静脉注射丙种球蛋白无反应型川崎病遗传学进展

摘要

Kawasaki disease(KD)has been the leading cause of acquired heart disease in children.Treatment with high dose intravenous immunoglobulin(IVIG) is effective,but about 10％ to 20％ KD patients have no response to initial IVIG treatment.These patients always show persistent or recurrent fever after completion of IVIG,and have a particularly high risk of developing coronary artery lesions.Recent studies have showed that there was some relationship between genetic polymorphisms and IVIG resistance.The genetic polymorphisms of IgG-Fc region receptors and inositol 1,4,5-trisphosphate 3-kinase C have been well studied and showed significant relationship with IVIG resistance in KD.Here,we provide an overview of recent genetic studies of IVIG resistant KD.%川崎病(Kawasaki disease,KD)是儿童获得性心脏病的首要病因.目前大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,ⅥG)是川崎病的经典疗法.然而,约10％～20％的KD患者IVIG治疗无反应,且其冠状动脉损害的发生率也随之增加.业已证明,IVIG治疗无反应与基因有一定相关性.免疫球蛋白Fc受体相关基因、1,4,5-三磷酸肌醇3激酶C基因是其中研究深入且与IVIG无反应有明确相关性的基因.该文就IVIG无反应型KD的遗传学进展作一综述.