烷化剂在1例特发性膜性肾病合并肾病综合征的循证应用

目的报告应用烷化剂对1例特发性膜性肾病(IMN)合并肾病综合征(NS) 患者进行的循证治疗过程。方法计算机检索Cochrane图书馆(2005年第3期)、MEDLINE(1978-2005)、 CBM disc(1978～2005),查找烷化剂治疗IMN合并NS及与病情缓解有关的系统评价、临床随机对照试验等,获取最佳证据用于临床治疗。结果高质量的临床证据表明,烷化剂能明显提高IMN合并NS患者蛋白尿的完全缓解率,其中糖皮质激素 环磷酰胺(MP CTX)的方案是较安全和有效的,但其能否提高肾脏的长期存活率尚不确定。据此,笔者结合临床经验及患者意愿,对患者实施MP CTX免疫抑制治疗,6个月后患者蛋白尿情况有所缓解,目前仍在随访中。结论 MP CTX方案能明显提高IMN合并NS患者的蛋白尿缓解率,但能否提高患者肾脏长期存活率,尚有待进一步观察。     

Immunosuppression and outcome in idiopathic membranous nephropathy

From 1986 to 1996, 53 patients with idiopathic membranous nephropathy (IMN) presented to the Glasgow Royal Infirmary renal unit: 19 (36%) were treated because of progressive disease. We compared outcomes of treated and untreated patients with 37 historical and untreated controls. Five- and 10-year survival rates off dialysis were 83.5 and 53.5%, respectively. At the end of a mean observation period of 5.9 years, 47% of patients were in remission, 13% had reached end-stage renal failure, 15% had died, 13% had persistent proteinuria but stable renal function, and 11% had declining renal function. These results are better than those in historical controls, with a reduction in the number of patients reaching ESRF (13% vs. 22%), a larger proportion of patients achieving remission (47% vs. 30%) and smaller numbers of patients with declining renal function (11% vs. 19%) at the end of a similar follow-up period. These data suggest that the use of immunosuppression in selected patients with IMN improves prognosis, although the results did not achieve statistical significance.      

The beneficial effects of lymphocytapheresis for treatment of nephrotic syndrome.

A considerable permeability factor (or factors) derived from circulating T cells has a crucial role in proteinuria of nephrotic syndrome (NS). We attempted to remove pathogenic T cells through lymphocytapheresis (LCAP) in 6 patients with primary NS, 2 patients with minimal change nephrotic syndrome (MCNS), 2 patients with focal segmental glomerulosclerosis (FSGS), 1 patient with membranous nephropathy (MN), and 1 patient with MN and FSGS using Cellsorba (Asahi Medical Co., Osaka, Japan). LCAP was performed 2 times in 2 consecutive weeks and was followed with corticosteroid therapy with or without cyclosporine A in 5 patients. Two patients with MCNS, 1 with FSGS, and 1 with MN and FSGS showed a dramatic decrease of proteinuria (-30% and -94%) in their urine protein/creatinine ratio. Three out of 4 patients had a complete or partial remission (proteinuria <1g/day) within 8 weeks following immunosuppressive therapy. During the LCAP, T cells, especially activated T cells, decreased significantly in the response group. The other 2 patients, 1 with FSGS and 1 with MN, however, had no response to LCAP and following immunosuppressive therapy or low-density lipoprotein apheresis and suffered from end-stage renal failure or death by pneumonia. These results suggested that LCAP might have a beneficial effect on the treatment of NS, especially MCNS and in some patients with FSGS, despite varying responses to LCAP and concomitant immunosuppressive therapy.     

Use of Rituximab as an Off-Label Medication in Glomerular Diseases: Clinical Perspective

ObjectivesThe aim of this study was to review the use rituximab (RTX) and outcomes in immune-mediated glomerular diseases (glomerulonephritis [GN]) and to compare it to the established literature.MethodsAdult GN patients who received RTX between January 2014 and January 2018 in three public hospitals were reviewed. Membranous nephropathy (MN) and minimal change disease (MCD) were considered diseases with the literature supporting RTX use. Lupus nephritis (LN), primary focal segmental glomerulosclerosis (1^o FSGS), IgA nephropathy, IgG4-related disease (IgG4-RD), and C3GN had insufficient literature support for RTX use. Clinical remission was assessed 6 months after receiving RTX.ResultsA total of 61 cases were analyzed. RTX was an add-on therapy in 87%. The remission rate was 95% in the MCD and MN versus 56% in the off-label group (p = 0.002). LN patients had a mean initial estimated glomerular filtration rate (eGFR) of 69 mL/min. All class III LN achieved remission, and 11 of 21 class IV achieved remission. The mean initial eGFR for 1^o FSGS was 33 mL/min, and it did not improve, and only 2 of 5 had partial resolution of proteinuria. Proteinuria improved in 3 of 5 IgG4-RD cases with eGFR stabilization but failed to improve in C3GN cases with eGFR deterioration. Vasculitis cases (6 ANCA-associated vasculitis and 2 IgA vasculitis) were analyzed separately. Remission was achieved in only 2 ANCA-associated vasculitis cases, and none in IgA vasculitis cases.ConclusionsOur data support the use of RTX in resistant MCD and MN. RTX showed success in LN and IgG4-RD but not FSGS or C3GN. The small number of cases of vasculitis does not allow drawing a conclusion on RTX effectiveness.     

Restrictive use of immunosuppressive treatment in patients with idiopathic membranous nephropathy: high renal survival in a large patient cohort

BACKGROUND: Immunosuppressive treatment initiated at an early stage in patients with idiopathic membranous nephropathy (iMN) improves renal survival. Treatment should ideally be restricted to high-risk patients. AIM: To evaluate the efficacy of a restrictive immunosuppressive treatment strategy for patients with iMN. DESIGN: Prospective cohort study evaluating a predefined treatment protocol. METHODS: From 1988, we adopted a restrictive treatment strategy: immunosuppressive treatment, mainly consisting of cyclophosphamide and steroids, was advised only in patients with renal insufficiency or severe intolerable nephrotic syndrome. We evaluated this strategy in a large patient cohort. To exclude any bias, we included all adult patients with iMN biopsied in the study period with a serum creatinine (Scr) < 135 micromol/l, a proteinuria > or = 3.0 g/day and/or a serum albumin (Salb) < or = 30 g/l at the time of biopsy. Analysis was according to the intention-to-treat principle. RESULTS: We studied 69 patients. At the time of biopsy, mean age was 51 years, Scr 90 micromol/l, Salb 23 g/l and proteinuria 6.7 g/day. Average follow-up was 5.5 years. Thus far 33 (48%) patients have received immunosuppressive therapy, mainly because of renal insufficiency (n = 24). Status at the end of follow-up was: complete remission n = 22 (32%), partial remission n = 24 (35%), nephrotic syndrome n = 15 (22%), persistent proteinuria n = 1 (1.4%), ESRD n = 6 (8.7%), death n = 1 (1.4%; due to bladder carcinoma after cyclophosphamide therapy). Patient survival was 100% at 5 and 7 years. Renal survival was 94% at 5 years and 88% at 7 years. DISCUSSION: In patients with iMN, a restrictive treatment policy assures a favourable prognosis, while preventing exposure to immunosuppressive therapy in >50% of the patients.     

霉酚酸酯循证治疗特发性膜性肾病合并肾病综合征1例报告

目的报告1例特发性膜性肾病(IMN)合并肾病综合征(NS)患者应用霉酚酸酯进行循证治疗的结果。方法计算机检索Cochrane(2005年第3期)、MEDLINE(1978～2006)和CNKI(1978～2006),查找霉酚酸酯治疗IMN合并NS及与病情缓解有关的系统评价、临床随机对照试验、队列研究和病例对照研究等,并对所获证据进行质量评价,进而应用于本例患者的临床治疗。结果现有C级临床证据表明,霉酚酸酯能提高IMN合并NS患者的蛋白尿缓解率,对激素或烷化剂耐药的患者也有一定疗效,但能否提高肾脏的长期存活率尚无可靠证据。据此,结合医生经验及患者意愿,对患者采取霉酚酸酯联合糖皮质激素治疗,按患者体重(50kg)使用霉酚酸酯2．0g／d和强的松25 mg/d,并同时给予活血化瘀及对症支持治疗,3月后患者蛋白尿有所缓解。目前仍在随访中。结论现有C级证据表明短期内霉酚酸酯能提高IMN合并NS患者的蛋白尿缓解率,但能否提高患者肾脏长期存活率以及卫生经济学评价有待进一步研究。     

Rituximab in adult patients with multi-relapsing/steroid-dependent minimal change disease and focal segmental glomerulosclerosis: a report of 5 cases

Background

Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are common causes of nephrotic syndrome in children and adults. However, frequent relapses, steroid dependence, steroid resistance, and side effects of immunosuppressive therapy remain a therapeutic challenge. Rituximab (RTX) has evolved as an efficacious alternative in childhood MCD/FSGS. We report the effect of RTX in 5 adult patients with multirelapsing/steroid-dependent nephrotic syndrome due to MCD or FSGS.

Results

All five patients treated with RTX achieved sustained complete remission and additional immunosuppression was withdrawn. One patient had a relapse after 23 months, which was successfully treated with a further series of RTX infusion without reinitiation of steroid therapy. Serious adverse events related to RTX therapy were not observed in our patients.

Conclusions

Our results indicate that RTX is effective and cessation of additional immunosuppressants could be achieved in all patients reported in this study. RTX may be an effective alternative therapy in adult patients with multirelapsing/steroid-dependent nephrotic syndrome due to MCD or FSGS.     

The Nephrotic Syndrome in Adults with 'Minimal Change' Glomerular Lesions

We describe 49 patients who developed a nephrotic syndrome afterthe age of 15 years, and who showed a ‘minimal change’pattern in their renal biopsies. Patients with sclerosing lesionsaffecting only part of the glomerulus were excluded, but 39biopsies showed some minor changes. Glomerulosclerosis affectingwhole glomeruli was present in 21 biopsies, and related stronglyto increasing age. Focal tubular atrophy and vascular changeswere also common but less related to age. Mesangial matrix increasewas not age-related, and presumably is a component of the glomerulardisease. At onset, these patients showed features differing from thosefound in nephrotic children with similar histology: the sexincidence of the adults was equal, non-selective differentialprotein clearances (51 per cent) hypertension (31 per cent)and diminished renal function (70 per cent) were all more common.No clinical tests distinguished these patients clearly fromother adult nephrotics, and renal biopsy remains essential intheir management. The subsequent behaviour of the adult patientshowever, resembled that of the childhood group very closely.Forty four patients were treated with prednisolone for at leasteight weeks, and 82 per cent responded with early loss of proteinuria.Of the 36 responding patients, 70 per cent later relapsed, 63per cent repeatedly. Five corticosteroid-resistant, and 12 corticosteroidintolerantpatients were treated with cyclophosphamide: one died aftera pulmonary embolus, but 14 lost their proteinuria and 11 remainin remission. Follow-up data were available for all 49 patients for up to19 years (mean 4.1 years). Nine patients were dead, only onein uraemia but three from probable complications of treatment.Ten still relapse and require corticosteroid treatment, butonly one now has persistent symptomless proteinuria. Twenty-nineare well, off all treatment.     

Membranous nephropathy. Long-term follow-up and association with neoplasia.

Sixty-six patients of all ages whose renal biopsy appearances satisfied strict criteria for the histopathological diagnosis of membranous nephropathy were studied and followed for a mean of 5-4 years (range 1 to 20 years). From initial investigation seven patients were found to have associated neoplasia, and in two patients the condition followed treatment with a mercurial diuretic and gold. One patient was Australia antigen positive. Two patients developed renal vein thrombosis, but in both this appeared to follow not precede their nephrotic syndrome. In the remaining 56 patients there was no associated factor. During the follow-up period, approximately one-quarter of the patients (15) died, nine from renal failure; one-quarter (10) had a persistent nephrotic syndrome, another one-quarter (15) proteinuria of lesser degree. The final one-quarter (16) are now in complete remission. The prognosis of the 54 patients with an initial nephrotic syndrome was poorer than the 12 with lesser proteinuria and no oedema at onset; five of 11 children were in complete remission when last seen. All but one of the nine patients who developed terminal chronic renal failure 4 to 18 years from onset had an unremitting nephrotic syndrome, eight of the 10 currently alive with a persistent nephrotic syndrome have reduced renal function. Renal functional deterioration did not occur in the absence of proteinuria. There was only slight correspondence between the stage of biopsy appearance, glomerular filtration rate at time of biopsy, time of the biopsy from apparent onset, or status at last follow-up. Staging is therefore of limited prognostic value. Twenty-two patients were treated with corticosteroids for 2 to 36 months; we detected no short or long-term benefit when compared to patients not so treated.     

Membranous Nephropathy: LONG-TERM FOLLOW-UP AND ASSOCIATION WITH NEOPLASIA

Sixty-six patients of all ages whose renal biopsy appearancessatisfied strict criteria for the histopathological diagnosisof membranous nephropathy were studied and followed for a meanof 5-4 years (range 1 to 20 years). From initial investigationseven patients were found to have associated neoplasia, andin two patients the condition followed treatment with a mercurialdiuretic and gold. One patient was Australia antigen positive.Two patients developed renal vein thrombosis, but in both thisappeared to follow not precede their nephrotic syndrome. Inthe remaining 56 patients there was no associated factor. During the follow-up period, approximately one-quarter of thepatients (15) died, nine from renal failure; one-quarter (10)had a persistent nephrotic syndrome, another one-quarter (15)proteinuria of lesser degree. The final one-quarter (16) arenow in complete remission. The prognosis of the 54 patientswith an initial nephrotic syndrome was poorer than the 12 withlesser proteinuria and no oedema at onset; five of 11 childrenwere in complete remission when last seen. All but one of thenine patients who developed terminal chronic renal failure 4to 18 years from onset had an unremitting nephrotic syndrome,and eight of the 10 currently alive with a persistent nephroticsyndrome have reduced renal function. Renal functional deteriorationdid not occur in the absence of proteinuria. There was only slight correspondence between the stage of biopsyappearance, glomerular filtration rate at time of biopsy, timeof the biopsy from apparent onset, or status at last follow-up.Staging is therefore of limited prognostic value. Twenty-twopatients were treated with corticosteroids for 2 to 36 months;we detected no short or long-term benefit when compared to patientsnot so treated. ¹Present address: Princess Alexandra Hospital, Ipswich Road,Woolloongabba, Brisbane, Queensland, Australia.     

钙调磷酸酶抑制剂和环磷酰胺对2型糖尿病患者中特发性膜性肾病的疗效和预后比较

  目的  比较不同初始治疗方案对2型糖尿病患者中特发性膜性肾病(idiopathic membranous nephropathy,IMN)的疗效及预后。  方法  回顾性收集并分析2004年1月至2015年4月在北京协和医院确诊的2型糖尿病合并IMN患者临床资料。根据初始治疗方案将患者分为环磷酰胺(cyclophosphamide,CTX)组、钙调磷酸酶抑制剂(calcineurin inhibitors,CNIs)组和其他治疗组。采用Kaplan-Meier生存分析和Cox多因素回归分析,观察不同治疗方案对患者总体缓解 (total remission,TR)率、完全缓解 (complete remission,CR)率、估算肾小球滤过率 (estimated glomerular filtration rate,eGFR)下降 ≥ 30%或进入终末期肾病 (end-stage renal disease,ESRD)、空腹血糖>8 mmol/L的影响。  结果  共纳入89例符合入选和排除标准的2型糖尿病合并IMN患者。多因素回归分析显示,CNIs组和CTX组在CR(HR=0.793,95% CI:0.315~1.999,P=0.623)、TR (HR=0.647,95% CI:0.334~1.252,P=0.196) 及空腹血糖>8 mmol/L(HR=1.709,95% CI:0.669~4.369,P=0.263)方面均无统计学差异。但CNIs组出现eGFR下降 ≥ 30%或进入ESRD的风险显著高于CTX组(HR=13.505,95% CI:1.512~120.665,P=0.020)。  结论  在2型糖尿病合并IMN患者中,CNIs与CTX作为初始治疗蛋白尿缓解率相当,但CNIs治疗后肾功能下降的风险显著增加。对此类患者需谨慎选择初始治疗方案。     

Primary glomerulonephritis: an update on renal survival and determinants of progression

Background: Few epidemiological studies have investigated thelong-term outcome of primary glomerulonephritis (GN) and itsdeterminants in the decade since angiotensin-converting enzymeinhibitors entered widespread use. Aim: To study several traditional and less traditional riskfactors for kidney disease progression in a cohort of patientswith primary GN. Design: Retrospective cohort study. Methods: We included 536 patients with primary GN first diagnosedbetween 1994 and 2001: 283 IgA nephropathy (IgA), 129 membranousnephropathy (MN), and 124 focal and segmental glomerulosclerosis(FSGS). Adjusted hazard ratios (HR) or dialysis or preemptivetransplantation for end-stage renal disease (ESRD) accordingto various characteristics were estimated with Cox proportional-hazardmodels. Results: At diagnosis, mean patient age was 43 ± 17 years,74% were men, and the mean estimated glomerular filtration rate(eGFR) was 69 ± 31 mL/mn/1.73m². After a mean follow-upof 7-years, 104 patients had started ESRD treatment and 14 haddied before reaching ESRD. The 7-year renal survival rate was69% for FSGS, 88% for MN, and 82% for IgAN (p < 0.01). Inpatients with FSGS, younger age was associated with a higherrisk of ESRD. Baseline proteinuria, diabetes, and haemoglobin(Hb) concentration were strongly associated with shorter timeto ESRD independent of baseline eGFR, but gender, hypertensionand smoking were not. Adjusted HRs for ESRD were 2.6 [95% confidenceinterval, 1.2–5.8] for diabetes and 2.4 [1.3–4.5]for the lowest and 1.9 [1.0–3.6] for the intermediateHb tertiles versus the highest. Discussion: In patients with primary GN, renal survival is clearlylower for FSGS than for IgAN and MN. Independent predictorsfor progression were baseline diabetes and anaemia, as wellas proteinuria, for all GN types, and younger age, for FSGS.     

雷公藤多苷片联合缬沙坦治疗IgA肾病蛋白尿的疗效

目的探讨雷公藤多苷片联合缬沙坦治疗IgA肾病蛋白尿的临床疗效。方法将23例原发性IgA肾病患者（24 h尿蛋白定量1.0～3.0 g.d-1）按随机数字表法分为观察组12例与对照组11例。对照组给予缬沙坦胶囊80～160 mg,1次.d-1,顿服;观察组在此基础上给予雷公藤多苷片1 mg.Kg-1.d-1,分3次口服。疗程均为3个月。比较2组治疗后24 h尿蛋白定量缓解情况。结果对照组完全缓解1例,显著缓解3例,部分缓解3例,无效4例,总有效率63.64%。观察组完全缓解3例,显著缓解5例,部分缓解4例,无效0例,总有效率100.00%。2组总有效率相比差异有统计学意义（χ2=5.282,P=0.037）。结论雷公藤多苷片联合缬沙坦治疗IgA肾病蛋白尿的临床疗效确切,能更好地降低IgA肾病患者蛋白尿水平,保护患者的肾脏功能,且不良反应少。     

吗替麦考酚酯、小剂量激素、拉米夫定联合治疗原发性肾病综合征合并慢性乙型病毒性肝炎的疗效

目的 探讨吗替麦考酚酯、小剂量激素、拉米夫定联合治疗原发性肾病综合征合并慢性乙型病毒性肝炎的疗效及临床病理关系.方法 回顾性分析73例采用吗替麦考酚酯、小剂量激素、拉米夫定联合治疗的原发性肾病综合征合并慢性乙型病毒性肝炎患者的临床资料.结果 73例中完全缓解51例,部分缓解13例,无效9例,总有效率87.7%(64/73).治疗时间最短12个月,最长36个月.随访12个月,血清HBV-DNA转阴率54.8%(40/73).微小病变(MCD)完全缓解率100.0%(8/8 ),膜性肾病(MN)完全缓解率20.0%(3/15),系膜增殖性肾炎(MsPGN)完全缓解率83.3%(25/30),局灶节段增殖性肾炎(FSPGN)完全缓解率93.7%(15/16 ),局灶节段肾小球硬化(FSGS)完全缓解率50.0%(2/4 ).至末次随访时,所有患者均无肝肾功能减退.结论 吗替麦考酚酯、小剂量激素、拉米夫定联合治疗原发性肾病综合征合并慢性乙型病毒性肝炎安全有效,必要时疗程可适当延长.疗程与病理类型有关,以MCD治疗反应最好,FSPGN、MsPGN、FSGS次之,MN较差.     

A comparison of a standard-dose prednisone regimen and mycophenolate mofetil combined with a lower prednisone dose in chinese adults with idiopathic nephrotic syndrome who were carriers of hepatitis B surface antigen: A prospective cohort study

Background: When receiving immunosuppressive therapy, patients with idiopathic nephrotic syndrome who are also carriers of hepatitis B virus (HBV) surface antigen (HBsAg) are at risk for reactivation of HBV.Objective: This study compared the effectiveness and tolerability of a standard-dose prednisone regimen with those of the combination of mycophenolate mofetil (MMF) and a lower prednisone dose for the treatment of idiopathic nephrotic syndrome characterized by minimal-change nephropathy or slight mesangial proliferative glomerulonephritis in Chinese adults who were also carriers of HBsAg, a combination here termed MSNS-HBV.Methods: This was a prospective, open-label cohort study in Chinese adults with MSNS-HBV. Patients were self-assigned to 1 of 2 treatment groups: the standard prednisone regimen of 1 mg/kg daily or oral MMF 0.5 to 1.0 g BID combined with the lower pred-nisone dose of 0.5 mg/kg daily. The planned duration of treatment was 36 weeks, with an additional 60 weeks of follow-up. The primary outcome measures were rates of complete remission of idiopathic nephrotic syndrome (a decrease in daily proteinuria to within the normal range [<0.3 g]) and rates of HBV reactivation (detectable serum HBV DNA). Secondary outcome measures included relapse rates (>1+ albuminuria on dipstick urinalysis on 3 consecutive days), alanine ami-notransferase (ALT) elevations (>50 U/L), use of la-mivudine 100 mg/d (added if HBV DNA titers reached ≥10⁵ copies/mL), and adverse effects.Results: The intent-to-treat population included 41 patients (22 prednisone, 19 MMF). In patients who completed the study, rates of complete remission after 24 weeks of treatment were 78.9% (15/19) in the prednisone group and 76.5% (13/17) in the MMF group; 2 and 3 patients in the respective groups had a partial remission, and 2 and 1 patient had no response. HBV reactivation occurred in 63.6% (14/22) and 36.8% (7/19) of patients (P = 0.047). The only significant difference in the study was in the probability of HBV reactivation between groups (P = 0.043, log-rank test). During follow-up, at least 1 relapse occurred in 46.7% (7/15) and 30.8% (4/13) of patients. Elevations in ALT were observed in 36.4% (8/22) and 26.3% (5/19) of patients, and the addition of lamivu-dine was required in 40.9% (9/22) and 21.1% (4/19) of patients. The most frequent adverse effects in both groups were infections (27.3% and 26.3%), followed by gastrointestinal symptoms (13.6% and 21.1%). Two MMF patients developed leukopenia. One patient in the prednisone group discontinued treatment because of severe hepatitis, and 1 patient in the MMF group discontinued because of severe pulmonary infection.Conclusions: Among the adult Chinese patients with MSNS-HBV who completed this study, there were no significant differences in remission rates of idiopathic nephrotic syndrome between the standard prednisone regimen and the combination of MMF and a reduced prednisone dose. Rates of HBV reactivation, however, were significantly lower in the combination-therapy group.     

成人霍奇金淋巴瘤69例临床预后分析

目的 探讨霍奇金淋巴瘤(HL)的临床特点与预后的关系。方法 回顾2004年1~12月在我院初治的69例成人HL患者的临床资料,分析各临床特征与预后的关系。结果 ①50例(72.5%)患者以颈部淋巴结肿大起病,病理以混合细胞型最多见。②治疗有效率达76.8％(53/69)。5年总生存率及无失败生存率分别为80.0%和72.0%。③单因素分析显示年龄、分期、结外器官侵犯、脾大、淋巴瘤国际预后指数(International Prognostic Index,IPI)分组、血红蛋白、一线化疗方案、化疗疗程、缓解情况是影响生存的主要因素(P＜0.05)。④多因素分析显示缓解情况是影响HL患者生存的主要因素(P＜0.05)。结论 HL患者以颈部淋巴结肿大起病最常见,治疗后缓解情况是HL独立预后危险因素。     

Treatment of Idiopathic Membranous Nephropathy. Is the Anti-CD20 Antibody Rituximab a Reasonable Option?

BACKGROUND: Membranous nephropathy (MN) is characterized by proteinuria and other symptoms of the nephrotic syndrome. In many cases, the etiology is unknown. Whether and how to treat MN is still a controversial question. Despite the use of corticosteroids and alkylating agents, up to 40% of patients still progress to end-stage renal failure. CASE REPORT: A 40-year-old male patient with biopsy-proven idiopathic MN was initially treated with prednisolone and chlorambucil because of a proteinuria of 22 g/d. Treatment with cyclosporine was started because the nephrotic syndrome failed to improve. Proteinuria was reduced to a minimum of 4 g/d. Cyclosporine was stopped after 17 months leading to a fast relapse. Therapy with an ACE inhibitor and AT(1) receptor antagonist and retreatment with cyclosporine improved proteinuria. Cyclosporine was terminated after a total of 24 months. 5 months later, relapse occurred with a high proteinuria of 34 g/d. The monoclonal anti-CD20 antibody rituximab (375 mg/m(2)) was given four times every 4 weeks. 4 weeks and 4 months after the end of treatment, proteinuria decreased to 780 mg/d and < 150 mg/d, but renal function remained impaired (creatinine clearance 65 ml/min, stage 2 according to K/DOQI). Now, remission of proteinuria (< 150 mg/d) has been stable for almost 2 years. However, renal insufficiency progressed further (creatinine clearance 45 ml/min, stage 3 according to K/DOQI). CONCLUSION: Rituximab offers the possibility for a targeted treatment of idiopathic MN. Based on the existing evidence and experience from this case, rituximab can be recommended as a new treatment option for MN, possibly before starting any treatment with cytotoxic agents and high-dose prednisolone carrying the risk of severe side effects. However, long-term results of this treatment are still lacking.     

Treatment of nephrotic syndrome associated with idiopathic rapidly progressive glomerulonephritis and cyclosporin A

Recent reports suggest that cyclosporin A is beneficial in inducing remission of idiopathic nephrotic syndrome. Nephrotic syndrome is seen in 10–30% of patients with rapidly progressive glomerulonephritis. We report a case of a 69–year–old man with nephrotic syndrome, associated with idiopathic rapidly progressive glomerulonephritis, who was treated initially with corticosteroid and cyclophosphamide. Three months later he developed thrombophlebitis and leucopenia and cyclophosphamide was suspended. Relapse of nephrotic syndrome associated with rapidly progressive glomerulonephritis developed and therapy with cyclosporin A was used with a good response.     

地西他滨联合CAG方案与单纯CAG方案治疗中、高危骨髓增生异常综合征的疗效比较

本研究比较小剂量地西他滨联合CAG方案（阿克拉霉素、阿糖胞苷、粒细胞集落刺激因子）与单用CAG方案治疗中高危骨髓增生异常综合征的临床疗效及不良反应,探讨前方案作为中高危骨髓增生异常综合征新的治疗方法的可行性及有效性.2011年起小剂量地西他滨联合CAG治疗中高危骨髓增生异常综合征（MDS-RAEB）12例及2005年起既往单纯CAG方案治疗中高危MDS患者10例（治疗1个疗程后评估,至少使用2个疗程）疗效评估.分析接受两种方案的患者1个疗程的完全缓解率、总有效率、无病生存时间及总的生存时间.结果表明：小剂量地西他滨联合CAG方案治疗中高危MDS的12例患者一疗程后有完全缓解9例,2例部分缓解,1例完全无效,缓解率75.0％,总有效率91.7％,中位无病生存时间9（0-27）个月,中位总生存时间16（3-28）个月.治疗后肺部感染发生4例,予以抗感染治疗后均好转.既往2005年起10例中高危MDS患者予以单纯CAG方案治疗,完全缓解5例,部分缓解伴临床症状改善3例,2例无效,骨髓及血液学均无好转.缓解率50％,总有效率80％,中位无病生存时间6（0-18）个月,中位总生存时间13（3-31）个月.治疗后肺部感染3例,肠道感染1例,大肠杆菌败血症1例,予以积极抗感染治疗后均好转.两组患者治疗过程中未发生严重并发症,患者均能耐受,无治疗相关死亡.经统计分析,小剂量地西他滨联合CAG较单用CAG治疗中高危MDS有较长的无病生存时间（P值为0.013）,总体生存时间有延长趋势,但无统计学意义（P值0.087）.结论：小剂量地西他滨联合CAG治疗中高危MDS有较高的治疗效果,不增加治疗风险,可在临床推广.     

雷公藤多甙联合福辛普利治疗儿童肾病综合征型紫癜性肾炎疗效分析

目的观察雷公藤多甙联合福辛普利治疗儿童肾病综合征型紫癜性肾炎的治疗效果。方法2006年12月至2009年12月在我院儿科就诊的38例诊断为肾病综合征型紫癜性肾炎的患儿,分别将患儿随机分为治疗组20例（口服雷公藤多甙联合福辛普利）和对照组18例（口服雷公藤多甙）,观察药物对两组患儿水肿、高血压、血尿、蛋白尿的疗效,采用SPSS10．0软件进行统计学分析。结果治疗组完全缓解18例（90％）,部分缓解2例（10％）。对照组完全缓解11例（61％）,部分缓解6例（39％）,无效1例（6％）两组缓解率比较,差异无显著性（P〉0．05）,但治疗组水肿消退、血压恢复正常、血尿消失、尿蛋白转阴的平均时间均明显短于对照组,差异有显著性（P〈0．01）。结论雷公藤多甙联合福辛普利治疗儿童肾病综合征型紫癜性肾炎疗效优于单用雷公藤多甙,值得临床应用。