Summary： In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schoenlein purpura nephritis （HSPN）, 31 children with IgA nephrop- athy aged between 3 ...Summary： In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schoenlein purpura nephritis （HSPN）, 31 children with IgA nephrop- athy 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, lodse 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 G 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.展开更多
目的探讨尿IgG/尿肌酐(UGCR)、24 h尿蛋白定量(24 h UP)、尿蛋白定量/尿肌酐(UPCR)在预测IgA血管炎肾炎(IgAVN)患儿肾脏病理进展中的价值。方法选取2018年1月—2022年10月收治的IgAVN患儿234例,均采集24 h尿液标本检测24 h UP。根据肾...目的探讨尿IgG/尿肌酐(UGCR)、24 h尿蛋白定量(24 h UP)、尿蛋白定量/尿肌酐(UPCR)在预测IgA血管炎肾炎(IgAVN)患儿肾脏病理进展中的价值。方法选取2018年1月—2022年10月收治的IgAVN患儿234例,均采集24 h尿液标本检测24 h UP。根据肾小球损伤程度不同将IgAVN患儿分为Ⅱ级47例、Ⅲ级175例、Ⅳ级12例,分析UGCR、24 h UP、UPCR与肾脏病理分级的相关性,通过受试者工作特征曲线分析上述指标预测肾脏IgAVN病理分级进展为Ⅲ级及以上的价值。结果肾脏病理分级Ⅲ级、Ⅳ级患儿UGCR、24 h UP、UPCR水平高于Ⅱ级患儿(P<0.01)。UGCR、24 h UP、UPCR与病理分级呈正相关,但相关性较弱(r=0.500、0.451、0.356,P<0.01)。UGCR、24 h UP和UPCR对IgAVN患儿肾脏病理分级进展为Ⅲ级及以上均具有预测价值,且三者联合检测预测价值更高(曲线下面积>0.9,P<0.01)。结论UGCR联合24 h UP、UPCR对IgAVN肾脏病理分级进展为Ⅲ级及以上的预测价值更高。展开更多
IgA血管炎(IgA vasculitis,IgAV)是儿童时期最常见的全身性小血管炎,其累及肾脏时被称作IgAV肾炎(IgA vasculitis with nephritis,IgAVN),约20%IgAVN患者可发展为慢性肾脏病,其所致的儿童终末期肾病(ESRD)占ESRD病因构成的1%~2%。识别Ig...IgA血管炎(IgA vasculitis,IgAV)是儿童时期最常见的全身性小血管炎,其累及肾脏时被称作IgAV肾炎(IgA vasculitis with nephritis,IgAVN),约20%IgAVN患者可发展为慢性肾脏病,其所致的儿童终末期肾病(ESRD)占ESRD病因构成的1%~2%。识别IgAVN预后的危险因素并及时给予有效干预,对改善患儿预后具有重要意义。本文就影响儿童IgAVN预后的危险因素进行阐述,以期为临床决策提供参考。展开更多
文摘Summary： In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schoenlein purpura nephritis （HSPN）, 31 children with IgA nephrop- athy 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, lodse 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 G 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.
文摘目的探讨尿IgG/尿肌酐(UGCR)、24 h尿蛋白定量(24 h UP)、尿蛋白定量/尿肌酐(UPCR)在预测IgA血管炎肾炎(IgAVN)患儿肾脏病理进展中的价值。方法选取2018年1月—2022年10月收治的IgAVN患儿234例,均采集24 h尿液标本检测24 h UP。根据肾小球损伤程度不同将IgAVN患儿分为Ⅱ级47例、Ⅲ级175例、Ⅳ级12例,分析UGCR、24 h UP、UPCR与肾脏病理分级的相关性,通过受试者工作特征曲线分析上述指标预测肾脏IgAVN病理分级进展为Ⅲ级及以上的价值。结果肾脏病理分级Ⅲ级、Ⅳ级患儿UGCR、24 h UP、UPCR水平高于Ⅱ级患儿(P<0.01)。UGCR、24 h UP、UPCR与病理分级呈正相关,但相关性较弱(r=0.500、0.451、0.356,P<0.01)。UGCR、24 h UP和UPCR对IgAVN患儿肾脏病理分级进展为Ⅲ级及以上均具有预测价值,且三者联合检测预测价值更高(曲线下面积>0.9,P<0.01)。结论UGCR联合24 h UP、UPCR对IgAVN肾脏病理分级进展为Ⅲ级及以上的预测价值更高。
基金国家自然科学基金(No.81300583)福建省引导性(重点)、联合创新项目(No.2019Y0069,No.2019Y9043,No.2023Y0068)第九〇〇医院院内课题(No.2021 MS 15,No.2021 JQ 10,No.2022 MS 30,No.2022ZD05)。
文摘IgA血管炎(IgA vasculitis,IgAV)是儿童时期最常见的全身性小血管炎,其累及肾脏时被称作IgAV肾炎(IgA vasculitis with nephritis,IgAVN),约20%IgAVN患者可发展为慢性肾脏病,其所致的儿童终末期肾病(ESRD)占ESRD病因构成的1%~2%。识别IgAVN预后的危险因素并及时给予有效干预,对改善患儿预后具有重要意义。本文就影响儿童IgAVN预后的危险因素进行阐述,以期为临床决策提供参考。
文摘【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。
