7.1 CF & Bronchiectasis Flashcards
define bronchiectasis
chronic irreversible dilatation of 1 or more bronchi
underlying mechanism of bronchiectasis
chronic inflammation causing destruction of elastic and muscular parts of bronchial walls, and perichondria fibrosis
classic CXW sign of bronchiectasis
tram track (thickens bronchi walls)
gold standard diagnostic test for bronchiectasis
-what is shown?
high resolution CT
shows bronchial dilation bigger than adjacent pulmonary artery (signet ring sign)
symptoms of bronchiectasis
-chronic cough
-sputum
-fatigue
-some haemoptysis
-breathless on exertion
signs of bronchiectasis
-hypoxaemia if severe
-haemoptysis
-fine crackles
-inspiratory squeaks
-fever
-rhonchi
2 organisms that can cause bronchiectasis and COPD
haemophilus influenzae
mortadella catarrhalis
difference in auscultation of COPD vs bronchiectasis
COPD
-diminshed breath sounds
-no inspiratory squeaks
define exacerbation of bronchiectasis
3 deteriorations of
-cough
-sputum volume
-blood sputum
-fatigue
-fever
for 48 hours
most common cause of bronchiectasis
CF
explain the mechanism of CF
-mutation of CFTR gene
-less Cl transported out of cells into airway, so less water follows
-produces thick and sticky mucous in airways
-impairs mucociliary clearance
-also less Na+ brought into cells via less regulation of ENaC
most common mutation in CF
Phe508del
intestinal malabsorption in CF can cause DM- how?
CF could affect the endocrine function of the pancreas
why are some people only diagnosed with CF in their 20s?
could have residual CFTR function e.g. mutation different, or heterozygous mutation
when to suspect atypical CF
-recurrent idiopathic pancreatitis
-recurrent sinusitis/lung infections
-infertility
list some complications of CF
-respiratory failure
-haemoptysis
-DM
-distal intestinal obstruction syndrome
-chronic liver disease
-gallstones
-HF
-arthritis (malabsorption of Ca2+)
why do CF patients need to avoid each other?
may be colonised by different strains of pseudomonas aeruginosa so could spread antibiotic resistance
why do CF patients need NaCl tablets in hot wetaher/exercise?
lose excess NaCl in their sweat
key way to get rid of mucous in CF patients?
saline
why might CF patients need a heart transplant?
cure cor pulmonate caused by R heart strain from years of pulmonary HTN
