Clinical manifestations of sickle cell anemia: Infants and children

Abstract

Children with sickle cell disease (SCD) have varied clinical problems. The hallmark manifestation of SCD, the pain crisis, typically first occurs in early childhood presenting as dactylitis. Pain increases in frequency and severity as children age, especially during adolescence. Due to functional asplenia, children with SCD are at signifi cantly increased risk for certain infections, most notably Streptococcus pneumoniae. Other infections like parvovirus B19 are also special threats and can trigger an aplastic crisis. Unique, potentially life-threatening acute complications like splenic sequestration and acute chest syndrome occur in these children. They are at risk for neurologic disease, the most serious being stroke. In addition, the chronic hemolysis of SCD causes gallstones, which can lead to biliary tract disease. Children with SCD also frequently face chronic issues that include nocturnal enuresis and decreased growth. Despite these many potential problems, with advances in care including antibiotic treatment, stroke screening, blood transfusions, and hydroxyurea therapy, children with SCD rarely die in childhood and can become productive adults.