ABSTRACT
I. Introduction The gastrointestinal (GI) tract is affected in all patients with cystic fibrosis (CF), whether or not they are pancreatic sufficient (PS) or insufficient (PI). The incidence of the GI manifestations varies depending on the age of the patient, genotype, and presence of pancreatic involvement. Meconium ileus (MI) is the first manifestation of CF and can be detected in utero. The pancreas is affected in most patients. Gastroesophageal reflux (GER), distal intestinal obstruction syndrome (DIOS) or MI equivalent, constipation, small bowel bacterial overgrowth (SBBO), and pancreatic insufficiency are the most common manifestations seen. Animal models [mouse and piglet (1,2)] provide excellent modalities for looking at GI abnormalities, including DIOS, bacterial overgrowth and intestinal inflammation, MI, focal biliary cirrhosis, and pancreatic destruction with resulting PI. While all models do not necessarily translate well to CF-related disease in humans, they nonetheless pave the way for future exciting research to help elucidate the mechanisms for the GI manifestations of CF.
