Finally, a consensus statement on sickle cell disease manifestations: A critical step in improving the medical care and research agenda for individuals with sickle cell disease

Comparison of automated red cell exchange transfusion and simple transfusion for the treatment of children with sickle cell disease acute chest syndrome

“…Patients with sickle cell disease were determined to have ACS if they developed a new pulmonary infiltrate involving at least one complete lung segment, consistent with infiltration rather than atelectasis, and associated with at least 1 additional finding including chest pain, fever, tachypnea, wheezing, rales, cough, increased work of breathing (intracostal retractions, nasal flaring, use of accessory muscles), a >2% decrease in room air oxygen saturation, or a PaO 2 < 60 mm Hg . Patients were admitted to the hospital, started on IV hydration at a rate of 1–1.5 times maintenance, and given pain control as needed, typically morphine delivered by patient controlled analgesia (PCA), and ketorolac, but other medications were used at the discretion of the attending hematologist.…”

“…Acute chest syndrome is defined as a new pulmonary infiltrate involving at least one complete lung segment, consistent with infiltration rather than atelectasis, and associated with at least 1 additional finding including chest pain, fever, tachypnea, wheezing, rales, cough, increased work of breathing (intracostal retractions, nasal flaring, use of accessory muscles), a >2% decrease in room air oxygen saturation, or a PaO 2 < 60 mm Hg in a patient with sickle cell disease . ACS is the second most common cause of hospitalization and the most frequent cause of death in patients with sickle cell disease .…”

Asthma morbidity and treatment in children with sickle cell disease

“…Multiple ACS definitions have been proposed; although significant differences exist in terms of clinical symptoms needed, it is accepted that an infiltrate or new radiodensity on imaging is an important criterion that needs to be fulfilled. Other clinical features include hypoxemia, respiratory distress, fever, need for blood transfusion and rapid deterioration in clinical status [25,26]. Differentiating between an acute asthma exacerbation and ACS is a challenge clinically as they can present with similar symptoms.…”

Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype

“…Since ACS was first described in 1979 , various clinical definitions and severity criteria have been proposed . However, none of the most commonly cited definitions include the temporal relationship between the onset of respiratory symptoms and abrupt decline in respiratory status . Toddlers with bronchiolitis who develop ACS with a subacute course over a course of several days are considered to have the same clinical entity as an adult with rapidly progressive ACS that results in respiratory failure within 24 hours, a well‐recognized clinical presentation .…”