成人原发性肾小球肾炎远期预后的观察：（附1071例？…

目的：前瞻性观察各种病理类型原发性肾小球肾炎患者的远期预后,了解影响肾小球肾炎预后的各种因素。方法：１９８０年１月～１９９６年月１２月间,在本院经肾活检确诊并常年定期随访的原发性肾炎患者共１０７１例,其中ＩｇＡ肾病（ＩｇＡＮ）４７１例（４４．３％）、系膜增生性肾炎（ＭｓＰＧＮ）２６８例（２５．０％）、ＩｇＡ肾病（ＩｇＭＮ）８０例（７．３０％）、膜增生性肾小球肾炎（ＭＰＧＮ）６８例（６．３０％）、膜     

4298例成年人肾小球疾病病理类型及流行病学特点

了解我国成年人肾小球疾病病理类型及流行产现学特点。方法：分析解放军肾脏病研究所１９８０－１９９６年间经肾活检确诊的肾小球疾病４２９８例。结果：原发性肾小球肾炎占７６．８％，继发性肾小球肾炎２２．８％，遗传性肾病０．４％；原发性肾小球肾炎男：女＝１．８１：１，继发性肾小球肾炎男：女＝０．４６：１，两组间存在统计学差异；原发性肾小球肾炎各型所占比例依次是：ＩｇＡ肾病（ＩｇＡＮ）３６．９％、系膜增生性肾     

慢性肾小球疾病高血压及其对肾功能的影响

作者分析５２０例经肾活检的慢性肾小球疾病患者的临床资料，发现其高血压发病率为３２．７％，膜增生性肾炎、新月体肾炎及硬化性肾炎高血压发病率较高，ＩｇＡ肾病和微小病变肾病Ｙ高血压发病率较低。不同病理类型慢性肾脏病高血压发病率不同，降压疗法可改善肾功能，延缓肾衰发展速率。     

狼疮性肾炎17例病理和临床分析

对１７例狼疮性肾炎行肾穿刺活检，病理形态学分为：系膜增生型（Ⅱ型）３例（１７．６％），弥漫增生型（Ⅳ型）３例（１７．６％），膜型（Ⅴ型）１０例（５８．８％），进行性硬化型（Ⅵ型）１例（５．９％）。以膜型狼疮性肾炎最多。病程在５年内者１３例（７６．５％），疾病处于活动期，病理损害可见于各种类型，病理改变与病程无一定的关系，不同病理类型的狼疮性肾炎，其临床表现及预后有明显的差异。     

原发性干燥综合征26例合并肾脏损害临床及病理分析

为探讨原发性干燥综合征（ＳＳ）肾损害的临床及病理特征，对２６例住院原发性ＳＳ合并肾损害行肾活检病人的临床及病理表现进行了总结。结果显示：其肾损害的主要表现为肾小管酸中毒２０例、蛋白尿２１例及肾功能不全５例；２４例肾活检免疫病理示ＩｇＧ９例、ＩｇＡ８例、ＩｇＭ７例及Ｃ３１０例肾小球基底膜及系膜区沉积；病理表现为慢性间质性肾炎（ＩＮ）１０例，ＩＮ合并局灶节段增殖性肾小球肾炎４例、硬化性肾小球肾炎４例，弥漫系膜增殖性肾炎和膜增殖性肾炎各１例及膜性肾炎４例。该结果提示：原发性ＳＳ肾损害时，肾小球内免疫复合物沉积及肾小球肾炎并不少见，不容忽视。     

纤维样变肾小球病：——附四例报告

报告４例纤维样变肾小球病。４例病人无系统性疾病，主要临床表现为高血压，大量蛋白尿、镜下血尿。２例有不同程度的肾功能减退。光镜下病理类型为膜增殖性肾炎（２例）、膜性肾病（１例）和系膜增生性肾炎（１例），病理改变均较重，刚果红染色阴性；免疫荧光多数病例（３１４例）以ＩｇＧ、Ｃ＿３为主，呈颗粒样在肾小球系膜区和（或）沿肾小球毛细血管壁沉积；电镜下可见大量纤维样物质在肾小球系膜区和（或）肾小球基底膜内分布，纤维样物质直径经图象分析仪测量为２０．５０±１０．３７ｎｍ。本病诊断主要依靠超微结构检查。本病为国内首次报道。     

1217例肾活检患者临床与病理资料分析

目的 了解行肾穿刺活检患者的流行病学特点及病理类型与临床表现间的关系.方法 回顾性分析1217例行肾活检者的性别、年龄、病理类型及临床表现等相关资料.结果 1217例患者行肾活检时的平均年龄为（30.86±13.41）岁,男性612例（50.3％）,女性605例（49.7％）.男性占本组原发性肾小球疾病（PGN）的52.2％,继发性肾小球疾病（SGN）的39.0％.肾脏病患者的高发年龄段为20 ～39岁（50.9％）.本组患者最常见的临床表现为肾病综合征（NS）610例（50.1％）,其余分别为尿检异常型（Uab）453例（37.2％）,反复发作性肉眼血尿型（rGH）89例（7.3％）,慢性肾衰竭（CRF）31例（2.5％）,急性肾损伤（AKI） 12例（1.0％）,高血压型（HT）11例（0.9％）,急性肾炎综合征（ANS）8例（0.7％）,孤立性肉眼血尿型（iGH）3例（0.2％）.原发性肾小球疾病以系膜增生性肾炎（MsPGN）,继发性肾小球疾以狼疮性肾炎（LN）为主要病理类型.本组患者中各临床分型均以系膜增生性肾炎为主要病理类型,除系膜增生性肾炎外,肾病综合征以MN,Uab以IgAN为主要病理类型.结论 原发性肾小球疾病是徐州地区最常见的肾脏疾病,男性、青壮年是高发人群,系膜增生性肾炎是最常见病理类型,肾病综合征是最常见的临床表现.     

成人肾小球疾病145例临床病理分析

目的了解成人肾小球疾病的病理类型及其特点。方法分析我院145例成人肾小球疾病患者的年龄性别分布、肾病理类型及其与临床表现的关系。结果145例患者中，原发性肾小球疾病占72．4％，男／女为1／1．1；继发性肾小球疾病占22．8％，男／女为1／1．54；遗传性肾小球疾病占4．8％，男／女为2．5／1。原发性肾小球疾病病理类型以IgA肾病(IgAN)为主，占43．9％，膜性肾病(MN)占17．1％，微小病变(MCD)占17．1％，系膜增生性肾小球肾炎(MsPGN)占15．2％，毛细血管内增生性肾小球肾炎(EnPGN)占3．8％，膜增生性肾小球肾炎(MPGN)占1．0％，局灶节段性肾小球硬化(FSGS)占1．0％。继发性肾小球肾炎中以狼疮性肾炎(LN)为主，占27．3％，乙型肝炎相关性肾炎占21．2％，过敏性紫癜肾炎占18．2％，系统性血管炎占18．2％，肾淀粉样变占6．0％，流行性出血热占3．0％，多发性骨髓瘤占3．0％，糖尿病肾病(DN)占3．0％。临床表现蛋白尿和(或)血尿占34．0％，肾病综合征占47．3％，急性肾衰竭占10．7％，急性肾炎综合征占8．0％。结论原发性肾小球疾病是成人最常见的肾小球疾病，以IgAN为主。高发年龄虽在青壮年，但不应忽视老年发病。继发性肾小球疾病中以LN多见，女性多发，其它继发性肾小球疾病逐渐增多。     

老年肾脏病152例临床与病理特点分析

目的了解老年肾脏病患者的临床表现及病理特点。方法回顾性分析152例经皮肾活检确诊的老年（≥60岁）肾脏病患者的临床表现和病理类型。结果肾活检患者中原发性肾小球疾病最为常见（114例,占75％）,主要表现为。肾病综合征（53．51％）、慢性肾炎综合征（30．70％）、隐匿性肾炎（8．77％）、急进性肾炎综合征（4．39％）和急性肾炎综合征（2．63％）。病理改变主要为系膜增生（48．25％）、局灶性节段性肾小球硬化症（13．16％）、膜性肾病（11．40％）、新月体性肾炎（6．14％）,轻微病变（6．14％）为主。继发性肾脏疾病占全部病例的25％,原发病以糖尿病肾病（28．95％）、急性肾小管坏死（23．68％）居多。以急性。肾衰竭为临床表现的23例患者的病理类型主要为急性肾小管坏死（39．13％）和新月体性肾炎（30．43％）。将本组患者与同期行肾活检的非老年组比较,在原发性肾脏病中,膜性肾病和新月体性。肾炎的检出率明显高于非老年组,而IgA肾病的检出率明显低于非老年组;继发性肾脏病患者中,本组以糖尿病肾病及急性肾小管坏死居多,而非老年组则以狼疮性肾炎（65．84％）和乙型肝炎病毒相关性肾炎（14．01％）居多。结论老年肾活检患者以原发性肾小球疾病最常见,肾病综合征是最常见的临床表现,病理改变则以系膜增生最为多见。     

儿童原发性肾病综合征并发特发性急性肾衰竭12例临床分析

回顾性分析12例原发性肾病综合征（PNS）并发特发性急性肾衰竭（IARF）患者的临床资料。结果显示，12例均为突然发生少尿或无尿，血肌酐及尿素氮进行性升高，肾活检病理检查示微小病变7例（58．3％）、系膜增生性肾炎3例（25．0％）、局灶节段性肾小球硬化2例（16．7％），其共同病理变化是小管间质病变广泛；经泼尼松、血液透析等对症治疗肾功能均迅速改善。认为PNS并发IARF多见于肾小球微小病变者，早期行综合治疗者预后较好。     

Mycophenolate mofetil in lupus nephritis

The treatment of severe lupus nephritis is based on the combination of steroids and cytotoxic drugs. Intravenous cyclophosphamide administered in "pulses" is effective in the induction of remission but other therapeutic alternatives are sought in refractory cases or severely relapsing patients. Mycophenolate mofetil, used in renal transplantation, also can be useful in severe lupus nephritis. We describe the evolution of 6 patients (5 women and 1 man; age 17-45 years) with severe lupus nephropathy who after achieving remission with intravenous cyclophosphamide and steroids (5 cases) or cyclosporin A (1 case) showed relapse of proteinuria and were treated with mycophenolate mofetil (dose 1000-2000 mg/day). Two patients have completed 24 months, 1 patient two cycles of 12 months, 2 patients 18 months and 1 patient 6 months. After this treatment, all patients have achieved remission (3 partial and 3 complete). There was no treatment failure and no one patient discontinued medication; however 1 case relapsed. There were no changes in leucocytes, haemoglobin, serum creatinine and serum albumin. ANA and alpha DNA antibodies decreased. Proteinuria (measured as protein/creatinine urine ratio: initial 3 and final 0.3) and dose of steroids (initial: 17.5 mg/d and final 5 mg/d) decreased significantly (p < 0.05 Wilcoxon t-test). The most common side effects were nausea and abdominal discomfort that improved without discontinuation of treatment. We conclude that mycophenolate mofetil is effective and a safe drug in severe relapsing lupus nephritis.     

对拉米夫定治疗无应答或变异的乙型肝炎病毒相关性肾炎的治疗

目的探讨对拉米夫定治疗无应答或变异的乙型肝炎病毒相关性肾炎(HBV-GN)的治疗方法。方法 2005年1月至2009年1月南京军区福州总医院诊治的HBV-GN且对拉米夫定治疗无应答或变异的患者共9例。采用恩替卡韦(0.5 mg/d)为主方案治疗9例对拉米夫定治疗无应答或变异的HBV-GN且蛋白尿>1.5 g/d、血HBV-DNA≥1.0×108拷贝/L的患者,观察其疗效及血HBV-DNA的变化。结果 9例患者肾病综合征(NS)7例、蛋白尿伴血尿2例,其中系膜增生性肾炎3例,膜性肾病3例,膜增生性肾炎、IgA肾病及局灶节段系膜增生性肾炎各1例。拉米夫定治疗(14.1±10.3)个月(其中6例联合激素治疗),5例部分缓解(PR)、4例无效(均为NS),改用恩替卡韦治疗后,治疗12个月时7例完全缓解(CR)、1例PR、1例NR,7例检测血清HBV-DNA水平患者中5例降至正常,随访观察了19～27个月,平均(23.7±3.0)个月,至随访结束,完全缓解(CR)7例,NR 2例,均停用激素。未见副反应。结论恩替卡韦为主的治疗对对拉米夫定治疗无应答或变异的HBV-GN是安全有效的,疗程以1年半为宜。     

微小病变样IgA肾病的临床病理特征及激素疗效分析

目的:了解微小病变样IgA肾病(IgA nephropathy,IgAN)的临床病理特征及激素疗效。方法:回顾性分析61例微小病变样IgAN患者[男性46例,女性15例,平均年龄(23.92±8.65)岁]的临床和病理特征及激素诱导治疗疗效。结果:61例患者均以水肿为首发症状,其中15例(24.6%)有明确感染诱因。临床表现主要为肾病综合征,14例(23.0%)伴急性肾损伤,5例(8.2%)伴高血压,14例(23.0%)伴镜下血尿。尿N-乙酰-β-D-葡萄糖苷酶(NAG酶)(78.7%)、尿视黄醇结合蛋白(RBP)升高(75.4%)较多见。肾小球病理表现轻微,肾小管、间质急性损伤突出,比例分别为80.3%、65.6%,其中小管中～重度损伤占14.7%。足量激素诱导治疗有效率为91.8%,平均随访(46.00±13.93)月(24～70月),复发率为76.8%。与激素治疗有效患者相比,无效患者血尿发生率高且较严重(P<0.01),血浆白蛋白相对较高(P<0.05),肾小管萎缩、间质纤维化、小动脉透明变性多见(P<0.01)。随访结束时,59例(96.7%)肾功能正常。结论:微小病变样IgAN临床及病理特征均类似微小病变,激素治疗缓解率高、总体预后好。     

Idiopathic nephrotic syndrome in the elderly

The most frequent primary glomerular diseases (PGD) associated with nephrotic syndrome (NS) in the elderly are membranous nephropathy (MN), minimal change nephropathy (MCN), and focal and segmental glomerulosclerosis (FSGS). In older patients MN may be secondary to drugs or neoplasia in 20 to 25% of cases. The natural renal outcome of idiopathic MN is similar in elderly patients and in those of the second age. However, elderly patients are more exposed to the extra-renal complications of NS. Corticosteroids alone do not seem to modify the course of the disease. A 6-month regimen with corticosteroids alternated to chlorambucil, which has proven to improve the outcome of MN in adults, may increase the chances of remission and protect renal function also in the elderly patients but side effects increase with age. Elderly patients with MCN are more prone than younger adults to the complications of the NS and to the development of renal failure. Only 60% of older patients enter remission with an 8-week course of prednisone, but about 80% can achieve complete remission with corticosteroids if treatment is prolonged to 12–16 weeks. Relapses are more rare in the elderly. In patients with contraindications to prolonged corticosteroid therapy, a course of 12 weeks with a cytotoxic agent may obtain stable remission in most cases. Little information is available about the natural course and the management of idiopathic FSGS in the elderly. A recent report showed that more than 40% of older patients may obtain stable remission after an initial treatment with corticosteroids for 6 months. For those patients who do not respond or have contraindications to steroid therapy, a cautious trial with cyclophosphamide may be tried.     

Propylthiouracil-associated antineutrophil cytoplasmic autoantibody-positive vasculitis: retrospective study of 19 cases

OBJECTIVE: To investigate the features, followup data, and outcomes of patients with propylthiouracil (PTU)-associated antineutrophil cytoplasmic autoantibody (ANCA)-positive vasculitis. METHODS: Nineteen patients with PTU-associated ANCA-positive vasculitis diagnosed in our hospital from 2000 to 2006 were analyzed retrospectively. RESULTS: Our data showed a female predominance among the patients. Eleven patients had involvement of more than one organ. Renal involvement was the most common manifestation. Fourteen patients underwent renal biopsy. Four patients had focal proliferative glomerulonephritis with crescent formation. Two had necrotizing glomerulonephritis with crescent formation. Two patients had minor glomerular abnormalities, 2 had IgA nephropathy, one had membranous nephropathy, one had focal proliferative glomerulonephritis, one had granulomatous interstitial nephritis, and the remaining one had focal segmental glomerular sclerosis. Immune complex glomerulonephritis was found in 3 patients. On indirect immunofluorescence, 17 patients were perinuclear-pattern ANCA-positive, one was positive for atypical ANCA, and one was positive for cytoplasmic-pattern-ANCA. By ELISA, 4 patients were positive for both myeloperoxidase (MPO)-ANCA and proteinase-3 (PR3)-ANCA, one was positive for PR3-ANCA only, and the others were positive for MPO-ANCA only. For the treatment of vasculitis, 5 patients received prednisone alone, 10 received prednisone and cyclophosphamide, and the remaining 4 did not receive prednisone or cyclophosphamide. During followup, 15 patients achieved remission, 3 patients died, and one patient depended on dialysis. In general, MPO-ANCA concentration did not correlate with disease progression, and a delayed decrease of MPO-ANCA concentration was found in most patients who achieved remission. CONCLUSION: Most patients with PTU-associated ANCA-positive vasculitis had good outcomes; however, severe cases existed. We suggest early recognition and adequate treatment are necessary to improve outcome.     

慢性肾功能衰竭患者肾活检的临床意义

目的：对慢性肾功能衰竭（ＣＲＦ）患者肾活检的临床意义进行评价。方法：对２２２例临床诊断为ＣＲＦ,ＳＣｒ〉１７８μｍｏｌ／Ｌ（２ｍｇ／ｄｌ）的口才行肾活检术,分析其病理类型、标本合格率、穿刺成功率、并发症发生率及并发症的危险因素。根据肾穿后有无并发症,将患者分为并发症且和无并发症组两组,对并发症影响因素进行统计学分析。结果：肾活检后能明确诊断者占８９．１％,其中ＩｇＡ肾病、血管炎、慢性间质性肾炎、狼     

Drug-induced interstitial nephritis. Coexistence with glomerular disease

Drug-induced interstitial nephritis is being recognized with increasing frequency. Pharmacologic agents responsible for inducing this entity include antibiotics, diuretics, and nonsteroidal anti-inflammatory drugs. We recently examined five patients with glomerular disease and drug-induced interstitial nephritis. In three patients prior biopsy specimens documented their glomerular disease (membranous nephropathy, crescentic glomerulonephritis, and presumptive lipoid nephrosis). A second biopsy specimen showed acute interstitial nephritis and the glomerular lesion. Two additional patients had single biopsy specimens demonstrating acute interstitial nephritis and either membranous nephropathy or crescentic glomerulonephritis. Our cases emphasize the need for recognizing this complex pattern of renal disease and the difficulties encountered in rendering a proper diagnosis.     

Increased expression of selectins in kidneys of patients with diabetic nephropathy

Summary In diabetic nephropathy leukocytes, mainly composed of monocytes/macrophages, which accumulate in the glomeruli and the interstitium, play an important part in the progression of glomerulosclerosis. The infiltration of leukocytes into inflammatory tissues or atherosclerotic lesions is mediated by adhesion molecules, which are expressed on the vascular endothelial cells, although little is known about the mechanism of leukocyte infiltration into diabetic renal tissues. P- and E-selectin are leukocyte adhesion molecules, which are expressed on the vascular endothelial cells and promote the adhesion of leukocytes to the endothelium. We investigated the expression of P- and E-selectin in the kidney tissue of patients with diabetic nephropathy and compared it with that of patients with other glomerular diseases (minimal change nephrotic syndrome, membranous nephropathy, IgA nephropathy, mesangioproliferative glomerulonephritis, and lupus nephritis). Expression of P- and E-selectin were both significantly increased in the glomeruli and the interstitium of patients with diabetic nephropathy as compared with those with other glomerular diseases. P- and E-selectin were both expressed along the glomerular capillaries and the peritubular capillaries in the interstitium. Neither P- nor E-selectin were correlated with the number of infiltrated leukocytes in the glomeruli, however, interestingly the E-selectin expression on peritubular capillaries was correlated with the number of infiltrated CD14 positive cells in the interstitium. These results suggest that E-selectin may play a key role in leukocyte infiltration into the renal interstitium in patients with diabetic nephropathy. [Diabetologia (1998) 41: 185–192] Received: 22 April 1997 and in final revised form: 29 September 1997     

Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

Background and objectives

Lupus podocytopathy, which is characterized by diffuse foot process effacement without peripheral capillary wall immune deposits and glomerular proliferation, has been described in SLE patients with nephrotic syndrome in case reports and small series. This study aimed to better characterize the incidence, clinical–morphologic features, and outcomes of such patients from a large Chinese cohort.

Design, setting, participants, & measurements

Lupus podocytopathy was identified from 3750 biopsies of SLE patients obtained from 2000 to 2013 that showed mild glomerular histology in patients with a clinical sign of nephrotic syndrome. The biopsy results were divided into three groups: glomerular minimal change, mesangial proliferation, and FSGS.

Results

Fifty (1.33%) cases were identified as lupus podocytopathy and included minimal change in 13 cases, mesangial proliferation in 28 cases, and FSGS in nine cases. Extensive foot process effacement appeared in all the biopsies and mesangial electron-dense deposits were present in 47 biopsies. All patients demonstrated nephrotic syndrome, and the median proteinuria was 5.72 g/24 h (interquartile range [IQR], 3.82, 6.92). Seventeen (34%) cases presented with AKI. Forty-seven (94%) patients achieved remission after immunosuppressive therapy for a median time of 4 weeks (IQR, 2, 8). Compared with the patients with minimal change and mesangial proliferation, patients with FSGS showed significantly higher incidence of AKI and severe tubule–interstitial injury and a much lower complete remission rate. During follow-up of a median of 62 (IQR, 36, 84) months, renal relapses occurred in 28 (59.6%) patients. No patient died or developed ESRD.

Conclusions

The findings from this cohort study suggest that lupus podocytopathy may represent a special entity of lupus nephritis with distinct clinical–morphologic features. The differences in AKI incidence, tubular injury severity, and response to treatment between the patients with minimal change/mesangial proliferation and those with FSGS patterns indicate two different subtypes of lupus podocytopathy.