Associated Factors of Acute Chest Syndrome in Children with Sickle Cell Disease in French Guiana

A matched case-control study was performed in order to identify some associated factors for ACS or to confirm the published data. Controls were children hospitalized during the same period for pain crisis who did not develop an ACS during hospitalization. Between January 2006 and October 2010, there were 24 episodes of ACS distributed among 19 patients (8 girls and 11 boys). The median age was 7.5 years (range: 3 to 17 years) for the cases and 7 years (range: 3–18 years) for the controls. Four cases and 11 controls were treated with hydroxyurea (HU). In 75% of the cases, the ACS had arisen 24–72 hours following admission. The independent factors associated with ACS were average Hb rate <8？g/dL (OR = 4.96, 95% CI = 1.29–27.34, and ), annual number of hospitalizations >3 (OR = 5.44, 95% CI = 3.59–8.21, and ), average length of hospitalization >7 days (OR = 3.69, 95% CI = 3.59–8.21, and ), and a pathological transthoracic echocardiography (TTE) (OR = 13.77, 95% CI = 2.07–91.46, and ). Although the retrospective design and small sample size are weaknesses of the present study, these results are consistent with those of previous studies and allowed identifying associated factors such as a pathological TTE. Sickle cell disease (SCD) is a major public health concern in French Guiana, a French region with 230,000 inhabitants located in South America [1]. The incidence of major SCD from birth screening is 1/227, and the overall frequency of AS carriers is 10% [2]. The major SCD groups include the three main genetic forms: hemoglobin (Hb) SS (68%), Hb SC (25%), and Sβ thalassemia (7%). The acute chest syndrome (ACS) is a complication of SCD characterized by pleuritic chest pain, fever, rales on lung auscultation, and pulmonary infiltrates on chest X-ray [3]. It is the most frequent cause of mortality in children with SCD [3–8]. In 1979, Charache et al. first suggested using the term acute chest disease (ACD) for this complication, acknowledging the difficulties in determining its pathogenesis [9]. We report here the results of a case-control study of risk factors for ACS in children with SCD in French Guiana, in order to find some associated factors for ACS or to confirm the published data. We hypothesized that HbSS, age, high Hb level, and high steady-state leukocyte count could be risk factors for ACS. This matched case-control study concerned all cases of ACS hospitalized in the pediatric unit in French Guiana from 2006 to 2010. The cases were children hospitalized between January 2006 and October 2010 for pain crisis and who developed an ACS. The controls