12c- Bronchiectasis and Cystic Fibrosis

Question 1

What is bronchiectasis?

Answer 1

Chronic dilation of 1+ bronchi

• Bronchi have poor mucus clearance
• Predisposition to bacterial infection

Question 2

What are the causes of bronchiectasis?

Answer 2

Question 3

Patients with bronchiectasis are more susceptible to infections. Which organisms are likely to cause infections in patients with bronchiectasis?

Answer 3

• Bacteria
  
  • Haemophilus influenzae
  • Pseudomonas aeruginosa
  • Moraxella catarrhalis
  • Staph aureus (CF)
• Fungi
  
  • Aspergillus
  • Candida

Question 4

What are the symptoms of bronchiectasis?

Answer 4

(common to less common)

• Chronic cough
• Daily sputum production
• Breathlessness on exertion
• Intermittent haemoptysis
  
  • coughing up blood
• Nasal symptoms
• Chest pain
• Fatigue
• Wheeze

Question 5

What are the signs of bronchiectasis?

Answer 5

• Hypoxaemia on Pulse Oximetry
• Fever
• Fine crackles
• High pitched inspiratory squeaks
• History of weight loss

Question 6

How would you investigate a patient with suspected bronchiectasis?

Answer 6

• Lung function test eg spirometry
• CT scan

Question 7

How is bronchiectasis managed?

Answer 7

1. Treat underlying cause
2. Physiotherapy- mucus clearance
3. Antibiotics - do sputum culture
4. Supportive- flu vaccine, bronchodilators

Question 8

What is the Signet Ring sign?

Answer 8

Question 9

What is cystic fibrosis? Explain how it’s caused.

Answer 9

Autosomal recessive

Mutation: CFTR Cystic Fibrosis Transmembrane Conductance Regulator

-thickened secretions

Question 10

Cystic fibrosis is usually diagnosed prenatally (CF known in family/ pre-natal screening). What features might be looked at post natally to confirm a diagnosis of cystic fibrosis?

Answer 10

Question 11

What other symptoms might CF patients present with (ie in infancy which may lead to a diagnosis of cystic fibrosis)? (3)

Answer 11

1. Recurrent chest infections
2. Meconium ileus
   
   1. Blockage in small intestine- meconium= stickier than usual
      
      1. Signs: bilious vomiting, abdominal distension, delay passing meconium
3. Intestinal malabsorption (>90% of CF patients)
   
   1. Deficiency in pancreatic enzymes (thick mucous blocks ducts)

Question 12

Increased chance of respiratory infections and low birth weight are complications caused by cystic fibrosis. How are each of these complications manage?

Answer 12

Question 13

What lifestyle advice should be given to patients living with cystic fibrosis?

Answer 13

• No smoking
• Avoid people with infections
• Avoid jacuzzies (pseudomonas)
• Avoid stables, compost etc- aspergillus fumigatus
• Annual influenza immunisation
• Sodium chloride tablets in hot weather

Question 14

Why is it not uncommon for people to be diagnosed with cystic fibrosis later in life?

Answer 14

Question 15

Why are patients with cystic fibrosis encouraged to not interact with one another?

Answer 15

Pseuomonas- resistant to many antibiotics

To avoid further infection by different strains of pseudomonas

Question 16

Cystic fibrosis patients can also suffere from CF related diabetes and Distal Intestinal Obstruction Syndrome (DIOS). Outline how DIOS is caused and how it will present.

Answer 16

• Caused:
  
  • Thick, dehydrated faeces in distal ileum and proximal colon
    
    • Insufficient pancreatic enzymes, salt deficiency/hot weather
• Presentation:
  
  • Palpable mass in right iliac fossa
  • AXR- faecal unloading at junction of small and large bowel

Question 17

Useful summary of effects of CTFR mutuation

Answer 17