Growth in prepubertal children with cystic fibrosis, homozygous for the F508 mutation

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Subject: Growth in prepubertal children with cystic fibrosis, homozygous for

the F508 mutation

Journal of Cystic Fibrosis

Volume 2, Issue 2 , June 2003, Pages 76-83

Copyright 2003 European Cystic Fibrosis Society. Published by Elsevier

Science B. V.

Growth in prepubertal children with cystic fibrosis, homozygous for the

F508 mutation

Bigna M. Keller, Carmen Casaulta Aebischer, , Kraemer and

H. Schöni

Department of Pediatrics, University of Bern, 3011, Bern, Switzerland

Background and methods: In cystic fibrosis, growth and lung function have

been identified as prognostic markers of both severity of pulmonary

disease and survival. Cross-sectional studies in patients with cystic

fibrosis (CF) including all genotypes have shown that in prepubertal

patients with lifetime continuous care within a specialised CF centre,

growth can normalise. No corresponding improvement in lung function has

been found. We used a longitudinal design to determine whether

normalisation of growth could be found in the genetic subgroup of

prepubertal children with CF with the homozygous F508 mutation, which is

one of the known severe mutations. Methods: Data of all children born

after 1980 with the homozygous F508 mutation, diagnosed in early

childhood at the specialised centre of the Children's Hospital of Berne

were systematically assessed up to the age of 11 years and

retrospectively analysed. Follow-up data of height, weight and BMI were

compared to the Swiss reference population using z-scores. The

correlations between lung function parameters (FEV1, MEF50, VC) and age,

as well as lung function parameters and growth indices, were calculated.

Additionally, the same correlations were examined in a cohort with the

same mutation born 10 years earlier. Results: In the study, cohort growth

(height, weight and BMI) was significantly below that of the normal Swiss

population. A significant decline of lung function with age was also

found, however, no association between lung function and growth could be

seen. Compared to an earlier cohort, an improved growth over the last

decade could be shown but no improvement on lung function could be

detected. Lung function varied widely in both groups. Conclusion: In

contrast to sequential cross-sectional studies of children with CF, the

present longitudinal study of children with homozygous for the F508

mutation failed to confirm normalisation of growth over time. However,

compared to the data of children born in the previous decade, improved

growth was observed. Author Keywords: Height; Weight; Body mass index;

Pulmonary function

Corresponding author. Tel.: +41-31-632-9551