BIO 115 Lecture Notes - Lecture 24: Cystic Fibrosis, Epithelial Sodium Channel, Sweat Gland

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7 Dec 2020
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1/3000 caucasians 1/17000 african americans: identify three methods of making the diagnosis of cystic fibrosis, sweat chloride test (pilocarpine iontophoresiss) cf patients should have salty sweat. 60 is the cut off. below 30 (infants) or 40 (adults) you"re good: genotyping. Describe the relationship of cftr dysfunction and development of abnormal sweat gland and lung function. Decreased cftr in the airway increases enac activity. Decreased cftr in sweat glands decreases enac activity. Hypothesized that the altered ion concentration (and ph) of airway secretions leads to impaired innate immunity with which leads to airway infection. Increased viscosity of mucus in the airways which is harder to clear and contributes to airway obstruction. Bronchiectasis is the serious airway pathological changes and can lead to respiratory failure and death in 85%-90% of affected individuals. Sinusitis and nasal polyposis affect the upper airways. Staph aureus and pseudomonas mainly cause chronic airway infection in cf.

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