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Prognostic aspects of cystic fibrosis in Brazil
P A Camargos1, M D Guimarães, F J Reis
1Department of Paediatrics, School of Medicine, Federal University of Minas Gerais, Avenida Alfredo Balena, 190/Sala 4061, 30130-100 Belo Horizonte, Brazil. pcamargs@medicina.ufmg.br
Insights
Early diagnosis of cystic fibrosis (CF) in children significantly impacts survival. Children diagnosed before 12 months face a higher mortality risk, necessitating intensive multidisciplinary care.
Area of Science:
- Pediatrics
- Pulmonology
- Genetics
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
- Prognostic factors in pediatric CF populations require ongoing investigation for improved patient outcomes.
Purpose of the Study:
- To identify prognostic factors influencing mortality in Brazilian children with cystic fibrosis.
- To assess the impact of age at diagnosis and clinical presentation on survival rates.
Main Methods:
- Retrospective cohort study of 111 Brazilian children diagnosed with CF between 1970 and 1994.
- Data collected via standardized protocol from medical charts, including demographics and clinical information.
- Cox's proportional hazard model used for univariate and multivariate risk analysis.
Main Results:
- Age at diagnosis was the sole significant prognostic factor for mortality (RH: 19.4 univariate, 17.6 multivariate).
- Children diagnosed before 12 months had a substantially increased risk of death.
- Mean age at death was lower for infants diagnosed early (3.10 years).
Conclusions:
- Early diagnosis of cystic fibrosis in children is critical for survival.
- Children diagnosed before 12 months require specialized, intensive multidisciplinary care.
- Age at diagnosis is a key predictor of mortality in pediatric CF.
Abstract:
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.