Prediction of Mortality in Patients with Cystic Fibrosis

DURING the past three decades the life expectancy of patients with cystic fibrosis has improved dramatically, but the majority still die in early adulthood from respiratory failure associated with pulmonary hypertension and cor pulmonale. 1 In patients with this disease, it is important that the prognosis be assessed so that the physician may intensify medical therapy, consider new approaches to treatment, or refer the patient for lung transplantation. At present, double-lung or heart-lung transplantation is the only definitive treatment for patients with advanced cystic fibrosis. The results of lung transplantation in adults 2 and children 3 with this disorder have been encouraging in . . .