Bronchiectasis and Cystic Fibrosis Flashcards
What is bronchiectasis?
permanent dilatation of bronchi and bronchioles due to destruction of airways muscles and elastic connective tissue
What are the different aetiologies of bronchiectasis?
postinfectious APBA COPD traction secondary to fibrosis aspiration obstruction immunological defects congenital defects inflammatory young's syndrome amyloidosis CF
What investigations should be ordered in all bronchiectasis patients?
CT chest sputum test for non tuberculosis mycobacterium IgE, aspergillus precipitins immunoglobulins 6MWT
What investigations should be considered to look for underlying cause of bronchiectasis?
CF genotyping TTE bronchoscopy immune tests (e.g. HIV) nasal brushings upper GI endoscopy
What are the virulence factors of pseudomonas?
pili, capsule, toxins, proteases, adhesins
What are the important organisms in bronchiectasis?
nontuberculous mycobacteria, aspergillus, stenotrophomonas maltophilia, staphylococcus aureus, pseudomonas
What are the principles of bronchiectasis treatment?
treat the cause
improve airway clearance (physio)
treat airway infection
treat airway obstruction (PRN SABA, consider ICA/LABA)
treat chronic inflammation (consider macrolides)
What are the benefits of macrolide treatment?
reduced exacerbation rate
improved QOL and sputum production
mild improvement in lung function
What are the potential risks of macrolide treatment?
increased resistance
increased non tuberculous mycobacteria infections
prolonged QT interval
What current therapies are available for cystic fibrosis?
CFTR modulators inhaled dnase macrolide antibiotics hypertonic saline lung transplantation
Why are male cystic fibrosis patients infertile?
absent vas deferens
