ABSTRACT
Objective:
To describe causes of death and mortality data related to cystic fibrosis (CF) using a multiple-cause-of-death methodology.
Methods:
Annual mortality data for the 1999-2017 period were extracted from the Brazilian National Ministry of Health Mortality Database. All death certificates in which category E84 (CF) of the ICD-10, was listed as an underlying or associated cause of death were selected. Epidemiological and clinical data were described, and standardized mortality rates were calculated per year and for the 2000-2017 period. A joinpoint regression analysis was performed to detect changes in the mortality rates during the study period.
Results:
Overall, 2,854 CF-related deaths were identified during the study period, ranging from 68 in 1999 to 289 in 2017. CF was the underlying cause of death in 83.5% of the death certificates. A continuous upward trend in the death rates was observed, with a significant annual percent change of 6.84% (5.3-8.4%) among males and 7.50% (6.6-8.4%) among females. The median age at death increased from 7.5 years in 1999 to 56.5 years in 2017. Diseases of the respiratory system accounted for 77% of the associated causes in the death certificates that reported CF as the underlying cause of death.
Conclusions:
A significant and continuous increase in CF-related death rates was found in Brazil in the last years, as well as a concurrent increase in the median age at death.
Keywords:
Cystic fibrosis/mortality; Cause of death; Death certificates; Brazil
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