小儿IgA肾病临床病理分析

目的:探讨小儿IgA肾病(IgAN)临床病理特点。方法:对我院1984～2005年6月经皮肾活检病理检查确诊的小儿原发性IgA肾病的临床病理资料进行回顾性分析。结果:小儿IgAN临床以单纯血尿最常见(36.8%),其次是血尿伴蛋白尿(31.6%);临床表现及病理改变以肾病综合征最重,其次是血尿伴蛋白尿,易合并肾功能不全和高血压;肾病综合征的免疫复合物沉积以IgA+IgG+IgM型多见,单纯血尿、血尿伴蛋白尿以IgA+IgG型免疫复合物沉积为主。结论:小儿IgAN的临床及病理分级与治疗及其预后有关,只有通过肾活检病理检查才能更好地认识小儿IgAN,制定正确的治疗方案,客观的评估预后。     

IgA 肾病与妊娠相关的研究进展

Ig A肾病是最常见的原发性肾小球肾炎之一,好发于育龄期,本文主要概述了Ig A肾病与妊娠的相互影响及IgA肾病患者妊娠的时机选择和妊娠前准备。研究表明,慢性肾脏病（CKD）1～2期的IgA肾病患者妊娠不增加肾功能下降的风险,但Ig A肾病患者的剖腹产比例、早产发生率及低体质量儿发生率均高于正常人群。肾功能不全、蛋白尿、高血压是不良妊娠的危险因素。建议Ig A肾病患者具备血压维持正常、尿蛋白排泄率＜1．0 g／24 h、肾功能正常或仅轻度损伤等条件时开始妊娠。     

IgA肾病的治疗现状

IgA肾病是全球最常见的原发性肾小球疾病中的一种,其发病机制复杂,临床表现多样化,故缺乏统一的治疗标准。目前临床治疗主要是结合肾组织病理改变对症治疗。反复发作肉眼血尿型主要是去除诱因,扁桃体切除可作为一种治疗手段。孤立性镜下血尿型主要是定期复查。有蛋白尿和／或高血压、肾功能不全患者,血管紧张素转换酶抑制剂可根据情况选择,必要时应用免疫抑制剂。     

类风湿性关节炎肾损害的组织病理改变——附4例报告及文献复习

目的　探讨类风湿性关节炎 (RA)肾损害的组织病理学改变特点。方法　总结 4例RA肾损害的临床病理资料 ,并结合文献进行分析。结果　 4例均呈蛋白尿伴血尿 ,其中 3例肾功能不全 ,组织病理为IgA肾病 2例、节段系膜增生性肾炎及局灶节段性肾小球硬化各 1例 ,文献报道临床表现以蛋白尿、血尿为主 ,可呈肾病综合征或伴肾功能不全 ,病理类型以系膜增生性肾炎 (包括IgA肾病 )、膜性肾病和肾淀粉样变性多见。结论　类风湿性关节炎的组织病理学改变以系膜增生性肾炎最常见 ,临床表现多样化     

The incidence of renal diseases as diagnosed by biopsy in Hungary

AIM AND METHODS: The authors analysed the incidence of renal diseases as diagnosed by biopsy in the population living on the southern Great Hungarian Plain. 798 biopsy specimens were examined between 1990 and 2002. RESULTS: The most common diseases in decreasing order of frequency were IgA nephropathy (15%), membranous nephropathy (12%), thin-basement-membrane nephropathy (8%), minimal change nephropathy (7%), lupus glomerulonephritis (7%), focal sclerosis (6%), hypertensive kidney disease and arteriolosclerosis (5%), diabetic nephropathy (5%), and crescentic glomerulonephritis (4%). The most frequent diseases in decreasing order of frequency in children were minimal change nephropathy, thin-basement-membrane nephropathy, Henoch-Sch?nlein nephropathy and IgA nephropathy; in adults were IgA nephropathy, membranous nephropathy, lupus glomerulonephritis and thin-basement-membrane nephropathy; and in the elderly were membranous nephropathy, amyloidosis, crescentic glomerulonephritis and diabetic nephropathy. The incidence of the diseases differed significantly between the genders in IgA nephropathy, thin-basement-membrane nephropathy, lupus glomerulonephritis, chronic sclerosing nephropathy and Alport nephropathy. At the time of the biopsy, 69 patients were suffering from diabetes mellitus. 37 patients were diagnosed as having diabetic nephropathy, and 32 as having non-diabetic nephropathy. In 6 cases, the diabetic nephropathy was accompanied by other glomerular disorders. In more than half of the diabetic patients with non-diabetic nephropathy, membranous nephropathy or focal sclerosis was diagnosed. Crescentic glomerulonephritis was diagnosed on 30 occasions, which was due to vasculitis in 20 cases, proliferative glomerulonephritis in 7 cases and anti-glomerular-basement-membrane nephritis in 3 cases. In the middle-aged and the elderly, the renal disease was relatively often a consequence of systemic disease. CONCLUSION: The incidence and the gender distribution of renal diseases diagnosed by biopsy were similar to those reported by other European kidney biopsy centres. IgA nephropathy was the most frequent disease in the biopsy registry of the authors. The high incidence of thin-basement-membrane nephropathy seems to be related to consequent biopsy examinations of glomerular haematuria. In diabetics and the elderly, the diagnosis of the renal disease may be challenging.     

The emerging role of omega-3 polyunsaturated fatty acids in the management of patients with IgA nephropathy.

Immunoglobulin A nephropathy (IgAN), the most common primary glomerulonephritis in the world, affects mostly young adults, and shows a widely variable clinical course with many patients developing progressive renal disease, culminating in terminal renal failure in 20% to 40% of those afflicted. Until recently, no treatment options have been available for IgAN. Although a cure for the disease remains elusive, drugs that slow disease progression are becoming available, including omega-3 (n-3) fatty acids. The largest long-term clinical trial evaluating n-3 fatty acids in high-risk patients with IgAN showed that early and prolonged treatment with n-3 fatty acids retards renal progression. The rationale for using these fats involves potential mechanisms that reduce renal inflammation and glomerulosclerosis, hallmarks of progressive disease.     

186例成人IgA肾病临床病理分析

目的研究成人IgA肾病(IgAN)的临床、病理特点及相关性. 方法将我科于1996年8月～2003年12月收治的原发性成人IgAN186例按照年龄分成2组,根据各年龄组的临床及其组织学特点进行临床与病理分型的分析. 结果 186例成人IgAN临床表现以无症状性尿检异常最常见,占122例(65.6%),其次是慢性肾炎和肾病综合征,分别占28例(15.1%)和25例(13.4%).老年组肾病综合征和急性肾功能衰竭的发生率高于青壮年组.病理类型以局灶节段硬化性肾小球肾炎最常见,占67例(36%),其次是系膜增生性肾小球肾炎、轻微病变肾小球肾炎,分别为47例(25.3%)和35例(18.8%). 结论成人IgAN的临床病理表现多样化并且有一定特点.临床表现以无症状性尿检异常最常见,病理类型以局灶节段性肾小球病变最常见.     

IgA肾病肾小球足细胞损伤与高脂血症的关系

目的 探讨IgA肾病患者高脂血症对肾小球足细胞损伤的作用.方法 选择51例经肾穿刺活检明确诊断的IgA肾病患者,肾组织病理定量采用免疫组织化学技术借助肾小球足细胞表面特异性标记物WT1对肾小球足细胞进行准确的密度定量分析.结果 IgA肾病患者三酰甘油与足细胞病变呈正相关(r=0.549,P＜0.05),与血清肌酐、肾小球硬化程度呈正相关(r=0.770、0.698,P＜0.01),与24h尿蛋白定量无相关性.随着病变加重,硬化肾小球增加,肾小球内足细胞表达减少(P＜0.01),IgA肾病患者足细胞病变与24h尿蛋白定量无相关性,但与血清肌酐、肾小球硬化程度呈正相关(r=0.765、0.679,P＜ 0.01).结论 IgA肾病脂代谢紊乱可能导致肾小球足细胞损伤,加重IgA肾病进展.     

原发性IgA肾病患儿肾组织免疫病理及CD44的表达

目的:探讨细胞表面糖蛋白(CD44)在儿童IgA肾病(IgAN)发病中的作用及其与肾小管间质损害的关系。方法:46例IgAN患儿按临床表现分为3组:孤立性血尿组14例,血尿蛋白尿组17例,肾病综合征组15例;按病理分级分为3组:病理Ⅰ+Ⅱ级组23例,Ⅲ级组14例,Ⅳ级组9例。设肾活检正常对照组4例。采用免疫组化SP法检测病例组与对照组肾组织CD44的表达,并分析其与肾小管间质病变程度的相关性。结果:①正常肾组织仅有少量CD44表达。②IgAN患儿肾组织中CD44的表达均较对照组明显增高,不同临床表现组中肾病综合征组最高,孤立性血尿组最低,且与IgAN患儿的病理分级密切相关。③IgAN患儿肾组织CD44的表达与肾小管间质损害程度呈正相关。结论:CD44参与了儿童IgAN的发病和肾小管间质损害,与IgAN的进展及预后有关。     

尿足细胞阳性IgA肾病患者临床病理特点

目的探讨尿足细胞阳性原发性IgA肾病（IgAN）患者临床病理特点。方法50例肾活检明确诊断的IgAN患者和10例健康志愿者,利用足细胞的标记蛋白Podocalyxin（PCX）标记尿液和肾组织足细胞,采集IgAN患者肾活检时临床资料、肾活检光镜结果,其中光镜参照Hass分级,各项病理指标参照Fofi半定量积分法进行评分,荧光显微镜观察肾组织足细胞PCX荧光表达程度,电镜观察足细胞足突形态学改变。结果①尿足细胞阳性的IgAN患者尿蛋白水平、血肌酐水平、平均动脉压较尿足细胞阴性患者增高,血浆白蛋白、肾小球滤过率（GFR）较尿足细胞阴性患者降低（P〈0．05）;②光镜示尿足细胞阳性的IgAN患者肾小球硬化程度、新月体发生率较尿足细胞阴性患者增高（P〈0．05）,而两组比较,系膜增生、基质增多、肾小管萎缩、间质纤维化、间质炎细胞浸润、间质血管硬化程度的差异无统计学意义（P〉0．05）;③肾组织足细胞PCX抗体荧光表达显示：肾小球节段硬化和球性硬化处足细胞PCX抗体荧光表达缺失;④电镜观察提示,无论是否伴足细胞尿,其足细胞足突均可出现一系列形态学改变。结论足细胞尿是反映肾脏疾病轻重的一个指标,与肾脏病理类型有一定关系。     

肾动态显像在临床-病理分离的IgA肾病治疗中的应用

目的研究肾动态显像在指导治疗临床-病理分离的IgA肾病中的应用价值。方法选择经病理学证实为IgA肾病而临床表现轻微患者62例,对其肾小球、肾小管间质和血管损害程度进行LEE氏分级。全部患者均接受99mTc-DTPA肾动态显像,计算出灌注指数(PI)、肾小球滤过率(GFR)、20m in清除率(C20)分析不同病理分级之间显像指标与病理积分间的关系。结果 PI与血管损害积分正相关(P<0.01)GFR与肾小球损害(P<0.01)及肾小管间质损害积分(P<0.05)呈负相关,C20与肾小管间质损害积分(P<0.01)呈负相关。结论肾动态显像结果与肾脏组织病理学改变明显相关、能较好地反映病理损伤的进程,对指导治疗临床-病理分离的IgA肾病有重要价值。     

IgA肾病合并代谢综合征患者生活方式干预的临床研究

目的观察IgA肾病合并代谢综合征(MS)患者生活方式干预的临床效果。方法选择我科2007-2011年确诊IgA肾病合并代谢综合征患者62例,随机分为2组,研究组为生活方式干预(运动锻炼、合理饮食、戒烟限酒)合并药物治疗组,对照组为单用药物治疗组6,个月后比较两组代谢综合征相关的参数及24小时尿蛋白定量、肾功能等。结果研究组生活方式干预6个月后,代谢综合征相关危险因素,如肥胖、高血压、高血糖、高甘油三酯、高尿酸均有明显改善(,P<0.01,或P<0.05)。研究组与对照组治疗后比较肾功能明显好转,24小时尿蛋白定量明显降低(P<0.05)。结论对IgA肾病合并代谢综合征患者应用活方式干预联合药物治疗,可有效控制病情的进展。     

老年肾病综合征120例临床和病理特点

目的探讨、分析老年肾病综合征（NS）的临床与肾病理特点。方法回顾性分析2008年1月~2010年12月某院〉60岁120例老年NS患者的临床表现和肾活检病理诊断类型及其之间的关系。结果男性75例,女性45例,平均年龄（69.9±5.1）岁。120例NS患者中合并肾炎综合征有56例（46.7%）,合并的其他主要疾病有高血压（50例）、慢性肾病（34例）、2型糖尿病（23例）、冠心病（19例）、急性肾损伤（10例）。肾活检病理诊断中膜性肾病62例（51.7%）;其余分别为局灶节段性肾小球硬化症11例、系膜增殖性肾炎9例、IgA肾病8例、微小病变7例、糖尿病肾病5例、糖尿病肾病合并膜性肾病2例、淀粉样变肾病3例、紫癜性肾炎3例、肾小球轻微病变2例、ANCA相关性肾炎2例、多发性骨髓瘤肾损害、慢性间质性肾炎、狼疮性肾炎各1例。结论老年人发生NS临床表现与年轻患者显著不同,老年NS的病理改变仍以膜性肾病为主,存在多种继发因素。     

扁桃体切除术对IgA肾病的治疗及预后因素预测

目的 了解扁桃体切除术对IgA肾病的治疗效果,探讨手术的适应证及禁忌证.方法 经肾活检确诊IgA肾病行扁桃体切除术患者35例为手术组,分别在手术前IgA肾病发作期及术后1周、2周、4周、半年检测24h尿蛋白定量、1 h尿RBC及血清肌酐.经肾活检确诊IgA肾病仅行内科治疗患者40例(非手术组)比较.结果 手术组的有效率是71.4%,非手术组的有效率是52.5%,两组比较差异有统计学意义.结论 扁桃体切除术对具有慢性扁桃体炎的IgA肾病患者疗效较好,尤其是肾小球轻度受损者,效果肯定;肾功能明显损害是手术禁忌证.     

MCP-1的变化与蛋白尿的关系及其在儿童IgA肾病中的作用

目的:探讨血液、尿液单核细胞趋化蛋白-1(MCP-1)的含量变化与蛋白尿的关系及其在儿童IgA肾病中的作用。方法:根据24 h尿蛋白定量将2008年1月～2011年10月收治的33例IgA肾病儿童分为大量蛋白尿组(>1.5 g)22例和小量蛋白尿组(≤1.5 g)11例,另外选取同期健康儿童50例作为正常对照组。采用ELISA法测定所有儿童血液和尿液MCP-1,对MCP-1与24 h尿蛋白定量、血肌酐、血尿β2微球蛋白以及肾脏病理分级的相关性进行分析。结果:大量蛋白尿组、小量蛋白尿组和正常对照组尿液MCP-1含量分别为(138.8±58.5)pg/ml、(76.4±36.2)pg/ml、(22.6±12.1)pg/ml,血液MCP-1含量分别为(23.9±5.1)pg/ml、(19.6±4.2)pg/ml、(17.6±2.5)pg/ml,尿液MCP-1含量两两比较,差异均有统计学意义(P<0.05),血液MCP-1含量三组比较差异均无统计学意义(P>0.05)。结论:MCP-1可能在儿童IgA肾病的病理损害中发挥重要作用,尿液MCP-1可作为评价IgA肾病肾功能的一项辅助指标。     

尿激酶联合苯那普利治疗IgA肾病的疗效观察

[目的]探讨尿激酶联合苯那普利治疗IgA肾病的临床疗效。[方法]140例IgA肾病患按随机数字表法分为对照组(70例)和观察组(70例),观察组患者应用尿激酶加苯那普利联合治疗,对照组单纯应用苯那普利。比较两组的治疗效果。[结果]6个疗程后,观察组的尿蛋白量和Scr水平与治疗前相比明显降低,Alb水平和Ccr显著升高(P﹤0.05,0.01),且改善水平显著优于对照组(P﹤0.05);观察组的总有效率为74.3%(52/70),明显高于对照组51.4%(36/70),差异有统计学意义(χ2=7.83,P﹤0.01)。[结论]尿激酶联合苯那普利治疗IgA肾病疗效确切,能显著改善肾功能,优于单纯使用苯那普利,值得临床进一步推广应用。     

扁桃体切除术对IgA肾病的治疗及预后因素预测

目的了解扁桃体切除术对IgA肾病的治疗效果，探讨手术的适应证及禁忌证。方法经。肾活检确诊IgA肾病行扁桃体切除术患者35例为手术组，分别在手术前IgA肾病发作期及术后1周、2周、4周、半年检测24h尿蛋白定量、1h尿RBC及血清肌酐。经。肾活检确诊IgA肾病仅行内科治疗患者40例（非手术组）比较。结果手术组的有效率是71．4％，非手术组的有效率是52．5％，两组比较差异有统计学意义。结论扁桃体切除术对具有慢性扁桃体炎的IgA肾病患者疗效较好，尤其是肾小球轻度受损者，效果肯定；肾功能明显损害是手术禁忌证。     

IgA肾病中关于细胞凋亡的研究进展

IgA肾病(IgAN)是全球范围内最常见的原发性肾小球疾病之一,近年来在儿童中的患病率明显升高,部分患儿最终发展为终末期肾病(ESRD),对儿童的生活质量及生命造成了严重威胁;由于其发病机理尚不明确,以及临床表现的多样性和预后的差异性,在临床上尚无疗效确切公认的治疗方法,药物治疗是常用的治疗手段.细胞凋亡是细胞生命活动中的基本现象,是近年生命科学研究的热点.该文对IgAN中有关细胞凋亡的研究进展进行综述,指出对细胞凋亡的干预可能是一种对IgAN新的治疗靶点.     

IgA and IgG antibodies to Chlamydia in IgA nephropathy as well as in mesangiocapillary and membranous glomerulonephritis

It might be supposed that, among the antigens causing chronic immune complex glomerulonephritis (IC GN), there are foreign materials, e.g. bacterial antigens penetrating the mucosal barrier. To put this hypothesis to the test, the presence and titres of IgA and IgG antibodies against Chlamydia (C., one of the most frequent bacteria causing mucosal inflammation) have been studied in the sera of 70 patients with IgA nephropathy (IgA NP), of 25 with mesangiocapillary GN (MCGN) and of 27 with membranous GN (MGN) using a single serovar (L2) inclusion immunoperoxidase assay. Significantly more IgA (titres greater than or equal to 8) and IgG (titres greater than or equal to 32) antibodies were found in the sera of IgA NP and MCGN patients than in healthy controls. These results are compatible with the hypothesis that there are some similarities between the clinical and morphological picture of IgA NP and MCGN. Furthermore, it may be assumed that in renal patients with an active C. infection (high IgG titres with IgA seropositivity) C. antigens may play a role in the production of nephropathogenic IC developing in antibody excess.     

Silicosis and renal disease: insights from a case of IgA nephropathy

A 68-yr-old male, smoker, is admitted for proteinuria (2,800 mg/24 h) and reduced renal function (serum creatinine 2 mg/dl, GFR 35 ml/min). Renter, he started working 20-yr-old as a sandstone cave miner. Despite the high levels of silica dusts, he reported no mandatory use of airways protection devices during the first 25 yr of activity. No clinical or radiological signs of silicosis or pneumoconiosis where reported until the year of retirement (1997). Erythrocyte sedimentation rate (91 mm/h) and C reactive protein (35 mg/l) suggested a pro-inflammatory status. High serum IgA was found (465 mg/dl). A renal biopsy identified glomerular sclerosis with IgA deposition, signs of diffuse vasculitis and tubular atrophia suggesting a diagnosis of IgA nephropathy. Chest X-Rays showed emphysema and diffuse nodularity suggesting diagnosis of silicosis. Chest tomography was also positive for mild signs of silicosis with silicotic nodules and without honeycombing. IgA nephropathy is the most common type of glomerulonephritis worldwide. Several clues suggest a genetic or acquired abnormality of immune system as a trigger of the increased production of IgA. In our case report, simultaneous kidney and pulmonary disease could suggest same triggers (e.g. exposure to virus, bacteria or environmental agents) inducing IgA synthesis and pulmonary immune system activation.