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镰状细胞病的心血管并发症:普通临床医生指南。

Cardiovascular complications of sickle cell disease: A primer for the general clinician.

机构信息

Division of Hematology and Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, United States.

Division of Cardiovascular Diseases, Department of Internal Medicine, University of Tennessee Health Science Center, Memphis, TN, United States.

出版信息

J Natl Med Assoc. 2024 Oct;116(5):517-525. doi: 10.1016/j.jnma.2023.11.010. Epub 2023 Dec 14.

Abstract

Sickle cell disease (SCD) is the most common hereditary hemoglobinopathy and mainly affects individuals of African ancestry. As survival has improved especially in high-income countries, increased rates of cardiopulmonary complications such as pulmonary hypertension, heart failure with diastolic dysfunction, and sudden death are encountered in clinical practice. These complications are the leading causes of morbidity and mortality as these individuals survive into adulthood. Understanding the need for, early identification, timely intervention, and implementation of preventive strategies are critical in reversing this trend and improving quality of life and survival rates. This manuscript aims to provide a comprehensive review of the pathogenesis of cardiovascular complications associated with sickle cell disease and equip the clinician with tools to facilitate the early diagnosis and management of patients with SCD as increasing numbers survive into adulthood.

摘要

镰状细胞病(SCD)是最常见的遗传性血红蛋白病,主要影响非洲血统的个体。随着生存状况的改善,特别是在高收入国家,临床实践中越来越多地遇到肺高血压、舒张功能障碍性心力衰竭和猝死等心肺并发症。这些并发症是发病率和死亡率的主要原因,因为这些个体已经进入成年期。了解这方面的需求、早期识别、及时干预和实施预防策略对于扭转这一趋势、提高生活质量和生存率至关重要。本文旨在全面综述与镰状细胞病相关的心血管并发症的发病机制,并为临床医生提供工具,以促进 SCD 患者的早期诊断和管理,因为越来越多的患者已经进入成年期。

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