Abstract
Sickle cell disease is the most common blood disorder in the United States, affecting 100 000 people. A genetic mutation creates hemoglobin S. In the deoxygenated state, hemoglobin S polymerizes, creating sickled hemoglobin. Sickled hemoglobin causes a cascade of complex pathophysiologic events that lead to hemolysis, chronic anemia and endothelial damage. This results in clinical complications, end organ dysfunction and a shortened life expectancy. The acute nature of many sickle cell complications makes the emergency department a common setting where sickle cell patients present. Common complications (vaso-occlusive episode, fever, acute chest syndrome, stroke) and less common complications (splenic sequestration, priapism, aplastic crisis, ocular emergencies) will be discussed. Public health implications will be discussed briefly.
Original language | English (US) |
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Pages (from-to) | 103-109 |
Number of pages | 7 |
Journal | Clinical Pediatric Emergency Medicine |
Volume | 19 |
Issue number | 2 |
DOIs | |
State | Published - Jun 2018 |
Keywords
- acute chest syndrome
- anemia
- aplastic crisis
- complications
- priapism
- sickle cell disease
- splenic sequestration
- stroke
- vaso occlusive crisis
- vaso occlusive episode
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Emergency Medicine