膜性肾病静脉血栓栓塞症的临床研究

目的:观察临床表现肾病综合征(NS)的膜性肾病患者静脉血栓栓塞症(VTE)的发生率,并探讨发生肾静脉血栓(RVT)的危险因素。方法:临床诊断NS且经肾活检确诊膜性肾病的患者100例,采用双源CT血管成像观察VTE的发生率。结果:36例(36%)患者共计发生血栓部位78处,平均每例2.2处。RVT33例(33%),下腔静脉19例,髂静脉2例,生殖静脉5例,腘静脉2例。17例(17%)患者发现肺动脉栓塞(PE),其中9例有胸闷、呼吸困难等症状。RVT中仅2例有肉眼血尿,5例有腰痛症状。与无血栓组相比,血栓组蛋白尿明显高于无血栓组(P<0.05)。两组患者其他指标(如血清白蛋白、血清肌酐、镜下血尿及肾小管功能)均无明显差异。凝血指标中,血栓组患者D二聚体阳性率显著高于无血栓组(P<0.01),血小板计数低于无血栓组(P<0.05)。两组患者血清抗凝血酶Ⅲ水平下降率无明显差异。结论:膜性肾病NS患者的血栓栓塞症发生率高达36%,其中RVT33%,PE17%。多数患者并无典型血栓栓塞的临床症状,D二聚体阳性率与血栓栓塞密切相关。因此,临床医师必须意识到RVT是膜性肾病的常见并发症,应常规行D二聚体监测,必要时行CT血管成像检查,以确诊有无RVT和PE。     

肾病综合征静脉血栓栓塞症的临床表现与诊断

静脉血栓栓塞症(venous thromboembolism,VTE)主要包括下肢深静脉血栓(deep venous thrombosis,DVT)、肾静脉血栓(renal vein thrombosis,RVT),以及由此引起的肺动脉栓塞(pulmonary embolism,PE),是肾病综合征(NS)常见的并发症之一.RVT的形成可进一步加重肾脏损害,使病情恶化.     

膜性肾病伴发静脉血栓栓塞症的超声诊断及相关因素分析

目的:回顾性分析膜性肾病(MN)患者伴发静脉血栓栓塞症(VTE)的超声特点及临床意义。方法:经肾活检确诊MN患者104例,应用彩色多普勒超声观察肾静脉、下腔静脉、髂静脉、下肢静脉及肺动脉、右心腔内部结构,了解是否存在异常回声及肺动脉高压。结果:104例患者中,发现肺栓塞14例,肾静脉血栓5例,下腔静脉血栓2例,髂静脉血栓4例,下肢静脉血栓4例。血栓组与无血栓组比较,血清白蛋白、肌酐、D-二聚体及纤维蛋白原,差异具有统计学意义(P0.05),24h尿蛋白定量及凝血酶原时间,两组差异无统计学意义(P0.05)。结论:彩色多普勒超声可作为MN患者筛查静脉血栓的首选影像学检查方法,为患者疗效评估提供了简便、无创、无辐射的检测方法。     

肾病综合征并发血栓的危险因素、病理机制及预防

<正>血栓形成是肾病综合征(NS)最常见的并发症之一,国外文献报道NS患者发生静脉血栓栓塞的发病率为3%~69%,其中肾静脉血栓(RVT)最为常见,其次为肺栓塞(PE)和深静脉血栓(DVT),而冠状血管、脑血管等重要脏器血管血栓也不少见,亦是NS最严重并发症之一,下肢静脉血栓未及时溶栓治疗,可造成终身残废,合并心、脑、肺、肾动脉血栓者可危及生命,合并PVT者可使病情持续发展和肾功能恶化。因此了解     

肾病综合征抗凝治疗的时机和药物选择

肾病综合征(NS)患者体内存在高凝状态,易导致静脉血栓栓塞症(VTE)发生,包括下肢深静脉血栓塞和肾静脉血栓,并可引起肺动脉栓塞(PE),严重威胁患者的生命安全.文献报道NS患者VTE的发生率在7.2％ ～62％.南京军区南京总医院全军肾脏病研究所一项前瞻性研究观察了膜性肾病患者VTE的发生率证实,VTE发生率高达36％,其中肾静脉血栓33％,PE17％,且多数患者并无典型血栓栓塞症状.因此,对于可能发生VTE的患者抗凝治疗显得尤为必要.但长期抗凝治疗可导致患者医疗费用的增加,出血并发症增多,严重者甚至威胁生命.因此,正确把握抗凝治疗的时机,选择合理的抗凝药物,对改善NS患者预后,减少并发症,提高生活质量有重要意义.     

肾病综合征患者血浆抗凝血酶Ⅲ检测及其临床意义

目的:比较分析不同病理类型肾病综合征患者血浆抗凝血酶III(antithrombin Ⅲ,AT-Ⅲ)浓度的差异及相关影响因素。方法:比较微小病变(minimal change disease,MCD,n=26)、局灶节段性肾小球硬化(focalsegmental glomerulosclerosis,FSGS,n=26)及膜性肾病(membranous nephropathy,MN,n=20)三组成人肾病综合征患者肾活检术前血尿AT-Ⅲ浓度及临床特点。选取20例健康成人作为正常对照。血尿AT-III浓度采用免疫速率比浊法检测。采用Person、Sperman方法及多元线性回归分析FSGS、MCD患者中影响血浆AT-III浓度的因素。结果:肾活检术时MN组尿蛋白、血清白蛋白、肌酐、总胆固醇、IgG与MCD和FSGS组差异显著(P0.05);后两组间以上指标的差异无统计学意义(P0.05)。FSGS组尿C3、α2巨球蛋白、N-乙酰-β-D-葡萄糖苷酶、视黄醇结合蛋白高于MCD和MN组(P0.05)。26例(100%)MCD、23例(88.46%)FSGS、3例(15%)MN患者血浆AT-Ⅲ降低(P0.05)。FSGS与MCD患者血浆AT-III浓度分别为(15.64±3.86)mg/dl、(18.10±4.8)mg/dl(P0.05),均低于MN患者(28.08±4.23)mg/dl(P0.05)。FSGS、MCD组尿AT-III5mg/dl者分别为8/20例(40%)、4/22例(18.18%)(P0.05),而MN组患者尿液AT-III含量均5mg/dl(P0.05)。一元回归发现FSGS患者中血浆AT-III浓度与尿蛋白(r=-0.505,P0.05)负相关、血清白蛋白(r=0.559,P0.01)正相关;MCD患者中血浆AT-III浓度与尿蛋白(r=-0.429,P0.05)负相关,与血清白蛋白(r=0.564,P0.01)、IgG(r=0.529,P0.01)正相关。多元回归分析提示FSGS、MCD患者中血浆AT-III与血清白蛋白相关(R2=0.312、0.320,P0.05)。随访中2例A-TⅢ降低的FSGS患者发生静脉血栓。结论:肾病综合征患者血浆AT-III浓度与病理类型有关。FSGS、MCD血浆AT-Ⅲ浓度较MN明显下降,可能源于尿液丢失的差异。     

肾病综合征血栓栓塞症的病理生理学基础

肾病综合征(nephrotic syndrome,NS)是一组表现为蛋白尿大于3.5 g/d,伴有水肿、低蛋白血症、高脂血症的临床综合征,可合并感染和血栓[1].血栓栓塞症是NS一个重要并发症,包括动脉和静脉血栓,其中最常见是肾静脉血栓(renal vein thrombosis,RVT),且多为无症状性[2],部分患者预后严重.关于NS和血栓之间存在很多尚未解决或有争议的问题,其中包括NS高凝状态的病理生理学特点[1]、血栓形成机制、最佳的诊断和抗凝治疗策略等.本文将重点讨论NS血栓并发症的病理生理学特点,阐明其高凝状态的机制.     

老年肺血栓栓塞症患者血流动力学的变化及影响因素

目的探讨老年肺血栓栓塞症患者血流动力学的变化及影响因素。方法选择70岁以上老年肺血栓栓塞症患者100例作为肺血栓栓塞症组,70岁以上老年健康体检者100例作为对照组。肺血栓栓塞症组患者根据是否合并肺动脉高压分为肺动脉高压组(59例)和非肺动脉高压组(41例)。收集患者的临床资料及平均动脉压(MAP)、心脏指数(CI)、每搏输出量(CO)、每搏指数、心率、胸腔静脉血压、体血管阻力、肺血管阻力等血流动力学指标。结果肺血栓栓塞症组MAP、CI、CO、每搏指数明显均明显低于对照组(P<0.05),肺血栓栓塞症组心率、胸腔静脉血压、体血管阻力、肺血管阻力均明显高于对照组(P<0.05)。肺动脉高压组年龄明显高于非肺动脉高压组,血小板计数低于非肺动脉高压组,女性、晕厥、V1~3 T波倒置比例明显高于非肺动脉高压组;肺动脉高压组吸烟、高血压、脑卒中、癌症、外伤或手术、呼吸困难、胸膜炎性胸痛、咯血、胸闷、心悸、咳嗽、D-二聚体、右束支传导阻滞、深静脉血栓形成的比例与非肺动脉高压组比较均无统计学差异(P>0.05)。Logistic多因素回归分析结果显示:性别和年龄是肺血栓栓塞症合并肺动脉高压的独立危险因素(P<0.05)。结论老年肺血栓栓塞症患者存在血流动力学改变,性别和年龄是老年肺血栓栓塞症合并肺动脉高压的独立危险因素。     

肾病综合征合并静脉血栓栓塞症的治疗及预防

静脉血栓栓塞症(VTE)包括深静脉血栓形成(DVT)及肺栓塞(PE),是同一疾病在不同部位、不同发病阶段的不同的临床表现,肾病综合征(NS)患者VTE发生率相当高[1,2],需要早期诊断和正确治疗.本文着重介绍NS患者合并VTE治疗及预防.     

肾病综合征相关静脉血栓栓塞症的发病机制及防治研究进展

静脉血栓栓塞症（VTE）是肾病综合征（NS）常见、多发的严重并发症,包括深静脉血栓形成（DVT）和肺血栓栓塞症（PTE）。NS患者长期处于“血栓形成倾向”状态,其VTE发病率逐年增高。对于疑诊PTE患者,可先应用临床可能性评分进行临床评估,并联合D-二聚体检测进一步筛查,PTE的确诊检查包括CT肺动脉造影、核素肺通气/灌注显像、磁共振肺动脉造影、肺动脉造影等;DVT确诊影像学检查包括加压静脉超声、CT静脉造影、核素静脉显像、静脉造影等。NS患者常存在肾功能不全,需临床医师谨慎选择适合的检查方式。NS相关VTE的治疗通常包括肝素、华法林,需根据不同患者制定个体化治疗方案,未确诊VTE的NS患者是否常规预防性抗凝存在争议。     

老年肾病综合征肾活检病理类型及血清抗M型磷脂酶A2受体IgG水平的研究

目的探讨老年肾病综合征肾活检病理类型及血清M型磷脂酶A2受体(PLA2R)IgG水平,为老年肾病综合征肾脏病理类型的非肾活检诊断提供依据。方法回顾分析2009年1月至2019年6月在中日友好医院肾内科诊断为肾病综合征,并住院期进行肾穿刺活检的老年患者临床数据和肾活检资料。结果共纳入研究对象250例,年龄平均(65.85±4.68)岁,其中男152例,女98例。(1)常见肾活检病理类型:膜性肾病154例(61.6%),微小病变性肾小球病31例(12.4%),局灶节段性肾小球硬化症20例(8.0%),IgA肾病11例(4.4%);继发性肾小球疾病中,糖尿病肾病11例(4.4%),乙型肝炎病毒相关性膜性肾病5例(2.0%),淀粉样变性肾损害5例(2.0%)。(2)对比前后5年间肾活检病理谱变化,膜性肾病(由61.11%增至62.36%)、微小病变性肾小球病(由9.72%增至13.48%)、糖尿病肾病(由4.17%增至4.49%)所占比例略有增加,IgA肾病(由11.11%降至1.69%),局灶节段性肾小球硬化症(由11.11%降至7.30%)所占比例显著下降。近5年来,膜性肾病和肾小球微小病变病两者所占比例达到了75.9%。(3)86例原发膜性肾病检测了血清PLA2R抗体水平,其中44例(51.2%)阳性,肾小球微小病变病和局灶节段性肾小球硬化症患者血清PLA2R抗体均阴性。结论老年肾病综合征中以膜性肾病最常见,其次是肾小球微小病变病,且所占比例有增多趋势。血清PLA2R抗体检测对老年肾病综合征中原发性膜性肾病诊断具有参考意义。     

老年肾病综合征的临床表现和病理类型分析

目的：进一步了解老年肾病综合征的临床表现和病理特点。方法：对北京协和医院1980－1999年底住院老年肾病综合征病人进行临床表现和病理类型分析。结果：住院成人肾病综合征病人共513例,其中老年肾病综合征61例,占11．9％,原发病为原发性肾小球肾炎者49例,占80．3％,糖尿病肾病者7例,占11．5％。临床表现以浮肿、血尿为主,贫血的发生率为54．1％,慢性肾功能不全（CRF）为32．8％。肾活检发现,在非糖尿病肾病综合征病人用激素或激素加免疫抑制剂治疗的缓解率为47．85,有效率为30．4％,未缓解率为21．7％,一般激素或激素加免疫抑制剂效果较好,其临床表现与年轻人的肾病综合征相似,但合并贫血、急性肾功能不全（ARF）、CRF和感染的比例较高,最常见的病因是慢性肾小球肾炎,其病理类型以系膜增殖型肾小球肾炎最常见,其次是局灶增殖型肾小球肾炎,老年肾病综合征患者对激素或激素加免疫抑制剂治疗的反应尚可。     

老年人原发性肾病综合征52例临床分析

目的 观察老年原发性肾病综合征患者临床特征及预后.方法 回顾性分析52例老年原发性肾病综合征患者的临床病理特点,并与64例非老年原发性肾病综合征患者进行比较.对老年原发性肾病综合征患者给予常规治疗,口服糖皮质激素泼尼松1 mg·kg-1·d-1,维持治疗8周后,若症状得到缓解,逐渐降低剂量至维持量后,再逐渐停药;若症状没有缓解,则加用环磷酰胺或环孢霉素、骁悉等治疗.结果 老年组并存高血压、感染、慢性肾功能不全和血尿者,与非老年组比较差异均有统计学意义(P＜0.05),两组血白蛋白、24 h尿蛋白定量和急性肾功能不全发生率,差异无统计学意义(P＞0.05).老年人肾病综合征病理类型以膜性肾病、IgA肾病和局灶节段性肾小球硬化为主,分别占46.2%、23.1%和11.5%,非老年组以系膜增生性肾小球肾炎IgA肾病和微小病变性肾病为主,分别占32.8%、25.0%和20.3%.治疗后完全缓解31例,部分缓解18例,无效3例,完全缓解率为59.6%.结论 老年人原发性肾病综合征临床病理以膜性肾病为主,采用糖皮质激素和免疫抑制剂治疗临床疗效显著,但在不同病理类型中具有差异.

Abstract：

Objective To observe the clinical characteristics and prognosis of primary nephrotic syndrome (PNS) in the elderly. Methods The clinical and pathological features of 52 elderly patients with PNS and 64 non-elderly patients with PNS were retrospectively analyzed and compared.The elderly patients with PNS received routine treatment:oral corticosteroids prednisone 1mg·kg-1 ·d-1.After 8 weeks maintenance treatment,if symptoms were alleviated,the prednisone dose was gradually reduced to a maintenance dose, then was stopped gradually. If symptoms were not alleviated, the cyclophosphamide or cyclosporine, mycophenolate mofetil was added. Results There were significant differences in the numbers of patients complicated with hypertension, infection,chronic renal insufficiency and hematuria between the elderly group and non-elderly group (P＜0. 05＝.But there were no significant differences in the level of blood albumin, quantitative measurement of 24 hours urinary protein and incidence rate of acute renal insufficiency between the two groups (P＞0.05). The major pathological types of PNS in the elderly were membranous nephropathy (46.2 %),IgA nephropathy (23. 1 %) and focal segmental glomerulosclerosis ( 11.5 %), respectively. And the major pathological types of PNS in non-elderly group were mesangial proliferative glomerulonephritis (32.8%), IgA nephropathy ( 25.0% ) and minimal change nephropathy ( 20. 3 %), respectively.Complete remission after treatment was found in 31 patients(59.6%), partial remission in 18 cases and inefficacy in 3 cases. Conclusions The major clinicopathological type of PNS in the elderly is membranous nephropathy and should be treated using corticosteroids and immunosuppressive agents,with different effects in different pathological types.     

Markers of endothelial cell injury and thrombin activatable fibrinolysis inhibitor in nephrotic syndrome.

Thromboembolic complications are often seen in patients with nephrotic syndrome. Markers of endothelial cell injury [thrombomodulin, intracellular adhesion molecule, vascular cell adhesion molecule, thrombin activatable fibrinolysis inhibitor (TAFI), protein Z, vascular endothelial growth factor, markers of thrombin and plasmin generation] were studied in 22 patients with nephrotic syndrome. All these parameters studied, except protein Z and D-dimers, were significantly higher in patients with nephrotic syndrome, whereas protein Z was significantly lower when compared with the healthy volunteers. None of the endothelial cell markers (thrombomodulin, P-selectin, E-selectin, intracellular adhesion molecule, vascular cell adhesion molecule), thrombin and plasmin generation markers (thrombin-antithrombin complexes, prothrombin fragments 1 + 2, plasmin-antiplasmin complexes, D-dimers), protein C, protein Z, vascular endothelial growth factor, and TAFI concentration and activity were directly correlated with the level of proteinuria, albumin, cholesterol, triglycerides or creatinine, except significant positive correlations between TAFI activity and serum creatinine, E-selectin and albumin as well as negative correlations between plasmin-antiplasmin complexes and proteinuria. In these patients, there is evidence of endothelial cell injury and probably secondary activation of the coagulation cascade. Elevated circulating TAFI antigen and activity might be a new link in the pathogenesis of impaired fibrinolysis and the progression of atherosclerosis in nephrotic syndrome. Protein Z deficiency might also contribute to the enhanced risk of thromboembolic complications in nephrotic syndrome.     

Renal biopsy in elderly adults over 75 years of age

The results of 177 renal biopsies (RB) in patients over 75 years of age were analysed. The three most frequent histological types were: Overall: membranous nephritis (MN), minimal change disease (MCD) and IgA Nephropathy (IgAN); In nephrotic syndrome (51% of RB): MN (36%), MCD (33%) and amyloidosis (12%); In chronic renal failure without nephrotic syndrome (25% of RB): chronic interstitial nephritis (17%), benign nephrosclerosis (12%) and IgAN (12%); In acute or progressive renal failure (18% of RB): acute tubular necrosis (36%), crescentic GN (16%) and IgAN (12%). Isolated proteinuria was most frequently associated with IgAN. In only 40% of patients was the medical history relevant, and only in selected cases it allowed for accurate prediction of the histological findings. Our data favor a more liberal use of biopsy in the elderly patients.     

Renal complications in chronic lymphocytic leukemia and monoclonal B-cell lymphocytosis: the Mayo Clinic experience

While the renal complications of plasma cell dyscrasia have been well-described, most information in patients with chronic lymphocytic leukemia and monoclonal B-cell lymphocytosis is derived from case reports. This is a retrospective analysis of patients with chronic lymphocytic leukemia or monoclonal B-cell lymphocytosis who underwent kidney biopsy for renal insufficiency and/or nephrotic syndrome. Between January 1995 and June 2014, 49 of 4,024 (1.2%) patients with chronic lymphocytic leukemia (n=44) or monoclonal B-cell lymphocytosis (n=5) had a renal biopsy: 34 (69%) for renal insufficiency and 15 (31%) for nephrotic syndrome. The most common findings on biopsy were: membranoproliferative glomerulonephritis (n=10, 20%), chronic lymphocytic leukemia interstitial infiltration as primary etiology (n=6, 12%), thrombotic microangiopathy (n=6, 12%), and minimal change disease (n=5, 10%). All five membranoproliferative glomerulonephritis patients treated with rituximab, cyclophosphamide and prednisone-based regimens had recovery of renal function compared to 0/3 patients treated with rituximab with or without steroids. Chronic lymphocytic leukemia infiltration as the primary cause of renal abnormalities was typically observed in relapsed/refractory patients (4/6). Thrombotic microangiopathy primarily occurred as a treatment-related toxicity of pentostatin (4/6 cases), and resolved with drug discontinuation. All cases of minimal change disease resolved with immunosuppressive agents only. Renal biopsy plays an important role in the management of patients with chronic lymphocytic leukemia or monoclonal B-cell lymphocytosis who develop renal failure and/or nephrotic syndrome.     

Incidence of thromboembolic complications in hospitalized COVID-19 patients in a medical ward in Japan: A single-center retrospective and prospective observational study

Background:A high incidence of thromboembolic complications is one of the hallmarks of COVID-19. However, there may be a difference in the incidence of thromboembolic complications between Asian and Western people. In addition, few prospective studies have been conducted to determine the incidence of thromboembolic complications in hospitalized COVID-19 patients in medical wards in Japan.Methods:A single-center retrospective and prospective cohort study was conducted to determine the incidence of thromboembolic complications in symptomatic COVID-19 patients in a medical ward in a Japanese hospital. All 1116 consecutive COVID-19 patients who were admitted to our hospital from November 1, 2020, to October 26, 2021, were included. The primary outcome was any thromboembolic complications, which included venous thromboembolism, myocardial infarction, ischemic stroke, and other arterial embolisms.Results:The median patient age was 50 (IQR, 37–61), 402 (36.0%) were women, 1005 (90.1%) were Japanese, the median body mass index was 24.1 (IQR, 21.6–27.2), and 43 (3.9%) had Padua scores of at least 4 points at admission. Regarding the severity of COVID-19, 543 (48.7%), 315 (28.2%), 204 (18.3%), and 54 (4.8%) patients had mild, moderate, severe, and critical COVID-19, respectively. Nine patients (0.8%) died, and 47 patients (4.2%) were transferred to other hospitals for intensive care. The primary outcome occurred in only 5 patients (0.5%; 95% CI, 0.1–0.8) and consisted of 3 ischemic strokes, 2 limb ischemia events, and one asymptomatic pulmonary embolism. Even in the 204 patients with severe COVID-19, the prevalence of thromboembolic complications was only 2.5% (95% CI, 0.3–4.6).Conclusion:Thromboembolic complications of COVID-19 are rare even in severe cases in a medical ward in a Japanese hospital. Further studies are needed to identify severe COVID-19 patients with a higher risk for thromboembolic complications in Japan.     

Incidence and etiology of thrombocytosis in an adult Turkish population

The aim of the study was to determine the incidence and etiology of thrombocytosis in an adult Turkish population. The medical records of 2000 patients were reviewed retrospectively to determine (i) the etiology of elevated platelet counts, (ii) additional laboratory parameters (i.e., complete blood counts), and (iii) the presence of thromboembolic complications. Within 5 years, 174 678 platelet counts had been performed for 124,340 patients. Of the 124,340 patients, 2000 (1.6%; mean age, 55.2 +/- 17.4 years; range 16-94 years; male-to-female ratio, 3.6:1 [1570:430]) had one or more platelet counts of 500 x 10(3)/mm3 or higher during the study period. Secondary thrombocytosis was the most frequent cause of an elevated platelet count and occurred in 1934 cases (96.7%). Infection was the most common cause of secondary thrombocytosis, occurring in 1001 patients (50.1%). Compared with secondary thrombocytosis, primary thrombocytosis was significantly associated with a higher rate of thromboembolic complications. It is concluded that a high platelet count may have both diagnostic and therapeutic implications. Secondary thrombocytosis is a common finding and mostly secondary to infection. Primary thrombocytosis has a higher risk for thromboembolic events than does secondary thrombocytosis.     

Transient ischemic attack and nephrotic syndrome: Case report and review of literature

Thrombotic complications in patients with nephrotic syndrome are attributed to a hypercoagulable state. Venous thrombosis is common, but arterial thrombosis occurs less frequently in adult nephrotic patients. We report a case of recurrent transient ischemic attacks as an initial manifestation of nephrotic syndrome due to early-stage membranous glomerulonephritis, review the literature for similar cases, and briefly discuss this potentially life-threatening condition. We observed that transient ischemic attack or ischemic stroke could be the initial manifestation of nephrotic syndrome. Our observation may serve as reminder to consider nephrotic syndrome as a possible contributor when evaluating patients with transient ischemic attacks with no other discernable clues. A high index of suspicion alone avoids the unnecessary withholding of prophylaxis or treatment that can be life saving.     

轻链沉积病肾损害的临床和病理分析

目的：了解轻链沉积病(LCDD)肾损害的临床和病理特征。方法：回顾性分析26例经临床和肾活检确诊的LCDD患者的临床和病理改变。结果：26例LCDD患者起病时的平均年龄为49.4岁(27—72岁)，其中男性21例，女性5例。8例确诊为多发性骨髓瘤(MM)，2例伴有浆细胞异常增生，另有16例病因不明。临床表现为急性肾衰3例(11．5％)，慢性肾衰17例(65．4％)，肾病综合征17例(65．4％)，15.4％的患者起病时即需行肾脏替代治疗。实验室检查发现血清和尿液游离κ、λ轻链的阳性率分别为56．5％和91．7％。肾脏病理改变以系膜结节样病变多见，占53．8％，2例表现为膜增生样病变、1例为膜性病变，中—重度小管间质慢性化病变为本组患者较特征性的病变(92．3％)。肾组织中以λ轻链沉积为主者占65．4％(17例)，κ轻链沉积占34．6％(9例)。18例患者行肾组织电子显微镜检查，均显示肾小球和(或)肾小管基膜内(外)侧不规则的纤细颗粒样电子致密物。结论：LCDD患者临床以肾功能不全伴肾病综合征多见，部分患者合并浆细胞增生性疾病。病理以系膜结节样伴严重的小管间质病变为特征。血清、尿液以游离λ轻链增高多见，肾组织中也以λ轻链沉积居多。