Cystic fibrosis (CF) is an autosomal recessive genetic disorder that mostly affects the lungs, but also the pancreas, liver, kidneys, and GI tract.

CF typically leads to frequent lung infections, sinus infections, poor growth, fatty stool, and infertility in males due to congenital absence of the vas deferens.

There is no cure. CF is managed with pulmonary rehabilitation, antibiotics, mucolytics (drugs that loosen and thin mucus) and pancreatic enzyme replacement; lung transplantation may be considered. Ivacaftor may also be considered.

NICE Guideline (NG78) - Cystic fibrosis: Diagnosis and management

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