系膜增殖性肾炎伴急性间质性肾炎

系膜增殖性肾炎伴急性间质性肾炎陈惠萍，张景红许多类型的肾小球肾炎可以存在程度不一的肾间质、小管损害；在原有肾小球疾病的基础上也能发生急、慢性间质性肾炎［１、２］。由于二者的临床经过与预后、治疗完全不同，因而必须加以区分，对症治之。现就笔者近期遇到的一...     

益肾胶囊治疗系膜增殖性肾小球肾炎35例

系膜增殖性肾小球肾炎 (以下简称MesPGN)是成人肾病综合征的主要病理类型之一。西药治疗以糖皮质激素和(或 )细胞毒类药物为主。近三年 ,我们在应用西药治疗的基础上 ,在激素减量阶段加用益肾胶囊取得满意的疗效 ,现报告如下。资料与方法1　肾病综合征的诊断标准　参照《内科学》(第 5版 .北京 :人民卫生出版社 ,2 0 0 0 .5 32 - 5 4 1)的诊断标准。 (1)2 4h尿蛋白定量≥ 3.5 g ;(2 )血浆白蛋白 <35g/L ;(3)水肿 ;(4)高脂血症。其中 (1) (2 )条必须具备。2　病例入选标准　所有患者都是 3年内经肾活检病理诊断为MesPGN的病例。并排除Ig…     

表现为系膜增殖性肾炎的抗肾小球基底膜病一例

患者男性,２１岁,因间断咯血９个月、尿检异常４个月于１９９８年３月３日入院。患者１９９７年６月元诱因出现咳嗽、咯痰,痰中带血,为鲜红色,不伴有发热、胸痛,在当地医院诊断为“支气管扩张”予‘杭生素”静滴后,咳嗽、咯血消失。同年９月再次出现上述症状,咯血量约为１００ ｍｌ,肺部ＣＴ检查示双肺布满结节状阴影,怀疑“粟粒性肺结核”,但痰查抗酸杆菌３次均阴性,结核菌素（ＰＰＤ）试验阴性,于抗结核治疗（异烟肼、奥复星及利福平）１个月后,于同年１０月复查胸片肺部阴影全部消失。检查尿常规时发现：蛋白＋＋＋,潜血…     

肾小球肾炎患者活检标本中肾小管间质损害的意义

已知在原发性肾小球肾炎(GN)的肾活检标本尤其是含有硬化肾小球的标本中可以观察到肾小管间质损害(TIL),然而这类损害有时常被忽视或认为意义不大。作者对成人GN的常见类型作了回顾性研究,以评价TIL在估计GN的严重性和预后方面的意义。本研究包括IgA肾病(IgA-N)101例,IgA阴性的增殖性肾炎(PGN)31例,特发性膜性肾小球肾炎(IMGN)75例。平均随访时间为72个月。对上述病例的肾活检标本作了常规染色及检     

活血软坚方对大鼠膜性肾小球肾炎肾小管间质损害影响的实验研究

目的：观察活血软坚方对大鼠膜性肾小球肾炎（MN）肾小管间质损害的影响，并探讨活血软坚中药对MN伴发小管间质损伤的作用机制。方法：用阳离子化牛血清白蛋白复制大鼠MN模型，将实验动物随机分为正常组、模型组、雷公藤组、治疗组，观察24h尿蛋白、血浆白蛋白、胆固醇、三酰甘油、血肌酐、尿素氮等生化指标，并对肾组织进行光镜、电镜、免疫荧光观察；采用RT—PCR的方法检测ColⅠmRNA和ColⅢmRNA的表达。结果：本方能明显降低蛋白尿及血清胆固醇、三酰甘油、血肌酐、尿素氮，升高血清白蛋白，减少免疫复合物沉积，改善肾小球及肾小管的病理损伤。结论：活血软坚方能减轻尿蛋白对肾小管的损伤，减少细胞外基质在肾间质的积聚，减轻肾脏病理损伤，从而达到保护肾功能的作用。     

骨碎补类黄酮对系膜增殖性肾小球肾炎大鼠模型的抑制作用

目的:探讨骨碎补类黄酮提取物(flavonoid fraction,FF)对单克隆抗体OX-7诱导的系膜增殖性肾小球肾炎大鼠模型(anti-Thy 1.1 GN)的作用.方法:利用单克隆抗体OX-7诱导的anti-Thy 1.1 GN动物模型,将大鼠随机分成正常对照组、模型组(Thy 1.1 GN)、Thy 1.1 GN FF组、FF组.检测尿蛋白,三色染色评估肾小球细胞外基质改变,免疫组化染色检测增殖细胞核抗原(PCNA)、ED-1及α-SMA在肾小球的表达,同时检测肾皮质超氧化物歧化酶(SOD)活性.结果:Thy 1.1 GN FF组尿蛋白量及三色染色面积均比Thy 1.1 GN组减少(分别P＜0.05和P＜0.01);免疫组化显示PCNA、ED-1阳性细胞数及α-SMA在肾小球表达下调,均与Thy 1.1 GN组有统计学差异(P＜0.01);FF还可加快anti-Thy 1.1 GN模型肾皮质SOD活性的恢复(P＜0.01).结论:FF通过抗氧化活性,抑制大鼠系膜增殖性肾小球肾炎系膜细胞的增殖及基质的增加.     

肾疏宁对大鼠系膜增生性肾炎的肾小管间质损害的形态学影响

目的 :观察中药肾疏宁对系膜增生性肾炎 (MsPGN )的肾小管间质损伤的影响。方法 :制作大鼠MsPGN模型。予肾疏宁治疗并以肾炎康复片为对照组 ,观察肾脏形态学及 2 4h尿蛋白定量。结果 :肾疏宁能明显减轻MsPGN大鼠的蛋白尿 ,减轻肾组织病理病变尤其是肾小管间质损伤 ,且对损伤的肾组织有修复作用。与模型组相比 ,有显著性差异 (P <0 .0 1) ,与肾炎康复片组相比 ,有显著性差异 (P <0 .0 5 ) ;与正常组相比 ,无显著性差异 (P >0 .0 5 )。结论 :肾疏宁能降低尿蛋白、保护肾功能、减轻MsPGN的肾小球病变及肾小管间质损伤。     

亲属活体肾移植术后新发或复发移植肾新月体形肾小球肾炎一例

患者,男性,24岁,于1999年11月因"慢性肾功能衰竭、尿毒症期"在我院接受亲属活体肾移植术.供者为患者的胞兄(30岁),HLA位点为:A 2、11;B 13、67;DR 51、52;受者HLA位点为:A11;B13、54(22);DR 51、53;交叉反应组错配:1;淋巴细胞毒交叉配合试验阴性.切取供者的左肾,供肾动脉与受者髂内动脉端端吻合,供肾静脉与受者髂外静脉端侧吻合,供肾输尿管断端与受者膀胱黏膜间断吻合,浆肌层包埋.术后以大剂量甲泼尼龙及环磷酰胺冲击治疗,术后第3天开始口服环孢素A、霉酚酸酯及泼尼松.术后第7天血肌酐正常,2周后出院.     

YB-1蛋白在大鼠系膜增殖性肾小球肾炎及体外培养大鼠肾小球系膜细胞中的表达及其意义

肾小球系膜细胞增殖是系膜增殖性肾小球肾炎(MsPGN)的常见病理特征,活化后的系膜细胞可分泌多种细胞因子,导致系膜细胞增殖及细胞外基质增生.     

泌尿系结核引起轻度系膜增生性肾小球肾炎一例

患者,男,29岁,1个月前无明显诱因出现眼睑水肿和间歇性肉眼血尿,拟诊急性肾小球肾炎.经青霉素治疗2周,无明显缓解,3 d前症状加重,于2007年3月13日入院.既往病史无特殊.其父于20年前曾有活动性肺结核病史,经抗结核治疗后至今未再复发.体格检查:BP140/96 mm Hg(1 mm Hg=0.133 kPa).     

A case of mesangial proliferative glomerulonephritis with endothelial damage

We describe a 71-year-old man, who had been treated for hypertension, myocardial infarction and abdominal aortic aneurysm, and was admitted to our hospital because of proteinuria(3.9 g/day at the outpatient clinic and 1.5 g/day at the time of admission) and edema in the extremities. Light microscopic study of the kidney biopsy specimen revealed mesangial proliferative glomerulonephritis and glomerular paralysis. Electron microscopic findings showed endothelial damage, including widening of the subendothelial space and detachment of endothelial cells from the glomerular basement membrane. Deposition of immunoglobulins and complement was not detected by immunofluorescence studies. These pathological findings resemble the findings of thrombotic microangiopathy, but there were no clinical pictures of HUS/TTP. These findings suggest that hypertension, atherosclerosis and circulating turbulence caused by an aortic aneurysm induced severe glomerular endothelial damage leading to mesangial proliferative glomerulonephritis without an immune response.     

Endocapillary proliferative glomerulonephritis with crescent formation and concurrent tubulointerstitial nephritis complicating retroperitoneal fibrosis with a high serum level of IgG4

Renal lesions of IgG4-related disease have been reported recently. Most of them are tubulointerstitial nephritis, and a definite glomerulonephritis complicating IgG4-related disease is very rare. We report here a case of definite glomerulonephritis and concurrent tubulointerstitial nephritis complicating retroperitoneal fibrosis with a high serum level of IgG4. A 68-year-old Japanese woman was referred to our hospital for investigation of anasarca. We diagnosed her disease as a nephrotic syndrome and left hydroureteronephrosis due to retroperitoneal fibrosis. Her laboratory data revealed a high serum level of IgG4, renal injury, hypoproteinemia, hypocomplementemia, a positive finding of circulating immunocomplex (CIC), and negative findings ofautologous antibodies suggesting systemic lupus erythematosus (SLE) or Sj?gren's syndrome (SS). A diagnosis of SLE or SS could not be made clinically. Right renal biopsy revealed endocapillary proliferative glomerulonephritis with crescent formation and concurrent tubulointerstitial nephritis. Infiltration of plasma cells in interstitium was more conspicuous than seen with ordinary tubulointerstitial nephritis, and in most of them IgG4 was positive. We placed a percutaneous nephrostomy catheter in her left kidney, and prescribed prednisolone and cyclosporine. The responses to prednisolone and cyclosporine therapies were very good. Further studies are needed to clarify the relationship between glomerulonephritis and IgG4-related disease. However, when considering renal lesions of IgG4-related disease, we think that hypocomplementemia, a positive finding of CIC, negative findings of autologous antibodies suggesting SLE or SS, conspicuous interstitial infiltration of IgG4-positive plasma cells, and a good response to steroid or immunosuppressant therapy are key points.     

A case of fulminant acute poststreptococcal glomerulonephritis showing mesangiolysis and crescent formation preceded by erysipelas

A 66-year-old man with erysipelas was admitted with complaints of oliguria and massive proteinuria/hematuria. He was diagnosed as having acute poststreptococcal glomerulonephritis(APSGN) due to erysipelas infected by group A streptococcus pyogenes. On admission, his white cell count increased to 31,000, and CRP was 27.3 mg/dl. Serum urea nitrogen and creatinine were increased to 90.1 mg/dl and 4.5 mg/dl, respectively. He had diabetes mellitus(HbA1c 7.9%) and liver dysfunction(total bilirubin 3.5 mg/dl, AST 76 IU, ALT 41 IU) caused by alcoholic liver cirrhosis. Hypocomplementemia was found in addition to ASO 216 U/ml and ASK 10,240 x. After antibiotics treatment was initiated, inflammation of the erysipelas began to improve. Disseminated intravascular coagulation syndrome, probably due to sepsis, occurred on the 5th hospital day. He died of gastrointestinal bleeding on the 18th hospital day. Renal autopsy revealed 37% formation of fibrocellular crescents, and marked mesangiolysis was noted by light microscopy. Granular deposition of C3 and IgG was seen along the capillary walls on immunofluorescence study. Intramembranous deposits were scattered on electron microscopy. This case illustrates a fulminant type of APSGN, which was in part attributed to the presence of diabetes and alcoholic liver cirrhosis. Histological findings of crescent formation and marked mesangiolysis may account for the fulminant clinical course.     

黑热病继发膜增生性肾小球肾炎一例

患者，男，42岁，因间断性头晕10年，水肿2年，发热、恶心1个月，以“高血压良性小动脉性肾硬化、发热原因待查”收住院。患者于10年前出现头晕症状，多次测血压高于正常，最高时达210／160mmHg。近2年多次于劳累后出现足踝部水肿，未行诊治。1个月前，患者出现发热、乏力、恶心等症状，在当地医院测体温38℃，查血常规“全血细胞减少”，尿蛋白（＋），BUN16．4mmol／L，Scr174μmol／L,B超示肝脾肿大，给予抗感染等治疗未见明显好转，故来我院。     

Unusual presentation of mesangial proliferative glomerulonephritis in HELLP syndrome associated with acute renal failure

Acute renal failure in pregnancy is not common in industrialized countries. HELLP syndrome (hemolysis, elevated liver enzyme, and low platelets) was one of the causes of acute renal failure in pregnancy, but renal pathological findings in case of acute renal failure had rarely been reported. We reported an unusual case of HELLP syndrome with acute renal failure requiring renal replacement therapy and which histopathologic findings of kidney biopsy showed mesangial proliferative glomerulonephritis and her renal function completely recovered after immediate artificial abortion, supportive management, transfusion of blood products, and hemodialysis.     

原发性胆汁性肝硬化合并轻度系膜增生性肾小球肾炎一例

我们报告1例原发性胆汁性肝硬化合并轻度系膜增生性肾小球肾炎及其诊治经过。患者，女性，68岁，因眼睑、双下肢水肿3个月于2004年3月22日入院。血压正常，体检无异常发现。     

Long-term renal outcome in pediatric glomerulonephritis associated with crescent formation

Background

Information on long-term renal outcome of pediatric glomerulonephritis associated with crescent formation is limited. A single center retrospective study was conducted to assess long-term renal survival and to determine whether the 2010 classification for antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis can predict renal outcome in pediatric glomerulonephritis associated with crescent formation.

Methods

Biopsy and clinical data of children, aged ≤ 18 years with ≥ 10 glomeruli and ≥ 10% crescentic glomeruli during January 1998 to December 2015, were reviewed. Biopsies were classified according to the 2010 classification into focal, crescentic, mixed, and sclerotic classes. The clinical endpoint was end-stage renal disease (ESRD).

Results

Of 72 children, 14 patients (19.4%) had positive ANCA. The biopsy indication was rapidly progressive glomerulonephritis in 38 patients (52.8%) and 22 patients (30.6%) required dialysis at onset. Lupus nephritis was the most common diagnosis (43.1%), followed by IgA nephropathy/Henoch–Schoenlein purpura (HSP) (22.2%). ESRD occurred in 18 patients (25%) and the risk of ESRD differed among the histological classifications (p < 0.001). Dialysis at onset and sclerotic class was independent predictors of ESRD in an adjusted model. The risk of ESRD was four-fold higher in patients requiring dialysis at onset and 7.7-fold higher in sclerotic patients than in crescentic patients.

Conclusions

The probability of ESRD was substantial in pediatric glomerulonephritis associated with crescent formation. The 2010 classification is useful for establishing long-term renal prognosis. Future research is required to validate whether histological classification could be a determinant in therapeutic guideline modification, since long-term renal prognosis is different in each class.