Outcome of assisted ventilation for acute respiratory failure in cystic fibrosis.
OBJECTIVE
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.


METHODS
Retrospective cohort study.


METHODS
Two large CF centres in the Netherlands.


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


METHODS
None.


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.
Slieker
Martijn G
MG
Cystic Fibrosis Centre Utrecht, University Medical Centre Utrecht, and Paediatric Intensive Care Unit, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
van Gestel
Josephus P J
JP
Heijerman
Harry G M
HG
Tramper-Stranders
Gerdien A
GA
van Berkhout
Ferdinand Teding
FT
van der Ent
Cornelis K
CK
Jansen
Nicolaas J G
NJ
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000503:physiopathology; D005260:Female; D006801:Humans; D007362:Intensive Care Units; D008297:Male; D009426:Netherlands; D012121:Respiration, Artificial; D012128:Respiratory Distress Syndrome; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome