表现为急性肾衰竭的IgA肾病11例临床与病理分析

目的：探讨IgA肾病并发急性肾衰竭(ARF)的临床与病理特点。方法：回顾分析11例IgA肾病并发ARF的临床与病理资料。结果：(1)本组11例IgA肾病患者并发ARF，占我院同期IgA肾病的1．28％(11／860)；(2)本组患者7例患者以浮肿、少尿为首发症状，3例以反复肉眼血尿为主诉、1例以恶性高血压为主要症状，6例患者蛋白尿≥2．0g／d；(3)肾活检示系膜增生性肾炎7例、新月体肾炎1例、增生硬化性肾炎伴新月体形成1例，余2例为In肾病的基础上伴发急性肾小管间质性肾炎。(4)本组资料中8例患者行激素加MP／CTX冲击治疗，4例予以单纯对症处理；8例肾功能恢复正常，1例肾功能部分缓解，2例患者肾功能进展行维持性透析治疗，总有效率为81．8％。结论：IgA肾病并发ARF发生率不低，病理可表现为多种病理类型，病理改变轻，预后好，多数患者肾功能可以恢复正常。     

IgA肾病合并新月体肾炎三例报告及文献复习

IgA肾病的临床谱很广，几乎涵盖了肾小球肾炎的各种表现，但同时合并急性肾功能衰竭（ARF）和新月体肾炎者并不多。我们报道了我院近期诊治的3例患者，探讨其临床特点和治疗。一、对象和方法1．病例资料：1979年至1999年5月在我院肾和住院，肾活检确诊为lgA肾病患者250例，其中3例诊断为lgA肾病合并新月体肾炎及ARF。2．观察指标：（）常规检查：尿常规、24小时尿蛋白定量、尿渗透浓度、肾功能、血浆白蛋白、蛋白电泳、血脂、血Ig、补体、ANA、抗一DSDNA、ENA、RF、抗肾抗体。ANCA等。（2）病理学检查：肾活检标本石蜡切片按常…     

20例毛细血管内增生患者临床与病理分析

目的：观察以肾小球毛细血管内增生为主患者的临床病理特点及预后。方法：回顾性分析我院近5年内收治的20例毛细血管内增生为主患者的临床表现、中医证候特征、肾脏病理特点及随访结果等。结果：20例患者中,临床表现为急性肾炎综合征2例（10.0%）、慢性肾炎综合征1例（5.0%）、肾病综合征17例（85.0%）;中医证型中,气阴两虚证占60.0%、湿热证占75.5%、血瘀证占50.5%。肾脏病理诊断为毛细血管内增生性IgA肾病6例（30.0%）、毛细血管内增生性非IgA系膜增生性肾炎5例（25.0%）、毛细血管内增生性狼疮性肾炎5例（25.0%）、急性链球菌感染后肾炎2例（10.0%）、肾小球毛细血管内皮病2例（10.0%）,除肾小球毛细血管内皮增生外,伴有系膜增生20例（100%）、基底膜增厚8例（40.0%）、新月体形成12例（60.0%）;按照个体化治疗原则并随访1年以上,完全缓解5例（25.0%）、部分缓解8例（40.0%）、未缓解7例（35.0%）。结论：毛细血管内增生患者临床以肾病综合征多见,中医证型以气阴两虚、挟湿挟瘀为主;除肾小球内皮及系膜细胞增生外,常合并基底膜增厚及新月体形成;不同病因毛细血管内增生患者预后差别较大,如急性链球菌感染后肾炎大多预后良好、毛细血管内增生性狼疮性肾炎、肾小球毛细血管内皮病大部分能完全或部分缓解,而毛细血管内增生性IgA肾病及毛细血管内增生性非IgA系膜增生性肾炎治疗反应较差。     

原发性肾病综合征并发急性肾损伤48例临床病理分析

目的探讨原发性肾病综合征合并急性肾损伤的临床及病理特点,提高此类并发症的防治水平。方法对我院原发性肾病综合征合并急性肾损伤患者的临床和病理改变进行回顾性分析。结果原发性肾病综合征合并急性肾损伤的临床特征表现为大量蛋白尿、高度水肿,常合并胸腹腔积液。肾脏病理类型：系膜增生性肾小球肾炎、肾小球微小病变及IgA肾病多见。其中系膜增生性肾小球肾炎22例,占46%;微小病变型10例,IgA肾病9例。所有患者均依据病理分型给予激素和（或）细胞毒药物,同时行利尿、控制感染、抗凝等综合治疗,其中5例进行血液透析治疗,肾损伤大多好转,但增生硬化型肾炎等预后较差。结论原发性肾病综合征并发急性肾损伤临床并不少见,多发生于系膜增生性肾小球肾炎、肾小球微小病变及IgA肾病,尽早明确病理诊断和去除诱因,并予相应治疗,大多患者预后良好,肾功能可恢复正常。     

肾病综合征1009例病理类型及流行病学分析

目的探讨肾活组织病理检查（下称肾活检）在肾病综合征诊治中的意义。方法选择近10年诊断为肾病综合征患者1009例,分析肾活组织穿刺检查术后的病理结果。结果原发性肾小球肾炎占71．9％,病理类型以非IgA系膜增生性肾炎、IgA肾病及膜性肾病多见;继发性肾病综合征占28．1％,其中居前3位的是狼疮性肾炎、过敏性紫癜性肾炎和乙肝病毒相关性肾炎。结论肾活检对肾病综合征的诊断、治疗及评估预后有重要意义。     

IgA肾病尿蛋白波动性与预后的相关性分析

IgA肾病在我国高发,占原发性肾小球肾炎的20%～30%,是我国终末期肾病（ESRD）发生的主要原因之一[1]。控制病情,延缓肾功能进展是我们治疗IgA肾病的最终目标。众多研究表明肾衰竭、蛋白尿、肾小球硬化、新月体形成和肾间质纤维化是影响IgA肾病预后的独立危险因素[2]。     

急性肾功能衰竭误漏诊原因分析及肾活检的意义

目的：探讨急性肾功能衰竭（ARF）误漏诊原因,提高ARF诊断水平。方法：分析入院前诊断与肾活检与肾活检后诊断的符合率,寻找误漏诊原因。结果：经肾活检证实68例ARF病例中20例（29．6％）存在病因误诊,包括急性肾小球肾炎（AGN）、急进性肾炎（RPGN）、 急性肾小管坏死（ATN）、膜增生性肾小球肾炎（MPGN）、原发性肾病综合征伴特发性急性肾功能衰竭（NS＋IARF）、狼疮肾炎（LN）、急性间 质性肾炎（AIN）、慢性肾炎急性发作等之间混淆。有30．9％（21／68）例改变了治疗方案,35．3％（24／68）部分改变了治疗方案,33．8％（23／68）按原方案治疗。结论：临床上有相当部分ARF的病因被误诊,在诊断困难时应及时肾活检,以免延误治疗。     

表现为肾病综合征的毛细血管内增生性肾炎临床分析

病理为毛细血管内增生性肾炎（endocapillary proliferative glomerulonephritis,EPGN）通常表现为急性感染后肾小球肾炎即急性肾炎,临床上主要表现为。肾炎综合征,但表现为肾病综合征的也并非少见。现对我科住院的经皮肾脏活体组织检查（简称肾活检）证实为EPGN的合并肾病综合征10例患者进行回顾性临床分析,报道如下。     

新月体性肾炎的中西医结合治疗

新月体性肾炎为一病理诊断,其病情发展急骤,由蛋白尿、血尿迅速发展为无尿或少尿的急性肾衰竭,预后恶劣.有学者报道8 828例肾活检病例中,新月体性肾炎占肾活检总数的1.74%[1].其肾穿刺病理改变特征为肾小球囊内细胞增生、纤维蛋白沉积.     

新月体性IgA肾病的临床研究进展

IgA肾病是最常见的原发肾小球疾病之一,其临床和病理表现呈明显的异质性,20世纪70年代末期,新月体性IgA肾病为人类所认识.现有研究表明在出现新月体的IgA肾病亚组中,其临床表现和预后仍不一致,少数可表现为慢性肾炎综合征,不伴肾功能的快速恶化;但大部分新月体性IgA肾病患者临床表现为急进性肾小球肾炎,预后差.新月体性IgA肾病为IgA肾病中的严重亚型,是急进性肾炎综合征的常见病因,其发病率低,关于此病的研究很少,目前国内外尚缺乏相关的专题讨论.我们将有关新月体性IgA肾病的最新研究进展综述如下.     

Acute reversible renal failure with macroscopic haematuria in IgA nephropathy

Macroscopic haematuria is common in IgA nephropathy, but itssignificance and influence on prognosis remains uncertain. Wecompared the clinical and pathological features of 11 adultpatients with primary IgA nephropathy who had had a renal biopsyduring or shortly after a bleeding episode. Six patients developedtransient acute renal failure (ARF) (group 1) and five did not(group 2). Patients of group 1 had a higher percentage of tubularred-blood-cell (RBC) casts (P<0.05) and of glomerular crescents(P<0.001). However, crescents were focal and involved lessthan 50% of glomeruli. Acute tubular necrosis was only presentin patients of group 1, and ARF was attributed to the acutetubular changes rather than to the glomerular lesions. Despitea prolonged duration of ARF (mean: 38 days), further outcomedid not differ in patients of both groups. We suggest that acutetubular damage and/or tubular obstruction by RBC casts shouldbe considered in any patient who develops ARF soon after a haematuricepisode.     

Randomised controlled trial of leflunomide in the treatment of immunoglobulin A nephropathy

AIM: To investigate the effect of leflunomide for treatment of immunoglobulin A (IgA) nephropathy. METHODS: Sixty IgA nephropathy patients were divided into two groups at random. Patients in the test group received leflunomide and patients in the control group received fosinopril. Clinical data were obtained at weeks 2, 4, 6, 8, 12, 16, 20, 24 and 28. RESULTS: The complete remission rate was 62.1% and the total effectiveness rate was 72.4%. In the leflunomide group, proteinuria significantly decreased from 1.66 +/- 0.42 g to 0.60 +/- 0.68 g (P < 0.05). The efficacy rate of leflunomide compared with fosinopril in treating IgA nephropathy was not statistically different (P > 0.05). Side-effects were mild in both treatment groups. CONCLUSION: These preliminary results are encouraging, but further randomised studies are required before leflunomide can be recommended for the treatment of IgA nephropathy.     

Acute renal failure in IgA nephropathy: aggravation by gross hematuria due to anticoagulant treatment

IgA nephropathy is one of the most common forms of glomerulonephritis. Macroscopic or microscopic hematuria with mild proteinuria are the main symptoms. Without complicating factors, IgA nephropathy has a favourable long-term prognosis. We report a case of reversible acute renal failure (ARF) as a complication of mild IgA nephropathy while oral anticoagulants were administered. Diagnosis was based on a renal biopsy showing marked granular mesangial IgA-deposition. In addition, numerous tubules were extended and completely obstructed by red blood cell casts. After hemodialysis treatment and parallel anti-inflammatory steroids and after stopping anticoagulation, renal function gradually improved up to complete remission. This report indicates that anticoagulatory treatment may have negative effects on the long-term prognosis of IgA nephropathy with respect to development of ARF or tubulo-interstitial inflammation.     

伴有慢性肾衰竭的马兜铃酸肾病与IgA肾病的配对研究

目的:了解伴有慢性肾衰竭的马兜铃酸肾病患者与IgA肾病患者的临床病理差异.方法:分析11例马兜铃酸肾病患者的临床病理资料,并与经肾活检确诊的IgA肾病患者进行配对比较.结果:两组患者的年龄、性别、血肌酐水平无明显差异(P>0.05),马兜铃酸肾病患者镜下血尿发生率低于IgA肾病患者(P<0.05).在血肌酐水平无明显差异时,马兜铃酸肾病患者血红蛋白浓度明显低于IgA肾病患者(P<0.01),尿蛋白定量和肾脏的长径少于IgA肾病患者(P<0.05).间质纤维化程度重于IgA肾病患者(P<0.05),而间质炎细胞浸润少(P<0.05).结论:伴有慢性肾衰竭的马兜铃酸肾病患者临床发展隐匿,与IgA肾病比较,肾小管间质损伤是造成肾功能损害的主要原因,因此要重视对马兜铃酸肾病的早期防治.     

Urinary liver-type fatty acid-binding protein: discrimination between IgA nephropathy and thin basement membrane nephropathy

BACKGROUND: Microscopic hematuria without proteinuria is a common clinical finding in cases of immunoglobulin A (IgA) nephropathy and of thin basement membrane nephropathy. Liver-type fatty acid-binding protein (L-FABP) is expressed in renal proximal tubules and is reported to be a useful marker of the progression of chronic glomerulonephritis. AIM: To assess urinary L-FABP levels for differential diagnosis in patients with microscopic hematuria but without proteinuria. METHODS: This was a multi-center retrospective study. Thirty adult patients who underwent renal biopsy for microscopic hematuria and 20 healthy adult volunteers were included in this study. Urinary L-FABP levels were measured by enzyme-linked immunosorbent assay and compared, particularly between those diagnosed with IgA nephropathy and those diagnosed with thin basement membrane nephropathy. RESULTS: Twelve (40%) patients had IgA nephropathy, 6 (20%) had thin basement membrane nephropathy and 12 (40%) had normal biopsy findings. The urinary L-FABP level was significantly higher in patients with IgA nephropathy (38.4 +/- 26.8 microg/g Cr) than in healthy subjects (5.8 +/- 4.0 microg/g Cr) (p < 0.01); however, the level in patients with thin basement membrane nephropathy or normal biopsy results was comparable to that in healthy subjects. Follow-up data were available for 11 of the 12 patients with IgA nephropathy who initially had no proteinuria. After 24 months, 4 of the 11 were found to have proteinuria, and the urinary L-FABP level had increased from 40.6 +/- 30.5 microg/g Cr to 58.8 +/- 40.5 microg/g Cr (p < 0.01). CONCLUSIONS: Our data suggest that the urinary L-FABP level can be used to discriminate between IgA nephropathy and thin basement membrane nephropathy in patients with microscopic hematuria.     

Discrimination between postinfectious IgA-dominant glomerulonephritis and idiopathic IgA nephropathy

Background: A unique form of postinfectious glomerulonephritis (PIGN) with IgA-dominant deposition mimicking IgA nephropathy has been increasingly reported. Methods: We compared the clinical and histological features of 12 patients with postinfectious IgA-dominant glomerulonephritis to 134 patients with idiopathic IgA nephropathy. Results: In addition to hypocomplementemia and subepithelial hump-shaped deposits characteristic of PIGN, patients with postinfectious IgA-dominant glomerulonephritis had older age (62.3 ± 16.9 vs. 37.9 ± 16.3 years; p < 0.001) and more frequently presented with acute renal failure (83.3% vs. 10.4%; p < 0.001) than patients with idiopathic IgA nephropathy. Moreover, glomerular changes including endocapillary proliferation, neutrophil infiltration, and capillary loops deposits by immunofluorescence were more commonly present in postinfectious IgA-dominant glomerulonephritis group (p < 0.001). Conclusions: PIGN could be characterized by glomerular IgA-dominant deposition resembling idiopathic IgA nephropathy. It is essential to differentiate postinfectious IgA-dominant glomerulonephritis from idiopathic IgA nephropathy because of the different treatments and prognosis of the two diseases.     

Exclusive characteristics of graft survival and risk factors in recipients with immunoglobulin A nephropathy: a retrospective analysis of registry data

BACKGROUND: Some studies have claimed that patients with immunoglobulin A (IgA) nephropathy have better graft survival than other renal graft recipients, whereas others have rejected this statement. We have addressed this paradox in the present study. METHODS: In all, 1,207 patients with IgA nephropathy who received a primary cadaveric renal graft from 1990 to 2002 were identified in the Eurotransplant database. For comparison, we analyzed 7,935 patients with nonglomerular diseases. Death-censored graft survival was calculated using Kaplan Meier estimates and a multivariable Cox regression analysis was used for risk calculations. RESULTS: Death-censored graft survival was superior in patients with IgA nephropathy in the first period after transplantation. After 3 years posttransplant, however, there was an accelerated decline in graft survival in recipients with IgA nephropathy. The fully adjusted risk of graft loss in the first year was increased by 40% in the control group compared to IgA nephropathy (hazard ratio [HR] 1.40, 95% CI 1.12-1.75), whereas the risk was significantly lower in the control group after the first year posttransplant (HR 0.75, 95% CI 0.63-0.88). Cold ischemia time, immunization and HLA-DR mismatch were risk factors for graft loss in the control group but not for IgA nephropathy, whereas HLA-AB mismatch was an independent risk factor, exclusively for the IgA nephropathy group. CONCLUSIONS: Recipients with IgA nephropathy have better 1-year graft survival, presumably due to favorable immunological behavior. This benefit was however abolished in the long-term by increased graft loss with time. Studies are needed to explain the difference in graft survival and the reason why different risk factors are involved in graft failure.     

Free radical release and progression of renal failure

Summary: IgA nephropathy, formerly known as Berger's Disease, is now recognized as the world's most common primary nephropathy. the rate of progression has recently been shown to be the same in childhood as well as adulthood (i.e. slightly more than 1/4 of the patients had end-stage renal disease at follow up of 20 years). the pathogenesis may be associated with free-radical release as demonstrated in experimental IgA nephropathy and indirectly in leucocytes of these patients. the use of vitamin E and fish oil is discussed.     

Oxidative stress and non-enzymatic glycation in IgA nephropathy

AIM: Approximately 20-50% of IgA nephropathy patients develop end-stage renal disease. We have previously found enhanced oxidative stress and decreased antioxidant capacity in red blood cells of IgA nephropathy patients. In this study we assess oxidative stress, non-enzymatic glycation, oxidative resistance of low-density lipoprotein and its alpha-tocopherol content in these patients. PATIENTS AND METHODS: Non-enzymatic glycation and oxidative stress were assessed in 88 IgA nephropathy patients by measuring advanced glycation end products, Nepsilon-carboxymethyl-lysine, thiobarbituric acid reactive substances, oxidative resistance of low-density lipoprotein and its alpha-tocopherol content. RESULTS: Advanced glycation end products (2659 +/- 958 a.u.) and Nepsilon-carboxymethyl-lysine (563 +/- 215 ng/ml) were significantly higher in IgA nephropathy patients with decreased renal function compared to those with normal renal function (p < 0.002) or controls (p < 0.001). Thiobarbituric acid-reactive substances in plasma and associated with low-density lipoprotein were significantly elevated and oxidative resistance of low-density lipoprotein was significantly reduced in all groups of IgA nephropathy patients. There was no significant difference in circulating fluorescent advanced glycation end products, Nepsilon-carboxymethyl-lysine, thiobarbituric acid-reactive substances levels, oxidative resistance of low-density lipoprotein and its alpha-tocopherol content between patients with normal vs. impaired glucose metabolism. Low alpha-tocopherol content of low-density lipoprotein was accompanied with decreased oxidative resistance, depletion in polyunsaturated fatty acids, elevated saturated fatty acids and thiobarbituric acid-reactive substances within low-density lipoprotein suggesting enhanced lipid peroxidation. CONCLUSIONS: Decreased oxidative resistance of low-density lipoprotein and enhanced oxidative stress are common features in IgA nephropathy, while increased non-enzymatic glycation occurs as renal function declines.     

VCAM-1, ICAM-1, and E-selectin in IgA nephropathy and Schönlein-Henoch syndrome: differences between tissue expression and serum concentration

The tissue expressions of vascular cell adhesion molecule 1 (VCAM-1), intercellular adhesion molecule 1 (ICAM-1) and endothelial leukocyte adhesion molecule 1 (E-selectin-1) were investigated in biopsy specimens from 28 patients with different stages of IgA nephropathy (IgAN) and 20 patients with acute renal failure (ARF) or chronic renal diseases (amyloidosis, Alport's glomerulopathy) by immunohistochemistry. The results were compared with the serum levels of the three adhesion molecules. VCAM-1 expression was significantly increased on parietal/tubular epithelial cells in IgAN and ARF. Significantly elevated circulating VCAM-1 levels were measured in IgAN and amyloidosis, but did not correlate with renal function (creatinine clearance). Significantly increased glomerular endothelial/epithelial ICAM-1 expression was found in IgAN and ARF. Intense mesangial ICAM-1 expression was found in mild stages of IgAN and in Sch?nlein-Henoch syndrome. Circulating ICAM-1 was not significantly elevated in IgAN and different renal diseases. VCAM-1 and ICAM-1 expressions of interstitial infiltrating cells were significantly higher in severe than in mild IgAN and associated with an increased infiltration of inflammatory leukocytes. Patients with IgAN and different renal diseases had decreased mesangial and almost absent interstitial E-selectin expression as compared with controls. The circulating E-selectin levels were significantly elevated in ARF. In conclusion, the tissue expression of adhesion molecules in IgAN reflects a continuous inflammatory renal activity. However, only increased circulating VCAM-1 serum levels correlated significantly with the histological state of renal inflammation and could be used as a disease marker.