The Value of Echocardiography in Evaluating Left Ventricular Dysfunction in Patients with Fabry Disease

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The Value of Echocardiography in Evaluating Left Ventricular Dysfunction in Patients with Fabry Disease

更新时间：2023-10-11

- The Value of Echocardiography in Evaluating Left Ventricular Dysfunction in Patients with Fabry Disease
- Journal of Clinical Ultrasound in Medicine Vol. 24, Issue 1, (2022)
- 作者机构：
  
  首都医科大学附属北京地坛医院
- 作者简介：
- 基金信息：
- DOI：
  CLC：
- Published：2022，
  
  Published Online：9 February 2022，
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The Value of Echocardiography in Evaluating Left Ventricular Dysfunction in Patients with Fabry Disease. [J]. Journal of Clinical Ultrasound in Medicine 24(1).(2022)
DOI：

The Value of Echocardiography in Evaluating Left Ventricular Dysfunction in Patients with Fabry Disease. [J]. Journal of Clinical Ultrasound in Medicine 24(1).(2022) DOI：

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，有助于患者的及早诊断及治疗。本文就超声心动图在评估Fabry患者左心室结构和功能损伤中的应用价值进行综述。

Abstract

Fabry disease is an X chromosome recessive genetic disorder. Due to the deficiency of α -galactosidase A activity

the accumulation of α-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病左心室功能

Keywords

EchocardiographyFabry diseaseLeft ventricular function

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Related Institution

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川北医学院

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