GP51 Pandoraea sputorum in a paediatric cystic fibrosis patient

Abstract

Introduction Cystic fibrosis (CF) is the most frequent fatal genetic disease among caucasians. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which is found in all exocrine tissues, leading to thick, viscous secretions in several organs. The major cause of morbidity and mortality in CF’s patients is lung function’s deterioration due to pathogenic colonization with several bacteria. One emerging multidrug resistant pathogen that has been identified in CF is Pandoraea sputorum, a non-fermenting Gram negative bacteria. Case report A 8-year-old boy, diagnosed with CF at 4 years old (homozygous genotype F508del; sweat test positive: 150 mEq/L) was admited to the hospital for intravenous treatment of Pandoraea sputorum bronchopulmonary colonization. This pathogen was identified by matrix-assisted laser desorption ionization–time of flight mass spectrometry. He had a history of Pseudomonas aeruginosa lung colonization at 4 years old, and of Stenotrophmonas maltophila at 7 years old, all of which had been eradicated after intravenous antibiotic treatment. All prior respiratory exacerbations were also treated empirically. His previous pulmonary function test revealed a forced expiratory volume in 1second (FEV1) of 89%. He was assymptomatic before, and throughout his inpatient stay, that lasted for 14 days while he received intravenous imipenem (80 mg/Kg per day). Two weeks after being discharged he was assessed at a paediatric pneumology appointment, remaining without symptoms, and with a FEV1 of 88%. His follow-up sputum culture was negative for Pandoraea sputorum. Discussion In this clinical case the authors followed the protocol for early eradication, treating when the pathogen is first acquired, regardless of age or clinical signs or symptoms. This was based on several case reports that compared Pandoraea sputorum to Pseudomonas aeruginosa in terms of pathogenicity and decline of lung function. There is still a need to better understand this microorganism, and its correlation with disease outcomes, that can arise from the description of more case reports.