Table 1.
Patients characteristics and clinical outcome
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | ||
|---|---|---|---|---|---|---|---|
| Age (years) | 64 | 23 | 39 | 29 | 18 | 18 | |
| Sex | M | M | M | F | M | M | |
| CFTR mutation | 1717-1GA/2789 + 5GA | F508del/F508del | F508del/F508del | F508del/F508del | F508del/R553X | F508del/F508del | |
| Date of first P. pulmonicola isolation | 2000 | March 2009 | June 2009 | October 2009 | November 2009 | December 2009 | |
| Associated pathogens | mucoid P. aeruginosa | mucoid P. aeruginosa | mucoid P. aeruginosa | mucoid P. aeruginosa | S. aureus, mucoid. P. aeruginosa, A. xylosoxidans, A. fumigatus | S. aureus, mucoid P. aeruginosa, A. fumigatus, G. argillacea | |
| Best Forced Expiratory Volume in 1 second (FEV1) value per year | 2009 | 25 | 45 | 33 | 43 | 47 | 61 |
| 2010 | / | 41 | 25 | 35 | 44 | 42 | |
| 2011 | / | 40 | 20 | / | 42 | 41 | |
| 2012 | / | 31 | 19 | / | / | 59 | |
| 2013 | / | 42 | 19 | / | / | 60 | |
| Number of intravenous antibiotic courses per year | 2009 | 8 | 2 | 6 | 10 | 7 | 8 |
| 2010 | / | 3 | 5 | 3 | 8 | 5 | |
| 2011 | / | 1 | 4 | / | 7 | 6 | |
| 2012 | / | 3 | 6 | / | / | 1 | |
| 2013 | / | 0 | 3 | / | / | 0 | |
| Organ transplantation | No | No | No (listed for heart and lung transplantation) | Lung transplantation (March 2010) | Lung and liver transplantation (April 2012) | Lung transplantation (January 2012) | |
| Clinical outcome | Death (October 2009) from severe comorbidities | Stable | Decline in lung function | Death (April 2010) from post transplantation bacteremia due to P. aeruginosa | Death (May 2012) from severe multiple post transplantation organ failure | Stable |