Pancreatitis and Mutations of the Cystic Fibrosis Gene

In patients with cystic fibrosis, the inability to maintain the luminal hydration of ducts that contain or secrete large molecules may affect multiple organs. The protein product of the gene that causes the disease — the cystic fibrosis transmembrane conductance regulator (CF TR) gene — functions as a cyclic AMP–regulated chloride channel.1 The exocrine pancreas and, in males, the wolffian ducts are most susceptible to the disease and are affected earliest. In both cases, high concentrations of macromolecules can block the narrow, tortuous, and lengthy ducts. Nevertheless, the severity of the disease varies considerably. This heterogeneity must be . . .