Sickle cell crises in the lung - otherwise referred to as acute chest syndrome (ACS) - are characterised by pleuritic chest pains, fever, abnormal chest examinations and presence of new infiltrations in the chest X ray (1).
They are of mixed pathology; caused by a combination of infection, fat embolism and vaso occlusion of the pulmonary vasculature (2)
A combination of clinical signs or symptoms of chest consolidation and worsening hypoxia is sufficient for the diagnosis of ACS (since X ray changes often lag behind clinical signs) (3)
Local protocols should be prepared for management of ACS and should include:
Longterm treatment with hydroxycarbamide (former British Approved Name, hydroxyurea) has led to a reduction by 50% in acute chest syndrome in some studies.
Reference:
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