Cardiovascular complications of sickle cell disease *

Cardiovascular complications of sickle cell disease *
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DOI:
10.1016/j.tcm.2020.02.002
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发表时间:
2021-03-03
影响因子:
9.3
通讯作者:
Lay, Swee
Lay, Swee
中科院分区:
医学2区
文献类型:
--
作者:
Sachdev, Vandana;Rosing, Douglas R.;Lay, Swee

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镰状细胞病(SCD)是美国最常见的遗传性血液疾病,也是一个全球性的健康问题。异常血红蛋白(HbS)的病理特征导致了该疾病的两个特征——急性微血管闭塞和慢性溶血性贫血的反复发作——对多个器官造成持续和潜伏的损害。随着儿童生存率的提高,成人SCD已演变为一种慢性退行性疾病,对包括心脏和肺在内的多个器官造成潜在损害。心肺并发症,包括心肌病、舒张功能障碍、肺动脉高压(PH)和心源性猝死是最常见的发病和死亡原因。意识到镰状病相关的心血管表型对于筛查、早期诊断和心脏并发症的干预是重要的。Elsevier Inc.出版。
Sickle cell disease (SCD) is the most common inherited blood disorder in the United States, and a global health problem. Pathological features of the abnormal hemoglobin (HbS) result in 2 hallmarks of the dis-ease -recurrent episodes of acute microvascular occlusion and chronic hemolytic anemia -that inflict continuous and insidious damage to multiple organs. With improved childhood survival, SCD in adults has evolved into a chronic degenerative disease with underlying damage to multiple organs including the heart and lungs. Cardiopulmonary complications, including cardiomyopathy, diastolic dysfunction, pul-monary hypertension (PH), and sudden cardiac death are the most common causes of morbidity and mortality. Awareness of the sickle-related cardiovascular phenotypes is important for screening, early di-agnosis, and intervention of cardiac complications in this disorder.Published by Elsevier Inc.