Normal cystic fibrosis (CF) transmembrane regulator (CFTR) protein has multiple functions
in health and disease. Many mutations in the CFTR gene produce abnormal or absent
protein. CFTR protein dysfunction underlies the classic CF phenotype of progressive
pulmonary and GI pathology but may underlie diseases not usually associated with CF. This
review highlights selected extrapulmonary disease that may be associated with abnormal
CFTR. Increasing survival in CF is associated with increasing incidence of diseases …