Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012 Oct;47(10):966-72.
doi: 10.1002/ppul.22519. Epub 2012 Feb 22.

Risk factors for onset of persistent respiratory symptoms in children with cystic fibrosis

Susanna A McColley  1 , Clement L Ren, Michael S Schechter, Warren E Regelmann, David J Pasta, Michael W Konstan; Epidemiologic Study of Cystic Fibrosis
Affiliations

Risk factors for onset of persistent respiratory symptoms in children with cystic fibrosis

Susanna A McColley et al. Pediatr Pulmonol. 2012 Oct.

Abstract

Objectives: To characterize the onset of persistent signs and symptoms of cystic fibrosis (CF) lung disease and identify characteristics that predict onset.

Study design: Patients in the Epidemiologic Study of CF who were <4 years of age at enrollment and had ≥2 years of follow-up were included. We defined persistence as a sign or symptom that was present during two consecutive encounters separated by 60-365 days, and persistent clubbing as ≥50% of encounters with clubbing within 365 days. Predictors were assessed in a Cox proportional hazards model for age at first occurrence of each symptom.

Results: Each sign or symptom met the criterion of persistence in a substantial proportion of patients during a follow-up period of 7 ± 3 years (mean ± SD; range 2-12). Risk factors that predicted earlier onset of signs and symptoms included pancreatic enzyme use, Pseudomonas aeruginosa infection, and prior diagnosis of asthma. Other risk factors had variable effects on signs and symptoms.

Conclusions: Signs and symptoms of lung disease begin early in CF. Risk factors previously reported for lower forced expiratory volume in 1 sec are also associated with earlier onset of persistent signs and symptoms of CF lung disease, but their impact varies.

PubMed Disclaimer

Conflict of interest statement

Disclosure of Conflict of Interest: S. McColley, C. Ren, M. Schechter, W. Regelmann, and M. Konstan have received honoraria from Genentech, Inc., for serving as members of the Scientific Advisory Group for the Epidemiologic Study of Cystic Fibrosis (ESCF). No compensation was provided to these authors in exchange for production of this manuscript. D. Pasta is an employee of ICON Clinical Research. ICON Clinical Research was paid by Genentech for providing biostatistical services for this study.

The decision to submit the manuscript was made by the authors and other members of Scientific Advisory Group and was approved by Genentech, Inc.

Figures

Fig. 1
Fig. 1
Cumulative percentage of patients reaching onset of persistent symptoms, by age 0 to 15, for cough, crackles, sputum, wheezing, and clubbing.
Fig. 2
Fig. 2
Hypothetical cumulative percentage of patients reaching onset of persistent symptoms according to risk factors, by age 0 to 15. Panel A: Daily cough by risk factors PA (Pseudomonas aeruginosa), asthma, Medicaid status, pancreatic enzymes. Panel B: Daily sputum by risk factors PA, asthma, Medicaid status, Site Quartile 1 (sites with best FEV1). Panel C: Wheezing by risk factors PA, asthma, Medicaid status, pancreatic enzymes. Panel D: Clubbing by risk factors PA, asthma, pancreatic enzymes, liver disease.
See this image and copyright information in PMC

References

    1. Linnane BM, Hall GL, Nolan G, Brennan S, Stick SM, Sly PD, Robertson CF, Robinson PJ, Franklin PJ, Turner SW, Ranganathan SC, AREST-CF Lung function in infants with cystic fibrosis diagnosed by newborn screening. Am J Respir Crit Care Med. 2008;178:1238–1244. - PubMed
    1. Long FR, Williams RS, Castile RG. Structural abnormalities in infants and young children with cystic fibrosis. J Pediatr. 2004;144:154–161. - PubMed
    1. Stick SM, Brennan S, Murray C, Douglas T, von Ungern-Sternberg B, Garratt LW, Gangell CL, De Klerk N, Linnane B, Ranganathan S, Robinson P, Robertson C, Sly PD, Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr. 2009;155:623–628. - PubMed
    1. Konstan MW, Butler SM, Wohl MEB, Stoddard M, Matousek R, Wagener JS, Johnson CA, Morgan WJ, Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr. 2003;142:624–630. - PubMed
    1. Morgan WJ, Butler SM, Johnson CA, Colin AA, FitzSimmons SC, Geller DE, Konstan MW, Light MJ, Rabin HR, Regelmann WE, Schidlow DV, Stokes DC, Wohl ME, Kaplowitz H, Wyatt MM, Stryker S. Epidemiologic Study of Cystic Fibrosis: design and implementation of a prospective, multicenter, observational study of patients with cystic fibrosis in the U.S. and Canada. Pediatr Pulmonol. 1999;28:231–241. - PubMed

Publication types

MeSH terms

Substances