Bronchiectasis

Question 1

What is bronchiectasis?

Answer 1

-chronic lung disease caused by permanent destruction of the bronchial walls. This destruction leads to increased mucus because the cilia are not able to clear it away which leads to LRTI

Question 2

What are the causes of bronchiectasis?

Answer 2

1. Cystic Fibrosis
2. Non-cystic fibrosis
   - bronchial lumen obstruction(TB or foreign body aspiration)
   - lung parenchyma destruction-necrotising pneumonia
   - repeated LRTI like pneumonia, HIV infected children, malnourished, aspiration penumonia, primary cilairy dyskinesia

Question 3

What do children with bronchiectasis present with?

Answer 3

1. Recurrent respiratory tract infections
2. Failure to thrive with stunting
3. Repeatedly treated for pulmonary TB because of their symptoms
4. Chronic productive cough(large amounts of sputum and halitosis)
5. Clubbing
6. Air trapping (barrel chest, displaced liver, decreased cardiac dullness)
7. On auscultation: widespread coarse crackles
8. If localized disease-trachea displaced and bronchial breathing and crackles over the pathology

Question 4

How do we diagnose bronchiectasis?

Answer 4

1. Definitive test is chest CT
2. CHEST X-Ray-will see honeycomb appearance(small cysts) which are indicative of small cysts and areas of non-specific consolidation, bronchial dilation and fibrosis

Question 5

What is the approach to bronchiectasis in South Africa?

Answer 5

1. Determine the cause of the bronchiectasis
   - usually cystic fibrosis,complicated HIV, TB
2. Determine anatomical extent of disease using CT scan
3. Determine functional extent of disease using 6 minute walk test and lung function test
4. Manage the complications of bronchiectasis
   - bacterial superinfection: haemophillus
   - TB because bronchiectasis can cause deactivation and even reinfection of TB
   - cor pulmonale leads to right sided heart failure

Question 6

How does the complication of cor pulmonale lead to heart failure?

Answer 6

The bronchiectasis causes destruction of the pulmonary vascular bed and coupled with hypoxia leads to right sided cardiac failure

Question 7

What is the management of a child with bronchiectasis?

Answer 7

1. Find the cause of the acute exacerbation by taking sputum specimen and and treating with broad spectrum AB
2. Focus on nutrition because they are often stunted
3. Make sure immunizations are up to date and if older give pneumococcal vaccine
4. Physiotherapy daily
5. Bronchodilator- salbutamol
6. Macrolide antibiotic -azithromycin to control the airway inflammation

Question 8

How many children with bronchiectasis have reversible airway obstruction?

Answer 8

40%

Question 9

When would we consider surgical management of bronchiectasis?

Answer 9

If the child presents with unilateral bronchiectasis , remains symptomatic with treatment and has no pulmonary hypertension

Question 10

What is primary cilia dyskinesia?

Answer 10

It is a genetic abnormality resulting in dysfunctional cilia in which the mucociliary blanket does not clear secretions from the respiratory system

Question 11

What infections does primary ciliary dyskinesia cause?

Answer 11

1. Otitis media
2. Sinusitis
3. Recurrent pneumonia

Question 12

How do we diagnose primary ciliary dyskinesia?

Answer 12

By radioisotope clearance test or measuring the amount of nitric oxide in the exhaled air

Question 13

How do children usually present at birth with primary ciliary dyskinesia?

Answer 13

1. Blocked nose from birth

2. Dextrocardia-

Question 14

What are the causes of focal bronchiectasis?

Answer 14

1. Obstruction of the bronchus
   - intraluminal like foreign body, tumor or granuloma
   - extra-luminal like lymphadenopathy
2. Bronchial stenosis

Question 15

What are the causes of generalised bronchiectasis?

Answer 15

1. Upper lobes-cystic fibrosis, sarcoidosis, TB
2. Middle lobe- primary ciliary dyskinesia
3. Lower lobe- chronic aspiration, immunodeficiency