Management of acute chest syndrome in patients with sickle cell disease: a systematic review of randomized clinical trials.

Muhammad Rafay Khan Niazi, Divya Chukkalore, Abdullah Jahangir, Syeda Sahra, Kira Macdougall, Maryam Rehan, Marcel Odaimi

Expert review of hematology 2022 Jun

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Acute chest syndrome (ACS) accounts for the highest mortality in Sickle cell disease patients. Early diagnosis and timely management of ACS results in better outcomes. However, the effectiveness of most treatment modalities for ACS management has not been established. To review the treatment modalities management protocols and highlight the effectiveness of each option a literature search was done. Randomized controlled trials that assessed the efficacy of different treatment modalities in ACS management in SCD patients were chosen and reviewed. 11 randomized controlled trials were found that evaluated the efficacy of incentive spirometry, positive expiratory pressure device, intravenous dexamethasone, oral vs. intravenous morphine, inhaled nitric oxide, unfractionated heparin, and blood transfusion in the prevention or treatment of ACS. Although there are guidelines for ACS treatment, the available evidence is very limited to delineating the effectiveness of various interventions in ACS management. More high-quality studies and trials with a larger patient population can benefit this area to support the recommendations with stronger evidence.

Citation

Muhammad Rafay Khan Niazi, Divya Chukkalore, Abdullah Jahangir, Syeda Sahra, Kira Macdougall, Maryam Rehan, Marcel Odaimi. Management of acute chest syndrome in patients with sickle cell disease: a systematic review of randomized clinical trials. Expert review of hematology. 2022 Jun;15(6):547-558