IgA型感染后肾小球肾炎的临床病理与预后研究

目的观察IgA型感染后肾小球肾炎(PIGN)的临床与病理特征,及其与金黄色葡萄球菌感染及潜在糖尿病的相关性。方法回顾性分析IgA型PIGN患者25例,均进行肾组织活检,分析其在光镜病理、免疫荧光显微镜和电子显微镜下变化特点,总结其临床结局。结果所有患者在发病时均为临床金黄色葡萄球菌感染其中MSSA感染11例,MRSA感染14例。肾活检显示:急性期PIGN 6例,表现为弥漫性增生性肾小球肾炎,恢复期或亚急性期显示系膜细胞增生。免疫荧光显示IgA沉积。超微结构分析表明局灶性上皮下驼峰状电子致密物沉积。25例均使用抗生素治疗12例联合应用甲泼尼龙治疗,血液透析依赖8例。其中16例出院,完全缓解6例,部分缓解10例;9例持续住院,死亡5例。结论 IgA型与金黄色葡萄球菌感染有关。诊断主要依靠IgA的免疫荧光染色和存在毛细血管内增殖,电镜检查示上皮下电子致密沉积。     

7例原发膜性肾病合并IgA肾病的临床与病理特点分析

目的 探讨膜性肾病合并IgA肾病患者的临床与病理特点。方法 回顾分析天津市第一中心医院肾内科2012年1月—2018年2月经肾穿刺活检确诊的7例膜性肾病合并IgA肾病患者的临床和病理特点。结果 男性4例,女性3例;年龄28～63岁,平均44.1岁;5例为双下肢浮肿,1例为尿中泡沫增多,1例以尿蛋白增多为主要表现;其中4例临床诊断为肾病综合征,3例诊断为慢性肾小球肾炎,仅1例有高血压病史,6例伴有镜下血尿,7例均有蛋白尿,肾功能均正常。肝炎、自身免疫性疾病、肿瘤等相关检查均为阴性。光光学显微镜下可见肾小球基底膜均弥漫增厚,6例伴有较明显的钉突形成,系膜细胞及系膜基质轻度增生,肾小管及肾间质未见明显病变。电子显微镜下7例毛细血管襻上皮下均见大小不等块状电子致密物沉积,6例可见节段钉突形成,系膜区及系膜旁区均可见少量中度电子致密物沉积。免疫荧光见IgG均沿毛细血管襻颗粒状沉积,IgA系膜区团块状及分支状沉积。免疫组织化学见C4d均沿毛细血管襻颗粒状沉积。结论 膜性肾病合并IgA肾病发病率较低,临床以蛋白尿及镜下血尿为主要表现,发病时肌酐大部分正常。光学显微镜下以膜性肾病特点为主,肾小管及肾间质病变轻微,免疫荧光IgA是诊断该病的必要条件,C4d是诊断该病的重要标志物;治疗尚无统一标准,需要更多病例的进一步研究。     

肾病综合征表现的急性肾小球肾炎鉴别

目的研究和分析肾病综合征表现的急性肾小球肾炎鉴别方式。方法共计24例急性肾小球肾炎误诊为肾病综合征患者,通过行病理学检查进行鉴别诊断,包括：局麻,B超下行肾穿刺,标本固定、切片,通过HE、六胺银、Masson和免疫荧光染色进行疾病分析。结果 HE染色：肾小球内增生的细胞主要为系膜细胞和内皮细胞。免疫荧光检查可见沿毛细血管壁和系膜区有弥漫粗颗粒免疫复合物沉积其中主要成分是IgG和C3,IgA和IgM少见。电镜检查可见上皮细胞下有＂驼峰状＂电子致密物沉积。PSGN病理改变呈自限性。结论表现为肾病综合征的急性肾小球肾炎：蛋白尿明显的急性肾炎可出现低蛋白血症、高脂血症和凹陷性水肿。通过尿检动态观察、血清补体检测及病理学检查可与肾病综合征相鉴别。     

肾功能正常的抗肾小球基底膜病临床分析

目的 了解肾功能正常的抗肾小球基底膜(GBM)病的临床和病理特点.方法 回顾性分析6例长期保持肾功能正常的抗GBM病患者的临床病理特点,并与同期伴肾功能损害的29例抗GBM病患者进行比较,分析影响预后的因素.结果 35例患者中6例(17.1％)肾功能一直保持正常.5例患者有不同程度肾脏受累,1例仅有肺出血,3例表现为肺肾出血综合征.6例均行肾活检病理检查,免疫荧光:4例表现为IgG2+ ～3+沿肾小球毛细血管襻呈线样沉积;光镜差异较大,从肾小球轻微病变到新月体肾炎伴大量毛细血管襻坏死,但大多数患者病变轻微.5例患者给予免疫抑制和(或)血浆置换治疗.6例肾功能正常者与同期肾功能异常的29例抗GBM病相比,贫血轻[血红蛋白(99.67±19.80)比(77.97±20.62) g/L,P＝0.024]、抗GBM抗体滴度低[(80.23±85.73)比(224.34±145.79)EU/ml,P＝0.027]、肾小球新月体比例低(0.17±0.27比0.58±0.29,p＝0.005).平均随访12～133个月,6例肾功能正常者4例完全治愈,2例遗留少量蛋白尿、镜下血尿.结论 肾功能正常的抗GBM病并不少见,多数患者肾脏临床病理轻,预后良好.     

纤维样肾小球病病理分析

目的：光镜及电镜下观察纤维样肾小球病的病理特征,为纤维样肾小球病的诊断及鉴别诊断提供依据。方法：2例纤维样肾小球病患者（其中1例并发免疫触须样肾小球病）经皮肾穿刺取活检组织,HE、PAS、PAM-Masson染色,IgG、IgM、IgA、C3、C4、C1q免疫组化染色,于光镜下观察;病理分析（光镜和电镜）及免疫组织化学染色;铀铅双重染色透射电镜观察。结果：光镜下PAM-Masson染色膜性肾病示系膜区团块状、肾小球基底膜细小嗜复红蛋白沉积,而膜增殖性肾小球肾炎示肾小球基底膜局灶增厚、“双轨化”伴嗜复红蛋白沉积。免疫组织化学染色膜性肾病和膜增殖性肾小球肾炎在肾小球系膜区有IgG、IgM、IgA、C3免疫复合物沉积,而在血管襻区膜性肾病见IgG、C3免疫复合物沉积,膜增殖性肾小球肾炎可见IgM、C3、C1q免疫复合物沉积,但其刚果红染色阴性。电镜下膜性肾病和膜增殖性肾小球肾炎在系膜区、基底膜上皮下和（或）内皮下有电子致密物沉积,见无分支的纤维样结构（直径15~25 nm）,而膜增殖性肾小球肾炎还可见微管状结构（直径30~50 nm）。结论：纤维样肾小球病具有典型的形态学特征,确诊需依赖电镜特征性纤维样结构。     

水痘病毒感染伴肾小球肾炎和脑炎1例报告

患者15岁,男性,因水痘、肾炎和肾病综合征、肾功能损伤、补体C3下降2个月而入北京中日友好医院肾内科治疗,肾活检证实为毛细血管内增生性肾小球肾炎伴足细胞增生和肾小管损伤,直接免疫荧光显示IgG、IgA、IgM、C3、C1q和纤维蛋白原沿肾小球毛细血管壁和系膜区颗粒状和团块状沉积,透射电子显微镜检查显示电子致密物在肾小球多部位沉积,血清中抗水痘病毒抗体(IgM)阳性,免疫组织化学和原位杂交显示水痘病毒抗原和mRNA存在于肾小球和肾小管上皮细胞,特殊染色和透射电子显微镜显示病毒颗粒和病毒包涵体存在于肾小球和肾小管上皮细胞内。患者住院期间尚有癫痫发作,脑电图及核磁共振证实为病毒性脑炎导致的癫痫,故确诊为水痘病毒感染伴发肾小球肾炎和脑炎。这是1例通过分子病理学方法证实水痘病毒在肾内感染导致的肾小球肾炎。     

浅谈急进性肾小球肾炎的护理

急进性肾小球肾炎(RPGN)简称急进性肾炎,是一组临床综合征,表现为起病急,进展迅速,由少尿、蛋白尿、血尿迅速进展为急性肾功能衰竭,预后恶劣.急进性肾小球肾炎的基本发病机制为免疫反应,根据免疫病理表现不同可分为三型.I型为抗肾小球基膜型,系抗肾小球基膜抗体与肾小球基膜抗原结合,激活补体而致病;Ⅱ型为免疫复合物型,系循环免疫复合物沉积于或原位免疫复合物种植于肾小球,激活补体而致病,该型发病前常有上呼吸道感染史,其致病抗原可能为细菌或病毒;Ⅲ型为非免疫复合物型,其发生可能与肾微血管炎有关,病人血清抗中性粒细胞胞浆抗体(ANCA)常呈阳性[1].     

急进性肾小球肾炎动物模型研究进展

急进性肾小球肾炎根据免疫病理特征分为Ⅰ型抗GBM抗体型、Ⅱ型免疫复合物型和Ⅲ型微量免疫复合物型,代表性的动物模型分别是实验性自体免疫肾小球肾炎、肾毒血清肾炎和实验性自体免疫脉管炎.本文就这些模型的造模方法、免疫机制和应用范围进行综述,以供读者参考.     

氯化汞诱导大白兔双相自身免疫性肾小球肾炎

本实验采用日本纯种大耳白免、给予小剂量氯化汞反复肌肉注射,成功地制成双相自身免疫性肾小球肾炎模型,即免疫荧光可见IgG沿肾小球基膜(GBM)呈线样和颗粒状沉积两时相。病理改变为类狼疮性肾炎及膜性肾病二种组织学改变。该模型为进一步研究人类慢性汞中毒所致的自身免疫性肾损伤有重要的现实意义。     

肾小球肾炎肾组织中HBV抗原的表达及意义

目的观察肾小球肾炎患者肾组织中乙型肝炎病毒(HBV)表达情况,探讨肾组织HBV抗原的来源及其与组织病变的关系。方法应用于免疫组化(S-P)法检测肾炎肾组织中的HBV抗原(HBsAg、HBcAg)。5例膜性肾病作透射电镜观察。结果血清HBV感染标志阴性肾炎与阳性者的肾组织中HBV抗原沉积一致,且肾小管HBcAg高表达。肾小管HBcAg阳性组的肾组织病变程度明显严重于阴性组,差异有显著性(P<0.05)。电镜下,血清HBV标志阴性肾炎与阳性者表现不同。结论肾组织中HBV抗原抗体免疫复合物有原位形成的可能;血清HBV感染与肾炎〔膜性肾病(MGN)、膜增生性肾小球肾炎(MPGN)、系膜增生性肾小球肾炎(MsPGN)、IgA肾病(IgAN)〕肾组织感染HBV不一致。肾小管中HBcAg可能与上述肾炎的肾组织病变程度有关。     

肾穿刺的病理分析及免疫学模式探讨

用光学、免疫荧光及透射电子显微镜对72例肾炎患者进行了肾穿刺的前瞻性研究。结果发现肾小球毛细血管丛系膜细胞不同程度的增生;用电镜观察,见上皮细胞足突融合是多数肾炎的非特异性病理特征。在相同病因和机理支配下产生的沉积物既可以是线型的,也可以是颗粒状的。因此,免疫荧光线型沉积不仅是在抗基底膜型肾小球肾炎中出现,而且可在其他型的肾小球肾炎中查见。     

实验性家兔慢性肾小球肾炎——免疫荧光、光镜和电镜研究

本文采用静脉注射小牛结晶性血清白蛋白(由小剂量逐渐增大),用弗氏佐剂作辅助免疫,诱发家兔慢性肾小球肾炎,结果免疫荧光显示肾小球系膜区及毛细血管丛基底膜逐渐出现强度不等的IgG颗粒状沉积;光镜下,系膜细胞呈进行增生伴有系膜基质增多和局限性肾小球硬化;电镜下见肾小球毛细血管丛系膜区及基底膜上皮侧和内皮下电子致密物沉积。上述病变类似人类系膜增生性肾炎,进一步说明循环免疫复合物沉着可引起多种类型慢性肾炎。     

抗基底膜性肾小球肾炎的实验研究

对肾毒性血清诱发的大白鼠抗基底膜性肾小球肾炎进行动态观察。光镜下肾小球内细胞数目明显增多。12h内兔抗鼠IgG呈线型分布于鼠肾小球基底膜上。鼠抗兔IgG抗体在7天内产生并沉积于肾脏。电镜下肾小球病变在3周内逐渐加重,并可见电子致密物沉积于肾小球基底膜与内皮细胞之间或肾小球基底膜内。鼠的抗基底膜性肾小球肾炎诱发率高,其病变与人类某些肾炎相似。     

乙肝病毒感染后肾小球肾炎的病理变化

本文对肾小球内有HBsAg沉积的27例各型肾炎的肾组织进行病理分析,其中膜性肾炎8例,膜增殖性肾炎3例,系膜增殖性肾炎13例,局灶硬化性肾炎3例,在各种病理类型的肾炎肾小球中都有不同程度的IgG、IgA、IgM、C_3呈粗大颗粒状沉积,以IgG的荧光强度最大,其轻链以K链为主。通过对沉积在肾小球内凝血纤溶因子的测定,证实其中11例存在高凝状态。透射电镜观察到部分病例肾小球的上皮下、内皮下系膜区有直径在30 nm～70 nm的病毒颗粒。     

Childhood Henoch-Sch?nlein purpura nephritis and IgA nephropathy: one disease entity?--A clinico-pathologically comparative study.

In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Sch?nlein purpura nephritis (HSPN), 31 children with IgA nephropathy aged between 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were compared with each other in clinical manifestation, blood biochemistry, serum immunology and follow-up study. Renal pathological findings under light microscope, immunofluorescence and electronic microscope were analyzed and also compared between 31 children with IgA nephropathy and 32 biopsied children with HSPN. The results showed that the onset age was over 12 years in 25.8% children with IgA nephropathy, but only 10% in HSPN (P < 0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were more often in HSPN, all of them had skin purpura, 59% had gastrointestinal symptoms and 47% suffered from arthralgia, compared with only abdominal pain in 3.2% children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5% of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9% of IgA nephropathy and 6.3% of HSPN, but endothelial proliferation in 65.6% of HSPN and 29% of IgA nephropathy (all P < 0.01). Thin basement membrane nephropathy was only found in 6.5% children with IgA nephropathy, no in HSPN. The electronic dense deposits in HSPN were sparse, loose and wildly spread in glomerular mesangium, subendothelial area and even intra basement membrane, but it was dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. Predominant IgA deposits were found in 81.2% of HSPN, and overwhelming IgG deposits in 12.5% of HSPN with relatively weak IgA deposits, moreover 6.3% of HSPN showed linear IgG deposits in glomerular capillary. Totally 71.9% of HSPN had IgG deposits in glomeruli and only 19.4% of IgA nephropathy showed glomerular IgG deposits (P < 0.01). No IgG deposit was observed in 81.6% of IgA nephropathy, among them most showed IgA and IgM and/or C3 deposits, moreover overwhelming IgG deposits and linear IgG deposits couldn't be found in IgA nephropathy. Mean 20 months follow-up showed complete remission in 72.5% of HSPN, but only 19.4% in IgA nephropathy after 34 months follow-up. Moreover, 64.5% of IgA nephropathy had consistent hematuria and proteinuria and 16.1% had active nephritides (P < 0.05). It was concluded that significant clinico-pathological difference was found between HSPN and IgA nephropathy, which didn't support the one disease entity hypothesis. HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities.     

实验性IgA肾小球肾炎——抗原的电荷在肾炎发病机理中的意义

本文选用阳、阴离子化右旋糖酐作抗原,分别诱发小鼠IgA肾小球肾炎。用显微分光光度计测定肾小球内IgA荧光强度,并结合形态学观察。结果显示阳离子组荧光强度显著高于阴离子组(P<0.01)。电镜下,两实验组均呈现肾小球系膜基质增生、细胞轻度增生及基质内电子致密物沉积;阳离子组基底膜增厚,其上沉积物及足突融合更为明显。显示抗原所带电荷可影响肾炎病变程度及其定位,而阳离子化比阴离子化物质对肾小球肾炎病变形态发生的影响更为显著。且阳离子化右旋糖酐引起的IgA肾炎属于原位免疫复合物性肾小球肾炎。     

Childhood Henoch-Schönlein purpura nephritis and IgA nephropathy: one disease entity?

Summary In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Sch?nlein purpura nephritis (HSPN), 31 children with IgA nephropathy aged between 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were compared with each other in clinical manifestation, blood biochemistry, serum immunology and followup study. Renal pathological findings under light microscope, immunofluorescence and electronic microscope were analyzed and also compared between 31 children with IgA nephropathy and 32 biopsied children with HSPN. The results showed that the onset age was over 12 years in 25.8% children with IgA nephropathy, but only 10% in HSPN (P<0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were more often in HSPN, all of them had skin purpura, 59% had gastrointestinal symptoms and 47% suffered from arthralgia, compared with only abdominal pain in 3.2% children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5% of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9% of IgA nephropathy and 6.3% of HSPN, but endothelial proliferation in 65.6% of HSPN and 29% of IgA nephropathy (allP<0.01). Thin basement membrane nephropathy was only found in 6.5% children with IgA nephropathy, no in HSPN. The electronic dense deposits in HSPN were sparse, loose and wildly spread in glomerular mesangium, subendothelial area and even intra basement membrane, but it was dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. Predominant IgA deposits were found in 81.2% of HSPN, and overwhelming IgG deposits in 12.5% of HSPN with relatively weak IgA deposits, moreover 6.3% of HSPN showed linear IgG deposits in glomerular capillary. Totally 71.9% of HSPN had IgG deposits in glomeruli and only 19.4% of IgA nephropathy showed glomerular IgG deposits (P<0.01). No IgG deposit was observed in 81.6% of IgA nephropathy, among them most showed IgA and IgM and/or C3 deposits, moreover overwhelming IgG deposits and linear IgG deposits couldn't be found in IgA nephropathy. Mean 20 months follow-up showed complete remission in 72.5% of HSPN, but only 19.4% in IgA nephropathy after 34 months follow-up. Moreover, 64.5% of IgA nephropathy had consistent hematuria and proteinuria and 16.1% had active nephritides (P<0.05). It was concluded that significant clinico-pathological difference was found between HSPN and IgA nephropathy, which didn't support the one disease entity hypothesis. HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities. ZHOU Jianhua, male, born in 1964, Professor     

Childhood Henoch-Sch(o)nlein Purpura Nephritis and IgA Nephropathy:One Disease Entity?--A Clinico-pathologically Comparative Study

In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schonlein purpura nephritis (HSPN), 31 children with IgA nephropathy aged between 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were compared with each other in clinical manifestation, blood biochemistry, serum immunology and followup study. Renal pathological findings under light microscope, immunofluorescence and electronic microscope were analyzed and also compared between 31 children with IgA nephropathy and 32 biopsied children with HSPN. The results showed that the onset age was over 12 years in 25.8 %children with IgA nephropathy, but only 10 % in HSPN (P＜0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were more often in HSPN, all of them had skin purpura, 59 % had gastrointestinal symptoms and 47 % suffered from arthralgia,compared with only abdominal pain in 3.2 % children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5 % of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9 % of IgA nephropathy and 6.3 % of HSPN, but endothelial proliferation in 65.6% of HSPN and 29 % of IgA nephropathy (all P＜0.01). Thin basement membrane nephropathy was only found in 6.5 % children with IgA nephropathy, no in HSPN. The electronic dense deposits in HSPN were sparse, loose and wildly spread in glomerular mesangium, subendothelial area and even intra basement membrane, but it was dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. Predominant IgA deposits were found in 81.2 %of HSPN, and overwhelming IgG deposits in 12.5 % of HSPN with relatively weak IgA deposits,moreover 6.3 % of HSPN showed linear IgG deposits in glomerular capillary. Totally 71.9 % of HSPN had IgG deposits in glomeruli and only 19.4 % of IgA nephropathy showed glomerular IgG deposits (P＜0.01). No IgG deposit was observed in 81.6 % of IgA nephropathy, among them most showed IgA and IgM and/or C3 deposits, moreover overwhelming IgG deposits and linear IgG deposits couldn't be found in IgA nephropathy. Mean 20 months follow-up showed complete remission in 72.5 % of HSPN, but only 19.4 % in IgA nephropathy after 34 months follow-up. Moreover, 64.5 % of IgA nephropathy had consistent hematuria and proteinuria and 16.1% had active nephritides (P＜0.05). It was concluded that significant clinico-pathological difference was found between HSPN and IgA nephropathy, which didn't support the one disease entity hypothesis.HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities.     

The clinical and pathological characteristics of Chinese patients with pauci-immune crescent golmerulonephritis

Objective　To investigate the clinical and pathological characteristics of pauci-immune crescent glomerulo～nephritis (PICGN) in Chinese patients. Methods　During 13 years (1985-1998), 6400 patients underwent non-transplanting renal biopsy. Twenty-four patients were diagnosed as PICGN. All clinical and laboratory data of these patients were collected from the patients’ records and used for detailed analysis. The diagnosis is based on clinico-pathologic findings. Results　Of the 24 patients, 16 were females and 8 were males, with median age of 33 years (ranged 10-76 years). Microscopic polyarteritis (MPA) (33.3%) and systemic vasculitis (8.3%) were the secondary diseases. The incidence of PICGN was 0.38% in renal biopsies and 22.9% in crescentic glomerulonephritis. Clinically, most patients (75.0%) showed rapidly progressive nephritis with enlarged kidneys. At onset, gross hematuria was noted in 58.3% of patients, hypertension in 45.8%, nephrotic syndrome in 41.7%, and oliguria in 25.0%. However, systemic symptoms were rare except for anemia. Pathologically, necrosis of glomerular capillaries (62.5%), infiltration of monocytes and neutrophil cells in glomeruli (66.7%), and vasculitis in the interstitium (53.3%) were observed. In addition, glomerulosclerosis was noted in 45.8%, severe tubular atrophy in 83.3% and interstitial fibrosis in 75.0%. Anti-neutrophil cytoplasmic antibodies (ANCAs) were positive in 52.2%. All patients except two received intensively immunosuppressive therapy. Sixteen patients were available for long-term follow up (median 29.8 months, range 8-92 months). Twelve of them had life-sustaining renal function, four had normal serum creatinine (&lt;124?μmol/L) and only 4 patients were dialysis-dependent. Conclusion　PICGN is not rare in China. Early diagnosis and administration of immunosuppressive therapy, particularly in patients with rapidly progressive glomerulonephritis (RPGN), are important for good prognosis.     

实验性抗基底膜肾小球肾炎——免疫荧光、光镜和电镜研究

采用自制兔抗大鼠肾免疫血清,制造大鼠抗基底膜肾小球肾炎模型,并进行了重复试验。用免疫荧光技术、光镜和电镜进行观察,前后两批实验结果相同,提示在肾小球基底膜上显示出典型的光滑线型荧光图象,在电镜下肾小球毛细血管丛基底膜呈现局灶性电子透明区而疏松增厚,光镜下主要表现为增生性肾小球肾炎变化,未见新月体形成。实验结果可与人类某些肾炎相比较,对了解人类某些类型肾炎的病因、发病机理、病理变化、临床表现及其防治有一定意义。