急进性IgA肾病

1969年Berger首先描述了一种在肾小球系膜区有IgA和IgG、C_3沉积的肾小球肾病,其典型病例为无症状性镜下血尿,常伴有蛋白尿和/或发作性肉眼血尿。多见于男性、学龄儿和青年。早先报道资料认为,此病预后良好,而近年资料表明有些病人经过数年至数十年后,肾功能慢性衰退。方法及诊断标准作者15年中进行肾活检819例,其中5例病理和临床表现符合以下标准而确定为急进性IgA肾病,即:(1)肾小球系膜区有IgA和C_3沉积,可伴有或不伴有IgG或IgM沉积;(2)40%以上肾小球有新月体形成或肾小球荒废;(3)无全身     

伴有新月体形成的原发性IgA肾病的临床与病理分析

目的了解儿童伴有新月体形成的原发性IgA肾病的临床与病理特点.方法对29例伴新月体形成的原发性IgA肾病患儿的临床及病理资料进行分析,并依受新月体累及的肾小球比例分组比较,≥50%(A组),9例;<50%(B组),20例.结果 (1)临床方面29例均有血尿+蛋白尿,尿蛋白≥1 g/24 h 者22例(76%)和肉眼血尿86%,水肿、高血压、肾功能异常者均不及半数.A组以肾病综合征和急进性肾炎为主,持续性肉眼血尿、大量蛋白尿、高血压、肾功能衰竭均较B组明显(P<0.05).B组无症状性血尿+蛋白尿者65% .(2)病理方面新月体形成累及肾小球5%～85%, A组为52%～85%(其中新月体型IgA 肾病10%),B组5%～40%,以细胞性为主.均有系膜增生和小管-间质病变,球囊粘连易见. 两组比较A组系膜增生严重、小球硬化和小管灶状萎缩明显(P<0.05),B组球囊粘连多见(P<0.05).(3)免疫荧光均有IgA+IgM+C3沉积,合并IgG沉积者18例(62%),其中5例(17%)为"满堂亮"(A组占4例).未见一例单纯IgA沉积.结论伴有新月体形成的原发性IgA肾病临床均有血尿合并蛋白尿,以持续性肉眼血尿和大量蛋白尿为主;以弥漫性系膜增生为主要病理改变,易见球囊粘连和小管-间质病变;沉积物以IgA+IgM型或IgA+IgM+IgG型多见,部分呈"满堂亮";较一般型IgA肾病临床、病理明显加重.     

江西地区肾脏病儿童757例肾活检的病理与临床资料分析

目的 探讨儿童肾脏疾病的病理特点及其与临床表现的关系.方法 回顾性分析2002年2月-2010年6月在江西省儿童医院行肾活检的757例肾病患儿的病理及临床资料.将肾活检组织分别行光镜、免疫荧光、免疫组织化学及电镜检查.肾活检组织均作苏木精-伊红(HE)、过碘酸雪夫反应(PAS)、六胺银(PASM)及Masson染色;免疫荧光检测IgG、IgA、IgM、C3、C4、C1q.有乙型肝炎病毒感染证据者肾组织同时行乙型肝炎表面抗原(HBsAg)、乙型肝炎e抗原(HBeAg)、乙型肝炎核心抗原(HBcAg)免疫组织化学.参照中华医学会肾脏病分会2000年制定的标准进行病理分型,结合临床和病理资料进行统计分析.结果 1.肾活检病例757例中原发性肾小球疾病537例(70.97%),其中肾病综合征265例(49.35%),孤立性血尿99例(18.44%);继发性肾小球疾病211例(27.84%),其中紫癜性肾炎144例(68.25%),乙肝相关性肾炎47例(22.27%);遗传性肾小球疾病9例(1.19%).2.原发性肾小球疾病病理类型最多的是系膜增生性肾小球肾炎277例(51.58%);继发性肾小球疾病中紫癜性肾炎最多,为144例(68.25%),其病理分级以Ⅱb～Ⅲb为主,占79.17%;遗传性肾小球疾病中Alport综合征8例;薄基底膜肾病1例.结论 江西地区儿童肾脏疾病以原发性肾小球疾病为主,病理改变以系膜增生性肾小球肾炎占绝大多数;继发性肾小球疾病中除以紫癜性肾炎为主外,乙肝相关性肾炎并不少见.     

单纯性血尿患儿肾组织免疫病理及肾小管CD_(40)的表达

目的观察单纯性血尿患儿肾组织免疫病理变化及肾小管CD40的表达,探讨免疫炎症反应在其发病机制中的作用。方法选取2004年1月-2007年3月在苏州大学附属儿童医院就诊并行肾活检的单纯性血尿患儿32例。全部患儿肾活检后,取部分肾组织经快速冷冻切片,进行常规病理和免疫荧光分析。同时采用免疫组织化学方法检测其肾小管CD40表达情况,采用计算机图像分析系统拍摄数字图像。采用Image-Pro Plus 5.02病理图文分析系统,计算各处理组肾小管CD40阳性表达率,并进行统计学分析。结果不同临床类型的单纯性血尿患儿肾组织病理主要表现为系膜增生性改变,其中轻度增生和弥漫性增生分别占全部患儿的37.50%和46.88%,伴局灶/阶段性细胞增生、坏死、纤维化和肾小球硬化者5例(占15.63%),未见膜性肾病和弥漫性硬化性肾小球肾炎病例。不同临床类型、病理类型的患儿均见IgA和(或)IgM沉积,其中系膜区以IgA沉积为主者占全部患儿的62.50%,且常伴IgM和(或)IgG沉积,单纯表现为IgA沉积者仅1例。以IgM沉积为主(不伴IgA)者占全部患儿的37.50%,其中仅有IgM者9例,占全部患儿的28.13%,伴IgG沉积...     

儿童紫癜性肾炎和IgA肾病临床病理对比分析

目的：探讨儿童紫癜性肾炎（HSPN）和IgA肾病在临床和肾脏病理上的异同。方法：回顾性分析103例HSPN患儿和61例IgA肾病患儿的临床资料和肾脏病理资料。结果：HSPN患儿与IgA肾病患儿在年龄、性别及病程方面比较差异无统计学意义（P>0.05）。与HSPN患儿比较,IgA肾病患儿临床分型更重,且更易出现肉眼血尿,差异有统计学意义（P<0.05）。IgA肾病患儿的血肌酐及胆固醇水平较HSPN患儿增高,差异有统计学意义（P<0.05）。光镜病理显示HSPN患儿较IgA肾病患儿易出现纤维蛋白原相关抗原沉积,而IgA肾病患儿较HSPN患儿易出现补体C3沉积,且间质纤维化、肾小管管型、肾小管炎症细胞浸润在IgA肾病患儿更常见（P<0.05）。结论：儿童HSPN和IgA肾病在临床表现、肾脏病理等方面存在较大差异,这些差异不支持两者为同一疾病的假说。     

儿童紫癜性肾炎55例临床与病理分析

目的 探讨儿童紫癜性肾炎(Henoch-Sch(o)nlein purpura nephritis,HSPN)的临床和病理特点及其相关性,以指导治疗,改善预后.方法 收集2000年1月至2008年10月收治的55例紫癜性肾炎患儿的临床及病理资料,并且根据不同的条件分组进行网顾性分析.结果 临床分型中以血尿和蛋白尿最多,为22例(40.0%);肾病综合征型次之,16例(29.1%);单纯性蛋白尿或单纯性血尿型、急性肾炎型和急进性肾炎型占少数,分别为6例(10.9%)、4例(7.3%)和1例(1.8%).伴有消化道症状的紫癜性肾炎患儿,临床上较无消化道症状的患儿易有肾病水平蛋白尿(P<0.05).伴肉眼血尿患儿比镜下血尿者易出现肾病综合征(P<0.05).根据ISKDC标准,HSPN病理分级以Ⅱ级和Ⅲ级最多,分别为27例(49.1%)和16例(29.1%).免疫荧光检查显示IgA+IgG+IgM型为32例(58.2%),伴C3沉积41例(74.6%),病理分级主要在Ⅲ级以上.免疫沉积类型、C3沉积与病理分级有相关性(P<0.05).临床表现为单纯性血尿型或单纯性蛋白尿型的HSPN病理损害较轻,7例(58.3%)为Ⅱ级;血尿和蛋白尿型的病理分级以Ⅱ级和Ⅲ级多见,分别为12例(54.5%)和5例(22.7%);肾病综合征型的病理分级多见Ⅲ级以上,为10例(62.5%).结论 儿童紫癜性肾炎临床以血尿和蛋白尿型及肾病综合征型为主,病理分级以Ⅱ级和Ⅲ级最常见,免疫沉积物类型与C3沉积和病理分级有相关性.HSPN临床症状和病理损伤程度基本一致;消化道受累程度越重,出现蛋白尿的比例越高;肉眼血尿与肾脏损害的严重程度有关.     

47例Alport综合征临床与病理分析

目的 分析不同年龄组Alport综合征患儿的临床与病理特点.方法 回顾性分析我院1990年1月-2007年1月47例住院并且明确诊断为Alport综合征患儿的临床及病理资料.结果 男32例,女15例,男女比例2.1:1,年龄15个月～13岁,平均9.0岁.47例患儿中39例有明确家族史,其中X连锁显性遗传37例;常染色体隐性遗传2例.28例(59.3%)患儿首发症状为肉眼血尿或镜下血尿,14例(29.8%)患儿为蛋白尿、水肿.Alport综合征临床表现为孤立性血尿11例(23.4%)、血尿合并蛋白尿17例(36.2%)、肾病综合征14例(29.8%)、肾功能不全5例(10.6%);孤立性血尿型、血尿合并蛋白尿型见于研究病例中的所有年龄组儿童,而肾病综合征型、肾功能不全型仅见于7～13岁年龄组儿童.肾组织病理显示,33例(70.2%)光镜改变为系膜增生性病变(MsPGN),13例(27.6%)表现为局灶节段性肾小球硬化(FSGS),1例(2.1%)表现为膜增生性肾小球肾炎(MPGN).免疫荧光多以IgM沉积为主19例(40.4%),以IgA沉积为主9例(19.1%),以IgG沉积为主9例(19.1%),免疫荧光阴性10例(21.4%).电镜下39例出现典型的肾小球基底膜病变,8例显示基底膜变薄.47例患儿中46例肾脏和(或)皮肤Ⅳ胶原a链分布异常.结论 Alport综合征男性患儿发病率高于女性.不同年龄组Alport综合征肾脏表现有明显差异,血尿伴随疾病始终,但随着病程延长,尿蛋白量逐渐增加.Alport综合征肾脏病理光镜下无特征表现,主要以系膜增生为主,小年龄组患儿电镜下GBM病变不典型,需结合Ⅳ胶原a链免疫荧光检测明确诊断.     

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目的探讨儿童特发性膜性肾病(IMN)的临床和病理特征。方法 1999年7月至2009年7月在中山大学附属第一医院确诊的IMN患儿13例,回顾性分析其临床病理特征、治疗及转归情况。结果 13例IMN患儿中男11例,女2例;就诊时中位年龄为11.4(3.0～14.5)岁;肾穿时中位病程为3.7(1.4～65.3)个月。确诊时临床表现为肾病综合征(NS)9例(单纯型1例,肾炎型8例),血尿蛋白尿3例,复发性肉眼血尿1例,伴高血压1例(7.7%)。所有患儿的肾功能均正常。肾脏病理结果:(1)光镜病理分期:Ⅰ期4例,Ⅱ期9例。4例伴肾小球球性硬化(球性硬化肾小球百分比分别为11.8%、2.9%、7.5%、4.5%),1例伴节段性硬化(节段硬化肾小球百分比为4.5%),3例伴肾小管萎缩,1例伴细胞纤维性新月体形成(3.8%)。(2)免疫荧光:以IgG、C3在肾小球基底膜(GBM)上皮下沉积为主(前者12/12例、后者10/12例),少数伴IgM、Fg、C1q和IgA沉积,但沉积强度较IgG、C3弱。(3)电镜:13例患儿GBM均有不同程度增厚,2例毛细血管腔内出现微血栓。根据患儿临床表现及病理分期予激素和(或)免疫抑制剂...     

紫癜性肾炎患儿肾脏病理与足细胞损伤的关系

目的 探讨儿童紫癜性肾炎(HSPN)肾脏病理分级、免疫荧光分型和肾小球足细胞损伤的关系.方法 回顾性分析2008年1月至201 1年1月南京军区南京总医院儿科肾活检病理检查诊断为HSPN的住院病例72例,对其进行病理分级和免疫荧光分型,并在电镜下观察足细胞损伤情况;分析足细胞损伤和病理分级的关系.结果 病理分级集中在Ⅱ级和Ⅲ级,其中病理分级为Ⅱ级的患儿34例(占47.2％),病理分级为Ⅲ级的患儿38例(占52.8％).电镜下观察肾小球足细胞损伤情况:足突广泛融合21例,足突节段融合35例和无足突融合11例.5例电镜下未见肾小球.足突广泛融合患儿病理分级为Ⅲ级者较足突节段融合、无足突融合者多.足突广泛融合患儿中免疫荧光分型以IgA+ IgM+ IgG型最多;足突节段融合患儿中免疫荧光分型以IgA+ IgG型较多,无足突融合患儿中免疫荧光分型以单纯IgA型最多.结论 儿童HSPN的肾小球病理改变除系膜细胞的增生外,还存在足细胞损伤的情况,足细胞损伤情况越重病理改变越明显.     

儿童肾脏疾病临床与病理753例分析

目的分析和总结儿童肾脏疾病的临床资料和病理类型,探讨肾穿刺活检术在儿童肾脏疾病中的重要性。方法回顾性分析1995—2015年在山东大学附属省立医院小儿肾脏风湿免疫科接受经皮肾穿刺活检的753例肾脏疾病患儿临床资料和病理资料。结果 753例经皮肾穿刺活检患儿中,原发性肾小球疾病428例(56.84%)、继发性肾小球疾病306例(40.64%)、先天性遗传代谢性肾脏疾病17例(2.26%)、肾小管间质性疾病2例(0.27%)。临床诊断最常见的为原发性肾病综合征。原发性肾小球疾病中最常见的临床表现和病理类型分别为原发性肾病综合征和IgA肾病,继发性肾小球疾病中常见的临床表现和病理类型分别为紫癜性肾炎和系膜增生性肾小球肾炎。先天性遗传代谢性肾脏疾病中最常见的为薄基底膜肾病和Alport综合征。8例重复肾活检患儿的病理类型及临床表现均出现改变。结论儿童肾脏疾病以原发性肾小球疾病为主,最常见的临床诊断和病理类型分别为原发性肾病综合征和系膜增生性肾小球肾炎。重复肾穿刺活检有助于及时掌握患儿病理类型转化,调整治疗方案。     

Henoch-Schönlein purpura nephritis: an update

Henoch-Sch?nlein purpura (HSP) is a form of systemic vasculitis characterised by vascular wall deposits of predominally IgA typically involving small vessels in skin, gut and glomeruli and associated with purpura, colic, haematuria and arthralgia or arthritis. HSP nephritis (HSPN) leads to chronic renal failure in up to 20% of paediatric patients after 20 years of follow-up in selected series. The risk is related to the initial clinical presentation and the percentage of glomeruli presenting with epithelial crescents. The pathogenesis of HSPN might be related to an increased production of abnormally glycosylated IgA, which is not sufficiently cleared by the liver and leads to the formation of IgA macromolecules, accumulating in the circulation with subsequent deposition in vessel walls and in the glomerular mesangium. HSPN is related to IgA nephropathy. These two diseases can be encountered consecutively in the same patient, have been described in identical twins and bear similar pathological and biological abnormalities. No consensus about treatment has been reached up to now. Recent studies indicate that early treatment with methylprednisolone or a combination of steroids and cytotoxic drugs might prevent evolution to chronic renal failure. CONCLUSION: Despite numerous studies, the pathogeny of Henoch-Sch?nlein nephritis remains incompletely elucidated and controlled therapeutic trials are still needed.     

Glomerular Membranopathy in Children with IgA Nephropathy and Henoch Schönlein Purpura

We evaluated renal biopsies from 34 children with IgA nephropathy or Henoch Schönlein purpura to further characterize the ultrastructural features of the glomerular membranopathy that occurs in these disorders. Focal glomerular basement membrane damage was identified in 29 children and was severe in 4 of the children. Alterations included focal and segmental attenuation, splitting, duplications, and spike-like subepithelial protrusions of the lamina densa, along with saccular glomerular microaneurysms arising at the paramesangium. Those cases with extensive glomerular basement membrane lesions had either moderate or severe glomerular alterations apparent by light microscopy. Over half of the cases with glomerular membranopathy had immunohistological or ultrastructural evidence of focal peripheral glomerular capillary wall immune deposits and electron-dense deposits occurred at sites of glomerular basement membrane splitting. Despite the focal attenuation of the glomerular basement membrane, we did not identify any biopsy with findings of thin basement membrane disease. The glomerular basement membrane ultrastructural findings we describe are characteristic of IgA nephropathy and Henoch Schönlein purpura, are common in children with these disorders, and are similar to the ultrastructural alterations of the basement membrane that occur in other glomerulonephritides. These basement membrane injuries may be inflammatory cell or immune mediated but their pathogenesis requires further study.     

IgA肾病和紫瘢性肾炎循环免疫复合物IgA检测的研究

目的 探讨小儿IgA肾病（IgAN）、过敏性紫瘢肾炎（HSPN）检测血甭循环免疫复合物IgA（IgA－IC）的临床意义。方法 应用胶固素酶联免疫吸附试验（ELISA）对IgAN、HSPN及其他泌尿系统疾病198例检测血清IgA－IC，观察IgA-IC与IgAN病理的关系。结果 IgAN、HSPN组检测IgA－IC与正常对照组比较无显著性差异，各组之间比较也无显著差异。结论 IgAN有HSPN患儿检测IgA－IC与正常对照组及他肾脏疾病组比较无显著差异，IgA－IC对IgAN及HSPN无诊断价值。IgA－IC升高与IgAN的病理分级无关。     

肾活检免疫病理呈“满堂亮”现象临床意义的探讨(附50例病例分析）

目的 探讨肾活检免疫病理“满堂亮”（指肾组织免疫荧光染色IgG、IgA、IgM、C3、C1q同时阳性）的现象与临床肾脏疾病关系。方法 对1984－1999年有完整临床资料及肾活检免疫荧光呈“满堂亮”的50例闰例进行分析。结果 50例“满堂亮”的诊断为：（1）狼疮性肾炎21例,其病理改变Ⅱ型（系膜增生型）4例,Ⅲ型（局灶节段型）5段,Ⅳ型（弥漫增殖型）9例,Ⅴ型（膜型）3例。（2）乙型肝炎病毒相关肾炎13例,其病理为轻系膜增生型4例;膜性肾病7例;膜增生型肾小球肾炎2例。（3）过敏性紫瘢性肾炎（均为肾病型）9例。（4）IgA肾病6例。（5）抗中性粒细胞胞浆抗体阳性的急进性肾小球肾炎1例。结论 （1）“满堂亮”现象可见于多种肾小球疾病,最多见于狼疮性肾炎,是狼疮性肾炎最有特片性的免疫病理改变。（2）“满堂亮”现象也可出现于乙型肝炎病毒相关肾炎。（3）对呈‘满堂亮‘而临床诊断为非狼疮性肾炎的病例应追踪观察,尤应注意其与狼疮性肾炎的密切关系。以便发现些延迟出现狼疮血清学阳性反应的和（或）狼疮临床表现的狼疮患者。     

Serum anti-streptococcal IgA,IgG and IgM antibodies in IgA-associated diseases

Serum anti-streptolysin-O antibody (ASO) and anti-streptococcal polysaccharide antibody (ASP) of IgA, IgG and IgM classes were measured using an enzyme-linked immunosorbent assay in 41 children with IgA nephropathy (Group A), 15 children with uncomplicated anaphylactoid purpura (Group B) and 13 children with purpura nephritis (Group C). The serum concentrations of the IgA, IgG and IgM classes were measured by single radial immunodiffusion. When compared with sex- and age-matched controls, the concentrations of serum IgA (but not of IgG or IgM) were significantly increased in the three groups studied. The titers of ASO of the IgA and IgM classes, and those of ASP of the IgA and IgG classes, were significantly increased in Group A. In Group B, only the ASP titers of the IgA class were significantly increased. No significant difference was noted in the titers of either ASO or ASP of any class in Group C. Thus, increased antibody response in IgA nephropathy is not restricted to IgA. Anaphylactoid purpura with or without renal disease appears to be different in its humoral anti-streptococcal response from IgA nephropathy.     

Mechanism of onset and exacerbation of chronic glomerulonephritis and its treatment

Immunoglobulin A nephropathy (IgAN) is one of the most common causes of chronic glomerulonephritis (CGN) in the world. The proliferative and crescentic forms of IgA are found in up to 30% of cases and are associated with nephritic‐range proteinuria, accelerated hypertension, and accelerated decline toward end‐stage renal disease. On the other hand, Henoch–Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. Two entity diseases are important as renal diseases in childhood. We herein review the mechanism of the onset and exacerbation of IgAN and HSP nephritis (HSPN) and its treatment. As to the pathogenesis, we found that CB4 provoked exacerbation of renal pathologic findings in hyper IgA mice via endothelial injury, γ‐interferon production, and dysfunction of the mesangial pathway and could possibly become one of the factors involved in the mechanism of the onset or evolution of human IgAN. As to the treatment of IgAN and HSPN, we evaluated the efficacy of multidrug combination therapy (prednisolone, warfarin, and dipyridamole, including mizoribine) for diffuse IgAN and the efficacy of methylprednisolone and urokinase pulse therapy plus immunosuppressive drugs for severe HSPN in children. These therapies were effective in ameliorating the proteinuria and histologic severity of patients with IgAN or HSPN. In future, detailed investigations into the pathogenesis of CGN and double‐blind randomized control studies on children with IgAN or HSPN will be necessary.     

Significance of Fibrin/ Fibrinogen Related Antigen in Glomeruli in Children with IgA Nephropathy

The relationship between the deposition of fibrin/fibrinogen related antigen (FRA) and morphologic findings in glomeruli was investigated in 59 children with IgA nephropathy. After treatment of renal biopsy sections with monochloroacetic acid (MCA) solution, MCA-insoluble FRA, suggesting cross-linked fibrin, was observed within the capillary walls, the mesangium and/or the sclerotic area in children with severe proliferative lesions, such as segmental sclerosis, capsular adhesions or crescent formation. Mesangial deposits of FRA were confined to electron-dense deposits, and the MCA-soluble FRA did not seem to be closely related to glomerular damage. FRA deposits within crescents may consist of MCA-soluble FRA.     

肾脏疾病患儿血清免疫球蛋白及其亚类的变化

目的通过对儿童各类肾脏疾病进行血清免疫球蛋白(Ig)及亚类测定,探讨不同肾小球疾病体液免疫功能的变化。方法采用酶联免疫吸附法(ELISA)分别对急性肾炎26例、肾病综合征(NS)30例、紫癜性肾炎(HSPN)28例进行Ig及其亚类测定,20例正常儿童作为对照组。结果NS、急性肾炎、HSPN患儿IgG1和IgG2亚类明显低于正常对照组,差异有显著意义;IgG、IgG3、IgG4降低不明显,而急性肾炎组IgG3明显高于NS组及对照组,差异有显著意义(P<0.05)。结论小儿不同肾小球疾病具有不同的免疫特点,尤其是在总Ig无明显变化的状况下,各亚类的变化在其疾病的发生发展过程中占有重要地位。     

1002例慢性肾脏病患儿临床及病理分析

目的 了解慢性肾脏病(CKD)儿童的临床及病理特点.方法 回顾分析1002例住院CKD患儿的临床及病理资料.结果 1 002例CKD患儿中,男635例,女376例;中位发病年龄7岁;CKD Ⅰ期973例,CKD Ⅱ期4例,CKDⅢ期7例,CKD Ⅳ期6例,CKD V期12例;病因以后天获得性肾小球疾病为主,原发性肾病综...