急性间歇性卟啉病并发肝细胞癌的发病机制与预防策略

Pathogenesis and prevention strategy of hepatocellular carcinoma in acute intermittent porphyria

随着遗传学、分子生物学的发展,急性间歇性卟啉病(AIP)的相关研究不断深入,其并发肝细胞癌(HCC)也日益引起关注。主要总结了AIP并发HCC的发病机制及预防研究进展。AIP并发HCC的机制包括氧化应激、p53突变、B淋巴细胞瘤-2下调、致炎细胞因子增多、铁超载等有关。其预防策略包括他汀类药物的应用、严格管理、肝移植和基因及酶替代治疗。对AIP并发HCC的发病机制及预防新进展作一综述。

With the development of genetics and molecular biology,the research on acute intermittent porphyria(AIP)has been gradually deepened,and its complication hepatocellular carcinoma(HCC)has attracted more and more attention.This article mainly summarizes the research advances in the pathogenesis and prevention of HCC in AIP.The pathogenesis of AIP with HCC is associated with oxidative stress,p53 mutation,downregulation of Bcl-2,increase in inflammatory cytokines,and iron overload,and its prevention strategies include the use of statins,strict management,liver transplantation,gene therapy,and enzyme replacement therapy.This article reviews the latest advances in the pathogenesis and prevention of AIP with HCC.