原发性IgA肾病113例牛津病理分型研究

目的 探讨IgA肾病新型评价标准--"牛津病理分型"与以往评价系统的相关性,以加深对此新型评价标准的认识.方法 对113例经肾活检确诊的原发性IgA肾病患者的病理资料分别按照改良HS Lee分级标准及IgA肾病牛津分型标准进行评价.结果 IgA肾病牛津病理分型与改良HS Lee分级标准间有相关性(P＜0.05).结论 IgA肾病牛津病理分型标准继承了以往评价标准的优势,而且可以更全面的评价病理资料,但新型评价标准尚存在一定的局限性,需进一步完善及更大样本的资料研究验证.     

成人IgA肾病的临床与病理探讨

目的 回顾分析73例IgA肾病（IgAN）的临床表现与病理改变及其内在联系。方法 经常规肾穿刺活检获取年龄大于18岁的IgAN忠者的病理结果，并将临床表现及病理结果进行分类分型，同时获取IgAN的病理资料。结果 成人IgAN的临床表现以慢性肾炎为常见。占30例（41．1％）．其次是无症状血尿／尿检异常及肾病综合症。病理类型以系膜增生型肾小球肾炎常见，占39例（53．4%）。其次为局灶节段硬化性肾小球肾炎。慢性肾炎常见的病理类型为系膜增生性肾小球肾炎，其次为局灶节段硬化性肾小球肾炎和膜增殖性肾小球肾炎；无症状血尿／尿检异常及肾病综合症常见症理类为系膜增生型肾小球肾炎，其次为局灶节段硬化型肾小球肾炎；急性肾炎综合症以局灶节段增生性肾小球肾炎和系膜增生性肾小球肾炎为主，急进性肾炎主要表现为新月体肾炎．肾病综合征表现为系膜增生性肾小球肾炎和局灶节段硬化性肾小球肾炎。IgAN的病理特点以轻系膜增生为主，伴有部分及全球的硬化及间质的慢性化表现。结论 成人IgAN的临床表现以慢性肾炎最常见，病理类型以系膜增生性肾小球肾炎常见．慢性肾炎除局灶节段增生性肾小球肾炎外，其余各种病理类型均可以见到。急进性肾炎仅见于无症状性血尿／尿检异常，除新月体肾炎外。各种病理类型均可见到。IgAN的病理特点以轻系膜增生为主，同时中度及重度系膜增生占一定比例，并伴有肾小球硬化及间质纤维化，提示预后差，建议早期肾活检。积极干预治疗。     

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。     

IgA肾病肾血管病变与临床病理改变及肾功能相关性的研究进展

IgA肾病(IgA nephropathy,IgAN)已经成为全世界最常见的原发性肾小球疾病,在我国原发性肾小球疾病中所占比例更是高达45.2％～58.2％,是导致终末期肾功能衰竭的常见原因之一,超过30％的患者20年内可以进展为终末期肾衰竭.而血管病变在IgA肾病中有较高的发生率,是判断IgA肾病预后不良的重要指标之...     

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。     

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。     

Ki67表达在IgA肾病中的临床及病理意义

采用适当的抗原修复技术 ,Ｋｉ6 7抗体已可用于石蜡包埋的组织进行免疫组化染色。Ｋｉ6 7主要标记除了Ｇ0 期及早期Ｇ1期的所有增殖细胞〔1〕。笔者采用单克隆抗体Ｋｉ6 7/ＭＩＢ1,对ＩｇＡ肾病肾活检标本进行免疫组化染色 ,以研究ＩｇＡ肾病与细胞增殖的关系。1　材料与方法1.1　临床资料　收集我院 1996～ 1999年间 ,资料完整、并经肾活检确诊为ＩｇＡ肾病的住院患者 4 3例。其中男 2 3例 ,女 2 1例 ,年龄 (34 15± 11 0 0 )岁。所有病例均排除肝硬化、过敏性紫癫等继发性ＩｇＡ肾病。临床指标包括 :(1) 2 4ｈ尿蛋白定量 ;(2 )血清肌酐 ;(…     

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.     

68例维吾尔族IgA肾病患者MBL基因多态性与临床病理相关性分析

目的 探讨甘露糖结合凝集素(mannose-binding lectin,MBL)基因第54位密码子多态性与维吾尔族IgA肾病患者临床和病理的关系.方法 应用PCR-RFLP方法对68例维吾尔族IgAN患者进行MBL多态性检测,并与患者临床和病理特点进行相关性分析.结果 ①维吾尔族IgAN中表现为蛋白尿的患者突变型等位基因GAC的发生频率显著高于表现为单纯血尿的患者(P＜0.05);②维吾尔族IgAN中表现为复合性免疫沉积的患者等位基因GAC的发生频率显著高于表现为单纯免疫沉积的患者(P＜0.05).结论 MBL突变型等位基因GAC与维吾尔族IgAN 蛋白尿发生和免疫复合沉积相关.     

IgA肾病肾小球毛细血管内血浆蛋白淤积的临床病理意义

观察ＩａＡ肾病肾小球毛细血管内血浆蛋白平均吸光度变化与微血栓形成率、ＩｇＡ沉积量、临床表现及病理类型之间的关系。方法用图像分析仪定量测定５５例ＩｇＡＮ肾穿标本ＭＡＰＧ和ＩｇＡ沉积物,比较高ＭＡＰＧ组与低ＭＡＰＧ组之间微血栓形成率、ＩｇＡ沉积量、临床表现及病理类型的差异。     

Smoking-Related Renal Histologic Injury in IgA Nephropathy Patients

Purpose

Smoking reportedly exerts deleterious effects on renal function; however, its effects on histology have not been clarified in patients with IgA nephropathy (IgAN).

Materials and Methods

Renal histology was evaluated in a cohort of 397 patients diagnosed with IgAN according to smoking status and dose in relation to renal function.

Results

Among the study cohort, which was predominantly male (88.5%), 52 patients (13%) were current smokers. These current smokers demonstrated more frequent hypertension and higher serum creatinine levels than non/ex-smokers at the time of diagnosis, which was apparent with increased smoking dose. The percentages of global glomerulosclerosis and arteriolar hyalinosis increased with increased smoking dose, whereas tubulointerstitial fibrosis or arterial intimal thickening did not. Glomerular mesangial alpha-smooth muscle actin expression were similar between current and non/ex-smokers matched for age, gender, hypertension, and histologic severity, although the number of glomerular CD68+ cells was significantly fewer in smokers. Initial serum creatinine level, estimated glomerular filtration rate (eGFR), and global glomerulosclerosis were found to be risk factors of serum creatinine doubling in both smokers and non/ex-smokers by univariate analysis during a mean follow-up of 3.8 years.

Conclusion

In addition to dose dependent renal functional decline and hypertension, smoking contributes to renal disease progression by eliciting microvascular injury in IgAN patients.     

IgA肾病中肾小动脉增厚及其临床评价

目的:目的观测IgA肾病肾小动脉病理学改变,探讨小动脉增厚与临床症状及病理学改变的关系.方法:选内蒙古地区汉族110例IgA肾病患者,医学图像分析系统单盲法测算小动脉血管腔内径与血管外径比值,分析小动脉增厚与临床症状及病理学改变的关系.结果:①IgA肾病小动脉增厚可表现为肌层增厚或玻璃样变性,造成管腔狭窄,两者可共存....     

Rapid Immunofluorescence Staining by Microwave Incubation in Patients with IgA Nephropathy

Abstract

The effect of microwave incubation on the immunofluorescence staining findings in renal tissues in patients with IgA nephropathy is described. Ten patients with IgA nephropathy were examined. The renal sections were incubated with FITC-labeled rabbit anti-human IgA, IgM, IgG, or C3 antisera in a microwave oven that was operated at 2,450 MHz with an output of 500 W. There was no significant difference in the distribution of IgA or C3 deposition in the glomeruli between incubation in the microwave for 3–7 sec and 4°C overnight incubation. However, the immunofluorescence intensity of IgA after microwave incubation was less than that after incubation at 37°C for 1 min to 2 hr or 4°C overnight incubation. The intensity or distribution of IgG and IgM deposition in the glomeruli was markedly less after microwave incubation than that after incubation of 4°C overnight. We conclude that rapid immunofluorescence by microwave incubation is useful in the examination of renal biopsy specimens, especially IgA and C3 staining findings in patients with IgA nephropathy. (The J Histotechnol 13:175, 1990)     

纤维连接蛋白及其受体在IgA肾病患者肾组织中的表达

为探讨纤维连接蛋白（ＦＮ）及其受体（ＦＮＲ）与ＩｇＡ肾病的关系，利用免疫组织化学和计算机图像分析的方法对４１例ＩｇＡ肾病患者和６例正常人肾组织中的纤维连接蛋白及其受体进行定量研究。结果表明，正常肾组织肾小球ＦＮ分布于系膜区，ＦＮＲ在肾小球系膜区毛细血管袢均有分布Ｉdisplay status     

Computer Imaging Analysis of Glomerular Extracellular Components in Patients with IgA Nephropathy

Computer imaging analysis was used for quantitative evaluation of the extents, amounts and distributions of glomerular extracellular components, such as the 7S and NC 1 domains of type IV collagen, laminin (LN), fibronectin (FN) and IgA, in glomeruli from patients with IgA nephropathy. Renal biopsy specimens from 13 patients with IgA nephropathy were incubated with mouse monoclonal antibodies against the FN or non collagenous (NC 1) domain of type IV collagen or polyclonal antiserum against the LN or 7S domain of human type IV collagen, and then stained with appropriate dilutions of FITC labeled anti mouse Ig antisera. Marked staining of the 7S or NC 1 domain of type IV collagen, LN or FN was detected in the glomerular capillary walls and/or mesangial areas in patients with IgA nephropathy. In particular, a prominent increase of FN was observed in the subendothelial regions of glomerular capillary walls, i.e. mesangial interposition, in the moderate or advanced stage of IgA nephropathy. Therefore, computer imaging analysis was shown to be useful for the quantitative determination of such components distributed in glomeruli from patients with IgA nephropathy. Acta Pathol Jpn 39: 296 305, 1989.     

尿NGAL在IgA肾病肾小管间质损伤评估中的意义探讨

目的 探讨尿中性粒细胞明胶酶相关脂质运载蛋白(NGAL)在IgA肾病(IgAN)患者肾小管间质损伤(TIL)评估中的意义.方法 应用酶联免疫法(ELISA)检测110例IgAN患者,30例非IgAN肾小球疾病患者以及30例健康对照者的尿NGAL水平.结果 IgAN组、非IgAN疾病对照组较健康对照组尿NGAL水平均明显升高(P＜0.01);IgAN组尿NGAL水平与多项临床病理指标明显相关;影响尿NGAL水平的独立影响因素有血白蛋白水平,尿蛋白定量、尿NAG酶、尿渗透压,以及球性硬化、间质炎细胞浸润、间质纤维化、肾小管萎缩;以尿NGAL水平超过正常对照组2个标准差视为显著增高,联合NAG酶对有无TIL进行评估,ROC曲线下面积为0.850,明显优于血肌酐的0.675,灵敏度83.3％,特异度73.3％,准确率81.2％.结论 IgAN患者尿NGAL水平可以反映IgAN病情程度,在联合尿NGAL与尿NAG酶判别有无TIL时明显优于血肌酐及单纯的尿NGAL、尿NAG酶、尿渗透压等指标.     

IgA Nephropathy: An Ultrastructural Study

From a series of 333 renal biopsies examined by immunofluorescence microscopy, 231 specimens corresponded to primary glomerulonephritis unassociated with systemic diseases. Of those 231 biopsies, 39 had diffuse mesangial deposits of IgA (16.8%). Thirty cases of IgA nephropathy had one to several glomeruli in the Epon-embedded tissue. All cases showed mild to moderate increase of mesangial cells and matrix. Mesangial deposits were present in all cases; subendothelial (5/30), intramembranous (3/30), and subepithelial (10/30) deposits were also found. Occasional dense granular deposits involved the basement membrane of the capsule of Bowman (1/30) and the subendothelial region of some extraglomerular arterioles (2/30). Thinning (6/30) and splitting (4/30) of the glomerular lamina densa appeared focally in some cases.     

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。