PEDIATRIC ANNALS 42:3 | MARCH 2013 Healio.com/Pediatrics | 115 FEATURE Hussein A. Abbas, PhD, is a medical student at American University of Beirut Medical Center, Lebanon; and Assistant Professor at American University of Science and Technology. Mario Kahale, MD, is a clinical research fellow, Inati Re- search Group. Maen Aboul Hosn, MD, is PGY-4 general surgery resident, American University of Beirut Medical Center. Adlette Inati, MD, is Head, Division of Pediatric Hematology-Oncology, Raic Hariri University Hospital; and Associate Professor in Pediatrics, Lebanese American Uni- versity, School of Medicine, Byblos, Lebanon. Address correspondence to: Adlette Inati, MD, Lebanese American University, University Medical Center – Rizk Hospi- tal, PO Box 11-3288, Beirut, Lebanon; email: adlette.inati@lau.edu.lb. Disclosure: The authors have no relevant i- nancial relationships to disclose. doi: 10.3928/00904481-20130222-11 S ickle cell disease is an autoso- mal recessive blood disorder that affects millions of individu- als worldwide. A single mutation in the beta globin gene resulting in a single amino acid substitution underlies the pathophysiology of this highly prevalent disorder. The disease is characterized by chronic hemolysis, recurrent vaso-oc- clusion, predisposition to infections, and end-organ failure. Phenotypic heteroge- neity characterizes sickle cell disease, with pain as the hallmark of this disease. The past 2 decades have witnessed an influx of information that has improved our current knowledge of the molecular, clinical, and therapeutic aspects of the disease. Multiple randomized, obser- vational, and interventional trials have demonstrated that with early diagno- sis and timely introduction of modern therapy in a comprehensive care setting, the survival rates and quality of life for affected patients have remarkably im- proved over recent years. Acute chest syndrome (ACS) is the most common cause of sickle cell dis- ease (SCD)-related death, accounting for one-fourth of these fatalities. ACS is also the second most common cause of hospitalization in patients with SCD. Many patients have multiple severe epi- sodes of ACS, and recurrent ACS events have been associated with chronic lung disease and early death. Rapid diagnosis and administration of appropriate therapy can help reduce morbidity and mortality associated with ACS. Because of improved survival and increased numbers of SCD patients liv- ing to adulthood, it is important for pri- mary care providers to become familiar with all of the diagnostic, therapeutic, and preventive aspects of this frequent complication. A Review of Acute Chest Syndrome in Pediatric Sickle Cell Disease Hussein A. Abbas, PhD; Mario Kahale, MD; Maen Aboul Hosn, MD; and Adlette Inati, MD © Shutterstock