7岁以下小儿肾小球疾病病理特点与临床研究

目的探究7岁以下小儿肾小球疾病的病理特点与临床研究。方法在医院临床儿科2016年2月至2019年2月诊断为10种肾小球疾病患儿中选取68例,并对这68例患儿进行肾组织相关的病理检查。通过对患儿进行经皮肾活检术,对患儿穿刺后提取的肾组织分为三个部分,并对选取的所有患儿进行免疫荧光、光镜以及电镜等检查,其标本分别包括PASM、HE、Masson染色及PAS,同时都利用免疫荧光来进行检测患儿肾组织里的IgA、IgG、IgM、Firibn、C3以及C4,有些患儿则依据血化验乙肝抗原性添加免疫荧光来进行检测其肾组织里的HBeAg、HBcAg以及HBsAg。其中有63患儿接受了电镜检查。结果 68例患儿的主要临床表现特点所占比例分别为:紫癜性肾炎(25.00%)、孤立性血尿(10.29%)、肾病综合征(41.18%)、孤立性蛋白尿(4.41%)、遗传性肾病(4.41%)、乙肝肾(1.47%)、急性肾炎综合征(7.35%)、慢性肾炎(1.47%)、狼疮性肾炎(1.47%)、急进性肾炎(2.94%);患儿病理类型分别为:C3肾病(2.94%)、系膜增生性肾脏肾炎(7.35%)、微小病变(44.12%)、IgM肾病(4.41%)、IgA肾病(7.35%)、局灶节段性肾小球硬化(7.35%)、Alport综合征(4.41%)、薄基底膜肾病(1.47%)、肾淀粉样变(1.47%)、新月体性肾炎(4.41%)、肾型过敏性紫癜(继发性IgA肾病)(14.71%)等。结论在临床中,临床疾病表现相似但是其病理类型却并不相同,同时相同的疾病病理类型,其临床表现多种多样。对7岁以下的患儿利用肾活检术进行肾小球疾病的估计预后、诊断以及治疗都占据着相当重要的地位,同时在进行肾病理检查的过程中利用电镜检查也是非常重要的。     

儿童单纯性血尿105例病理分析

目的 探讨小儿单纯性血尿的病理类型.方法 对105例符合单纯性血尿诊断标准的患儿行肾活检术,肾组织进行光镜、电镜及免疫荧光检查.结果 薄基底膜肾病36例(34.3%),IgA肾病35例(33.3%),系膜增生性肾小球肾炎17例(16.2%),轻微病变7例(6.7%),Alport综合征6例(5.7%),局灶节段性硬化2例(1.9%),IgM肾病2例(1.9%).结论 遗传性肾小球基底膜病占小儿单纯性血尿的第一位,但有明确家族史者不足50%.IgA肾病也是小儿单纯性血尿的主要原因之一,表现为单纯性血尿的IgA肾病病理变化相对较轻.部分单纯性血尿患儿肾小球未见明显病变.少数严重肾小球病变临床也可表现为单纯性血尿.     

小儿肾小球疾病肾活检110例临床分析

目的探讨小儿肾小球疾病的病理特点及其与临床表现的关系.方法对110例符合肾小球疾病诊断标准的小儿行肾活检术,肾组织送光镜、电镜及免疫荧光检查.结果 110例肾组织病理改变为:IgA肾病32例(29.1%);系膜增生21例(19.1%);薄基底膜肾病18例(16.4%);膜增生性肾炎、Alport综合征及微小病变各6例(5.5%);局灶增生和轻微病变各4例(3.6%);新月体肾炎及增生硬化性肾炎各3例(2.7%);局灶节段性硬化、膜性肾病和IgM肾病各2例(1.8%);毛细血管内皮增生1例(0.9%).结论相同的临床诊断,其病理表现可不同;相同的病理诊断,也可有不同的临床表现.     

儿童单纯性血尿37例临床和病理分析

目的 探讨小儿单纯性血尿的病理类型.方法 对37例临床诊断为单纯性血尿的患儿进行肾组织活检,并进行光镜、电镜及免疫荧光检查.结果 系膜增生性肾小球肾炎13倒,肾小球轻微病变9例,IgA肾病8例,IgM肾病4例,局灶节段性肾小球硬化症3例.结论 本组资料单纯性血尿最常见的病理类型是系膜增生性肾小球肾炎,其次是肾小球轻微病变及IgA肾病.     

67例儿童单纯性血尿的肾脏病理及临床分析

目的 探讨儿童单纯性血尿的病理类型.方法 对67例临床诊断为单纯性血尿的患儿进行肾组织活检,并行光镜、电镜和免疫荧光检查.结果 本组单纯性血尿肾脏病理类型最常见的依次为IgA肾病22例(32.8%)、系膜增生性肾小球肾炎17例(25.3%)和毛细血管内增生性肾小球肾炎8例(11.9%).结论 本组资料单纯性血尿最常见的病理类型是IgA肾病,其次是系膜增生性肾小球肾炎、毛细血管内增生性肾小球肾炎.     

江西省儿童单纯性血尿的肾脏病理分析

目的 探讨江西省儿童单纯性血尿的病理类型.方法 对159例单纯性血尿的患儿进行肾组织活检,并行光学显微镜、电子显微镜和免疫荧光检查.结果 单纯性血尿肾脏病理类型最常见的依次为轻度系膜增生性肾小球肾炎69例(43.4%)、IgA肾病36例(22.6%)和局灶节段性肾小球肾炎24例(15.1%),其次为轻微病变17例(10.7%)、局灶节段性肾小球硬化4例(2.5%)、微小病变1例(0.6%)、膜性肾病1例(0.6%)、新月体肾炎1例(0.6%)、膜增殖性肾炎1例(0.6%)和IgM肾病1例(0.6%).结论 单纯性血尿最常见的病理类型是轻度系膜增生性肾小球肾炎,其次是IgA肾病及局灶阶段性肾小球肾炎.     

326例经皮肾活检临床与病理分析

目的:回顾总结本院2010年9月至2012年4月进行肾穿刺活检的标本,分析肾脏疾病经皮肾活检的病理类型构成比例。方法:参照WHO肾小球疾病的组织学分型方案,通过光镜及免疫荧光检测明确病理诊断。结果:326例肾活检病人中,原发性肾小球疾病258例占首位(79.14%),继发性肾小球疾病39例占11.96%,其它占8.90%。原发性肾小球疾病病理类型中最多的是IgA肾病(43.41%),其次为微小病变肾病(25.97%)和膜性肾病(13.18%)。继发性肾小球疾病中狼疮性肾炎占首位(41.03%),其次为紫癜性肾炎(35.89%)。结论:原发性肾小球疾病为常见的肾小球疾病,其中以IgA肾病最为常见;继发性肾小球疾病中以狼疮性肾炎最多见。随着肾活检的广泛开展,其它少见的肾脏疾病也有检出。     

2型糖尿病并发肾脏损害19例临床与病理分析

目的 探讨2型糖尿病患者并发肾脏损害的临床与病理特点,并指出肾脏活检在2型糖尿病患者并发肾脏损害中的诊断与治疗价值.方法 分析我院行肾活检的19例2型糖尿病患者并发肾脏损害的临床表现与病理分型.结果 19例患者中,糖尿病肾脏损害病(DKD)6例(占31.6%),非糖尿病性肾脏疾病13例(占68.4%),其中包括各种继发性肾脏疾病,病理类型分布分别是IgA肾病Lee'sⅡ级3例(占23.1%),IgA肾病Lee'sⅢ级1例(占10%),系膜增生性肾小球肾炎3例(占23.1%),膜性肾病3例(占23.1%),其中2例为乙肝病毒相关性肾炎,1例为狼疮性肾炎,局灶阶段性肾小球硬化症1例(占7.69%),肾淀粉样变性病1例(占7.69%),膜增生性肾炎1例(占7.69%).结论 2型糖尿病肾病并发肾脏损害相当部分是非糖尿病性肾脏病变,当临床有非糖尿病肾病线索时,应及早行肾活检.     

2型糖尿病并发非糖尿病肾损害26例临床与病理分析

目的:探讨2型糖尿病患者并发非糖尿病性肾损害患者的临床与病理特点,并指出肾活检在2型糖尿病患者并发肾脏损害中的诊断及治疗中的价值。方法:回顾性分析我院行肾活检的42例2型糖尿病患者并发肾损害患者的临床表现、病理分型。结果:42例糖尿病合并肾损害的患者中,糖尿病肾病(DN)16例(占34.8%),非糖尿病性肾脏疾病26例(占56.5%),其中包括各种继发性肾脏疾病,病理类型分布分别是IgA肾病Lee’sⅡ级6例(占23.1%),IgA肾病Lee’sⅢ级3例(占11.5%),系膜增生性肾小球肾炎5例(占19.2%),膜性肾病4例(占15.4%),其中1例为乙肝病毒相关性肾炎,1例为狼疮性肾炎,免疫荧光表现为"满堂亮",局灶阶段性肾小球硬化症2例(占7.7%),肾淀粉样变性病1例(占3.9%),骨髓瘤肾病1例(占3.9%),膜增生性肾炎2例(占7.7%),其中1例为丙肝病毒相关性肾炎,紫癜性肾炎1例(占3.9%),慢性间质性肾损害1例(占3.9%)。结论:2型糖尿病肾病并发肾脏损害相当部分是非糖尿病性肾脏病变,当临床有非糖尿病肾病线索时,应及早行肾活检。     

临床诊断为原发肾病综合征的病理研究

目的:分析临床诊断为原发肾病综合征(PNS)患者的病理类型及其与临床表现之间的联系。方法:对42例临床诊断为PNS患者在彩超引导下,应用自动肾活检枪做经皮肾活检术,标本行光镜、免疫病理及电镜检查,并进行病理分型。结果:患者行肾活检时平均年龄为(32.5±15.3)岁;常见的病理类型为膜性肾病(占38.1%)、IgA肾病(占42.6%)、系膜增生性肾小球肾炎(占9.5%)、IgM肾病(占4.8%)及微小病变肾炎(占4.8%),通过肾活检纠正4例临床诊断。结论:本地区PNS患者多见于青壮年,其中IgA肾病、膜性肾病为最常见的病理类型。肾活检病理对肾病综合征的诊断、治疗及预后评估有重要意义。     

以单纯蛋白尿为主要表现IgA肾病患者的临床与病理分析

目的分析以单纯蛋白尿为主要表现的IgA肾病的临床与病理特点,并进行相关性分析。方法回顾性分析79例以单纯蛋白尿为主要表现的IgA肾病患者的临床和病理资料,采用Spearman相关分析方法进行病理损伤和免疫球蛋白沉积类型与病理分级的相关分析。结果按Lee氏分级,II级28例(35.44%),III级37例(46.84%),IV级14例(17.72%),Katafuchi积分与血尿酸、血肌酐、平均动脉压呈正相关,与尿蛋白、总胆固醇、甘油三酯无相关性。免疫病理分型:IgA型8例(10.13%),IgA+C3型8例(10.13%),IgA+IgM型14例(17.72%),IgA+IgM+C3型46例(58.23%),IgA+IgG+IgM+C3型3例(3.8%),以IgA+IgM+C3型多见。结论单纯蛋白尿IgA肾病患者病理损伤均较重,以III～IV级为主,免疫球蛋白沉积类型以IgA+IgM+C3多见。     

62例肾病综合征临床病理分析

目的探讨肾病综合征患者临床表现与肾脏病理的关系。方法对符合尿蛋白〉3．5g／24h及血清白蛋白〈30g／L诊断标准的62例肾病综合征患者进行经皮肾活检术，通过光镜、免疫病理及电镜检查明确病理分型。结果62例肾病综合征患者中常见的病理类型为系膜增生性肾小球肾炎（25．81％）、微小病变（17．74％）、IgA肾病（11．29％）、膜性肾病（8．07％）和狼疮性肾炎（27．42％），通过肾活检纠正5例临床诊断。结论肾活检病理对肾病综合症征诊断、治疗及预后评估有重要意义。     

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。     

38例儿童特发性IgM肾病临床与病理分析

目的 探讨小儿IgM肾病的临床病理特点及预后。方法 收集整理同济医院38例IgM肾病患儿病例，对其临床表现、病理及疗效进行分析。结果 IgM肾病多表现为肾病综合征(17例)，其次为单纯性血尿(13例)，也可表现为持续性蛋白尿、血尿伴蛋白尿。38例均可见IgM弥漫性沉积于肾小球系膜区，31例(81．58％)可见电子致密物的沉积，20例(52．63％)存在球囊粘连，9例(23．68％)伴有间质炎性细胞浸润。17例肾病综合征者经激素或激素加免疫抑制剂治疗后均可完全缓解。13例单纯性血尿者经中药及对症治疗后，肉眼血尿消失，镜下血尿有不同程度的减轻但仍持续存在。随访25例，21例复发(84％)但无患儿发生肾功能不全，其预后有待进一步观察。结论 儿童IgM肾病有其独特的临床病理特征及预后，应将其作为一种独立的疾病，以便更好地指导临床治疗。     

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.     

IgA肾病临床与病理分型

目的探讨原发性IgA肾病的病理与临床疗效及预后之间的关系。方法对55例原发性IgA肾病的患者进行肾穿刺后分析其病理分型、临床表现、疗效及预后。结果病理分型轻者多表现为无症状血尿和／或蛋白尿,疗效较好,而病理分型重者临床表现多为肾病综合征伴高血压、肾功能不全,疗效差,预后差,提示病理分型与临床表现、疗效及预后有明显相关。结论早期肾穿刺活检及早治疗,可以延缓肾功能衰竭进展。     

肾活检在非典型狼疮性肾炎诊断中的价值

目的探讨肾活检在非典型狼疮性肾炎（LN）诊断中的价值。方法对23例非典型LN患者的临床和病理资料进行回顾性分析。结果23例患者肾活检前均未达到ACR诊断标准，其中误诊为原发性肾病综合征13例，慢性肾炎4例．无症状性血尿或（和）蛋白尿、过敏性紫癜、特发性膜性肾病各2例；所有病例经肾活检确诊为LN，病理分型为：Ⅱ型2例，Ⅲ型3例，Ⅳ型6例，Ⅴ型11例，Ⅲ型±Ⅴ型1例；临床表现为肾病综合征者肾脏病理分型多为Ⅴ型和Ⅳ型，慢性肾炎综合征者病理分型呈多样化。结论非典型LN临床表现无特异性，易误诊误治，肾活检对非典型LN的诊断具有重要价值。     

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     

银屑病合并IgA肾病的临床特征分析探讨

目的：分析银屑病合并IgA肾病的临床表现、免疫学特点和肾脏病理特点。方法：回顾性分析5例2006年1月至2012年12月在本院诊治的银屑病合并IgAN患者的临床表现、实验室指标和肾脏病理特点。结果：多见于男性,其中男4例、女1例,平均年龄50．8岁;5例患者均为寻常型银屑病,发生肾损害时银屑病痛程为l－30年;在银屑病发病后平均28月发现肾脏损害,以无症状性镜下血尿和蛋白尿起病1例、以发作性肉眼血尿起病2例、以肾病综合征起病l例、以慢性肾炎综合征起病l例;5例患者均无严重肾脏病理表现,光镜下表现为轻度系膜细胞增生和系膜基质稍增多。结论：银屑病合并IgAN多见于男性患者,临床主要表现为尿检异常或肾病综合征、肾小管功能受损。肾组织光镜下多见节段系膜增生性病变。