Pathology - Cystic Fibrosis ( CF)
Pathophysiology The CFTR gene has an autosomal recessive mutation that causes this multisystem genetic disease. CFTR, a Cl– channel that controls the release of water and salt, is expressed in a number of epithelia throughout the body. Reduced fluid secretion due to mutations in this gene causes viscous mucus to build up in the GI tract, reproductive system, pancreatic ducts, and lungs. This dense mucus in the lungs works to block the medium- and large-sized airways' ability to breathe, leading to wheezing and rhonchi as well as other frequent obstructive lung disease aftereffects. One of the architectural lung abnormalities associated with cystic fibrosis (CF) is bronchiectasis, which can lead to pulmonary vascular exposure and hemoptysis. Hypoxemia and recurrent lung Infections with pseudomonas aeruginosa are also frequently observed in this illness. Malabsorption and stunted growth may be caused by exocrine pancreatic and gastrointestinal tract dysfunction. Male infertility results from a vas deferens defect that is congenital. Clubbing of the fingernails is a typical observation in this and other chronic hypoxemic lung disorders, however its exact cause is unknown.
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