Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis☆,☆☆,★
Section snippets
Patients
The study cohort was retrospectively assembled from the CF database at the Hospital for Sick Children in Toronto.9 All patients who were born in the years 1960 to 1974, who had at least two recorded pulmonary function tests, and whose first test was performed before the age of 10 years were included. The HSC clinic began in 1960, and routine pulmonary function testing began in 1967. Nineteen seventy-four was selected as the cutoff birth year to allow at least 10 years of follow-up from the age
Results
There were 553 patients identified in the CF database who were born between 1960 and 1974, of whom 366 satisfied the study criteria. Forty-six patients died before the age of 10 years and without pulmonary function follow-up. Another 40 patients in the cohort were lacking pulmonary function records, either because they transferred out of the clinic before pulmonary function testing was initiated or because HSC was not the site of their regular follow-up. The remaining 101 patients did not have
Discussion
This analysis has quantified the differences in rate of pulmonary function decline in patients who died at a young age compared with those who died later. All but the most severely affected patients, the relatively small proportion who died before age 15, appeared to have normal pulmonary function when first tested in early childhood. This suggests that in future studies of young patients with normal pulmonary function, it is highly desirable to consider other genetic factors, such as
Acknowledgements
We thank Ken Oliver, MD, for his review and useful comments on the manuscript.
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Supported by research grants from the Canadian Cystic Fibrosis Foundation and the Medical Research Council of Canada.
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Reprint requests: Mary Corey, PhD, Division of Gastroenterology and Nutrition, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario, M5G 1X8 Canada.
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