Improvement of digestive health and reduction in proteobacterial populations in the gut microbiota of cystic fibrosis patients using a Lactobacillus reuteri probiotic preparation: A double blind prospective study

BackgroundAlthough scientific knowledge about the benefits of probiotic use in cystis fibrosis (CF) is scarce, their expectative is promising. The aim of this work was to analyze the effect of a Lactobacillus reuteri probiotic preparation versus placebo in CF patients.MethodsA prospective, double blind, crossover and with placebo study was carried out in 30 CF patients from two Spanish hospitals. Patients were randomized in Group A (6 months of probiotic followed by 6 months of placebo) and Group B (6 months of placebo followed by 6 months of probiotic). GIQLI (gastrointestinal) and SF-12 (general) health tests were performed after probiotic and placebo intakes. Fat absorption coefficient, calprotectin, and inflammatory interleukin quantification were determined in fecal samples. Total fecal DNA was obtained and metagenomic 454-pyrosequencing was applied to analyze the microbiome composition. STATA v12 MP software was used for statistical analyses.ResultsStatistically significant improvement in the gastrointestinal health and decrease of the calprotectin levels were demonstrated in patients after probiotic exposure, in comparison with placebo. All CF subjects reported good tolerance to L. reuteri without secondary effects. Metagenomic analysis showed an important dysbiosis in CF gut microbiota associated with a high concentration of Proteobacteria. Probiotic intake was followed by a reduction in the total bacterial density, mostly due to a considerable reduction in the γ-Proteobacteria phylum; and an important increase of the microbial diversity with a higher representation of Firmicutes.ConclusionsProbiotics might ameliorate the dysbiosis of CF gut microbiota, characterized by a high density of Proteobacterial organisms. L. reuteri significantly decrease intestinal inflammation and increase digestive comfort.     

Contribution of HLA DRB1, PTPN22, and CTLA4, to RA dysbiosis

This narrative review gathers current evidence for a contribution of rheumatoid arthritis (RA) HLA-DRB1, PTPN22 and CTLA4 polymorphisms to the gut dysbiosis observed in RA, especially at its onset (transient excess of Prevotella). The gut microbiome contains elements which are 30% heritable, including genera like Bacteroides and Veillonella, and to a lesser extent Prevotella. The first months/year seems a critical period for the selection of a core of microbiota, that should be considered as a second self by the immune system, and tolerized by regulatory T and B cells. Imperfect tolerization may increase the risk of RA following further repeated silent translocations of various gut microorganisms, including Prevotella copri, from gut to joints (fostered by a concurrent loss in gut mucosa of protective bacteria like Faecalibacterium prausnitzii). Genetics studies confirmed that Prevotella copri was partly heritable, and strong associations were observed between the overall microbial composition of stools and the HLA-DRB1 RA risk allele, either in a US cohort (P = 0.00001), or the Twins UK cohort (P = 0.033). This finding also stands for persons still free from RA, and was replicated in the Swiss SCREEN-RA cohort. Gene variants of PTPN22 also modify intestinal microbiota composition, compromise granulocyte-mediated antibacterial defence in gut, and reduce the suppressive effect of gut regulatory B cells. CTLA4 variants may similarly contribute to RA dysbiosis, since immunotherapy by CTLA-4 blockade depends on microbiota, and CTLA4 activates T follicular regulatory cells to reduce immune responses to segmented filamentous bacteria. Suggestions for future works are made.     

Expression of the inflammatory regulator A20 correlates with lung function in patients with cystic fibrosis

BackgroundA20 and TAX1BP1 interact to negatively regulate NF-κB-driven inflammation. A20 expression is altered in F508del/F508del patients. Here we explore the effect of CFTR and CFTR genotype on A20 and TAX1BP1 expression. The relationship with lung function is also assessed.MethodsPrimary nasal epithelial cells (NECs) from CF patients (F508del/F508del, n = 7, R117H/F508del, n = 6) and controls (age-matched, n = 8), and 16HBE14o- cells were investigated. A20 and TAX1BP1 gene expression was determined by qPCR.ResultsSilencing of CFTR reduced basal A20 expression. Following LPS stimulation A20 and TAX1BP1 expression was induced in control NECs and reduced in CF NECs, broadly reflecting the CF genotype: F508del/F508del had lower expression than R117H/F508del. A20, but not TAX1BP1 expression, was proportional to FEV₁ in all CF patients (r = 0.968, p < 0.001).ConclusionsA20 expression is reduced in CF and is proportional to FEV₁. Pending confirmation in a larger study, A20 may prove a novel predictor of CF inflammation/disease severity.     

Small macrophages are present in early childhood respiratory disease

BackgroundRecently, an established “small macrophage” phenotype has been observed in the sputum of patients with CF and COPD. However, little is known about the prevalence of this phenotype in the airways of young children. Since respiratory inflammation begins early in CF, we hypothesised that these small macrophages would be increased in paediatric CF bronchoalveolar lavage (BAL).MethodsMacrophage populations in CF and disease control BAL were assessed by multicolour flow cytometry. BAL inflammatory indices were collected as part of the AREST-CF programme.ResultsSmall macrophages were present in CF (n = 35, mean 36 ± 12% of BAL macrophages) but not significantly different to the respiratory disease controls (n = 7, mean 40 ± 21%). Number of small macrophages correlated significantly with number of BAL neutrophils (r = 0.44, p < 0.01) but not infection or IL-8.ConclusionsIn paediatric patients small macrophages are not unique to CF, but their establishment as the dominant phenotype in adults may be due to chronicity of inflammation and infection.     

Chronic Mycobacterium abscessus infection and lung function decline in cystic fibrosis

BackgroundAlthough nontuberculous mycobacteria (NTM) are recognized pathogens in cystic fibrosis (CF), associations with clinical outcomes remain unclear.MethodsMicrobiological data was obtained from 1216 CF patients over 8 years (481 ± 55 patients/year). Relationships to clinical outcomes were examined in the subset (n = 271, 203 ± 23 patients/year) with longitudinal data.ResultsFive hundred thirty-six of 4862 (11%) acid-fast bacilli (AFB) cultures grew NTM, with Mycobacterium abscessus (n = 298, 55.6%) and Mycobacterium avium complex (n = 190, 35.4%) most common. Associated bacterial cultures grew Stenotrophomonas or Aspergillus species more often when NTM were isolated (18.2% vs. 8.4% and 13.9% vs. 7.2%, respectively, p < 0.01). After controlling for confounders, patients with chronic M. abscessus infection had greater rates of lung function decline than those with no NTM infection (− 2.52 vs. − 1.64% predicted FEV₁/year, p < 0.05).ConclusionsNTM infection is common in CF and associated with particular pathogens. Chronic M. abscessus infection is associated with increased lung function decline.     

A retrospective analysis of biofilm antibiotic susceptibility testing: A better predictor of clinical response in cystic fibrosis exacerbations

BackgroundBacteria grow as biofilms within CF airways. However, antibiotic susceptibility testing is routinely performed on planktonically-growing bacteria. This study assessed whether CF patients infected with multiresistant organisms had improved clinical outcomes if given antibiotics that inhibited their biofilm-grown bacteria.Methods110 patients with pulmonary exacerbations were treated with intravenous antibiotics based on susceptibility testing of planktonically-growing bacteria. A retrospective analysis was done using bacterial isolates grown from their sputum at exacerbation. Each isolate was grown as a biofilm and combination antibiotic susceptibility testing was performed. Clinical outcomes in patients treated with biofilm-susceptible antibiotics were compared to those that were not.Results66 of 110 patients (60%) were treated with antibiotic combinations that inhibited all of their planktonically-grown bacterial isolates, however, when the same isolates were grown as biofilms, only 24 patients (22%) had all of their biofilm-grown isolates remaining susceptible to the antibiotics (P = < 0.001 ). When patients with at least one biofilm-grown susceptible isolate (n = 61) were compared to those with none (n = 49), there was a significant decrease in sputum bacterial density (P = 0.02) and length of stay (P = 0.04) and a non-significant decrease in treatment failure. Survival analyses of time to next exacerbation showed non-significant trends favoring patients treated with biofilm-effective antibiotics.ConclusionsMost patients with CF exacerbations do not receive antibiotics that inhibit all biofilm-grown bacteria from their sputum at exacerbation. Patients treated with biofilm-effective therapy seemed to have improved clinical outcomes.     

Age-related levels of fecal M2-pyruvate kinase in children with cystic fibrosis and healthy children 0 to 10 years old

Background

The pathogenesis of gut inflammation, bacterial dysbiosis and increased rates of malignancy in CF is unclear. Fecal M2-pyruvate kinase (M2-PK) is a biomarker indicative of cellular proliferation that may be raised in intestinal malignancy and inflammation. Biomarkers, including M2-PK, may be useful in assessing effects of novel therapies on the gastrointestinal tract.

Long-term follow-up of cystic fibrosis newborn screening: Psychosocial functioning of adolescents and young adults

BackgroundLong-term psychosocial outcomes of cystic fibrosis (CF) patients diagnosed through newborn screening remain unknown.MethodsThis cross-sectional study compared three groups of youths (16 to 22 years): CF patients diagnosed through NBS (CF-NBS, n = 13), CF patients diagnosed through standard practice (CF-SP, n = 26) and healthy peers (H, n = 42), plus 72 of their parents. We hypothesized that adolescent psychological functioning would be mediated by parent depression and quality of parent–child communication and cohesiveness.ResultsA path analysis showed significantly more depression among CF-NBS group parents (p = .006–.008). Parent–child cohesiveness was related to communication (p < .001). Cohesiveness and communication were associated with youth Internalizing Problems (p = .037, p = .009), Emotional Symptoms (p = 0.018, p = 0.022), and Personal Adjustment (communication only, p = 0.009). Parent depression was related to youth Personal Adjustment (p = 0.022).ConclusionsCF patients report psychosocial function similar to healthy peers. Parents of children diagnosed with CF through NBS may be at risk for depressive symptoms when their children reach adolescence.     

Multidisciplinary management of posterior fossa dural arteriovenous fistula: A single-center experience

PurposeThe management of posterior fossa dural arteriovenous fistulas (pfDAVFs) is challenging. Here, we show how multidisciplinarity leads to their successful management, even in complex cases.MethodsAll pfDAVFs managed from 2010 to 2019 at our center were reviewed. The preoperative clinical and radiological characteristics, their management and the occlusion rate were retrieved. The radiological and functional outcomes were retrieved at discharge and last follow-up (FU).Resultsn = 27 patients were included (6 females, mean age: 61-years-old, mean FU: 22.5 months). n = 8 patients presented with cerebral hemorrhage. Among patients with ruptured pfDAVFs, n = 7 had headache, n = 4 had ataxia, and n = 2 had impaired level of consciousness. In the unruptured group N (n = 19), n = 7 patients had headache, n = 6 patients had focal neurological deficit, n = 4 patients had tinnitus, n = 3 (had ataxia, and one presented with seizure. n = 24 patients were treated by endovascular therapy (EVT), n = 2 patients were treated by microsurgery (MS) and n = 1 patient was managed with a combined approach. Re-treatment was necessary in n = 6 patients. n = 24 patients showed total exclusion at last FU. n = 2 patients died during the first 30 days; n = 1 patient died during FU.ConclusionsWhile EVT should be advocated as the first line therapy whenever possible, MS should not be banned from the treatment armamentarium. Neurosurgeons must be able to achieve direct surgical occlusion when the angioarchitecture speaks against EVT.     

Extraluminal helicoidal stretch (Helixtretch): A novel method of intestinal lengthening

PurposeWe sought to test a novel, extraluminal method of intestinal lengthening that precludes violation of the intestinal wall.MethodsSprague–Dawley rats (n = 45) with size-matched bowel segments isolated by Roux-en-Y reconstruction were divided into three groups. Group 1 (n = 14) had no further manipulations. In Groups 2 (n = 12) and 3 (n = 19), the isolated segment was wrapped around a length-matched device in a helicoidal fashion. In Group 2, the device consisted of plain polyurethane tubing. In Group 3, it consisted of a gradually expanding hygroscopic hydrogel (12.5 mm final diameter). Euthanasia was performed at 8–21 days. Statistical analysis was by two-way ANOVA (P < 0.05).ResultsOverall survival was 87% (39/45). There was a statistically significant increase in bowel length in Group 3 compared to the other two groups (P < 0.001). This increase correlated with the number of helicoidal coils (P = 0.018), but not with post-operative time (P > 0.50). There were no significant differences in total DNA/protein ratio across the groups (P = 0.65). Histologically, there was an apparent increase in the goblet cell density in Group 3.ConclusionsMeasured extraluminal helicoidal stretch (Helixtretch) is tolerated by the intestine. Helixtretch induces bowel lengthening in a rodent model. Further analysis of this novel, minimally invasive alternative for intestinal augmentation is warranted.     

Mycobacterium avium and Mycobacterium abscessus complex target distinct cystic fibrosis patient subpopulations

BackgroundClinical observations suggest that Mycobacterium avium complex (MAC) and Mycobacterium abscessus complex (MABSC) may affect cystic fibrosis (CF) patients with different characteristics and risk factors, but this has never been demonstrated within a single prospective cohort.MethodsWe studied 50 MABSC-positive and 23 MAC-positive patients from a French prevalence study of non‐tuberculous mycobacteria (NTM) in CF. Risk factors specifically associated with MABSC and MAC were analyzed by nested case–control studies, with two NTM-negative controls matched by age, sex and center for each case.ResultsMAC-positive patients were significantly older than MABSC-positive patients (mean [SD] age, 23.1 [10.2] vs 17.4 [8.3] years, p = 0.013), and were also older at CF diagnosis (mean [SD] age, 12.9 [16.1] vs 3.1 [7.7] years, p = 0.015); they tended to be less frequent of the ΔF508/ΔF508 genotype (33.3 vs 61.1%, p = 0.17) and to use pancreatic extracts less frequently (82.4 vs 97.6%, p = 0.07). Risk factors identified by multivariate analysis were: i) in the MAC case–control study, an older age at CF diagnosis (p = 0.004); ii) in the MABSC case–control study, at least one course of intravenous antibiotics (p = 0.01) and more frequent isolation of Aspergillus (p = 0.03).ConclusionsMAC affects adult patients with a mild form of CF, whereas MABSC affects younger patients with more severe CF and more frequent intravenous antimicrobial treatment.     

Lung clearance index during hospital admission in school-age children with cystic fibrosis

BackgroundThere is currently limited information regarding lung clearance index (LCI) and its response to treatment of pulmonary exacerbations in CF. We aimed to examine the utility of LCI for assessing short term clinical response to IV antibiotic therapy in school-age children with CF.MethodsSubjects experiencing exacerbations and hospitalised for IV antibiotics performed both multiple breath nitrogen washout (MBNW) and spirometry on admission to hospital and prior to discharge.Results27 patients (aged 6–20 years) had paired data for MBNW and spirometry. Mean LCI reduced from 12.18 to 11.65 (4.4%) by time of discharge and FEV₁ z-score improved from − 3.05 to − 2.86 (6.2%). Overall, LCI improved in n = 15 (55%) patients compared with n = 18 (67%) where FEV₁ improved.ConclusionsIn summary, these findings do not support the use of LCI (or indeed, FEV₁) to gauge the short term clinical response to IV antibiotic therapy in school-age children with cystic fibrosis.     

Levels of moderate–vigorous physical activity are low in Spanish children with cystic fibrosis: A comparison with healthy controls

BackgroundPhysical activity (PA) is recommended as part of the treatment regimen for cystic fibrosis (CF) although objective methods have been scarcely used to monitor achievement of PA guidelines.MethodsPA was measured by accelerometer in outpatient CF children (n = 47) and results were compared with those obtained in age- and gender-matched healthy controls (n = 39).Results2.1% of the outpatients fulfilled PA guidelines (i.e. ≥ 60 min·day^− 1 of moderate-to-vigorous PA (MVPA)) vs. 34.2% of controls. Overall, lower MVPA levels were observed in CF patients than controls despite the former undergoing more light or total PA. Peak oxygen uptake was also lower in the CF group than in controls (37.5 ± 7.8 vs. 43.5 ± 7.6 ml·kg^− 1·min^− 1, p = 0.002) and was correlated with MVPA and vigorous PA in the former.ConclusionsThese findings support a need to promote PA interventions (including MVPA) targeted at improving cardiorespiratory fitness in CF children.     

Development of a quantitative immunofluorescence assay for detection of Stenotrophomonas maltophilia antibodies in patients with cystic fibrosis

BackgroundTo describe a simple quantitative immunofluorescence assay (IFA) for the detection of specific Stenotrophomonas maltophilia antibodies in serum of CF patients.MethodsA total of 100 sera (64 CF patients and 36 healthy subjects) were collected over a period of 2 years at the University Hospital Essen, Germany. Sputum culture status classified CF patients into groups. Serologic response was determined after Pseudomonas aeruginosa absorption by indirect IFA to Sm whole cell.ResultsCF patients with “chronic S. maltophilia” showed significantly higher S. maltophilia antibody levels compared with healthy individuals (P < 0.0001) and CF patients with “intermittent” (P = 0.0315) or “never S. maltophilia/P. aeruginosa” (P = 0.0002). A discriminant cut-off value of > 1:120 titre was established to differentiate “CF chronic S. maltophilia” from the other groups. For “CF chronic S. maltophilia”, the IFA showed sensitivity and specificity values of 70.7% and 84.7%, respectively.ConclusionOur data demonstrated that quantitative IFA is a simple serological assay for the detection of specific S. maltophilia antibodies, which could be useful as a diagnostic tool for monitoring immune response of CF patients to S. maltophilia.     

Health related quality of life in cystic fibrosis: To work or not to work?

BackgroundThe present study investigated whether patients with CF who are studying or working report a better HRQoL in comparison to non-working/studying patients.Methods57 adult CF patients completed the Cystic Fibrosis Questionnaire—Revised, a CF-specific measure of HRQoL. Medical condition was quantified in terms of FEV1 % predicted, BMI, Pseudomonas aer. status, pancreatic status (PS), having an indwelling catheter device (PAC), CF Related Diabetes Mellitus and nutritional status.ResultsMean age was 26.7 years (SD 8.1), mean FEV1 % predicted was 65.09 (SD 22.18), mean BMI was 21.23 (SD 3.45). FEV1 % predicted was related to HRQoL domains Physical Functioning and General Health (r = 0.27 and 0.38 respectively, p < 0.05). A higher BMI was associated with better scores on Eating Disturbances (= fewer problems; r = 0.44, p < 0.01) and a better perception of Weight (r = 0.43, p < 0.01) and Body Image (r = 0.28, p < 0.05). Analysis of variance showed that specific domains of HRQoL were related to diabetes (Weight), taking caloric supplements (Body Image and Weight) and/or PAC (Physical Functioning, Treatment Burden, Role, Weight). Twenty-four patients worked/studied, these patients had a higher FEV1, and fewer had Pseudomonas aer. or a PAC or took high caloric supplements, compared to non-working/studying patients. After controlling for medical parameters, patients who were working/studying scored higher than non-working/studying patients on Physical Functioning, Role Functioning and Social Functioning.ConclusionsCF patients' HRQoL is related to medical status. The non-working/studying CF patients in this sample had greater disease severity and reported a lower quality of life than their working/studying peers, even after controlling for relevant medical parameters. The decision to stop work/study for CF patients is difficult and affects patients' personal, social and financial well-being.     

Blunt splenic injury: Outcomes of proximal versus distal and combined splenic artery embolization

PurposeTo assess clinical outcomes of blunt splenic injuries (BSI) managed with proximal versus distal versus combined splenic artery embolization (SAE).Materials and methodsAll consecutive patients with BSI admitted to our trauma centre from 2005 to 2010 and managed with SAE were reviewed. Outcomes were compared between proximal (P), distal (D) or combined (C) embolization. We focused on embolization failure (splenectomy), every adverse events occurring during follow up and material used for embolization.ResultsFifty patients were reviewed (P n = 18, 36%; D n = 22, 44%; C n = 8, 16%). Mean injury severity score was 20. The technical success rate was 98%. Four patients required splenectomy (P n = 1, D n = 3, C n = 0). Clinical success rate for haemostasis was 92% (4 re-bleeds: P n = 2, D n = 2, C n = 0). Outcomes were not statistically different between the materials used. Adverse events occurred in 65% of the patients during follow up. Four percent of the patients developed major complications and 56% developed minor complications attributable to embolization. There was no significant difference between the 3 groups.ConclusionSAE had an excellent success rate with adverse events occurring in 65% of the patients and no significant differences found between the embolization techniques used. Proximal preventive embolization appears to protect in high-grade traumatic injuries.     

Minimally invasive radiologic techniques in the treatment of uretero-enteric fistulas

ObjectivesThe goal of this study was to assess the efficacy of minimally invasive interventional radiologic (IR) techniques in the management of uretero-enteric fistulae in comparison to established surgical modalities.Materials and methodsTwenty-five patients (16 men, 9 women) with a mean age of 47 (range: 19–77 years) with uretero-enteric fistulae were treated with percutaneous nephrostomy, double “J” stent, radiologic uretero-neocystostomy, and radiologic uretero-pyelocalicostomy. All patients had a single fistula each. Uretero-enteric fistulas were due to direct or iatrogenic trauma in 14 patients (uretero-ileal fistulas, n = 6; uretero-colonic fistulas, n = 4; uretero-duodenal fistulas, n = 2; uretero-pancreatic fistula, n = 1; uretero-fallopian tube, n = 1), complications of pelvic neoplasms in 4 patients (uretero-sigmoid fistulas, n = 4), inflammatory disease in 4 patients (uretero-ileal fistulas, n = 2; uretero-sigmoid fistulas, n = 2), and avascular necrosis of renal transplants in 3 patients (uretero-sigmoid fistulas, n = 3).ResultsDrainage by percutaneous nephrostomy and double “J” stent resulted in closure of 8 uretero-enteric fistulae over 7–16 weeks. Four uretero-enteric fistulae obliterated after re-routing urine flow using 3 radiologic uretero-neocystostomies and one IR pyelocalicostomy. In other patients, flow through the fistulae was substantially decreased by five double “J” stents and 3 percutaneous nephrostomies. The duration of inpatient hospitalization was significantly less for patients managed successfully by IR procedures than those treated by surgical modalities, 5.07 versus 10.5 days mean (P < 0.05).ConclusionsIR procedures provided definitive treatment in 48% of uretero-enteric fistulae at significantly reduced inpatient hospitalization and cost. As palliative treatment, it improved the quality of life.     

Daily serum creatinine monitoring promotes earlier detection of acute kidney injury in children and adolescents with cystic fibrosis

BackgroundThe epidemiology of aminoglycoside-associated acute kidney injury (AG-AKI) has not been well described in pediatric patients with cystic fibrosis (CF). We aimed to assess the impact of daily serum creatinine (SCr) measurement on detection of AG-AKI at our institution.MethodsWe examined a cohort of hospitalized patients with CF who received an intravenous (IV) aminoglycoside for ≥ 3 days. We compared the rate, timing, and medical management surrounding detection of AG-AKI during 2 periods: January 2010–May 2011 (Era 1, SCr measured at the discretion of the medical team, N = 124) and June 2011–June 2012 (Era 2, SCr measured daily, N = 103). Our primary outcome was detection of AG-AKI defined as ≥ 50% increase in SCr from baseline (lowest value in prior 6 months), or ≥ 0.3 mg/dL rise within 48 h, occurring after day 2.ResultsThe use of once daily tobramycin (p = 0.02) and IV fluids (p < 0.001) was higher during Era 2, while AG courses were shorter (p = 0.04), and fewer concomitant nephrotoxins (p = 0.04) were given; higher daily tobramycin doses (p < 0.001) were administered. Although the rate of AG-AKI was not significantly different (12% during Era 1 vs. 20% during Era 2, p = 0.09), the number of AG-AKI days detected increased (5.5 vs. 2.9 per 100 AG days, p = 0.003), and detection occurred earlier (median 6 vs. 9 days, log rank test p = 0.02) during the daily SCr period.ConclusionsDaily SCr measurement promoted earlier and increased detection of AG-AKI in patients with CF at our institution. We suggest systematic evaluation for AKI during aminoglycoside administration in patients with CF.     

Non-invasive liver elastography (Fibroscan) for detection of cystic fibrosis-associated liver disease

BackgroundCystic fibrosis-associated liver disease (CFLD) is the second cause of mortality in CF. The prevalence is estimated to be 26–45%, but sensitive diagnostic tools are lacking.We investigated whether non-invasive liver elastography (Fibroscan) could serve as a screening tool.MethodsFibroscan measurements were performed in 66 CF patients. Age-specific cutoff values were determined in a control population (n = 59). The measurements were compared to clinical data, biyearly biochemistry and ultrasound.ResultsFibroscan was easy to perform in this patient population. There were 14 patients (21%) with abnormal liver stiffness measurements.Liver stiffness was significantly increased in patients with clinical CFLD (11.2 kPa versus 5.1 kPa), biochemical CFLD (7.4 kPa versus 5.4 kPa) or ultrasonographical CFLD (8.2 versus 4.3 kPa) (p < 0.02 for all).ConclusionsFibroscan is an objective measure and is easy to perform in CF patients, even in children and could provide a valuable tool to detect, and quantify CFLD.     

Differences in prevalence and treatment of Pseudomonas aeruginosa in cystic fibrosis centres in Denmark,Norway and Sweden

BackgroundChronic Pseudomonas aeruginosa (PA) infection causes increased morbidity and mortality in cystic fibrosis (CF). This study aimed to answer the following questions: Does the prevalence of chronic infection with PA differ between the CF centres in Scandinavia? Which differences exist concerning segregation and treatment of PA?Methods989 patients (86%) from all eight CF-centres in Scandinavia were included. Demographic and clinical data, including PA colonisation status based on cultures and serology, were recorded at inclusion. The patients were followed prospectively for 1 year, recording number of days with anti-PA antibiotic treatment.ResultsIn all pancreatic insufficient (PI) patients (n = 890) the prevalence of chronic PA infection at each centre ranged from 25.8% to 48.9%, but were not significantly different. In PI patients < 19 years the prevalence was 14.5% in Copenhagen compared to 30.9% in the Swedish centres pooled (p = 0.001). In intermittently colonised PI patients < 19 years the median number of days per year on anti-PA antibiotics was almost 6 times higher in Copenhagen (mean 86 (110), median 61 days) compared to the Swedish centres pooled (mean 27 (52), median 11 days) (p = 0.037). The pulmonary function was similar.ConclusionsIt is possible to maintain a very low prevalence of chronic PA infection in CF patients < 19 years. We speculate that this was most likely due to a very intensive treatment of intermittently colonised patients with inhaled anti-PA antibiotics over prolonged periods of time in some centres. Since lung function was similar in centres with less intensive use of inhaled antibiotics, studies comparing different treatment modalities and other parts of CF care are needed to define the best clinical practice, including how to use antibiotics in the most rational way.