Journal
PEDIATRIC CLINICS OF NORTH AMERICA
Volume 60, Issue 6, Pages 1363-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.pcl.2013.09.006
Keywords
Sickle cell disease; Genetics; Diagnosis; Pathophysiology; Treatment; Survival
Categories
Funding
- NHLBI
- Eli Lilly and Co
- MAST Therapeutics, Inc.
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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.
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