小儿毛细血管内增生性IgA肾病38例临床与病理分析

目的探讨毛细血管内增生性IgA肾病患儿临床与病理之间的关系。方法选择38例肾脏病理诊断为毛细血管内增生性IgA肾病的患儿,分为肾病综合征组和非肾病综合征组以及轻、中、重度内皮细胞增生组,对其尿常规、尿蛋白定量、血清白蛋白等进行检测,对其肾组织中内皮细胞和系膜细胞增生程度进行评分。结果（1）肾病综合征组与非肾病综合征组比较,内皮细胞增生指数偏高,而系膜细胞增生指数无差异。（2）重度与轻度内皮细胞增生组比较,在前驱感染率、肉眼血尿发生率以及尿蛋白好转率上均存在差异,轻、中、重度内皮细胞增生组之间24h尿蛋白定量上均有差异。结论 在小儿毛细血管内增生性IgA肾病患者中,呈肾病综合征表现者有更严重的内皮细胞增生,而其增生程度的轻重与临床中蛋白尿的多寡有关,镜下血尿与内皮细胞增生程度无关,感染似乎是小儿毛细血管内增生性IgA肾病患者发生肉眼血尿的更主要影响因素。     

20例毛细血管内增生患者临床与病理分析

目的：观察以肾小球毛细血管内增生为主患者的临床病理特点及预后。方法：回顾性分析我院近5年内收治的20例毛细血管内增生为主患者的临床表现、中医证候特征、肾脏病理特点及随访结果等。结果：20例患者中,临床表现为急性肾炎综合征2例（10.0%）、慢性肾炎综合征1例（5.0%）、肾病综合征17例（85.0%）;中医证型中,气阴两虚证占60.0%、湿热证占75.5%、血瘀证占50.5%。肾脏病理诊断为毛细血管内增生性IgA肾病6例（30.0%）、毛细血管内增生性非IgA系膜增生性肾炎5例（25.0%）、毛细血管内增生性狼疮性肾炎5例（25.0%）、急性链球菌感染后肾炎2例（10.0%）、肾小球毛细血管内皮病2例（10.0%）,除肾小球毛细血管内皮增生外,伴有系膜增生20例（100%）、基底膜增厚8例（40.0%）、新月体形成12例（60.0%）;按照个体化治疗原则并随访1年以上,完全缓解5例（25.0%）、部分缓解8例（40.0%）、未缓解7例（35.0%）。结论：毛细血管内增生患者临床以肾病综合征多见,中医证型以气阴两虚、挟湿挟瘀为主;除肾小球内皮及系膜细胞增生外,常合并基底膜增厚及新月体形成;不同病因毛细血管内增生患者预后差别较大,如急性链球菌感染后肾炎大多预后良好、毛细血管内增生性狼疮性肾炎、肾小球毛细血管内皮病大部分能完全或部分缓解,而毛细血管内增生性IgA肾病及毛细血管内增生性非IgA系膜增生性肾炎治疗反应较差。     

小儿毛细血管内增生性IgA肾病的临床与牛津病理分型特点分析

目的：探讨儿童毛细血管内增生性IgA肾病临床特点与牛津病理分型的关系。方法：选取我院肾脏穿刺术后经病理确诊为毛细血管内增生性IgA肾病患儿38例,根据临床特点分为肾病综合征组及非肾病综合征组、肉眼血尿组及非肉眼血尿组,根据尿蛋白定量分为3组,分别为〈2 g组,2 g-3 g组,〉3 g组,分别对牛津分型中系膜细胞增生（M）、内皮细胞增生（E）的分布进行分析,来观察临床与病理之间的关系。结果：内皮细胞增生E1在临床各分组分布中差异均具有统计学意义,而系膜细胞增生M1在临床各分组分布中无差异。结论：在毛细血管增生性IgA肾病的小儿患者中,呈肾病综合征表现的患儿较无肾病综合征表现的患儿有更严重的内皮细胞增生,有肉眼血尿病史的患儿较无肉眼血尿的患儿有更严重的内皮细胞增生,且内皮细胞增生的程度随尿蛋白的增多逐渐加重,而系膜细胞增生与肉眼血尿与否及尿蛋白多寡无明确关系。     

李平教授辨治乙肝病毒相关性肾炎经验

正乙肝病毒相关性肾炎(hepatitis B virus associated-glomerulonephritis,HBV-GN),简称乙肝肾,是乙肝病毒直接或间接诱发的继发性肾小球肾炎。常见的病理类型有:膜性肾病、膜增生性肾小球肾炎、系膜增生性肾小球肾炎(包括IgA肾病)、毛细血管内增生性肾小球肾炎、局灶节段性肾小球肾炎等。其中,最常见的病理类型为膜性肾病,为继发性膜性肾病中最常     

IgA肾病诊疗进展

IgA肾病是全球最常见的原发性肾小球肾炎,亚洲人群中发病率高于其他人种。IgA肾病是目前导致终末期肾病的重要原因之一。临床上以血尿为特点,常伴随蛋白尿、高血压。其病理表现主要为IgA免疫复合物在肾小球系膜区的沉积、系膜细胞增生、毛细血管内皮细胞增生等。其发病机制可能为血液循环中半乳糖缺乏的IgA1增多,在內外界环境刺激下,产生过多的、能沉积于肾小球系膜区的免疫复合物。目前,对IgA肾病的诊断主要依靠病理检查。治疗方面,以肾素-血管紧张素系统阻断剂、控制血压为基础,恰当联合免疫抑制剂、细胞毒性药物、鱼油等或能延缓IgA肾病的进展。本文的目的是对IgA肾病的诊疗现状进行总结和分析,为临床工作及进一步科研提供指导和参考。     

IgA肾病:239例形态学和临床参数对长期预后的重要性

本文对239例IgA肾病进行了形态学和临床参数的研究,分析了肾小管间质损害与肾小球损害两者的关系及其对预后的重要性。肾活检时平均年龄33.0±11.8岁,30岁以上肾小球肾炎的进展较年轻者快。总肾存活率5年为91.8%,10年为80.5%。30岁以上肾存活率(KSR)5年为88.7%,10年为72.6%。在176例男性中KSR5年及10年分别为91.1%和83.7%,33例女性KSR5年及10年分别为93.8%和69.1%,无显著差异。在组织学研究中,中度到严重的系膜增生改变的IgA肾病较轻微或微小毛细血管内损害的IgA肾病预后差。以往对系膜增生性肾类(mesGN)的研究中,中度及重度的mesGN较之轻微或微小增生性毛细血管内肾炎(MPIGN)常合并肾小管间质性损害。无肾小管间质改变     

系膜区IgG沉积为主的原发性系膜增生性肾小球肾炎的临床病理特征

肾小球系膜区免疫球蛋白沉积是原发性系膜增生性肾小球肾炎(MsPGN)常见的病理形态学特征,而最常见的是系膜区IgA为主的免疫复合物沉积引起的IgA肾病(IgAN).近年来提出了一种以系膜区IgM为主的免疫复合物沉积引起的IgM肾病.IgG作为一种最常见的血清免疫球蛋白,其常在多种肾小球肾炎中沉积,例如膜性肾病、系膜毛细血管性肾小球肾炎和急性感染性肾小球肾炎等.     

IgA肾病的病理与临床

IgA肾病（IgA nephropathy）是我国最常见的肾小球疾病,约占原发肾小球疾病的30％～40％。自从40多年前Berger首次报道IgA肾病以来,越来越多的学者认为IgA肾病不是单一疾病,而是一组以IgA为主的免疫球蛋白颗粒状弥漫沉积在肾小球系膜区及毛细血管袢的临床综合征。IgA肾病的临床表现可从无症状镜下血尿伴或不伴有蛋白尿到典型肾病综合征,或严重的急进性肾炎综合征,     

杨霓芝教授治疗系膜增生性肾小球肾炎的临证经验

系膜增生性肾小球肾炎（mesangial proliferative glomerulonephritis,MsPGN）是一种以弥漫性肾小球系膜细胞增生及不同程度系膜基质增多为主要病理特征的原发性肾小球疾病。据其免疫病理可将其分为IgA肾病（以IgA沉积为主）及非IgA肾病两大类。     

IgA肾病伴毛细血管袢坏死的临床病理分析

目的:分析IgA肾病伴毛细血管袢坏死(Necrotizing IgA nephropathy, NIgAN)患者的临床和病理特点.方法:对15例NIgAN患者,进行回顾性分析其临床及肾脏病理参数,并和不伴毛细血管袢坏死的IgA肾病(Non-NIgAN)患者进行比较.结果:两组患者在临床指标,如蛋白尿程度、高血压发生率、肾功能水平之间无统计学差异;在肾活检指标中,NIgAN组与Non-NIgAN组相比,仅在新月体形成比例有统计学差异(P<0.01).结论:IgA肾病伴毛细血管袢坏死在总体IgA肾病中并不少见,临床表现无特殊,故常规肾活检显得尤为重要.     

Peculiar renal endarteritis in a patient with acute endocapillary proliferative glomerulonephritis

Acute glomerulonephritis (AGN) is one of the most common renal diseases. They are often associated with infections and can result in diffuse proliferative glomerulonephritis (GN). This case report reviews an interesting case in which renal endarteritis coexisted in AGN with diffuse endocapillary proliferation. The discussion highlights important pathological findings and clinical aspects in acute endocapillary proliferative GN with renal endarteritis. Coexisting endarteritis should be in the differential diagnosis of AGN in patients with persistent clinical courses.     

Focal segmental glomerulosclerosis plays a major role in the progression of IgA nephropathy. II. Light microscopic and clinical studies

It is well known that lesions morphologically identical with focal segmental glomerulosclerosis (FSGS) may appear in IgA nephropathy (IgAN). Capsular adhesions without underlying abnormalities in the tuft, often the first sign of FSGS, are frequent in IgAN. In this retrospective study, a new cohort of 128 adult patients with IgAN was used to validate the new Oxford classification system of IgAN, and shown to have highly significant associations with clinical and outcome parameters. We then used these patients to determine the extent to which IgAN could be accounted for in terms of FSGS. Some form of lesion consistent with FSGS, notably hyalinosis and collapsing glomerulopathy, was found in 101 of these patients. No glomerular lesions were found in 16 patients, and 11 had mild lesions not definable as FSGS. Those with FSGS had significantly worse renal survival at 80 months than those without. Comparison of pure forms of FSGS (excluding collapsing glomerulopathy) with cases of FSGS having other glomerular lesions (mesangial hyperplasia, endocapillary hypercellularity, glomerular necroses, extracapillary proliferation) revealed that those with FSGS and other superimposed lesions did significantly worse than cases of pure FSGS at 80 months following diagnosis. Importantly, patients with pure FSGS had relatively poor survival even without other superimposed glomerular abnormalities. Thus, the majority of cases of IgAN can be interpreted as representing one or another variant of FSGS. Hence, interpreting IgAN in terms of FSGS emphasizes the role that podocyte lesions may play in the pathogenesis and progression of this disease.     

Acute poststreptococcal glomerulonephritis superimposed on IgA nephropathy

Superimposition of poststreptococcal glomerulonephritis (PSGN) on the course of IgA nephropathy (IgAN) is uncommon. A case of PSGN during IgA nephropathy is presented. A 30-year-old man who had alternating gross and microscopic hematuria for 7 months underwent a renal biopsy. The first renal biopsy revealed IgAN with mesangial deposits of IgA and C3. Two months later, the patient suffered generalized edema, proteinuria, hematuria, an increased ASO titer and a decreased C3 level. A second renal biopsy revealed diffuse endocapillary proliferative glomerulonephritis with epimembranous hump-like electron-dense deposits of C3, but the original mesangial IgA deposits had disappeared. A diagnosis of acute PSGN was indicated. Two months after the onset of acute nephritic syndrome, the patient remained asymptomatic, except for microscopic hematuria and proteinuria. Some cases with persistent proteinuria or hematuria after PSGN are probably related to preexisting IgAN.     

IgA肾病520例临床病理分析

目的研究IgA肾病(IgAN)的临床和病理特点及其相互关系。方法对1992年11月～2003年6月温州医学院附属第一医院肾内科病理室肾活检诊断的原发性IgAN520例进行临床与病理分型关系的分析。结果520例IgAN临床表现以无症状性尿检异常最常见,占346例(66.5%),其次是慢性肾炎和肾病综合征,分别占77例(14.8%)和66例(12.7%)。病理类型以局灶节段硬化性肾小球肾炎最常见,占186例(35.8%),其次是系膜增生性肾小球肾炎、轻微病变肾小球肾炎和局灶节段增生性肾小球肾炎,分别为116例(22.3%)、104例(20%)和63例(12.1%)。结论IgAN的临床病理表现多样化并具有一定特点。临床表现最常见为无症状性尿检异常,在病理上最常见的是局灶性肾小球病变类型。     

1224例肾活检病理资料分析

目的 探讨本院近5年1224例肾活检资料的流行病学特点及病理类型分布特征.方法 回顾性分析2008年1月至2012年12月1224例肾脏疾病经皮肾活检的病理类型.结果 1224例肾脏疾病患者行肾活检时的年龄范围为4 ～ 82岁,男661例,女563例.本组原发性肾小球疾病占81.70％,继发性肾小球疾病占12.17％,小管间质疾病占5.07％,代谢相关性肾病占0.82％,移植肾占0.16％,未确定诊断0.08％.原发性肾小球疾病以系膜增生性肾小球肾炎最为常见,占30.30％,其次分别为IgA肾病28.00％,膜性肾病17.50％,局灶节段性肾小球硬化10.50％,增生硬化性肾炎3.90％,膜增生性肾小球肾炎3.10％,新月体肾炎2.60％,微小病变性肾病2.50％,毛细血管内增生性肾小球肾炎1.60％.继发性肾损害最常见的继发肾脏病是狼疮性肾炎31.54％,其次分别是高血压肾损害27.52％,紫癜性肾炎13.42％,肾脏淀粉样变性11.4％,糖尿病肾病7.38％,乙肝病毒相关性肾损害2.68％,血栓性微血管病2.01％.结论 原发性肾小球疾病仍为最常见的肾小球疾病,其中以系膜增生性肾小球肾炎最为常见,IgA肾病次之.且IgA肾病发病率呈逐年上升趋势.另外,继发性肾脏病逐渐增多,继发性肾脏疾病中仍以狼疮性肾炎最为多见.通过肾活检和临床病理讨论,不仅能明确疾病的病理类型,更重要的是指导临床治疗,选择最佳的治疗方案,并对疾病的预后作出比较正确的判断.     

Renal involvement of monoclonal immunoglobulin deposition disease associated with an unusual monoclonal immunoglobulin A glycan profile

A 38-year-old man was admitted to the hospital for the evaluation of proteinuria, microscopic hematuria, and monoclonal IgA-κ gammopathy. The initial renal pathological findings showed mesangial proliferative glomerulonephritis with endocapillary proliferation, a necrotizing lesion, and cellular crescent formation accompanied by IgA1-κ deposition in the mesangium. Neither typical immune-complex deposits nor organized-structure deposits were detected. We diagnosed the patient with monoclonal immunoglobulin deposition disease (MIDD) associated with monoclonal IgA (mIgA). After the initiation of a monthly treatment with melphalan and predonisolone (MP therapy), the patient’s serum IgA levels declined, and clinical remission was ultimately achieved. The follow-up renal biopsy showed reduced IgA-κ staining, and both the endocapillary proliferation and the necrotizing lesion had disappeared. To elucidate the mechanism of IgA deposition, we investigated the glycan profile of the patient’s serum mIgA using a mass spectrometry technique. The results revealed an unusual N-glycan profile compared to that of another patient with circulating mIgA lacking renal involvement and that of a healthy control. mIgA deposition in the mesangial area is a rare disease, and the glycan profiling of MIDD with renal involvement has not been reported previously. Thus, the present case suggests that any variation in Ig glycosylation may be a step in the pathogenesis of MIDD with renal involvement and/or contribute to some cases of IgA nephropathy.     

Validation of the Oxford classification of IgA nephropathy

The Oxford classification of IgA nephropathy (IgAN) identified four pathological elements that were of prognostic value and additive to known clinical and laboratory variables in predicting patient outcome. These features are segmental glomerulosclerosis/adhesion, mesangial hypercellularity, endocapillary proliferation, and tubular atrophy/interstitial fibrosis. Here, we tested the Oxford results using an independent cohort of 187 adults and children with IgAN from 4 centers in North America by comparing the performance of the logistic regression model and the predictive value of each of the four lesions in both data sets. The cohorts had similar clinical and histological findings, presentations, and clinicopathological correlations. During follow-up, however, the North American cohort received more immunosuppressive and antihypertensive therapies. Identifying patients with a rapid decline in the rate of renal function using the logistic model from the original study in the validation data set was good (c-statistic 0.75), although less precise than in the original study (0.82). Individually, each pathological variable offered the same predictive value in both cohorts except mesangial hypercellularity, which was a weaker predictor. Thus, this North American cohort validated the Oxford IgAN classification and supports its utilization. Further studies are needed to determine the relationship to the impact of treatment and to define the value of the mesangial hypercellularity score.     

Detection of urinary macrophages expressing the CD16 (Fc gamma RIII) molecule: a novel marker of acute inflammatory glomerular injury

BACKGROUND: The CD16 antigen is the Fc gamma receptor III. CD14+CD16+ cells are proinflammatory monocytes/macrophages (Mo/M phi) that constitute a minor population in the peripheral blood of healthy individuals. Little is known about the expression of CD16 antigen on Mo/M phi in glomerulonephritis. METHODS: Flow cytometric analyses were performed on urine and blood samples obtained from 209 patients with various renal diseases. Patients variously suffered from rapidly progressive crescentic glomerulonephritis (RPGN), membranoproliferative glomerulonephritis (MPGN), postinfectious acute glomerulonephritis (AGN), Henoch-Sch?nlein purpura nephritis (HSPN), IgA nephropathy (IgAN), membranous nephropathy (MN), minimal change nephrotic syndrome (MCNS), lupus nephritis (LN), acute interstitial nephritis, hereditary nephropathy, idiopathic renal hematuria (IRH), and renal stone. RESULTS: The CD16+ M phi population of cells was present in the urine of hematuria-positive patients with proliferative glomerulonephritis, including AGN, IgAN, RPGN, MPGN, and LN with acute inflammatory lesions, such as endocapillary proliferation, tuft necrosis, and cellular crescents. In contrast, the urinary CD16+ M phi population was negligible in hematuria-positive patients with nonproliferative renal disease, including hereditary nephropathy, IRH, and renal stone and also in patients with proliferative glomerulonephritis lacking acute inflammatory lesions. Total urinary M phi of these patients were much less than those of patients having proliferative glomerulonephritis with acute inflammatory lesions. Transient expansion of the CD16+ M phi population in urine was observed during the acute exacerbation of urinary abnormalities, whereas the disappearance of CD16+ M phi closely preceded the amelioration of urinary abnormalities in patients with proliferative glomerulonephritis. In 38 of the 98 patients positive for CD16+ M phi population in urine, the CD16+ Mo population was negligible in peripheral blood. Immunohistochemically, CD16+ M phi were present in the glomeruli of active proliferative glomerulonephritis, whereas such cells were absent in inactive proliferative glomerulonephritis or nonproliferative glomerular diseases. CONCLUSION: CD16+ M phi may be effector cells involved in the acute inflammation common to all types of proliferative glomerulonephritis. Furthermore, the detection of CD16+ M phi in urine, as well as urinary M phi counts, may serve as a useful indicator of the active stage of proliferative glomerulonephritis.     

Clinical and histopathologic profile of patients with primary IgA nephropathy seen in a tertiary hospital in India

Background IgA nephropathy (IgAN) is known to have an aggressive course in Asians. There is a paucity of data regarding the Oxford classification pattern of Indian patients with IgAN. This study aims to characterize the clinical and histopathologic profile of these patients. Methods All patients diagnosed to have primary IgAN by kidney biopsy in the nephrology department from July 2009 to July 2014 were included in this study. All kidney biopsies were reviewed and the MEST score was assigned as per the Oxford classification. The clinical features and Oxford classification score of patients were characterized. Results Nephrotic range proteinuria (NRP) (65/103, 63.1%) with or without edema was the commonest presentation. 67.0% patients had eGFR ≥?60 mL/min and 16.5% patients had eGFR Conclusion Indian patients with primary IgA nephropathy have a unique profile. They commonly present with nephrotic range proteinuria. A significant proportion of these patients have normal renal function despite heavy proteinuria. Mesangial proliferative lesions are predominant with a paucity of endocapillary proliferation and crescents compared to other Asian populations. Immunosuppressive use is more common in Indian patients.