J. Bartošová

Antineutrophil cytoplasmic antibodies directed against bactericidal/permeability-increasing protein detected in children with cystic fibrosis inhibit neutrophil-mediated killing of Pseudomonas aeruginosa.

Antineutrophil cytoplasmic antibodies (ANCA) directed against bactericidal/permeability-increasing protein (BPI) were repeatedly found in cystic fibrosis (CF) patients. We analyzed the effect of BPI-ANCA in inhibiting neutrophil-mediated killing of Pseudomonas aeruginosa. The bactericidal effect expressed as percentage of killed bacteria after 1 h incubation with neutrophils was 55% when the neutrophils were pretreated with normal human serum, ranged from 49 to 63% with the sera from control BPI-ANCA-negative groups and sharply decreased to the mean 30.5% (range 8-51%) in the presence of BPI-ANCA. Furthermore, the effect mediated by BPI-ANCA was dose dependent and reflected the titer of BPI-ANCA in tested sera.

17 Cystic fibrosis newborn screening (CF NBS): 4 year experience of the Prague Centre with the IRT/DNA/IRT protocol

Background: Cystic Fibrosis Newborn screening (CFNBS) as a pilot study started at the Institute of Mother and Child (IMC) Centre in 1999 and 444 063 newborns were examined until 2003. Current CFNBS has started gradually in Poland in 2006, covering the whole country in 2009 and is ongoing. 582 693 children were screened until the end of 2011. During CFNBS different protocol’s strategies were used. Aim: Impact of the implementation of NBS on the age of CF diagnosis. Methods: The study involved children diagnosed and treated only in IMC CF Centre (from 1999 until the end of 2011). Three groups were formed: 1. Pilot group (p-NBS) − 56 children, according to protocol strategy IRT/IRT and IRT/IRT/DNA, only F508del mutation was assessed; 2. Current group (c-NBS) − 92 children, IRT/IRT/DNA and IRT/DNA protocol, with expanded DNA analysis panel; 3. Patient diagnosed clinically (non-NBS) − 56 children. Moreover, DNA analysis in c-NBS group contains also frequent mutation in Polish population: 3849+10kbC>T, which is combined with low sweat test values. All patients underwent sweat tests. Results: The age of diagnosis was significantly lower (both p T than p-NBS and non-NBS group (both 0.9%). Conclusion: IRT/DNA strategy with extended DNA analysis provides an opportunity of earlier CF diagnosis even in children with normal sweat test values.

137* The end of a long-term outbreak with highly transmissible Burkholderia cenocepacia ST32

Persons with CF can acquire Burkholderia cepacia complex (Bcc) infection through patient-to-patient contacts or from the environment. The former way of acquisition was well documented in the Prague CF centre where infection rate with a single B. cenocepacia strain ST32 reached over 30% in 1997–2003. The aim of this study was to compare epidemiological situation in the Centre in 2003 vs. 2010, and to evaluate efficacy of infection control in tackling the spread of ST32. System of surveillance exploiting typing techniques (RAPD and MLST) was set up in 2008. A total of 76 out of 211 patients examined by the end of 2003 (36.0%) were infected with Bcc; majority of them (78.9%) harboured epidemic strain ST32. Seven years later, Bcc positivity was detected in 72 out of 374 patients who attended the Prague CF clinic (19.3%). While 39 patients still suffered from infection with ST32 (54.2% of infected), a substantial portion of patients carried strains other than ST32 (see Table; note that ST number is specified only if more than two patients harboured the strain). Notably, only 2 patients within the ST32 group became positive after 2003, with the last case dated in May 2007. Epidemiological situation characterized by increasing heterogeneity of the Bcc population and no occurrence of new ST32 cases is a likely consequence of both healthcare workers’ and patients’ good compliance with strict infection control rules. Supported by NS10543−3, MSM0021620812 and MZ0FNM2005.