Cystic Fibrosis

Question 1

In CF patients, mucus in the respiratory tract is _________, often leading to ____________ _________.

Answer 1

1. Viscous
2. Respiratory
3. Infections
   (Mucus cant be easily moved to the oropharynx to swallow)

Question 2

The most common mutation in CF leads to what structural change in CFTR?

Answer 2

Misfolding.

Question 3

How could the mucous of a CF patient be described?

Answer 3

Viscous

Question 4

What does viscous mucous in CF lead to?

Answer 4

• Pulmonary infection
• Constipation
• Meconium ileus in newborns

Question 5

How is sweat affected in CF patients?

Answer 5

Less Cl- reabsorbed into sweat gland, so sweat is rich in NaCl…‘salty skin’.

Question 6

Name 4 parts of the body where CF manifests.

Answer 6

1. Respiratory tract
2. Gastrointestinal tract
3. Sweat glands of skin
4. Pancreas

Question 7

How is the pancreas affected in CF patients?

Answer 7

• Thickened exocrine secretions blocks ducts, leading to less digestive enzymes reaching gut–> malabsorption and diarrhoea (undigested fat)
• Can cause pancreatitis and become fibrotic