[Increased survival rates of children with cystic fibrosis].

Seventy-two patients with cystic fibrosis were under care between January 1st 1972 and December 31st 1988, and 75 until now (July 1989). Fifty are alive, 24 died and 1 was lost to follow-up. The number of alive patients under control at the end of every year rose from 5 in 1972 up to 47 in 1988 and 50 at present. In order to assess the progress of survival rates, we compared two periods: 1972-1980 and 1981-1988. Twenty-eight were under control during the first period and 16 (57%) died, these figures for the second period being 55 and 8 (14%) (p 0.001) respectively. A remarkable increase in cumulative survival rates during the second period was observed at ages 1 (96% vs 68%), 5 (94% vs 55%) and 10 (86% vs 28%). Greater experience and closer follow-up, together with more aggressive treatment are likely to account for the increase in survival. It is vital that reference cystic fibrosis centers should be set-up in our country for the management of so complex a disease as 'cystic fibrosis.