Therefore, SLC26A9 is an attractive target not only for the treatment of CF lung disease, but also for systemic therapy that may be beneficial for the prevention and/or treatment of several extrapulmonary disease manifestations including meconium ileus and distal intestinal obstruction syndrome (DIOS), exocrine pancreatic insufficiency and CFRD. Here, SLC26A9 is linked to exocrine pancreatic insufficiency.