Cystic Fibrosis

• Abnormally thickened mucous due to chloride channel deletion of a phenylalanine residue - all tubes/mucous places have issues
• Dehydrated mucus that blocks lumen \(\rightarrow\) infections, inflammation and scarrinng (S. aureus in childhood, Pseudomonas aeruginosa)
• Bronchitis, bronchiectasis, pancreas - exocrine aatrophy and fibrosis, seminiferous tubules blockage 95% males infertile
• Chronic cough, lung infections malabsorption and eventual right sided heart failure