Acute chest syndrome in pediatric sickle cell disease: Associations with racial composition and neighborhood deprivation.

BACKGROUND: Acute chest syndrome (ACS) is the leading cause of death for children with sickle cell disease (SCD). Recurrent ACS has detrimental effects on pulmonary health and health care costs. Neighborhood characteristics affect the outcomes of many pediatric chronic diseases, but their role in SCD is not well studied. In this study, we investigated the effects of area-level socioeconomic deprivation and racial composition on the recurrence of ACS. STUDY DESIGN: We performed a retrospective cross-sectional analysis of clinical data from a large pediatric SCD center. Patients' residential addresses were geocoded and linked to a composite area deprivation index (ADI) and percent African American population at the level of Census block groups. The association of recurrent ACS with neighborhood characteristics was evaluated using logistic regression analysis. RESULTS: The sample included 709 children with SCD. Residence in a socioeconomically deprived neighborhood was associated with 27% less risk of recurrent ACS, and residence in a predominantly African American neighborhood was associated with 41% less risk of ACS recurrence. The racial composition explained the protective effect of living in a high-deprivation area after adjusting for sociodemographic and clinical covariates. Demographic and clinical factors associated with recurrent ACS included older age, male gender, asthma, hydroxyurea use, and chronic transfusion therapy. CONCLUSIONS: This is the first study to report a protective effect of residing in a predominantly African American community for ACS recurrence. Further prospective studies are needed to confirm the association and to understand the mechanisms of such relationship.

The association of area deprivation and state child health with respiratory outcomes of pediatric patients with cystic fibrosis in the United States.

BACKGROUND: Differences in socioenvironmental exposures influence overall child health, but their association with pediatric cystic fibrosis (CF) outcomes is less clear. This observational study investigated the relationship between area-level socioeconomic deprivation, state child health, and CF respiratory outcomes in a national cohort. METHODS: We assessed relationships between the 2015 area deprivation index, a composite measure of socioeconomic disadvantage; the 2016 child health index, a state-specific measure of overall child health; and CF respiratory outcomes in the 2016 CF Foundation Patient Registry. RESULTS: The sample included 9934 individuals with CF, aged 6-18 years. In multiple regression analysis adjusted for demographic and clinical covariates, those residing in the worst tertile for area deprivation had 2.8% lower percent predicted forced expiratory volume in 1 s (ppFEV1 ; 95% confidence interval [CI]: -4.1 to -1.5), 1.2 more intravenous (IV) treatment nights (CI: 0.1-2.4), and 20% higher odds of ≥2 pulmonary exacerbations (odds ratio [OR]: 1.2, CI: 1.0-1.5) than best-tertile counterparts. Children with CF in states at the worst tertile for child health had 2.3% lower ppFEV1 (CI: -4.5 to -0.2), 2.2 more IV treatment nights (CI: 0.5-3.6), and 40% higher odds of ≥2 exacerbations (OR: 1.4, CI: 1.1-1.8) than best-tertile counterparts. State child health accounted for the association between area deprivation and multiple exacerbations and more IV treatment nights. CONCLUSIONS: Both area socioeconomic characteristics and state child health play a role in pediatric CF outcomes. The residual association of the state child health with CF outcomes after controlling for area deprivation reflects the ability of state programs to mitigate the effect of poverty.