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Predictors of deterioration of lung function in cystic fibrosis.

Schaedel, Charlotta LU ; de Monestrol, I; Hjelte, L; Johannesson, M; Kornfält, Ragnhild LU ; Lindblad, A; Strandvik, B; Wahlgren, Lars LU and Holmberg, Lars LU (2002) In Pediatric Pulmonology 33(6). p.483-491
Abstract
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... (More)
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. (Less)
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organization
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Contribution to journal
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published
subject
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
in
Pediatric Pulmonology
volume
33
issue
6
pages
483 - 491
publisher
John Wiley & Sons
external identifiers
  • pmid:12001283
  • wos:000175797600012
  • scopus:0036112237
ISSN
8755-6863
DOI
10.1002/ppul.10100
language
English
LU publication?
yes
id
c84dd260-b230-45f7-849c-d236d45300dc (old id 108420)
alternative location
http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12001283&dopt=Abstract
date added to LUP
2007-07-19 13:56:19
date last changed
2017-12-10 03:47:06
@article{c84dd260-b230-45f7-849c-d236d45300dc,
  abstract     = {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.},
  author       = {Schaedel, Charlotta and de Monestrol, I and Hjelte, L and Johannesson, M and Kornfält, Ragnhild and Lindblad, A and Strandvik, B and Wahlgren, Lars and Holmberg, Lars},
  issn         = {8755-6863},
  keyword      = {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},
  language     = {eng},
  number       = {6},
  pages        = {483--491},
  publisher    = {John Wiley & Sons},
  series       = {Pediatric Pulmonology},
  title        = {Predictors of deterioration of lung function in cystic fibrosis.},
  url          = {http://dx.doi.org/10.1002/ppul.10100},
  volume       = {33},
  year         = {2002},
}