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Cystic fibrosis is a secretory problem. Thick tenacious mucus is produced and hard to remove from the alveoli. If the mucous lines the alveolar wall, gas exchange is more difficult because diffusion is impaired. That's what happens, the further the gases have to travel to reach the blood stream (or the alveoli in the case of CO2), the slower and more difficult the diffusion.

2007-03-23 13:02:48 · answer #1 · answered by misoma5 7 · 0 0

Cystic fibrosis is loss of function mutation is a chloride channel. This channel normally allows the flow of the chloride ion from outside the cell into the cell. In people with CF, this allows the build up of chloride outside the cell, which then attracts sodium.

Exactly how this abnormal Sodium Chloride regulation effects lung function is unknown. The classical theory was that water is attracted by osmosis, leading to a build up of nutrient rich mucous. However, that theory does not quite make sense, as osmosis would draw out only water, you would get a build up fluid more like sweat than mucous. (Indeed, people suffering from CF have high salt concentrations in their sweat)

Either way, the end result of the mutation is a build up of thick mucous in the lungs. This not only blocks of small passage ways, but increases bactarial and fungal infections in the lungs, which eventually leads to scaring AKA fibrosis.

Also, the large volumes of mucous slow the diffusion of gas through the lungs, simply because the molecules have further to move before they get into the blood.

2007-03-23 20:03:48 · answer #2 · answered by Bill C 3 · 0 0

Yes, definitely, it killed my father.

2007-03-23 19:47:26 · answer #3 · answered by Anonymous · 2 1

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