CF and Bronchiectasis

Question 1

`Which type of bronchiectasis is characterised by walls that appear ‘beaded’ due to alternating dilation and constriction?
A Cylindrical
B Traction
C Varicose
D Cystic

Answer 1

C

Question 2

A chest X-ray of a patient with suspected bronchiectasis shows parallel lines extending from the hilum. What is this radiological sign called?
A Signet ring sign
B Tram-track sign
C Air bronchogram
D Silhouette sign

Answer 2

B

Question 3

Kartagener’s syndrome is a triad of conditions associated with a recognised cause of bronchiectasis. Which of the following correctly identifies this triad?
A
Infertility, liver cirrhosis, bronchiectasis
B
Situs inversus, chronic sinusitis, bronchiectasis
C
Asthma, nasal polyps, bronchiectasis
D
Immunodeficiency, ABPA, bronchiectasis

Answer 3

B

Question 4

A patient on NIV for CF presents with increased headaches and daytime sleepiness during an exacerbation. What is the most likely physiological explanation?
A
Hypoxaemia from worsening V/Q mismatch
B
CO₂ retention due to worsening ventilatory failure
C
Anaemia secondary to haemoptysis
D
Hypoglycaemia from CF-related diabetes

Answer 4

B

Question 5

The CFTR gene responsible for cystic fibrosis is located on which chromosome?
A
Chromosome 14
B
Chromosome 21
C
Chromosome 7
D
Chromosome 12

Answer 5

C

Question 6

Which of the following is NOT a recognised cause of bronchiectasis?
A
Allergic bronchopulmonary aspergillosis (ABPA)
B
Diffuse panbronchiolitis
C
Alpha-1 antitrypsin deficiency causing emphysema
D
Primary ciliary dyskinesia

Answer 6

C

Question 7

Traction bronchiectasis differs from other types primarily because:
A
It produces the most excessive secretions of all types
B
It is caused by parenchymal distortion, often from pulmonary fibrosis, and patients frequently do not produce excessive secretions
C
It is associated exclusively with cystic fibrosis
D
It resolves spontaneously once the underlying fibrosis is treated

Answer 7

B

Question 8

Which scoring tools are used specifically to predict mortality and exacerbation rates in non-CF bronchiectasis?
A
FEV1/FVC ratio and MRC dyspnoea scale
B
CURB-65 and APACHE II
C
Bronchiectasis Severity Index and Bronchiectasis Aetiology and Co-morbidity Index
D
CAT score and GOLD staging

Answer 8

C

Question 9

In the context of CF pathophysiology, what is the primary role of the CFTR protein under normal conditions?
A
Transport of sodium ions from outside to inside the cell
B
Regulation of water absorption across the gastrointestinal epithelium only
C
Movement of chloride ions from inside to outside the cell
D
Active transport of bicarbonate to acidify airway secretions

Answer 9

C

Question 10

A physiotherapist identifies a patient with bronchiectasis associated with rheumatoid arthritis. What is the clinical significance of this aetiology?
A
These patients have the best prognosis and rarely need airway clearance
B
It is associated with a poorer prognosis compared to other causes
C
The condition is fully reversible with disease-modifying antirheumatic drugs
D
RA-associated bronchiectasis is predominantly traction-type and secretion-free

Answer 10

B

Question 11

Which of the following best describes the ‘signet ring sign’ seen on CT in bronchiectasis?
A
Parallel lines extending toward the periphery representing thickened bronchial walls
B
A dilated bronchus appearing larger than its adjacent pulmonary artery in cross-section
C
Mucus plugging visible as dense opacities within the bronchi
D
Air trapping causing mosaic attenuation on expiratory CT

Answer 11

B

Question 12

Distal intestinal obstruction syndrome (DIOS) is a complication of which condition managed by respiratory physiotherapists with expertise in this area?
A
COPD with cor pulmonale
B
Severe bronchiectasis with RA
C
Primary ciliary dyskinesia
D
Cystic fibrosis

Answer 12

D

Question 13

Which pattern of lung disease is bronchiectasis classified under?
A
Obstructive — reduced FEV1/FVC ratio
B
Restrictive — reduced lung volumes and increased FEV1/FVC
C
Mixed restrictive-obstructive only in CF variant
D
Purely vascular with no spirometric changes

Answer 13

A

Question 14

What is the approximate proportion of bronchiectasis cases classified as idiopathic (no identifiable cause)?
A
Around 50%
B
25–30%
C
10–15%
D
Over 70%

Answer 14

A

Question 15

A physiotherapist supporting a CF patient post-operatively notices increasing sputum volume, darker and thicker sputum, and a raised CRP. Which of the following additional findings would most strongly confirm a pulmonary exacerbation?
A
Mild peripheral oedema and new onset hypertension
B
Worsening liver enzymes on routine bloods
C
New onset glycosuria and raised HbA1c
D
Drop in lung function and reduced exercise tolerance alongside the above

Answer 15

D

Question 16

Young’s syndrome shares some features with CF. Which combination of features correctly characterises Young’s syndrome?
A
Pancreatic insufficiency, diabetes, and bronchiectasis
B
PCD, ABPA, and cystic fibrosis
C
Situs inversus, infertility, and liver disease
D
Obstructive azoospermia, chronic sinusitis, and bronchiectasis

Answer 16

D

Question 17

When prescribing exercise for a patient with CF or bronchiectasis, what is the primary physiotherapy rationale beyond cardiovascular fitness?
A
To suppress the cough reflex and reduce haemoptysis risk
B
To increase thoracic kyphosis to improve breathing mechanics
C
To assist airway clearance, improve sputum mobilisation, and support overall lung health
D
To reduce pancreatic enzyme supplementation requirements

Answer 17

C

Question 18

A patient with bronchiectasis presents with haemoptysis, weight loss, fever, and a history of recurrent respiratory infections without a smoking history. P. aeruginosa is cultured from their sputum. What does the presence of P. aeruginosa in non-CF bronchiectasis indicate?
A
It is a normal commensal organism at this site and requires no action
B
It indicates early-stage, reversible disease
C
It is a clinical feature associated with more severe bronchiectasis and worse outcomes
D
It confirms the diagnosis of CF rather than idiopathic bronchiectasis

Answer 18

B

Question 19

Which of the following statements about the physiotherapy role in non-invasive ventilation (NIV) for CF patients is most accurate?
A
Physiotherapists support CPAP/NIV use in the context of respiratory failure and monitor for signs of CO₂ retention
B
NIV is contraindicated in CF patients with respiratory failure
C
Physiotherapists only set up NIV equipment; clinical monitoring is solely a nursing role
D
NIV replaces airway clearance techniques in advanced CF disease

Answer 19

A

Question 20

What is lung compliance

Answer 20

Ability of lungs to expand (change in volume for a change in pressure)

Question 21

Fall in compliance = more or less pressure needed to achieve a change in volume?

Answer 21

More

Question 22

Normal V/Q ratio

Answer 22

0.8

Question 23

Decreased ventilation (eg atelectasis) causes what to the V/Q ratio and why

Answer 23

Decreases ratio as less air gets to alveoli

Question 24

Decreased V/Q ratio does what to PaCO2 and why?

Answer 24

Increases as less oxygen in alveoli means less gas exchange = CO2 retention

Question 25

What does lung dependence mean

Answer 25

The lower areas of the lungs