C1Q nephropathy in children
C1Q nephropathy in children
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DOI：
10.1007/s00467-005-2040-4
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发表时间：
2005-12-01
影响因子：
3
通讯作者：
Vizjak, A
中科院分区：
文献类型：
--
作者：
Levart, TK;Kenda, R;Vizjak, A
C1q肾病（C1qNP）是一种特殊形式的肾小球肾炎，其特征是系膜免疫球蛋白和补体沉积，主要是C1q，没有系统性红斑狼疮的证据。我们描述了C1qNP的发病率，表现，组织病理学检查结果，随访，治疗和结果。在131例接受肾活检的儿童中发现12例C1qNP患者，占C1qNP的9.16%。光镜检查显示局灶节段性肾小球硬化（FSGS）伴或不伴弥漫性系膜增生（n=6）、微小病变（MCD）（n=4）和局灶性肾小球肾炎（n=2）。C1q沉积物中发现的所有，而电子显微镜显示可见存款9例。八名儿童表现为肾病综合征，而一名患有肾病蛋白尿和肾功能不全，进展为终末期肾衰竭。其余3例患者表现为非肾病性蛋白尿伴微量血尿、高血压或肾功能不全。只有1例肾病综合征患者对皮质类固醇反应良好，而4例成为皮质类固醇依赖性，3例皮质类固醇耐药，对其他免疫抑制治疗反应也很差。非肾病蛋白尿患者表现出固定的实验室检查结果。大多数C1qNP患者有FSGS或MCD，其中大多数表现为皮质类固醇依赖性或皮质类固醇抵抗性肾病综合征。后者对任何免疫抑制治疗的反应都非常差，并且进行性肾功能不全的风险很高。
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.