Cause of thick pancreatic secretions in cystic fibrosis?
Overproduction of mucin
Failure to clear mucin due to epithelial dysfunction
Defect in chloride channel leading to water reabsoption
Defect in sodium channel leading to water reabsorption
CF occurs because of mutations in the gene that makes a protein called CFTR (cystic fibrosis transmembrane regulator). A person with CF produces abnormal CFTR protein -- or no CFTR protein at all, which causes the body to make thick, sticky mucus instead of the thin, watery kind. This blockage causes a drop in the amount of digestive enzymes the pancreas puts out. Because of this, a child with CF has trouble absorbing fats, some proteins, and fat-soluble vitamins A, D, E, and K. The problems with the pancreas can get very severe. Some of the hormone cells in the pancreas can be destroyed Ref Robbins 9/e pg 178
Genetics