S8) Cystic Fibrosis and Bronchiectasis

Question 1

What is bronchiectasis?

Answer 1

Bronchiectasis is the chronic dilatation of one or more bronchi wherein the bronchi exhibit poor mucus clearance and there is predisposition to recurrent/chronic bacterial infection

Question 2

Identify 5 causes of bronchiectasis

Answer 2

• Post infective e.g. whooping cough, TB
• Immune deficiency e.g. hypogammaglobulinaemia
• Obstruction e.g. foreign body, tumour, extrinsic lymph node
• Inhalation of toxic chemicals/gases
• Secondary immune deficiency e.g. HIV, malignancy

Question 3

Identify 4 genetic causes of bronchiectasis

Answer 3

• Cystic fibrosis
• Primary ciliary dyskinesia
• Young’s syndrome (bronchiectasis, sinusitis, reduced fertility)
• Kartagener syndrome (bronchiectasis, sinusitits, situs inversus)

Question 4

Identify 3 causative organisms of bronchiectasis

Answer 4

• Haemophilus influenzae
• Fungi e.g. aspergillus, candida
• Non-tuberculous mycobacteria

Question 5

Describe the management of bronchiectasis

Answer 5

• Treat underlying cause
• Physiotherapy for mucus clearance
• Antibiotics according to sputum cultures
• Flu vaccine
• Bronchodilators

Question 6

What is cystic fibrosis?

Answer 6

Cystic fibrosis is a multisystem, autosomal recessive disease occurring due to a mutation in the CFTR molecule which is characterised by thickened secretions

Question 7

How does cystic fibrosis present?

Answer 7

• Meconium ileus – intestinal obstruction by sticky secretions (bilous vomiting & abdominal distension)
• Intestinal malabsorption – severe deficiency of pancreatic enzymes
• Recurrent chest infections

Question 8

How does one confirm the diagnosis of Cystic fibrosis?

Answer 8

• 1/more characteristic phenotypic features
• History of CF in a sibling
• Positive newborn screening test result
• Increased sweat [Cl^-]
• Identification of two CF mutations (genotyping)

Question 9

Identify 4 common cystic fibrosis complications and describe how they are treated

Answer 9

• Respiratory Infections – aggressive therapy with physio and prophylactic antibiotics
• Low body weight – pancreatic enzyme replacement therapy, high calorie intake and extra supplements
• Distal Intestinal Obstruction Syndrome (DIOS)
• CF Related Diabetes

Question 10

Describe the management of cystic fibrosis

Answer 10

• Avoid smoking
• Avoid other CF patients
• Avoid jacuzzis (pseudomonas)
• Annual influenza immunisation
• Sodium chloride tablets in hot weather / vigorous exercise