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Cystic fibrosis (CF) is a systemic disease that affects multiple organ systems, especially the respiratory and gastrointestinal tracts and the exocrine glands. Comprehensive care is usually delivered at CF centers, but a patient with a complication may present to any hospital. The most common pulmonary complications are pulmonary exacerbations, pulmonary hemorrhage/hemoptysis, and pneumothorax. The most urgent gastrointestinal concern is distal ileal obstruction syndrome (DIOS), an acute intestinal obstruction. Cystic fibrosis–related diabetes (CFRD) is now the most common complication as a result of improved survival. If possible, always coordinate the management of a patient with CF with the staff of the...

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