内科理论与实践2024，Vol.19Issue(2)：140-143,4.DOI:10.16138/j.1673-6087.2024.02.10
磷脂酰肌醇3激酶δ过度活化综合征治疗进展
Progress in treatment of activated phosphoinositide 3-kinase δ syndrome
摘要
Abstract
Phosphoinositide 3-kinase δ(PI3Kδ)activated phosphoinositide 3-kinase δ syndrome(APDS)was first reported in 2013.APDS is an autosomal dominant primary immunodeficiency disease(PID)caused by mutations in the PIK3CD gene or PIK3R1 gene.The clinical manifestations of APDS are recurrent respiratory tract infection,non-neoplastic lymphoid hyperplasia,autoimmune diseases and lymphoma.In this paper,the treatment of APDS is introduced in detail.In addition to conventional treatment for immunodeficiency,treatment such as antimicrobial prophylaxis,immunoglobulin replacement therapy and hematopoietic stem cell transplantation were reviewed,and more specific mammalian target of rapamycin(mTOR)inhibitors and PI3Kδ inhibitors were also discussed.关键词
PI3Kδ过度活化综合征/造血干细胞移植/mTOR抑制剂/PI3Kδ抑制剂Key words
PI3Kδ overactivation syndrome/Hematopoietic stem cell transplantation/mTOR inhibitor/PI3Kδ inhibitor分类
医药卫生引用本文复制引用
吴涛,刘文慧..磷脂酰肌醇3激酶δ过度活化综合征治疗进展[J].内科理论与实践,2024,19(2):140-143,4.基金项目
甘肃省科技计划项目(21JR7RA015、22YF7FA106) （21JR7RA015、22YF7FA106）
