儿童肾活检977例病理及临床分析

目的回顾性分析小儿肾脏疾病的病理类型及临床特点。方法收集977例接受经皮肾活检患儿的病理诊断和临床资料,分析比较原发性肾小球疾病与继发性肾小球疾病的病理类型和临床特征。培果成功肾活检971例,其中原发性肾小球疾病755例（77．8％）,继发性肾小球疾病183例（18．8％）,先天性遗传性代谢性肾小球疾病18例（1．9％）。原发性肾小球疾病中,临床诊断前3位依次为孤立性血尿、肾病综合征和迁延性肾小球肾炎;病理类型前3位依次为肾小球轻微病变、局灶节段性肾小球硬化和局灶性肾小球肾炎。继发性肾小球疾病中,临床诊断前3位依次为紫癜性肾炎、乙型肝炎相关性肾小球肾炎和狼疮性肾炎;病理类型前3位依次为系膜增生性肾小球肾炎、肾小球轻微病变和膜性肾病。在18例先天性遗传性代谢性肾脏疾病中,有7例为薄基膜肾病。在临床诊断为孤立性血尿的患儿,肾活检病理类型中肾小球轻微病变所占百分比随患儿年龄的增长逐渐降低（P〈0．05）,而IgA肾病和局灶性肾小球肾炎所占百分比随患儿年龄的增长逐渐升高（P〈0．05）。结论本组资料中,儿童肾小球疾病以原发性肾小球疾病为主,临床表现和病理类型以血尿和肾小球轻微病变最为常见;小儿继发性肾小球疾病以紫癜性肾炎最为常见,病理类型以系膜增生性肾小球肾炎、肾小球轻微病变和膜性肾病为主;先天性遗传性肾脏疾病中薄基膜肾病最为常见。建议对6岁以上的孤立性血尿患儿行肾穿刺活检术。     

Pattern of renal pathology among renal biopsy specimens in Eastern Saudi Arabia

OBJECTIVE: To identify the pattern of renal pathology among renal biopsy specimens, and to study the clinical correlation in a general hospital in the eastern region of the Kingdom of Saudi Arabia. METHODS: All patients who underwent native kidney biopsy by the author at Dhahran Health Center (DHC) between June 1998 and April 2005 were included and prospectively followed-up. RESULTS: One hundred native kidney biopsies were performed on 95 patients with a mean age of 40.8+/-18 years, and a glomerular filtration rate of 57+/-42 ml/min/1.73 m2. Patients were followed up for a mean of 28+/-22.5 months. Primary renal pathology was identified in 72 specimens and secondary in 28. Primary renal pathologies included focal and segmental glomerulosclerosis (FSGS) (35%), immunoglobulin A nephropathy (IgAN) (14%), tubulo-interstitial nephritis (12%), minimal change disease (10%), membranous nephropathy (4%), mesangioproliferative glomerulonephritis (6%), mesangiocapillary glomerulonephritis (4%), thin glomerular basement membrane disease (8%), and miscellaneous (7%). Secondary lesions included lupus nephritis (LN) (36%), sickle cell nephropathy (SCN) (18%), diabetic nephropathy (14%), hypertensive nephrosclerosis (11%), Henoch Schonlein purpura (7%), and miscellaneous (14%). Obesity was particularly prevalent among patients with FSGS. Among the entire group, 12 patients (13%) progressed to end stage renal disease (ESRD) at a mean of 17.6+/-17 months (range, 1-45 months), and the overall mortality rate was 5.3%. CONCLUSION: At DHC, FSGS was the most common primary renal pathology, followed by IgAN. There was an association between FSGS and obesity. Lupus nephritis was the predominant secondary renal pathology followed by SCN. Within the time of follow up, primary renal lesions were associated with a low rate of progression to ESRD.     

儿童肾活检病理分析与临床意义探讨(附1 100例肾活检资料)

目的：了解1100例小儿肾小球疾病的病理类型分布及其与临床表现之间的关系。方法：总结我院1100例住院患儿的肾活检资料，按WHO1982例制定的肾小球疾病病理分类及1995年肾小球疾病组织学分型修订方案，结合临床资料、实验室检查结果，确定诊断。结果：原发性肾小球疾病919例（占83.5％），继发性肾小球疾病154例（14.0％），遗传性/先天性肾小球疾病19例（1.7％），其他无法归类的8例（0.7％）；其中微小病变102例（9.3％），轻微病变54例（4.9％），系膜增生583例（53.0％）膜性肾病62例（5.6％），膜增殖112例（10.2％），毛细血管内增生76例（6.9％），局灶节段性硬化71例（6.5％），新月体性肾炎6例（0.5％），急性肾小管坏死3例（0.3％），间质性肾炎5例（0.5％），溶血性尿毒综合征6例（0.5％），Alport性病1例（0.1％），肝豆状核变性1例（0.1％），脂蛋白肾小球病1例（0.1％）。结论：本组资料表明国内儿童阶段的肾小球疾病仍以原发性多见，病理类型以系膜增生性肾炎、轻微/微小病变性肾炎、膜增殖性肾炎等为主；在继发性肾小球疾病中以紫癜性肾炎占绝大多数，狼疮性肾炎次之。另外在儿童阶段亦不应忽视遗传性/先天性肾小球疾病的诊断。     

足细胞病的发病机制研究进展

足细胞是位于肾小球基底膜外部（GBM）的终末分化细胞,其形成的裂孔膜是肾小球滤过的最后屏障。足细胞损伤后,其结构完整性受损,足突消失,滤过膜孔径增大或断裂,大分子蛋白质滤出,超出近端肾小管重吸收能力而形成蛋白尿。研究表明,原发性足细胞病是以足细胞结构和功能异常为主要特点的肾小球疾病。本文主要对原发性足细胞病的几种类型,包括膜性肾病（MN）、微小病变型肾病（MCN）和局灶节段性肾小球硬化（FSGS）与足细胞的关系及其发病机制作一概述。     

Remission of idiopathic nephrotic syndrome after treatment with cyclosporin A

Nephrotic syndrome in minimal change lipoid nephrosis and focal segmental glomerulosclerosis may be due to alteration of glomerular anionic sites by a lymphokine. Six adults with nephrotic syndrome who were resistant to treatment with corticosteroids and immunosuppressants were treated with cyclosporin A. In three patients with minimal change lipoid nephrosis who had been nephrotic for 3.5 to 23 years proteinuria resolved within 12 to 42 days. Subsequently, these patients became dependent on cyclosporin A. In three patients with focal segmental glomerulosclerosis who had been nephrotic for four to six years mean (SD) 24 hour urinary protein decreased from 14.7 (8.4) g to 3.6 (0.6) g within 20 to 25 days, serum albumin concentration rose, and oedema subsided. One patient died of myocardial infarction when still in partial remission after 11 weeks' treatment. Two patients remained proteinuric despite continuing treatment with cyclosporin A, but control of sodium balance was easy and serum albumin concentrations remained higher than without cyclosporin A. In all patients renal function improved during treatment. These preliminary results show that cyclosporin A may be effective in the treatment of patients with nephrotic syndrome that resists every other form of treatment and especially in the treatment of those with minimal change lipoid nephrosis. The results are in keeping with a T lymphocyte mediated mechanism of minimal change lipoid nephrosis and focal segmental glomerulosclerosis, but they also suggest that minimal change lipoid nephrosis and focal segmental glomerulosclerosis are separate entities.     

慢性肾功能不全患者的病理类型分析

目的分析肾活检时表现为慢性肾功能不全患者（Scr≥130μmol/L）的患者病理类型及特点。方法选择我院2006年1月～2008年12月行肾脏活检时表现为血清肌酐升高（Scr≥130μmol/L）的所有慢性肾脏病患者共104例作为研究对象,分析其临床和病理特点。同时重点分析了增生硬化型IgA肾病的特点。结果 104例慢性肾脏病患者在肾活检时表现为肾功能异常,占同期肾活检患者的11.9%。这些患者的平均血清肌酐（235.11±114.4）μmol/L。临床诊断主要为：慢性肾小球肾炎63例,肾病综合征27例,系统性血管炎4例,系统性红斑狼疮3例。其中78例（75.0%）患者有高血压。病理类型主要表现为IgA肾病（46.2%,其中有70.8%的患者主要表现为增生硬化性肾炎）,其次是间质性肾炎,系膜增生性肾小球肾炎,新月体肾炎,局灶节段肾小球硬化,IV型狼疮性肾炎。其中,增生硬化型的IgA肾病患者血压升高更为突出,但是尿蛋白量更少。结论表现为慢性肾功能不全的患者病理上主要为IgA肾病,其中增生硬化性肾炎是主要病理表现,其次是肾小管间质病变。慢性病变是主要的病理特点。     

313例6岁以下小儿肾脏疾病病理特点及其与临床表现的关系

目的:了解6岁以下小儿肾脏疾病病理特点及其与临床表现的关系.方法:对313例临床诊断为14种肾脏疾病的6岁以下小儿进行肾组织病理检查.采用快速经皮肾活检术,将穿刺取得的组织分成3部分,按常规方法所有病例分别进行光镜、电镜及免疫荧光检查,标本均作HE,PAS,PASM及Masson染色,均应用免疫荧光检测肾组织中IgG,IgM,IgA,C3,C4,C1q及Firibn,部分病例根据血化验乙肝抗原阳性者加做免疫荧光检测肾组织中的HBsAg,HBeAg和HBcAg.本组290例(92.65%)进行了电镜检查.结果:313例肾活检成功率为100%;临床主要表现为持续性血尿103例(32.92%),单纯性肾病82例(26.21%),急性肾炎综合征63例(20.14%),紫癜性肾炎26例(8.32%),乙肝相关性肾炎15例(4.79%),孤立性蛋白尿8例(2.56%)等;病理改变主要为系膜增生性肾A炎162例(51.75%),IgM肾病26例(8.31%),微小病变扣轻微病变25例(7.99%),IgA肾病23例(7.35%),毛细血管内增生性肾小球肾炎16(5.11%),局灶节段性肾小球硬化14例(4.47%),薄基底膜病14例(4.47%),膜性肾病14例(4.47%)等.通过电镜检查,使Alport综合征、先天性肾病、薄基底膜病得以明确诊断.通过肾活检组织免疫病理学检查,使IgA肾病、IgM肾病及C1q肾病得以确诊.结论:临床表现类似的疾病病理类型不同,同一病理类型的疾病,临床表现可以多样.肾活检病理诊断对6岁以下小儿肾脏疾病的诊断、治疗、估计预后均有重要价值.电镜在肾病理检查中起到不可忽视的作用.     

尿视黄醇结合蛋白测定在小儿肾脏疾病中的意义

采用ELISA方法对255例不同肾脏疾病患儿的尿视黄醇结合蛋白（RBP）进行了检测，结果表明：各种肾脏疾病尿RBP水平高低不同，狼疮性肾炎和急性肾功能衰竭最高，其次为肾病综合征、乙肝肾及急性肾小球肾炎，对17例肾病综合征患儿进行尿RBP动态观察，结果发现尿RBP在活动期最高，随着尿蛋白下降和阴转而逐渐减低至正常。对55例不同病理类型肾脏疾病患儿进行尿RBP检测，结果发现局灶节段性肾小球硬化最高，增殖性肾炎其次，而膜性肾病和微小病变升幅最低。结果提示尿RBP能准确敏感地反映肾脏病变，适于临床推广应用。     

原发性肾小球疾病97例临床病理分析

目的: 探讨原发性肾小球疾病患者临床表现与病理之间的关系、肾活检的并发症及其临床意义。方法: 分析97例原发性肾小球疾病患者的临床表现、病理类型及肾活检资料。结果: 97例原发性肾小球疾病中,IgA肾病30例,系膜增生性肾小球肾炎26例,膜性肾病10例,局灶性节段性硬化性肾小球肾炎14例,IgM肾病2例,毛细血管内增生性肾小球肾炎1例,肾小球轻微病变8例,增生硬化性肾小球肾炎4例,新月体性肾小球肾炎2例。肾活检术后并发症:肉眼血尿3例,肾包膜下小血肿2例,无其它严重并发症。结论: 肾活检病理检查与临床密切结合,有助于原发性肾脏疾病正确诊断和治疗。肾穿刺术虽有一定并发症,但多不严重,是一项安全、简便的诊断操作。     

Max—Core^TM自动活检枪在小儿肾穿刺中的应用

目的 探讨肾活检在小儿肾脏病诊断中的意义及Max—Core^TM活检枪在小儿肾穿刺中的应用。方法 在B超引导下用Max—Core^TM一次性活检枪行经皮肾活检术，标本分别做光镜、免疫荧光和电镜检查。结果 6例肾小球疾病患儿均一次成功，其中轻微病变1例，系膜增生2例，膜性肾病1例，局灶节段性硬化1例，弥漫性基底膜变薄1例。经肾活检纠正诊断2例。3例患儿术后出现了轻微并发症。结论 Max-Core^TM自动活检枪操作简单、安全、成功率高，适用于儿科临床应用。     

儿童单纯性血尿105例病理分析

目的 探讨小儿单纯性血尿的病理类型.方法 对105例符合单纯性血尿诊断标准的患儿行肾活检术,肾组织进行光镜、电镜及免疫荧光检查.结果 薄基底膜肾病36例(34.3%),IgA肾病35例(33.3%),系膜增生性肾小球肾炎17例(16.2%),轻微病变7例(6.7%),Alport综合征6例(5.7%),局灶节段性硬化2例(1.9%),IgM肾病2例(1.9%).结论 遗传性肾小球基底膜病占小儿单纯性血尿的第一位,但有明确家族史者不足50%.IgA肾病也是小儿单纯性血尿的主要原因之一,表现为单纯性血尿的IgA肾病病理变化相对较轻.部分单纯性血尿患儿肾小球未见明显病变.少数严重肾小球病变临床也可表现为单纯性血尿.     

成人特发性局灶节段性肾小球硬化病248例临床病理分析

目的 重新评价肾活检标本中特发性局灶节段性肾小球硬化病（FSGS）的发生率、主要临床及病理特点。方法 收集1994年7月～2000年7月间248例特发性FSGS的患者,观察肾组织的光镜、 荧光及电镜的特点,根据肾小球病变将特发性FSGS分为3种亚型。结果 248例成人特发性局灶节段肾小球硬化占同期肾活检的12.4%,占成年人原发性肾小球疾病的19.0%,以18～30岁为发病高峰年龄（约占44.4%）。临床表现为不同程度的蛋白尿,以肾病综合征为表现者143例（占57.7%)。FSGS的3种病理亚型中,经典型FSGS仍占绝大多数（91.5%),尖端型为4.4%,塌陷型为4.1%,肾活检时,约1/3以上为肾功能不全（约占35.1%）。13例尿毒症中76.9%病理亚型为塌陷型。结论 局灶节段性肾小球硬化病约占原发性肾小球病1/5,FSGS患者肾活检时,除表现为蛋白尿、肾病综合征外,肾功能不全也较常见。塌陷型FSGS常表现为进行性的不可逆的肾功能衰竭,其预后差。     

成人特发性局灶节段性肾小球硬化症的临床及病理分析

目的:了解特发性局灶节段性肾小球硬化症(focal segmental glomerulosclerosis,FSGS)在肾脏疾病中的构成变化及其临床病理诊断要点和意义.方法:对1990年1月至2001年12月在我院肾科住院行肾活检确诊为特发性FSGS的65例患者的临床资料及病理改变进行总结分析.结果:(1)特发性FSGS占肾活检患者的2.2%,占原发性肾小球疾病的3.2%,占肾病水平蛋白尿患者的5.8%,12年中发病无上升趋势.(2)其病变类型为单纯门部病变占12.3%,单纯周缘部病变占23.1%,两者的混合型占60.0%,尖端型占1.5%,塌陷性占3.1%.同时伴随的病变有:足细胞增生、肥大和空泡变性(58.5%),球囊粘连(90.8%),肾小管间质病变(92.3%)以及节段性内皮和系膜细胞增生(20.0%).由于病变分布呈局灶节段的特点,且病变多样、重叠出现,在病理诊断中应警惕误、漏诊的可能.(3)伴节段性内皮和系膜细胞增生的患者常表现为肾病水平蛋白尿(92.3%).结论:在肾脏病理工作中,提高警惕性和检查方法对于正确诊断特发性FSGS具有重要意义.特发性FSGS不是构成本组肾活检患者的常见病理类型.足细胞病变以及节段性内皮和系膜细胞增生可能与节段性硬化形成有关.     

成年人原发性肾病综合征的不同病理类型对泼尼松的治疗反应

目的 明确成年人原发性肾病综合征的常见病理类型及对泼尼松的治疗反应。方法 对220例原发性肾病综合征患者进行了经皮肾穿刺病理检查并给予泼尼松口服治疗10周，通过测定治疗前后24h尿蛋白定量及血浆白蛋白的变化评价治疗效果。结果 成年人原发性肾病综合征的常见病理类型为：系膜增生性肾炎、膜增生性肾炎、轻微肾小球病变、膜性肾病、局灶性节段性硬化。泼尼松治疗10周后总有效率为63％。结论 原发性肾病综合征对泼尼松的治疗反应与病理类型及是否合并肾小管损伤有关。     

158例肾活检病理及临床分析

目的回顾性总结158例肾活检的病理结果,分析其病理类型及临床分类特点。方法总结我院158例肾活检病理资料,分析其病理类型与临床分类特点。结果158例肾活检中原发性肾小球疾病125例(79.11%),继发性肾小球疾病33例(20.89%)。原发性肾小球疾病病理类型最多的是系膜增生型肾炎,第二位是IgA肾病,继发性肾小球疾病以紫癜性肾炎病理类型最多。原发性肾小球疾病临床分布最多的前三位依次是肾病综合征、慢性肾炎、IgA肾病。其中肾病综合征的病理类型主要是系膜增生型和膜性肾病,慢性肾炎的主要病理类型是系膜增生型和肾小球硬化。结论原发性肾小球疾病是最常见的肾小球疾病,肾活检病理类型以系膜增生型肾炎、IgA肾病最多,临床类型则以肾病综合征、慢性肾炎、IgA肾病最为多见。     

Glomerulonephropathy of Laurence-Moon-Biedl syndrome

A patient with Laurence-Moon-Biedl syndrome and nephrotic range proteinuria is presented. Radiological investigation of the urinary tract revealed clubbed calyces but no evidence of obstruction or vesicoureteric reflux. Renal biopsy revealed occasional sclerotic glomeruli, extensive foot-process fusion and segmental glomerular basement membrane abnormalities with negative immunofluorescence for immunoglobulins and complement. Nephrotic proteinuria responded to steroid therapy but mild proteinuria persisted. The findings were consistent with minimal change nephropathy superimposed on the glomerular lesions of Lawrence-Moon-Biedl syndrome.     

2型糖尿病合并非糖尿病性肾损害的临床病理分析

目的　分析 2型糖尿病 (2 DM )患者合并非糖尿病性肾损害 (NDRD)的临床病理特点 ,提出对 2 DM合并肾损害患者进行肾活检的推荐指征。方法　 2 3例 2 DM接受肾穿刺患者 ,回顾性分析其年龄、性别、糖尿病史、肾脏病史、糖尿病并发症情况、尿检、尿蛋白、血糖、血肌酐等临床资料和病理活检的光镜、免疫光镜、电镜等病理资料。结果　 2 DM可合并多种原发性或继发性NDRD ,包括IgA肾病、非IgA系膜增生性肾小球肾炎、膜性肾病、微小病变、局灶节段性硬化、高血压肾小动脉硬化、淀粉样变、系统性血管炎等。临床可表现为肾功能不全、肾病综合征、血尿、蛋白尿等。血尿、无肾功能不全的肾病综合征、糖尿病史短高度提示NDRD的存在。糖皮质激素治疗总体上较安全。结论　 (1) 2 DM合并肾脏病变可以是NDRD ;(2 )NDRD的疾病谱多种多样 ,以IgA肾病最多见 ;(3)当出现血尿、无肾功能不全的肾病综合征、糖尿病史短等可疑因素时 ,应积极进行肾穿刺检查 ;(4 )对于NDRD ,糖尿病并非糖皮质激素的绝对禁忌证     

Thin-membrane nephropathy--a common cause of glomerular haematuria

Thin-membrane nephropathy recently has been described as a cause of glomerular haematuria. The prognosis of the condition is unclear but it generally is considered to be benign. In a series of 92 patients with glomerular haematuria, thin-membrane nephropathy was found to be a common cause, occurring in 26 (28%) patients. Sixteen patients were women. The mean age was 42 years. Four patients had a family history of renal disease or haematuria and no patient was deaf. Haematuria had been present from six days to 30 years. Loin pain occurred in 31% of patients. Hypertension was not a feature and mild renal impairment was present in one case only, while a further three cases showed proteinuria at a level of greater than 500 mg of protein per day. Glomerular basement membranes in patients with thin-membrane nephropathy gave a mean (+/- standard deviation) width of 319 + 37 nm which was significantly (P less than 0.002) less than the control value of 394 +/- 61 nm. On the basis of clinical features and serological parameters, thin-membrane nephropathy could not be separated from other renal causes of haematuria but required careful electronmicroscopic examination of renal biopsy material to establish the diagnosis. Limited follow-up has confirmed the good prognosis of the condition.     

Glomerular Disease Associated with Takayasu Arteritis： 6 Cases Analysis and Review of the Literature

Objective To evaluate the clinical features, renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis （TA）. Methods Patients with TA and renal biopsy-confirmed glomerular disease were investigated retrospectively. None of them had renal artery stenosis or occlusive changes.Results Six patients with glomerulopathy, accounting for 3.75% of the 160 TA patients admitted to our hospital at the same period, were analyzed. All of them were females with a mean age of 35.5 ± 10.0 years. Four cases presented with lower extremity edema. Laboratory tests showed that one was nephrotic syndrome, three were nephrotic range proteinuria, and two of them had mild renal dysfunction. The other two patients were asymptomatic microscopic hematuria and proteinuria. Renal pathology revealed mild immunoglobulin A nephropathy in two cases, mild mesangial proliferative glomerulonephritis （GN）, membranoproliferative GN, minimal change disease, and fibrillary GN in one case respectively. Five cases received glucocorticoids and cyclophosphamide therapy. Proteinuria and microscopic hematuria disappeared in 2 to 4 weeks after the initiation of therapy in three cases. The patient with membranoproliferative GN also reached complete remission of proteinuria and recovered renal function 6 months after the treatment. Conclusions TA may induce glomerular disease as a part of its histological spectrum. Apart from ischemic glomerular disease, glomerular disease should be suspected when TA patients have microscopic hematuria or proteinuria, that may be therapeutically responsive to glucocorticoids and immunosuppressive agent in relative early phase.     

Clinicopathological spectrum and treatment outcome of idiopathic steroid-resistant nephrotic syndrome in children at a tertiary care center

BackgroundIdiopathic steroid-resistant nephrotic syndrome (iSRNS) is a common problem in pediatric nephrology. About 10%–20% of children with nephrotic syndrome have iSRNS and almost 50 percent progress to end-stage renal disease. Very few studies have tried to study the histopathological spectrum, correlate clinical features, renal biopsy patterns and treatment outcome in children presenting with iSRNS in India. In this study, we compared the histopathologic distribution of different subtypes of glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy.MethodsIt is a retrospective cross-sectional study involving review of records of all children with iSRNS presenting to our pediatric nephrology OPD who underwent renal needle biopsy and followed-up for a period of atleast six months post biopsy. Histopathological subtypes were correlated with clinical features (viz. age of onset, gender, oliguria, microscopic hematuria, hypertension, serum creatinine at presentation, serum total protein, serum albumin, 24 h urine protein, persistent proteinuria after 12 weeks of calcineurin inhibitor therapy, and progress to end stage renal disease (ERSD), if any, outcome.ResultIt was found that minimal change disease (MCD) was overall the most common cause of iSRNS. These patients have a significantly greater incidence of remission with immunosuppression as compared with focal segmental glomerulosclerosis (FSGS) and IgA nephropathy subgroup.ConclusionKidney biopsy is of prognostic value in children with iSRNS. The prognosis of children with SRNS owing to MCD is much better than with other nephropathy.