Acute chest syndrome is one of the complications of sickle cell disease.

Pathogenesis: pulmonary infection, pulmonary fat embolism from necrotic bone marrow and/or pulmonary infarction


Clinical features (vary with age):

(1) acute illness

(2) cough

(3) chest pain

(4) fever (>= 38.5°C)

(5) hypoxemia

(6) tachypnea

(7) dyspnea

(8) variable crepitations on auscultation


Findings on imaging studies: segmental pulmonary infiltrate consistent with consolidation



(1) progression to acute respiratory failure

(2) multi-organ failure

(3) neurological complications (stroke, posterior reversible leukoencephalopathy, etc)

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