Prognostic factors in cystic fibrosis in a single center in Brazil: A survival analysis.

摘要

The purpose of the present study was to identify prognostic factors related to death in patients with cystic fibrosis (CF). Records of 127 patients with CF submitted to a systematic protocol were retrospectively reviewed. Prognostic factors associated with demographic, nutritional, clinical, and laboratory findings on admission were studied. The median follow-up was 44 months. The analysis was conducted in two steps. First, in a univariate analysis, variables associated with death outcome were identified by the log-rank test. Then the variables that were significantly associated with adverse outcome were included in a multivariate analysis. This analysis, using the Cox proportional hazards model, was performed to identify variables that were independently associated with a worse prognosis. Only variables that remained independently associated with adverse outcome were included in the final model.Three variables were identified as independent predictors of adverse outcome: age at diagnosis under 3 months (relative risk (RR) = 13, 95% CI = 4.5-34, P < 0.001); Shwachman-Kulczycki (S-K score) score below 70; and birth weight under 3,000 g. An interaction effect between S-K score and birth weight was identified. The relative risk of death for the S-K score was 24 (95% CI = 3-195) when birth weight was lower than 3,000 g; on the other hand, when birth weight was 3,000 g or more, the relative risk for the S-K score was 1.4 (95% CI = 0.24-8.83).The combination of three factors (low S-K score, low birth weight, and young age at diagnosis) is indicative of a poor prognosis in CF patients. Pediatr Pulmonol. 2002; 34:3-10.