Abstract

Cystic Fibrosis (CF) is a rare genetic disease, of autosomal recessive transmission, with multiple organ involvement, a progressive course and is potentially lethal. We studied the factors associated with the reduced survival. In an open cohort of cases diagnosed between 01 January 1990 and 10 October 2009 in a CF reference centre in Rio de Janeiro, we analysed survival and risk factors associated with survival. Information on patients included that on CF diagnostic criteria follow-up and outcome. The model included variables on gender, genotype, number of involved organs, nutritional state, bacterial colonisation, enzyme replacement and calendar-time of diagnosis. Survival was estimated by Kaplan–Meier (KM) method and covariates examined by log-rank tests. HRs were estimated by a Cox model and evaluated by the likelihood ratio, deviance and residual analysis. The majority of the population (n=177) was female (56%) and the median age at diagnosis was 14 months. The median survival was 19 years. After diagnosis, 81% survived up to 5 years, 70% up to 10 and 61% up to 14.5. The model explained 19.9% of the effects and included six covariates. HRs were 10.30 (2.41–43.97) for isolated pseudomonas colonisation, 4.50 (0.93–1.85) for Staphylococcus aureus, 3.38 (0.92–1.32) for other bacteria, 1.95 (0.96–3.96) for gender, 1.94 (0.94–3.98) for nutritional state and 4.34 (1.50–12.52) for decade of diagnosis. Risk factors obtained at diagnosis were associated with prognosis suggesting that interventions may reduce morbidity by nutritional improvement and pseudomonas eradication.