儿童无症状尿检异常IgA肾病的临床病理和预后分析

目的 探讨儿童无症状尿检异常的IgA肾病的临床病理特征和预后。 方法 对54例IgA肾病儿童的临床和病理特征进行分析。根据起病时有无临床症状分为无症状尿检异常组和有症状肾炎组。组织病理学分级参照Lee氏和Katafuchi氏半定量积分法。 结果 无症状尿检异常组18例，有症状肾炎组36例。有症状肾炎组尿蛋白量（24 h）明显高于无症状尿检异常组[（2.3±2.2） g比（0.4±0.3） g，P < 0.05]。无症状尿检异常的IgA肾病儿童表现为镜下血尿者，87％有尿微量白蛋白增高。无症状尿检异常IgA肾病患儿病理表现以Lee 氏Ⅰ~Ⅱ级为主，2例表现为Lee氏Ⅳ~Ⅴ级和 5例发生Katafuchi Ⅱ~Ⅲ级肾小管间质病变。有症状肾炎组Lee氏病理分级以Ⅱ~Ⅲ级为主，两者病理分级分布差异无统计学意义（P > 0.05）。全组患儿平均随访（26.9±8.8）月后，1例病理为Lee 氏Ⅴ级患儿进入终末期肾衰竭，其余患儿Scr均无升高1倍以上。 结论 无症状尿检异常的儿童IgA肾病虽临床症状轻微，但可出现病理损害严重的病例，并影响其预后。     

IgA肾病简明半定量病理评分方法及其与预后的关系

目的 建立一个用于预测预后且相对简明的IgA肾病组织学半定量评分方法。方法回顾性分析北京大学第一医院肾内科确诊为原发性IgA肾病并有2年以上随访资料的患者155例,终点事件为进入不可逆的终末期肾衰竭（ESRD）。所有病理切片均经重新阅片,其中91例由一位病理医师分别两次阅片,56例由两位病理医师分别独立阅片评分,判断重复性。初始的8个病理指标指数：（1）内皮细胞增生（endoI）;（2）活动性新月体及节段性袢坏死（dGAI）;（3）系膜细胞增生（MsHI）;（4）系膜基质增多（MsMI）;（5）肾小球慢性病变（GCI）;（6）肾间质炎症细胞浸润（infl）;（7）肾小管萎缩和肾间质纤维化（TCI）;（8）小动脉慢性病变（VCI）。结果 肾穿时Scr为（112．18±83．13）μmol/L。25例患者（16．13%）在随访期内[（69．07±28．66）月,10～170个月]达到终点（ESRD）。对初始的8个病理指标进行多变量生存分析,选出以下3个与预后最相关的变量组成评分方法：dGAI、GCI和TCI。后两项之和组成慢性指数CI。在多因素生存分析中,dGAI和CI都与肾脏生存率呈正相关（RR分别为1．255和1．691,P＜0．05）,是影响预后的独立危险因素。根据患者的dGAI和CI进行分组．显示dGAI≥4且CI≥6者预后最差（P＜0．01）。对CKDⅠ、Ⅱ期患者的多种临床病理指标进行多因素生存分析,仅CI是影响预后的独立危险因素。评分法具有良好的重复性,kappa值均大于0．4。结论 由代表活动性病变的dGAI和代表慢性病变的CI组成的IgA肾病组织学半定量评分法能够有效地判断预后,且具有良好的重复性。     

742例原发性IgA肾病患者的临床病理特征及其预后分析

目的:探讨原发性IgA肾病（IgA nephropathy,IgAN）患者的临床病理特征及IgAN患者肾脏预后的影响因素。方法:回顾性分析2015年1月至2019年9月在安徽医科大学第一附属医院经肾活检确诊原发性IgAN的患者资料,并根据肾活检时基线肾功能（estimated glomerular filtration...     

强直性脊柱炎相关IgA肾病临床病理分析

目的了解强直性脊柱炎（AS）相关IgA肾病的临床病理特点。方法自1997年1月至2006年12月10年间在北京协和医院接受肾活检确诊为IgA肾病的AS患者10例，回顾性分析其临床及病理特点。结果男性9例，女性l例，平均年龄（28．6＋6．8）岁（16～53岁）。4例患者表现为无症状镜下血尿；6例表现反复血尿合并蛋白尿，其中2例有发作性肉眼血尿。平均尿蛋白量（24h）为（1．56±1．53）g（0．02-5．26g）。2例患者有血压升高。所有患者的血肌酐水平均在正常范围。光镜下，8例患者呈轻度系膜细胞增生，IgA肾病Lee氏分级均为Ⅰ或Ⅱ级；另外2例呈中重度系膜增生性改变，IgA肾病Lee氏分级分别为Ⅲ级和Ⅵ级。结论AS相关IgA肾病临床表现为隐匿性肾炎或慢性肾小球肾炎，病理改变以轻度系膜增生为主。     

伴足细胞尿的IgA肾病的临床病理特征

目的 探讨伴足细胞尿的IgA肾病（IgAN）患者的临床病理特点。方法 入选IgAN患者36例,其中男性20例,女性16例,平均年龄（34．1±12．2）岁。10例健康志愿者为健康对照。足细胞排泄的定量检测采用尿沉渣涂片免疫组化染色直接计数。进行尿液足细胞排泄与肾脏病理的相关分析。结果 （1）IgAN患者尿细胞podocalyxin阳性率为61%,健康对照组为0（P＜0．05）。（2）与非大量蛋白尿（＜3．0 g/24 h）IgAN患者比较,大量蛋白尿（≥3．0 g/24 h）IgAN患者的尿液足细胞检测阳性率、尿液足细胞排泄数、足细胞与尿肌酐的比值以及足细胞占尿液小管上皮细胞的百分数均显著增高（P＜0．05）。IgAN患者足细胞排泄水平与蛋白尿水平呈正相关（r=0．446,P=0．007）。（3）与无足细胞尿的患者比较,伴足细胞尿的IgAN患者的蛋白尿水平显著增高,血浆白蛋白水平显著降低,肾小管上皮细胞与尿肌酐的比值亦显著增高（P＜0．05）。但伴与不伴足细胞尿的2组IgAN患者在年龄、性别、血压、Scr、血红蛋白水平以及血浆脂质代谢等方面差异均无统计学意义（P＞0．05）。（4）尿足细胞的排泄与细胞新月体或细胞纤维性新月体、小球血管襻腔狭窄和足突广泛融合病变有关,而与系膜、内皮细胞病变及局灶基底膜增厚无关。伴足细胞尿的患者肾小球和肾小管间质纤维化更明显（P＜0．05）。伴有新月体的患者其尿液足细胞排泄水平、尿液上皮细胞和管型的排泄均增加（P＜0．05）。结论 足细胞尿不仅是IgAN患者肾小球损伤的结果,也是IgAN患者活动性损伤的指标。足细胞尿排泄的水平与蛋门尿水平呈正相关,与肾脏病理类型也有一定的关系。     

血清IgM水平与IgA肾病疾病严重程度及预后的相关性

本研究纳入252例原发性IgA肾病(IgAN)患者为研究对象,按照肾穿刺时血清IgM中位数水平分为低IgM水平组和高IgM水平组。结果示低IgM水平组(<1.015 g/L)患者的年龄、男性占比、血尿素氮、血尿酸、尿蛋白量、收缩压、用激素/免疫抑制剂占比、肾脏病理肾小管萎缩或间质纤维化分型占比较高IgM水平组高,血清IgG、C3水平较低(均P<0.05)。Kaplan-Meier生存曲线分析结果显示,低IgM水平组肾脏累积生存率低于高IgM水平组(χ2=7.123,P=0.008)。多因素Cox回归分析结果显示,低IgM水平是IgAN患者肾脏不良预后的危险因素。提示低血IgM水平IgAN患者的临床及肾脏间质病理改变更严重,低IgM水平是IgAN患者进展至终末期肾脏病的独立危险因素。     

影响原发性IgA肾病预后的临床病理因素研究进展

IgA肾病(IgA Nephropathy,IgAN)具有慢性进展性质,需要识别该病进展的标志和预后的影响因素.现已明确肾活检时的血肌酐水平、持续性蛋白尿、高血压对预测IgAN预后的价值最大.广泛的肾小球硬化及/或肾间质纤维化是最强的独立的预测疾病进展的病理学参数.新近提出的病理评分系统能独立于临床地反映IgAN预后.还有研究显示结合多个参数(特别是临床病理结合)可更好地预测疾病进展和判断预后.     

贫血加重IgA肾病患者的临床和病理改变

目的 分析IgA肾病合并贫血患者的临床病理特征.方法 收集经肾活检确诊的IgA肾病患者临床资料409例,按照贫血与否分为非贫血组和贫血组,回顾性分析两组患者的临床和病理资料.结果 与非贫血组比较,贫血组患者的肾小球损伤和肾小管间质萎缩程度较重、24 h尿蛋白增多和eGFR降低.Spearman相关分析结果显示,血红蛋白、eGFR与肾脏病理损伤呈负相关(P＜0.05),血尿酸、24h尿蛋白与肾脏病理损伤呈正相关(P＜0.05).多因素Logistic回归分析发现贫血是肾小管间质萎缩的独立危险因素.结论 IgA肾病合并贫血患者的临床和病理损伤重于IgA肾病非贫血的患者,贫血参与IgA肾病的进展.     

IgA肾病临床与病理分析—附61例报告

目的：探讨IgA肾病患者临床与病理类型间的关系。为临床治疗及判断预后提供依据。方法：对61例IgA肾病患者的临床特点，病理及免疫病理等资料进行回顾性分析。结果：IgA肾病以血尿并发蛋白尿最多见，占49．18％，病理分级与临床类型未发现有相关性，与免疫病理学型无密切关系，但病理分级高，提示肾损害严重，预后不良，重度蛋白尿组的血肌酐，血尿素氮显著高于轻，中度蛋白尿组。结论：大量蛋白尿可能促进IgA肾病的肾功能损害进展。     

代谢综合征对IgA肾病患者病情的影响

目的 观察代谢综合征（MS）对IgA肾病（IgAN）患者病情的影响。 方法 从确诊为IgAN的病例中,以并发MS的118例作为IgAN-MS组;另从同年龄范围的IgAN病例中随机抽取118例无并发MS者作为IgAN-非MS组,对比分析两组患者的临床病理资料。 结果 IgAN-MS组的尿蛋白量、Scr、体质量指数、平均动脉压、血三酰甘油、空腹血糖及血尿酸水平均显著高于IgAN-非MS组（P ＜ 0.05或P ＜ 0.01）;血高密度脂蛋白（HDL-C）水平显著低于IgAN-非MS组（P ＜ 0.01）;高血压、糖代谢异常及脂代谢异常患者的百分率也显著高于IgAN-非MS组（P ＜ 0.01）。IgAN-MS组的病理改变显著重于IgAN-非MS组（P ＜ 0.01）。Spearman相关分析显示MS与尿蛋白量、Scr、肾小球损伤指数及肾小管间质损伤指数均呈正相关（P ＜ 0.01）。 结论 MS是IgAN进展的一个危险因素。     

Clinicopathological and prognostic analysis of IgA nephropathy patients with anemia

Objective To analyze the clinicopathological features of IgA nephropathy (IgAN) patients with anemia and the influencing factors of prognosis. Methods The clinical and pathological data of patients diagnosed with primary IgAN at the First Affiliated Hospital of Fujian Medical University from January 1, 2006 to December 31, 2016 were retrospectively analyzed. The patients were divided into anemia group and non-anemia group according to whether the patient was anemia or not. The clinical and pathological data of the two groups were collected. All of them were followed up from the date of renal biopsy to January 1, 2018. Survival curves of the two groups were drawn by Kaplan-Meier method, and compared by Log-rank test. Multivariate Cox proportional hazards regression model was adopted to explore the influencing factors of prognosis in IgAN patients. Results A total of 231 subjects were enrolled, including 122 males (52.8%), and the male-female ratio was 1.12∶1. Their age was (34.8±10.1) years (15-68 years). There were 70 patients (30.3%) in anemia group, 161 cases (69.7%) in non-anemic group. Compared with non-anemia group, anemia group had higher proportion of females, lower serum albumin, higher proportion of tubular atrophy/interstitial fibrosis (T1/2), endothelial cell proliferation (E1) and crescent formation (C1/2), which were statistically significant (all P＜0.05). The patients had a median follow-up time as 6.3 years (0.3-12.9 years). Survival analysis showed that patients in anemia group had lower cumulative renal survival rate than that in non-anemia group ( χ2=15.234, P＜0.001). Multivariate Cox hazards regression analysis revealed that anemia (HR=3.820, 95%CI 1.674-8.719, P=0.001), tubular atrophy/interstitial fibrosis (T1/2) (HR=3.770, 95%CI 1.026-13.852, P=0.046), glomerular segmental sclerosis/adhesion (S1) (HR=4.211, 95%CI 1.139-15.576, P=0.031), hypertension (HR=2.988, 95%CI 1.276-6.999, P=0.012), increased 24 h urinary protein (HR=1.103, 95%CI 1.046-1.163, P＜0.001) and estimated glomerular filtration (eGFR)＜60 ml?min-1?(1.73 m2)-1 (HR=3.725, 95%CI 1.639-8.462, P=0.002) were the independent risk factors for poor renal prognosis in patients with IgAN. Conclusions The clinicopathological features of IgAN patients with anemia are relatively serious, and the renal cumulative survival rate is lower. Anemia, tubular atrophy/interstitial fibrosis (T1/2), glomerular segmental sclerosis/adhesion (S1), hypertension, increased urinary protein and eGFR＜60 ml?min-1?(1.73 m2)-1 are the independent risk factors for poor renal prognosis in patients with IgAN.     

Clinical-pathologic features and outcomes of IgA nephropathy patients with IgM deposition

Objective To determine the correlation of IgM deposition with clinic-pathological features and outcomes of IgA nephropathy patients. Methods A total of 1060 patients, who were diagnosed as IgA nephropathy by renal biopsies between 2001 and 2007 in Guangxing Hospital were enrolled. According to immunofluorescent test, patients were divided into patients with mesangial IgM deposition and patients without IgM deposition. Renal survival curves were assessed by Kaplan-Meier method. The effect of IgM deposition on outcomes of IgA nephropathy patients was examined by univariate and multivariable Cox proportional-hazards regression. Results Among 1060 IgA nephropathy patients, there were 750 patients with IgM deposition and 310 patients without IgM deposition. (1) Urinary protein and uric acid in patients with IgM deposition were significantly higher than those in patients without IgM deposition (all P＜0.05). Other clinical indicators shown no statistical difference (all P＞0.05). Moreover, IgM deposition patients had higher serum IgA, serum IgG and serum IgM (all P＜0.05). (2) In pathological indicators, IgM deposition patients had more segmented sclerosis or adhesions (S1 of Oxford classification), activity lesions as inflammatory cell infiltration and mesangial proliferation, and chronic pathological changes as tubular atrophy, segmented glomerular damage than patients without IgM deposition (all P＜0.05). (3) All patients were followed-up for a median of 89.7(61.8, 113.4) months, Kaplan-Meier analysis revealed that kidney survival rate was significantly lower in IgM deposition patients compared with patients without IgM deposition (Log-rank χ2=4.95, P=0.026). In a univariate Cox hazards regression mode, IgM deposition was a risk factor for poor prognosis of IgA nephropathy patients (HR=1.597, 95%CI 1.053-2.422, P=0.027). However, in a multivariable Cox analysis, IgM deposition shown no influence on outcomes of IgA nephropathy patients (HR=1.409, 95%CI 0.921-2.156, P=0.114). Conclusions IgA nephropathy patients with IgM deposition have higher urinary protein, and more serious pathological damage and immune fluorescence deposition. IgM deposition affects renal survival of IgA nephropathy, while IgM deposition is not an independent risk factor for prognosis of IgA nephropathy.     

Clinico-pathological features and renal outcomes of primary IgA nephropathy with IgM deposition

Objective To investigate the clinico-pathological features and renal outcomes of primary IgA nephropathy (IgAN) with glomerular IgM deposition. Methods Primary IgAN diagnosed with biopsy from January 2006 to December 2011 were recruited. Patients were divided into groups according to IgM deposition (Group A) and without IgM deposition (Group B). In addition, Group A was subdivided into two groups based on the position of IgM deposits as the mesangium (Group A1) and both mesangium and capillary wall (Group A2). Renal outcomes were defined as end stage renal disease (ESRD) and/or the doubling of baseline serum creatinine. Clinico-pathological features were retrospectively compared. Kaplan-Meier was conducted for renal outcomes, and Cox regression model was used to analyze the prognostic value of IgM deposition and the position of IgM deposition in the progression of nephropathy in IgAN patients. Results 939 patients were enrolled with 422 (44.9%) having IgM deposition (Group A). Of the 422 patients, 382 patients were divided as Group A1, whereas 40 patients were noted as Group A2. Compared to Group B, hemoglobin, serum protein, albumin and serum IgG levels in group A were significantly lower, and the cholesterol and serum IgM levels were significantly higher (all P＜0.05). There was no significant difference in serum creatinine, estimated glomerular filtration rate (eGFR), urinary protein, blood pressure and uric acid between group A and B. In terms of pathological manifestations, patients in Group A exhibited more severe histological lesions including glomerular sclerosis, S1, M1 and interstitial inflammatory cell infiltration (all P＜0.05). Immunofluorescence showed that the proportion of IgG, C1q and Fg deposition in group A was significantly higher than that in group B (all P＜0.05). By Kaplan-Meier, cumulative renal survival rate has no significant difference between Group A and B (Log-rank test χ2=0.019, P=0.891). Univariate and multivariable Cox regression analysis showed that IgM deposition had no significant effect on the renal progression in IgAN patients. Subgroup analysis showed that patients in Group A2 exhibited higher urine protein, creatinine and blood pressure, and lower eGFR and serum albumin, also had worse histological lesions including M1, E1 and T1-2 of Oxford classification (all P＜0.05), Immunofluorescence showed that the proportion of IgG, C1q and Fg deposition in group A2 was significantly higher than that in group A1 (all P＜0.05). By Kaplan-Meier, renal survival rates calculated from outcomes were lower in Group A2 (Log-rank test χ2=18.207, P＜0.001). In addition, IgM deposited both in the mesangium and capillary wall was a risk factor for renal progression of IgAN patients with IgM deposition by a univariate Cox hazards regression mode and multivariable-adjusted Cox models (HR=3.621, 95%CI 1.924-6.814, P＜0.001; HR=2.309, 95%CI 1.176-4.533, P=0.015 respectively). Conclusions The IgAN patients with IgM deposition relatively had more severe clinico-pathological changes, especially those with IgM deposited both in the mesangium and capillary wall. In this study, IgM deposition was not found to be an independent risk factor for the prognosis of kidney in IgAN patients. However, IgM deposited both in the mesangium and capillary wall was an independent risk factor for renal prognosis in IgAN patients with IgM deposition.     

原发性IgA肾病中的微血管损害

目的 了解原发性IgA肾病中微血管损害及新月体形成(V/C)的临床、病理特点。方法 以2004年确诊并行肾穿刺活检证实的87例伴V/C损害的原发性IgA肾病与同期135例不伴V/C损害的原发性IgA肾病以及伴有V/C的狼疮肾炎患者的临床、病理资料进行比较。结果 原发性IgA肾病中较常发生V/C损害,发生率为39.19%;而V/C损害受累小球数占肾小球总数的(14.08±12.75)%。37.9%伴V/C损害的IgA肾病患者血清肌酐升高。血压、尿蛋白等临床指标在有与无V/C损害的两组IgA肾病间均无显著性差异。原发性IgA肾病患者的球性硬化发生率(135例/222例,64.86%)、球性硬化数与肾小球总数的比率[(26.98±24.68)%]均显著高于LN组[30例/73例,40.00%,(16.18±18.80)%]。结论 原发性IgA肾病中V/C损害发生率较高,出现常缺乏明显临床表现,并可能导致肾单位的缓慢、持续性、“非显性”丢失, 最终进展至终末期肾衰。     

Effect of serum uric acid level on prognosis in maintenance hemodialysis patients

Objective To analyze the relationship between serum uric acid (SUA) level and clinical indicators in maintenance hemodialysis (MHD) patients, and explore its influence on all-cause mortality and cardiovascular mortality. Methods This study was a retrospective cohort study. Patients who received MHD from the blood purification center of the Third Affiliated Hospital of Sun Yat-sen University from January 1, 2011 to December 30, 2015 were enrolled in the queue. They were divided into 3 groups according to the first and third quantile of the SUA level quartiles, and the baseline data of clinical and laboratory examinations were compared. The correlation between SUA level and clinical indicators was analyzed by Pearson correlation coefficient. Kaplan-Meier method and Cox proportional hazard regression model were used to examine the association between SUA and all-cause mortality and cardiovascular mortality in MHD patients. Results A total of 201 patients were enrolled in the study. The age of the patients was (56.9±16.7) years and the baseline SUA level was (531.1±137.9) μmol/L. Patients were divided into 3 groups with the first quantile (442 μmol/L) and the third quantile (620 μmol/L) of the SUA quartiles as the boundary points: group 1 (SUA＜442 μmol/L, n=52), group 2 (SUA 442-620 μmol/L, n=101) and group 3 (SUA＞620 μmol/L, n=48). The results showed that the patients in group 1 were older and had more proportion of patients with diabetes mellitus and cardiovascular diseases than those in group 3 (all P＜0.05). Compared to group 3, the serum albumin, serum phosphorus and serum creatinine were lower in group 1, while the hypersensitive C-reactive protein was higher (all P＜0.05). Pearson correlation analysis showed that SUA level was positively correlated with albumin (r=0.135, P=0.047), blood phosphorus (r=0.269, P＜0.001) and serum creatinine (r=0.333, P＜0.001), and negatively correlated with hypersensitive C-reactive protein (r=-0.216, P=0.002). After a median follow-up of 49.8 months, 66(32.8%) all-cause deaths and 32(15.9%) cardiovascular deaths were recorded. Kaplan-Meier method showed that with the decrease of SUA, all-cause mortality (Log-rank χ2=18.27, P＜0.001) and cardiovascular mortality (Log-rank χ2=15.04, P=0.001) increased. After adjusting for age, gender, comorbidity and other factors using the Cox proportional hazards model, the all-cause mortality and cardiovascular mortality decreased by 20.1% (HR=0.799, 95% CI 0.651-0.980, P=0.031) and 29.6% (HR=0.704, 95% CI 0.524-0.946, P=0.020) for each 100 μmol/L increase in baseline SUA. Compared to group 1, all-cause mortality (HR=0.332, 95%CI 0.142-0.774, P=0.011) and cardiovascular mortality (HR=0.140, 95%CI 0.030-0.657, P=0.013) were lower in the group 3. Conclusion Low SUA level increases the risk of all-cause mortality and cardiovascular mortality in MHD patients.     

伴恶性高血压IgA肾病的临床病理特征及其与肾血管病变的相关性

目的 分析伴恶性高血压IgA肾病（IgAN-MHT）的临床病理特征并探讨其与肾血管病变的相关性。 方法 从我科1997年4月至2007年5月间肾活检确诊的2000例原发性IgA肾病中筛选出29例IgAN-MHT患者，收集其临床、病理及预后资料。半定量分析肾小球、肾小管间质及血管（肾内微动脉、小动脉）的病理改变，包括436条入球动脉、124条小叶间动脉及5条弓形动脉。分析血管病变与肾脏病理改变、临床指标及预后的相关性。终点事件为基线Scr水平增加1倍或终末期肾病。 结果 原发性IgAN中MHT发生率约为1.5%。IgAN-MHT患者的临床表现主要为肾功能不全（100%）、高尿酸血症（62.7%）、高三酰甘油血症（51.7%），尿蛋白量（24 h）平均为2.8 g。常见肾脏病理改变为中度系膜细胞增殖、重度肾小球硬化、重度间质炎细胞浸润、重度肾小管萎缩及间质纤维化。IgAN-MHT患者的肾内小动脉（弓形动脉和小叶间动脉）及微动脉（入球动脉）均可受累。常见肾内血管病变特点为动脉闭塞、动脉中膜增厚、增生性动脉内膜炎（洋葱皮样改变、黏液样变性）、血管壁透明样变性，其中以肾内动脉闭塞为主（86.2%）。微动脉病变程度与年龄、总蛋白水平呈负相关；血管闭塞程度与尿酸水平呈正相关。平均随访21.1个月（1~84个月），14例患者达到终点。肾内微动脉病变是IgAN-MHT患者预后不良的主要危险因素(RR = 10.21，95%CI = 1.16~89.67)。 结论 IgAN-MHT的主要临床特点是肾功能不全；主要病理特征是以动脉闭塞为主的微动脉病变。微动脉病变是IgAN-MHT患者预后不良的主要危险因素。