help button home button
AJRCCM
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Stanke, F.
Right arrow Articles by Tümmler, B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Stanke, F.
Right arrow Articles by Tümmler, B.
American Journal of Respiratory and Critical Care Medicine Vol 173. pp. 1047, (2006)
© 2006 American Thoracic Society

Correspondence

Cystic Fibrosis Disease–specific Centiles in 2000 and 2005

To the Editor:

Kulich and colleagues recently reported disease-specific reference percentiles in a cystic fibrosis (CF) patient population for FEV1 (1), a clinical parameter that is frequently used to describe disease severity. This laudable approach to correct for the age-, height-, and sex-dependent decline of lung function in CF is appreciated. However, we were taken by surprise by Kulich and coworkers' claim that they "have developed the first disease-specific lung function reference equations" (1). Based on the 1995 Epidemiologic Registry of CF on more than 25,000 subjects, we derived CF-specific percentiles for lung function (2) to rank our cohort of European CF twins and siblings by disease severity. Subgroups of extreme phenotypes were investigated during the last 10 years for associations between clinical status and basic defect (3) or genetic modifiers (3–5). The impact of the careful selection of cohorts by CF-specific lung function percentiles on the information available from a clinical study was addressed in a letter to the AJRCCM (6). We fully support Kulich and colleagues' conclusion that the newly developed centiles for FEV1 (1) or our centiles for FEVPerc (2) should improve the generalizability of CF clinical trials, but we wonder how related work presented at numerous CF conferences in the United States and elsewhere could not be noticed by this distinguished Czech-American consortium of CF experts.

Frauke Stanke, Manfred Ballmann and Burkhard Tümmler

Medizinische Hochschule Hannover, Hannover, Germany

FOOTNOTES

Conflict of Interest Statement: F.S. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript. M.B. has been reimbursed by Hoffmann La Roche for attending several conferences, and by Chiron for several conferences. B.T. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript.

REFERENCES

  1. Kulich M, Rosenfeld M, Campbell J, Kronmal R, Gibson RL, Goss CH, Ramsey B. Disease-specific reference equations for lung function in patients with cystic fibrosis. Am J Respir Crit Care Med 2005;172:885–891.[Abstract/Free Full Text]
  2. Mekus F, Ballmann M, Bronsveld I, Bijman J, Veeze H, Tümmler B. Categories of deltaF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics. Twin Res 2000;3:277–293.[CrossRef][Medline]
  3. Bronsveld I, Mekus F, Bijman J, Ballmann M, de Jonge HR, Laabs U, Halley DJ, Ellemunter H, Mastella G, Thomas S, et al. Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings. J Clin Invest 2001;108:1705–1715.[CrossRef][Medline]
  4. Mekus F, Laabs U, Veeze H, Tümmler B. Genes in the vicinity of CFTR modulate the cystic fibrosis phenotype in highly concordant or discordant F508del homozygous sib pairs. Hum Genet 2003;112:1–11.[CrossRef][Medline]
  5. Stanke F, Becker T, Cuppens H, Kumar V, Cassiman JJ, Jansen S, Radojkovic D, Siebert B, Yarden J, Ussery DW, et al. The TNFalpha receptor TNFRSF1A and genes encoding the amiloride-sensitive sodium channel ENaC as modulators in cystic fibrosis. Hum Genet (In press)
  6. Mekus F, Tümmler B. Genes, environment, ion transport, and cystic fibrosis. Am J Respir Crit Care Med 2004;169:770.[Free Full Text]



This article has been cited by other articles:


Home page
 Eur Respir JHome page
G. de Laurentiis, D. Paris, D. Melck, M. Maniscalco, S. Marsico, G. Corso, A. Motta, and M. Sofia
Metabonomic analysis of exhaled breath condensate in adults by nuclear magnetic resonance spectroscopy
Eur. Respir. J., November 1, 2008; 32(5): 1175 - 1183.
[Abstract] [Full Text] [PDF]

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Stanke, F.
Right arrow Articles by Tümmler, B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Stanke, F.
Right arrow Articles by Tümmler, B.

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Proc. Am. Thorac. Soc. Am. J. Respir. Cell Mol. Biol.
Copyright © 2006 American Thoracic Society