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…     

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。     

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。     

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     

C1q肾病的儿科临床病理研究

Clq肾病是一种免疫复合物(IC)介导的肾小球病变,患者的尿蛋白量常在肾病综合征(NS)范围。Jennette和Hipp首先报道15例年龄14～27岁的本病患者时使用这一名称。ClqNP在免疫荧光(IF)检查中都可见到非全身性系膜病变的肾小球肾炎。ClqNP与其它原因引起的NS不同,其特点为,IF可见系膜区形成明显的Clq沿着免疫球蛋白沉积,而无膜增殖性肾小球炎(MPGN)的特征性肾小球毛细血管壁损伤和SLE的临床表现。 15例ClqNP患儿年龄2～16岁,男女之比1∶2。其中3例持续肾小球炎患儿表现为中度蛋白尿、血尿和细胞管型;1例肾炎性肾病;9例特发性NS;     

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，…     

表现为单纯镜下血尿的C1q肾病报道1例

<正>C_1q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C_1q沉积,临床表现多为持续性蛋白尿或肾病范围内蛋白尿,伴有或不伴有血尿和肾功能损伤。本病例为以单纯血尿为表现的一例C_1q肾病,特报道如下。临床资料患者男,30岁,因体检发现尿潜血2年余于2016年6月10日     

儿童C1q肾病的临床病理特点及治疗

目的 探讨儿童C1q肾病的临床、病理特点及治疗方法。 方法 回顾性分析本院8年来经肾活检确诊的23例C1q肾病患儿临床、病理和预后资料。 结果 C1q肾病占同期肾活检的原发性肾小球疾病的4.78%。23例患儿中,男15例,女8例;年龄10个月~12岁5个月,平均发病年龄（5.0±3.4）岁;肾病综合征（NS） 18例（2例伴镜下血尿）,肾病水平蛋白尿4例（1例伴镜下血尿）,单纯镜下血尿1例。1例NS起病前曾服用2周中药,发病时同时并发急性肾功能不全。3例患儿有肾脏病家族史,其中2例（肾病水平蛋白尿）为姐弟,父亲亦有蛋白尿,基因检测证实为家族性Denys-Drash综合征并发C1q肾病。1例患儿（NS）姐姐亦有大量蛋白尿（未行肾活检）。所有患儿起病时血压均正常,补体正常,抗核抗体、抗dsDNA抗体、抗Sm抗体及乙肝两对半均阴性。18例NS中13例激素耐药（72.2%）,4例激素依赖,1例激素敏感。光镜下,13例为微小病变（MCD）（其中1例伴间质性肾炎）;6例为系膜增生性肾小球肾炎（MsPGN）;4例为局灶节段性肾小球硬化（FSGS）。另9例患儿伴有不同程度的小管萎缩和间质纤维化。免疫荧光下,所有患儿均见系膜区弥漫性C1q≥2+沉积,其中伴IgG沉积18例,IgM沉积18例,IgA沉积8例,C3沉积11例,6例患儿呈“满堂亮”表现。除4例患儿电镜下未见肾小球外,其余19例中4例系膜区见电子致密物沉积。12例激素耐药（包括2例肾病水平蛋白尿者）及3例激素依赖患儿在激素治疗基础上加用静脉CTX冲击;3例激素耐药者加用环孢素A（CsA）口服;1例激素依赖患儿给予足量激素重新诱导;1例单纯镜下血尿患儿及2例Denys-Drash综合征并发C1q肾病患儿仅给予血管紧张素转换酶抑制剂（ACEI）治疗。其中1例患儿CTX冲击满疗程无效后换用CsA治疗;1例患儿CTX冲击满疗程无效后换用FK506治疗。23例患儿中,1例失访,1例治疗时间<3个月未纳入随访对象,2例Denys-Drash综合征目前不能通过药物治疗好转未纳入疗效统计,余19例中,15例完全缓解（78.9%）,2例部分缓解（10.5%）,2例无效（10.5%）。NS患儿总缓解比例94.4%（17/18）,肾病水平蛋白尿患儿总缓解比例50.0%（2/4）。病理为MCD者总缓解比例100.0%,MsPGN者缓解比例83.4%,FSGS缓解比例50.0%。随访末所有患儿血压、肾功能均正常,自身抗体均阴性,补体水平均正常。 结论 C1q肾病罕见,临床以NS或肾病水平蛋白尿为主,且往往激素耐药或激素依赖;病理以MCD为主,也可表现为MsPGN或FSGS。加用其他免疫抑制剂治疗后,MCD和MsPGN者多可获缓解,但FSGS预后欠佳。     

Membranous nephropathy with predominance of C1q: another variant of C1q nephropathy?

Originally described as a proliferative glomerulonephritis, C1q nephropathy is nowadays mostly recognized as a variant of focal segmental glomerulosclerosis or minimal change disease. We describe a 30-year-old male patient with nephrotic range proteinuria. Kidney biopsy demonstrated a membranous nephropathy with predominant staining for C1q. Under conservative therapy the outcome was favorable. We suggest that this case represents another variant of C1q nephropathy, thus broadening the spectrum of the disease.     

C1q nephropathy: a case with severe atopic dermatitis

A 9-year-old boy with nephrotic syndrome who had a history of atopic dermatitis since 5 years of age is presented in this report. Generalized edema and hyperpigmented and desquamated pruritic lesions were detected on the trunk and extremities. Mesangial C1q deposition was seen on kidney biopsy. A high serum IgE level was also detected. Complete remission was achieved with steroid therapy.     

C1q nephropathy in association with Gitelman syndrome: a case report

There have been rare reports of glomerulopathies developing in patients with Bartter syndrome (BS) and its milder variant, Gitelman syndrome (GS). We present the first case of C1q nephropathy (C1qN) in an African American child with GS. This child was diagnosed with GS at 9 years of age and subsequently developed nephrotic range proteinuria 3 years later. Renal biopsy revealed mesangial hypercellularity and focal segmental glomerulosclerosis (FSGS). The segmental lesions were generally located at the vascular pole. Dominant C1q (2+) staining along with IgG (1–2+) was demonstrated in the mesangium, which correlated with scattered electron dense mesangial deposits demonstrated by electron microscopy. Treatment with an angiotensin-converting enzyme inhibitor led to an improvement in proteinuria to near-normal values (urine protein/creatinine ratio down to 0.5), but the creatinine clearance declined to approximately 58 ml/min/1.73 m². This case highlights the possible association between the milder hypokalemic tubulopathy, GS, and glomerular disease, including C1qN. Prompt evaluation of proteinuria with renal biopsy in these patients is recommended to detect significant glomerular pathology. Further research is needed to define risk factors for this complication.     

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 nephropathy in two young sisters

C1q nephropathy (C1qNP) is a controversial and uncommon form of glomerulonephritis, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. Clinically, it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microhematuria, hypertension, or renal insufficiency. We describe two sisters with C1qNP, who presented with steroid-resistant nephrotic syndrome. Both sisters presented before the age of 2 years, and they showed a poor response to other immunosuppressive therapy. Both girls had normal serum complement levels, negative antinuclear antibodies (ANAs) and negative hepatitis B antigen. Renal biopsy in both patients showed histological features of mesangioproliferative glomerulonephritis, with diffuse “full-house” positive immunofluorescence reaction in the mesangial area. The immunofluorescence reaction for C1q was most intense and co-dominant with IgG in both patients. Correspondingly, electron microscopy demonstrated dense deposits mainly in the mesangial areas too. We report on two young sisters with the characteristic features of C1qNP presented in early childhood. To the best of our knowledge, this is the first report of C1qNP in siblings.     

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.