Sickle Cell Disease in Childhood From Newborn Screening Through Transition to Adult Medical Care

被引:68
作者
Quinn, Charles T. [1 ,2 ]
机构
[1] Cincinnati Childrens Hosp Med Ctr, Div Hematol, Cincinnati, OH 45229 USA
[2] Univ Cincinnati, Dept Pediat, Coll Med, Cincinnati, OH 45229 USA
关键词
Sickle cell disease; Genetics; Diagnosis; Pathophysiology; Treatment; Survival; ACUTE CHEST SYNDROME; PULMONARY-HYPERTENSION; ADVERSE OUTCOMES; CHILDREN; ANEMIA; STROKE; TRANSFUSIONS; HOSPITALIZATION; DEXAMETHASONE; HYDROXYUREA;
D O I
10.1016/j.pcl.2013.09.006
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Sickle cell disease (SOD) is the name for a group of related blood disorders caused by an abnormal hemoglobin molecule that polymerizes on deoxygenation. SOD affects the entire body, and the multisystem pathophysiology begins in infancy. Thanks to prognostic and therapeutic advancements, some forms of SOD-related morbidity are decreasing, such as overt stroke. Almost all children born with SOD in developed nations now live to adulthood, and lifelong multidisciplinary care is necessary. This article provides a broad overview of SOD in childhood, from newborn screening through transition to adult medical care.
引用
收藏
页码:1363 / +
页数:20
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