在原先急性肾炎基础上发生的IgA肾病

IgA 肾病是由一组疾病组成的综合征。尽管具有共同的免疫病理特点,但临床上可见于很多疾病,病因不清。于急、慢性肾小球肾炎,其 IgA 沉着很少。本文介绍一个特殊病例,IgA 肾病发生于急性肾小球肾炎(AGN)之后。     

糖尿病肾病患者凝血与纤溶平衡变化的研究

目的　探讨糖尿病肾病患者的凝血、纤溶状态。方法　 35例 2型糖尿病、糖尿病肾病 (DN)患者和慢性肾小球肾炎 (CGN)患者 2 9例 ,按是否合并肾病综合征 (NS)分为 :DN- NNS组 19例 ,DN- NS组 16例 ,CGN- NNS组 16例 ,CGN- NS组 13例。酶联免疫吸附法系统检测各项凝血和纤溶指标。结果　无论是否合并 NS,DN患者较 CGN组血中TM、PF1 2 、TAT、FPA及 PAI-水平明显增加。结论　DN患者较慢性肾小球肾炎患者合并更强的血液高凝状态     

血凝、纤溶与肾脏疾病

机体的正常止血功能由血凝与纤维蛋白溶解之间的平衡得以维持,止血平衡紊乱与某些肾脏疾病的发病机理密切相关。本文详述溶血性尿毒症综合征、感染后急性肾小球肾炎、急进性肾炎、慢性肾小球肾炎及肾病综合征等肾脏疾病与血凝、纤维蛋白溶解间的关系。     

肾病综合征蛋白质饮食的新观点

本文总结了八十年代以来的有关文献,提出表现为肾病综合征的慢性肾小球疾病,在没有肾功能不全的情况下,也应该给予低蛋白饮食。     

2289例慢性肾脏病临床及病理分析

目的 总结我院近3年来慢性肾脏病患者肾脏病理类型以及不同年龄段肾脏疾病的构成比,分析慢性肾脏病分期、临床分型与病理分型之间的关系.方法 收集2009年1月～2011年12月在我科行肾活检的慢性肾脏病患者2289例,对其性别、年龄、病理类型、临床分型、CKD分期等进行回顾性分析.结果 (1)2289例患者平均年龄(46.6±25.7)岁,男女比例1.22∶1;(2)原发性肾小球疾病占68.3％,IgA肾病最常见(占40.3％);继发性肾脏疾病占31.4％,狼疮性肾炎发病率最高(28.4％),其次是糖尿病肾病(19.7％);(3)年龄＜18岁组患者以肾小球轻微病变最为多见(30.4％),年龄＞ 60岁的老年患者中以膜性肾病和糖尿病肾损害最常见;(4)慢性肾炎综合征是最常见的临床类型(37.9％).结论 慢性肾脏病好发于中青年,不同年龄段患者肾脏疾病的病理分型明显不同.原发性肾病中IgA肾病最常见,继发性肾脏疾病以狼疮性肾炎最多见.导致慢性肾功能不全最常见的病理类型为局灶节段性肾小球硬化、IgA肾病以及糖尿病肾病.最常见的临床类型为慢性肾炎综合征和肾病综合征.     

老年人肾脏疾病的临床表现与病理特点分析

目的分析肾脏疾病在老年人中的临床表现和病理特点。方法收集60岁以上老年肾脏病患者51例,对其临床表现及肾活检病理进行分析。结果 51例老年肾脏病患者中,原发性肾小球疾病33例,继发性肾脏疾病18例。临床表现为肾病综合征(NS)28例,肾炎综合征10例,慢性肾功能衰竭(CRF)5例,急性肾损伤(AKI)4例,肾病综合征合并急性肾损伤4例。老年原发性肾小球疾病最常见的病理类型为膜性肾病(17例),其次是Ig A肾病(5例),系膜增生性肾小球肾炎(4例)。在继发性肾脏疾病中,最常见的诊断是乙型肝炎病毒相关性肾炎(4例),其次为ANCA相关性血管炎肾损害和肾淀粉样变(各3例)。结论老年原发性肾小球疾病多见,且以膜性肾病为主。老年继发性肾脏病以乙型肝炎病毒相关性肾炎、血管炎肾损害和肾淀粉样变多见。肾病综合征是老年肾脏病患者最常见的肾活检适应证。对病因不明的老年肾脏病患者,应及早行肾脏病理活检,有助于明确诊断及治疗。     

膜性肾小球肾炎的研究进展

膜性肾病或膜性肾小球肾炎是免疫复合物沉积在肾小球基底膜和脏层上皮之间,并引起基底膜弥漫性增厚的慢性原发性肾小球疾病。其临床特征为大量蛋白尿或肾病综合征。近年来,临床上对该病的研究日趋重视。现概述如下。     

单中心规律血液透析患者常见病因及其与年龄、超重关系的临床研究

目的探讨规律血液透析患者导致慢性肾衰竭的病因及其与年龄、超重的关系。方法选择符合入选标准的患者92例，调查导致慢性。肾衰竭的病因、年龄及体重指数，并进行回顾性分析。结果我中心血液透析患者以老年为主，常见病因是慢性。肾小球。肾炎，糖尿病。肾病，高血压肾病。糖尿病。肾病、高血压。肾病患者中60岁以上老年患者所占例数明显高于慢性肾小球肾炎组。糖尿病。肾病、高血压肾病患者中超重患者所占百分比明显高于慢性。肾小球。肾炎组。结论血液透析患者以老年为主，超重可能是加速慢性肾小球肾炎、糖尿病肾病、高血压肾病进展至慢性肾衰竭的主要原因之一。     

慢性肾病患者肾功能指标与肾脏病理变化的关系研究

目的探讨几种常见肾功能指标对评估慢性肾病患者肾脏病理变化的价值。方法选择2003年11月至2004年11月在苏州大学附属第二医院肾内科住院的54例慢性肾病患者,用RXL生化分析仪测定血肌酐(Scr)并通过MDRD方程计算肾小球滤过率(GFR)。检测血清胱蛋白酶抑制剂(CysC)、β2微球蛋白(β2-MG),以24h尿蛋白定量及蛋白尿持续时间的乘积计算尿蛋白指数,根据肾穿刺检查病理结果衡量肾小球指数和肾间质病变程度判定疾病进展情况。结果慢性肾病患者的血清CysC、Scr、血β2-MG、蛋白尿指数、GFR与肾脏病理变化程度相关。其中GFR和血清CysC与肾脏病理进展程度高度相关。结论在慢性肾病进展评估中应该同时考虑CysC及蛋白尿指数,能更全面地反映慢性肾脏疾病的进展情况。     

慢性肾病合并冠状动脉粥样硬化的他汀治疗

近年来,冠心病非传统危险因素逐渐受到业内重视,慢性肾病是其中之一.根据美国肾病系统数据(USRDS)分析显示,2005年美国慢性肾病患者中因心血管疾病住院率达44.5%,心脑血管疾病是慢性肾病最主要的并发症及死亡原因,其中心源性死亡占39%,终末期肾病患者的心血管病死亡率为普通人群的500倍,其中主要死因为急性冠脉综合征(ACS)、心肌梗死后心衰和猝死.     

Focal and segmental glomerular sclerosis in reflux nephropathy

Reflux nephropathy was diagnosed in 23 patients (14 per cent of all the patients who received transplants) between 1973 and 1977, and nephrectomy was performed in all. Histology and immunofluorescence revealed a glomerular sclerosis associated with the idiopathic nephrotic syndrome. No focal and segmental glomerular sclerosis was seen in kidneys removed from patients with nonglomerular renal disease. Twenty-four hour urinary protein excretion in grams was 3.1 ± 0.3 (mean ± SEM) and was greater than that in our patients with end-stage nonglomerular renal disease. Thirty-one renal transplants were performed in these 23 patients; thereafter, maximum protein excretion was 1.4 g. Focal and segmental glomerular sclerosis was seen in only one (chronic rejection, protein excretion < 0.5) of the 20 kidneys available for histologic study. Thus, focal and segmental glomerular sclerosis is extremely common in reflux nephropathy, accounts for “glomerular” proteinuria and may contribute importantly to progressive renal failure but, unlike that associated with the idiopathic nephrotic syndrome, rarely recurs after renal transplantation.     

A review of nephrotic syndrome associated with chronic lymphocytic leukemia

Four patients had the nephrotic syndrome and chronic lymphocytic leukemia (CLL), which may be pathogenetically related. Membranoproliferative glomerulonephritis appears to be the most common glomerular lesion in patients with CLL. Available evidence suggests that nephrotic syndrome associated with CLL is often related to immune-complex disease. It is also possible that disorders of immunoglobulin production and cellular immunity contribute to renal disease in patients with CLL.     

Immunopathology of the nephrotic syndrome associated with renal vein thrombosis. Report of two cases and brief review of the literature

Renal tissue from two patients with the nephrotic syndrome and renal vein thrombosis was studied by immunofluorescence microscopy in addition to conventional histologic and electron microscopic technics. Granular deposits of immunoglobulins G (IgG), M (IgM) and beta₁ C/beta₁ A globulin (one case) were seen by fluorescence microscopy along the basement membranes in a pattern similar to that observed in patients with chronic membranous nephropathy and in the experimental model of chronic serum sickness. Renal vein thrombosis, associated with the nephrotic syndrome, is not clearly separated clinically or pathologically from primary glomerular disease with the nephrotic syndrome. In view of the lack of experimental evidence to show that either the glomerular lesion or the proteinuria is the result of elevated venous pressure alone, the pathogenesis of the lesion must remain in doubt.     

Myelodysplastic syndromes with nephrotic syndrome

It is sometimes reported that the immunological abnormalities in myelodysplastic syndromes (MDS) induce autoimmune disease (i.e., acute systemic vasculitic syndrome, chronic cutaneous vasculitis, polyneuropathy, relapsing polychondritis, and steroid-responsive pulmonary disorders). We investigated the clinical features of patients with MDS accompanied by nephrotic syndrome. We enrolled 125 patients with MDS who were admitted between January 1979 and May 1996 in this study. The renal function was assessed based on the laboratory data and the findings at the physical examination. The diagnoses of nephrotic syndrome and glomerular disease were established when 24-hr urinary excretion was more than 3.5 g and serum total protein was less than 6.0 g/dl, and when the 24-hr protein excretion was more than 1.5 g. Five patients (4%) had glomerular disease, and three (2.4%) had nephrotic syndrome. Of the five patients with glomerular disease, two had refractory anemia (RA), and three had chronic myelomonocytic leukemia (CMMOL). Three of the total 11 patients with CMMOL were diagnosed as having nephrotic syndrome. Among the CMMOL patients, those with nephrotic syndrome showed higher absolute monocyte numbers than did those without nephrotic syndrome (8830 +/- 4677/microl vs. 3061 +/- 2887/microl, P = 0.03). One CMMOL patient was treated with VP-16 and hydroxyurea. As the white blood cell count in this patient decreased, the 24-hr urine protein excretion and the serum tumor necrosis factor alpha level decreased. The relationship between nephrotic syndrome and CMMOL was not clear. High monocyte count and the serum cytokines in MDS patients may play a partial role in the evolution of glomerulonephritis, and CMMOL may be closely related to nephrotic syndrome.     

Glomerular lesions in the acquired immunodeficiency syndrome

Between January 1982 and December 1983, 75 patients with the acquired immunodeficiency syndrome were identified in our hospitals: 35% used intravenous drugs, 50% had proteinuria in excess of 0.5 g/dL, and 10% were nephrotic. Glomerular changes seen at autopsy in 36 patients included frequent mesangial lesions and deposits associated with mild asymptomatic proteinuria. Focal and segmental glomerular sclerosis was found in 5 patients and 4 of these had the nephrotic syndrome. Whereas reversible episodes of acute renal failure were not uncommon, terminal episodes of acute renal insufficiency occurred in 14 patients. The short survival of these patients may prevent the development of chronic renal failure.     

Medullary nephrocalcinosis, distal renal tubular acidosis and polycythaemia in a patient with nephrotic syndrome

ABSTRACT: BACKGROUND: Medullary nephrocalcinosis and distal renal tubular acidosis are closely associated and each can lead to the other. These clinical entities are rare in patients with nephrotic syndrome and polycythaemia is an unusual finding in such patients. We describe a patient with medullary nephrocalcinosis, distal renal tubular acidosis and polycythaemia in a patient with nephrotic syndrome due to minimal change disease. Proposed mechanisms of polycythaemia in patients with nephrotic syndrome and distal renal tubular acidosis include increased erythropoietin production and secretion of interleukin 8 which in turn stimulate erythropoiesis. CASE PRESENTATION: A 22 year old Sri Lankan Sinhala male with nephrotic syndrome due to minimal change disease was investigated for incidentally detected polycythaemia. Investigations revealed the presence of renal tubular acidosis type I and medullary nephrocalcinosis. Despite extensive investigation, a definite cause for polycythaemia was not found in this patient. Treatment with potassium and bicarbonate supplementation with potassium citrate led to correction of acidosis thereby avoiding the progression of nephrocalcinosis and harmful effects of chronic acidosis. CONCLUSION: The constellation of clinical and biochemical findings in this patient is unique but the pathogenesis of erythrocytosis is not clearly explained. The proposed mechanisms for erythrocytosis in other patients with proteinuria include increased erythropoietin secretion due to renal hypoxia and increased secretion of interleukin 8 from the kidney. This case illustrates that there may exist hitherto unknown connections between tubular and glomerular dysfunction in patients with nephrotic syndrome.     

Nephrotic syndrome in childhood

Childhood nephrotic syndromes are most commonly caused by one of two idiopathic diseases: minimal-change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS). A third distinct type, membranous nephropathy, is rare in children. Other causes of isolated nephrotic syndrome can be subdivided into two major categories: rare genetic disorders, and secondary diseases associated with drugs, infections, or neoplasia. The cause of idiopathic nephrotic syndrome remains unknown, but evidence suggests it may be a primary T-cell disorder that leads to glomerular podocyte dysfunction. Genetic studies in children with familial nephrotic syndrome have identified mutations in genes that encode important podocyte proteins. Patients with idiopathic nephrotic syndrome are initially treated with corticosteroids. Steroid-responsiveness is of greater prognostic use than renal histology. Several second-line drugs, including alkylating agents, ciclosporin, and levamisole, may be effective for complicated and steroid-unresponsive MCNS and FSGS patients. Nephrotic syndrome is associated with several medical complications, the most severe and potentially fatal being bacterial infections and thromboembolism. Idiopathic nephrotic syndrome is a chronic relapsing disease for most steroid-responsive patients, whereas most children with refractory FSGS ultimately develop end-stage renal disease. Research is being done to further elucidate the disorder's molecular pathogenesis, identify new prognostic indicators, and to develop better approaches to treatment.     

Nephrotic syndrome in Hodgkin's disease. Evidence for pathogenesis alternative to immune complex deposition.

The nephrotic syndrome has been reported to occur in patients with Hodgkin's disease even in the absence of amyloidosis, tumor infiltration of renal vein thrombosis. Three patients are presented with Hodgkin's disease and the nephrotic syndrome whose renal biopsy specimens studied with light, immunofluorescence and electron microscopy were compatible with "lipoid nephrosis" (minimal change disease). A review of the literature reveals 35 patients with Hodgkin's disease and the nephrotic syndrome. Renal tissue was available for examination in only 27 patients. The majority of patients apparently had glomerular alterations consistent with lipoid nephrosis. The nephrotic syndrome in most of these patients remitted with a variety of methods of therapy (including excision, irradiation, prednisone and cyclophosphamide) and tended to relapse with a recurrence of Hodgkin's disease. In three-fourths of the patients with Hodgkin's disease and the nephrotic syndrome, the Hodgkin's disease was of a mixed cellularity type. The etiology of lipoid nephrosis, although unclear, may be a consequence of altered lymphocyte function. Hodgkin's disease is a malignancy involving T lymphocytes, and the nephrotic syndrome occurring in the course of Hodgkin's disease may be a result of an adverse effect of glomeruli by products of tumor lymphocytes rather than of glomerular deposition of immune complexes.     

粘附分子P选择素在老年肾病综合征患者肾组织中的表达和周围血流式细胞分析

目的：探讨粘附分子Ｐ选择素（ＣＤ６２Ｐ）在老年肾病综合征（ＮＳ）中的变化。方法：采用流式细胞术和直接免疫荧光标记单克隆抗体对老年ＮＳ患者周围血和肾活检组织进行了ＣＤ６２Ｐ表达的研究。结果：①２６例老年ＮＳ患者周围血ＣＤ６２Ｐ表达量较正常组显著增高（Ｐ＜０．０１）；②肾组织中显示ＣＤ６２Ｐ表达２２例（８４．６２％），表达部位常见于肾小球系膜区、血膜袢，其次为肾小管、间质小血管及球囊壁。③ＣＤ６２Ｐ在周围血表达水平与肾组织表达程度呈显著正相关（Ｐ＜０．０１），血、肾组织的表达与患者尿蛋白定量呈正相关（Ｐ＜０．０５）。结论：ＣＤ６２Ｐ在老年ＮＳ患者肾组织和周围血中表达增强，提示其在ＮＳ发病机制中具有重要作用     

Abnormal Renal Sodium Excretion in the Nephrotic Syndrome after Furosemide: Relation to Glomerular Filtration Rate

ABSTRACT. Danielsen H, Pedersen EB, Madsen M, Jensen T. (Department of Internal Medicine C, Aarhus Kommunehospital, and University of Aarhus, Aarhus, Denmark.) Abnormal renal sodium excretion in the nephrotic syndrome after furosemide: relation to glomerular filtratoni rate. The effect of 40 mg furosemide intravenously on sodium excretion, the renin-aldosterone system and arginine vasopressin (AVP) was studied in 14 patients with the nephrotic syndrome and in 13 control subjects. Creatinine clearance (C_cr) was reduced in all patients but four. Before furosemide, AVP, but not angiotensin II (AII) or aldosterone (Aldo), was increased in the nephrotic patients. After fursoemide, sodium excretion (Na_E) increased less and changes in AVP, AII and Aldo were blunted in the patients. C_cr and Na_E were positively correlated in the nephrotic syndrome. The reduced sodium response after furosemide in the nephrotic syndrome seems to be closely correlated to a reduced glomerular filtration rate but not to an increased activity of the renin-angiotensin-aldosterone system. The reduced response of AVP, AII and Aldo after furosemide is consistent with a lower degree of volume depletion in nephrotic patients.