258例儿童原发性IgA肾病临床与病理分析

目的分析258例儿童原发性IgA肾病的临床和病理特点。方法收集自2008年12月至2017年12月于成都市妇女儿童中心医院儿童肾脏内科肾活检诊断为IgA肾病患儿258例。所有肾活检样本均行光镜、免疫荧光和电镜检查。比较IgA肾病不同病理分级和临床特点的相关性。结果 258例IgA肾病患儿平均年龄为(9.3±3.2)岁,男女比为2.23∶1。临床表现为孤立性血尿型84例,肾脏病理Lee氏分级以Ⅱ级为主(51.1%);孤立性蛋白尿型45例,Lee氏分级以Ⅱ级为主(40.0%);血尿和蛋白尿型72例,Lee氏分级以Ⅲ级为主(56.9%);急性肾炎型4例,其中Lee氏分级为Ⅲ级者2例,Ⅱ、Ⅳ级各1例;肾病综合征型50例,Lee氏分级以Ⅲ级为主(80.0%);慢性肾炎型3例,其中Lee氏分级为Ⅳ级者2例,Ⅴ级1例。不同临床型IgA肾病患儿的免疫球蛋白主要沉积形式为IgA、IgM、C3共沉积;随着病理分级的加重,IgA、IgM、C3共沉积所占比率显著增多(P0.05)。结论 IgA肾病临床分型与病理分级有一定相关性,急性肾炎型、肾病综合征型及慢性肾炎型患儿病理损伤程度重,通过临床分型间接判断IgA肾病进展情况,对于指导临床治疗及判断疾病预后有一定临床价值。     

儿童IgA肾病的临床与病理分析

目的 探讨IgA肾病患儿临床表现和病理改变的关系及疾病转归.方法 分析30例IgA肾病患儿的临床和病理改变,病理分型采用改良Lee分型法,其中28例进行随访,4例重复肾活检.结果 临床表现孤立性血尿型10例,病理为Ⅰ、Ⅱ级;血尿和蛋白尿型11例,病理为Ⅰ～Ⅲ级;急性肾炎型2例,病理Ⅲ级;肾病综合征型7例,病理为Ⅱ～Ⅳ级,以Ⅱ、Ⅲ级为主.4例行重复肾活检,病理变化为改善、加重和无明显变化,但免疫复合物沉积均有加重.28例随访1年2个月～9年,其中9例预后不乐观;死亡1例;预后不良患儿7例治疗依从性差.结论 IgA肾病临床表现与病理有一定相关性,急性肾炎型、肾病综合征型患儿损伤重、预后欠佳,同时提示临床医师重视IgA肾病患儿随访和健康宣教,提高治疗依从性,以利疾病康复.     

伴高血压IgA肾病患者临床病理特征

目的探讨伴有高血压的IgA肾病患者临床及病理结果特征。方法经肾活检确诊的117例IgA肾病患者,分为高血压组（A组）与非高血压组（B组）,比较两组实验室检查及病理特征。结果A组中伴有高血压者占28．21％,患者蛋白尿、肾功能不全、高尿酸血症、低蛋白血症的发生率显著高于B组患者（P〈0．01）。其病理改变也较重,肾小球Lee’s分级：Ⅳ型占48．49％,Ⅴ型占21．21％;肾小管间质病变：中度占45．45％、重度占24．25％,与B组比较有统计学差异（P〈0．01）。A组肾血管病变占78．79％,中度病变为24．24％,重度病变为15．15％,两组比较有统计学差异（P〈0．05）。结论伴有高血压的IgA肾病患者,其临床表现和肾脏病变较重,高血压是预后不良的重要指标。     

IgA肾病肾病综合征的临床和病理研究

目的：了解IgA肾病肾病综合征的临床和病理的特点及其与预后的关系。方法：观察72例经肾活检确诊为IgA肾病表现为肾病综合征的患的临床表现、实验室检查、病理改变、免疫组化、组织学定量分析及其与转归的关系。结果：患常见表现有高血压、肉眼和镜下血尿、贫血、氮质血症,病理改变多样;治疗后有22例完全缓解,15例部分缓解,16例呈持续性非肾病性蛋白尿,8例呈持续肾病性蛋白尿,10例发展为肾功能衰竭;其转归或激素敏感性与临床表现、蛋白尿选择性、小球病理形态、区膜区增宽和免疫沉着、小管间质病变及肾间质T淋巴和单核细胞分布显相关。结论：IgA肾病肾病综合征可有不同预后,其预后与临床表现、实验室发现、病理特点和免疫发病机理有关。     

IgA肾病患者尿蛋白成分与临床病理指标的相关性

目的：探讨IgA肾病（IgAN）患者尿中白蛋白（Alb）、转铁蛋白（TRF）、免疫球蛋白（IgG）、α1-微球蛋白（α1-MG）、β2-微球蛋白（β2-MG）与临床及病理指标的关系。方法：采集143例原发性IgAN患者肾穿刺前新鲜晨尿,用免疫散射比浊法检测Alb、TRF、IgG、α1-MG、β2-MG的浓度;收集患者血压、血肌酐、24h尿蛋白定量以及病理分级、肾小球病变积分、肾小管间质损害积分、血管病变等资料,并进行对比分析。结果：143例IgAN患者,尿Alb、TRF、IgG、α1-MG、β2-MG升高的比例分别为95.8%、100%、91.6%、63.6%、74.1%。IgAN患者血压升高组较血压正常组尿Alb、IgG、α1-MG明显升高;肾功能异常组较肾功能正常组尿IgG、α1-MG、β2-MG明显升高。随病理Lee氏分级、肾小球硬化程度、肾小管间质损害加重,尿Alb、IgG、α1-MG明显升高;随肾小球系膜增殖程度加重,尿Alb、IgG、α1-MG以及TRF明显升高;随血管病变出现,尿α1-MG明显升高。结论：IgAN患者尿Alb、TRF、IgG、α1-MG、β2-MG与多种反应疾病进展的临床及病理指标变化一致而又各有侧重,可以更全面地反映IgAN患者肾脏病变的程度,对判断病情、随访疗效具有一定的实际意义。     

IgA肾病49例临床与病理的关系

ＩｇＡ肾病４９例临床与病理的关系黄建榕朱起之黄云ＩｇＡ肾病（ＩｇＡＮ）是原发性肾小球疾病最常见的类型之一，临床表现多样化，组织形态学轻重不一，预后相差甚远。我们报告４９例ＩｇＡＮ的临床和病理的关系，揭示其间规律性。一、病例与方法１．病例选择：资料来源...     

IgA肾病520例临床病理分析

目的研究IgA肾病(IgAN)的临床和病理特点及其相互关系。方法对1992年11月～2003年6月温州医学院附属第一医院肾内科病理室肾活检诊断的原发性IgAN520例进行临床与病理分型关系的分析。结果520例IgAN临床表现以无症状性尿检异常最常见,占346例(66.5%),其次是慢性肾炎和肾病综合征,分别占77例(14.8%)和66例(12.7%)。病理类型以局灶节段硬化性肾小球肾炎最常见,占186例(35.8%),其次是系膜增生性肾小球肾炎、轻微病变肾小球肾炎和局灶节段增生性肾小球肾炎,分别为116例(22.3%)、104例(20%)和63例(12.1%)。结论IgAN的临床病理表现多样化并具有一定特点。临床表现最常见为无症状性尿检异常,在病理上最常见的是局灶性肾小球病变类型。     

肾病综合征为表现的IgA肾病病理分析

ＩｇＡ肾病临床表现多种多样,有以单纯血尿为主、或无症状肉眼及镜下血尿、或肾病综合征或肾炎综合征,我们分析我院１９９３年～１９９８年３７例以肾病综合征为表现ＩｇＡ肾病的肾脏病理,着重分析此类ＩｇＡ肾病的肾小球内免疫球蛋白的分布与肾小球、肾小管损伤程度及...     

IgA肾病患者合并高尿酸血症临床及病理特征分析

目的探讨IgA肾病高尿酸血症的临床病理特征及相关危险因素。 方法选取2010年1月至2015年7月于山西医科大学第二医院行经皮肾穿刺活检确诊为IgA肾病的188例患者,根据血尿酸水平将患者分为正常血尿酸组与高尿酸血症组,收集患者一般资料、尿蛋白定量、肾功能等临床生化指标以及病理指标,并对肾脏病理组织进行牛津病理分型。分析两组患者临床表现、肾脏病理特点,应用多元回归统计学方法分析高尿酸血症发生的影响因素。其他数据采用SPSS13.0软件进行统计分析。 结果本研究中心188例IgA肾病患者中合并高尿酸血症的患者有42例,高尿酸血症发生率为22.3%;高尿酸血症组中男性患者36例,女性患者6例;与正常血尿酸组(男性患者61例、女性患者85例)相比,高尿酸血症组男性患者明显增多(χ²＝25.2,P<0.001)。本组研究IgA肾病患者肾脏组织牛津病理分型以M1E1S0T0多见;与正常血尿酸组比较,高尿酸血症组患者肾小管－间质损伤重,差异有统计学意义(χ²＝5.056,P＝0.025)。肾组织免疫复合物IgA沉积于毛细血管袢者高尿酸血症发生率明显升高(χ²＝44.69,P<0.001)。IgA肾病患者合并高尿酸血症的相关因素为性别、体质量指数、甘油三酯、IgA沉积于毛细血管袢。 结论IgA肾病高尿酸血症的危险因素为男性、肥胖、高甘油三酯血症,并可能与IgA在毛细血管区沉积相关。     

Clinical and histopathologic profile of patients with primary IgA nephropathy seen in a tertiary hospital in India

Background IgA nephropathy (IgAN) is known to have an aggressive course in Asians. There is a paucity of data regarding the Oxford classification pattern of Indian patients with IgAN. This study aims to characterize the clinical and histopathologic profile of these patients. Methods All patients diagnosed to have primary IgAN by kidney biopsy in the nephrology department from July 2009 to July 2014 were included in this study. All kidney biopsies were reviewed and the MEST score was assigned as per the Oxford classification. The clinical features and Oxford classification score of patients were characterized. Results Nephrotic range proteinuria (NRP) (65/103, 63.1%) with or without edema was the commonest presentation. 67.0% patients had eGFR ≥?60 mL/min and 16.5% patients had eGFR Conclusion Indian patients with primary IgA nephropathy have a unique profile. They commonly present with nephrotic range proteinuria. A significant proportion of these patients have normal renal function despite heavy proteinuria. Mesangial proliferative lesions are predominant with a paucity of endocapillary proliferation and crescents compared to other Asian populations. Immunosuppressive use is more common in Indian patients.     

IgA肾病合并高尿酸血症患者的临床病理分析

目的探讨IgA肾病合并高尿酸血症患者的临床及病理变化的特点,以期揭示IgA肾病伴有高尿酸血症的临床意义。方法回顾性分析2006年6月至2012年12月厦门大学附属中山医院肾内科收治的270例经肾活检确诊的原发性IgA肾病患者,依据血尿酸水平,将270例IgA肾病患者分为高尿酸血症组和尿酸正常组,测定记录所有患者的性别、发病年龄、收缩期血压、24 h尿蛋白定量、血尿酸、血肌酐、血白蛋白、血脂等临床指标,所有患者均进行肾脏病理检查并行Lee分级,统计分析2组的临床和病理特点,并对肾功能正常患者(135例)的病理指标进一步行亚组分析。结果IgA肾病患者高尿酸血症的患病率为25.19%,高尿酸血症组患者年龄、血白蛋白、血三酰甘油、血清总胆固醇水平与尿酸正常组比较,差异无统计学意义,患者男性比例、收缩期血压、24 h尿蛋白定量、血肌酐水平均高于尿酸正常组(P0.05),高尿酸血症组患者肾脏病理Lee分级严重的比例及发生肾小管间质病变、肾内动脉病变的比例均高于尿酸正常组(P0.05)。正常肾功能患者中,高尿酸血症组出现动脉壁肥厚等肾内动脉病变及肾小管间质慢性病变的比例亦高于尿酸正常组(P0.05)。结论 IgA肾病合并高尿酸血症患者与尿酸正常组患者比较,临床表现及肾脏病理损伤多较重,尤其对肾小管间质病变及肾内血管病变影响更明显,临床预后不佳,应予重视并及时有效地进行干预治疗。     

Clinical and pathological analysis of IgA nephropathy with chronic renal failure

Objective: To investigative clinical and pathological characteristics of IgA nephropathy with chronic renal failure.

Method: Clinical and pathological findings from 65 cases of IgA nephropathy with chronic renal failure were reviewed. Pathological characteristics of all the cases were analyzed according to WHO definition and Oxford Classification. Evaluating the severity of pathological lesions by the Katafuchi R semiquantitative scoring system, and analyzing their relationship with clinical indexes of renal function.

Results: Of all 65 cases the male and female ratio was 1.4, and the mean age was 37?±?13 years old. Levels of systolic pressure, mean arterial pressure (MAP), blood urea nitrogen (BUN), serum creatinine (Scr), uric acid (UA), album (Alb), serum IgG and 24?h urinary protein were related with eGRF level (p < 0.05, respectively). The most common pathological type was proliferative sclerosis glomerulonephritis (PSGN) and M1S1E0T0 according to WHO definition and Oxford Classification, respectively, and most of the 65 cases had glomerulosclerosis. Simple IgA deposition was the most common immunopathologic type. Of all the cases, 44.6% accompanied with C3 while 4.6% with C1q. Further analysis revealed there were no relationships between severity of pathological lesion and levels of clinical indexes (Scr and eGRF) (p?>?0.05).

Conclusion: IgA nephropathy with chronic renal failure usually occurred in young adults, and it had severe clinical condition and pathological changes, while there was no significant relationship between them.     

IgA肾病Lee分级及慢性肾脏病分期与高血压的相关性分析

目的分析IgA肾病Lee分级及慢性肾脏病(chronickindney disease,CKD分期与高血压之间的相关关系,进一步了解IgA肾病,为指导诊疗提供依据。方法回顾性分析深圳市第二人民医院肾内科经过肾活检确诊的430例原发性IgA肾病患者的临床资料,包括性别、年龄、体质量、血压、血白蛋白、血肌酐、尿酸、血总胆固醇、三酰甘油、低密度脂蛋白胆固醇、高密度脂蛋白胆固醇、24 h尿蛋白定量和肾脏病理Lee分级。根据血肌酐水平估算出肾小球滤过率分为CKD 1~5分期,根据血压水平,分为高血压组和非高血压组,高血压组分为高血压1级、高血压2级和高血压3级三个亚组,进行临床相关指标、肾脏病理Lee分级、CKD分期与高血压的相关分析。结果 430例IgA肾病患者中,男女比例1.05:1,年龄13~72岁,平均年龄(34.5±9.4)岁,发病高峰年龄段是21~30岁。高血压组的年龄和体质量均高于非高血压组(P0.05)。在本组资料中,伴有高血压201例,患病率为46.7%。高血压组中高血压1级42例(占总例数9.8%),2级高血压57例(占总例数13.2%),3级高血压102例(占总例数23.7%)。与非高血压组相比,高血压组患者的年龄、体质量、24 h尿蛋白定量、血肌酐、尿酸、血总胆固醇、三酰甘油和低密度脂蛋白胆固醇水平明显升高,高密度脂蛋白胆固醇水平明显降低(P0.05)。高血压组中肾脏病理Lee分级Ⅲ、Ⅳ、Ⅴ级分别有66例、63例、44例,经Spearman秩相关法分析得高血压与肾脏病理Lee分级之间有相关关系(r=0.394,P=0.000)。高血压组中CKD3~5期分别有60例、36例和11例,经Spearman秩相关法分析得高血压与CKD分期之间有相关关系(r=0.477,P=0.000)。多因素Logistic回归分析,结果提示血肌酐、Lee分级和CKD分期与IgA肾病患者伴发高血压有关。结论 IgA肾病伴有高血压的患病率较高,其临床指标、CKD分期和肾脏病理Lee分级均相对较重,故应加强血压的控制,有助于延缓肾功能的进展。     

伴高尿酸血症的IgA肾病临床和病理分析

目的探讨伴有高尿酸血症的IgA肾病的临床和病理特点。方法将经过肾活检明确诊断的原发性IgA肾病110例患者分为2组：尿酸正常组55例，尿酸升高组55例，比较2组临床和病理的差异。结果尿酸升高组与尿酸正常组比较，血尿素氮、血肌酐、24h尿蛋白定量、血总胆固醇、三酰甘油、低密度脂蛋白升高；肾小球损伤加重，肾小管萎缩、肾间质纤维化明显。结论伴有尿酸升高的kA肾病患者临床和病理损伤均重于尿酸正常的IgA肾病，临床上应给与重视。     

The level of serum secretory IgA of patients with IgA nephropathy is elevated and associated with pathological phenotypes.

BACKGROUND: Mucosal infection associated episodic macroscopic haematuria is observed in many patients with IgA nephropathy (IgAN), however, the mechanism has not been elucidated. Recent study suggested that secretory IgA (SIgA) might play an important role in the pathogenesis of IgAN. The aim of this study is to investigate the level of serum SIgA and the deposition of SIgA in glomeruli in IgAN patients with different pathological phenotypes. METHODS: The levels of serum SIgA were detected in 57 patients with IgAN and 48 normal controls. The associations between the levels of SIgA and the pathological phenotypes of IgAN as well as clinical parameters were investigated. Frozen renal sections from 34 of the 57 patients without IgM deposition were immunofluorescence stained and examined by confocal microscopy to detect the co-deposition of IgA and secretory component (SC). The association between deposition of SIgA and the level of serum SIgA was analysed. RESULTS: The level of serum SIgA in patients with IgAN was significantly higher than that of normal controls. The level of serum SIgA in patients with focal proliferative sclerosing IgAN (fpsIgAN) was much higher than that in patients with mild mesangial proliferative IgAN (mIgAN) (P<0.001). The level of serum SIgA correlated with the level of serum creatinine (R=0.509, P<0.001), degree of proteinuria (R=0.643, P<0.001) and creatinine clearance (R= -0.454, P=0.002) in patients with IgAN. Significant co-deposition of SC and IgA were found in 11 of the 34 patients. Although the level of serum SIgA in patients with SC deposits was higher than those without SC deposits, the difference was not significant. CONCLUSIONS: It was concluded that mesangial IgA, at least partly, was originated from mucosal immune sites. The levels of serum SIgA were significantly increased in patients with IgAN and were closely associated with pathological phenotypes.     

呈局灶节段性肾小球硬化的IgA肾病临床病理特点

目的探讨呈局灶节段性肾小球硬化（FSGS）的IgA肾病（IgAN）的临床和病理特点。方法选取我院1988年1月至2002年2月经肾活检确诊为IgAN的患者587例,其中呈FSGS85例,呈弥漫性系膜增生性肾小球肾炎（MsPGN）162例,呈弥漫性系膜增生性肾小球肾炎伴局灶节段性肾小球硬化（MsPGN伴FSGS）185例,比较3种类型IgAN临床和病理资料。结果FSGS型IgAN占同期所有IgAN的14．5％,临床类型以大量蛋白尿型为主,占37．64％。肾小球球囊黏连发生率高达74．12％,小管间质纤维化发生率97．65％,病理分级以LeeⅣ～Ⅴ级为主,免疫病理以IgA—MG型为主,与MsPGN伴FSGS型和MsPGN型的IgAN相比,FSGS型IgAN病程较长,高血压、肾功能不全发生率较高（P〈0．05）,而血尿的发生率与后两者无明显区别。结论呈FSGS型IgAN大量蛋白尿、高血压、肾功能不全的发生率高,病变较重,预后较差。     

少量蛋白尿和（或）血尿IgA肾病临床病理分析

目的 了解表现为少量蛋白尿和（或）血尿IgA肾病（IgAN）患者的肾脏病理特征及其与临床表现的关系。 方法 对1993年1月至2009年10月肾活检确诊为IgAN,且表现为少量蛋白尿 （<1 g/24 h）和（或）血尿,Scr<133 μmol/L的患者的临床和病理资料进行回顾性分析。病理学分级参照Lee分级及Katafuchi半定量积分标准。应用多因素logistic回归法分析肾脏病理损伤的危险因素。 结果 符合入选标准共316例,男123例,女193例,肾穿时年龄（33.10±10.69）岁。蛋白尿伴血尿占84.5%、单纯血尿占7.6%、单纯蛋白尿占7.9%。16.5%患者伴有高血压。CKD1、2、3期分别占76.9%、20.9%和2.2%。Lee Ⅲ级及以上患者占31.3%。52.8%患者有不同程度肾小球硬化;20.3%伴新月体形成;22.5%伴小管萎缩;16.8%有间质纤维化;24.7%有血管病变。肾小球硬化积分与估算肾小球滤过率（eGFR）呈负相关;与蛋白尿及平均动脉压（MAP）呈正相关。肾小管间质病变积分与eGFR及血红蛋白(Hb)呈负相关;与尿蛋白量呈正相关。血管病变积分与MAP呈正相关;与eGFR呈负相关(均P < 0.05)。多因素logistic回归分析结果显示,肾活检时尿蛋白量（OR = 8.564,P < 0.01）、Scr（OR = 1.031,P< 0.01）及Hb（OR = 0.975,P < 0.01）是肾脏病理损伤（LeeⅢ级以上）的独立危险因素。 结论 部分表现为少量蛋白尿和（或）血尿IgAN患者的病理改变并不轻,且肾功能已减退。尿蛋白量、Scr、Hb是预测肾脏病理损伤程度的独立危险因素。肾活检对这些患者明确诊断、判断病情和预后、制定个体化治疗方案十分重要。     

Correlation analysis between expression of histone deacetylase 6 and clinical parameters in IgA nephropathy patients

BackgroundIt has been demonstrated that histone deacetylase 6 (HDAC6) is involved in various kidney diseases in experimental study. However, correlation between HDAC6 and clinical parameters in IgA nephropathy (IgAN) patients is still unknown.MethodsA total of 46 human kidney biopsy specimens with IgAN were selected as observation group, specimens of normal renal cortex tissue that was not affected by the tumor from patients with renal carcinoma (n = 7) served as control. We investigated the relationship between HDAC6 and clinical parameters in IgAN.ResultsHDAC6 was highly expressed in human kidney biopsy specimens with IgAN compared with control group, while the number of acetyl histone H3 positive cells were significantly decreased. There was a statistical difference in the indexes of albumin, estimated glomerular filtration rate (eGFR), serum urea, serum creatinine, serum uric acid, β2-microglobulin, cystatin C, cholesterol, high-density lipoprotein, low-density lipoprotein, and HDAC6 positive area among the different Oxford Classification (p < 0.05). The expression of HDAC6 was different in various eGFR levels, the expression of HDAC6 increased with the decreasing of eGFR level, the expression of acetyl histone H3 decreased with the decreasing of eGFR level. In addition, the expression of HDAC6 positively correlated with Masson trichrome positive area, serum urea, serum creatinine, β2 macroglobulin, and cystatin C, while negatively correlated with eGFR and acetyl histone H3. Multivariate linear regression analysis demonstrated that eGFR and cystatin C were independently associated with HDAC6, respectively (p < 0.05).ConclusionsThese results suggested that high level of HDAC6 expression in IgAN is correlated with renal dysfunction.