C1q肾病临床病理特征研究进展  

Clinicopathological features and research progress of C1q nephropathy

作　　者：何根娅(综述) 马宏 赵青霞(审校) He Genya;Ma Hong;Zhao Qingxia(Department of Pediatrics,the First School of Clinical Medicine,Shanxi Medical University,Taiyuan 030001,China)

机构地区：[1]山西医科大学第一临床医学院小儿内科,太原030001

出　　处：《国际儿科学杂志》2020年第3期193-196,共4页International Journal of Pediatrics

摘　　要：C1q肾病是一种特殊类型的肾小球肾炎,以肾小球系膜补体C1q大量沉积为特征,病理学上常表现为微小病变型肾小球肾炎、局灶节段性肾小球硬化及增生性肾小球肾炎,易发生激素依赖或抵抗,预后相对较差,目前发病机制尚不明确。临床表现差异性较大,糖皮质激素仍是治疗首选药物,免疫抑制剂常用于激素依赖或抵抗的患者,近年来还发现利妥昔单抗对C1q肾病具有较好的疗效。虽然人们对C1q肾病的认识和研究不断深化,但能否将C1q肾病划分为一类独立的疾病及其归类的临床意义还存在较大的争议。该文就C1q肾病的发病机制、病理组织学、临床特点、治疗及预后进行综述,以期对当前C1q肾病的相关研究有更好的理解。C1q nephropathy is a special type of glomerulonephritis,which characterized by massive deposition of C1q on glomerular mesangial.The pathological manifestations of C1q nephropathy are minimal change disease,focal segmental glomerulosclerosis and proliferative glomerulonephritis.C1q nephropathy is prone to hormone dependence or resistance,and the prognosis is not good relatively.The pathogenesis of the disease is not clear.The clinical manifestations are quite different.Glucocorticoid is still the first choice for treatment.Immunosuppressive agents are often used in patients with hormone dependence or resistance.It has been found that the rituximab has good curative effect on C1q nephropathy in recent years.Although the understanding and research on C1q nephropathy is deepening,there is still much controversy about whether to classify C1q nephropathy into a class of independent diseases and the clinical significance of their classification.In this paper,the pathogenesis,pathological histology,clinical characteristics,treatment and prognosis of C1q nephropathy are reviewed in order to have a better understanding of C1q nephropathy.

关 键 词：C1Q肾病 发病机制 病理组织学 临床特征 治疗 

分 类 号：R69[医药卫生—泌尿科学]