ACUTE CHEST SYNDROME OF SICKLE-CELL DISEASE - RADIOGRAPHIC AND CLINICAL ANALYSIS OF 70 CASES

Background. Acute chest syndrome (ACS) is a pulmonary illness with fev er, chest pain, leukocytosis and new pulmonary opacity in a patient wi th sickle cell disease. It is a common reason for hospitalization in s ickle cell patients, and a significant cause of mortality, The etiolog y of ACS is unclear. Lung or bone infarction and infection, among othe r possible causes, have been proposed. Objective. We reviewed the ches t radiographs and medical records of 41 patients with 70 episodes of A CS and correlated the clinical and radiographic courses in an attempt to better characterize and understand the syndrome. Results. In 87 % o f episodes, no identifiable etiology of ACS was found. This group of p atients had a median age of 14 years and showed dramatic clinical and radiographic improvement within 24 h of therapy. In the remainder of e pisodes (13 %), an identifiable etiology was found, usually bacterial pneumonia. These patients were younger than the group without an ident ifiable etiology (median age 2 years) and had a prolonged radiographic course of illness. Conclusion. The chest radiographs of children with ACS without an identifiable etiology have an extremely typical appear ance and evolution, Only in cases which do not have this typical patte rn should infection be suspected as the underlying cause.