儿童C1q肾病临床病理研究

目的 :探讨儿童C1q肾病临床病理特征及诊断与治疗。方法 :分析 8例C1q肾病患儿临床病理特点及激素或免疫抑制剂治疗效应 ,并与同期 77例原发性NS患儿作比较。结果 :8例C1q肾病患儿临床上大多表现为原发性NS(6例 ) ,仅 2例表现为肾炎综合征和单纯性血尿。LM主要包括MC(3例 )、MsPGN(2例 )、FSGS(2例 )和ECPGN(1例 )。IF显示明显的系膜区C1q沉积 ,伴或不伴有Ig和补体沉积。EM检查仅 1例有系膜区和内皮下电子致密物沉积。与原发性NS相比 ,6例表现为NS的C1q肾病患儿对泼尼松初次治疗产生耐药的相对危险度为 2 1(P <0 .0 0 1) ,但对免疫抑制剂治疗均敏感。结论 :儿童C1q肾病临床上以对激素耐药的NS为常见表现 ,IF是其主要诊断依据 ,使用免疫抑制剂治疗有效     

伴C1q沉积的原发性IgA肾病患儿临床病理及预后分析

目的 探讨伴C1q沉积的IgA肾病患儿的临床、病理改变特征及其预后分析。 方法 回顾性分析2000年1月至2017年12月于东部战区总医院肾活检确诊为原发性IgA肾病患儿的临床病理资料,根据肾小球免疫荧光检查是否有C1q沉积分为C1q沉积组和C1q阴性组。随访终点事件包括血肌酐翻倍、或估算肾小球滤过率（eGFR）下降超过50%、或进入终末期肾脏病期、或接受肾脏替代治疗、或死亡。采用Kaplan-Meier曲线比较两组患儿肾脏生存率的差异;单因素及多因素Cox回归模型法分析C1q沉积对IgA肾病患儿预后的影响。 结果 120例IgA肾病患儿入选本研究,其中C1q沉积组60例,C1q阴性组60例。C1q沉积组基线eGFR、血浆白蛋白水平低于C1q阴性组,血肌酐、血总胆固醇、24 h尿蛋白量水平高于C1q阴性组（均 P<0.05）。C1q沉积组系膜细胞增殖（M）、肾小管萎缩/间质纤维化（T）、细胞/纤维细胞性新月体（C）评分高于C1q阴性组（均 P<0.05）。中位随访时间78.9（66.3,184.1）个月,Kaplan-Meier生存曲线分析结果显示,两组患儿肾脏累积生存率的差异有统计学意义（Log-rank检验 χ ²=6.801, P<0.01）。Cox回归分析结果显示,C1q沉积组患儿出现肾脏终点事件的风险较C1q阴性组患儿增加了5.772倍（ HR=5.772,95% CI：1.353~24.6211, P=0.018）。 结论 伴C1q沉积的IgA肾病患儿临床、肾脏病理改变程度较C1q阴性患儿严重,肾脏预后较差。C1q沉积是儿童IgA肾病肾脏预后不良的独立危险因素。     

C1q肾病的诊断及治疗

C1q肾病是一种以系膜增生为主的肾小球疾病,其特点为免疫荧光染色可见系膜区高强度C1q沉积,电镜下可见系膜区电子致密物沉积,根据组织病理学特点主要分为3类,包括微小病变(MCD)、局灶节段性肾小球硬化(FSGS)和免疫介导的增生性肾小球肾炎。C1q肾病的临床表现具有多样性,可表现为肾炎或肾病范围内蛋白尿,伴有或不伴有血尿和肾功能损伤。虽然目前糖皮质激素是治疗C1q肾病的主要方法,但多数研究认为C1q肾病对糖皮质激素治疗反应较差。中西医结合治疗有望提高C1q肾病的疗效。     

C1q肾病伴氮质血症1例

1　病　例　　患者 ,男 ,63岁 ,农民 ,主诉 1月前因劳累后 ,出现两下肢浮肿 ,无发热、皮疹、咽痛、关节痛及腰痛 ,在当地医院作“青霉素”及对症治疗无效 ,且浮肿加重。 1周来 ,发现尿色混浊 ,呈泡沫状 ,无排尿痛 ,经尿常规检查尿蛋白 ++++,拟诊肾病 ,收住入院。查体Ｔ 36.9℃ ,Ｐ 84次 /ｍｉｎ ,Ｒ 2 0次 /ｍｉｎ ,ＢＰ1 77/74ｍｍＨｇ。神清 ,呼吸平稳 ,颜面无明显浮肿 ,巩膜皮肤未见黄染 ,未见出血点及瘀点 ,浅表淋巴结未及 ,咽不充血 ,扁桃体不肿大。心律整 ,未闻及病理性杂音。两肺音清。腹平软 ,肝脾肋下未及。肾区三联症 (- ) ,两下…     

对强的松敏感的C1q肾病1例报告并文献复习

Ｃ１ｑ肾病是临床上比较少见的一种原发性肾小球疾病，大多数对口服激素治疗不敏感。笔者近遇１例Ｃ１ｑ肾病，且对强的松高度敏感，报道如下。 病 例 患者，男性，３３岁，农民，住院号１６１８７８，因“间断颜面、双下肢水肿５年，加重半月”于２００１年１月４日第二次入院。患者于５年前因“颜面、下肢水肿”入住本院，入院时化验尿蛋白＋＋＋，２４ ｈ尿蛋白定量３．２４ ｇ，诊为“原发性肾病综合征”。同时Ｂ超、ＣＴ提示“双肾多发错构瘤”。予强的松６０ｍｇ／ｄ口服，一周后尿蛋白即转阴，水肿消退，好转出院。出院后病情稳定，…     

儿童IgA肾病病理与临床分型及血尿酸的关系

目的:探讨原发性IgA肾病(IgA nephropathy,IgAN)患儿肾脏病理分级与临床分型及血尿酸水平的关系。方法:对2006年1月～2011年12月经肾活检确诊的84例原发性IgAN患儿的临床和病理资料进行回顾性分析。结果:临床分型为无症状性血尿和(或)蛋白尿者,病理改变最轻;单纯性肾病综合征、急性肾炎综合征和慢性肾炎综合征者,病理改变逐渐加重;肾炎性肾病综合征者,病理改变最重。高尿酸组肾脏病理改变以Ⅲ级为主,正常尿酸组以Ⅱ级为主,高尿酸组Ⅳ级、Ⅴ级病变较正常尿酸组多见,正常尿酸组Ⅰ级病变较高尿酸组多见。结论:病理分级与临床分型和血尿酸水平相关联,通过IgAN患儿的临床分型和血尿酸水平有助于了解其肾脏病理损伤的程度。     

C1q肾病17例临床病理分析

目的探讨成人C1q肾病的临床表现、病理改变和治疗效果,以加强对成人C1q肾病的认识。方法选择我院明确诊断为C1q肾病的患者17例,从临床表现、病理改变、治疗效果3个方面进行分析。结果17例C1q肾病者,1例血肌酐超出正常水平。肾脏病理检查显示均为系膜增生性。免疫荧光检查以C1q沉积为主。电子显微镜下,除1例未见肾小球外,其余16例均可见电子致密物分布在系膜区和（或）内皮下。激素治疗效果不好。结论成人C1q肾病临床表现多样化,免疫荧光检查表现为不同的相伴沉积形式。     

狼疮肾炎患者肾小球外补体C1q沉积的临床病理意义

目的 了解狼疮肾炎（lupus nephritis,LN）患者肾小球外补体C1q的沉积情况,探讨其临床病理意义,为LN肾小管间质损伤机制提供免疫学证据。 方法 该研究为横断面研究。收集2019年3月1日至2023年6月30日河南中医药大学第一附属医院经肾活检证实的LN患者的临床资料。石蜡切片直接免疫荧光法检测肾小球外即肾小管基底膜、肾小管周毛细血管和肾间质小动脉C1q的沉积,连续切片分析C1q表达与相应组织病变的关系。Pearson相关分析法或Spearman秩相关分析法分析C1q表达水平与肾组织病理评分（活动性肾小管间质指数、活动性肾小球指数、活动性指数、慢性肾小管间质指数、慢性肾小球指数、慢性指数）、肾损伤临床指标（24 h尿蛋白量、尿α1微球蛋白、尿N-乙酰β- D-葡萄糖苷酶、血肌酐）的相关性。 结果 该研究纳入LN患者71例,年龄（20.83±11.77）岁（7～48岁）,其中男性14例（19.72%）,女性57例（80.28%）。LN病例分型Ⅱ型6例,Ⅲ型21例,Ⅳ型18例,Ⅴ型8例,Ⅲ+Ⅴ型12例,Ⅳ+Ⅴ型6例。石蜡切片分析结果显示,肾小管基底膜和/或肾小管周毛细血管和/或肾间质小动脉C1q阳性60例（84.51%）,以肾小管基底膜阳性率最高（50例,70.42%）。C1q在肾小管基底膜呈散在或局灶沉积44例（61.97%）。C1q在肾小管周毛细血管呈散在或局灶沉积32例（45.07%）。肾小管基底膜C1q阳性比例以LN Ⅳ型（15/18）最高。肾小管周毛细血管C1q阳性比例以LN Ⅱ型（5/6）最高。肾组织连续切片结果显示,C1q主要表达在肾小管间质炎症病变区域,肾小管萎缩区域无C1q阳性表达。相关性分析结果显示,肾小管基底膜C1q阳性百分比与活动性肾小管间质指数（ r=0.640, P<0.001）、尿α1微球蛋白（ r=0.573, P<0.001）、尿N-乙酰-β- D-葡萄糖苷酶（ r=0.404, P=0.008）及血肌酐（ r=0.399, P=0.001）均呈正相关,与其他肾组织病理评分和24 h 尿蛋白量均无相关性。C1q肾小管周毛细血管与24 h 尿蛋白量、尿α1微球蛋白、尿N-乙酰β- D-葡萄糖苷酶、血肌酐和肾组织病理评分均无相关性。 结论 C1q在肾小管基底膜沉积可能是肾小管间质损伤的原因之一,建议在常规肾脏病理诊断中予以标注。     

Current status and issues of C1q nephropathy

C1q nephropathy, first proposed by Jennette and Hipp [Am J Clin Pathol 83:415-420, 1985; Am J Kidney Dis 6:103-110, 1985], was described as a distinct glomerular disease entity characterized by extensive mesangial deposition of C1q, with associated mesangial immune complexes, and the absence of any clinical and laboratory evidence of systemic lupus erythematosus. Now, 20 years since the first report, the disease entity is gradually attaining recognition, particularly in the field of pediatrics. C1q is the subcomponent of C1 in the classical pathway of complement activation. Generally, C1q deposition is caused by the activation of C1 by immunoglobulin G (IgG) and IgM; therefore, C1q nephropathy is considered as an immune complex glomerulonephritis. However, in C1q nephropathy, it remains unclear whether the deposition of C1q in the glomeruli is in response to the deposition of immunoglobulin or immune complex, or whether deposition is non-specific trapping that accompanies increased glomerular protein trafficking associated with proteinuria. Since not only the pathogenesis of C1q deposition in glomeruli but also its significance are still uncertain, it has not yet been established as an independent disease. From recent publications of the clinical and pathological characterizations, C1q nephropathy has been thought to be a subgroup of primary focal segmental glomerular sclerosis. However, many reports describe different symptoms, histopathologies, therapeutic responses and prognoses, suggesting that C1q nephropathy is not a single disease entity, but that it may be a combination of several disease groups. There are many uncertain areas requiring further investigation, though it is hoped that a detailed examination of future cases will clarify the subgroups making up C1q nephropathy and their clinicopathological characteristics, and will lead to the establishment of C1q nephropathy as an independent disease entity.     

14例儿童IgM肾病临床与病理探讨

目的：探讨儿童IgM肾病临床与病理之间的关系。方法：对首都儿科研究所附属儿童医院2004年10月～2009年3月经肾组织活检明确诊断为IgM肾病14例患儿的临床资料、肾组织病理、治疗方法和随访情况进行回顾性分析。结果：本资料50%IgM肾病临床表现为肾病综合征;肾脏病理显示以系膜增生病变为主,免疫荧光示8例单纯IgM沉积,5例IgM＋IgG沉积;结合临床和病理给予个体化治疗,有效率可达81.8%。结论：IgM肾病临床和病理存在着一定的关联性,临床表现为肾病综合征者病理损害较重。早期、规律、个体化治疗,近期疗效好。大量蛋白尿是预后不良的因素。     

A case of congenital nephrotic syndrome associated with positive C1q immunofluorescence

We present a 1-month-old girl with a congenital nephrotic syndrome and unusual histological findings. Immunofluorescence microscopy demonstrated granular mesangial deposition of C1q and electron microscopy revealed electron-dense mesangial deposits. Her heavy proteinuria gradually decreased and the steroid therapy did not have a significant effect. Her renal function was normal throughout the entire period of observation. The clinical evidence and histopathological features of this patient were compatible with C1q nephropathy.     

儿童IgA肾病的临床与病理分析

目的 探讨IgA肾病患儿临床表现和病理改变的关系及疾病转归.方法 分析30例IgA肾病患儿的临床和病理改变,病理分型采用改良Lee分型法,其中28例进行随访,4例重复肾活检.结果 临床表现孤立性血尿型10例,病理为Ⅰ、Ⅱ级;血尿和蛋白尿型11例,病理为Ⅰ～Ⅲ级;急性肾炎型2例,病理Ⅲ级;肾病综合征型7例,病理为Ⅱ～Ⅳ级,以Ⅱ、Ⅲ级为主.4例行重复肾活检,病理变化为改善、加重和无明显变化,但免疫复合物沉积均有加重.28例随访1年2个月～9年,其中9例预后不乐观;死亡1例;预后不良患儿7例治疗依从性差.结论 IgA肾病临床表现与病理有一定相关性,急性肾炎型、肾病综合征型患儿损伤重、预后欠佳,同时提示临床医师重视IgA肾病患儿随访和健康宣教,提高治疗依从性,以利疾病康复.     

Steroid-sensitive nephrotic syndrome associated with positive C1q immunofluorescence

A 21-year-old woman showed heavy proteinuria and edema. A light microscopic study of a renal biopsy specimen showed diffuse mild mesangial expansion, with borderline mesangial hypercellularity. An immunofluorescence study revealed dominant positive staining (3+) of C1q in the glomerular mesangium. Stainings for C3, C4, IgG, and IgM were weak or 1+. Staining for IgA was negative. Electron-dense deposits were present in the mesangial area. There was significant fusion of foot processes. There was no serological or clinical evidence of collagen disease. She was treated with oral prednisolone (initially, 40mg/day). The proteinuria was alleviated and the patient remains in complete remission. The histopathological studies were compatible with C1q nephropathy, although the clinical outcome differed in a number of aspects. The clinical picture in the current patient appears to represent a very rare phenotype of nephritis.     

C1Q nephropathy in children

C1q nephropathy (C1qNP) is a peculiar form of glomerulonephritis characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. We describe the incidence, manifestation, histopathologic findings, follow-up, treatment and outcome of C1qNP. Twelve C1qNP patients were identified among 131 children who had undergone renal biopsy, accounting for a 9.16% incidence of C1qNP. Light microscopy examination showed focal segmental glomerulosclerosis (FSGS) with or without diffuse mesangial proliferation (n=6), minimal change disease (MCD) (n=4) or focal glomerulonephritis (n=2). C1q deposits were found in all, while electron microscopy revealed visible deposits in nine cases. Eight children presented with nephrotic syndrome, while one had nephrotic proteinuria and renal insufficiency that progressed to end-stage renal failure. The remaining three patients presented with nonnephrotic proteinuria associated with microhematuria, hypertension or renal insufficiency. Only one nephrotic syndrome patient responded excellently to corticosteroids, while four became corticosteroid dependent, and three were corticosteroid resistant, showing a very poor response to other immunosuppressive therapy as well. Patients with non-nephrotic proteinuria demonstrated fixed laboratory findings. Most C1qNP patients had FSGS or MCD, the majority of them presenting with corticosteroid-dependent or corticosteroid-resistant nephrotic syndrome. The latter showed a very poor response to any immunosuppressive therapy and high risk for progressive renal insufficiency.     

C1q nephropathy: features at presentation and outcome

The study population comprised all 20 patients followed since 1990 through December 2004 at the Le Bonheur Childrens Medical Center with diagnosis of C1q nephropathy (55% boys; 60% African Americans). All were aged under 18 years at biopsy (mean 11.2 years, 65% aged 11 or over); the youngest presented at age 10 months and progressed to end-stage renal disease at 14 months. None had clinical or laboratory features of systemic lupus erythematosis or membranoproliferative glomerulonephritis. Clinical features assessed at diagnosis were age, gender, blood pressure, history of macroscopic hematuria, urinary protein to creatinine ratio, serum creatinine, estimated glomerular filtration rate, renal histology, and pattern for immunofluorescent reactants. At the time of biopsy 40% had nephrotic syndrome and 30% nephrotic range proteinuria without nephrotic syndrome. Three patients with nephrotic syndrome also had chronic renal insufficiency at diagnosis. The most common histological feature was focal segmental glomerulosclerosis in 40%, but 30% had minimal change lesion. Four patients, all with nephrotic syndrome at diagnosis, progressed to end-stage renal disease. Of the 12 patients not presenting with nephrotic syndrome, none had chronic renal insufficiency at last follow-up. Kidney survival was 94% and 78% at 1 and 5 years, respectively, in all patients and 88% and 49% in those presenting with nephrotic syndrome.     

C1q nephropathy in an old woman with acute renal failure: a case report and literature review

Reports on the clinical entity of C1q nephropathy have focused on older children and young adult, data on old people are rare. In this report, we would introduce a 77-year-old woman who was diagnosed as C1q nephropathy by means of electron microscopic and immunofluorescence examination. Facial and lower extremity edema was the main reason for her to go for medical treatment, and she developed into acute renal failure within 5?d. Complete remission was observed after hemodialysis and steroid drugs treatments.     

C1q nephropathy with asymptomatic urine abnormalities

We found four cases of C1q nephropathy (C1qN) among a total of 193 pediatric series of first renal biopsies. Among them, 94 biopsies were performed because of asymptomatic urine abnormalities detected by school urinary screening program in Japan; three cases out of these 94 biopsies (3.2%) met the criteria of C1qN. One case out of the remaining 99 biopsies with symptomatic renal diseases (1%) also met the criteria of C1qN. Three cases with asymptomatic onset presenting with mild proteinuria with or without hematuria equally showed histologic features of membranoproliferative glomerulonephritis and showed improvements in urinalysis without corticosteroid treatment. Our data suggest that membranoproliferative glomerulonephritis may be a common histological feature of asymptomatic pediatric C1qN in Japan and that this type of glomerulopathy may follow a relatively good clinical course without steroid therapy.     

C1q的沉积对V型狼疮性肾炎和原发性膜性肾病的鉴别诊断意义

目的:研究C1q在V型狼疮性肾炎、原发性膜性肾病及病理组织学为不典型膜性肾病肾活检标本中的沉积,分析其不同及意义。方法:对V型狼疮性肾炎、原发性膜性肾病和不典型膜性肾病的患者的肾活检组织进行C1q免疫组化染色,并收集临床和血清学指标,进行统计学分析。结果:V型狼疮性肾炎会出现C1q的沉积,原发性膜性肾病不会出现C1q的沉积,一些病理组织学表现不典型的膜性肾病,会出现C1q的沉积,后者阳性率与狼疮性肾炎接近,与膜性肾病相比,差异有统计学意义。结论:C1q阳性且病理组织学为不典型膜性肾病的患者,极有可能是早期的V型LN。