Division of Hematology, Cincinnati Children's Hospital Medical Center, MC 11027, 3333 Burnet Avenue, Cincinnati, OH 45229, USA; Department of Pediatrics, University of Cincinnati College of Medicine, 231 Albert Sabin Way, Cincinnati, OH 45229, USA.
Pediatr Clin North Am. 2013 Dec;60(6):1363-81. doi: 10.1016/j.pcl.2013.09.006.
Sickle cell disease (SCD) is the name for a group of related blood disorders caused by an abnormal hemoglobin molecule that polymerizes on deoxygenation. SCD affects the entire body, and the multisystem pathophysiology begins in infancy. Thanks to prognostic and therapeutic advancements, some forms of SCD-related morbidity are decreasing, such as overt stroke. Almost all children born with SCD in developed nations now live to adulthood, and lifelong multidisciplinary care is necessary. This article provides a broad overview of SCD in childhood, from newborn screening through transition to adult medical care.
镰状细胞病（SCD）是一组由异常血红蛋白分子引起的相关血液疾病的名称，该分子在脱氧时聚合。SCD 影响全身，多系统病理生理学始于婴儿期。由于预后和治疗的进步，一些与 SCD 相关的发病率正在下降，例如明显的中风。在发达国家，现在几乎所有患有 SCD 的儿童都能活到成年，需要终身多学科护理。本文从新生儿筛查到向成人医疗过渡，对儿童期的 SCD 进行了广泛概述。
