霉酚酸酯对局灶节段性肾小球硬化大鼠肾脏的保护作用

目的观察霉酚酸酯对局灶节段性肾小球硬化大鼠残肾的保护作用。方法给予局灶节段性肾小球硬化大鼠霉酚酸酯（MMF）50mg·kg^-1·d^-1、雷公藤20mg·kg^-1·d^-1。8周后观察大鼠24h尿蛋白定量、血尿素氮（BUN）、血肌酐（SCr）、血浆白蛋白（Alb）的含量以及肾脏病理改变。结果两药均能减少尿蛋白,降低BUN。在肾脏病理上,局灶节段性肾小球硬化大鼠出现局灶性肾间质纤维化,肾小球硬化,用药后病变减轻,以MMF减轻更为显著。结论MMF能抑制局灶节段性肾小球硬化大鼠肾脏中细胞的异常增殖,减少间质纤维化及肾小球硬化,减少蛋白尿,减轻肾脏的损害。     

局灶节段性肾小球硬化的新病理分型

局灶节段性肾小球硬化(FSGS)由Rich在1957年首先描述,其发生率有逐年增高趋势.FSGS是一种临床病理综合征,其特点为蛋白尿,常为肾病性蛋白尿,同时伴局灶节段性肾小球硬化和足突的消失.疾病早期,仅部分肾小球(＜50%)和/或部分毛细血管襻(<50%)发生硬化性改变.随着病变进展,肾小球逐渐弥漫硬化,甚至出现球性硬化.     

局灶节段性肾小球硬化的病理诊断及分型

局灶节段性肾小球硬化（focalsegmental glomerulosclerosis,FSGS）是一种临床以大量蛋白尿或肾病综合征为特征、病理以局灶和节段分布的硬化性病变为主要特征的肾小球疾病。分为原发性和继发性两类。继发性FSGS是指多种肾小球疾病导致的局灶肾小球节段硬化性病变。原发性FSGS是病因、发病机制、病变特点、临床表现和预后等各方面均具特点的肾小球疾病,是本文讨论的重点。     

局灶节段性肾小球硬化症诊治进展

局灶节段性肾小球硬化（FSGS）表现为蛋白尿,同时伴局灶节段性肾小球硬化和足突的消失。疾病早期,仅部分肾小球（〈50％）和毛细血管襻（（50％）出现硬化性改变。随着病变进展,肾小球逐渐出现球性硬化。根据病因可分为原发（特发）性、继发性FSGS两类,本文就原发性FSGS的诊治进展作一综述。     

肥胖相关性肾小球病发病机制的研究进展

1997年世界卫生组织确定肥胖是一种疾病,近20年其发病率明显升高。肥胖不仅是高血压、动脉粥样硬化病及糖尿病的高危因素,而且可以导致肾损害。1974年Weisinger等首次报告严重肥胖患者能出现大量蛋白尿,此其后此病被命名为“肥胖相关性肾小球病”（obesity-related glomerulopathy,ORG）。病理检查ORG可分成两型,仅表现为肾小球肥大者称为“肥胖相关性肾小球肥大症”（obesity-associated glomerulomegaly,OB-GM）,表现为肾小球肥大及局灶节段性肾小球硬化者称为“肥胖相关性局灶节段性肾小球硬化”（obesity-associated focal and segmental glomerulosclerosis,OB-FSGS）。     

局灶节段性肾小球硬化症的病理诊断及分型

局灶节段性肾小球硬化症(focal segmental glomerulosclelrosis，FSGS)是一种临床和病理均具特点的肾小球疾病，临床以大量蛋白尿或肾病综合征为特征．病理以局灶和节段分布的硬化性病变为主要变化。根据病因发病机制分为原发性和继发性FSGS两大类，继发性FSGS是指多种病因和发病机制明确的肾小球疾病所导致的局灶肾小球的节段硬化性病变．而原发性FSGS则在病因、发病机制、病变特点、临床表现和预后诸方面均具特点的肾小球疾病，是本文讨论的重点。     

局灶节段硬化性肾小球肾炎的中西医结合治疗研究进展

<正>局灶节段硬化性肾小球肾炎(focalsegmental glomerular sclerosis,FSGS)是一组病理诊断学名词,其病理表现为局灶性(50%的肾小球受累)、节段性(非累及整个肾小球)肾小球硬化性病变,常伴不同程度的小管间质损害。免疫荧光显示IgM及C3呈团块样沉积在肾小球病变区,电镜可见足突广泛融合。根据其硬化部位及细胞增殖特点的不同,可分为非特殊型、门周型、顶端型、塌陷型、细胞型5个亚型。临床上以非特殊型最多见,约占半数以上[1]。塌陷型治疗反应差,进展快,顶     

老年人局灶节段性肾小球硬化的激素治疗及预后

老年人局灶节段性肾小球硬化的激素治疗及预后［英］／ＮａｇａｉＲ…／／ＣｌｉｎＮｅｐｈｏｌ．－１９９４，４２（１）．－１８～２１在老年患者的肾活检中特发性局灶节段性肾小球硬化（ＦＳＧＳ）罕见．该文在８２２例肾活检中６０岁以上病人仅１７例为ＦＳＧＳ约占２...     

木村病合并肾病综合征临床病理特点和转归分析——2例并文献复习

报道2例木村病合并肾病综合征病例并进行文献复习,为临床诊治提供参考。两例患者均因水肿入院,既往组织活检诊断木村病明确,临床表现符合肾病综合征。病例1肾脏病理结果为局灶球性肾小球硬化,予糖皮质激素治疗后症状快速缓解。病例2肾脏病理结果为慢性肾小管间质性肾炎伴系膜增生性肾小球病变及局灶节段性肾小球硬化,使用糖皮质激素治疗后...     

刘光珍教授临床运用凉血散血法治疗慢性肾脏病的经验

正慢性肾脏病起病隐匿,病程长,病理表现复杂多样,常见病理表现有肾小球球性硬化、节段性毛细血管塌陷或狭窄,血管襻挤压、闭塞,球囊黏连,系膜基质增多,基底膜增厚,肾小管灶状萎缩,肾间质灶状淋巴细胞和单核细胞浸润及纤维化等[1]。导师刘光珍在临床诊疗过程中发现,中医肾络瘀阻、肾微癥瘕证候表现与肾脏病理诊断为肾小球硬化、肾间质纤维化等患者的临床表现甚为相似。导师在临床慢性肾脏病诊疗过程中衷中参西,注重中西医结合,辨证地认识中医肾病与现代医学肾脏     

Membranous nephropathy in Schimke immuno-osseous dysplasia

Schimke immuno-osseous dysplasia is a rare autosomal recessive multi-system disorder, with clinical features of growth retardation, spondylo-epiphyseal dysplasia, nephrotic syndrome and immunodeficiency beginning in childhood. Here, we report a new case, in a 10-year-old boy with characteristic symptoms of Schimke immuno-osseous dysplasia. The patient presented with short stature and, later, developed nephrotic syndrome and peritonitis. In addition, he had perinuclear anti-neutrophilic cytoplasmic antibody (p-ANCA)-positive arthritis. Renal pathology of the patients with this disease usually show focal segmental glomerulonephritis, whereas our patient had membranous nephropathy, which has not previously been reported.     

Generalized atherosclerosis sparing the transplanted kidney in Schimke disease

Schimke-immuno-osseous dysplasia (SIOD) is a multisystem disorder caused by a mutation of the chromatin remodeling protein. The main clinical findings are spondyloepiphyseal dysplasia with dysproportional growth deficiency, nephrotic syndrome with focal and segmental glomerulosclerosis, and defective cellular immunity. Transitory ischemic attacks due to vaso-occlusive processes are still an untreatable and life-limiting complication in patients with SIOD. The underlying pathophysiology of vaso-occlusive processes in SIOD is unclear. We report the clinical and pathological findings of the eldest published patient with the severe form of SIOD, who died at the age of 23 years due to pulmonary hypertension with subsequent right heart failure. The autopsy revealed a severe generalized atherosclerosis including the brain, heart, and pulmonary arteries. However, the kidney that was transplanted at the age of 5 years showed a good graft function without glomerular sclerosis and with only minimal nephrosclerosis on histology. Thus, the absence of severe vaso-occlusive processes in the transplanted organ and in the severely atherosclerotic host may indicate that the vaso-occlusive processes in SIOD are not caused by post-transplant cardiovascular morbidity such as arterial hypertension and hyperlipidemia. Instead, vascular factors of the host such as endothelial dysfunction may explain the pathophysiology of atherosclerosis in SIOD.     

脂蛋白肾病——一种新型的肾小球疾病伴进行性硬化

报道一例中国成年女性脂蛋白肾病。对肾穿组织进行光镜、电镜及免疫荧光观察。结果 本例临床上以大量蛋白尿、肾病综合征为主要表现。肾活检显示肾小球毛细血管腔高度扩张，腔内有多量脂蛋白血栓充填，苏丹３染色阳性，电镜显示腔内充满各种大小的空泡状脂肪凝聚物，呈簇状或层状排列。两个月后行重复肾活检显示，扩张的血管腔明显减少，脂蛋白血栓已逐渐为系膜增生及节段性硬化病变所取代。结论 经病理确诊为脂蛋白肾病。     

Dense intramembranous deposit disease: a clinical comparison of histological subtypes

The accumulation of osmiophilic dense deposits in glomerular mesangial and basement membranes (dense intramembranous deposit disease, or DIDD) is associated with variable histologic alterations of the kidneys. We compared clinical features and long-term renal outcome in 21 patients representing two histologic subtypes of DIDD, namely membranoproliferative glomerulonephritis (MPGN) and focal segmental glomerulonephritis (FSGN). We found that MPGN-type DIDD in 12 patients was associated with nephrotic syndrome in 12, persistent hypocomplementemia in 10 and progression to chronic renal insufficiency in 8. In 9 patients with the focal segmental variety of DIDD, nephrotic syndrome was observed in 3, persistent hypocomplementemia in none, and progression to renal insufficiency in 2 (significance: nephrotic syndrome, p = 0.001; persistent hypocomplementemia, p = 0.0001; chronic renal insufficiency, p = 0.02). In one patient transition from focal segmental to MPGN-type DIDD was observed. We conclude that DIDD is a heterogeneous disorder, and that certain clinical and histologic features may be useful in predicting ultimate outcome.     

Clinical follow-up of 54 patients with IgM-nephropathy

The clinical course of mesangial glomerulopathy with IgM deposits (IgM-nephropathy) was studied in 54 patients. The initial manifestations of the disease were nephrotic syndrome in 18, proteinuria in 21, proteinuria together with hematuria in 4 and isolated hematuria in 11 patients. The nephrotic syndrome was steroid-responsive in 60% of cases and of these 80% were steroid-dependent. During a 5-year postbiopsy follow-up 3 patients went into terminal uremia and in 6 more patients a milder renal insufficiency was observed. Three patients were rebiopsied and in 2 of these the second biopsy specimen disclosed typical focal and segmental glomerulosclerosis. Hematuria was a favorable sign, as no patient with hematuria showed progressive impairment of renal function. The prevalence of hypertension in the whole material was 37%. At close of follow-up 35% of all patients were in clinical remission. It is suggested that IgM-nephropathy associated with abundant proteinuria or the nephrotic syndrome represents a distinct disorder from that associated with hematuria. While the nephrotic type often manifested itself with a morphologic change and a tendency to develop renal insufficiency, the hematuric type showed female predominance, a high tendency to spontaneous clinical remission and a favorable clinical course.     

Clinical observations of mycophenolate mofetil therapy in refractory primary nephrotic syndrome

SUMMARY:   Mycophenolate mofetil (MMF) is an effective immunosuppressive agent in renal transplantation, and preliminary studies suggest that it may also be effective in the treatment of lupus nephritis. This study investigated the efficacy and safety of MMF therapy in patients with refractory primary nephrotic syndrome in a prospective multicentre clinical observation. Nineteen refractory nephrotic patients with minimal change disease or mesangial proliferative glomerulonephritis were enrolled in this study. Combined MMF and prednisone therapy was used for 6 months with an initial MMF dose of 1.0–2.0 g/day and a prednisone dose of 20–60 mg/day; both drugs were tapered gradually. It was found that all patients achieved clinical remission and 11 of 19 responded within 4 weeks, and 12 of 19 patients entered complete clinical remission. The prednisone dose in those patients who were previously steroid dependent could be successfully tapered. During follow up, three patients experienced transient increasing of proteinuria associated with infections and recovered without an adjustment of therapy. One patient was withdrawn from the study because of a fall in haemoglobin levels; other adverse effects did not necessitate withdrawal. Follow-up renal biopsies in two patients found no alteration in renal pathology. Mycophenolate mofetil is an effective and well-tolerated immunosuppressive agent for patients with refractory nephrotic syndrome.     

IgA肾病肾病综合征的临床和病理研究

目的：了解IgA肾病肾病综合征的临床和病理的特点及其与预后的关系。方法：观察72例经肾活检确诊为IgA肾病表现为肾病综合征的患的临床表现、实验室检查、病理改变、免疫组化、组织学定量分析及其与转归的关系。结果：患常见表现有高血压、肉眼和镜下血尿、贫血、氮质血症,病理改变多样;治疗后有22例完全缓解,15例部分缓解,16例呈持续性非肾病性蛋白尿,8例呈持续肾病性蛋白尿,10例发展为肾功能衰竭;其转归或激素敏感性与临床表现、蛋白尿选择性、小球病理形态、区膜区增宽和免疫沉着、小管间质病变及肾间质T淋巴和单核细胞分布显相关。结论：IgA肾病肾病综合征可有不同预后,其预后与临床表现、实验室发现、病理特点和免疫发病机理有关。     

成人特发性膜性肾病91例临床病理分析

目的:探讨成人特发性膜性肾病(IMN)的临床与病理特点,分析年龄因素和有无伴局灶节段性肾小球硬化(FSGS)对IMN临床病理的影响。方法:回顾性分析温州地区2006年9月～2011年4月经临床及肾活检诊断为IMN且具有完整临床病理资料的患者91例,分别按年龄(16岁～30岁,31岁～60岁和61岁～80岁)和有无FSGS(FSGS+和FSGS-)分层,分析其临床、病理特征的关系。结果:IMN好发于中年男性,31岁～60岁居多(62.6%),以浮肿(80.2%)、肾病综合征(52.7%)、高血压(47.3%)、镜下血尿(63.7%)为主要临床表现,肾衰竭(3.3%)较少,病理分期以Ⅰ、Ⅱ期为主。61岁～80岁组中肾病综合征以及小管间质和小血管病变重的相对较多,血尿素氮较高,血白蛋白和eGFR较低。FSGS+和FSGS-两组间临床表现、实验室指标及病理指标均差异无统计学意义。多因素二元Logistic回归分析显示血肌酐、病理分期是发生FSGS的主要危险因素。结论:年龄大的IMN患者临床和病理表现较重的比例相对增加,以血尿素氮、肾小球滤过率、小管间质和小血管病变方面较突出。血肌酐和病理分期是发生局灶节段性肾小球硬化的独立危险因素。     

Younger onset myeloperoxidase-specific antineutrophil cytoplasmic antibody- (MPO-ANCA) related glomerulonephritis accompanied with nephrotic syndrome

It is known that nephrotic syndrome rarely accompanies myeloperoxidase-specific antineutrophil cytoplasmic antibody- (MPO-ANCA) related glomerulonephritis. We present a case of younger onset MPO-ANCA-related glomerulonephritis accompanied with nephrotic syndrome in a female patient. It was diagnosed through the renal biopsy and the detection of a high titer of MPO-ANCA and steroid therapy (intravenous steroid pulse therapy and oral administration), anticoagulant therapy and antiplatelet therapy were initiated. Since her nephrotic syndrome persisted in spite of the decrease of MPO-ANCA, we conducted a second renal biopsy. We found active necrotizing crescentic glomerulonephritis with a small deposition of immunoglobulin and fibrinogen on the glomeruli. To suppress her disease activity, we administered second steroid-pulse therapy and MPO-ANCA titer disappeared. However, as her nephrotic syndrome, which was accompanied by severe hyperlipidemia, persisted, we tried to treat her using low-density lipoprotein (LDL) apheresis. It was effective temporarily, but she finally fell into end-stage renal failure. We discuss here the possibility of double nephropathy by considering her clinical and renal pathologic features.