Cystic fibrosis mortality: registry data of cystic fibrosis.
BACKGROUND
The Cystic Fibrosis Foundation has been collecting mortality and other data on cystic fibrosis patients from over 100 U.S. clinical centers since 1966, and publishes annual reports of the data. Cystic fibrosis is an important autosomal recessive genetic disease, affecting multiple body systems, with disability and early mortality as very common complications.


RESULTS
Observed data by age in the 1994 and 1995 REports are compared with the earliest Registry experience. 1966-1972, after age-matching observed with expected mortality in the U.S. population. Observed and expected results are given in four tables of comparative mortality.


CONCLUSIONS
Excess mortality as a rate difference has shown a marked decrease during this time period, ranging from 96% at age 0-1 to 52% at ages 20-25 years. This can be attributed to improvements in medical treatment.
Singer
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RB
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000401:mortality*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007352:Insurance, Life; D015993:Life Tables; D008297:Male; D012042:Registries; D014481:United States / Q000453:epidemiology