Acute chest syndrome in Omani children with sickle cell disease: epidemiology and clinical profile

Abstract

Aims: To describe the epidemiology and clinical profile of acute chest syndrome (ACS) in Omani Arab children.

Methods: Prospective, clinical study of consecutive episodes of ACS in patients with sickle cell disease (SCD) seen at Nizwa Regional Referral Hospital, Oman between June 2001 and May 2006.

Results: Of 240 patients registered with SCD, 52 (22%) developed 55 episodes of ACS. There were 31 (59.6%) males and ages ranged between 14 months and 12 years [median (SD) 6.5 (2.49) years]; ACS occurred fairly evenly in those aged between 2 and 10 years. Ten (18%) episodes were mild, 51% moderate and 31% severe. In general, there was no relationship between severity of SCD and the incidence of ACS, but in 71% of cases a vaso-occlusive crisis (VOC) preceded the episodes. Other probable precipitating factors were acute upper respiratory tract infections (in 29% of cases) and use of morphine (26%). The distribution of ACS during the year was bimodal, peaking in May and September and at its lowest in June and December/January. Fever, cough, chest pain, tachypnoea, reduced breath sounds, crackles and chest radiograph opacities were the main manifestations. However, respiratory physical signs were absent in 36% of episodes.

Conclusions: ACS is common in SCD. Irrespective of SCD severity, all patients appeared to be at risk of the syndrome, but the risk is increased by VOCs. Prevention of VOCs, prompt and effective treatment of respiratory infections and caution in the use of morphine during a VOC should reduce the incidence of ACS.