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儿童镰状细胞肺病的最新进展。

Updates in Pediatric Sickle Cell Lung Disease.

机构信息

Division of Pulmonary Medicine, The Ohio State University Wexner Medical Center, Nationwide Children's Hospital, 2050 Kenny Road, Suite 2200, Columbus, OH, USA.

Division of Pulmonary and Sleep Medicine, Children' Hospital of Philadelphia, 3500 Civic Center Boulevard, Philadelphia, PA, USA.

出版信息

Clin Chest Med. 2024 Sep;45(3):749-760. doi: 10.1016/j.ccm.2024.02.022.

Abstract

Sickle cell lung disease presents a challenging care paradigm involving acute and chronic lower airway disease, sleep-disordered breathing, pulmonary vascular disease, and modification by environmental factors. Understanding the presentation, pathophysiology, and diagnostic approaches is essential for accurate identification and management. While significant progress has been made, there remains a need for research to develop effective treatments and interventions to decrease disease burden in these children. Additionally, the long-term impact of interventions on cardiopulmonary outcomes is unknown. Collaborative efforts among health care providers, researchers, advocacy groups, and policy makers are crucial to improving the lives of children with SCD.

摘要

镰状细胞肺病呈现出具有挑战性的医疗模式,涉及急性和慢性下呼吸道疾病、睡眠呼吸紊乱、肺血管疾病,以及受环境因素的影响。了解其表现、病理生理学和诊断方法对于准确识别和管理至关重要。尽管已经取得了重大进展,但仍需要研究来开发有效的治疗和干预措施,以减轻这些儿童的疾病负担。此外,干预措施对心肺结局的长期影响尚不清楚。医疗保健提供者、研究人员、宣传团体和政策制定者之间的协作对于改善镰状细胞病儿童的生活至关重要。

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