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Published ahead of print on October 22, 2004, doi:10.1164/rccm.200405-667OC
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American Journal of Respiratory and Critical Care Medicine Vol 171. pp. 158-164, (2005)
© 2005 American Thoracic Society
doi: 10.1164/rccm.200405-667OC

Original Article

One-year Outcome after Severe Pulmonary Exacerbation in Adults with Cystic Fibrosis

Madiha Ellaffi, Christophe Vinsonneau, Joël Coste, Dominique Hubert, Pierre-Régis Burgel, Jean-François Dhainaut and Daniel Dusser

Service de Pneumologie; Service de Réanimation Médicale; and Service d'Informatique Médicale et de Biostatistique, Groupe Hospitalier Cochin, Assistance Publique Hôpitaux de Paris, Faculté Cochin Port-Royal, Université Paris 5, Paris, France

Correspondence and requests for reprints should be addressed to Daniel Dusser, M.D., Service de Pneumologie, Hôpital Cochin, 27 rue du faubourg Saint Jacques, 75679 Paris cedex 14, France. E-mail: daniel.dusser{at}cch.ap-hop-paris.fr

We retrospectively studied the outcomes of adult patients with cystic fibrosis (CF) hospitalized for severe pulmonary exacerbations (69 cases) between January 1997 and June 2001. Cases were treated either in the Pulmonary Department (n = 46) or in the intensive care unit (ICU) (n = 23) depending on severity. Noninvasive mechanical ventilation was used in 61% (14 of 23) and 33% (15 of 46) of cases treated in the ICU and the Pulmonary Department groups, respectively. Invasive ventilation was necessary in 4 of 23 cases treated in the ICU. The 1-year survival rate was 52% (12 of 23) and 91% (42 of 46) in the ICU and the Pulmonary Department groups, respectively. Lung transplantation was performed in two patients from the ICU group and in five patients from the Pulmonary Department group after hospital discharge. Factors predictive of death were prior colonization with Burkholderia cepacia and rapid decline in FEV1 before admission and severity of exacerbations (severity of hypoxemia and hypercapnia, simplified acute physiology score II and logistic organ dysfunction (LOD) scores, requirement of noninvasive mechanical ventilation, and hospitalization in the ICU) in the univariate analysis and were prior colonization with B. cepacia, the severity of hypoxemia at admission, and hospitalization in the ICU in the multivariate analysis. In 1-year survivors, pulmonary exacerbation did not affect the progression of the disease.

Key Words: cystic fibrosis • intensive care unit • nasal intermittent positive pressure ventilation • pulmonary exacerbation • respiratory failure




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