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After antibiotic eradication treatment for a first ever Pseudomonas aeruginosa isolation, the European consensus criteria (ECC) are widely used to assess colonization status with P. aeruginosa in CF-patients. We evaluated to what extent genotyping (GT) of subsequent P. aeruginosa isolates could predict/assess chronic colonization (CC), in comparison with the ECC., Methods: Over a 14-year period, sputa were cultured from 80 CF-patients (age range: 2-51 years), from a first ever isolation of P. aeruginosa onwards. Patients with a positive culture for P. aeruginosa received antibiotic eradication treatment. For the 40 patients for whom three or more P. aeruginosa isolates were available, these isolates were genotyped., Results: According to the ECC, 27 out of the 40 patients (67.5%) became CC during the study period (ECC-positive patients). Genotyping confirmed persistence of the same genotype for 25 of these ECC-positive patients. Genotyping indicated persistence of the same genotype for at least two subsequent isolates for 5 out of 13 ECC-negative patients. Culture-positivity characteristics of the 27 ECC-positive patients corresponded well to those of the 30 GT-positive patients, with an overall higher number of positive cultures as well as a shorter interval in between first and second isolate compared to ECC-negative and GT-negative patients. Genotyping indicated persistence of the same genotype on average 9.3 months earlier than CC according to the ECC (P < 0.01)., Conclusions: Genotyping of P. aeruginosa isolates confirmed CC for 25 out of 27 ECC-positive patients (92.6% specificity) and predicted CC 9.3 months earlier than the ECC., (Copyright © 2018 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Background: Long-term effect of enteral tube feeding (ETF) in cystic fibrosis (CF) remains equivocal., Methods: A Belgian CF registry based, retrospective, longitudinal study, evaluated the pre- and post- ETF (n = 113) clinical evolution and compared each patient with 2 age, gender, pancreatic status and genotype class-matched controls., Results: At baseline ETF had a worse BMI z-score (p < 0.0001) and FEV1% (p < 0.0001) compared to controls. Patients eventually receiving ETF, had already a significant worse nutritional status and pulmonary function at first entry in the registry. Both parameters displayed a significant decline before ETF-introduction. ETF had more hospitalization and intravenous antibiotic (IVAB) treatment days (p < 0.0001). After ETF introduction hospitalizations and IVAB decreased significantly. After ETF-introduction BMI z-score recuperated towards the original curve before the decline, but remained below the controls. Starting ETF had no effect on rate of height gain in children. The pre-index FEV1 decline (-1.52%/year (p = 0.002)) stabilized to +0.39%/year afterwards. Controls displayed decline of -0.48%/year (p < 0.0001)., Conclusion: ETF introduction improved BMI z-score and stabilized FEV1, associated with less hospitalizations and IVAB treatments. Higher mortality and transplantation in the ETF cases, leading to drop-outs, made determination of the effect size difficult., (Copyright © 2018 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Pseudomonas aeruginosa (Pa) is one of the most common and clinically important pathogens in patients with cystic fibrosis (CF). Chronic Pa colonization in CF patients is associated with increased morbidity and mortality. Pa strains causing early infection are usually antibiotic sensitive and have low bacterial density in the airways. As a result, the treatment strategy has shifted from suppressive therapy in patients chronically colonized by Pa to attempts at early eradication therapy as soon as Pa is detected. In the literature, different treatment regimens have been studied. However, the optimal treatment regimen and duration of treatment are not yet determined. In this article, an overview on the natural history of early Pa colonization and the history of eradication treatment is given. Moreover, the results of the different eradication treatment trials and directions for future research are discussed., (Copyright © 2012 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Purpose: Quantitative ultrasound bone sonometry (QUS) might be a promising screening method for cystic fibrosis (CF)-related bone disease, given its absence of radiation exposure, portability of the equipment and low cost.The value of axial transmission forearm QUS in detecting osteopenia in CF was therefore studied., Methods: We investigated the application of QUS in the evaluation of bone status in a group of 64 adolescents (>12 years) and young adults (<40 years) with CF in a comparison with a dual X-ray absorptiometry (DXA) of the whole body and peripheral quantitative computed tomography (pQCT) of the radius at 4% and 66% sites., Results: Mean (SD) Z-scores of speed of sound (SOS), whole body bone mineral content (BMC), radial trabecular bone mineral density (BMD), and radial cortical BMD were respectively -0.31 (0.78), -0.09 (1.28), 0.10 (1.16) and -0.62 (2.88). The pQCT determined bone geometry values (cortical bone area and cortical thickness) were more depressed than the BMD data. QUS had a sensitivity and specificity of respectively 0% and 96% for diagnosing osteopenia (based on a whole body BMC Z-score<-2)., Conclusions: QUS cannot replace DXA, but can screen out patients with normal bone mass. Further and larger studies are needed to examine if QUS may reflect other aspects than bone mass, or if it is possible to improve its sensitivity by supplementing the SOS results with clinical risk factors., (Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Previous studies proved the importance of rapid antibacterial intervention in case of Pseudomonas aeruginosa detection in respiratory samples of cystic fibrosis patients. To improve the early detection of P. aeruginosa, several culture, PCR and serology based approaches have been compared. Because an increasing number of routine microbiology laboratories have access to real-time PCR (qPCR), we reviewed the specificity and sensitivity of published PCR formats. The importance of choice of DNA-extraction methods and PCR formats and of the validation of their specificity and sensitivity with clinical samples is stressed., (Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Background: Longitudinal data regarding the genotypes of Pseudomonas aeruginosa isolates after eradication treatment are limited. We followed cystic fibrosis patients after a first ever isolation of P. aeruginosa and evaluated the P. aeruginosa-free time period after eradication therapy., Methods: Between January 2003 and December 2008 respiratory samples were cultured prospectively from 41 patients with a first ever P. aeruginosa isolate. Twenty five patients had at least one subsequent isolate. Treatment efficacy was assessed based on the time to a second isolation and on comparison of the RAPD genotypes of the P. aeruginosa isolates., Results: Eleven patients became chronically colonized during the study period. For ten of these the second isolate had the same genotype as the first isolate. Moreover, these patients had a significantly shorter P. aeruginosa-free time interval between the first ever and the second isolate compared to the 14 not chronically colonized patients (median 0 months versus 7.5 months, p<0.05)., Conclusion: Our results indicate that the presence of a genotypically identical subsequent P. aeruginosa isolate and/or a short P. aeruginosa-free time interval after treatment are ominous signs and might be useful additional tools to predict impending chronic colonization. Current routine bacteriological methods for the detection of P. aeruginosa may lack the sensitivity to discriminate between true eradication and low bacterial persistence., (Copyright (c) 2009 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Background: Achromobacter xylosoxidans is increasingly cultured in sputum from cystic fibrosis (CF) patients; nevertheless, there are few published data on the clinical impact of this infection or chronic colonisation., Methods: Relying on DNA fingerprinting techniques we studied the prevalence of A. xylosoxidans in our CF population. In a retrospective case control study the clinical status of patients with at least 3 sputum cultures positive for A. xylosoxidans over at least 9 months, at the moment of the first positive culture and during the period of colonisation were compared to age (+/-1 year), gender and to Pseudomonas aeruginosa colonisation controlled CF patients who had never A. xylosoxidans positive sputum cultures., Results: The prevalence of patients with at least one positive A. xylosoxidans culture was 17.9%. 5.3% of the patients fulfilled the criteria of our definition of colonisation. Colonised patients had a median age of 20 years (range 11-27 years) and a mean colonisation period of 1.5 (+/-0.9) years. At the moment of the first positive culture we found significantly lower Bhalla scores on HRCT scans of the lungs (11+/-3 versus 16+/-3, p<0.002), lower Brasfield chest X-ray scores (14+/-3 versus 18+/-3, p<0.019), lower FVC values (70%+/-22 versus 94%+/-12, p<0.017) and lower FEV(1) values (55%+/-32 versus 78%+/-23, p=0.123), although the latter did not reach significance. There was no significant difference in body mass index (BMI) (18.7+/-3 kg/m2 versus 19.6+/-3 kg/m2, p=0.8). Over the study period A. xylosoxidans-colonised patients did have more need for intravenous antibiotic treatment courses (19 versus 5, p<0.001); nevertheless, there was no significant difference in lung function decline over the study period (FVC: -6.25+/-12.34% versus -5.62+/-8.30%, p 0.77, FEV1: -5.62+/-8.30% versus -1.87+/-11.58%, p<0.47)., Conclusions: The prevalence of A. xylosoxidans infection or colonisation is probably underestimated. Colonised patients are mostly older, with more pronounced lung damage and lower lung function values. Although there was more need for intravenous antibiotic treatment courses, no faster decline in lung function was observed in A. xylosoxidans positive patients.