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Published ahead of print on October 22, 2004, doi:10.1164/rccm.200405-667OC

Am. J. Respir. Crit. Care Med., Volume 171, Number 2, January 2005, 158-164

A more recent version of this article appeared on January 15, 2005
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Submitted on June 1, 2004
Accepted on October 19, 2004

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

Madiha Ellaffi1, Christophe Vinsonneau2, Joel Coste3, Dominique Hubert1, Pierre-Regis Burgel1, Jean-Francois Dhainaut1, and Daniel Dusser1*

1 Service de Pneumologie, Hopital Cochin, Paris, France, 2 Service de Reanimation Medicale, Hopital Cochin, Paris, France, 3 Service d'Informatique Medicale et de Biostatistique, Hopital Cochin, Paris, France

* To whom correspondence should be addressed. E-mail: daniel.dusser{at}cch.ap-hop-paris.fr.

We retrospectively studied the outcomes of adult cystic fibrosis patients 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 ICU (n=23) depending on severity. The clinical characteristics prior to exacerbations were similar in both groups. Non invasive mechanical ventilation was used in 61% (14/23) and 33% (15/46) of cases treated in the Intensive Care Unit and the Pulmonary Department groups, respectively. Invasive ventilation was necessary in 4/23 cases treated in the ICU. The one-year survival rate was 52% (12/23) and 91% (42/46) 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, rapid decline in FEV1 prior to admission and severity of exacerbations (severity of hypoxemia and hypercapnia, SAPS II and LOD scores, requirement of non invasive mechanical ventilation, and hospitalization in the ICU) in the univariate analysis, and were prior colonization with Burkholderia cepacia, severity of hypoxemia at admission, and hospitalization in the ICU, in the multivariate analysis. In one-year survivors, pulmonary exacerbation did not affect the progression of the disease.


Key words: Pulmonary exacerbation, cystic fibrosis, intensive care unit, respiratory failure, nasal mechanical ventilation




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