Congenital Anomalies

Question 1

Congenital anomalies of the lung

Answer 1

1. Agenesis - failure to form
2. Pulmonary Hypoplasia - incomplete/defective development
3. Bronchial atresia
4. Bronchogenic cysts - accessory bronchial buds sealed off from rest of airway
5. Bronchopulmonary sequestration - portion of lung that does not communicate with normal bronchial tree
6. Kartagener syndrome
7. Cystic fibrosis
8. Neonatal respiratory distress syndrome

Question 2

Features of Kartagener syndrome

Answer 2

• immotile cilia syndrome
• cilia have abnormal structure/unable to beat in a coordinated fashion
• recurrent infections

Question 3

Features of cystic fibrosis

Answer 3

• common AR disorder in Caucasians
• caused by production of abnormally viscous mucus that cannot be cleared from lungs, pancreas, intestines
• stagnant - repeated infections & bronchiectasis
• require intensive physiotherapy
• death due to infection/respiratory failure

Question 4

Features of neonatal respiratory distress syndrome

Answer 4

• hyaline membrane disease
• due to deficiency of surfactant in the lungs, usually due to prematurity
• surfactant: secreted by type II pneumocytes, reduces surface tension, keeps alveoli open
• alveolar collapse - hypoxia, damage to endothelial & alveolar lining cells, fibrin exudation
• mothers are given steroids