Burkholderia cepacia in cystic fibrosis: Novel Australian cluster strain without accelerated respiratory deterioration (original) (raw)

2001, Journal of Paediatrics and Child Health

The isolation of Burkholderia cepacia (B. cepacia) from the sputum of patients with cystic fibrosis (CF) has been associated with rapid pulmonary decline 1,2 and increased mortality. 3-5 Colonization with B. cepacia has been considered an independent negative prognostic factor in CF which may effectively preclude patients from organ transplantation. 6 Retrospective studies in CF patients have identified advanced lung disease, female gender and increasing age as predisposing factors to colonization with B. cepacia. 6-8 Whilst B. cepacia is an environmental pathogen, 9 there is clear documentation of person to person spread of various B. cepacia epidemic strains in CF populations both within and outside hospitals. 8-12 Based upon North American and European data, the prevalence of B. cepacia in CF clinics is approximately 3-8%, with occasional epidemics increasing the prevalence to around 30%. 2,6,13,14 There is no accurate data on B. cepacia prevalence in CF centres in Australia. Despite the increasing recognition of B. cepacia in CF patients during the last 15 years, there have been comparatively few reports of unexpected deaths within 12 months of colonization of B. cepacia (Cepacia syndrome). 1,15 Interestingly, the majority of reported deaths have come from the Northern hemisphere, especially from centres around the Great Lakes in North America, and have been attributed to Genomovar III species of B. cepacia. 1,6,10,15 Moreover, considerable variability exists in the outcome of CF patients with similar disease severity following colonization with B. cepacia, suggesting that different strains are associated with differing pathogenicity. 1,2,10,15,16 In the CF clinic at the John Hunter Hospital, Newcastle, Australia, the presence of an endemic strain of B. cepacia as defined by pulse field gel electrophoresis in 19/20 patients colonized with B. cepacia was recently reported. 16 This represented more than 25% of all patients currently managed in our paediatric clinic between 1993 and 1997. B. cepacia was first isolated in one of our CF patients in 1987 and further cases appeared until the early 1990s. However, because of increasing numbers of positive isolates in our CF population, we screened all CF patients routinely for B. cepacia from 1992, with the first complete set of sputa being tested in 1993. The B. cepacia 'cluster' was attributed to cross-infection because of: (1) the novel single strain that was isolated and (2) the lack of a segregation policy in hospitalized patients and in the outpatient clinic. Whilst we have since instigated a segregation policy for our B. cepacia colonized patients in the outpatient clinic and in the hospital, our experience suggested that colonization with B. cepacia in our CF patients was not life-threatening, as reported previously. 1,6,14,15 Despite increasing numbers of B. cepacia colonized CF patients in our clinic, there was only