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.
Key points
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SCD affects the entire body, beginning in very early infancy, and a multidisciplinary team is needed to care for children with SCD.
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Some forms of SCD-related morbidity, such as overt stroke, are decreasing owing to prognostic and therapeutic advancements.
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Primary prevention of organ injury is an important focus of current research.
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Almost all children born with SCD in developed nations now survive to adulthood, but the transition to adult medical care is a high-risk period for death.
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