatment before the transplantation is of utmost importance in order to maintain a reasonable clinical condition and prepare the child for the dialysis and renal transplantation. Prenatal diagnosis is possible in some types of CNS by determination of the maternal serum and amniotic fluid alpha-fetoprotein (AFP). Increased AFP indicates fetal proteinuria, and thereby nephrotic syndrome before birth. In some cases with the onset of proteinuria after birth prenatal AFP measurement does not detect the disease.     

Applying decision analysis to management of adolescent idiopathic nephrotic syndrome

Due to the concern that adolescents presenting with nephrotic syndrome are less likely to have minimal change disease than younger children, pediatric nephrologists have tended toward renal biopsy-tailored treatment rather than corticosteroid use in this population. The need for biopsy prior to treatment of nephrotic syndrome in adults has been challenged. A similar challenge to the clinical need for this procedure in adolescents with idiopathic nephrotic syndrome is raised here. The principles of medical decision analysis were applied and calculations were made with the Decision Maker computer program. The life expectancy of an adolescent who receives biopsytailored treatment was found to be no different from that of an adolescent who receives empiric corticosteroid treatment. We conclude that renal biopsy is not mandatory for the clinical management of adolescents with idiopathic nephrotic syndrome. Prognostically, a response, or lack thereof, to empiric corticosteroid therapy may be just as informative as a histological diagnosis.     

A Case with Acute Renal Failure and Subsequent Nephrotic Syndrome

Nephrotic syndrome due to secondary amyloidosis is not so common, and the prognosis depends on primary disease. We report a case of secondary amyloidosis caused by Takayasu's arteritis. Sustained high fever and acute renal failure proceeded to the occurrence of nephrotic syndrome. Secondary amyloidosis was diagnosed by renal biopsy before the diagnosis of primary disease. She was completely recovered from nephrotic syndrome after two years' treatment with prednisolone, aspirin, and dimethyl sulfoxide. This rare case provides meaningful suggestions for the diagnosis and treatment of acute renal failure and nephrotic syndrome caused by secondary amyloidosis.     

Strongyloidiasis as a possible cause of nephrotic syndrome

Chronic strongyloidiasis is a mild disease and has never been reported to be associated with nephrotic syndrome. Disseminated strongyloidiasis is known to have high mortality, but it frequently is not diagnosed until autopsy. We report a patient with nephrotic syndrome developing disseminated strongyloidiasis after steroid therapy. The findings in renal biopsy, the time course of the development, and resolution of nephrotic syndrome after thiabendazole treatment suggested a possible causal relationship between chronic strongyloidiasis and nephrotic syndrome. The case also demonstrated the importance of early diagnosis in disseminated strongyloidiasis and the good clinical outcome of early treatment before the development of organ failure.     

New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome

Although many children with idiopathic nephrotic syndrome (INS) respond initially to steroid therapy, repeated courses for patients with relapses often cause significant steroid toxicity. Patients with frequent relapses who develop steroid dependency thus require alternative treatment. The first such options have been considered to be cyclophosphamide or levamisole, although the latter is no longer available in many countries. There is also an increasing body of data indicating that mycophenolic acid (MPA) may be an alternative for these patients. Calcineurin inhibitors (cyclosporine A or tacrolimus) are usually effective and often used after cytotoxic treatment, but long-term treatment with these agents is necessary, raising concerns of a possible accumulation of side effects. Some patients show a tendency to relapse even on such maintenance regimens, and some even have a refractory course that creates a medical dilemma. For this situation, recent data indicate that monoclonal antibodies directed to B-cells (e.g. rituximab) may have some effect and that such drugs may also prove to be a therapeutic option in less complicated cases. Patients that do not respond to steroid treatment need genetic testing and a renal biopsy since focal segmental glomerulosclerosis (FSGS) may be present. Treatment options include pulse methylprednisolone, often in addition to calcineurin inhibitors, mainly in the form of cyclosporine, but tacrolimus has also come into recent favor. Some studies have found cytotoxic treatment, especially intravenous cyclophosphamide, to be effective in steroid resistant nephrotic syndrome, but it seems to be inferior to calcineurin inhibitors. MPA and rituximab have also been used in children with primary FSGS, but the response seems to be inferior to that in patients with steroid sensitive nephrotic syndrome. Taken together, INS in both steroid-sensitive and steroid-resistant patients is a potentially complicated disorder, and despite a wide arsenal of immunological interventions, some patients have a treatment refractory course. Prospective studies or at least standardized treatment for complicated cases is urgently needed.     

小剂量环孢素A联合中药治疗难治性肾病综合征疗效观察

目的研究小剂量环孢素联合中药治疗难治性肾病综合征的疗效。方法23例患者均接受中药柴苓汤、百令胶囊及小剂量环孢素A（2mg·kg^-1·d^-1）治疗，观察治疗前、后24h尿蛋白定量、肝功能、肾功能变化。结果24h尿蛋白定量较治疗前明显下降，血浆白蛋白在治疗后明显升高，总有效率为74％。结论小剂量环孢素联合中药治疗难治性肾病综合征疗效安全、可靠。     

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.     

Prognosis and risk factors for idiopathic membranous nephropathy with nephrotic syndrome in Japan

BACKGROUND: Idiopathic membranous nephropathy (IMN) is a representative form of refractory nephrotic syndrome in Japan. Although IMN is thought to run a more benign course in Japanese than in the Caucasian population, risk factors and appropriate treatment are controversial issues. METHODS: The research group supported by a grant for "Progressive Renal Disease" from the Ministry of Health, Labor and Welfare, Japan, carried out a national survey of patients with IMN and nephrotic syndrome. Of 1066 nephrotic patients with histopathologically proven IMN registered from 1975 to 1993 in 85 institutions, 949 patients were studied. RESULTS: The overall renal survival rates were 95.8%, 90.3%, 81.1%, and 60.5% at 5, 10, 15, and 20 years after diagnosis, respectively. When clinical and histopathologic features at onset of nephrotic syndrome were evaluated by multivariate analysis, male gender, old age (> or =60 years), high serum creatinine concentration (> or =1.5 mg/dL), and the development of tubulointerstitial lesions (> or =20% of the biopsy sample area) were significant predictors of progression to end-stage renal disease (ESRD). The renal survival rate in patients on steroid therapy was significantly higher than in patients on supportive therapy alone. Patients achieving a remission showed a significant reduction of risk for progression. CONCLUSION: IMN is a disease with a comparatively good prognosis in Japan even when it is associated with nephrotic syndrome. Steroid therapy, which has not been recommended for IMN in most review articles, seems to be useful at least for Japanese patients. In particular, a remission from heavy proteinuria likely results in a favorable outcome.