Study objectives: To compare international trends in
mortality from cystic fibrosis.
Design: Comparison of
trends in median age at death using national mortality data.
Setting: Data from 10 countries in North America, Europe,
Participants: All persons registered
as having died of cystic fibrosis in specified years from 1980 to
Interventions: Comparison of relative odds of
death at the international median age at death for the year of death
between countries for two periods of time; from 1980 to 1987 (10
countries) and from 1980 to 1994 (7 countries).
and results: The international median age at death increased from
8 years in 1974 to 21 years in 1994. Median age at death also increased
within all countries, was consistently highest in the United States,
and varied significantly by a factor of > twofold between countries.
Women were significantly more likely to die at a younger
age than the median age at death than men.
Conclusions: Median age at death from cystic fibrosis is
increasing, but our findings imply that clinically significant
differences in survival with cystic fibrosis persist between