Bronchiectasis and Cystic Fibrosis

Question 1

Describe bronchiectasis. (4)

Answer 1

Chronic dilation of one of more bronchi so it appears bigger than the adjacent blood vessel, bronchial wall thickening, and poor mucus clearance.

Question 2

Describe the diagnosis of bronchiectasis. (2)

Answer 2

Signet ring sign on high resolution CT.

Question 3

Describe symptoms of bronchiectasis. (8)

Answer 3

Often non-specific
Chronic cough and sputum production
Breathlessness, chest pain, haemoptysis, fatigue
Wheeze less common.

Question 4

Describe the causes of bronchiectasis. (10)

Answer 4

Common:
Post infective - TB, pertussis toxin
Immune deficiency
Muco-ciliary clearing defects - CF, Young’s syndrome, Kartagener Syndrome.

Less common:
Obstruction - foreign body, tumour
Toxic insult - vomit aspiration, toxin inhalation

Question 5

Describe Young’s Syndrome. (3)

Answer 5

Sinusitis, bronchiectasis and reduced fertility.

Question 6

Describe Kartagener Syndrome. (3)

Answer 6

Sinusitis, bronchiectasis and situs invertus.

Question 7

Describe 4 common organisms in the sputum of a bronchiectasis patient.

Answer 7

Haemophilus influenzae
Pseudomonas aeruginosa
Streptococcus Pneumonia
Non-tuberculus mycobacterium

Question 8

When should you ask “could this be bronchiectasis?” (5)

Answer 8

“Asthmatics” with common severe chest infections, nasal/ear symptoms, IBD, RA, positive sputum culture.

Question 9

Describe the management of bronchiectasis (5)

Answer 9

Physio and airway clearance
Routine sputum sampling
Exclude immunodeficiency 
Treat identifiable cause
Consider daily abx and flu vaccines

Question 10

Describe a bronchiectasis exacerbation. (7)

Answer 10

Deterioration in 3 or more key symptoms in 48 hours: cough, sputum volume / consistency / purulence, breathlessness, fatigue, haemoptysis.

Question 11

Describe the pathophysiology of cystic fibrosis. (5)

Answer 11

Autosomal recessive defect in chromosome 7
CF transmembrane conductance regulator mutation
Ineffective cell surface Cl- excretion and increased ENaC activity (pumping Na back in) .
Dehydrated bodily fluid in eg lungs, liver, GI.

Question 12

Describe the diagnosis of CF. (5)

Answer 12

One or more phenotypic features OR history of a sibling OR postive newborn screening
AND increased sweat chloride test OR genotyping showing mutations.

Question 13

Describe the presentation of CF. (4)

Answer 13

Meconial delay at birth.
Intestinal malabsorption due to pancreatic enzyme deficiency
Chest infections common
New born screening.

Question 14

Describe CF complications relevant to the lungs. (5)

Answer 14

Bronchiectasis, pneumothorax, Resp failure, haemoptysis.

Question 15

Describe CF complications relevant to the Upper respiratory tract. (2)

Answer 15

Chronic sinusitis, nasal polyps.

Question 16

Describe CF complications relevant to the Pancreas (2)

Answer 16

Diabetes, pancreatic insufficiency

Question 17

Describe CF complications relevant to the Gut (1)

Answer 17

Oesophageal reflux.

Question 18

Describe CF complications relevant to the Liver (2)

Answer 18

Chronic liver disease, portal hypertension.

Question 19

Describe CF complications relevant to the Biliary tree. (1)

Answer 19

Gallstones

Question 20

Describe CF complications relevant to the Heart (1)

Answer 20

Cardiac failure

Question 21

Describe CF complications relevant to the Bones (2)

Answer 21

Arthritis, osteoporosis

Question 22

Describe CF complications relevant to the Reproductive system. (2)

Answer 22

Males congenitally infertile due to bilateral absence of Vad Deferens.

Question 23

Describe th lifestyle advice given to a patient with CF. (8)

Answer 23

No smoking
Avoid sites of infection - jacuzzis (pseudomonas), other CF patients, friends with colds, clean nebs properly, stables/rotting veg (aspergillus).
Annual flu jab
NaCl tablets for exercise in hot weather.

Question 24

Describe the management for CF. (3)

Answer 24

Maintain lung health and nutrition
Genotype specific therapies
Managing other co-morbidities - diabetes, liver disease.