纤维样变肾小球病：——附四例报告

报告４例纤维样变肾小球病。４例病人无系统性疾病，主要临床表现为高血压，大量蛋白尿、镜下血尿。２例有不同程度的肾功能减退。光镜下病理类型为膜增殖性肾炎（２例）、膜性肾病（１例）和系膜增生性肾炎（１例），病理改变均较重，刚果红染色阴性；免疫荧光多数病例（３１４例）以ＩｇＧ、Ｃ＿３为主，呈颗粒样在肾小球系膜区和（或）沿肾小球毛细血管壁沉积；电镜下可见大量纤维样物质在肾小球系膜区和（或）肾小球基底膜内分布，纤维样物质直径经图象分析仪测量为２０．５０±１０．３７ｎｍ。本病诊断主要依靠超微结构检查。本病为国内首次报道。     

吸烟与特发性结节样肾小球硬化症

结节样肾小球硬化是指系膜结节样硬化伴轻度肾小球分叶的一种肾脏病理改变。Kimmelstiel和Wilson在1936年首次描述结节样肾小球硬化是糖尿病肾病（diabetic nephropathy，DN）特征性病理表现，随后人们在膜增殖性肾炎、淀粉样变性等多种肾小球疾病也发现结节样肾小球硬化（表1）。     

脂蛋白肾小球病

脂蛋白肾小球病南京军区南京总医院解放军肾脏病研究所（南京，２１０００２）陈惠萍黎磊石关键词脂蛋白肾小球病脂蛋白栓塞中图法分类号Ｒ６９２６１病例报告患儿男性，１１岁。因浮肿、尿检异常半年入院。缘于１９９６年２月无诱因地出现双睑浮肿，无发热、少尿、肉眼...     

IgA型免疫管状肾小球病

老年男性患者,病程6年,肾脏损伤表现为肾病综合征、大量镜下血尿,肾功能不全及高血压,肾外表现为反复发作的双下肢紫癜样皮疹、补体C3偏低、贫血、血轻链κ/λ比值增高。肾活检组织学改变为肾小球膜增生样病变,免疫荧光以Ig A沿肾小球毛细血管袢沉积为主,电镜下肾小球系膜区、内皮下、上皮侧见平行排列、直径均一的中空微管状物质。该患者最终诊断为Ig A型免疫管状肾小球病。     

甲氨蝶呤对类风湿关节炎成纤维样滑膜细胞表达RANKL的影响

目的 探对类风湿关节炎（rheumatoid arthritis，RA）滑膜细胞中表达核因子kB受体活化子配体（RANKL）的细胞以及甲氨蝶呤（MTX）对其表达的影响。方法 收集原代RA及正常滑膜细胞，用免疫磁珠法筛选滑膜CD68^＋和CD68^-细胞，CD44免疫组织化学染色观察以及RANKL免疫荧光检测；细胞体外培养并在培养液中加入最终浓度为1mg／L的MTX，用双夹心法酶联免疫吸附试验（ELISA）检测培养液中RANKL含量以及评估每一细胞RANKL表达量。结果 在滑膜细胞中，CD68^＋滑膜细胞为巨噬样细胞，CD44、RANKL染色阴性；CD68^-滑膜细胞为纤维样细胞，CD44、RANKL染色阳性。RA滑膜CD44^＋CD68^-纤维样细胞RANKL蛋白表达量显著增高，MTX对其RANKL表达有抑制作用（P〈0．01）。正常滑膜纤维样细胞低表达RANKL，MTX对其RANKL表达抑制作用来自对细胞增殖的抑制。结论 滑膜组织中有RANKL表达，这些细胞为CD44^＋/CD68^-纤维样细胞，RA滑膜纤维样细胞RANKL分泌增高并受到MTX抑制。MTX可通过抑制RANKL减少关节周围骨破坏作用。     

纤维连接蛋白肾小球病

纤维连接蛋白肾小球病 ,也称纤维连接蛋白沉积肾小球病 (glomerulopathywithfibronectindeposits ,GFND) ,是近年来才被认识的一种遗传性肾小球病 ,其发病较为罕见。据不完全统计 ,自 1980年以来 ,全世界共报道了约 13个家系 ,患者总数约 4 8人[1～ 10 ] 。GFND病例始先报道于欧美国家 ,1998年日本人Sato等[8] 报道了亚洲第一例GFND ,随后日本人Uesugi等[9] 又报道了 4个家庭的 5例患者 ,而我国尚未见报道。本文就GFND的命名、临床表现、病理改变、诊断和鉴别诊断、治疗、遗传学特征、相关基因研究以及发病机制作一综述。1　命　　…     

新近认识的三种肾小球疾病

新近认识的三种肾小球疾病陈惠萍朱茂艳关键词肾小球疾病蛋白尿肾病综合征中图法分类号Ｒ６９２．６近年来，文献中报道了三种非免疫因素介导的肾小球疾病，它们是胶原纤维变性的肾小球病或称Ⅲ型胶原肾小球病（ｃｏｌ－ｌａｇｅｎｏｆｉｂｒｏｔｉｃ（ｔｙｐｅⅢｃｏｌａ...     

Toll样受体与炎症性肠病关系探讨

Toll样受体家族是一种识别微生物体上保守结构——病原体相关的分子结构的模式识别受体。它可以表达于肠道粘膜的多种细胞中，介导微生物与细胞之间的相互作用，在粘膜免疫中发挥重要作用。近年来的研究显示，炎症性肠病与肠道免疫异常和粘膜稳态破坏相关，而Toll样受体在其中具有重要的作用。     

Toll样受体在炎症性肠病发病机制、并发症及治疗中的作用研究进展

炎症性肠病（IBD）是一种多因素导致的慢性非特异性自身免疫性疾病，发病机制复杂。Toll样受体（TLR）是模式识别受体，可诱导炎症反应。TLR在IBD中异常表达，在IBD的发生发展中占重要地位。TLR通过髓样分化因子（MyD88）依赖性通路和MyD88非依赖性通路影响肠黏膜屏障与免疫细胞分化，参与IBD的发生发展；TLR与IBD并发症密切相关，TLR下游炎症因子可促进肠纤维化，诱导炎症相关性结直肠癌，还可促进IBD患者血栓形成；临床可基于TLR基因多态性治疗IBD并判断预后，益生菌或内外源性调控物质可通过TLR通路改善IBD病情。     

移植肾C3肾小球病的诊治

C3肾小球病是新近才被提出的一种以肾小球C3沉积为主的疾病,包括C3肾小球肾炎和致密物沉积病。C3肾小球病肾移植术后具有很高的复发风险。该病主要的病理生理改变是遗传性或者是获得性补体系统本身或者其调控系统的异常,最终导致C3持续的活化而沉积于肾小球系膜区和毛细血管襻,因此基因检测在C3肾小球病中具有重要作用。移植肾C3肾小球病临床表现各异,主要为血尿、蛋白尿以及血肌酐升高。移植肾活检多表现为膜增生样改变。移植肾C3肾小球病无特殊治疗方案,新近有报道针对补体的单克隆抗体Eculizumab对部分病人有效,但仍需要大样本研究进一步证实其疗效。     

肾小球微管状免疫球蛋白沉积

55岁男性,肾病综合征伴血清肌酐升高,肾外有贫血、低补体血症,肾活检组织学改变为肾小球系膜区及内皮下区域大量PAS强阳性物质沉积,免疫病理示多种免疫球蛋白及补体广泛沉积于肾小球,电镜证实沉积物为排列紊乱的中空管状物。该患者最终诊断为免疫管状肾小球病。     

肥胖相关性肾病的研究进展

随着肥胖成为流行性疾病,肥胖相关性肾病(ORG)的发生率明显升高.ORG的发生与胰岛素抵抗、高瘦素血症、脂代谢异常、肾素-血管紧张素-醛固酮系统(RAAS)激活和氧化应激等因素密切相关.生活方式改变、减轻体重、使用改善胰岛素抵抗药物、抑制RAAS活性药物以及选择性抗炎药物等有助于延缓ORG进展.     

塌陷性肾小球病

塌陷性肾小球病(collapsing glomerulopathy,CG)在病理上属于塌陷型局灶节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)的一种,病理特征表现为局灶或球性毛细血管袢塌陷伴足细胞肥大、增生,同时肾小管间质损伤显著。1979年报道了第一例CG,因其发病原因不清,传统免疫抑制治疗效果差,肾脏存活率低,目前已成为终末期肾病的重要原发病之一。过去20多年,CG发病机制的研究取得了长足进展,近年研究的重点在于CG易感基因筛选,足细胞受损、增生的机制,包括细胞周期调控紊乱、异常信号通路激活等。本文就CG发病机制的最新进展作一简述。     

Immunotactoid glomerulopathy

We present 11 patients with immunotactoid glomerulopathy, a new syndrome characterized clinically by proteinuria (11/11), microscopic hematuria (9/11) and hypertension (9/11). The patients consisted of six females and five males, aged 25 to 59 years (mean, 44.6). Proteinuria was the presenting feature and the reason for renal biopsy in all patients. The diagnosis of immunotactoid glomerulopathy was established at renal biopsy by the presence of glomerular extracellular microtubules composed of immune reactants. All the biopsies studied by immunofluorescence (10 cases) had glomerular deposits of IgG and C3. In three biopsies studied with IgG subclass specific antisera, only one patient had monoclonal immunoglobulin deposits (IgG3 kappa). In six cases the glomerular deposits were analyzed for light chains. In three the deposits contained kappa only, and three consisted of both kappa and lambda. In two cases the immune aggregates were confined to the mesangium, and in the remaining eight cases, the deposits were present in the mesangium and the glomerular basement membranes. Electron-dense deposits composed of microtubules were present in the same distribution within the glomerulus as the immune reactants. The microtubules had a uniform diameter in each biopsy, but they varied in size from case to case. They were approximately the same size in eight cases (mean, 22.3 +/- 3 [SD] nm). Three cases had much larger microtubules: 34.2 nm, 35.4 nm, and 48.9 nm in diameter. Although the 22.3-nm microtubules resembled amyloid in their appearance, glomerular distribution and random orientation in the tissue, they were more than twice the diameter of amyloid (8.9 nm), and Congo red and thioflavin T stains for amyloid were negative. Similar microtubular structures have been described in patients with cryoglobulinemia, SLE and paraproteinemia, but these diseases were excluded in our patients on clinical, serologic and in some cases histologic grounds. More important, none of our patients had clinical or histochemical evidence of amyloidosis, an entity which may be confused with immunotactoid glomerulopathy on a morphologic basis. Follow-up, from 22 to 94 months (mean, 52.6) was obtained in all 11 patients, and 2 clinical courses were noted. Six patients had progressive deterioration of renal function, with five requiring dialysis. This group had severe hypertension (4/6) and nephrotic-range proteinuria (5/6) at some point in their course. The remaining five patients with stable renal function had proteinuria of less than 2.0 g/24 hr in most cases (4/5), and none had severe hypertension. This dichotomy correlated with the distribution of immunotactoids.(ABSTRACT TRUNCATED AT 400 WORDS)     

C3肾小球病

以往研究证实多种肾小球肾炎发病机制与补体系统,尤其是补体旁路途径调节异常相关。近年有作者发现一组免疫荧光染色单纯补体C3沿肾小球毛细血管袢沉积的肾小球肾炎,不伴或伴少量免疫球蛋白沉积,其发病机制可能与先天或后天获得性补体系统调节异常相关,该作者将这一组疾病统一命名为C3肾小球病,并根据其临床表现及可能的发病机制分为不同类别。本文就这类疾病作一综述,旨在关注补体在疾病发生中的作用机制,并提高对此类肾小球肾炎的认识。