Answer: The correct answer is acute chest syndrome (a).
Acute chest syndrome (ACS) is a potentially life-threatening complication of sickle cell disease characterized by lung infiltrates, fever, and respiratory symptoms, including cough, tachypnea, wheeze, increased work of breathing or shortness of breath, and reduced oxygen saturation. ACS is the second most common reason for hospitalization in children and adults with sickle cell disease and is their leading cause of mortality. Management of ACS in children and adults is similar, although the disease is often more severe in adults, among whom it is associated with bone marrow and fat emboli. ACS can be triggered by an underlying infection or vaso-occlusive crisis, although in most cases, an identifiable trigger is not determined. Sickle cell disease is the primary risk factor for ACS. Within the population with sickle cell disease, the following are predictors for increased risk of developing ACS: young age, low fetal hemoglobin, leukocytosis, genotypes HbSS (sickle cell anemia) and HbSβ0, asthma, tobacco exposure, recent surgery, or three or more severe veno-occlusive crises in the past year.
Management requires hospitalization for hydration, oxygenation, intravenous antibiotics, pain control, and blood transfusions and, in some cases, exchange transfusion.
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- Winter MW, Ingerowski E, Prasad P. Acute chest syndrome. VisualDx.com website. Accessed April 4, 2023.