ACUTE CHEST SYNDROME IN ADULT AFRO-CARIBBEAN PATIENTS WITH SICKLE-CELL DISEASE - ANALYSIS OF 81 EPISODES AMONG 53 PATIENTS

Background: To evaluate the frequency, presentation, and course of the acute chest syndrome (ACS) in adult Afro-Caribbean patients with sick le cell disease (SCD). Patients and Methods: Retrospective cohort stud y during a 12-year period in patients with SCD at least 14 years of ag e, discharged with a diagnosis of ACS from the only hospital on the Ca ribbean island of Curacao, where 109 patients with SCD (62 HbSS, 47 Hb SC) were observed. Results: Eighty-one episodes of ACS occurred (57 in 34 patients with HbSS and 24 in 19 patients with HbSC):. The risk (od ds ratio, 1.80; P=.13) and incidence (7.6 vs 4.2 per 100 patient-years ; P>.2) of ACS did not differ between patients with HbSS and HbSC, but recurrent ACS affected patients with HbSS more (odds ratio, 2.96; P=. 09). Abnormal chest sounds (mainly bilateral crepitations) were found in 91% of cases at diagnosis, but 48% had normal chest roentgenograms at that time and had delayed development (5.4+/-3.4 days) of radiologi c abnormalities. Patients with HbSS and HbSC had similar clinical pres entations. Mortality (6%) and hospital stay (20 days) were not influen ced by the use of transfusions or anticoagulation. All five nonsurvivi ng female patients with HbSS had had more previous admissions for SCD and ACS. Conclusions: Acute chest syndrome occurs in 42% of adult Afro -Caribbean patients with SCD; patients with HbSS are more prone to rec urrences. Delayed development of radiologic infiltrates is common. Int erventions apart from supportive care do not influence the course of A CS. Fatal ACS occurs in patients with a more severe form of SCD.