超声心动图评估Fabry病患者左室功能障碍的应用进展  

Application progress of echocardiography in evaluating left ventricular dysfunction in patients with Fabry disease

作　　者：马晨瑶 张瑶 MA Chenyao;ZHANG Yao(Department of Ultrasound,Beijing Ditan Hospital,Capital Medical University,Beijing 100853,China)

机构地区：[1]首都医科大学附属北京地坛医院超声诊断科,北京市100853

出　　处：《临床超声医学杂志》2022年第1期53-56,共4页Journal of Clinical Ultrasound in Medicine

摘　　要：Fabry病是一种X染色体隐性遗传病,由于α-半乳糖苷酶A活性缺失导致α-D半乳糖残基鞘糖脂在体内蓄积,最终累及心、脑、肾等引发多系统疾病。超声心动图是一种有效评价Fabry病对心脏结构和功能影响的无创方法,有助于对患者的及早诊断并治疗。本文就超声心动图在评估Fabry患者左室结构和功能损伤中的应用进展进行综述。Fabry disease is an X chromosome recessive genetic disorder.Due to the deficiency ofα-galactosidase A activity,the accumulation ofα-D galactosyl residue glycosphingolipids in the body eventually affects the heart,brain and kidney,resulting in multi-system diseases.Echocardiography is an effective noninvasive method to evaluate the impact of Fabry disease on cardiac structure and function,which is helpful for early diagnosis and treatment of patients.This article reviews the application value of echocardiography in assessing left ventricular structural and functional damage in Fabry patients.

关 键 词：超声心动描记术 FABRY病 心室功能 左 

分 类 号：R540.45[医药卫生—心血管疾病]