Assessment of hospitalization rates, factors associated with hospitalization and in-patient mortality in pediatric patients with cystic fibrosis

Introduction: Cystic fibrosis (CF), an inherited autosomal recessive disease that results in the accumulation of mucus and damage primarily to the respiratory and digestive tracts is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In the United States, it has been estimated that CF occurs in 1 out of 3500 infants. The objective of this study was to explore the patient and hospital characteristics associated with CF hospitalizations and inpatient mortality in pediatric CF patients. Methods: Utilizing the National Inpatient Sample database from 2008 to 2017, a retrospective cohort study was conducted to analyze the hospitalization rates, associated factors, and the inpatient mortality of CF patients 0-17 years of age. Hospitalizations with a diagnosis of CF were identified with ICD-9-CM and ICD-10-CM codes. Adjusted survey logistic regression models were utilized to determine factors associated with CF hospitalizations and in-hospital deaths in CF patients. Results: There were a total of 98,660 (about 0.2%) CF hospitalizations in patients 17 years of age or younger during the study period. Non-Hispanic (NH) White CF patients had the highest prevalence of CF (26.30 per 10,000 hospitalizations). The prevalence of inpatient deaths were highest among those identified as NH-Others and NH-Blacks (71.35 and 68.83 per 10,000 CF hospitalizations, respectively. When compared with NH-White category, those belonging to NH-Black, Hispanic and Other racial/ethnic sub-group had reduced odds of being hospitalized with CF. Discussion: Despite our finding of an increased likelihood of being hospitalized for CF among NH-White and male pediatric patients, no association between race or sex and CF inpatient death was observed when adjusted for covariates. More research is needed to determine the impact of sex and race on CF mortality rates.