Acute Chest Syndrome: A Leading Cause of Death in Sickle Cell Disease

Abstract

Acute chest syndrome (ACS) is a leading cause of mortality in sickle cell disease (SCD), and is one of the numerous complications of sickle cell disease. The aetiological factors associated with the development of ACS could either be either infectious or non-infectious. ACS develops insidiously or suddenly, during admission for vaso-occlusive crises, or after surgery particularly abdominal surgery. Diagnosing and managing it may be challenging in in-experienced hands. The outcome of ACS depends on rapid diagnosis (clinical, radiological and laboratory), as well as institution of appropriate management, which aims to reduce the incidence of respiratory failure, and death. Therefore, this review article aims to elucidate on ACS in order to improve on the diagnostic and management skills of Emergency Physicians/ Surgeons, Pediatricians, Family Physicians, Internists and Haematologists who manage SCD patients, thus reducing associated mortality. The following search engines were used in this review article: Google, Pubmed and UpToDate. Key words used in the search were: Acute chest syndrome and Sickle cell disease. The number of articles initially obtained was 53; however 41 articles were included in this review. Literature search for this review article was conducted over a period of four (4) months from June 2016 to October 2016.