The decline and fall of pulmonary function in cystic fibrosis: new models, new lessons

PB Davis - The Journal of pediatrics, 1997 - jpeds.com
The explosion of knowledge about the basic defect and its consequences in cystic fibrosis
has raised hopes that new treatments, based on this information,~ 11 halt progression of
disease and increase life expectancy. Because lung disease accounts for most deaths
associated with CE the lung is the most important therapeutic target. It is therefore important
to assess the pulmonary disease in CF and its progression, to identify factors that modify the
course of the disease, to identify particular populations at risk for rapid decline, and to be …