Describing acute chest syndrome in children with sickle cell Anaemia

Acute Chest Syndrome (ACS) is a potential life threatening complication of Sickle Cell disease (SCD) characterized by the presence of new pulmonary infiltrates with respiratory findings in a patient with SCD. It is the second commonest cause of hospital admission in SCD, next to vasoocclusive crises and accounts for 25% of mortalities. The aetiology is unknown but infection and infarction play major roles. Common presenting features include fever, cough, wheezing, chest pain and pleural effusion. An abnormal Chest X-ray is generally required to confirm the diagnosis. The X-ray changes were previously thought to be caused mainly by infection but recent reports have suggested that pulmonary infarction due to local sickling and emboli may be more likely. Treatment includes intravenous antibiotics using a third generation cephalosporin and a macrolide or quinolone, blood transfusion, hydration and analgesics. NSAIDs may worsen ACS due to vasoconstriction and bronchospasm, corticosteroids are controversial, bronchodilators may be useful, Nitric oxide (NO) has been found to be beneficial and bronchoscopy is not routinely done. Because ACS is common in children and yet often underdiagnosed, all paediatric SCD patients who present with fever should be evaluated with a chest radiograph and daily fluid intake should not exceed 1,500mls/m2/day. The aim of this report is to add to the understanding of ACS in this region and also to sensitize physicians to the spectrum of clinical presentation of ACS so as to improve diagnosis and treatment and hence prevent morbidity and mortality.