中西医结合治疗特发性膜性肾病临床观察

目的 :观察中西医结合治疗特发性膜性肾病临床疗效。方法 :对 6 8例特发性膜性肾病随机分为对照组 32例 ,治疗组 36例 ;对照组单纯用强的松和环磷酰胺、潘生丁、洛丁新 ,治疗组在用上述西药同时辨证加用中药治疗 ,观察 3年～ 6年。结果 :对照组完全缓解率为 2 8.1% ,总缓解率为 6 5 .6 % ;治疗组完全缓解率为 5 5 .6 % ,总缓解率为 86 .2 %。治疗组完全缓解率和总缓解率明显优于对照组 ,两组比较P <0 .0 5 ,具有显著性差异。结论 :中西医结合是治疗膜性肾病的有效方法。     

Immunosuppression can arrest progressive renal failure due to idiopathic membranous glomerulonephritis

The effect of pulse intravenous methylprednisolone therapy followed by oral immunosuppression was evaluated in ten patients with idiopathic membranous glomerulonephritis who had developed progressive renal failure--a group generally considered to have a poor prognosis. The patients (six male, four female, mean age 50 years) were monitored over 9-30 months during which time creatinine clearance reduced from (mean +/- SEM) 83 +/- 10 to 29 +/- 6 ml/min, and plasma creatinine increased from 135 +/- 22 to 297 +/- 35 mumol/l. All patient were nephrotic with mean 24-h urinary protein excretion ranging from 5.8 to 19.6 g. Treatment administered was pulse intravenous methyl-prednisolone 1 g X 3 then oral prednisolone 30 mg and azathioprine 50 mg (nine patients) or cyclophosphamide 50 mg (one patient). Mean prednisolone dosage was 25 mg at 3 months, 16 mg at 6, and 10 mg at 12 months. Patients have been followed up for between 12 and 57 months on therapy. Creatinine clearance increased to 39 +/- 6, 47 +/- 5 and 48 +/- 18 ml/min after 3, 6 and 12 months treatment with a fall in proteinuria to 6.2 +/- 1.7, 5.7 +/- 1.4, and 3.1 +/- 1.1 g/24h. The deterioration of renal function was reversed in six patients (associated with a reduction in proteinuria to less than 1 g/24 hours in five), slowed in three (with a significant reduction in proteinuria in two), and only one patient with more advanced renal failure before treatment progressed to end-stage failure without any retardation of the rate of deterioration or change in proteinuria.(ABSTRACT TRUNCATED AT 250 WORDS)     

Characteristics of patients with coexisting IgA nephropathy and membranous nephropathy

Background: Coexistence of IgA nephropathy (IgAN) and membranous nephropathy (MN) in the same patient is rare. Few studies have reported the clinical and pathological features of patients with combined IgAN and MN (IgAN–MN).

Methods: The clinico-pathological features, levels of galactose-deficient IgA1 (Gd-IgA1) and autoantibodies against M-type transmembrane phospholipase A₂ receptor (anti-PLA₂R) in sera were compared among IgAN–MN, IgAN, and MN patients.

Results: Twenty-six patients with biopsy-proven IgAN–MN were enrolled. The mean age at biopsy was 43.6?±?15.9?years, and 65.4% were male. Proteinuria and estimated glomerular filtration rate (eGFR) levels in patients with IgAN–MN were similar to that of MN patients. Compared with the IgAN patients, IgAN–MN patients showed a higher median proteinuria level (4.3 vs. 1.2?g/day, p?2, p?p?=?.801). Percentage of IgAN–MN patients with detectable serum levels of anti-PLA₂R was lower than that of MN patients (38.5% vs. 68.6%, p?=?.011).

Conclusions: IgAN–MN patients display similar clinical features to MN patients and milder pathological lesions than IgAN patients. IgAN–MN patients have similar levels of Gd-IgA1 to those of IgAN patients, and a lower proportion of anti-PLA₂R than MN patients.     

改良Ponticelli方案治疗特发性膜性肾病的疗效观察与护理

目的探讨改良Ponticelli方案治疗中度及高度危险特发性膜性肾病过程疗效与临床护理对策。方法选取在我院肾内科住院治疗的诊断为特发性膜性肾病的患者22例,其危险度分级为中度及重度危险度,或者为既往经激素及环磷酰胺(CTX)治疗无效者,针对这些患者分别予以不同的护理措施。结果完全缓解14例,部分缓解5例,总有效率为86%。患者总体的耐受性较好,但是仍有少数患者有肝损、骨髓抑制及感染情况的发生。结论改良的Ponticelli方案治疗中度及高度危险特发性膜性肾病的疗效比较满意,需要在临床护理工作中密切监测,注意副反应的情况,指导患者配合治疗及做好相应的防范措施。     

Membranous nephropathy with light chain restricted deposits

The literature on membranous nephropathy (MN) with monoclonal deposits on immunofluorescence (IF) and their outcome is very scarce. We report our experience of managing five patients with this clinical entity. The mean age of the patients was 33.2 ± 6.55 years. The mean proteinuria, serum albumin and serum creatinine was 5.73 ± 2.17 g/day, 2.86 ± 0.51 g/dL and 1.34 ± 1.19 mg/dL, respectively. None of the patients had a lymphoproliferative disorder. Only one patient had an elevated free light chain ratio. Four (80%) patients were M‐type phospholipase A2 receptor (PLA2R) negative (tissue and serum), and one (20%) was PLA2R related. Three (60%) cases had monoclonal IgG3/k, one IgG3/λ, whereas one patient with PLA2R positivity had an IgG3/IgG4k subtype. Two (67%) patients treated with cyclical cyclophosphamide and steroids (cCYC/GC) achieved complete remission and one patient (33%) with elevated baseline creatinine had a reduction in serum creatinine with persistent proteinuria at the end of the 12th month of follow‐up. One patient with PLA2R positive MN was treated with Rituximab and is in complete remission. The patient with an elevated free light chain at baseline was treated with Bortezomib/Thalidomide/Dexamethasone, had complete remission at 12 months, however, had a progressive rise in creatinine over the next 40 months of follow‐up. The current series, though limited by numbers, documents the efficacy of conventional therapies in non‐malignant associated MN with monoclonal deposits on IF.     

霉酚酸酯治疗特发性膜性肾病的荟萃分析

目的 评价霉酚酸酯治疗特发性膜性肾病的临床疗效和不良反应。 方法 检索了PubMed-Medline、EMBASE和Cochrane Library电子数据库的霉酚酸酯治疗特发性膜性肾病的随机对照试验或临床对照试验。疗效使用优势比（OR）进行计算评估;不良反应用相对危险度（RR）进行计算评估。 结果 3个随机对照试验和1个临床对照试验,共计141例被纳入本次研究。3个随机对照试验分别比较了霉酚酸酯与环磷酰胺、非免疫抑制剂的保守治疗和苯丁酸氮芥的疗效。1个临床对照试验比较了霉酚酸酯与环磷酰胺的疗效。与对照组相比,霉酚酸酯治疗特发性膜性肾病的临床完全缓解、部分缓解和总缓解率的OR（95%CI）分别为0.74（0.32~1.75）、0.56（0.28~1.11）和0.46（0.23~0.92）。表明霉酚酸酯治疗特发性膜性肾病的临床完全缓解、部分缓解率与对照组相当（P > 0.05）,而总缓解率明显低于对照组（P < 0.05）。霉酚酸酯与其他免疫抑制剂在感染、胃肠道症状和贫血等不良反应差异无统计学意义,但霉酚酸酯可以降低白细胞减少症的发生率,其RR（95%CI）为 0.06（0.01~0.41)。 结论 不推荐霉酚酸酯作为特发性膜性肾病的首选用药。     

吗替麦考酚酯联合激素治疗膜性肾病临床对照观察

目的观察吗替麦考酚酯（MMF）联合激素治疗膜性肾病的临床疗效及安全性。方法将40例临床表现为肾病综合征肾活检明确为原发性膜性肾病（PMN）的患者随机分为两组。MMF治疗组采用MMF联合泼尼松龙治疗：环磷酰胺（CTX）对照组采用间断静脉CTX冲击联合泼尼松龙治疗：动态观察疗效及不良反应发生情况。结果经过6个月和12个月的治疗,与治疗前相比,两组尿蛋白均显著下降（P〈0．05）,血浆清蛋白均显著回升（P〈0．05）,血总胆固醇均显著下降（P〈0．05）,治疗12个月时,MMF治疗组完全缓解率和总有效率分另1为20％和60％,CTX对照组分别为20％和55％。两组间相同时间观察指标比较差异无统计学意义（P〉0．05）。MMF治疗组发生胃肠道症状及转氨酶升高各1例,而CTX对照组发生肺部感染、带状疱疹及闭经各1例,胃肠道症状2例,白细胞减少、转氨酶升高各3例,MMF治疗组不良反应发生轻微,且不良反应发生率明显低于CTX对照组（P〈0．05）。结论MMF联合激素治疗膜性肾病的疗效与环磷酰胺联合激素治疗的疗效相同,但MMF治疗的不良反应小,耐受性好。     

ACEI联合ARB治疗早期特发性膜性肾病疗效观察

目的探讨贝那普利联合缬沙坦对早期特发性膜性肾病的临床疗效。方法选择早期特发性膜性肾病患者62例,采用前瞻、随机、对照研究,分为对照组(Control)、缬沙坦组(valsartan)、贝那普利组(Benazepril)、贝那普利联合缬沙坦组(Benazepril+valsartan)。Control组仅给予限制蛋白饮食,血脂升高者给予降脂等一般治疗;缬沙坦组组在对照组基础上给予valsartan 80 mg/d,如无副作用及低血压增加剂量至160 mg/d;Benazepril组在对照组基础上给予Benaze-pril 10 mg/d,如无副作用及低血压增加剂量至20 mg/d;Benazepril+valsartan组在valsartan组基础上合用Benazepril 10～20 mg/d,1次/d;以上各组疗程共8周。结果与治疗前比较,除对照组外,valsartan组、Benazepril组与Benazepril+val-sartan组24小时尿白蛋白量均有下降(P<0.01);与其它组比较,Benazepril+valsartan组作用更显著(P<0.05)。结论贝那普利联合缬沙坦可以快速降低早期特发性膜性肾病的尿蛋白排泄量,提升白蛋白水平,促使病情缓解。     

Recurrent Idiopathic Membranous Nephropathy: Early Diagnosis by Protocol Biopsies and Treatment with Anti‐CD20 Monoclonal Antibodies

Membranous nephropathy (MN) recurs posttransplant in 42% of patients. We compared MN recurrence rates in a historical cohort transplanted between 1990 and 1999 and in a current cohort diagnosed by protocol biopsies, we analyzed the progression of the disease and we assessed the effects of anti‐CD20 antibodies (Rituximab) on recurrent MN. The incidence of recurrent MN was similar in the historical (53%) and the current cohorts (41%), although in the later the diagnosis was made earlier (median, 4[2–21] months vs. 83[6–149], p = 0.002) and the disease was clinically milder. Twelve out of 14 patients (86%) with recurrent MN in the current cohort had progressive increases in proteinuria. Eight recipients were treated with Rituximab after their proteinuria increased from median, 211 mg/day (64–4898) at diagnosis to 4489 (898–13 855) (p = 0.038). Twelve months post‐Rituximab, 75% of patients had either partial (PR) or complete remission (CR). After 24 months 6/7 (86%) had PR/CR and one patient relapsed. Posttreatment biopsies showed resorption of electron dense immune deposits in 6/7 cases and were negative for C3 (4/7) and IgG (3/7). Protocol biopsies allow early diagnosis of subclinical recurrent MN, which is often progressive. Treatment of recurrent MN with Rituximab is promising and should be evaluated in a prospective randomized controlled trial.