成人过敏性紫癜性肾炎与IgA肾病临床病理和转归对比分析

目的：分析成人过敏性紫癜性肾炎（HSPN）与IgA肾病（IgAN）临床病理和转归对比。方法选择广西贵港市第二人民医院42例HSPN患者和42例IgAN患者,分析临床病理改变和转归。结果 HSPN组合并肾外症状多于IgAN组;在肾病病理改变中IgAN组出现球形硬化11.9%,HSPN组为4.8%;HSPN组出现内皮增生19.4%;IgAN组为0%;HSPN组肾小管萎缩21.4%,IgAN组则为33.3%,差异有统计学意义（P＜0.05）;在HSPN组中肾小球免疫沉淀物中有IgG的有22例,而在IgAN组中则只有8例。结论 HSPN组与IgAN组在肾脏病例改变和免疫病理分型中明显差异,证实HSPN与IgAN是两种疾病实体。     

老年IgA肾病的临床病理特征和预后分析

目的:探讨老年IgA肾病(IgA nephropathy,IgAN)临床病理特征和预后.方法:选择肾活检确诊的年龄≥60岁的IgA肾病患者70例,与同期肾活检确诊的年龄<60岁的IgA肾病患者82例进行临床、病理及预后对比分析比较.结果:老年组与非老年组相比,收缩压[(142.0±20.4)mmHg vs (124.2±16.9)mmnHg,1 mmHg=0.133 kPa]、舒张压[(83.1 4±11.8)mmUg vs(78.9 4±12.3)millHg]、肾穿时血肌酐水平[(172.7-1±125.8)μmol/L vs(94.4 4±42.5)μmol/L]、血胆固醇[(5.7 4±1.6)mmoL/ vs (5.1±1.6)mmol/L]、24 h尿蛋白定量[(3.4±2.9)g/d vs(1.8 4±2.O)g/d]、高血压的发生率(57.1%vs32.5%)、慢性肾疾病(chronic kidney disease,CKD)分期3～5期的比例(64.O%伪14.6%)差异均有统计学意义(P<0.05).两组的病程、肉眼血尿的发生率、血甘油三酯、血WgA水平差异无统计学意义(P>o.05).病理资料上,老年组肾病理以慢性化病变为主,与非老年组相比,肾小球硬化[(19.7 4±20.1)%vs(13.4 4±17.8)%]、肾小管萎缩(>1分,34.2%vs25.6%)、间质纤维化(>1分,34.2%vs 18.2%)、肾小动脉硬化(>2分,20.O%vs 8.5%)的比例差异均有统计学意义(P<0.05).而系膜细胞增生、新月体比例、间质炎症细胞浸润在两组间差异无统计学意义(P>0.05).平均随访(34.6 4±33.3)个月,老年组3年和5年累计肾生存率显著低于非老年组(74.6%1.78 100%,62.2%vs92.9%,P=0.002).结论:老年组IgA肾病患者在确诊时高血压、肾功能不全、高脂 血症的发生率较高,肾脏病理改变以慢性病变为主,肾小动脉病变明显,这可能是导致老年IgA肾病患者预后差的原因.     

94例小儿IgA肾病分析

目的:评估湖南省湘雅医院、湘雅二医院儿科的94例小儿IgA肾病住院情况和临床、病理特点.方法:对1995～2004年间每年的儿科住院总人数、肾脏病住院人数及其肾活检人数和IgA肾病人数及其病理特征进行比较总结.结果:10年间,住院总人数年均增长率为9.98%(9.98%±4.46%),肾脏病为7.5%(7.5%±4.4%),二者相关系数为0.96(P＜0.001),56.3%的肾脏病患者接受肾活检,约每3～4年增长10%,接受肾穿刺者与肾脏病患儿人数之间的相关系数为0.45(P＜0.05),共发现小儿IgAN病例94例,占肾活检人数8.3%,IgAN患儿与肾活检人数之间相关系数为0.57(P＜0.01).94例IgAN患儿临床表现以血尿为主,占76%.病理特征主要为炎症细胞浸润(91%)、肾小管变性(81%)和肾间质纤维化(31%),尤以肾病综合征型IgAN为明显.结论:10年间随住院总人数、肾脏病住院人数和肾活检人数的增加,小儿IgAN的检出率也相应提高.血尿是小儿IgAN的主要症状,常规尿液分析和对无症状性血尿行常规的肾活检将提高小儿IgAN的检出率.炎症细胞浸润、肾小管变性和肾间质纤维化是IgAN的普遍性病理特征,尤以肾病综合征型IgAN为明显,与持续性蛋白尿的作用有关.早期控制蛋白尿可能会延缓或降低肾小管纤维化的发生.     

IgA肾病及过敏性紫癜性肾炎患儿血清半乳糖缺乏IgA1测定的临床意义

目的分析IgA肾病(IgA nephropathy,IgAN)及过敏性紫癜性肾炎(Henoch-Schnlein purpura nephri-tis,HSPN)患儿中IgA1(Gd-IgA1)水平的临床意义。方法 45例研究对象被分为IgAN组、HSPN组、对照组,每组15例。磁珠分选外周血初始B细胞(CD27-IgD+),加滤泡辅助性T细胞(T follicular helper,Tfh)相关因子共培养,酶联免疫吸附试验(enzyme-linked immunosorbent assay,ELISA)测定细胞培养上清液IgA分泌,散大蜗牛凝集素(helix asper-sa,HHA)ELISA测定半乳糖缺乏的IgA1(Gd-IgA1)水平,同时测定血清中的IgA、Gd-IgA1水平。结果 IgAN、HSPN及对照组细胞培养上清液IgA水平分别为(65.4±11.3)ng/ml、(63.6±9.4)ng/ml、(60.7±12.8)ng/ml;Gd-IgA1水平分别为(91.5±11.5)U/ml、(88.0±10.5)U/ml、(75.7±12.8)U/ml。IgAN、HSPN及对照组血清IgA水平分别为(537.0±100.0)μg/ml、(508.0±95.2)μg/ml、(498.0±85.4)μg/ml;Gd-IgA1水平分别为(2495.0±278.5)U/ml、(2580.0±254.0)U/ml、(1897.0±210.9)U/ml。与对照组相比,IgAN组和HSPN组细胞培养上清液及血清IgA水平无统计学差异(P>0.05);细胞培养上清液及血清Gd-IgA1水平显著升高,差异有统计学意义(P<0.01)。IgAN组及HSPN组细胞培养上清液IgA、Gd-IgA1及血清IgA、Gd-IgA1水平均无统计学差异(P>0.05)。结论Tfh细胞介导Gd-IgA1的生成增加,在IgAN和HSPN发病起作用;血清Gd-IgA1可能是间接反映IgAN和HSPN的无创性指标之一。     

IgA肾病患者血清IgA1对足细胞凋亡的影响

【目的】 探讨IgA肾病患者血清IgA1对足细胞凋亡的影响。 【方法】 Jacalin 亲和层析和Sephacryl S-200 分子筛用来纯化蛋白,单体IgA1(mIgA1)热聚合为聚合体IgA1(aIgA1), 同步化的足细胞与患者和健康对照来源的aIgA1共培养,流式细胞仪评价细胞凋亡情况,Real time PCR 检测Bcl-2, Bax, Fas and Fas-L mRNA表达情况。 【结果】 IgAN患者的aIgA1可诱导足细胞的凋亡,其凋亡率显著高于对照组[(30.5 ± 5.4)% vs (20.6 ± 4.5)%, P < 0.05];而健康对照的aIgA1可诱导足细胞的凋亡,凋亡率也显著高于对照[(31.4 ± 5.3)% vs (20.6 ± 4.5)%,P < 0.05];IgAN患者的aIgA1诱导足细胞的凋亡呈现时间和浓度依赖?与对照相比,IgAN患者和健康对照来源的aIgA1诱导足细胞Fas mRNA 升高2.4倍(P < 0.05),而Bcl-2 mRNA和Bax mRNA无显著性变化(P > 0.05)。【结论】 IgA肾病患者血清IgA1可诱导足细胞的凋亡,其可能参与IgAN的进展。     

血清中IgA/C3检测在IgA肾病诊断中的意义

目的:探讨血清IgA/C3比值在IgA肾病(IgA nephropathy,IgAN)诊断中的价值。方法:选择IgA肾病(IgAN)患者102例,其它非IgA型肾小球肾炎(GN)患者122例,健康人群53例。检测患者及健康人群血清中IgA、补体C3水平,计算IgA/C3比值,并进行ROC分析。结果:IgAN患者血清IgA/C3比值明显高于GN患者及健康人群,但ROC曲线分析显示(AUC=0.668),将血清IgA/C3用于IgAN的诊断预测无较大价值。但当IgA/C3比值≥4.5时,患IgAN的概率大于89%,且77%以上为男性患者。结论 :IgA/C3比值截点不同,对IgAN的诊断预测价值不同,以IgA/C3比值≥2.43时为截点时,无较大预测价值,但在以IgA/C3比值≥4.5时为截点时,具有较强的诊断意义。     

Characteristics of interstitial inflammation in drug-induced tubulointerstitial nephritis and IgA nephropathy

目的 探讨人类药物相关性间质性肾炎(D-IN)和IgA肾病(IgAN)肾小管间质免疫炎症反应特点的异同.方法 观察33例D-IN(急性23例,慢性10例)和32例IgAN患者肾活检组织中肾小管间质病变的特点,应用免疫组化法观察T淋巴细胞和单核/巨噬细胞浸润情况,α-平滑肌肌动蛋白(α-SMA)和骨桥蛋白(OPN)的表达,结合临床资料进行相关分析.结果 两组患者CD3+的T淋巴细胞和CD68+的单核/巨噬细胞均为肾间质的主要浸润细胞.D-IN组急性病例以CD3+细胞为主(CD3+/CD68+为3.7±1.0),慢性病例以CD68+细胞为主(CD3+/CD68+为0.3±0.1,P＜0.05).IgAN组两种细胞在数量上显著相关(r=0.839,P＜ 0.001),比例不随病变程度加重而变化.IgAN组,CD3+与CD68+细胞的浸润程度均与血肌酐、肾间质纤维化以及α-SMA阳性面积呈正相关(r=0.568,0.612;0.703,0.769; 0.597,0.655,P均＜ 0.001).D-IN组,CD3+/CD68+与肾间质纤维化和α-SMA阳性面积呈负相关(r=-0.587、-0.442,P＜0.05).两组肾小管上皮OPN表达均明显增加并与CD68+细胞数量呈正相关(r=0.7604,0.8547,P＜ 0.001).结论 在IgAN中,T淋巴细胞和单核/巨噬细胞可能共同参与肾小管间质的慢性损伤过程;而在D-IN中,单核/巨噬细胞在肾功能恶化和病情慢性化过程中可能起主导作用.     

不同IgA肾病病理评分方法的临床适用性评估

目的 比较不同IgA肾病病理评分方法的相关性,探讨简明半定量评分各病理指标与临床指标的关系.方法 112例IgA肾病患者的病理及临床资料.按Lee氏,Hass氏以及IgA肾病简明半定量病理评分方法(SQM)分别对肾组织切片进行评分.比较3种病理评分方法之间及3种病理评分方法与估算的肾小球滤过率(eCFR)及24h蛋白定量(24h-UPro)间的相关性.根据血肌酐(Scr)以及24h-UPro不同分别将患者分为两组(血肌酐:<1.5mg/dl组,≥1.5mg/dl组;24h-UPro:<30g/L组,≥30g/L组),分别比较两组患者SQM法8个病理指标有无差异.结果 ①Lee氏,Hass氏与SQM法有很好的相关性(P均≤0.001);3种病理评分(SQM,Lee氏与Hass氏)与eGFR值均呈负相关(P均≤0.001),与24h-UPro间无相关关系;②半定量评分系统中,肾间质炎症细胞浸润指数(InfI)、肾小管萎缩和间质纤维化指数(TCI)、小动脉慢性病变指数(VCI)、肾小球慢性病变指数(GCI)及系膜基质增多指数(MsMI)病理改变程度在Scr≥1.5mg/dl水平组积分明显高于Scr<1.5mg/dl组,(P<0.05);而内皮细胞增生指数(endoI)、活动性新月体及节段性袢坏死指数(dCAI)、系膜细胞增生指数(MsHI)病理评分在不同Scr水平两组阃差异无统计学意义(P均>0.05),24h-UPro两组同半定量评分各病理指标严重程度差异无统计学意义(P均>0.c15).结论 3种病理评分方法均能较好地反应IgA肾病的病变严重程度,而SQM法的相关性更好;肾组织病变中,小管间质病变、血管病变以及肾小球慢性化病变与肾功能eGFR的下降有关,而患者蛋白尿的多少尚未发现与肾组织光镜下病理改变之间有必然的关系.     

基质金属蛋白酶-9在IgA肾病足细胞损伤中表达变化的意义

目的 探讨基质金属蛋白酶-9(MMP-9)在IgA肾病患者肾组织足细胞损伤中表达变化的意义.方法 48例经肾穿刺活检明确诊断的IgA肾病,采用免疫组织化学技术定量肾组织足细胞表面特异性标记物Wilms肿瘤蛋白(WT1)和MMP-9的变化.结果 组织学分级Ⅰ～Ⅲ级组IgA肾病患者肾小球MMP-9表达量与足细胞WT1表达量呈正相关(r=0.556,P＜0.05),Ⅳ～Ⅴ级组IgA肾病患者肾小球MMP-9表达量与足细胞WT1呈负相关性(r=-0.773,P＜0.01),IgA肾病患者足细胞WT1表达与血压、尿蛋白无明显相关性,但与血清肌酐水平呈正相关(r=0.756,P＜0.01).MMP-9在正常肾小球少量表达或无表达;随着新月体、增殖性病变加重表达也增加,肾小球MMP-9的表达与血压无相关性,但与尿蛋白呈正相关(r=0.812,P＜0.01),与血清肌酐呈负相关(r=-0.657,P＜0.01).结论 MMP-9的异常表达可能是影响IgA肾病足细胞损伤的因素之一.     

恶性高血压为主要表现的IgA肾病的临床和病理特点分析

目的探讨以恶性高血压（MHT）为主要表现的IgA肾病（IgAN）的临床、病理特点和预后。方法对32例主要表现为MHT的IgAN患者（IgAN-MHT组）的临床、病理资料进行回顾性分析,随访其预后。并与30例无MHT的IgAN患者（IgAN组）进行比较。结果 IgAN-MHT组13例表现为急性肾损伤,8例为肾病综合征,7例为肉眼血尿。随访期内多数病例均表现为进行性肾功能损害。IgAN组4例表现为肾病综合征,3例呈肉眼血尿。随访期内肾功能无明显进展。IgAN-MHT组前驱感染发生率明显高于IgAN组（P〈0.05）,从发病至肾活检的时间明显较IgAN组短（P〈0.01）,血清肌酐、尿酸、C-反应蛋白及尿蛋白水平均较对照组明显升高（P〈0.01）。肾活检病理示IgAN-MHT组肾小球、间质、血管病变明显较IgAN组重,IgAN-MHT组Lee’s分级≥Ⅲ级所占比例明显较IgAN组高（100%比53.3%,P〈0.01）。结论主要表现为MHT的IgAN临床病情及肾脏病变严重,预后差。影响肾功能转归的因素可能包括肾活检前MHT持续的时间、肾活检时血肌酐值、肾脏病变的严重程度和降压治疗是否达标。     

大量蛋白尿表现的儿童IgA肾病和紫癜性肾炎的临床病理比较

目的对比研究临床大量蛋白尿表现的IgA肾病（IgA nephropathy,IgAN）和儿童紫癜性肾炎（HenochSchnlein purpura nephritis,HSPN）的临床和肾脏病理改变的异同。方法收集大量蛋白尿表现的26例IgAN和41例HSPN患儿的临床资料和病理资料并进行回顾性分析。结果大量蛋白尿表现的IgAN患儿和HSPN患儿在年龄、性别方面比较差异无统计学意义（P〉0.05）。与大量蛋白尿的HSPN患儿相比较,大量蛋白尿的IgAN更易出现血尿（P〈0.05）,24h尿蛋白、血胆固醇水平更高,血白蛋白更低（P〈0.05）,临床表现更重。有大量蛋白尿的IgAN患儿和HSPN患儿肾脏免疫复合物沉积比较差异无统计学意义（P〉0.05）,但IgAN患儿较HSPN患儿在肾小球硬化、肾小管炎症细胞浸润、肾小管萎缩和间质纤维化方面更常见（P〈0.05）。结论大量蛋白尿表现的的IgAN比HSPN患儿临床表现重,病理改变更趋慢性化。     

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.     

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.     

不同性别原发性IgA肾病临床病理特征

分析不同性别成人原发性IgA肾病(IgA nephropathy,IgAN)临床病理特征。方法回顾性调查2008年5月至2011年12月374例经肾活检确诊为原发性IgAN患者的临床病理资料,分析不同性别原发性IgAN患者的临床表现和病理特征差异性。结果不同性别原发性IgAN患者的发病年龄、24h尿蛋白定量、血胆固醇、血白蛋白、血IgA、血C3、血糖水平差异无统计学意义(均P>0.05),而男性患者收缩压、舒张压、血肌酐、血尿酸及血三酰甘油水平明显高于女性(均P<0.05);肾小球滤过率(eGFR)显著低于女性(P<0.05)。男女患者在肾小球积分、肾小管积分、肾小动脉积分差异无统计学意义(均P>0.05),但男性患者肾间质积分和总积分指标显著高于女性患者(均P<0.05)。结论不同性别原发性IgAN患者临床和病理特征存在一定差异。性别是导致原发性IgAN临床表现多样性的原因之一。     

伴有新月体形成的IgA肾病的临床特征及预后

目的 探讨伴有新月体形成的IgA肾病的临床特征及预后.方法 分析89例伴新月体形成的IgA肾病患者肾活检时的临床资料,以412例无新月体IgA肾病患者为对照.对其中276例患者进行随访观察,了解患者人、肾存活情况,用非参数乘积限估计法(Kaplan-Meier法)分析肾存活率.结果 新月体形成在IgA肾病中的发生率为17.8%,39例(43.9%)表现为急进性肾炎,明显高于不伴有新月体形成的IgA肾病患者.其肾小球、肾小管间质病变程度均较无新月体组重,并随着其形成范围及纤维性新月体增加而加重.新月体组平均随访(40.3±29.6)月,其1、3、5年肾存活率分别为95.24%、80.95%和61.9%;无新月体组平均随访(45.1±26.9)月,其1、3、5年肾存活率分别为100%、91.67%和79.17%.结论 伴新月体形成的IgA肾病患者临床表现和病理表现较重,预后较差.     

少量蛋白尿IgA肾病患者的临床、病理特点分析

目的:总结少量蛋白尿IgA肾病(IgAN)患者的临床病理特点,探讨影响其肾功能减退的可能因素。方法:对412例少量蛋白尿IgAN患者的临床、实验室及病理表现(包括病理半定量检查)进行回顾性对比分析。结果:5年间少量蛋白尿IgAN患者占全部肾活检病例的10.2%。其中,肾功能正常者占87.1%,肾功能异常者占12.9%。肾功能异常组高血压患病率、蛋白尿和血尿酸水平均显著高于肾功能正常组(P<0.01,P<0.05)。肾功能异常组肾小球硬化程度显著重于肾功能正常组(P<0.01),同时肾小管间质病变及血管病变亦较重(P<0.05)。结论:少量蛋白尿IgAN患者的肾功能及病理损伤亦可能较重。蛋白尿、高血压及高尿酸血症均可能影响患者的预后。临床中应积极行肾穿刺,及早发现有治疗价值的病变。     

小儿IgA肾病30例临床与病理分析

目的　了解小儿 Ig A肾病 ( Ig A nephropathy,Ig AN)临床、病理及与预后的关系。方法　对肾活检病理诊断为 Ig AN的 3 0例病例进行临床与病理分析。结果　小儿 Ig AN临床表现以单纯肉眼血尿最常见 ,其与血尿伴蛋白尿者免疫复合物沉积均多为 Ig A+Ig G型 ,但前者临床及病理改变相对轻于后者。肾病综合征 ( NS)的临床及病理改变最重 ,易合并肾功能不全、高血压 ,以 Ig A+Ig G+Ig M型的免疫复合物沉积为主。结论　小儿 Ig AN预后与临床及病理分级有关 ,单纯肉眼血尿预后较好 ,NS、病理改变有肾小球硬化及免疫复合物沉积为 Ig A+Ig G+Ig M型者预后不良。     

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     

活动性肾小球肾炎中过氧化脂质体增殖激活受体γ在肾脏的表达

目的 :研究过氧化脂质体增殖激活受体γ(PPAR γ)在人肾小球肾炎病变活动时肾组织中的表达变化。方法 :根据临床及肾活检病理诊断 ,选择炎症病变明显活动的病人 ,细胞性新月体肾小球肾炎 (RPGN) 17例、狼疮性肾炎 (Ⅳ型 ) (LN) 15例 ,并以炎症病变轻微的轻度系膜增生性IgA肾病 (IgAN) 7例和无炎症病变肾小球微小病变 (MCD) 10例为对照。分别收集各组病人临床资料 ,光镜下观察并计数肾脏病变活动指数 ;免疫组化和原位杂交方法观察肾组织PPAR γ蛋白和mRNA表达。结果 :LN和RPGN组病人临床上呈明显病变活动表现 ,肾脏病理活动指数显著高于IgAN和MCD组。肾组织PPAR γ免疫组化显示PPAR γ在MCD组未见表达 ;在IgAN组肾小球和肾小管少量表达 ;在病变活动的RPGN和LN组肾小球和肾小管表达显著上调。相关分析结果显示 ,在病变活动性指数与肾小球和肾小管PPAR γ染色阳性细胞数间呈显著相关性 (相关系数分别为 0 .4 78,P <0 .0 1;0 .5 13,P<0 .0 1)。原位杂交方法也进一步证实LN和RPGN组肾小管上皮细胞PPAR γmRNA表达较IgAN和MCD组显著上调。结论 :活动性肾小球肾炎病人的肾组织PPAR γ表达上调 ,这可能是肾组织对炎症应激反应的一种表现 ,并预示PPAR γ可能在肾小球炎症过程中发挥重要作用