Synopsis: Current Trends in the Management of Acute Chest Syndrome in Children with Sickle Cell Disease

Abstract

Acute chest syndrome (ACS) is one of the most serious and frequent pulmonary complications of sickle cell disease in childhood. Defined by the presence of a new pulmonary infiltrate on chest imaging accompanied by fever and/or respiratory symptoms. The clinical manifestation of ACS ranges from a mild, self-limited illness to fulminant respiratory failure requiring intensive care and life-saving interventions. Acute chest syndrome is one of the leading causes of hospitalization and a major contributor to morbidity and mortality in children with sickle cell disease (SCD). Early recognition and prompt institution of evidence-based management strategies are therefore essential to reduce the immediate complications and long-term pulmonary sequelae of the disease. This review discusses current trends in the management of ACS in children with SCD, as well as practical approaches to ACS management in resource-poor settings.