The acute chest syndrome is a common reason for patients with a sickling hemoglobinopathy to present for health care. It is a syndrome that may be due to several mechanisms.


The clinical and laboratory features vary with the cause and may include:

(1) fever, with a temperature > 38.5°C

(2) chest pain

(3) cough

(4) tachypnea

(5) wheezing and bronchospasm

(6) chills

(7) hypoxemia

(9) leukocytosis

(9) deterioration of pulmonary function tests

(10) imaging findings may include:

(10a) presence of a new pulmonary infiltrate involving at least 1 complete lung segment and/or

(10b) a perfusion defect on radionuclide imaging study of the chest


Criteria for the diagnosis of acute chest syndrome (Castro et al, pages 643 and 644):

(1) presence of respiratory signs and symptoms

(1a) pleuritic chest pain

(1b) fever

(1c) rales on lung auscultation

(1d) wheezing, dyspnea and/or cough

(2) presence of one or both of the following:

(2a) radiographic evidence of a new infiltrate on chest X-ray

(2b) perfusion defect on a ventilation-perfusion scan


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