Bronchiectasis & CF

Question	Answer
What is bronchiectasis in general?	Permanent, abnormal dilation and destruction of bronchial walls With chronic inflammation, airway collapse, and ciliary loss/dysfunction --> impaired clearance of secretions
Most common cause of bronchiectasis	CF (50%)
Other causes of bronchiectasis	- Recurrent infections - Primary ciliary dyskinesia (Kartagener's) - Autoimmune diseases - Humoral immunodeficiency - Airway obstruction
Clinical features of bronchiectasis (4)	- Chronic cough with foul-smelling, mucopurulent sputum - Dyspnea - Hemoptysis - Recurrent or persistent pneumonia
Diagnostic study of choice for bronchiectasis	High-res CT
Main goal in treating bronchiectasis	Preventing complications of pneumonia and hemoptysis
Treatment for bronchiectasis (2)	- Antibiotics for acute exacerbations - Bronchial hygiene: hydration, chest physiotherapy (postural drainage, chest percussion), inhaled bronchodilators
Mode of inheritance of CF	AR
Genetic defect in CF	Defective chloride channel protein Impaired chloride and water transport, leading to think, viscous secretions
Organs/systems affected by abnormal chloride channels in CF (5)	- Respiratory tract - Exocrine pancreas - Sweat glands - Intestines - GU tract
rhDNase	recombinant human deoxyribonuclease Inhaled medication that breaks down DNA in respiratory mucus that is clogging the airways

Resource summary

	Created by Aaron Samide over 9 years ago