Sandbox 49: Difference between revisions

[[Image:Cysticfibrosis01.jpg | thumb]]

One consequence of the autosomal recessive disease cystic fibrosis is a deficiency in transport of trypsin and other digestive enzymes from the pancreas. Cystic Fibrosis (CF) is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator. This gene is needed to control the amount of sweat, digestive juices, and mucous the body makes. CF is a common disease which affects the entire body, causing progressive disability and often early death. The name cystic fibrosis refers to the characteristic scarring or fibrosis and cyst formation within the pancreas. The most serious symptom is difficulty breathing caused by repetitive lung infections. A picture of a normal airway compared to an airway with cystic fibrosis can be seen to the right. The pancreas is a gland organ in the digestive and endocrine system of vertebrates. It is both an endocrine gland producing several important hormones, including insulin, glucagon, and somatostatin, as well as an exocrine gland, secreting pancreatic juice containing digestive enzymes that pass to the small intestine. This disease can lead to meconium ileus which involves an intestinal obstruction (ileus) due to thick meconium which is normally broken down by trypsins and other proteases, then passed in feces. Meconium is the earliest stool of an infant which is composed of materials ingested during the time the infant spends in the uterus such as intestinal epithelial cells, lanugo, mucus, amniotic fluid, bile, and water. Meconium is quite sterile and it is normally broken down by trypsin and other proteases, then passed in the feces.