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AAP Grand Rounds 4:17-18 (2000) Causes and Outcomes of Acute Chest Syndrome in Sickle Cell DiseaseSource: Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342:18551865.
Thirty United States centers evaluated 671 episodes of acute chest syndrome (ACS) in 538 patients from 19931997 in this prospective study to determine the cause, outcome, and response to therapy. ACS was defined as a new pulmonary infiltrate on chest radiograph in association with chest pain, temperature elevation (>38.5°C), tachypnea, wheezing, or cough in a patient known to have hemoglobin SS, SC or SS-beta thalassemia and at least one prior episode of ACS. Patients were treated with a standardized protocol including oxygen, antibiotics, fluid management, respiratory therapy and bronchodilators, pain management, and transfusion therapy. Laboratory evaluations included blood counts, blood gases, chest radiographs, pulmonary function testing, viral cultures, bacterial cultures, serologies (for Mycoplasma pneumoniae, Epstein-Barr virus, Chlamydia, and parvovirus), and histologic examination for fat embolism (lipid laden macrophages on bronchoalveolar lavage).
The mean age at the time of the first episode of ACS was 13.8 years, and slightly over half of the patients were male (58%). Only half
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