Management of Sickle Cell Disease in Children.
Journal Article (Journal Article;Review)
Sickle cell disease (SCD) is a heterogeneous inherited disorder of hemoglobin that causes chronic hemolytic anemia, vaso-occlusion, and endothelial dysfunction. These physiologic derangements often lead to multiorgan damage in infancy and throughout childhood. The most common types of SCD are homozygous hemoglobin S (HbSS disease), hemoglobin SC disease, and sickle β thalassemia. HbSS disease and sickle β(0) thalassemia often are referred to as sickle cell anemia because they have similar severity. Screening and preventive measures, including infection prophylaxis and vaccination, have significantly improved outcomes for children with SCD. Evidence-based therapies, such as hydroxyurea and transfusion, play an important role in preventing progression of select complications. Many chronic complications develop insidiously and require multidisciplinary care for effective treatment. Primary care physicians, as well as physicians in many other disciplines, may care for these patients and should be familiar with the potential acute and chronic complications of this disease. This review addresses healthcare maintenance guidelines, common complications, and recommendations for management of pediatric patients with SCD.
Full Text
Duke Authors
Cited Authors
- Noronha, SA; Sadreameli, SC; Strouse, JJ
Published Date
- September 2016
Published In
Volume / Issue
- 109 / 9
Start / End Page
- 495 - 502
PubMed ID
- 27598348
Electronic International Standard Serial Number (EISSN)
- 1541-8243
Digital Object Identifier (DOI)
- 10.14423/SMJ.0000000000000523
Language
- eng
Conference Location
- United States