Cystic fibrosis

Question 1

What is Cystic fibrosis?

Answer 1

It is a hereditary disease from the exocrine glands characterised by increased secretion production

Question 2

Pathophysiology of CF?

Answer 2

Increased electrolyte content in sweat (Na+ and Cl-)
Increasing obstruction in excorine ducts from secretions
Increased secretion retention in lung leading to recurrent infections, fibrosis and scarring
Malabsorption of nutrients

Question 3

Etiology of CF?

Answer 3

An autosomal recessive gene that affects exocrine glands, diagnosed in childhood

Question 4

CF clinical Presentation?

Answer 4

Genetic testing (Cl- sweat test)
1. Inspection: low weight, possible osteoporosis, barrel chest, chronic cough, copious mucus (often with blood), increased RR, clubbing
2. Palpation: increased tactile frem over secretion areas (CF can be obstructive, restrictive or both)
3. Percussion: dull over secretions (CF can be obstructive, restrictive or both)
4. Auscultation: decreased BS, insp and exp crackles, wheezing

Question 5

What is special about CF mucus and sputum?

Answer 5

Often, there is blood in the mucus or sputum, and it can be green from bacterial infection, usually yellow in colour