Exercise testing in cystic fibrosis

Abstract

Cystic fibrosis (CF) is the most frequent genetic disease in the Caucasian population. It is characterized by absent or incorrect function of the channel that regulates the chloride exchange at cell surface (CTRF). The lungs are particularly involved as the very thick and tenacious mucus leads to progressive airflow limitation, respiratory infections, bronchiectasis, lung destruction, and ultimately respiratory failure. Dyspnea and exercise intolerance are the hallmarks of the diseases. Patients, however, should be encouraged to exercise regularly since early childhood because it promotes expectoration and contributes to good nutritional status and overall level of fitness. In addition, there are no contraindications to agonist sports. It follows that a growing number of CF patients will be referred to exercise-based evaluations in the forthcoming years. Cardiopulmonary exercise testing (CPET), in particular, is useful to determine the mechanisms of exercise intolerance in individual patients which may have treatment and prognostic implications.