S8.3 Bronchiectasis & Cystic Fibrosis

Question 1

What is bronchiectasis?

Answer 1

Chronic dilatation of one or more bronchi.

The bronchi exhibit poor mucus clearance

Question 2

What are the clinical features and causes of bronchiectasis?

Answer 2

Clinical features – chronic cough, daily sputum production, breathless, chest pain.
Causes: Post infective (whooping cough, TB), Immune deficiency, Mucociliary clearance defects (Cystic fibrosis), obstruction, Young’s syndrome.

Question 3

What is cystic fibrosis?

Answer 3

Autosomal recessive disorder affecting exocrine glands causing thick mucus production.

Question 4

Outline the pathophysiology of cystic fibrosis

Answer 4

Long arm chromosome 7 defect causes a CFTR mutation.
This reduces Cl transport across memb so water does not leave epithelium to hydrate mucus causing thick mucus production.
This can block ducts: sperm canal, pancreatic duct, sweat glands release Cl and Na.

Question 5

What are the clinical features and management of cystic fibrosis?

Answer 5

Clinical features – phlegmy cough, wheezing, weight loss, infertility
Management – physio to maintain lung health, avoid smoking and CF patients.