[Cystic fibrosis lung disease and its management]

The function of the cystic fibrosis (CF) gene product, cystic fibrosis transmembrane conductance regulator (CFTR) is critical to normal lung defense. CFTR not only acts as a cAMP-dependent Cl-channel, but regulates other ion channels and several cellular functions. This article describes the current understanding of the clinical pathophysiology and the management of cystic fibrosis lung disease, particularly the CFTR-related bacterial colonization of the airways. Since chronic lung infection remains the major cause of early death, it is important to interrupt the cycle of infection and inflammation that leads to bronchiectasis and respiratory failure. Only a few cases have been reported in Japanese with no mutations including delta F508 identified. Those cases seem to be caused by unknown factors yet to be found.