Over time, these weaken the bronchial walls, leading to bronchiectasis 3. Haemophilus influenzae: especially in the first 6 months of lifeĪs a result of repeated and chronic infections, there is a marked increase in the number of polymorphonuclear (PMN) leukocytes and associated inflammatory agents, including elastase and collagenase.Staphylococcus aureus: especially in the first 6 months of life 1.The organisms most responsible for pulmonary infections are: The result is iso-osmotic, but low volume, secretions, which tend to dry out, or be thick as they still contain all the other constituents.Ībnormal CFTR function has other effects 1,3, e.g: In CF patients, too little chloride is pumped out, too much sodium is reabsorbed resulting in osmotic re-absorption of water from the lumen. Therefore, under normal circumstances, salt and chloride remain in the lumen and keep water there osmotically. In the lung, the cystic fibrosis transmembrane regulator (CFTR) is a protein responsible for efflux of chloride and inhibition of the sodium channel's activity which controls the influx of sodium. ![]() Later in the disease, larger volume hemoptysis, which may be life-threatening, as well as pneumothoraces become more common 2. Patients have a chronic cough and expectorate copious quantities of sputum, frequently blood stained and containing mucous plugs 2,7. Clinical presentation is with the expected recurrent bacterial infections and hemoptysis.
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