By Duncan M. Geddes MD, MRCP (auth.)
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Extra resources for Airways Obstruction
Since the air inside the bulla is not resorbed, there is always a connection between the bulla and the bronchial tree. Emphysema and Bullae 37 Pathogenesis Emphysema A small airspace is formed by the destruction of alveolar walls. Air continues to enter the space by collateral drift but does not leave during expiration because of airways closure. As the space enlarges, the surface tension of its wall decreases, predisposing to further enlargement. Valve Mechanism An airway may be distorted or narrowed by disease and so allow air to enter the bulla more easily than it leaves.
Any individual with a ZZ phenotype is likely to develop emphysema. Smoking should therefore be forbidden. All children of a ZZ will carry the Z allelle and if such an individual marries an MZ heterozygote, half their offspring will be at serious risk. Genetic counselling is important since ZZ homozygotes are usually of reproductive age. The management of the emphysema itself is conducted along conventional lines. Heterozygotes The phenotypes MS, MZ have intermediate levels of at antitrypsin. Detailed studies of such individuals have shown a slight impairment of lung function, especially among smokers.
In doubtful cases, fludrocortisone 4 mg daily is given for five days and the test repeated; in cystic fibrosis the sweat sodium level does not fall. Bacteriology Sputum culture is the most important investigation once the diagnosis is made. The same organism is usually grown from a patient over a long period. Staph. aureus, Haemophilus and Pseudomonas Bronchiectasis and Cystic Fibrosis 49 are the commonest pathogens but Proteus and anaerobes occasionally give problems. Tuberculosis is very rare.