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Self-Assessment Questions

Pulmonary Disease

Answer 3
  1. Lymphangiomyomatosis and chylothorax. There is no link between lymphangiomyomatosis and CF. Lymphangiomyomatosis may cause chylothorax. Hemoptysis (either scanty or profuse) and spontaneous pneumothorax are well-known complications of CF. Atelectasis can result from the mucus plugging of airways. Acute or chronic respiratory failures are also seen commonly in CF patients, as are pulmonary infections due to pseudomonads or staphylococci. CF patients are prone to develop allergic bronchopulmonary aspergillosis, which requires treatment with corticosteroids. Hypertrophic pulmonary osteoarthopathy, which is a triad of digital clubbing, arthritis, and periostitis, can occur in CF and is clearly associated with severity of pulmonary disease. In hypertrophic pulmonary osteoarthopathy, the long and tubular bones are most often affected. Hypertrophic pulmonary osteoarthopathy is treated with anti-inflammatory agents for symptomatic relief.

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