what is cystic fibrosis?
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a hereditary disorder affecting the exocrine glands. It causes the production of abnormally thick mucus, leading to the blockage of the pancreatic ducts, intestines, and bronchi and often resulting in respiratory infection.
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Cystic fibrosis- It is an autosomal recessive disorder. It caused due to a mutation in a gene on chromosome 7. The gene codes for chloride channel which allows diffusion of chloride ions into and out of epithelial cells andis located in the cell surface membranes of these cells. In the sufferers of this disease, this chloride channel does not function.One of the normal function of theepithelial cells is to form mucus glands which secrete mucus. In cystic fibrosis patients this mucus becomes abnormally thick and sticky because the normal outward flow of chloride ions is prevented. The parts of the body that is most affected are the lungs pancreas and liver. In the pancreas, fibrous patches called cysts develop which give the disease its name. The thick mucus clogs up the airway of lungs, and the branches of the pancreatic duct and the bile duct from the liver into the gut. Repeated lung infections are caused, frequent digestive problems, including poor release of pancreatic enzymes and poor absorption of digested food. Males and females are mostly infertile because of this disease.
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