Prognostic aspects of cystic fibrosis in Brazil

Selected prognostic factors were assessed in a cohort of 111 Brazilian children with cystic fibrosis diagnosed between 1 June 1970 and 31 December 1994 and followed in the Pediatric Pulmonology Unit of the Federal University of Minas Gerais Hospital. A standardized protocol was used to collect information retrospectively from medical charts which included date of birth, age at diagnosis, gender, ethnic group, predominant mode of presentation, duration of follow-up, status on 31 December 1994, date of death or date of the last clinical evaluation. The risk of death (univariate and multivariate) was assessed using Cox's proportional hazard model. Mean age at death was higher in males (5.10 years), when the diagnosis was made before the age of 12 months (3.10 years), when clinical presentation was other than respiratory (4.01 years old) and in 'non-whites' (4.86 years old). Age at diagnosis was the only factor associated with death in this population, with both Cox's univariate (RH: 19.4) and multivariate analyses (RH: 17.6; 95% CI: 4.8-64.1). The increased risk of dying in children diagnosed before 12 months of age found in our analysis indicates that they should receive differentiated and intensive multi-professional care.