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[Cystic fibrosis in adults]
1Service de pneumologie Hôpital Cochin 75679 Paris. dominique.hubert@cch.ap-hop-paris.fr
Abstract:
One third of patients with cystic fibrosis are adults. The respiratory disease, which conditions the vital prognosis, consists of bronchiectasis with chronic bronchial infection and bursts of acute infection, leading to respiratory insufficiency. Pseudomonas aeruginosa is the most frequent bacteria in adulthood. Exocrine pancreatic insufficiency is associated in 80% of patients. Some complications, such as diabetes, hepatic cirrhosis or osteoporosis, are more frequent in adults. Treatment is based on chest physiotherapy and antibiotics with intravenous courses when there is a colonisation with P. aeruginosa. Pregnancy can be considered in women when respiratory and nutritional status is acceptable. Men are sterile because of congenital bilateral atresia of vas deferens, but can be proposed medical assistance for procreation.