Congenital Anomalies Flashcards

1
Q

Congenital anomalies of the lung

A
  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
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2
Q

Features of Kartagener syndrome

A
  • immotile cilia syndrome
  • cilia have abnormal structure/unable to beat in a coordinated fashion
  • recurrent infections
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3
Q

Features of cystic fibrosis

A
  • 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
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4
Q

Features of neonatal respiratory distress syndrome

A
  • 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
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