肉眼血尿型和肾病综合征型IgA肾病组织学定量分析的差异

肉眼血尿型和肾病综合征型ＩｇＡ肾病组织学定量分析的差异杨剑辉，粱俊林，鲍旭东ＩｇＡ肾病（ＩｇＡＮ）是一类以肾小球系膜区弥漫性ＩｇＡ沉积为特征、排除全身性疾患的肾脏疾病。临床上可表现为孤立性肉眼血尿、无症状尿检异常、反复发作性肉眼血尿（ＥＭＨ）和肾病综...     

36例血尿患者的临床与病理分析

血屎是肾活检病理检查常见适应证之一。本文时３６例临床表现为单纯性血尿或以血尿为主要表现的患者肾活检病理类型进行了分析，发现系膜增生性肾小球肾炎和ＩｇＡ肾病占５５．６％。值得注意的是，２例ＩｇＡ肾病患者临床上表现为非肾小球性血尿，几例有较严重肾脏位置异常的病人，病理诊断为肾小球肾炎。同时指出了血尿病人行肾活栓病理检查的必要性。     

原发性肾病综合征病理与临床的联系

肾活检技术的广泛开展和电子显微镜、荧光免疫检查的应用,使人们对肾病综合征的发生、发展过程及病理变化的认识愈加深刻。本文对原发性肾病综合征病理变化与临床表现、治疗及预后关系综述如下。1原发性肾病综合征病理与临床表现的联系引起原发性肾病综合征表现的肾小球疾病常见病理类型有:轻微病变性肾炎,膜性肾病,系膜增生性肾小球肾炎,系膜毛细血管性肾小球肾炎和局灶节段性肾小球硬化等。1.1轻微病变性肾炎本型病变轻微,常见于儿童,约占65%-85%。光镜下肾小球形态正常,仅见肾小管上皮细胞肿胀,内有脂质沉积。电镜下上皮细胞肿胀,其足突广泛融合变平,基底膜正常。免疫学无阳性发现。临床上常表现为典型的肾病综合征,大量蛋白尿、高度水肿、低蛋白血症及高脂血症。部分患者镜下可见血尿。     

早期轻链沉积性肾病3例临床病理分析

目的探讨早期轻链沉积性肾病(LCDN)的临床病理学特征及诊断,提高对该病的认识。方法回顾性分析3例早期轻链沉积性肾病肾穿组织的临床资料,观察光镜、电镜病理改变及免疫组化染色特点,并复习相关文献。结果老年男性2例,中年女性1例。3例均有大量蛋白尿,其中前2例以水肿为首发症状,例3有多发性骨髓瘤(λ型)病史。例1肾小球呈轻度系膜增生,近曲小管上皮细胞弥漫肿胀;免疫组化标记近曲小管上皮细胞质弥漫性λ链强阳性;电镜下肾小管基底膜内侧见间断线性电子致密物沉积;病理诊断为轻链小管肾病。例2肾小球呈弥漫膜性(Ⅰ~Ⅱ期)病变,免疫组化示局灶节段肾小球系膜区单克隆性λ链块状沉积,提示伴早期轻链沉积性肾病。例3肾小球病变轻微,免疫组化示系膜区和入球动脉壁散在块状Igλ强阳性,蛋白管型呈Igλ强阳性,相同部位Igκ染色强度为弱阳性;病理诊断为轻微病变型轻链沉积性肾病。结论早期轻链沉积性肾病无典型的系膜结节状结构,肾小球及肾小管形态学可病变轻微,极易漏诊,应结合轻链蛋白的免疫组化和电镜检查进一步确诊。     

以肾病综合征为表现的毛细血管内增生性肾小球肾炎的临床与病理

目的探讨毛细血管内增生性肾小球肾炎并发肾病综合征的临床病理特点与发病机制。方法12例患者肾穿刺术后,标本经常规固定、包埋、切片,进行HE、六胺银、Masson和免疫荧光染色,其中3例予以电镜观察。结果肾小球系膜细胞、内皮细胞增生;小球内可见中性多核白细胞渗出。10例毛细血管襻和(或)灶性系膜区有免疫复合物沉积。3例上皮细胞足突融合消失,上皮下电子致密物沉积。结论EPGN发生大量蛋白尿的机制与肾小球滤过膜状态的改变关系密切,使其对蛋白的通透性大大增加。     

p21、p27和PCNA在IgA肾病系膜细胞中的表达水平研究

IgA肾病是最常见的原发性肾小球肾炎，是导致终末期肾功能衰竭最常见的原因之一，也是我国最常见的原发性肾小球疾病，发病率较高，占原发性肾小球疾病的30％～40％。IgA肾病肾小球系膜区有以IgA免疫复合物为主的颗粒样沉积，同时有系膜细胞的增生，基质增多，系膜区电子致密物沉积。通过长期观察发现约20％的IgA肾病患者经20年临床疾病演变后进展至终末期肾功能衰竭。现通过对肾脏不同病理损害程度系膜细胞中p21、p27、PCNA的表达水平，进一步研究抑制系膜细胞增生的调控因子。     

基质金属蛋白酶及其抑制剂与糖尿病肾病的关系

糖尿病肾病(DN)是引起终末期肾病最主要原因之一。但引起DN发生及发展的具体机制至今仍不完全明确。DN的早期病理改变是系膜区扩张,主要是由于细胞外基质(ECM)沉积和系膜细胞增生所致;肾小球基底膜(GBM)增厚也在早期可见,主要是由于ECM代谢失衡。由于在正常肾脏ECM重构中系膜     

IgA肾病

１概述ＩｇＡ肾病（ＩｇＡＮｅｐｈｒｏｐａｔｈｙ）是一免疫病理诊断名称。１９６８年由Ｂｅｒｇｅｒ和Ｈｉｎｇｌａｉｓ首先提出,故又可称Ｂｅｒｇｅｒｓ病。临床上以血尿为突出表现曾称局灶性肾小球肾炎。肾活检光镜下见弥漫性系膜增生为主也有诊断为系膜增生性肾小球...     

原发性膜性肾病合并IgA肾病3例临床病理分析

目的探讨原发性膜性肾病合并IgA肾病的临床病理特征。方法分析3例经肾活检病理检查及实验室检查证实的原发性膜性肾病合并IgA肾病患者的临床表现及病理特点。结果2例患者呈肾病综合征表现,伴或不伴轻微镜下血尿,肾小球基底膜增厚,系膜无明显增生/轻度增生;1例呈慢性肾炎综合征表现,有发作性肉眼血尿并伴有高血压,肾小球基底膜弥漫增厚,伴轻至中度系膜增生、内皮细胞节段增生、小细胞性新月体形成及纤维素样坏死。结论原发性膜性肾病合并IgA肾病较为罕见,部分患者临床表现与膜性肾病相近,也有患者病理及临床表现以IgA肾病为主。     

六次甲基四胺-硝酸银基底膜染色改良法在肾活检组织中的应用

<正>在肾小球肾病的肾活检病理诊断中,观察与研究肾小球系膜、小球毛细血管基底膜及球囊增生新月体的形态具有极为重要的诊断价值。目前常用的基底膜染色方法多从霉菌染色的六胺银法演化形成,该方法存在试剂浓度不当、染色     

Renal disorders in rheumatoid arthritis]

Glomerulonephritis has been believed to be a rare complication in rheumatoid arthritis (RA). However, recent studies have revealed a focal segmental increase in mesangial cells and matrix in RA patients with hematuria. In our series, proteinuria, hematuria or both abnormalities were recognized in 74 (22%) out of 336 RA cases. Among 119 patients examined by renal biopsy, mild mesangial proliferative glomerulonephritis (GN) was found in 25 patients, of which 22 demonstrated mesangial IgA deposits, by immunofluorescent microscopy. Membranous nephropathy was noticed in 26 cases. Three cases of membranous nephropathy had no history of gold or D-penicillamine treatment. Electron microscopy revealed diffuse thinning of the glomerular basement membrane in 12 cases. The average thickness of the glomerular basement membrane was significantly thinner in RA patients than in normal subjects. The immunological processes associated with rheumatoid factor do not seem to be related to the renal lesions in RA patients.     

Fabry病合并IgA肾病临床病理特点分析

目的总结Fabry病合并IgA肾病的临床病理特点。方法回顾性分析2例Fabry病合并IgA肾病患者的临床资料。结果2例患者均为男性,1例表现为蛋白尿并镜下血尿,高血压,肾功能正常;1例表现为蛋白尿,肾功能正常;2例外周血α-半乳糖苷酶A活性均较正常降低;光镜下均见肾小球足细胞空泡变性呈蜂窝状,足细胞胞质内见嗜甲苯胺蓝颗粒;电镜下足细胞胞质内大量髓样小体;均有肾小球系膜增生病变,伴有IgA弥漫颗粒状沉积于系膜区。结论Fabry病合并IgA肾病临床表现缺乏特异性,但具有特征性病理形态学改变,肾组织活检是确诊主要依据。     

123例老年肾脏病患者的肾病理和临床特征分析

目的：探讨老年人原发性肾小球疾病及继发性肾脏疾病肾活检病理类型及临床特点。方法：回顾分析2003年—2005年123例年龄≥65岁,资料完整,并经临床和肾活检确诊为原发性肾小球疾病或继发性肾脏疾病患者的肾脏活组织病理和临床资料,并与同期367例中青年患者的肾脏活组织检查和临床资料进行对比。结果：①老年患者原发性肾小球疾病中膜性肾病最为常见,占原发肾脏疾病的31.46%,其次为IgA肾病（25.84%）。中青年患者原发肾脏疾病中以IgA肾病最为常见,占39.49%,其次为系膜增生性肾小球肾炎,占22.88%。②老年IgA肾病患者病理以局灶节段性肾小球硬化（FSGS）为主,占21.74%,而中青年患者以弥漫系膜增生为主,占30.95%。③糖尿病肾病是老年人最常见的继发性肾脏疾病,占32.35%,狼疮性肾炎是中青年患者最常见的继发性肾脏疾病,占58.33%。④老年肾脏疾病患者尿蛋白定量（3.60±0.78g.24h^-1）显著高于中青年组（2.19±0.68g.24h^-1）,P〈0.05。高血压的发生率显著增高（73.98%比30.51%,P〈0.01）。结论：老年患者肾脏病理类型与中青年患者不一致,老年原发性肾小球疾病以膜性肾病为主,IgA肾病的病理类型以FSGS为主;中青年患者原发性肾小球疾病以IgA肾病为主,IgA肾病的病理类型以弥漫系膜增生为主。老年患者继发性肾脏疾病的发生率高,以糖尿病肾病最为常见。肾脏病理类型的不同,是老年肾脏病患者临床表现中尿蛋白多和高血压的发生率增高的原因之一。     

Study of lupus nephritis: its classification and the significance of subendothelial deposits

This study describes the clinical and pathological characteristics of 74 patients with lupus nephritis classified according to renal biopsy findings using light, electron and immunofluorescent microscopy, and further, assesses the significance of subendothelial deposits in evaluating disease activity. In membranous lupus nephritis (14 cases), many cases showed normal renal function even with the nephrotic syndrome, although five cases had little or no urinary abnormalities. Glomerular cellular proliferation was very mild and subepithelial deposits with a few mesangial deposits were the main pathological alterations. Mesangial proliferative lupus nephritis (17 cases) clinically had very mild renal disease. Renal biopsies in this group revealed mesangial deposits with slight cellular proliferation. Although clinical features of mild diffuse proliferative lupus nephritis (16 cases) were similar to those of mesangial lupus nephritis, glomerular loop deposits were seen in addition to mesangial deposits. In moderate diffuse proliferative lupus nephritis (17 cases), renal function was slightly decreased, moderate proteinuria with haematuria were found, and C3 level was low. Renal biopsies showed active proliferative changes, and subendothelial deposits were frequently seen. In severe diffuse proliferative lupus nephritis (10 cases), the duration from onset of SLE to renal biopsy was short. Impairment of renal function, and nephrotic syndrome with haematuria and hypocomplementemia were frequent. Only three patients survived in this group. Renal biopsies demonstrated highly active proliferative and necrotizing changes, and electron microscopy showed massive subendothelial and mesangial deposits accompanied by subepithelial and intramembranous deposits. The amount of subendothelial deposits correlated with those of mesangial deposits and subepithelial deposits in the cases with diffuse proliferative lupus nephritis. Urinary protein loss and histologic activity showed statistically significant correlations with the amount of subendothelial deposits, but C3 levels and creatinine clearance revealed negative correlations with those deposits.     

Detection of anionic sites and immunoglobulin A deposits in the glomerular capillary walls from patients with IgA nephropathy

A study of anionic sites in the glomerular basement membrane (GBM) from patients with immunoglobulin A (IgA) nephropathy is described. The relationship between the deposition of IgA and the detection of glomerular extracellular components, i.e., noncollagenous (NC-1) domain of Type IV collagen, in the glomerular capillary walls was examined by double immunofluorescence. Renal biopsy specimens from patients with IgA nephropathy were immersed in polyethyleneimine (PEI) as a cationic probe and then examined by electron microscopy. Renal specimens were also incubated with mouse monoclonal anti-NC-1 domain of Type IV collagen and then stained with fluorescein isothiocyanate (FITC)-labelled goat antimouse Ig antiserum. After these reactions, sections were stained with rhodamine-labelled rabbit antihuman IgA antiserum. GBM subepithelial anionic sites marked by PEI were altered by the deposition of electron-dense deposits (EDD) in patients with IgA nephropathy and there was a significant correlation between the levels of proteinuria and the incidence of EDD in the GBM in such patients. Marked proteinuria was observed in patients who showed loss of anionic sites in the GBM by electron microscopy. By double immunofluorescence, IgA was shown to be focally deposited outside the NC-1 domain of Type IV collagen-detected regions in the same patients. The authors concluded that high levels of proteinuria might be due to alterations of the size barrier and/or anionic sites of GBM in the moderate stage of IgA nephropathy.     

乙型肝炎病毒相关性肾炎的临床与病理分析

目的探讨乙型肝炎病毒（HBV）相关性肾炎临床病理特点。方法分析17例HBV相关性肾炎的临床表现及肾活检病理、免疫荧光和超微病理特点。结果17例HBV相关性肾炎临床表现以肾病综合征最常见10例（58.82%）,肾炎综合征7例（41.18%）。病理类型多样化,其中膜性肾病（MN）8例（47.06%）,系膜增生性肾炎（MsPGN）7例（41.18%）,系膜毛细血管性肾炎（MPGN）1例（5.88%）,IgA肾病（IgAN）1例（5.88%）。免疫荧光检查5.88%的病例呈＂满堂亮＂现象;电镜观察HBV相关性膜性肾病易见电子致密物系膜沉积。结论HBV-GN的病理类型以HBV-MN为主,多见于中青年人,肾组织HBVAg免疫组化标志为诊断HBV-GN的重要指标。鉴于HBV相关性肾炎的一些病理特点与狼疮性肾炎相似,有必要结合临床对二者进行鉴别。     

The clinico-pathologic features of hepatitis B virus-associated glomerulonephritis

The frequency of hepatitis B surface antigen (HBsAg) was studied in the sera of 311 patients with various forms of primary glomerulonephritis and 43 patients with lupus nephritis. HBs antigenaemia was detected in 69 of the 311 patients (22 per cent) with primary glomerulonephritis and this prevalence of HBsAg carrier was significantly higher than that in the general population (p less than 0.001). These patients had no clinical or biochemical findings to suggest acute or chronic liver disease. A higher HBs antigenaemia carrier rate was not observed in patients with lupus nephritis. Three glomerulopathological entities, membranous nephropathy, IgA nephropathy, and mesangial proliferative glomerulonephritis, were found to be associated with a higher prevalence of HBs antigenaemia compared with the general population (p less than 0.001). Glomerular deposits of HBsAg and/or hepatitis core antigen (HBcAg) were detected in 41, 61, and 60 per cent of renal biopsy specimens from patients with membranous nephropathy, IgA nephropathy, and mesangial proliferative glomerulonephritis associated with persistent HBs antigenaemia respectively. During the mean study period of 40 months (range 12-180), 14 per cent of these patients with hepatitis-associated glomerulonephritis developed progressive renal failure, although none required maintenance dialysis. Our study suggests that hepatitis B virus antigenaemia may play a significant role in the development of specific forms of glomerulonephritis and that these hepatitis B virus-associated glomerulonephritides can run an indolent but relentless progressive clinical course.     

小儿常见肾脏疾病尿电导率的变化分析

目的：检测小儿常见肾脏疾病尿电导率的变化,探讨该指标的临床意义。方法选择2011年3月至2012年3月本院小儿肾脏内科收治的首次诊断为肾脏疾病患儿986例及同期健康体检儿童350例的尿液,利用 Sysmex 公司的全自动尿液分析仪 UF-1000i 分别测定其尿电导率的变化。根据临床诊断将肾脏疾病患儿分为肾病综合征组、肾小球肾炎组、肾功能不全组、紫癜性肾炎组和狼疮性肾炎组。其中216例进行肾穿刺活检。根据肾穿刺活检病理诊断结果将患儿分为系膜增生性肾小球肾炎组、毛细血管内增生性肾小球肾炎组、膜性肾病组、IgA 肾病组、IgM 肾病组、过敏性紫癜性肾炎组和狼疮性肾炎组。结果1．肾病综合征组、肾小球肾炎组、肾功能不全组、紫癜性肾炎组、狼疮性肾炎组患儿的尿电导率明显低于健康对照组,差异具有统计学意义（P ＜0．05）。2．系膜增生性肾小球肾炎组、毛细血管内增生性肾小球肾炎组、膜性肾病组、IgA 肾病组、过敏性紫癜性肾炎组和狼疮性肾炎组患儿尿电导率明显低于健康对照组,差异具有统计学意义（P ＜0．05）。3．IgM 肾病组与健康对照组相比,尿电导率差异无统计学意义（P ＞0.05）。结论尿电导率可以作为小儿肾脏功能和尿液浓缩功能的重要指标。     

IgA-dominant postinfectious glomerulonephritis: a new twist on an old disease

IgA-dominant acute postinfectious glomerulonephritis (APIGN) is an increasingly recognized morphologic variant of APIGN, particularly in the elderly. In contrast to classic APIGN, in which there is typically glomerular deposition of IgG and C3 or C3 only, IgA is the sole or dominant immunoglobulin in IgA-dominant APIGN. Because the vast majority of reported cases occur in association with staphylococcal infections, the alternative designation 'IgA-dominant acute poststaphylococcal glomerulonephritis' has been applied. Diabetes is a major risk factor, likely reflecting the high prevalence of staphylococcal infection in diabetics, particularly involving skin. Patients typically present with severe renal failure, proteinuria and hematuria. Prognosis is guarded with less than a fifth of patients fully recovering renal function. This variant of APIGN must be distinguished from IgA nephropathy. Features that favor IgA-dominant APIGN over IgA nephropathy include initial presentation in older age or in a diabetic patient, acute renal failure, intercurrent culture-documented staphylococcal infection, hypocomplementemia, diffuse glomerular endocapillary hypercellularity with prominent neutrophil infiltration on light microscopy, stronger immunofluorescence staining for C3 than IgA, and the presence of subepithelial humps on electron microscopy. The pathogenetic mechanism of selective IgA deposition in patients with poststaphylococcal glomerulonephritis likely involves specific host responses to the inciting pathogen.