IgA肾病小管间质损害与预后相关分析

目的观察IgA肾病患者肾小管间质损害与临床表现及肾组织病理改变之间的关系。方法选取经临床病理诊断明确的IgA肾病患者143例,根据小管间质损害的程度进行临床和病理指标的比较。结果①143例IgA肾病患者中,83．92％出现小管间质损害,其中轻度占65．03％,中度占16．08％,重度占2．80％。②随着小管间质损害程度的加重,女性患者所占比例逐渐增大,年龄和血压呈逐渐升高的趋势,血浆白蛋白水平呈下降趋势,而24h尿蛋白定量、血肌酐、总胆固醇水平呈明显升高趋势。③随着小管间质损害的加重,患者肾小球损害和血管损害也随之加重（P〈0．05）。结论小管间质损害是决定IgA肾病预后的关键因素之一,但不是唯一因素,应将临床参数与病理参数,间质损害指标与肾小球、肾血管损害指标相结合,综合评价患者预后。     

伴高尿酸血症IgA肾病的临床、病理特点及预后的相关分析

目的：研究伴高尿酸血症IgA肾病患者的临床、病理特点及预后的相关因素。方法：对2006年～2009年经肾活检确诊为IgA肾病的72例患者的临床与病理资料进行分析。根据血尿酸水平,将72例IgA患者分为高尿酸血症组与血尿酸正常组,比较两组间临床指标、肾功能指标及病理学参数。结果：临床指标中年龄、血尿酸及尿蛋白定量均与血肌酐、肾小球滤过率有显著相关性（P〈0.05）。多元逐步回归分析血尿酸对IgA肾病肾功能进展的预测优于尿蛋白定量。病理参数中肾小球积分、肾小管间质积分与血肌酐、肾小球滤过率显著相关（P〈0.05）。高尿酸血症组肾功能指标均差于血尿酸正常组。高尿酸血症组肾小管间质损害均高于血尿酸正常组。结论：IgA肾病的预后主要与尿蛋白定量、血尿酸、肾小管间质损害、球性硬化有关,IgA肾病37.5%患者合并高尿酸血症、其临床尿蛋白、肾功能损害及病理肾小管间质损害均高于血尿酸正常组IgA肾病患者,应重视血尿酸在IgA肾病中的作用。     

儿童IgA肾病肾小管间质损害与其他临床病理指标的关系探讨

目的:探讨在儿童IgA肾病中肾小管间质损害与其他临床及病理指标的关系。方法:回顾性分析2014年01月~2017年09月在我院肾活检确诊的119例原发性IgA肾病患者的资料,对比不同程度及类型的肾小管间质损害与其他临床及病理指标之间的关系。结果:肾间质损害总体程度与年龄、性别、体块指数、肉眼血尿发生率、内皮细胞增生程度、肾小球硬化分值、纤维型新月体的发生没有关系。随着肾间质损害的加重,肾小球系膜细胞及基质增殖程度、细胞型及混合型新月体的发生率、尿蛋白的排出量逐渐增高同时肾小球滤过率逐渐下降。间质炎性细胞浸润与内皮细胞增生及细胞型新月体的发生关系密切,而存在肾小管萎缩和肾间质纤维化的患者更容易出现肾小球硬化。结论:IgA肾病患儿的肾小管间质损害与肾小球病变程度相平行,其与肾小球系膜细胞及基质增生程度、新月体形成、与蛋白尿及肾小球滤过率的改变密切相关。不同的小管间质病理类型与其他病理指标的关系各有区别但与各项临床指标的关系没有差别。     

单纯性肾小球性血尿90例临床和病理分析

目的分析成人单纯性肾小球性血尿临床和病理特点，探讨血尿类型与病理严重程度的关系，寻找能较好反映单纯血尿患者肾脏病变程度的指标。方法选取90例单纯性肾小球性血尿患者为研究对象，分析不同病理类型的临床和病理特点；比较不同血尿类型以及不同肾小管间质损害程度患者的临床和病理学指标。结果90例患者中IgA肾病32例、局灶增生性肾小球肾炎27例，为最主要的病理类型。镜下血尿组各指标中除球性硬化、血管玻璃样变及IgA沉积外，其余各临床与病理指标均与肉眼血尿组无明显统计学差异。随着单纯血尿患者肾小管间质损害程度的加重，各病理参数积分均显著增加。结论成人单纯性血尿最主要的原因是IgA肾病，病理类型以局灶增生性肾小球肾炎最多见。不能单纯以血尿类型来判断肾脏病理严重程度。肾小管间质损害可以反映单纯性血尿患者的病变程度。     

肾小球疾病中肾小管间质损害的研究进展

1990年,Bogenschutz等研究发现IgA肾病患者,若肾小球病变较重,但无明显间质损害者预后良好;而间质损害严重,肾小球病变不明显者预后不佳,并提出了肾脏间质纤维化是预测肾脏病情的重要指标。随后肾间质损害在肾脏病中的作用受到国内外学者的重视,相继的一些研究表明,肾脏间质纤维化对肾病的预后有着重要影响。因此,了解间质损害的机制,控制间质损害的发展对延缓肾脏疾病的进展有着重要意义。一、流行病学临床上,肾小管间质病变与肾功能进展和预后有密切联系。近20年有关慢性肾小球疾病进展和预后相关的临床和病理改变的临床研究,对象集中于全世界发病率较高及预后较差的几种肾病,如IgA肾病、膜性肾病、局灶节段性肾小球硬化（FSGS）等。在膜性肾病的多因素相关分析表明肾小管间质病变是决定10年肾脏存活率的最重要影响因素,其影响程度甚至超过了肾小球疾病本身。在FSGS中也     

122例老年人肾脏病的临床与病理分析

目的 探讨老年人肾脏病的临床与病理.回顾性分析122例住院行肾活检的老年人临床与病理资料。结果 ①临床特点：以肾病综合征最为常见，其次为慢性肾炎综合征、慢性肾功能不全，急性肾功能不全及发作性肉眼血尿较少见。②病理类型特点：原发性肾小球疾病占60．66％，以膜性肾病最为多见（19．67％）；继发性肾小球疾病占30．34％，以糖尿病肾病最为多见（9．02％）；肾小管－间质病变中间质性肾炎较多见（6．56％）。③病理改变特点：与青壮年相比，肾小管萎缩、间质炎细胞浸润、间质纤维化、血管壁增厚更为多见。④临床特点与病理类型的关系：以肾病综合征为临床表现者，病理类型原发性者以膜性肾病为主，继发性者以糖尿病肾病为主；以慢性肾炎综合征或慢性肾功能不全为临床表现者病理类型以IgA肾病为主；发作性肉眼血尿只见于系膜增殖性肾小球肾炎和IgA肾病；以急性肾功能不全为临床表现者病理上多为急性肾小管坏死或间质性肾炎。结论 老年人肾脏病临床与病理有着自己的特点：①临床上以肾病综合征最为常见。②病理类型上继发性肾小球疾病增加，糖尿病肾病成为主要原因。③病理改变特点上肾小管－间质及血管病变较重，可能与老龄化有关。④不同的临床表现可能提示不同的病理类型。     

福建地区571例IgA肾病流行病学特点

目的探讨福建地区IgA肾病的流行病学及临床病理特点。方法回顾性分析本地区571例IgA肾病的流行病学及临床病理特点。结果发病年龄≤20以下占17．0％，21～30岁占36．1％），31～40岁占24．5％，41～50岁占21．7％，50岁以上仅为1．7％。蛋白尿发生率高达93．0％；伴高血压病者15．9％0，肾功能不全者14．3％。临床表现以无症状性尿检异常型最常见（占39．4％0），病理类型以弥漫性系膜增生性肾小球肾炎（MsPGN）伴局灶节段肾小球硬化（FSGS）最为常见（占31．9％），MsPGN占27．5％，局灶节段系膜增生性肾炎（FsPGN）占22．6％，其他类型占18．0％），肾小管间质损害以中度损害为主（71．6％）。免疫病理类型以IgAMG型占53．1％，IgAG型占21．0％，其他类型占25．9％。IgA肾病出现慢性肾功能不全时，以FSGS病理类型最常见。结论福建地区IgA肾病流行病学及临床病理存在一定的地区特点：老年人比例少，蛋白尿比例高，最常见病理类型是MsPGN伴FSGS，免疫病理以IgAMG型最常见，肾小管间质病变较重。     

HBV-GN肾小管间质损害的发生影响因素及临床意义

目的:探讨乙肝相关性肾炎(HBV-GN)中肾小管间质损害(TIL)的发生情况、影响因素及临床意义。方法:单因素及多因素分析227例HBV-GN患者TIL发生的影响因素,比较不同程度TIL患者的临床病理资料。结果:肾小管间质损害轻度者占59.5%,中度者占17.6%,重度者占4.8%,无肾小管间质损害者仅占18.1%。不同病理类型HBV-GN患者其TIL发生率各不相同,年龄>14岁、高血压、尿蛋白≥0.3g/d、血肌酐≥132.6μmol/L、球囊黏连、新月体形成、系膜增殖程度和肾小球硬化率是TIL发生的危险因素,其中高血压、系膜增殖程度和肾小球硬化率是TIL发生的独立影响因素;随着肾小管间质损害的发生或加重,尿蛋白≥0.3g/d、高血压、高尿酸血症、肾功能异常和上述4种肾小球病理改变的发生率亦增高。结论:肾小管间质损害在HBV-GN患者的十分常见,高血压、系膜增殖程度和肾小球硬化率是TIL的独立危险因素,TIL发生或加重对其肾小球病理改变也有明显影响,是影响HBV-GN预后的不良因素之一。     

^99mTc—DTPA肾动态显像在IgA肾病慢性肾功能不全患者的临床应用

目的探讨^99mTc-DTPA肾动态显像在IgA肾病（kAN）慢性肾功能不全患者病情监测及预后判断时的应用价值。方法23例经肾活检确诊为IgAN的患者行常规^99mTc-DTPA肾动态显像，测定肾小球滤过率（GFR，包括总GFR和分肾GFR），同时行肾活检按Lee氏分级标准将肾脏病理改变分级，Katafuchi积分标准对患者的肾脏病理损害，包括肾小球损害、肾小管间质损害和血管损害进行积分，将积分与上述显像指标（GFR）进行相关分析。结果随着病理分级增高，GFR趋于降低，各级组间比较差异有显著性意义（P〈0．05或P〈0．01）；相关分析示GFR与肾脏病理总积分、肾小球损害（尤其是节段损害和全球硬化）、小管间质（尤其是间质纤维化和肾小管萎缩）及血管损害呈负相关（P〈0．05或P〈0．01）；在临床指标方面，GFR与血清肌酐和尿蛋白定量及血压（收缩压和舒张压）呈负相关（P〈0．05或P〈0．01）。结论通过^99mTc-DTPA肾动态显像获得的GFR与IgA肾病肾脏病理学改变及临床指标关系密切，其在IgA肾病患者病情监测、指导治疗及预后判断中具有重要临床应用价值。     

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。     

A clinicopathological study of IgA nephropathy in renal transplant recipients: beneficial effect of angiotensin-converting enzyme inhibitor.

BACKGROUND: Prolonging the survival of transplant kidneys is a major task of modern nephrology. It has recently been shown that deteriorating renal function and substantial graft loss were observed in 55% of renal allograft recipients with recurrent IgA nephropathy (IgAN) at long-term follow-up. To gain a useful insight into the therapeutic approach towards protecting allograft kidneys from deteriorating graft function, we compared the histological characteristics of post-transplant IgAN to primary IgAN and investigated the effects of an ACE inhibitor. METHODS: Twenty-one patients with post-transplant IgAN and 63 patients with primary IgAN were included in the histopathological study. The effectiveness of angiotensin-converting enzyme (ACE) inhibitor treatment in post-transplant IgAN was also studied in 10 patients. RESULTS: The prevalence of glomeruli with adhesions and/or cellular crescents in primary IgAN was significantly greater than in post-transplant IgAN (P<0.05), but the proportion of glomeruli with segmental sclerosis was similar in both groups. The rate of global obsolescence, and the degree of interstitial fibrosis in post-transplant IgAN were significantly greater than in primary IgAN (P<0.05). The degree of glomerular obsolescence and the severity of interstitial fibrosis correlated with the severity of glomerular lesion in primary IgAN, but not in post-transplant IgAN. In primary IgAN, glomerular diameter significantly correlated with the proportions of glomerular obsolescence, but not in post-transplant IgAN, suggesting that allograft kidneys may be in a hyperfiltration state. Both the blood pressure and the urinary protein excretion significantly improved after ACE-inhibitor treatment (P<0.001). CONCLUSION: In post-transplant IgAN, histopathological lesions indicative of acute inflammatory insults were suppressed, and glomerular hypertrophy, which may relate to haemodynamic burden such as hyperfiltration, was prominent. Preliminary study of ACE-inhibitor treatment in 10 patients showed favourable effects. A future long-term follow-up study is required to establish the effectiveness of ACE inhibitors in treatment of post-transplant IgAN.     

Clinicopathologic study on prognostic markers in IgA nephropathy

BACKGROUND/AIM: Few prognostic markers have found general acceptance in IgA nephropathy (IgAN). The aim of the present study was to search for significant predictor(s) at the time of biopsy. METHODS: Fifty-five patients with IgAN undergoing evaluation and treatment at our institution were examined regarding clinicopathologic features at the time of renal biopsy and, if possible, at follow-up. Factors predictive of outcome were evaluated. Renal histopathology was quantified using a glomerulosclerosis index (GSI), a tubulointerstitial index (TII), and a crescent index (CI). RESULTS: The serum creatinine concentration (S-Cr) showed positive correlations with proteinuria and serum total cholesterol concentration, as well as with histopathologic findings. Heavy proteinuria (> or =3.0g/24 h) was associated with higher S-Cr and greater severity of pathologic abnormalities than with milder proteinuria. At follow-up, 6 patients progressed to chronic renal insufficiency, in whom the S-Cr increased by at least 50% to reach or exceed 1.5 mg/dl (132.6 micromol/l). By univariate analysis, elevated GSI, TII, and S-Cr, presence of nephrotic syndrome, elevated CI, and elevated total cholesterol were found to be negative predictors, in descending order of odds ratio. In multivariate analysis, however, only TII independently predicted unfavorable outcome. Conclusion: Renal biopsy in IgAN may be the most powerful predictor for renal outcome; an advanced tubulointerstitial lesion is unfavorable.     

固本通络冲剂治疗IgA肾病的疗效特点分析

目的：研究中药复方固本通络冲剂治疗IgA肾病的疗效特点。方法：对肾活检证实的IgA肾病息随机分为固本通络冲剂治疗组(50例)及潘生丁对照组(30例)进行疗效特点分析，疗程3个月。结果：其疗效特点为对病理损害轻，临床表现为持续镜下血尿伴少量蛋白尿，中医辨证为气阴两虚的病人疗效最好；而对病理损害重，临床表现为肾病综合征，中医辨证为脾肾气虚疗效差。结论：固本通络冲剂对临床表现为持续镜下血尿伴少量蛋白尿，中医辨证属气阴两虚更为适宜。     

Characteristics and risk factors of intrarenal arterial lesions in patients with IgA nephropathy.

BACKGROUND: Although the clinical importance of immunoglobulin-A nephropathy (IgAN) is widely recognized, the characteristics of intrarenal arterial lesions in this disease and the main factors associated with them have not been studied extensively, and a large-scale analysis of intrarenal arterial lesions in IgAN has not been performed. METHODS: To clarify these issues, we investigated the prevalence, underlying factors and significance of intrarenal arterial lesions in 1005 patients with IgAN. We distinguished different degrees of severity of small artery and arteriolar lesions (mild, moderate and severe), using a semi-quantitative scoring system. We compared the arterial lesions of IgAN patients with those of 627 non-IgAN patients, who had mesangial proliferating glomerulonephritis without IgA deposits, and of 221 patients with membranous nephropathy (MN). RESULTS: The IgAN patients with arterial lesions were significantly younger than the non-IgAN and MN patients (mean ages 34.6 vs 40.4 and 47.7 years, respectively). The prevalence of intrarenal small artery and arteriolar lesions was 54.6% in IgAN patients, compared with 26.6 and 47.1% in non-IgAN and MN patients, respectively; the percentages of moderate/severe arterial lesions were 37.0 vs 21.6 and 23.1%, respectively; and the percentages of hyaline changes were 43.7 vs 16.8 and 21.2%, respectively. The differences in the prevalence of lesions between IgAN patients and the two other groups were statistically significant for all three parameters. Our search for possible relationships between arterial-arteriolar lesions and various indirect outcome markers disclosed significant associations with hypertension, higher serum creatinine and uric acid, high urinary protein excretion, glomerulosclerosis, tubular atrophy and interstitial fibrosis. Furthermore, these parameters were changed more markedly in IgAN patients with moderate/severe arterial lesions and hyaline changes than in IgAN patients who had mild arterial lesions and wall thickening alone. CONCLUSIONS: The prevalence of small intrarenal arterial-arteriolar lesions was higher in IgAN patients than in non-IgAN and MN patients; moreover, the lesions in IgAN patients were associated with younger age, were more severe and exhibited a higher degree of hyaline changes. Finally, the severity of small arterial- arteriolar lesions was linked to several markers of adverse outcome.     

毛细血管内增生性病变在IgA肾病中的研究现状

IgA肾病是以系膜增生为主要特征的原发性肾小球疾病,也可以伴有不同程度的毛细血管内增生性病变。本文结合相关文献,对合并毛细血管内增生性病变的IgA肾病和弥漫性毛细血管内增生型IgA肾病的临床病理及预后特点进行综述,以阐明毛细血管内增生性病变在IgA肾病中的临床意义。     

合并毛细血管襻纤维素样坏死的原发性IgA肾病患者的预后

目的 初步探讨合并毛细血管襻纤维素样坏死的IgA肾病(IgAN)的临床病理特点及纤维素样坏死对IgAN近期预后的影响。方法 回顾分析1997年至2004年10月在北京大学第一医院肾内科经肾活检诊断资料完整的780例原发性IgAN。在46例伴有纤维素样坏死的患者中，35例有完整的随访数据，平均随访时间26个月。随机选取同期的80例无毛细血管襻纤维素样坏死的IgAN患者，平均随访时间39个月，比较两组患者的临床病理及预后情况。终点事件定义为Scr上升50%或Ccr下降33%或ESRD。采用Kaplan-Meier方法进行生存分析及Cox风险比例模型筛选预后危险因素。结果 坏死组肾组织的淋巴单核细胞浸润更为明显(P = 0.004)；肾小球硬化比率较低(P = 0.002)。坏死组中仅有2例以急性肾功能衰竭(ARF)起病，3例达随访终点；80例非坏死组患者中有14例达终点。多因素分析提示，纤维素样坏死不是IgAN预后的独立危险因素，而严重慢性化病变是唯一提示预后不良的危险因素(RR = 23.13， P < 0.01)。结论 合并纤维素样坏死的IgAN在临床病理方面并无显著特点，纤维素样坏死不影响IgAN的短期预后。     

Glomerular growth under cyclosporine treatment in childhood nephrotic syndrome

BACKGROUND: Glomerular hypertrophy is important in children with idiopathic nephrotic syndrome in regard to diagnosis and pathogenesis. Moreover, glomerular growth may be altered by cyclosporine (CsA) treatment in these patients. METHODS: Bowman's area (BA) and the glomerular tuft area (GA) of pre- and post-treatment biopsies was measured by morphometry in 47 children with idiopathic nephrotic syndrome (39 MCD and 8 FSGS) treated with CsA and low-dose prednisolone for up to 2 years. RESULTS: BA and GA increased with age. The mean BA and GA were 1.2 times larger in FSGS than in MCD and the proportional increase was similar in both diseases after treatment. BA and GA decreased in 48.9% and 40.4% of cases after treatment, respectively, whereas tubulointerstitial lesion (TIL) developed in 27.7%. BA and GA decreased to 10.4% and 8.3%, respectively in children who developed TIL after treatment and the values were largely unchanged in those treated for more than 16 months. CONCLUSIONS: Glomerular growth is hampered by CsA nephrotoxicity, which is a more common complication than TIL. The impairment of glomerular growth is related to the duration of treatment and the development of TIL, but not to age or diagnosis.     

Aberrantly glycosylated serum IgA1 are closely associated with pathologic phenotypes of IgA nephropathy

BACKGROUND: IgA nephropathy (IgAN) is the most common glomerulonephritis with various histologic and clinical phenotypes. The mechanisms underlying the pathogenesis of IgAN remained unclear. But now altered O-glycosylation of serum IgA1 observed in these patients was considered to be a key contributory factor. The aim of the current study is to investigate whether aberrantly glycosylated IgA1 was associated with pathologic phenotypes of IgAN. METHODS: Sera from 107 patients with IgAN recently diagnosed were collected. Fifty patients were with mild mesangial proliferative IgAN, the others were with focal proliferative and sclerosing IgAN. Sera from 22 normal blood donors were used as normal controls. Biotinylated lectins were used in enzyme-linked immunosorbent assay (ELISA) to examine different glycans on IgA1 molecules. The alpha2,6 sialic acid was detected by elderberry bark lectin (SNA), the exposure of terminal galactose (Gal) and N-acetylgalactosamine (GalNAc) were detected by arachis hypogaea [peanut agglutinin (PNA)] and vilsa villosa lectin (VVL), respectively. The serum IgA1 glycans levels corrected by serum IgA1 concentrations were compared between patients and controls. RESULTS: Reduced terminal alpha2,6 sialic acid (1.16 +/- 0.21 vs. 0.98 +/- 0.31) (P= 0.008) and galactosylation (0.30 +/- 0.29 vs. 0.16 +/- 0.19) (P= 0.029) increased exposure of (GalNAc) (0.00 vs. 0.03) (P= 0.024) were demonstrated in serum IgA1 from patients with IgAN as compared with those in controls. More important, the exposures of 2,6 sialic acid and Gal were significantly decreased, especially in patients with focal proliferative and sclerosing IgAN compared with that in patients with mild mesangial proliferative IgAN (0.91 +/- 0.34 vs. 1.05 +/- 0.25) (P= 0.014) (0.108 +/- 0.137 vs. 0.221 +/- 0.219) (P= 0.018). However, no significant difference was found between patients with mild mesangial proliferative IgAN and normal controls (P > 0.05). The exposure of GalNAc of serum IgA1 from patients with focal proliferative and sclerosing IgAN was significantly higher than that of controls (P= 0.017), but had no statistical difference with that of patients with mild mesangial proliferative IgAN. CONCLUSION: The desialylation and degalactosylation of IgA1 in sera of patients with IgAN were closely associated with pathologic phenotypes.