title: [Management of acute and severe complications in adults with cystic fibrosis].
abstract: The natural history of cystic fibrosis (CF) may be associated both with acute respiratory complications (respiratory exacerbations, haemoptysis, pneumothorax) and with non-respiratory complications (distal intestinal obstruction syndrome, dehydration) that may result in hospitalizations. The aim of this article is to describe the main therapeutic approaches that are adopted in the management of acute complications occurring in CF adults, and to discuss indications for admission of these patients to intensive care units. Adult CF patients admitted to intensive care unit often benefit from antibiotic courses adapted to their chronic bronchial infection, especially when the hospitalization is related to respiratory disease (including haemoptysis and pneumothorax). Nutritional support, including hypercaloric diet, control of hyperglycemia and pancreatic enzyme supplementation is warranted. The recommended therapy for major haemoptysis is bronchial artery embolization. Patient with significant pneumothorax should have a chest tube inserted, while the treatment of distal intestinal obstruction syndrome will most often be medical. In case of respiratory failure, non-invasive ventilation is the preferred mode of ventilatory support because invasive ventilation is associated with poor outcomes. Therapeutic options should always have been discussed between the patient, family members and the CF medical team to allow for informed decision making.
authors.affiliation: Service de pneumologie, centre de ressources et de compétences pour la mucoviscidose de l'adulte, hôpital Cochin, AP-HP, université Paris Descartes, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: Chapron
authors.forename: B
authors.initials: B
authors.lastname: Zuber
authors.forename: R
authors.initials: R
authors.lastname: Kanaan
authors.forename: D
authors.initials: D
authors.lastname: Hubert
authors.forename: N
authors.initials: N
authors.lastname: Desmazes-Dufeu
authors.forename: J-P
authors.initials: JP
authors.lastname: Mira
authors.forename: D
authors.initials: D
authors.lastname: Dusser
authors.forename: P-R
authors.initials: PR
authors.lastname: Burgel
mesh_terms: D000208:Acute Disease; D000328:Adult; D003131:Combined Modality Therapy; D003550:Cystic Fibrosis / Q000150:complications* / Q000628:therapy*; D003681:Dehydration / Q000209:etiology* / Q000628:therapy*; D006469:Hemoptysis / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D007362:Intensive Care Units; D007415:Intestinal Obstruction / Q000209:etiology* / Q000628:therapy*; D010343:Patient Admission; D011030:Pneumothorax / Q000209:etiology* / Q000628:therapy*; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000628:therapy*; D012151:Resuscitation