Intensive Care Medicine

, Volume 32, Issue 5, pp 754–758 | Cite as

Outcome of assisted ventilation for acute respiratory failure in cystic fibrosis

  • Martijn G. Slieker
  • Josephus P. J. van Gestel
  • Harry G. M. Heijerman
  • Gerdien A. Tramper-Stranders
  • Ferdinand Teding van Berkhout
  • Cornelis K. van der Ent
  • Nicolaas J. G. Jansen
Brief Report

Abstract

Objectives

To assess outcome of assisted ventilation in cystic fibrosis (CF) patients with acute respiratory failure (ARF), to identify risk factors associated with poor outcome and to compare long-term outcome of CF children who were mechanically ventilated for ARF with unventilated CF controls.

Design

Retrospective cohort study.

Setting

Two large CF centres in the Netherlands.

Patients

CF patients who required assisted ventilation for ARF and unventilated CF controls.

Interventions

None.

Measurements and results

Thirty-one CF patients required assisted ventilation for ARF between January 1990 and March 2005. All five children (under 2 years of age) and seven adults (27%) survived. In the total population, age was a statistically significant risk factor for poor outcome (p = 0.02). In adult CF patients who required invasive mechanical ventilation, acute on chronic respiratory failure was associated with poor outcome. In children who required mechanical ventilation for ARF, lung function and CF related complications 5 years later were not significantly different compared with controls matched for age, gender and genotype.

Conclusions

CF patients younger than 2 years old, who are ventilated because of ARF, have a good prognosis and their long-term outcome seems identical to unventilated CF controls. ARF in adult CF patients still is associated with high mortality, especially among patients with acute on chronic respiratory failure.

Keywords

Cystic fibrosis Intensive care unit Mechanical ventilation Mortality Respiratory failure Risk factors Outcome 

Supplementary material

134_2006_85_MOESM1_ESM.doc (116 kb)
Electronic Supplementary Material (DOC 118kb)

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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Martijn G. Slieker
    • 1
  • Josephus P. J. van Gestel
    • 2
  • Harry G. M. Heijerman
    • 3
  • Gerdien A. Tramper-Stranders
    • 1
  • Ferdinand Teding van Berkhout
    • 1
  • Cornelis K. van der Ent
    • 1
  • Nicolaas J. G. Jansen
    • 2
  1. 1.Cystic Fibrosis Centre UtrechtUniversity Medical Centre UtrechtUtrechtThe Netherlands
  2. 2.Paediatric Intensive Care Unit, Wilhelmina Children's HospitalUniversity Medical Centre UtrechtUtrechtThe Netherlands
  3. 3.Adult Cystic Fibrosis CentreHaga Teaching HospitalThe HagueThe Netherlands

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