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Pediatr Clin North Am. 2013 Dec;60(6):1363-81. doi: 10.1016/j.pcl.2013.09.006.

Sickle cell disease in childhood: from newborn screening through transition to adult medical care.

Author information

1
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. Electronic address: charles.quinn@cchmc.org.

Abstract

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.

KEYWORDS:

Diagnosis; Genetics; Pathophysiology; Sickle cell disease; Survival; Treatment

PMID:
24237976
PMCID:
PMC4262831
DOI:
10.1016/j.pcl.2013.09.006
[Indexed for MEDLINE]
Free PMC Article

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