RESULTS
A total of 210 individuals with CF were followed in the CF Center from
2000 to 2020; five patients were excluded due to missing data and 205
patients were included in the study cohort. Ninety-nine patients were
female (48.3%). Mutation analysis was available for 156 patients
(76.1%). Patient characteristics are described in Table 1. Median age
at diagnosis was 1 year (1 month – 34 years). Median survival age for
the cohort was 21.37 years (95% CI 17.20 – 25.55). Median survival age
for high SES was 29.46 years (95% CI 24.09 – 34.83) vs 15.12 years
(95% CI 11.78 – 18.45) for low SES (p < 0.001). Patients
with a diagnosis of chronic infection of P. aeruginosa at 6 years
had a median survival of 12.41 years (95% CI 10.11 – 14.73) compared
to 35.25 years (95% CI 26.61 – 43.70) for patients with no evidence of
chronic infection at 6 years (p=0.030). Differences in median survival
age are presented in Table 2. Survival curves by SES and by P.
aeruginosa chronic infection status are displayed in Figure 1.
Differences in survival were statistically significant
(p<0.001, p<0.001).
Median age at death for the cohort was 12.46 years (1 month – 43.65
years). Median age at death in the first 5-year period (2000 – 2005)
was 9.55 years (1 month – 30.61 years), while for the last 5-year
period (2016 – 2020), it was 15.49 years (5 months – 39.27 years)
(p=0.012). Median age at death according to gender, SES, presence of
chronic P. aeruginosa infection at 6 years, pancreatic status,
and genotype are shown in Table 3.
Uni- and multivariable Cox regression analyses for mortality are shown
in Table 4. Variables that reached
significance in the multivariate model were chronic P. aeruginosainfection at 6 years, low SES, and pancreatic insufficiency.