Cystic fibrosis: conservative management

Abstract

Cystic fibrosis (CF) is the most frequent, lethal, autosomic recessive hereditary disorder in caucasian populations. It is caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene whose product functions as a cAMP-regulated chloride channel [1–6]. Many organs are involved, but the lung represents the major target, and the pulmonary manifestations are the most common life-threatening aspects of the disease. Mutations of the CFTR gene lead to altered CFTR function in the airway epithelium causing production of abnormally thick secretions. Mucous obstruction of small airways is associated with development of chronic inflammation and infection. Progressively the airways are irreversibly damaged and bronchiectatic cysts develop. The bronchioles become stenosed and obliterated, and the pathological processes extend to the peribronchiolar alveolar structures causing fibrosis and alveolar destruction.