ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2021, Vol. 30 ›› Issue (5): 470-475.DOI: 10.3969/j.jssn.1006-298X2021.5.015

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载脂蛋白E相关肾小球疾病的诊治进展

  

  • 出版日期:2021-10-28 发布日期:2021-10-28

Diagnosis and treatment of Apolipoprotein E related glomerular diseases

  • Online:2021-10-28 Published:2021-10-28

摘要: 载脂蛋白E(apoE)在脂蛋白代谢中起关键作用,其突变可引起apoE结构功能障碍,介导apoE相关肾小球疾病,如apoE2纯合子肾小球病和脂蛋白肾病(LPG)等。apoE2纯合子肾小球病组织学特征为肾小球硬化,可见来自巨噬细胞的泡沫细胞浸润,偶有非层状脂蛋白栓子形成。巨噬细胞不仅参与泡沫细胞的形成,其功能障碍也可导致脂蛋白在肾小球积聚,引起脂蛋白栓子。携带apoE Toyonaka(Ser197Cys)突变纯合子apoE 2/2基因型的患者,肾小球可以出现非免疫性膜性肾病样特征。LPG可见不伴泡沫细胞的片状脂蛋白栓子。不同apoE基因突变所致肾脏疾病的病理特征及临床表现,甚至治疗方式不尽相同。因此了解apoE基因多态性及apoE相关肾病的致病机制,为有效治疗和个体化药物治疗提供依据,可有效延缓慢apoE相关肾小球疾病的发展。


Abstract: Apolipoprotein E (apoE) plays a key role in lipoprotein metabolism,its mutation can cause dysfunction in apoE structure,causes apoErelated glomerular disorders,such as apoE2 homozygote glomerulopathy and lipoprotein glomerulopathy (LPG).ApoE2 homozygote glomerulopathy was characterized histologically by glomerulosclerosis with marked infiltration of foam cells .Macrophages are not only involved in formation of foam cells,but also their dysfunction can lead to accumulation of lipoproteins in glomeruli,causing lipoprotein thrombi derived from macrophagesIn patients with apoE Toyonaka (Ser197Cys) combined with homozygous apoE2/2,nonimmune membranous nephropathylike features were observed in glomeruli.LPG is histologically characterized by lamellated lipoprotein thrombi that lack foam cells.Therefore,the understanding of apoE gene polymorphism and pathogenesis of apoErelated glomerular disorders can provide means for effective treatment and individualized drug treatment,and prevent development of chronic kidney disease.