Bronchiectasis

Question 1

What is the definition of bronchiectasis?

Answer 1

-permanent abnormal irreversible dilation of the bronchial tree leading to excess mucous production

Question 2

What are the clinical features of bronchiectasis?

Answer 2

• chronic productive cough ++++ purulent mucous
• dyspnoea
• mild haemoptysis
• recurrent infective exacerbations

Question 3

What is the bug that causes frequent infective exacerbations of bronchiectasis?

Answer 3

p. aeruginosa

Question 4

Tests for bronchiectasis?

Answer 4

• CXR
• high resolution CT to confirm
• bloods - genetic testing for CF, IgA definciency
• FEV/FVC ratio decreased (obstructive pattern)

Question 5

Management of bronchiectasis?

Answer 5

• smoking cessation
• chest physio + hygiene
• Abx for infective exacerbation
• vaccinations
• surgery (lobectomy if bleeding/unilateral bad bad)

Question 6

Define cystic fibrosis.

Answer 6

an autosomal recessive genetic disorder leading to a mutation in the CFRT gene, leading to a dysfunctional cystic fibrosis transmembrane conductance regular protein. This leads to thicker secretions throughout the body.

Question 7

What are the systems CF can affect? and therefore the symptoms it presents with?

Answer 7

-GI tract - pancreatic insufficiency
=meconium ileus 
=failure to thrive
=malabsorption 
=steatorrhea
= CF related diabetes 

-Resp tract = thick secretions:
=COPD w/ bronchiectasis
=chronic productive cough
=recurrent infective exacerbation (p. aeruginosa adults, s. aureus kids)

-GU
=men infertile - azospermia
=women reduced fertility - thick vaginal mucous or amenorrhea

Question 8

What is the mainstay of investigation for CF? any other tests you might do?

Answer 8

• Chlorine sweat test >60mmol/L
• likely picked up on heel stick new born screen
• can be tested for prenatally - amniocentesis or chorionic villus sampling
• genetics can be run
• stool testing = increased faecal elastase
• CT chest in resp disease

Question 9

How do you manage a patient with CF?

Answer 9

• BD chest physio (teach parents)
• dornase alpha mucolytic
• high fat, high calorie diet
• vitamin supplement (ADEK)
• pancreatic enzyme supplement taken with each meal
• lung transplant in chronic burkholderia infection

Question 10

What mucolytic agent would you offer a CF patient?

Answer 10

• rhDNase first line

- inhaled manitol powder second line

Question 11

what other rogue drug is there for CF? when can it be given?

Answer 11

okrambi

• increases CFTR transported into cell surface membrane
• only given in patients with homozygous F508 gene mutations