Original ArticleCystic Fibrosis Birth Rates in Canada: A Decreasing Trend since the Onset of Genetic Testing
Section snippets
Data
A table of age-at-diagnosis by birth year for patients born and diagnosed between 1971 and 2000 was obtained from the CCFF Patient Data Registry. The registry collects data annually from 38 specialized CF clinics throughout Canada. Because of the single-payer government health care system in Canada, virtually all patients diagnosed with CF are seen in these specialized clinics. Canadian births were obtained from the federal demographics database, Canadian Vital Statistics, for the corresponding
Results
Between 1971 and 1987, there were no significant trends in the estimates of the CF birth rates obtained from the nonparametric Poisson model (Figure). Although estimated annual rates fluctuated considerably through the 1970s and 1980s, the overall CF birth rate obtained from the segmented linear Poisson model was 1/2714, with no increasing or decreasing trend. A decline in CF birth rates was observed from 1988 to 2000, yielding an estimated birth rate for year 2000 of 1/3608, a 25% decrease
Discussion
Determining an accurate and unbiased estimate of CF birth rates has been an issue ever since registries and large population surveys of the disorder became available. Gregg et al14 used data from the Wisconsin CF newborn screening program for the period of April 1985 to June 1991 to estimate the birth prevalence rate of CF. Newborns with serum immunoreactive trypsinogen levels ≥180 ng/mL on screening were then being diagnosed at 6 weeks of age by sweat chloride determinations after pilocarpine
References (23)
- et al.
First-trimester prenatal diagnosis of cystic fibrosis with linked DNA probes
Lancet
(1986) The changing epidemiology of cystic fibrosis
J Pediatr
(1993)- et al.
Cystic fibrosis
- et al.
Identification of the cystic fibrosis gene: chromosome walking and jumping
Science
(1989) - et al.
Identification of the cystic fibrosis gene: genetic analysis
Science
(1989) - Cystic Fibrosis Genetic Analysis Consortium (CFGAC). 2002. www.genet.sickkids.on.ca/cftr/. Accessed in April...
- et al.
Genetic determination of exocrine pancreatic function in cystic fibrosis
Am J Hum Genet
(1992) - et al.
Cystic fibrosis mutations in French Canadians: three CFTR mutations are relatively frequent in a Quebec population with an elevated incidence of cystic fibrosis
Am J Med Genet
(1992) - Canadian Cystic Fibrosis Foundation. Canadian Patient Data Registry National Report, 2000. Canadian Cystic Fibrosis...
- et al.
Cystic fibrosis: a worldwide analysis of CFTR mutations–correlation with incidence data and application to screening
Hum Mut
(2002)
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Supported by CCFF, CIHR, NSERC, Genome Canada.