TABLE 1.
Clinical characteristics
| Burkholderia isolation# | |
| Gender | |
| Female | 3 (42.9) |
| Male | 4 (57.1) |
| Age, years | 11.7 (9.0–19.6) |
| BMI, kg·m−2 | 17.1 (15.5–20.1) |
| CFTR mutation | |
| Homozygote Phe508del | 4 (57.1) |
| Heterozygote Phe508del | 3 (42.9) |
| Other | 0 |
| Comorbidities | |
| Cystic fibrosis-related diabetes | 0 |
| Cystic fibrosis-related liver disease | 4 (57.1) |
| Pancreatic insufficiency | 7 (100.0) |
| Osteoporosis | 0 |
| Forced expiratory volume in 1 s, % pred | 82.7±20.7 (58–107) |
| Forced expiratory volume in 1 s, L | 2.0±0.6 (1.2–3.1) |
| BCC infection initial/new¶ | 6/1 |
| Exacerbation during first isolation | 5 |
| Follow-up after eradication, years, median (range) | 1.5 (0.6–7.2) |
| Coinfection with pathogens | |
| Staphylococcus aureus | 7 (100.0) |
| Haemophilus influenzae | 5 (71.4) |
| Streptococcus pneumoniae | 1 (14.3) |
| Aspergillus spp. | 4 (57.1) |
| Pseudomonas aeruginosa | 1 (14.3) |
| Acinetobacter spp. | 0 |
| Stenotrophomonas maltophilia | 0 |
| Nontuberculosis mycobacteria | 0 |
| BCC antibiotic resistance | |
| Tobramycin | 7 |
| Trimethoprim/sulfamethoxazole | 0 |
| Comedication in year after isolation | |
| CFTR-modulator therapy | 0 |
| Extra course of trimethoprim/sulfamethoxazole during exacerbation in the 6 months after BCC isolation | 2 |
| Tobramycin nebulisation for 1 month for eradication therapy for P. aeruginosa | 1 |
| Sputum samples | |
| Positive for BCC | 10 |
| Total sputum samples | 60 |
Data are presented as n (%), mean (range), n or mean±sd (range), unless otherwise stated. Inclusion criteria: patients diagnosed with cystic fibrosis with clinical signs consistent with cystic fibrosis and sweat chloride >60 mEq·L−1 and/or two cystic fibrosis-causing mutations identified; an initial or new Burkholderia cepacia complex (BCC) isolation from sputum cultures (or pharyngeal swabs and bronchoalveolar lavage) during the study period, treated with inhalation therapy with amiloride and tobramycin, combined with oral cotrimoxazole; and multiple sputum cultures after the end of treatment. Treatment regimen consisted of nebulised amiloride (produced in the hospital pharmacy, with 1 mL amiloride 0.45% solution dissolved in 5 mL 0.9% saline, given thrice daily) followed by tobramycin inhalation (300 mg twice daily) but now with addition of oral trimethoprim/sulfamethoxazole (cotrimoxazole; 12/60 mg·kg−1·day−1). Exclusion criteria: chronic BCC infection; lung transplantation before BCC isolation; incomplete exposure and/or outcome data. During the study period, a total of 18 cystic fibrosis patients had at least one positive culture for BCC, of which 12 patients did not meet the inclusion criteria for this case series: six were already chronically infected (three with Burkholderia multocida, two with Burkholderia multivorans and one not otherwise specified); two received treatment other than the study therapy (both B. multivorans; both developed chronic BCC infection); and two were not treated at the patient's wish due to pregnancy (one with B. multocida and one with B. multivorans; both developed chronic BCC infection). Two patients were not treated because they had only one positive culture, were in good clinical condition and had repeat cultures that were negative (one with Burkholderia cenocepacia and one not otherwise specified). BMI: body mass index; CFTR: cystic fibrosis transmembrane conductance regulator. #: n=7. ¶: defined as an initial BCC isolation when the patient had never been infected with BCC prior to this BCC isolation; for a new BCC isolation, patients had to be free of BCC, defined as a BCC isolation in the past with all negative sputum cultures in the last 6 months.