This is a draft cheat sheet. It is a work in progress and is not finished yet.
• Autosomal recessive predominantly in white patients. Defect in chloride channel protein causes impaired chloride and water transport, which leads to excessively thick, vicious secretions in the respiratory tract, exocrine pancreas, sweat glands, GI, and GU tracts.
• Prognosis has improved with the median age of death now >30.
• Pancreatic enzyme replacement, fat-soluble vitamin supplements, chest PT, vaccinations (influenza and pneumococcal), antibiotics for infections, inhaled recombinant human deoxyribonuclease (rhDNAse) breaks does the DNA in respiratory mucus that clogs airways.
• Results in obstructive lung disease with chronic infections (frequently Pseudomonas), pancreatic insufficiency, and other GI complications.
| || |
Help Us Go Positive!
We offset our carbon usage with Ecologi. Click the link below to help us!