Early bronchiectasis in cystic fibrosis detected by surveillance CT

There is emerging evidence that cystic fibrosis lung disease begins early in infancy. Newborn screening allows early detection and surveillance of pulmonary disease and the possibility of early intervention in this life-shortening condition.We report two children with cystic fibrosis who underwent a comprehensive assessment from diagnosis that included measurement of lung function, limited-slice high-resolution CT and BAL performed annually. Early aggressive surveillance enabled significant lung disease and bronchiectasis to be detected during the first few years of life and led to a change in management, highlighting a clinical role for CT scanning during the preschool years in children with cystic fibrosis

Anti-RSV prophylaxis efficacy for infants and young children with cystic fibrosis in Ireland

Rationale: There is limited evidence supporting the routine use of palivizumab in paediatric cystic fibrosis (CF) patients to reduce respiratory syncytial virus (RSV) infection and related hospitalisation. Despite this, anti-RSV prophylaxis is increasingly common. This is the first report from Ireland regarding palivizumab outcomes for children with CF, under 2 years old, despite the greatest prevalence of CF globally. Methods: An audit was performed at a tertiary hospital in Ireland’s mid-West to document all children with CF, <24 months old, who received palivizumab over a five year period and comparision made with all eligible children for the prior five year period who had not received the product (also CF patients). Palivizumab was administered to both cohorts in their first year of life. Hospitalisation rates were compared using Fisher’s exact test. Incidence of RSV and Pseudomonas aeruginosa infection was recorded. Results: A total of 19 patients who received palivizumab were included in the study; comparision was made with a retrospective control group of 30 patients. Prophylactic palivizumab did not prevent hospitalisation for 10/19 patients, 3 of whom were affected by RSV. This was significantly greater than in the control group, where no hospitalisations were recorded (p < 0.0001). P. aeruginosa was isolated in one case from the study cohort, while no P. aeruginosa was detected in the control group. Conclusions: This study, the first of its kind from Ireland where CF prevalence is highest, does not provide unequivocal support for prophylactic use of palivizumab in CF patients under 2 years. Despite being derived from a small sample size, based on these data and complementary clinical observation, we have discontinued such prophylaxis. However, should reported incidence of RSV-related hospitalisation increase, there is scientific plausibility for appropriately powered, randomised, controlled trials of palivizumab

Medical devices for cystic fibrosis care may be portable reservoirs of potential pathogens

no abstract availabl

eOTUs present in one category and absent from the other.

<p>eOTUs present in one category and absent from the other.</p

Richness of the microbial communities present in the control lower airways and the CF upper and lower airways.

<p>Box and Whiskers plot of Shannon's diversity indices for microbial communities present in the control lower airway, CF lower airway and the CF upper airway. * represents statistical significance (p = 0.001; two-tailed t test).</p

Adonis scores for correlations between CF community diversity and discrete variables.

<p><i>The CFTR mutation category was Not Applicable (N/A) as all but one patient was F508del homozygous.</i></p><p><i>* Discrete variables that passed the Adonis test (p<0.05).</i></p><p>Adonis scores for correlations between CF community diversity and discrete variables.</p

Bacterial community structures in the control lower airways and the CF upper and lower airways.

<p>Principle coordinate analysis (PCoA) plots of A) UniFrac distances and B) weighted UniFrac distances of all airway microbial communities were created in R. Each community from each sample is represented as a filled circle and coloured by sample type and/or patient cohort; CF lower (CF BAL; red), CF upper (CF SWB; blue) and control lower (CN BAL; yellow) airway samples. The x-axis and y-axis represent 2-dimensions of percentage variation explained by the PCoA. Ellipses were included for visualisation purposes.</p

Taxa of differential abundance between the control and CF lower airway.

<p>Circular Phylogenetic Tree was rendered in iToL and illustrates abundance changes between eOTUs present in the control lower (inner ring) and CF lower (outer ring) airways. The colour saturation indicates the degree of difference from the mean control lower value for the eOTU; where dark blue indicates a HybScore difference of −52326, white  = 0 (no change from mean control lower), dark red  = +61480. Two eOTUs remained of significantly differential abundance following Benjamini-Hochberg correction; <i>Prevotella veroralis</i> (*) and a CW040 (**). Following a permutation test, 62 taxa were found to be of significantly differential abundance, <i>Corynebacterium</i> (***) is highlighted. A detailed list of the 59 taxa of significantly differential abundance between categories is provided in the supplementary data (<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0109798#pone.0109798.s001" target="_blank">S2 File</a>).</p

A case of failed eradication of cystic fibrosis-related sinus colonisation by Pseudomonas aeruginosa

Background: Pseudomonas aeruginosa is a pathogen associated with cystic fibrosis that has potential to decrease lung function and cause respiratory failure. Paranasal sinuses are increasingly recognised as potential reservoirs for intermittent colonisation by P. aeruginosa. This case documents investigation and outcome of P. aeruginosa recurrence in a male paediatric patient over an eight year period. Case presentation: A 12 year old Irish male paediatric cystic fibrosis patient experienced intermittent culturing of P. aeruginosa from the oropharyngeal region, indicating chronic infection of the sinuses despite absence of symptoms, retaining good lung function, and normal bronchoscopy and bronchoalveloar lavage. However, P. aeruginosa was isolated from a sinus wash-out and was identified as a unique strain of P. aeruginosa that was also cultured from cough swabs. Despite treatment, successful eradication from the paranasal sinuses was not achieved. Conclusions: Few reports have addressed the paranasal sinuses as a reservoir for lung infection in cystic fibrosis patients despite increased recognition of the need to investigate this niche. In this case, attempts at eradication of P. aeruginosa present in paranasal sinuses including oral and nebulised antimicrobials proved unsuccessful. However, detection of P. aeruginosa in the paranasal sinuses instigated antimicrobial treatment which may have contributed to prevention of migration to the lower airways. Our outcome provides additional insight and may indicate utility of nasal lavage or nasal endoscopy in paediatric cystic fibrosis patients’ annual review clinic visits

Baseline demographic and clinical characteristics of all patients included in the study.

<p><i>PA  =  Pseudomonas aeruginosa, SP  =  Streptococcus pneumoniae, MC  =  Moraxella Catarrhalis, SA  =  Staphylococcus aureus, HI  =  Haemophilus influenzae, NF  =  Normal Flora, ICS  =  Inhaled corticosteroids, HS  =  Hypertonic Saline.</i></p><p><i>0 = 0 CFU/ml, 1 = 1 to 100 CFU/ml, 2 = 100 to 10,000 CFU/ml and 3 = >10,000 CFU/ml.</i></p><p>Baseline demographic and clinical characteristics of all patients included in the study.</p