Acute chest syndrome in children with sickle cell disease: Saudi Arabian experience

Acute chest syndrome (ACS) is a known complication of sickle cell disease (SCD). It carries high morbidity and mortality. We conducted a retrospective study to evaluate the frequency, clinical, laboratory, and radiological features of this complication among children with sickle cell disease in the Southern Province of Saudi Arabia. Our results were generally comparable to international published data among similar population. Our results revealed that the frequency of ACS episodes are age dependent, which occurred more frequently in young children i.e. < 4 years of age (57%). Fever and cough were the most frequent symptoms; 93% and 86% respectively. Most of the cases experienced respiratory distress such as tachypnea (86%), chest retraction (64%), and decreased breath sounds (57%). On the other hand only small number of patients (11%) had complete normal chest examination. ACS, as other sickle cell crises, usually develops in association with other complication. In our study, painful crisis was the most commonly associated complication along with ACS (79%). All of the chest X-rays were positive at different anatomical sites; bilateral involvement was observed frequently (36%). This study is unique being the first description of such problem in children in the Southern Province of Saudi Arabia. It will serve as a base s for subsequent studies.