IgA肾病呈单纯血尿和(或)轻度蛋白尿临床病理分析

目的 探讨单纯血尿和／或轻度蛋白尿IgA肾病患者病理特点及临床病理相关性,并进行危险因素分析。方法 选取肾脏疾病数据库1988-2001年我科收住的248例临床表现为单纯血尿和／或伴有轻度蛋白尿(尿蛋白定量≤1.0 g／d)的IgA肾病患者,血压、肾功能均正常,采用单盲法进行临床和病理分级(Haas分级),多因素回归分析法进行病理损伤的危险因素评估。结果(1)临床特点:49％(121例)患者同时存在血尿和蛋白尿,47％(117例)为单纯血尿,4％(10例)为单纯蛋白尿;(2)病理特点:35.5％(88例)病理损伤偏重,Haas分级Ⅱ-Ⅳ级。蛋白尿是病理损伤偏重(HaasⅡ级以上)的独立危险因素(OR=3.46,95％CI:1.54-7.79,P=0.002)。随着蛋白尿程度的增加,其病理损伤偏重的危险性亦明显增加。结论 相当一部分临床表现轻微的IgA肾病患者病理偏重,蛋白尿是危险因子,即使轻度蛋白尿亦和病理损伤明显相关。对于怀疑IgA肾病患者,尽管只有少量蛋白尿亦应进行肾活检,有助于发现病理损伤偏重的患者。     

IgA肾病中足细胞损伤及其与蛋白尿的关系

目的 观察 IgA肾病（IgAN）患者足细胞损伤的各种表现，探讨其与蛋白尿的关系。 方法 收集35例伴有明显蛋白尿[尿蛋白量（24 h）>1.0 g]的IgAN患者肾活检组织作研究；以8例肾错构瘤患者术后切除肾和肾癌患者术后远离癌旁肾组织为正常对照。免疫组化方法观察肾组织细胞周期调节蛋白（p21、p27）、足细胞结构蛋白（nestin）、足细胞数目 （WT1）。用显微切割方法取出肾小球，通过实时定量PCR方法检测整合素（integrin）β1、nephrin和α辅肌动蛋白4（α-actinin 4）水平。电镜观察足细胞超微结构的改变。根据足细胞数目密度(Nv, n×106/μm3)将35例IgAN患者分为足细胞数目减少组（ Nv<52.49×106/μm3，n = 15）和足细胞数目正常组（Nv≥52.49×106/μm3，n = 20）。随访蛋白尿的转归情况，共18个月。 结果 （1）与正常对照组比较，IgAN患者肾小球内个别足细胞重新表达p21，而足细胞p27的表达明显降低（0.71±0.12比0.91±0.07，P < 0.05）。（2）IgAN患者足细胞nestin 蛋白表达比正常对照显著降低（13.40%±0.04%比 17.60%±0.04%，P < 0.05）；肾小球内integrin-β1 mRNA表达显著升高（12.54±5.20比1.02±0.30，P < 0.05），而nephrin及α-actinin4 mRNA无明显改变。（3）电镜下观察到明显的足突融合和足细胞从基底膜脱落。（4）IgAN患者足细胞数目密度比正常对照组显著减少(161.27±225.92比323.22±138.12，P < 0.05)，且与Lee氏分级相关。（5）足细胞数目密度、integrin-β1 mRNA与肾穿刺当时的尿蛋白量（24 h）呈负相关(r = -0.4483、-0.840, 均P < 0.05)。足细胞数目减少组较足细胞数目正常组的蛋白尿下降程度明显减少（P < 0.05）。 结论 伴蛋白尿的IgAN中存在足细胞的损伤，表现为足细胞周期调节蛋白、结构蛋白的改变，足突的融合及足细胞数目的减少，而足细胞损伤及足细胞数目减少会影响蛋白尿的发生和发展。     

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。     

IgA肾病患者高血压的相关因素分析

目的探讨IgA肾病患者高血压的相关因素。方法经肾脏活体组织检查确诊的IgA肾病患者120例,采用单因素和多因素Logistic回归分析IgA肾病患者高血压发生的相关因素。结果120例IgA肾病患者中伴有高血压患者39例（占32．5％）。单因素分析发现,24h尿蛋白定量≥2．0g、尿素氮（BUN）≥8mmol／L、血肌酐（SCr）≥133μmol／L、肾小球率过滤（GFR）〈60ml·min^-1·（1．73m^2）^-1、高尿酸血症、贫血、肾小球慢性病变指数≥4分、肾间质炎症细胞侵润〉25％、肾小管萎缩和问质纤维化〉25％、肾小动脉管壁增厚、Lee分级Ⅳ～Ⅴ级与IgA肾病患者高血压相关。多因素Logistic回归结果显示,蛋白尿程度、GFR水平为IgA肾病高血压发生的独立危险因素。结论32．5％的IgA肾病患者伴有高血压,蛋白尿程度、GFR水平是高血压的独立危险因素。     

Association of secretory IgA with clinical pathological characteristics and complement activation in IgA nephropathy

Objective To further investigate the association among clinical pathology, complement activation and renal secretory IgA (SIgA) deposition in patients with IgA nephropathy (IgAN). Methods The activation of serum complements were detected by immunoturbidimetry and ELISA. Renal deposition of SIgA and activation of complements were detected by immunofluorescence. Then the association among clinical pathology, complement activation and renal SIgA deposition were analyzed in IgAN patients. Results In all 201 patients with IgAN,59 patients had SIgA deposition with higher incidences of mucosal infection history and hematuria (P＜0.05), lower levels of serum cystatin C, β2 microglobulin and lower tubulointerstitial lesion grades and T?grade in the Oxford classification (P＜0.05), when compared with patients without SIgA deposition. Both alternative and mannose binding lectin (MBL) pathways were activated in patients with or without SIgA deposition. Patients with MBL pathway activation had lower estimate glomerular filtration rate (P＜0.01), higher serum creatinine, higher proportion of glomerulosclerosis and S?grade in the Oxford classification, more severe tubulointerstitial lesion (P＜0.05). Conclusions Compared with patients without SIgA deposition, patients with SIgA deposition have a stronger link to mucosal immune. The deposition of SIgA is associated with different clinical and pathological manifestations; however, the complement activation is similar in both groups of patients. Patients with MBL pathway activation show more severe kidney injury.     

Analysis of the clinical pathological characteristics and prognosis of primary IgA nephropathy with hypertension

Objective To investigate the clinic-pathological features and prognostic risk factors of IgA nephropathy (IgAN) with hypertension (HTN). Methods Primary IgAN patients diagnosed with biopsy from January 2016 to December 2017 were recruited. Patients were divided into IgAN with normal blood pressure (IgAN-NTN) group and IgAN with hypertension (IgAN-HTN) group based on the pressure value when performing the kidney biopsy. The clinical and pathological data were collected and compared between the two groups. Kaplan-Meier method was conducted for renal results, whereas the Cox regression model was exploited to analyze the prognostic factors in the progression of IgAN-HTN patients. Results The total number of enrolled patients was 275 cases, 170 (61.82%) of which had normal pressure and 105 individuals (38.18%) resulted in hypertension. The IgAN-HTN group in terms of male proportion, age, systolic pressure, diastolic pressure, serum urea nitrogen, serum creatinine, serum uric acid, 24 h urinary protein, triacylglycerol, complement C4 and so on were higher than those in the IgAN-NTN group (all P＜0.05). The incidence of gross hematuria and the level of estimated glomerular filtration rate (eGFR) were significantly lower than those in the NTN group (all P＜0.001). For the aspect of light microscope pathological manifestations, IgAN-HTN group exhibited more severe histological lesions including glomerular sclerosis, renal tubular atrophy or renal interstitial fibrosis, interstitial vascular injury than IgAN-NTN group (all P＜0.05). Immunofluorescence examination results showed that the deposition ratio of C1q in IgAN-HTN group was higher than that in IgAN-NTN group (P=0.015). By employing Kaplan-Meier method, the cumulative renal survival rate in the HTN group was much lower than that in the NTN group (Log-rank test: χ2=6.456, P=0.011). For the patients in IgAN-HTN group, the cumulative renal survival rate in the dyslipidemia group was much lower than that in the ortholiposis group (Log-rank test: χ2=5.093, P=0.024). There was no significant difference in the cumulative renal survival rate between the blood pressure control group and the unqualified group (Log-rank test: χ2=1.036, P=0.309). As a result of univariate and multivariable Cox regression analysis, total cholesterol, eGFR and 24 h urinary protein were risk factors for renal progression of IgAN patients with hypertension. Conclusions The clinical manifestations and renal pathological changes in patients with IgAN-HTN are more serious than those in IgAN-NTN patients, which result in worse prognosis. IgAN-HTN patients should be paid more attention to the management of serum lipid level during treatment and follow-up.     

The disease characteristics of patients both with IgA nephropathy and diabetes mellitus

Objective To sum up and analyze the clinical and pathological characteristics in patients with both IgA nephropathy (IgAN) and diabetes mellitus. Methods A total of 500 patients were recruited, including 25 patients with both IgAN and diabetes mellitus, and 475 patients with IgAN only, who were diagnosed by renal-biopsy during Jan 2015 to Jan 2017 at the First Affiliated Hospital of Zhengzhou University. The clinical and pathological data were collected and analyzed using SPSS 22.0. Propensity Score Matching was used to match and select the patients in the both groups, and thereafter the depth of the basement membrane from the matched patients were compared using electron microscopy. The data of the patients whose follow-up time was ≥3 months were retrospectively collected, and Kaplan-Meier analysis was used to compare the difference of the prognosis. Results Compared to the patients with IgAN only, patients with both IgAN and diabetes mellitus were older [(46.36±13.49) years vs (34.00±13.80) years, P＜0.001], had higher level of serum triglyceride [2.06(1.52, 3.11) mmol/L vs 1.51(1.01, 2.25) mmol/L, P=0.012] and thicker basement membrane [(384.33±61.20) nm vs (346.72±52.65) nm, P=0.044]. The patients with both IgAN and diabetes mellitus were more prone to reach the composite endpoint [4/7(57.14%) vs 25/265(9.33%), P＜0.001] and had worse prognosis (Log-Rank test, P=0.004). Conclusions IgAN patients with diabetes mellitus have different clinical, pathological characteristics and prognosis from patients with IgAN alone. These patients need to be closely monitored and actively treated.     

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。     

原发性IgA肾病与非IgA系膜增生性肾小球肾炎的临床病理分析

目的 比较原发性IgA肾病与非IgA系膜增生性肾小球肾炎（non-IgA mesangial proliferative glomerulonephritis,non-IgA MsPGN）的临床及肾脏病理改变特点.方法 选择我科经肾活检确诊的原发性IgA肾病患者（A组）和non-IgA MsPGN患者（B组）进行临床与病理资料对比分析.结果 A、B组的性别、前驱上呼吸道感染诱因、起病时伴发高血压、镜下血尿、血肌酐无统计学差异（P＞0.05）.B组较A组起病年龄小,起病时伴发肉眼血尿比率低,肾病综合征发生率高,血IgG水平低,差异均有统计学意义（P＜0.05）.A组肾小球、肾小管间质、肾小动脉病理改变发生率高于B组（P＜0.05）,IgM、C3沉积、系膜区电子致密物沉积、大块状致密物、足细胞微绒毛化、肾小球基底膜分层发生率均较B组高（P＜0.01）.结论 IgA肾病与non-IgA MsPGN在临床表现、病理改变上存在明显差异,IgA肾病较non-IgA MsPGN病理损伤重.     

The correlation between serum levels of oxidative stress indicators and renal interstitial fibrosis in patients with IgA nephropathy

Objective To investigate the relationship between serum levels of oxidative stress indicators and the degree of renal interstitial fibrosis in patients with IgA nephropathy (IgAN). Methods Seventy eight patients with confirmed primary IgAN in General Hospital of Ningxia Medical University from January 2013 to December 2014 were enrolled. The patients were divided into T0 group (n=30), T1 group (n=26) and T2 group (n=22) according to the grade of tubular atrophy/interstitial fibrosis of Oxford pathological classification criteria for IgAN in 2009. Meanwhile, thirty cases of health examiner were enrolled as control subjects. The levels of serum malondialdehyde (MDA), superoxide dismutase (SOD), catalase (CAT) and glutathione peroxidase (GSH-Px) were detected by xanthine oxidase method, thiobarbituric acid spectrophotometry method, ultraviolet spectrophotometry method, chemical colorimetric method, respectively. The levels of serum advanced oxidation protein products (AOPPs), transforming growth factor beta 1 (TGF-β1), monocyte chemotactic protein 1 (MCP-1), transforming growth factor alpha (TGF-α), interleukin 6 (IL-6) and hypoxia inducible factor 1 alpha (HIF-1α) were detected by enzyme linked immunosorbent assay (ELISA) in all groups. Spearman correlation analysis was used to analyze the correlation between serum oxidative stress indicators and traditional risk factors of tubular atrophy/renal interstitial fibrosis. Multivariable linear regression analysis was used to analyze the correlation between oxidative stress indicators and degree of renal tubular atrophy/renal interstitial fibrosis. Results There were differences in serum levels of AOPPs, MDA, SOD, CAT and GSH-Px in IgAN patients with different degrees of renal interstitial fibrosis (all P﹤0.05). With the increase of renal interstitial fibrosis, the levels of AOPPs and MDA increased gradually, while the levels of SOD, CAT and GSH-Px decreased gradually. Serum AOPPs, MDA, SOD, CAT, GSH-Px concentration in IgAN patients were correlated with the mean arterial pressure (MAP), total blood protein (TP), albumin (Alb), Scr, uric acid (UA), 24-hour urinary protein volume and estimated glomerular filtration rate (eGFR). Multivariate regression analysis showed that the AOPPs levels of blood were positively correlated with MAP, Scr, UA and 24-hour urinary protein (all P﹤0.01), and negatively correlated with TP, Alb, eGFR (all P﹤0.05). The serum levels of AOPPs and MDA in IgAN patients were positively correlated with the levels of TGF-β1, MCP-1, TGF-α, IL-6 and HIF-1α. The levels of SOD, CAT and GSH-Px were negatively correlated with the levels of TGF-β1, MCP-1, TGF-α, IL-6 and HIF-1α. Multivariate stepwise regression analysis showed that the degree of renal interstitial fibrosis in IgAN patients was positively correlated with serum AOPPs level (β=0.285, P=0.001), negatively correlated with CAT (β=-0.346, P﹤0.001), GSH-Px (β=-0.303, P﹤0.001). Conclusions The level of serum oxidative stress in IgAN patients is elevated and positively correlated with the degree of renal interstitial fibrosis, suggesting that oxidative stress may be involved in the occurrence and development of renal interstitial fibrosis.     

血浆凝血因子VIII水平与IgA肾病患者临床病理改变及预后的关系

目的探讨血浆凝血因子VIII(factor VIII,FVIII)水平与IgA肾病(IgAN)患者临床参数及预后的关系。方法收集2016年1月至2016年12月中南大学湘雅二医院确诊的IgAN患者的临床资料。按照时间依赖的受试者工作特征曲线(ROC)得出的血浆FVIII预测IgAN预后的临界值,将患者分为高FVIII组(FVIII>140.50%)和低FVIII组(FVIII≤140.50%),比较两组患者肾活检时基线临床参数的差异。以估算肾小球滤过率(eGFR)下降≥30%或进入终末期肾脏病(ESRD)为终点事件,采用Kaplan-Meier生存曲线及Cox回归方程法分析血浆FVIII水平对IgAN患者预后的影响。结果共93例IgAN患者纳入本研究,中位随访时间为35.15(33.77,36.76)个月,12例(12.90%)患者发生终点事件。高FVIII组患者年龄、血肌酐、尿素氮、血三酰甘油、血总胆固醇、血浆纤维蛋白原、D-二聚体、24 h尿蛋白量、蛋白C、蛋白S和eGFR下降速率高于低FVIII组(均P<0.05);eGFR、血白蛋白、中位随访时间低于低FVIII组(均P<0.05)。Kaplan-Meier生存分析结果显示,与低FVIII组比较,高FVIII组患者肾脏累积生存率降低(χ2=5.635,P=0.018)。在校正收缩压、eGFR、尿蛋白、肾小管萎缩/间质纤维化程度等因素后,多因素Cox回归分析结果显示,高血浆FVIII水平是IgAN患者肾脏预后不良的独立危险因素(HR=4.147,95%CI 1.055~16.308,P=0.042)。结论血浆FVIII水平与IgAN患者临床指标及预后相关,高血浆FVIII水平是IgAN患者肾脏预后不良的独立危险因素。     

IgA肾病肾组织中基质金属蛋白酶2和单核细胞趋化因子1的表达

目的探讨基质金属蛋白酶2（MMP-2）和单核细胞趋化因子-1（MCP-1）在IgA肾病肾组织的表达强度及其在该病早期肾损害发病机制中的作用。方法将30例IgA肾病患者经肾脏活体组织检查的。肾组织和1（）例切除肾肿瘤患者正常肾组织,进行MMP-2、MCP-1、Ⅳ型胶原免疫组织化学检测并进行半定量分析,按其组织学改变Lee分级标准和肾小管间质病变程度进行分组和分级,同时检测肾功能、24h尿蛋白定量等临床指标,并与组织学、免疫组织化学检测结果进行相关分析。结果在IgA肾病轻度肾损害阶段,MMP2和MCP-1表达水平显著高于正常对照（P〈0．05）,随着病变级别加重,MMP-2和MCP-1在肾小管间质的表达呈逐渐减少趋势（P〈0．05）,而Ⅳ型胶原在肾小管间质表达随肾损害加重呈逐渐增加趋势（P〈0．01）。Spearman相关分析显示,MMP-2和MCP-1表达水平与肾小管间质炎症细胞浸润程度呈正相关（P〈0．05）,与Ⅳ型胶原表达水平呈负相关（P〈0．05）。结论MMP-2和MCP-1可能参与IgA肾病早期单个核细胞浸润等早期肾损害发病机制。     

IgA肾病患者尿足细胞数及肾组织podocalyxin表达与临床病理的相关分析

目的 探讨尿足细胞数和肾组织中足细胞顶膜区的特异性标记蛋白podocalyxin（PCX）的表达与IgA肾病(IgAN)患者临床和病理改变的关系。 方法 收集50例IgAN患者活检前3 d的每天晨尿和20例体检健康对照者的晨尿各100 ml,离心去上清于TXD3细胞离心涂片机上制成涂片。用抗人PCX单克隆抗体分别对尿沉渣涂片和肾组织切片进行免疫组化染色,于光学显微镜下计数尿足细胞排泄数,以及用计算机图像分析系统测量和计算肾小球PCX平均吸光度值。IgAN按Lee分级分成5组,并用Katafuchi半定量积分法记分。比较各组尿足细胞数和肾组织PCX平均吸光度值,并与各项病理指标评分和临床生化指标进行相关性分析。 结果 IgAN组尿中足细胞数显著高于健康对照组（P < 0.01）。IgAN Lee分级各组组间尿足细胞中位数两两比较,Ⅰ-Ⅱ级组低于Ⅲ、Ⅳ、Ⅴ级组（P < 0.05）;Ⅲ级组显著低于Ⅴ级组（P < 0.05）;Ⅲ级组低于Ⅳ级组和Ⅳ级组低于Ⅴ级组,但差异无统计学意义。尿足细胞阳性率在Ⅳ、Ⅴ级组最高（100%）,在LeeⅠ-Ⅱ级组最低（55%）。IgAN患者随着Lee分级升高,肾小球足细胞PCX表达下调,两两比较结果显示,Ⅰ-Ⅱ级组显著高于Ⅲ、Ⅳ、Ⅴ级组（P < 0.05）;Ⅲ、Ⅳ级组显著高于Ⅴ级组（P < 0.05）;Ⅲ级组稍高于Ⅳ级组（P > 0.05）。IgAN患者尿足细胞排泄数与肾小球PCX表达量呈负相关（r = -0.702,P < 0.01);与24 h尿蛋白量呈正相关（r = 0.465,P < 0.01）;与肾小球和肾小管病理积分均呈正相关（r = 0.233,r = 0.307,均P < 0.05）。肾小球PCX表达量分别与肾小球和肾小管病理积分呈负相关（r = -0.560,r = -0.377,均P < 0.05）;与24 h尿蛋白量呈负相关（r = -0.367,P < 0.05）。 结论 IgAN患者尿足细胞排泄量可反映肾组织足细胞缺失程度,肾小球足细胞损伤脱落可能参与IgAN的发生发展,其尿足细胞数有可能作为反映疾病进展的重要指标。     

IgA肾病牛津病理分型适用性及其与临床关系的研究

目的探讨应用IgA肾病牛津分型法的临床适用性及其与临床指标的关系。方法收集191例原发性IgA肾病患者肾活检前血肌酐（SCr）,24h尿蛋白定量、平均动脉压（MAP）等临床资料;依据IgA肾病牛津分型定义对肾组织切片进行病理分型;对不同牛津分型间肾活检前SCr、MAP、尿蛋白定量进行比较;对随访时SCr〉129．9μmol／L的26例患者就临床有效性进行预后多因素Logistic回归分析。结果肾组织伴有S1、T1—2型的患者比S0、T0型者。肾活检前SCr水平高（P〈0．05）。24h尿蛋白定量在M、S、E分型间无统计学差异。MAP在伴有S1、T1—2型患者中高于S0、T0型患者（P〈0．05）;多因素Logistic回归分析显示伴有T1—2是导致疗效不佳的独立危险因素。结论牛津分型实际应用仍有局限性;IgA肾病患者伴有T1-2是导致疗效不佳的独立危险因素。     

不同性别成人IgA肾病患者临床特征

目的了解不同性别成人原发性IgA肾病（IgAnephropathy,IgAN）患者的临床特征。方法选择我科1996年3月至2009年11月经肾活检确诊为kAN的成人患者192例,对比分析男性和女性患者各临床表现和病理积分特点。结果成人IgAN患者中,不同性别患者的病程、发病年龄、血胆固醇、清蛋白、血IgA、血C3、尿红细胞计数和估算肾小球滤过率值的差异无统计学意义,但男性收缩压、舒张压、血肌酐、尿素氮、血尿酸及24h尿蛋白定量明显高于女性,差异显著。男性高血压、高尿酸血症、高三酰甘油血症、24h尿蛋白定量〉1g的比例显著高于女性。男女患者的肾小球积分、肾小管间质积分、血管积分及慢性病变、活动性病变积分差异均无统计学意义。肾功能中度损伤的独立危险因素为高尿酸血症（r=9．146,95．0％CI：2．322-36．026,P〈0．01）、总积分（r=2．772,95．0％CI：1．709～4．496,P〈0．01）、肾小球积分（r=0．522,95．0％CI：0．287-0．952,P〈0．05）。结论成人IgAN患者肾活检时男女患者间的肾功能及。肾脏病理改变并无明显差异,但男性患者相关临床病变重,血压、血肌酐、血尿酸及24h尿蛋白定量高,需积极干预延缓其进展。     

Related factors of hyperuricemia in IgA nephropathy patients

Objective To investigate the influencing factors of hyperuricemia in patients with IgA nephropathy (IgAN). Methods A retrospective study was performed in patients with renal biopsy diagnosed as IgAN in the Department of Nephrology, Provincial Hospital of Anhui Medical University from January 2016 to October 2018. According to the blood uric acid level, they were divided into two groups: patients with hyperuricemia and patients without hyperuricemia. The general clinical indicators and renal pathological data were compared between the two groups. Logistic regression model was used to analyze the influencing factors of hyperuricemia in IgAN patients. Results A total of 125 IgAN patients with age of (35.70±11.16) years old were enrolled, including 63 males and 62 females. The morbidity of hyperuricemia was 44.0%(55/125). Compared with the normal blood uric acid group, the blood urea nitrogen, serum creatinine and the proportion of chronic kidney disease (CKD) stage 3-5, small arterial wall thickening, fibrous crescents/globules, renal interstitial fibrosis, renal tubular atrophy, glomerular sclerosis and inflammatory cell infiltration in the hyperuric acid group were higher, while the level of estimated glomerular filtration rate (eGFR) was lower. And the differences were statistically significant (all P＜0.05). Multivariate logistic regression analysis showed that the level of serum creatinine was an independent related factor of hyperuricemia in IgAN patients (OR=1.034, 95%CI 1.005-1.064, P=0.021). Conclusions IgAN patients with hyperuricemia presented more severe glomerular, tubular and interstitial lesions, and the level of serum creatinine is an independent related factor of hyperuricemia in IgAN patients. High uric acid level may have an important influence on the progression of IgAN, so good control of serum uric acid may improve the prognosis of patients with IgAN.     

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.     

伴有高脂血症的IgA肾病临床和病理分析

目的 探讨伴有高脂血症的IgA肾病临床表现和病理特点的关系.方法 回顾性分析我科经肾活检诊断为IgA肾病86例患者的一般情况、临床表现、病理资料.根据病程中患者血清脂质水平,分为高脂血症组40例和非高脂血症组46例.比较2组临床和病理资料.结果 高脂血症组血尿酸和24 h尿蛋白定量高于非高脂血症组,估算肾小球滤过率(e...     

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。     

血清低半乳糖化IgA1测定对鉴别IgA肾病的临床价值

目的 确定血清低半乳糖化IgA1对鉴别诊断IgA肾病的临床价值。 方法 以原发性肾小球疾病患者91例为研究对象，接受肾活检并留取血清；以健康体检者20例血清作为对照。血清标本先用装有耦联蚕豆凝集素的微球进行微量离心柱法分离并洗脱，获得低半乳糖化IgA1。再以凝集素HAA（Helix aspersa）用ELISA法定量检测异常糖基化IgA1（HAA-IgA1）。分析血清低半乳糖IgA1升高在鉴别诊断IgA肾病方面的临床价值。 结果 48例IgA肾病患者HAA-IgA1水平[（83.7±41.0） U]高于健康对照组[（52.6±22.9） U]及43例其他原发性肾小球疾病患者组[（49.2±27.3） U]（均P < 0.01）。而该43例中，非IgA系膜增殖性肾炎患者22例（51%）的HAA-IgA1水平[（47.6±21.5） U]亦显著低于IgA肾病患者。以肾穿刺病理诊断为金标准，所绘制ROC曲线面积为0.797，面积的标准误为0.047（P < 0.01）；鉴别诊断IgA肾病的灵敏度为72.9%，特异度为72.1%，准确度为72.5%。 结论 应用微量离心柱法联合ELISA法检测IgA肾病患者血清低半乳糖IgA1对于鉴别诊断IgA肾病具有一定临床价值。