摘要
心脏淀粉样变(cardiac amyloidosis,CA)是由于前体蛋白异常折叠形成不可溶的淀粉样物质,沉积于心肌细胞外间质而导致的一类浸润型心肌病。尽管有30种前体蛋白可引起淀粉样变,但导致淀粉样变心肌病主要为以下两类前体蛋白:免疫球蛋白轻链(immunoglobulin light chain)和转甲状腺素蛋白(transthyretin)。导致的淀粉样变相应称为免疫球蛋白轻链淀粉样变(light chain amyloidosis,AL)和转甲状腺素蛋白淀粉样变(transthyretin amyloidosis,ATTR)。由于ATTR起病隐匿,易误诊和漏诊,预后差,因此本文着重就转甲状腺素蛋白淀粉样变心肌病(ATTR-CA)的早期临床表现、诊断及预后和治疗做一综述。
Cardiac amyloidosis(CA)is a kind of invasive cardiomyopathy caused by the abnormal folding of precursor proteins to form insoluble amyloid substances and be deposited in the external stroma of cardiac cells.Although there are 30 precursor proteins that cause amyloidosis,amyloidogenic cardiomyopathy mainly causes two main types of precursor proteins:immunoglobulin light chain and transthyroidine protein.The resulting amyloidosis is correspondingly called light chain amyloidosis(AL)and transthyretin amyloidosis(ATTR).Due to the insidious onset of ATTR,easy misdiagnosis and missed diagnosis,and poor prognosis,this paper focuses on the early clinical manifestations,diagnosis,prognosis and treatment of ATTR amyloidosis cardiomyopathy.
作者
罗金勇
袁卫红
董庆斐
丁双
LUO Jinyong;YUAN Weihong;DONG Qingfei;DING Shuang(Fuwai Yunnan Cardiovascular Hospital,Kunming 650102,China;不详)
出处
《中国医学创新》
CAS
2023年第20期179-184,共6页
Medical Innovation of China
基金
云南省心血管病临床医学中心项目(FZX2019-06-01)
云南省心血管系统疾病临床医学研究中心-重大心血管疾病诊治新技术研发项目(202102AA310002)。
