Improved survival of patients with cystic fibrosis.

The survival of 320 patients, who were born in and after 1958 with cystic fibrosis and managed by the Departments of Thoracic Medicine and Gastroenterology, Royal Children's Hospital, Melbourne, is reviewed. Eighty per cent of patients survived to 11 years of age, and 64% to 18 years. Of patients managed between 1973 and 1977, 91% survived to 12 years of age, and 80% to 17 years of age. In the same period, 79% of patients survived for 16 years after diagnosis. Forty-four per cent of the 240 patients currently being managed have no significant permanent lung disease, and only 9% have advanced disease.