Chest
Clinical InvestigationsCystic FibrosisFactors Influencing Outcomes in Cystic Fibrosis: A Center-Based Analysis
Section snippets
Materials and Methods
The analysis examined ESCF data collected during the 2-year period from 1995 through 1996. Initiated in December 1993, the study had enrolled 18,411 patients by December 1995,14 which was estimated to include > 80% of the population of CF patients in the United States and approximately 10% of that in Canada. The 194 sites included > 90% of those accredited by the US Cystic Fibrosis Foundation. All Canadian and US sites were invited to participate if they had > 10 patients. Patients were
Results
Of the 194 participating sites in the ESCF, 132 with 8,125 patients met the eligibility criteria for this analysis. For the 6 to 12-year-old age group, 112 sites with 3,361 patients (mean, 30 patients per site; SD, 19 patients per site) were eligible. For the adolescent age group (13 to 17 years of age), 84 sites with 1,734 patients (mean, 21 patients per site; SD, 11 patients per site) were eligible. For the adult group (≥ 18 years of age), 103 sites with 3,030 patients (mean, 29 patients per
Discussion
This analysis takes advantage of the large number of sites and patients reporting to the ESCF. It presents an opportunity to study outcomes and practices in a comprehensive way. The approach was to compare CF care sites based on an important index of lung health (ie, the percentage of predicted FEV1 values). Large differences in lung health across sites were demonstrated. The sites reporting the highest values for median FEV1 monitored patients, obtained measurements of lung function, and
References (25)
- et al.
Outcome measures for clinical trials in cystic fibrosis: summary of a cystic fibrosis foundation consensus conference
J Pediatr
(1994) - et al.
Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis
J Pediatr
(1997) - et al.
Risk of death in cystic fibrosis patients with severely compromised lung function
Chest
(1998) - et al.
Progression of cystic fibrosis lung disease as a function of serum immunoglobulin G levels: a 5-year longitudinal study
J Pediatr
(1984) - et al.
A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis: Cystic Fibrosis Foundation Prednisone Trial Group
J Pediatr
(1995) Cystic fibrosis of the pancreas and its relation to celiac disease
Am J Dis Child
(1938)1999 annual data report
(2000)- et al.
Determinants of mortality from cystic fibrosis in Canada, 1970–1989
Am J Epidemiol
(1996) Management of pulmonary disease in patients with cystic fibrosis
N Engl J Med
(1996)- et al.
Cystic fibrosis
Am J Respir Crit Care Med
(1996)
Cystic fibrosis foundation guidelines for patient services, evaluation, and monitoring in cystic fibrosis centers
Am J Dis Child
Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
N Engl J Med
Cited by (0)
Drs. Johnson and Butler are employees of Genentech. Drs. Konstan, Morgan, and Wohl are consultants for the Epidemiologic Study of Cystic Fibrosis sponsored by Genentech. All sources of support for the Epidemiologic Study of Cystic Fibrosis in the form of grants, case report forms, and data analysis were provided by Genentech.