Chronic lung disease is the major cause of morbidity and mortality among patients suffering from Cystic Fibrosis (CF), the most common autosomal recessive disease in Caucasian population. Mutations in the CF transmembrane conductance regulator (CFTR) gene disrupt a cAMP-regulated chloride channel - the CFTR protein - which is mainly present at the apical plasma membrane of epithelial cells lining organs such as the sweat glands, pancreas and airways. Although impressive progress in the elucidation of the genetic and molecular basis of CF has been done, no effective therapy exists to control progressive lung deterioration in CF patients. The lung disease in CF is characterized by thick mucus, recurrent airway inflammation and chronic bacterial infections, which result in airway narrowing that ultimately lead to fatal respiratory failure. Since airflow obstruction is a component of CFâ lung disease, as it is in asthma and COPD, these diseases are also categorized as obstructive lung diseases, in opposition to restrictive lung diseases. In these obstructive diseases, a vicious cycle of repeated airway inflammation is associated with structural tissue âremodelingâ inappropriate to the maintenance of normal lung function, suggesting that similar molecular pathways may be involved in the pathogenesis of these diseases. Environmental factors and modifier genes other than CFTR genotype determine progression of lung disease in CF.
Journal of Pulmonary & Respiratory Medicine welcomes articles related to "lung disease"
Last date updated on July, 2014