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Cystic Fibrosis | Animation Videos | Medical Pathology Stu

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This Cystic fibrosis animation tells a hereditary disease affecting lungs and the digestive system. The body produces thick sticky mucus clogging the lungs and obstructing the pancreas. This sqadia.com sqatoon briefly explains cystic fibrosis pathophysiology and how is cystic fibrosis inherited.

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It requires two diseased alleles for the individual to be a patient of cystic fibrosis. Cilia cannot clear up the microbes. This then leads to the build of more mucus in the airway. The airway cells contain an ion channel called cystic fibrosis transmembrane conductance regulator (CFTR). Normally, this is responsible for the movement of chloride ions out of the cells into the mucus. However, in case of mutated CFTR, the chloride ions are unable to move outside the cell hence the concentration of chloride ions inside the cell increases.

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This mutated CFTR is also responsible for the increased activity of other ion channel called epithelial sodium channel ENaC. This CFTR gene is also found in digestive ducts where stomach and small intestine connect allowing bile and enzymes from pancreas to enter small intestine to digest food.

However, in case of mutated CFTR the entry of chloride ion into the cell is blocked causing increased ions in sweat and hence, salty sweat. This is also another very important symptom of cystic fibrosis cystic fibrosis. To treat the symptoms and provide a relief, one can use antibiotics, mucolytics and bronchodilators as well. This would help in slowing the progression of disease.
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