HTHSCI 1DT3 Lecture Notes - Lecture 6: Mucociliary Clearance, Idiopathy, Pancreatitis
13 Aug 2020
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Document Summary
Mod. 2-2 & 1-13, 14 cystic fibrosis (cf) 2. Cftr"s inhibition of enac is eliminated , resulting in increase na absorption. There are significant shunt pathways for cl- in airway, the increase na absorption drives increased absorption of cl- and water. In cf, airways display accelerated isotonic fluid absorption that depletes asl volume and dehydrates mucus, leading to obstruction and infection: staining of water content: With appropriate amount of water, the cilia stand up nice and straight to beat in coordinated fashion and clear pathogens and debris. Cf cftr: 3-4 um of water, there is insufficient air surface liquid cilia fall over and do not beat properly (unable to clear debris and pathogens: other hypothesis. Caucasian: risk of cf mutation (1 in 29). Risk of child with cf (1 in 3300) Asian: risk of cf mutation (1 in 90). Risk of child with cf (1 in 32,100: heterozygous advantage (from animal data)
