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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

Annals of Tropical Paediatrics
|February 24, 2001
PubMed

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.

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