摘要
黏多糖病是一种累及多个系统的罕见遗传代谢性疾病,临床表现类型多样,并随着年龄的增长而恶化,心脏受损是该病常见的致死原因,以黏多糖病Ⅰ、Ⅱ和Ⅵ型受累最为严重。影像学检查是该类患者心脏损害评估的重要手段,随着有效治疗方法的应用,早期、准确评估黏多糖病患者的心血管并发症可改善患者的生活质量与预后。现对黏多糖病的心脏受累特点及影像评估方法研究现状及进展做一综述。
Mucopolysaccharidosis( MPS) is a rare genetic metabolic disease involving several systems,with diverse clinical manifestations and worsening with age. Cardiac injury is the common cause of death,MPS Ⅰ,Ⅱ and Ⅵ are those with the most severe cardiac involvement. Imaging examination is an important means of assessing cardiac damage. With the application of effective treatment,early and accurate assessment of cardiovascular complications in MPS patients can significantly improve the quality of life and the prognosis of patients. In this paper,the characteristics of cardiac involvement in MPS and the research status and progress of imaging evaluation methods are reviewed.
作者
邹庆
郭应坤
张丽芝
ZOU Qing;GUO Yingkun;ZHANG Lizhi(Department of Radiology,West China Second University Hospital,Sichuan University,Chengdu 610041,Sichuan,China;Department of Radiology,West China Hospital,Sichuan University,Chengdu 610041,Sichuan,China)
出处
《心血管病学进展》
CAS
2020年第4期361-365,共5页
Advances in Cardiovascular Diseases
基金
国家科学自然基金项目(81701767,81771897)
四川省科技厅项目(2018SZ0148)。
关键词
黏多糖病
影像
心脏损伤
Mucopolysaccharidosis
Image
Cardiac injury
