纤维样肾小球病并发免疫触须样肾小球病一例

我科近期收治一例患者,病理示纤维样肾小球病并发免疫触须样肾小球病,报告如下. 患者女,35岁,因蛋白尿3+1个多月入本院.查体:T36.5℃,BP 110/60 mm Hg,心肺腹部无异常,颜面双下肢无水肿.实验室检查:尿蛋白3+,24 h尿蛋白量1.62 g.血WBC 3.14×109/L,Hb 89 g/L.自身抗体RNP、抗组蛋白抗体阳性,余阴性.补体C30.66 g/L,C40.08 g/L.乙肝三对阴性,HCV抗体阴性,类风湿因子阴性,冷球蛋白正常.     

纤维样肾小球病并发免疫触须样肾小球病一例

我科近期收治一例患者,病理示纤维样肾小球病并发免疫触须样肾小球病,报告如下. 患者女,35岁,因蛋白尿3+1个多月入本院.查体:T36.5℃,BP 110/60 mm Hg,心肺腹部无异常,颜面双下肢无水肿.实验室检查:尿蛋白3+,24 h尿蛋白量1.62 g.血WBC 3.14×109/L,Hb 89 g/L.自身抗体RNP、抗组蛋白抗体阳性,余阴性.补体C30.66 g/L,C40.08 g/L.乙肝三对阴性,HCV抗体阴性,类风湿因子阴性,冷球蛋白正常.     

免疫触须样肾小球病一例

正患者女性,63岁,2016年11月1日因"反复双下肢水肿2年,血压升高1年"入院。2年前患者无明显诱因出现双下肢凹陷性水肿,伴双膝关节间断疼痛,无膝关节肿胀发红,不伴畏寒、发热、咳嗽、咳痰、心累、气紧,不伴尿频、尿急、尿痛、血尿。患者未加重视,未予治疗。1年前发现血压升高,自服药物控制。水肿反复出现。患病以来患者睡眠差,精神、饮食尚可,大小便正常,体质量无明显变化。既往史否认糖     

免疫触须样肾小球病1例报告

免疫触须样肾小球病（immunotactoid glomerulopathy,ITG）是指肾小球内存在中空的微管样结构的纤维性物质,对淀粉样蛋白特殊染色阴性,是一种罕见的原发性肾小球疾病,目前国内仅见数例报道。我科曾收治1例,现报告如下。     

触须样免疫性肾小球病一例

触须样免疫性肾小球病(immuno-tactoid glomerulopathy,IT)是一种新认识的少见的肾小球病,目前国内仅见数例报道,我们报告1例。 患者男性,18岁,双下肢水肿2个月,加重1个月入院。血压140／80 mmHg,尿常规:蛋白3 ,RBC 5-8／HP。尿C30.45 mg／L,C4 0.11 mg／L。Scr 275txmol／L,Ccr 55.26 ml／min。空腹血糖正常。乙肝病毒免疫学检测各指标正常,肝功能无异常。     

触须样免疫性肾小球病

触须样免疫性肾小球病（immunotactoid glomerulopathy,ITG）是指电镜下肾小球系膜区和／或基底膜内存在直径30～51nm、排列规则的类似淀粉纤维丝样物质或呈中空微管样结构的纤维样物质,但对淀粉样蛋白质特殊染色阴性,一般不伴有系统性疾病的。肾小球疾病。该病于1980年由Schwatz等首次报道,他在电镜下观察到肾小球内较粗的平行排列管样物质,类似昆虫触须,因而命名为触须样免疫性肾小球病。据不完全统计,迄今为止国内外报道已超过20例。     

糖尿病伴触须样免疫性肾小球病一例

触须样免疫性肾小球病(immunotactoid glomerulopathy IT)是近年来由电子显微镜发现的新病种之一．以往报道尚未见并糖尿病者。我们发现一例，报道如下。     

纤维样肾小球病一例

患者：男,37岁,因“反复眼睑及颜面水肿1年多,发现肾功能异常1个多月”于2013年9月5日入院.患者1年前无明显原因出现眼睑及颜面稍浮肿伴泡沫尿,考虑“慢性肾炎”,未行肾活检,经金水宝胶囊及降压药治疗好转出院,出院后坚持服用金水宝胶囊,病情平稳,未定期复查尿蛋白及肾功能,1个多月前患者无明显诱因出现双下肢水肿伴头晕,偶有活动后心累、气促,到外院治疗,查血肌酐＞ 200μmol/L,给予保肾治疗.患者在住院过程中出现腹痛,经治疗无好转转入华西医院.     

膜性肾病并发纤维样肾小球病一例

纤维样肾小球病(fibrillary glomerulonephritis,FGN)是少见的肾小球病,电镜显示肾小球内存在杂乱排列的、直径约20 nm的纤维丝样结构为其特点.现报告1例纤维样肾小球病并发膜性肾病.     

纤维样肾小球病并发过敏性间质性肾炎一例

患者,女性,58岁.2006年7月感冒后眼睑及双下肢水肿,尿蛋白3+,11月水肿加重,乏力纳差.住本市某医院查尿蛋白量(24 h)4.3 g,Alb 28.8 g/L,Scr 162.6 μmol/L,接受泼尼松40 mg/d及对症治疗2个月无效;又接受红花注射液静滴,5 d后,全身出现出血性皮疹伴瘙痒,肾功能恶化出院.     

纤维样肾小球病合并慢性淋巴细胞白血病一例

<正>纤维样肾小球病(fibrillary glomerulopathy,FGP)是肾小球内存在类似淀粉样纤维物质或呈丝状结构的纤维样物质,对淀粉样蛋白质的特殊染色阴性,FGP是一种较为少见的肾小球疾病,占肾活检病例的0.6~1%[1-2],一般不伴有系统性疾病的一类肾小球疾病。近年发现少数纤维样肾小球病例     

A case of rheumatoid arthritis with immunotactoid glomerulopathy]

The patient was a 64-year-old female who had been treated by a local doctor for rheumatoid arthritis and hypertension for 10 years. Malaise and edema developed since July, 1990, and as proteinuria and renal dysfunction were noted, the patient was admitted to our hospital on November 2. On admission, BUN was 33mg/dl, creatinine was 2.5mg/dl, and proteinuria was about 3g/day. Renal biopsy was performed after admission. Light microscopy revealed nodular lobulation of glomeruli and occlusion of loops. Dylon staining was negative. Immunofluorescent study showed granular deposition of IgG, IgM, C3, C4, Clq in the glomerular basement membrane and mesangial area. Electron microscopy showed a large amount of electron dense deposits in the subendothelium and mesangial area and dense aggregation of tubular structure in the deposit, part of which exhibited a profile of fingerprint deposit. The tubular structures were classified into three major types, which were 120, 100, and 50nm in diameter. From these findings, a diagnosis of immunotactoid glomerulopathy was made. After renal biopsy, plasmapheresis and prednisolone were administered, and the patient has been managed conservatively to date.     

淋巴细胞增生症伴糖尿病合并免疫类晶体肾小球病一例

免疫类晶体肾小球病（immunotactoid glomerulopathy,ITG）是一种较为少见的肾小球病，电镜检查显示肾小球内存在有序性微管状或原纤维结构沉积为其特点。现报告1例淋巴细胞增生症伴糖尿病合并免疫类晶体肾小球病病例。     

Fibrillary glomerulonephritis and immunotactoid glomerulopathy

Fibrillary glomerulonephritis is a now widely recognized diagnostic entity, occurring in approximately 1% of native kidney biopsies in several large biopsy series obtained from Western countries. The distinctive features are infiltration of glomerular structures by randomly arranged fibrils similar in appearance but larger than amyloid fibrils and the lack of staining with histochemical dyes typically reactive with amyloid. It is widely but not universally recognized to be distinct from immunotactoid glomerulopathy, an entity characterized by glomerular deposits of immunoglobulin with substructural organization as microtubules and with clinical associations with lymphoplasmacytic disorders. The pathophysiologic basis for organization of the glomerular deposits as fibrils or microtubules in these entities remains obscure.     

Nonamyloidotic fibrillary glomerulopathy

A 29-year-old man had oedema, proteinuria in nephrotic range, haematuria and cardiac arrhythmia (second grade atrioventricular block). The pathologic findings of kidney biopsy showed in light microscopy diffuse mesangial matrix increase with mild mesangial proliferation and variable thickening of the glomerular capillary walls. IgG, c₃ and c_1q were intensely fluorescent and exhibited a diffuse granular pattern in mesangial areas and along the capillary walls. Both kappa and lambda chains were weakly positive in the same pattern. Ultrastructurally, microfibrils of about 20 nm in width were seen to be deposited in mesangial areas and along the glomerular basement membranes. Congo red stain and metachromasia were negative. Neither cryoglobulinaemia nor paraproteinaemia including light chains were found. The aetiology of nonamyloidotic fibrillary glomerulopathy is unknown and no clear-cut clinical or pathologic pattern has emerged. It may represent more than one disease process with a common morphologic expression.