IgA肾病高尿酸血症与牛津病理分型及临床特征

目的：探讨IgA肾病高尿酸血症与肾脏病理牛津分型、组织病理学及临床特征的关系。方法收集151例IgA肾病,将其分为血尿酸升高组与血尿酸正常组,对肾脏病理切片进行牛津分型、Lee氏分级和肾小球硬化、新月体及血管病变分析,并记录一般资料、血压、肾功能、尿蛋白等临床指标。结果151例中IgA肾病,高尿酸血症的发病率为48.3%,青壮年男性易发,高血压与高尿酸血症密切相关。肾脏病理牛津分型主要表现为M1E0S1T0,Lee氏分级主要表现为Ⅲ级,伴高尿酸血症患者肾脏病理突出表现为肾小管间质慢性化病变重,肾小球硬化比例增多,伴有肾小球滤过率下降,而血管病变差异不明显。结论 IgA肾病伴高尿酸血症发病率高,牛津分型显示伴有高尿酸血症的IgA肾病其肾小管间质慢性病变更明显,伴有肾小球滤过率下降,临床表现更重。     

IgA肾病患者临床与病理分析

目的探讨IgA肾病患者的临床表现和肾脏病理的特点及其相互关系。方法收集62例IgA肾病患者的临床资料及肾脏病理资料并进行统计学分析。结果病理类型中以系膜增生性、局灶节段增生性、局灶节段性硬化症三种最多，共占90．32％。Lee氏病理分级中以Ⅲ、Ⅳ级最多，共82．26％，随着Lee氏病理分级程度的增高。血压、血肌酐、血尿酸、24h尿蛋白量有不同程度的升高趋势（P〈0．05或P〈0．01）。Katafuchi积分与收缩压、血肌酐、血尿素氮、血尿酸、24h尿蛋白量呈显著正相关（P〈0．01）。结论IgA肾病肾脏病变与血压、肾功能、24h尿蛋白量等临床表现之间存在明显的相关关系。     

IgA肾病系膜细胞肌成纤维细胞表型转化与病理分级、肾功能相关性分析

目的:检测IgA肾病肾小球内α-平滑肌肌动蛋白(α-SMA)及波形蛋白等细胞骨架蛋白表达,探讨IgA肾病系膜细胞肌成纤维细胞表型转化与病理分级、肾功能关系.方法:对63例肾穿刺病理诊断为IgA肾病患者,回顾其临床资料及病理改变,通过免疫组织化学显色检测α-SMA及波形蛋白在肾小球的表达.参考Cockcroft-Gault公式计算肾小球滤过率(GFR).结果:α-SMA、波形蛋白在IgA肾病标本中均100％阳性表达,α-SMA及波形蛋白在肾小球内的表达呈正相关关系.α-SMA、波形蛋白表达与WHO分级、Katafuchi氏肾病积分及GFR均呈正相关关系.在与GFR相关性方面,α-SMA高于波形蛋白.结论:α-SMA可作为评估IgA肾病患者肾病变严重程度的分子生物学指标.     

表现为孤立性血尿的IgA肾病36例临床分析

目的探讨表现为孤立性血尿的IgA肾病的临床特点及相关因素的影响。方法分析36例经肾活检确诊为孤立性血尿的IgA肾病患者的临床表现特点、病理特征及相关因素。结果孤立性血尿的IgA肾病患者以镜下血尿为主要临床表现,伴有腰痛、咽干咽痛、疲劳者较多。病理特征以系膜增生性肾小球肾炎为主,Lee病理分级分布以Ⅱ～Ⅲ级为主。结论孤立性血尿的IgA肾病患病率较高,全身症状较轻,易被忽视,容易造成漏诊,应引起临床诊治的足够重视。     

不同性别IgA肾病患者相关临床指标及病理特点对比

目的 通过对不同性别IgA肾病患者临床指标及病理特点的对比,了解性别间相关指标的差异,为临床积极有效的治疗该病提供依据。方法 回顾性分析2017年1月1日~2018年8月30日我院经肾穿刺活检确诊的361例IgA肾病患者的临床资料,比较不同性别IgA肾病患者的临床资料、危险度分级、病理分级及免疫荧光分型。结果 不同性别IgA肾病患者年龄、病程、舒张压、水肿、血尿、蛋白尿、血尿+蛋白尿、白蛋白、IgA、IgG、IgM、IgE、C3、C4、IgA/C3、肾小球滤过率、血钾、血钙、血磷、APTT、PT、FIB、左肾及右肾大小比较,差异无统计学意义（P＞0.05）;男性收缩压、血压高、肾功异常占比、胱抑素、肌酐、尿素、尿酸、甘油三脂、胆固醇、血钠、尿蛋白定量高于女性,差异有统计学意义（P＜0.05）。不同性别IgA肾病患者在1~3级占比比较,差异无统计学意义（P＞0.05）;男性IgA肾病患者在4级占比多于女性,差异有统计学意义（P＜0.05）。不同性别IgA肾病患者病理分级比较,差异无统计学意义（P＞0.05）。不同性别IgA肾病患者在IgA+IgM+C3、IgA+IgM+IgG、IgA+IgM+IlgG+C3占比比较,差异无统计学意义（P＞0.05）;男性IgA肾病患者在IgA+IgG+C3、IgA+C3分型中占比多于女性,差异有统计学意义（P＜0.05）。结论 男性IgA肾病的发病人数多于女性,且在IgA肾病中,男性的肾功能较女性差,推测其预后可能较差,因此当男性确诊为IgA肾病后,应更加关注其临床指标及病理相关指标,及早干预、及早治疗,制定合理地个性化治疗方案,延缓其进展。     

血清IgA、C3及IgA/C3比值在IgA肾病诊断中的应用价值

目的：探讨原发性IgAN和非IgAN原发性肾小球肾炎患者血清IgA、C3水平和IgA/C3比值的差异及与病理Lee氏分级的相关性。方法：选择上海交通大学医学院附属新华医院肾脏内科经肾穿刺组织活检确诊为原发性IgAN患者167例与非IgAN原发性肾小球肾炎患者105例,以透射免疫比浊法测定其血清IgA和C3浓度,按Lee氏分级标准评估IgAN的病理分级。结果：①原发性IgAN患者与非IgAN原发性肾小球肾炎患者血清IgA水平分别为[（2.98±1.27）vs（2.15±0.88）g/L,P〈0.01];血清C3水平分别为[（1.11±0.27）vs（1.12±0.29）g/L,P〉0.05];IgA/C3比值分别为[（2.83±1.34）vs（2.05±1.12）,P〈0.01];②Lee氏分级为Ⅰ和Ⅱ级与Lee氏分级为Ⅲ、Ⅳ、Ⅴ级的IgAN患者血清IgA水平分别为[（2.73±0.95）vs（3.12±1.41）g/L,P〈0.05];血清C3水平分别为[（1.94±0.32）vs（1.10±0.24）g/L,P〉0.05];IgA/C3比值分别为[（2.70±1.45）vs（2.90±1.26）,P〉0.05]。③IgAN患者不同临床起病表现组间IgA、C3水平和IgA/C3比值的差异均无统计学意义。结论：血清IgA水平及IgA/C3比值可作为鉴别IgAN与非IgAN的参考指标,血清C3水平的降低程度与IgAN患者病理严重程度相关。     

2013~2017年我院402例肾活检临床病理分析

目的 分析北部湾沿海钦州地区肾脏疾病临床及病理特点。方法 对广西钦州市第一人民医院2013年~2017年共402例肾活检患者的临床和病理资料进行回顾性分析。结果 402例肾活检患者,原发性肾小球疾病(PGN)297例,占73.88%;继发性肾小球疾病(SGN)88例,占21.89%。PGN病理分型中最常见为膜性肾病,其次为微小病变型肾病及IgA肾病,SGN中狼疮性肾炎居于首位,其次为乙肝相关性肾炎。结论 本地区肾活检患者临床表现以肾病综合征为主,PGN最常见的病理类型是膜性肾病,非肾病综合征最常见的病理类型是IgA肾病,本地区无症状尿检异常有肾活检指征患者检出率低。     

DC细胞和过渡性B细胞水平与IgA肾病患者病理分型及患者预后不良的关系

目的探讨过渡性B淋巴细胞、树突细胞(dendritic cells, DCs)水平与IgA肾病(IgA nephropathy,IgAN)患者病理分型的关系及对患者预后不良的预测价值。方法选取2012年1月至2016年1月我院收治的85例IgAN患者以及同期108例健康体检者作为研究对象。采用免疫荧光法检测肾组织DC数目和分布情况,采用流式细胞仪检测外周静脉血中CD19+CD27-CD38hi细胞水平,对IgAN患者进行肾脏组织病理分型,并分析DC细胞和过渡性B细胞与临床病理指标及预后之间的关系。结果研究组IgAN患者肾小球和肾小管间质组织中的CD209细胞水平高于健康对照组,外周血中CD19+CD27-CD38hi占B淋巴细胞百分率低于对照组(P<0.05)。肾小球、肾小管间质的DC数目、免疫荧光DC水平与牛津病理分型具有相关性(P<0.05)。Spearman相关分析显示,肾小管间质中DC数目与肾小管间质纤维化比例、肾小球硬化比例之间存在显著正相关性(P<0.05),CD19+CD27-CD38hi细胞比例与血尿素氮、血肌酐、尿蛋白量、肾小球硬化比例、节段性硬化比...     

IgA肾病的病理探讨

目的了解IgA肾病的病理特点。方法以皮肾穿刺活检获得IgA肾病的病理资料。将病理资料分为成人组和青少年组加以分析。结果IgA肾病可见于各种病理类型。其中以系膜增生性肾小球肾炎为主,病理特点以轻度系膜增生为主,伴有慢性化的表现。成人IgA肾病以难治性的病理类型较青少年组多见,以慢性化的病理特点较青少年组多见。结论成年人的IgA肾病与青少年的IgA肾病的病理类型相比,病理类型较严重,病理特点表现为慢性化程度高,提示成年人IgA肾病应早期行肾活检。     

糖基化异常IgA1自身聚合或与IgG形成的大分子可能与IgA肾病病理表型相关

目的 IgA肾病是最常见的原发性肾小球疾病之一,其临床病理表型多种多样.血清中糖基化异常的IgA1及其与其他免疫球蛋白所形成的大分子复合物可能是本病重要的发病原因.本文探讨IgA1大分子复合物的组成和结构特征,及其与IgA肾病不同病理表型之间的关系.方法 制备偶联有去唾液酸IgA1（DesIgA1）和去唾液酸去半乳糖IgA1（DesDeGalIgA1）的琼脂糖亲和层析柱（DesIgA1/Sepharose,DesDeGalIgA1/Sepharose）.取10名轻度系膜增生性IgA肾病患者、10名局灶增生硬化性IgA肾病患者及10名正常人血清,分别经DesIgA1/Sepharose和DesDeGalIgA1/Sepharose分离,测定IgA1结合蛋白（IgA1-BP）含量及其中IgA1和IgG浓度,并检测IgA1-BP中IgA1糖基化程度,比较其在IgA肾病不同病理表型间的差别.结果 从两种亲和层析柱上所洗脱的IgA1-BP含量,在不同病理类型IgA肾病患者及正常人间无明显差别.在DesDeGalIgA1/Sepharose上洗脱的IgA1-BP中,两种病理类型IgA肾病患者IgA1唾液酸均严重缺失;在局灶增生硬化性IgA肾病患者中,IgA1分子半乳糖缺失比正常人严重.同时,局灶增生硬化性IgA肾病患者血清中与DesDeGalIgA1/Sepharose结合的IgG的含量显著多于正常人.结论 糖基化缺陷的IgA1自身聚合及与IgG聚合形成的大分子复合物可能与IgA肾病的病理表型相关.     

Familial IgA nephropathy. Evidence of an inherited mechanism of disease

The evaluation of familial glomerulonephritis in patients with IgA nephropathy who were from central and eastern Kentucky resulted in the discovery of potentially related pedigrees containing 14 patients. An additional 17 members of the pedigrees had clinical glomerulonephritis, and 6 had "chronic nephritis" noted on their death certificates. Six patients with IgA nephropathy had a common ancestor. In addition, both parents of six patients with the disease came from families with other cases of IgA nephropathy. No single HLA haplotype or antigen was found in all the patients with IgA nephropathy. Our data on these pedigrees strongly support an inherited mechanism in the pathogenesis of IgA nephropathy in some patients.     

Immunopathological similarities between IgA nephropathy and Henoch-Schoenlein purpura (HSP) nephritis

A study on the immunopathological similarities between IgA nephropathy and Henoch-Schoenlein purpura (HSP) nephritis is described. Various examinations were performed as follows. (1) Pathological studies: light microscopic findings and immunofluorescent staining; (2) Measurement of the levels of IgA in pharyngeal washings and sera, and those of IgA quantitated by radial immunodiffusion; (3) Elution studies: renal biopsy specimens obtained from patients with IgA nephropathy and HSP nephritis were treated with citrate buffer (pH 3.2) and the "eluate" was neutralized by sodium hydroxide. The "eluate" was then applied to the acid-treated sections obtained from the same and other patients with IgA nephropathy as well as sections from patients with HSP nephritis and other glomerular diseases. The sections were stained with FITC-conjugated heavy chain specific antihuman IgA antisera and then examined with a fluorescent microscope. There were no differences in pathological findings of IgA nephropathy and HSP nephritis in the light microscopic and immunofluorescent examinations. The levels of IgA in pharyngeal washings and sera were significantly increased in patients with both diseases. IgA antibodies deposited in kidneys from patients with HSP nephritis crossreacted with kidneys from some patients with IgA nephropathy, and vice versa. However, antibodies from patients with IgA nephropathy and HSP nephritis did not react with normal glomeruli or other nephritic glomeruli. It is concluded that there are some immunopathological similarities between IgA nephropathy and HSP nephritis.     

IMMUNOPATHOLOGICAL SIMILARITIES BETWEEN IgA NEPHROPATHY and HENOCH-SCHOENLEIN PURPURA (HSP) NEPHRITIS

A study on the immunopathological similarities between IgA nephropathy and Henoch-Schoenlein purpura (HSP) nephritis is described. Various examinations were performed as follows. (1) Pathological studies: light microscopic findings and immunofluorescent staining; (2) Measurement of the levels of IgA in pharyngeal washings and sera, and those of IgA quantitated by radial immunodiffusion; (3) Elution studies: renal biopsy specimens obtained from patients with IgA nephropathy and HSP nephritis were treated with citrate buffer (pH 3.2) and the "eluate" was neutralized by sodium hydroxide. The "eluate" was then applied to the acid-treated sections obtained from the same and other patients with IgA nephropathy as well as sections from patients with HSP nephritis and other glomerular diseases. The sections were stained with FITC- conjugated heavy chain specific antihuman IgA antisera and then examined with a fluorescent microscope. There were no differences in pathological findings of IgA nephropathy and HSP nephritis in the light microscopic and immunofluorescent examinations. The levels of IgA in pharyngeal washings and sera were significantly increased in patients with both diseases. IgA antibodies deposited in kidneys from patients with HSP nephritis crossreacted with kidneys from some patients with IgA nephropathy, and vice versa. However, antibodies from patients with IgA nephropathy and HSP nephritis did not react with normal glomeruli or other nephritic glomeruli. It is concluded that there are some immunopathological similarities between IgA nephropathy and HSP nephritis.     

IgA nephropathy: A brief review

IgA nephropathy is a lifelong disease that is the most common primary glomerulopathy worldwide. It has a complicated and incompletely understood pathogenesis that is theorized as a four ‘hit’ process involving an improperly produced IgA. While it has a variety of histologic appearances, it is diagnosed by the presence of bright IgA deposits within the mesangium as seen on immunofluorescence and mesangial hypercellularity by light microscopy. This brief review explains the varied histologic features that are important in the diagnosis of IgA nephropathy and the calculation of the MEST-C score that was first introduced by the 2009 Oxford Classification working group.     

IgA肾病患者纤溶酶原激活物的变化及临床意义

目的：探讨IgA肾病患者纤溶酶原激活物（PA）的变化及临床意义。方法：以纤维蛋白平板法检测108例IgA肾病及34例健康自愿者尿PA活性，同时以免疫组化方法观察了27例IgA肾病及6例正常人肾组织t-PA、u-PA抗原表达，分析其与临床病理资料的关系。结果：正常人肾组织t-PA偶见少量表达于肾小球毛细血管袢，u-PA则表达于所有节段的肾小管上皮细胞。IgA肾病肾组织t-PA阳性率及单个肾小球t-PA平均积分明显高于正常人。轻度增生的肾小球其t-PA阳性率明显增高，中度增生的肾小球t-PA阳性率明显高于轻度增生者，硬化的肾小球不表达t-PA。u-PA表达明显下调，尿PA活性下降。伴血肌酐升高、肾小管间质病变严重、肾小动脉病变较重或大量蛋白管型形成的患者尿PA活性下降更为明显。结论：IgA肾病早期肾组织t-PA表达增加，晚期下降。肾组织u-PA表达减少。蛋白管型的形成可能与尿PA活性下调有关。尿PA活性的检测有助于判断IgA肾病的病情。