Pulmonary complications of sickle cell disease in children

Abstract

Sickle cell disease is one of the most prevalent genetic diseases, affecting 1 in 600 African-Americans. The lung is one of the major organs affected in sickle cell disease and the pulmonary complications of sickle cell disease result in significant morbidity and mortality in children. Recent literature has provided evidence supporting the strong association between asthma and airway hyperreactivity and sickle cell disease, as well as a link with acute chest syndrome and vaso-occlusive crisis. Additionally, children with sickle cell disease who have acute chest syndrome episodes have worse pulmonary function than those who have not experienced acute chest syndrome episodes. An increasing amount of literature highlights the link between pulmonary hypertension and sickle cell disease, and multiple mechanisms have been invoked to explain why patients with sickle cell disease are prone to the development of pulmonary hypertension. Previous and current studies have also highlighted the manifestations of sleep disordered breathing in children with sickle cell disease. The pulmonary complications of sickle cell disease include airway hyperreactivity, acute chest syndrome, chronic sickle lung disease, pulmonary hypertension, and sleep disordered breathing. Further understanding of the interrelationships between these disorders will lead to improved therapies.