Study of clinical presentation and factors affecting the outcome of acute chest syndrome in sickle cell disease
Background: Acute chest syndrome (ACS) is a frequent and potentially severe pulmonary illness in sickle cell disease (SCD) and it is a leading cause of death and hospitalization among patients with SCD. Aim of the present research was to study the clinical profile, predictors and outcomes of ACS in sickle cell patients.
Method: It was a cross sectional hospital based observational study enrolled total 226 admitted patients of SCD with age more than 12 years. Data on presenting signs and symptoms, laboratory findings, predictors of mortality and outcomes were recorded.
Results: Among the total 226 patients of SCD, 82 (36%) patients were diagnosed to have ACS. Joint pain (72 patients), chest pain (68 patients) and fever (55 patients) were the most common presenting symptoms. Out of 82 patients, 24 (29%) died and 58 (71%) survived. Thus the mortality rate of the study was 29%. The predictors of mortality were respiratory failure; septicemia, multi organ failure and SOFA score ˃10. Patients with severe anaemia, leucocytosis or leucopenia, thrombocytopenia, raised billirubin or elevated liver enzymes, deranged creatinine and hypotension were associated with poor outcome. Blood transfusion was given to 56 patients while oral hydroxyurea was given to 30 patients in the dose of 5 mg/kg.
Conclusion: A multidisciplinary approach to the management of patients of ACS is needed to improve outcome in ACS in SCD. A national consensus guideline should be performed regarding various complications of SCD including ACS.
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