Blinder Morey A, Russel Sarah
Departments of Medicine and Pathology and Immunology, Washington University , St. Louis, MO.
University of Notre Dame , Notre Dame, IN, USA.
Hematol Rep. 2014 Dec 3;6(4):5502. doi: 10.4081/hr.2014.5502. eCollection 2014 Nov 19.
Sickle cell trait (SCT) occurs in about 8% of African-Americans and is often described to be of little clinical consequence. Over time, a number of risks have emerged, and among these are rare but catastrophic episodes of sudden death in athletes and other individuals associated with physical activities which is often described as exercise collapse associated with sickle trait (ECAST). Despite an epidemiologic link between SCT and sudden death as well as numerous case reports in both medical literature and lay press, no clear understanding of the key pathophysiologic events has been identified. Strategies for identification of individuals at risk and prevention of ECAST have been both elusive and controversial. Stakeholders have advocated for different approaches to this issue particularly with regard to screening for hemoglobin S. Furthermore, the recommendations and guidelines that are in place for the early recognition of ECAST and the prevention and treatment of the illness are not well defined and remain fragmented. Among the cases identified, those in collegiate football players in the United States are often highlighted. This manuscript examines these case studies and the current recommendations to identify areas of consensus and controversy regarding recommendations for prevention, recognition and treatment of ECAST.
镰状细胞性状(SCT)在约8%的非裔美国人中出现,通常被认为临床影响不大。随着时间的推移,出现了一些风险,其中包括运动员和其他从事体育活动的个体中罕见但灾难性的猝死事件,这通常被描述为与镰状细胞性状相关的运动性虚脱(ECAST)。尽管SCT与猝死之间存在流行病学关联,且医学文献和大众媒体都有大量病例报告,但尚未明确关键的病理生理事件。识别高危个体和预防ECAST的策略一直难以捉摸且存在争议。利益相关者主张针对这个问题采取不同方法,特别是在筛查血红蛋白S方面。此外,目前关于ECAST早期识别以及疾病预防和治疗的建议和指南并不明确,仍然零散。在已识别的病例中,美国大学橄榄球运动员的病例经常受到关注。本文探讨这些案例研究和当前建议,以确定关于ECAST预防、识别和治疗建议的共识和争议领域。