my personal edition > cystic fibrosis > news


  E-Mail this DGReview to a colleague

DGReview

Long Term Survival in Cystic Fibrosis Patients Not Improved by Neonatal Screening

While cystic fibrosis is recognised very early by neonatal screening, compared with diagnosis by symptoms in early infancy such programmes do not necessarily improve long-term survival.

In a population-based study, researchers at the Cystic Fibrosis Center, Verona, Italy, analysed data on 593 cystic fibrosis patients living in Veneto and Trentino in north-western Italy where neonatal screening began in 1973.

Since the early 1980s it has been virtually universal in this region, which has a population of five million. During the study period, there was an estimated incidence of the disease of 1/2,650 live births per year.

All the patients, who were followed by a single referral centre, were born in the years 1938 to 2000. After screening, the median time from birth to confirmation of diagnosis was 32 days, with a range of 0-1,531.

Median age at diagnosis for subjects in whom cystic fibrosis was recognised after the occurrence of symptoms was invariably less than one year.

Median age for survival was found to be 37.7 years.

The method of diagnosis, either screened or unscreened, did not significantly influence long-term survival to age 20-30 years. Neither did gender or age at diagnosis for unscreened patients (<1, 1-5, >5 years).

In the last years of the study, improving survival was shown to vanish in current survival analysis of three consecutive decades.

The investigators conclude there could be no inference that a regional neonatal screening programme improved "long term survival if compared with diagnosis by symptoms in early infancy."
Am J Epidemiol 2002 Sep 1;156(5):397-401 "Epidemiology and survival analysis of cystic fibrosis in an area of intense neonatal screening over 30 years."

E-Mail this DGReview to a colleague

To print, use this version