Sinonasal pathology in Cystic Fibrosis

This thesis describes the pathology of the mucosa of the nose and the paranasal sinuses in patients with Cystic Fibrosis (CF). CF is a genetic disorder with a high morbidity and mortality in relatively young patients. The genetic defect encodes for a defective Cystic Fibrosis Transmembrane conductance Regulator (CFTR) protein, a chloride channel present in many epithelial cells. As a consequence, the secretions of these epithelial cells are higher in viscosity compared to patients without CF. This leads to different pathology in different organs: lung infection and bronchiectasis, pancreatic insufficiency, intestinal obstruction, biliary cirrhosis and absence of the vas deferens. Often these disorders co-exist. Surprisingly, pathology of the upper airways in patients with CF has received little attention from researchers in the recent decades. This changed approximately 20 years ago with the gradual acknowledgement of the impact of infections of the nose and the paranasal sinuses on quality of life and on pulmonary pathology.
Over the last four decades the life expectancy of a patient with CF has increased enormously from approximately 10 to 40 years of age, due to the introduction of several innovative therapies. Improvements in life expectancy have allowed a shift in focus to quality of life factors and disorders impacting this.
One of the factors impacting quality of life in CF patients is sinonasal pathology. This thesis explores sinonasal pathology in cohorts of adults and children with CF. In chapter 2 to 4 different aspects of sinonasal disease in a group of 104 adult patients are described.