Abstract
Although pulmonary involvement caused by P. aeruginosa (PA) infections accounts for most of the morbidity associated with cystic fibrosis (CF), exacerbations characteristically lack the classic signs of infection. Recently, the incidence of other gram negative nonfermentors has increased, and approximately 17% of the CF patients at this center are colonized by P. cepacia (PC). We report here four female CF patients with previously only mild pulmonary involvement, who developed rapidly progressive fatal PC infection, characterized by spiking fever, leukocytosis, and bacteremia. These patients demonstrated a dramatic fulminant course with PC rather than the chronic deterioration more frequently associated with PA infections. In three of the patients the interval between colonization and death was 7 weeks or less. Colonization in one instance was probably hospital-associated. Treatment with aminoglycosides and ureidopenicillins was ineffective; ceftazidime offered temporary clinical improvement. Female CF patients, especially after puberty, appear at greater risk for PC infections than males. PC is usually an environmental commensal, but appears to have increased virulence in CF patients through as yet undefined mechanisms. Clinicians should be aware of PC as a new significant pathogen in CF. Investigations of altered host response, nosocomial spread, and selection due to antibiotic pressure should be addressed.
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Boxerbaum, B., Klinger, J. PSEUDOMONAS CEPACIA BACTEREMIA IN CYSTIC FIBROSIS. Pediatr Res 18 (Suppl 4), 269 (1984). https://doi.org/10.1203/00006450-198404001-01059
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DOI: https://doi.org/10.1203/00006450-198404001-01059
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