312 Outcomes and factors associated with poor outcome of children with cystic fibrosis admitted to the intensive care unit.

Prognosis of adult patients with cystic fibrosis (CF), admitted to intensive care unit (ICU), has improved. Few data are available for children. The aim is to describe clinical course and outcomes of CF children hospitalized in ICU. Baseline characteristics of children, reasons for admission in ICU, ventilatory support and outcomes at discharge from ICU were retrospectively analyzed. Non parametric statistical test compared data of survivors at ICU to those with poor outcome (death or lung transplantation in super emergency). From 2000 to 2013, 26 children, median age 13.4 years (1.9–17.6), were admitted to ICU (Necker-Enfants Malades, Paris). Before admission, 15 children (58%) had a non-invasive ventilation (NIV) and 23 (88%) were colonized with Pseudomonas aeruginosa . The median forced expiratory volume (FEV 1 ) was 32% (13–101). Eighteen children (69%) were admitted for respiratory exacerbation, NIV was initiated for 18 (69%) and 9 (35%) were intubated. Half of the 26 children had a poor outcome, 5 (19%) died and 8 (31%) needed urgent lung transplantation. Poor outcome-associated factors were female sex (p = 0.015), history of hemoptysis (p = 0.03), ventilation (p = 0.001) or nutritional (p = 0.017) support before ICU, chronic intravenous antibiotics use (p = 0.001), lower FEV 1 before ICU (27% (13–49) for poor outcome group and 40% (17–101) for survival group, p = 0.036), admission for respiratory exacerbation (p = 0.030), hypercapnia (p = 0.010), metabolic acidosis (p = 0.007) and hypochloremia (p = 0.013). Our study highlights poor outcomes in children hospitalized in ICU and points factors associated with poor outcomes. This will help to improve indications of pediatric lung transplant. [ABSTRACT FROM AUTHOR]