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Predictors of deterioration of lung function in cystic fibrosis.

På svenska

Author

  • Charlotta Schaedel
  • I de Monestrol
  • L Hjelte
  • M Johannesson
  • Ragnhild Kornfält
  • A Lindblad
  • B Strandvik
  • Lars Wahlgren
  • Lars Holmberg

Summary, in English

The severity of lung disease in cystic fibrosis (CF) may be related to the type of mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, and to environmental and immunological factors. Since pulmonary disease is the main determinant of morbidity and mortality in CF, it is important to identify factors that can explain and predict this variation. The aim of this longitudinal study of the whole Swedish CF population over age 7 years was to correlate genetic and clinical data with the rate of decline in pulmonary function. The statistical analysis was performed using the mixed model regression method, supplemented with calculation of relative risks for severe lung disease in age cohorts.The severity of pulmonary disease was to some extent predicted by CFTR genotype. Furthermore, the present investigation is the first long-term study showing a significantly more rapid deterioration of lung function in patients with concomitant diabetes mellitus. Besides diabetes mellitus, pancreatic insufficiency and chronic Pseudomonas colonization were found to be negative predictors of pulmonary function. In contrast to several other reports, we found no significant differences in lung function between genders. Patients with pancreatic sufficiency have no or only a slight decline of lung function with age once treatment is started, but an early diagnosis in this group is desirable.

Publishing year

2002

Language

English

Pages

483-491

Publication/Series

Pediatric Pulmonology

Volume

33

Issue

6

Document type

Journal article

Publisher

John Wiley & Sons Inc.

Topic

  • Pediatrics

Keywords

  • Cystic Fibrosis : physiopathology
  • Cystic Fibrosis : surgery
  • Cystic Fibrosis Transmembrane Conductance Regulator : genetics
  • Disease Progression
  • Female
  • Human
  • Longitudinal Studies
  • Lung : physiopathology
  • Male
  • Lung Transplantation
  • Mutation
  • Predictive Value of Tests
  • Regression Analysis
  • Support
  • Risk Factors
  • Non-U.S. Gov't
  • Cystic Fibrosis : microbiology
  • Preschool
  • Child
  • Alleles
  • Adult
  • Adolescence

Status

Published

ISBN/ISSN/Other

  • ISSN: 8755-6863