Bronchiectasis

Question 1

What is bronchiectasis?

Answer 1

Permanent dilation of the airways caused by chronic inflammation and inability to clear secretions.

Question 2

Describe the pathophysiology of bronchiectasis.

Answer 2

Dependent on cause. Initially, there is infection of the smaller distal airways that results in inflammation and the release of inflammatory mediators. This impairs ciliary action, allows for bacterial proliferation and tissue damage and causes bronchial dilation.

Question 3

What are the causative organisms of bronchiectasis?

Answer 3

Streptococcus pneumoniae.
Haemophilus influenzae.
Staphylococcus aureus.
Pseudomonas aeruginosa: common in patients with cystic fibrosis.

Question 4

What are the congenital causes of bronchiectasis?

Answer 4

Cystic fibrosis.
Young’s syndrome: associated with azoospermia.
Kartagener’s syndrome: causes cilia to become immobile, removing the defence mechanism of the respiratory tract; also associated with situs inversus.

Question 5

What are the acquired causes of bronchiectasis?

Answer 5

Tumours.
Rheumatoid arthritis.
Inflammatory bowel disease.

Question 6

Give a complication of bronchiectasis.

Answer 6

Massive haemoptysis- medical emergency.

Question 7

How is bronchiectasis treated?

Answer 7

Antibiotics
Bronchodilators
Corticosteroids
Postural drainage
Surgery (if indicated)

Question 8

What are the symptoms of bronchiectasis?

Answer 8

Purulent sputum
Persistent cough
Fever

Question 9

What are the signs of bronchiectasis?

Answer 9

Clubbing
Crepitations
Coarse inspiratory crackles

Question 10

What investigations should be performed for suspected bronchiectasis?

Answer 10

Bloods: FBC, WCC, U&Es, LFTs, TFTs, CRP, ESR.
CXR: shows tram track opacities of bronchi and bronchioles.
Sputum culture and sensitivity.
Aspergillus screen if cause suspected.

Question 11

What is cystic fibrosis?

Answer 11

Autosomal recessive condition occurs in 1/2500 births.

Question 12

What causes cystic fibrosis?

Answer 12

Mutation of cystic fibrosis transmembrane conductance regulator gene (CFTR) on chromosome 7.

Question 13

How is cystic fibrosis diagnosed?

Answer 13

Sweat test. In neonatal period, diagnosed by Guthrie’s test, which detects rated serum immunoreactive trypsinogen.

Question 14

What other conditions is cystic fibrosis associated with?

Answer 14

Lung disease
Pancreatic insufficiency
Diabetes
Infertility in males