Cystic fibrosis: A decade of progress

Cystic fibrosis (CF) is an autosomal recessive, multisystem disorder which greatly reduces the lifespan of its victims, largely by gradual, infection- driven destruction of the lungs. Advances in therapy to date have improved median survival from less than 10 years to over 30 years of age. New scient ific advances in the past 10 years, notably discovery of the CF gene and in creasing understanding of its protein product, hold forth hope of even furt her therapeutic advances. This review will highlight the clinical character istics of CF, current standard therapies and directions of ongoing research . (C) 1999 Prous Science. All rights reserved.