| 1. |
- Andersson, Tommy
(författare)
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Moment-method calculations of scattering by a square plate using singular basis functions and multipole expansions
- 1993
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Ingår i: Journal Electromagnetic Waves and Applications. - 1569-3937. ; 7:1, s. 93-121
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Tidskriftsartikel (refereegranskat)abstract
- The method of moments is used to solve electromagenetic boundary value problems numerically. It is known that the choice of basis functions is crucial for the numerical efficiency. Fast convergence is achieved provided the basis functions efficiently approximate the unknown function. In this paper the far field (incl. RCS) of a thin square conducting plate is calculated. Basis functions with correct edge and corner singularities are shown to greatly enhance the convergence compared to ordinary "rooftop" functions. The calculations of the matrix elements as well as the right side of the matrix equation and the scattered field are simplified by the use of a multipole technique.
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| 2. |
- Abid, Nosheen, 1993-, et al.
(författare)
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UCL: Unsupervised Curriculum Learning for Water Body Classification from Remote Sensing Imagery
- 2021
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Ingår i: International Journal of Applied Earth Observation and Geoinformation. - : Elsevier. - 1569-8432 .- 1872-826X. ; 105
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Tidskriftsartikel (refereegranskat)abstract
- This paper presents a Convolutional Neural Networks (CNN) based Unsupervised Curriculum Learning approach for the recognition of water bodies to overcome the stated challenges for remote sensing based RGB imagery. The unsupervised nature of the presented algorithm eliminates the need for labelled training data. The problem is cast as a two class clustering problem (water and non-water), while clustering is done on deep features obtained by a pre-trained CNN. After initial clusters have been identified, representative samples from each cluster are chosen by the unsupervised curriculum learning algorithm for fine-tuning the feature extractor. The stated process is repeated iteratively until convergence. Three datasets have been used to evaluate the approach and show its effectiveness on varying scales: (i) SAT-6 dataset comprising high resolution aircraft images, (ii) Sentinel-2 of EuroSAT, comprising remote sensing images with low resolution, and (iii) PakSAT, a new dataset we created for this study. PakSAT is the first Pakistani Sentinel-2 dataset designed to classify water bodies of Pakistan. Extensive experiments on these datasets demonstrate the progressive learning behaviour of UCL and reported promising results of water classification on all three datasets. The obtained accuracies outperform the supervised methods in domain adaptation, demonstrating the effectiveness of the proposed algorithm.
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| 3. |
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| 4. |
- Alexopoulou, Sofia, 1984-
(författare)
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The portrait of older people as (non) users of digital technologies: A scoping literature review and a typology of digital older (non) users
- 2020
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Ingår i: Gerontechnology. - : International Society for Gerontechnology. - 1569-1101 .- 1569-111X. ; 19:3, s. 1-15
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Forskningsöversikt (refereegranskat)abstract
- Background: The images of older people while using (or not) technology is a theme that plays a crucial role not only in the implementation of policies, but also in the design of e-services or more broadly e-governance. Older people is a complex and a non-homogenous group that requires public (welfare) services which in many cases have been moved to a digital interface. The real challenge is to provide these services without excluding anyone.Objective: This paper aims to investigate how older people are represented as (non)users of technology in the digital literature and public discourse and to produce a typology of older digital users based on the work of Schneider and Ingram (1993). Method: The study followed established methods for a scoping literature review to discover the profile of older digital (non) users and their relationship with technology. Results: Based on this literature review, two positive profiles of different power were found: the silver surfers or “athletes” who are proficient digital users and the “older people with borrowed access” to digital technologies who are less powerful and independent while using technology. On the other hand, we also found some negative images of older adults: the “laidback” who are reluctant to use digital technologies but they have the necessary intellectual capacity to acquire IT skills on their own (strong in terms of power). The biggest group entails older people as technophobic, non-users, want-nots, digitally backward/internet laggards, digital immigrants, needy and those who are unaware of their digital condition.Conclusion: This research could offer a substantial contribution to policy-makers and public servants to provide better and friendlier online services, digital tools and applications in conjunction with the supply of IT courses for older individuals.
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| 5. |
- Bahmanyar, Shahram, et al.
(författare)
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Cystic fibrosis gene mutations and gastrointestinal diseases
- 2010
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 9:4, s. 288-291
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Tidskriftsartikel (refereegranskat)abstract
- Background: This study examined if CF mutation heterozygosity is associated with diseases of gastrointestinal epithelial barrier function. Design and methods: Swedish registers identified 865 patients with a diagnosis of CF between 1968 and 2003 and matched with 8101 individuals without CF. Gastrointestinal disease risk was examined among 1534 biological parents and 1396 siblings of CF patients, compared with 15,526 parents and 15,542 siblings of individuals without CF. Results: First-degree relatives of CF patients were not at lower risk of the gastrointestinal diseases, in contrast with a raised risk among CF patients. Conclusion: Heterozygosity for CF gene mutations does not protect against gastrointestinal diseases where impaired barrier function may be relevant. (C) 2010 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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| 6. |
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| 7. |
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| 8. |
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| 9. |
- Bresso, F, et al.
(författare)
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The cystic fibrosis F508del mutation in Crohn's disease
- 2011
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Ingår i: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. - : Elsevier BV. - 1873-5010. ; 10:2, s. 132-132
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)
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| 10. |
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| 11. |
- Carlsson, Malin, et al.
(författare)
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Pseudomonas aeruginosa in cystic fibrosis: Pyocyanin negative strains are associated with BPI-ANCA and progressive lung disease
- 2011
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier Science B.V., Amsterdam.. - 1569-1993 .- 1873-5010. ; 10:4, s. 265-271
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Tidskriftsartikel (refereegranskat)abstract
- The clinical consequence of chronic Pseudomonas aeruginosa colonization in cystic fibrosis (CF) varies between individuals for unknown reasons. Auto-antibodies against bactericidal/permeability increasing protein (BPI-ANCA) are associated with poor prognosis in CF. We hypothesize that there is a correlation between the presence of BPI-ANCA, the properties of the colonizing bacteria and the clinical conditions of the host. We compared isolates of P. aeruginosa from BPI-ANCA positive CF patients who have deteriorating lung disease with BPI-ANCA negative CF patients who are in stable clinical conditions. Epithelial cells (A549) and isolated polymorphonuclear granulocytes (PMNs) were stimulated with the isolates and cell death was analyzed with flow cytometry. We found that the ANCA associated strains in most cases showed pyocyanin negative phenotypes. These strains also induced less inflammatory response than the non-ANCA associated strains as shown by apoptosis and necrosis of epithelial cells and neutrophils. Our results suggest that colonization with strains of P. aeruginosa that induce a weak inflammatory response is associated with unfavorable outcome in CF. We speculate that inadequate control of pathogen proliferation through an insufficient inflammatory response results in a slowly increasing number of bacteria and accumulation of dying PMNs in the airways, contributing to progression in CF lung disease.
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| 12. |
- Carr, S.B., et al.
(författare)
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Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study
- 2022
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993. ; 21:4, s. E221-E231
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Tidskriftsartikel (refereegranskat)abstract
- Background: This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen. Results: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007). Conclusions: This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2. © 2022
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| 13. |
- Castellani, Carlo, et al.
(författare)
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Benchmarks for Cystic Fibrosis carrier screening: A European consensus document
- 2010
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 9:3, s. 165-178
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Forskningsöversikt (refereegranskat)abstract
- This paper presents an overview of the conclusions from an international conference convened to address current issues related to the provision of Cystic Fibrosis carrier screening within Europe. Consensus was not aimed at stating whether such a programme should be implemented. Instead the focus was to provide a framework for countries and agencies who are considering or planning its establishment. The general principles and target population of Cystic Fibrosis carrier screening, advantages and disadvantages, health economics, monitoring and future evaluative and research directions were covered. A range of screening strategies have been assessed and compared: pre-conceptional and prenatal screening; individual and couple screening; sequential and simultaneous sampling or testing. Furthermore, technical issues were examined with respect to the choice of the panel of mutations, its detection rate, sensitivity, management of intermediate 'at-risk' couples, screening approach to different populations and ethnic minorities, and assurance of laboratory quality control. The consensus statement also aims to establish the benchmarks for communicating with health care providers, the general public and potential and actual participants before and after the genetic test. (C) 2010 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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| 14. |
- Castellani, Carlo, et al.
(författare)
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Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice
- 2008
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 7:3, s. 179-96
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Tidskriftsartikel (refereegranskat)abstract
- It is often challenging for the clinician interested in cystic fibrosis (CF) to interpret molecular genetic results, and to integrate them in the diagnostic process. The limitations of genotyping technology, the choice of mutations to be tested, and the clinical context in which the test is administered can all influence how genetic information is interpreted. This paper describes the conclusions of a consensus conference to address the use and interpretation of CF mutation analysis in clinical settings. Although the diagnosis of CF is usually straightforward, care needs to be exercised in the use and interpretation of genetic tests: genotype information is not the final arbiter of a clinical diagnosis of CF or CF transmembrane conductance regulator (CFTR) protein related disorders. The diagnosis of these conditions is primarily based on the clinical presentation, and is supported by evaluation of CFTR function (sweat testing, nasal potential difference) and genetic analysis. None of these features are sufficient on their own to make a diagnosis of CF or CFTR-related disorders. Broad genotype/phenotype associations are useful in epidemiological studies, but CFTR genotype does not accurately predict individual outcome. The use of CFTR genotype for prediction of prognosis in people with CF at the time of their diagnosis is not recommended. The importance of communication between clinicians and medical genetic laboratories is emphasized. The results of testing and their implications should be reported in a manner understandable to the clinicians caring for CF patients.
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| 15. |
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| 16. |
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| 17. |
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| 18. |
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| 19. |
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| 20. |
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| 21. |
- Döring, Gerd, et al.
(författare)
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Clinical trials in cystic fibrosis
- 2007
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 6:2, s. 85-99
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Tidskriftsartikel (refereegranskat)abstract
- In patients with cystic fibrosis (CF), clinical trials are of paramount importance. Here, the current status of drug development in CF isdiscussed and future directions highlighted. Methods for pre-clinical testing of drugs with potential activity in CF patients including relevantanimal models are described. Study design options for phase II and phase III studies involving CF patients are provided, including requiredpatient numbers, safety issues and surrogate end point parameters for drugs, tested for different disease manifestations. Finally, regulatoryissues for licensing new therapies for CF patients are discussed, including new directives of the European Union and the structure of aEuropean clinical trial network for clinical studies involving CF patients is proposed.
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| 22. |
- Edenborough, F. P., et al.
(författare)
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Guidelines for the management of pregnancy in women with cystic fibrosis
- 2008
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 7 Suppl 1, s. S2-32
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Tidskriftsartikel (refereegranskat)abstract
- Women with cystic fibrosis (CF) now regularly survive into their reproductive years in good health and wish to have a baby. Many pregnancies have been reported in the literature and it is clear that whilst the outcome for the baby is generally good and some mothers do very well, others find either their CF complicates the pregnancy or is adversely affected by the pregnancy. For some, pregnancy may only become possible after transplantation. Optimal treatment of all aspects of CF needs to be maintained from the preconceptual period until after the baby is born. Clinicians must be prepared to modify their treatment to accommodate the changing physiology during pregnancy and to be aware of changing prescribing before conception, during pregnancy, after birth and during breast feeding. This supplement offers consensus guidelines based on review of the literature and experience of paediatricians, adult and transplant physicians, and nurses, physiotherapists, dietitians, pharmacists and psychologists experienced in CF and anaesthetist and obstetricians with experience of CF pregnancy. It is hoped they will provide practical guidelines helpful to the multidisciplinary CF teams caring for pregnant women with CF.
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| 23. |
- Ehre, Camille, et al.
(författare)
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Mucus aberrant properties in CF: Insights from cells and animal models
- 2023
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 22:Suppl 1
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Forskningsöversikt (refereegranskat)abstract
- Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus accumulation in the lungs, the intestinal tract, and the pancreatic ducts. Mucins are high-molecular-weight glycoproteins that govern the biochemical and biophysical properties of mucus. In the CF lung, increased mucus viscoelasticity is associated with decreased mucociliary clearance and defects in host defense mechanisms. The link between defective ion channel and abnormal mucus properties has been investigated in studies involving cell and animal models. In this review article, we discuss recent progress toward understanding the different regions and cells that express CFTR in the airways and how mucus is produced and cleared from the lungs. In addition, we reflect on animal models that provided insights into the organization and the role of the mucin network and how mucus and antimicrobial activities act in concert to protect the lungs from invading pathogens.
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| 24. |
- Elidottir, Helga, et al.
(författare)
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Abnormal glucose tolerance and lung function in children with cystic fibrosis. Comparing oral glucose tolerance test and continuous glucose monitoring
- 2021
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 20:5, s. 779-784
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Cystic fibrosis (CF) related diabetes (CFRD) is a common complication of CF. CFRD is associated with declining lung function even before its onset. Regular screening for CFRD using oral glucose tolerance test (OGTT) is recommended. Additionally, continuous glucose monitoring (CGM) has surfaced as a possible surveillance method, but evidence for its use and concordance with OGTT has not been established.METHODS: Children were prospectively recruited at CF center Lund to undergo both intermittent scan CGM (isCGM) and OGTT. Lung function was evaluated by spirometry and multiple breath washout. Demographic and clinical data were collected from the Swedish national CF registry.RESULTS: 32 patients participated in the study, yielding 28 pairs of isCGMs and OGTTs. The OGTTs showed that two patients met the criteria of CFRD, seven had impaired glucose tolerance (IGT) and indeterminate glycemia (INDET) was found in eleven cases. The isCGM percent of measurements >8mmol/L and the number of peaks per day >11 mmol/L have correlations with intermediate OGTT glucose time points, but not the 2hour glucose value. Patients with abnormal glucose tolerance (AGT) had lower lung function than those with normal glucose tolerance demonstrated by both FEV1% predicted and lung clearance index (LCI).CONCLUSION: Correlations can be found between isCGM and OGTT in regards to the latter's intermediate time points. LCI demonstrates as well as FEV1% of predicted, worse lung function in children and adolescents with abnormal glucose tolerance in CF.
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| 25. |
- Fluge, G., et al.
(författare)
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Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients
- 2009
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 8:3, s. 198-202
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Tidskriftsartikel (refereegranskat)abstract
- Background: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. Methods: Transglutaminase-IgA (TGA), endomysium-IgA (EMA) and total IgA in serum were measured in 790 CF patients (48% females, 86% with pancreatic insufficiency). Six patients were diagnosed with CD prior to the Study, all receiving a gluten-free diet. Patients with elevated TGA (>50 Units/mL) and a positive EMA test were offered a gastroscopy obtaining mucosal biopsies from the duodenum. Results: Four new cases of CD were diagnosed. Two additional patients had positive serological tests, but normal biopsies. In total, 10 cases of CD (1.2%, 1:83) indicate a prevalence rate about three times higher than the general prevalence of CD in Norway and Sweden. No CD patients were detected in the Danish CF cohort. Patients diagnosed with untreated CD reported symptoms typical of both CF and CD (poor weight gain, loose and/or fatty stools, fatigue, irritability, abdominal pain). They improved after introduction of a gluten-free diet. Conclusions: Systematic screening for CD in a Scandinavian cohort of CF patients revealed a higher prevalence of CD than in the general population. Clinical signs of CD are difficult to differentiate from CF with malabsorption, and patients may go undiagnosed for a long time. In a population where CD is common we recommend screening for CD in patients with CF. (C) 2009 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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| 26. |
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| 27. |
- Gilljam, Marita, et al.
(författare)
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Lung transplantation in patients with cystic fibrosis and Mycobacterium abscessus infection
- 2010
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 9:4, s. 272-276
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Tidskriftsartikel (refereegranskat)abstract
- Mycobacterium abscessus lung disease is difficult to treat and has been considered a strong relative contraindication to lung transplantation. We performed double lung transplantation in three cystic fibrosis patients with ongoing, and a fourth with recent treatment for Mycobacterium abscessus lung infection. Despite prolonged antibiotic courses and adjustment of immunosuppressive therapy the first three patients developed skin infection and abscesses. At follow-up after 1, 3, 5 and 7 years respectively no patient had evidence of M abscessus infection and all had stable lung function. Lung transplantation in patients with M abscessus lung infection is feasible but may involve severe complications.
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| 28. |
- H, Elidottir, et al.
(författare)
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2D Shear Wave Elastography, a promising screening tool for Cystic Fibrosis liver disease, shows a correlation between vitamin D and liver stiffness
- 2022
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993. ; 21:5, s. 873-877
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Tidskriftsartikel (refereegranskat)abstract
- Background: Liver disease in Cystic Fibrosis (CFLD) is an early complication of CF. Evidence of CFLD is often subclinical and screening is recommended. Screening includes a biochemical work-up and an ultrasound investigation. Non-invasive methods measuring liver stiffness such as shear wave elastography could be beneficial. This study describes the use of 2D Shear Wave Elastography (2D SWE) in screening for CFLD in a clinical setting and explores its correlation to other indicators of CFLD. Furthermore, a relationship between liver stiffness and nutritional status, lung function and glucose tolerance was explored. Material and Methods: A retrospective cohort study was performed at a pediatric CF center. Information was gathered from the patients’ charts and the Swedish national CF registry. The patients included had been evaluated for the presence of CFLD by ultrasound and 2D SWE during 2018-2020. Demographic data as well as data concerning nutritional status, lung function and glucose tolerance were collected. Results: Fifty-one subjects were included with a median age of 11 years. Four children who had biopsy confirmed liver cirrhosis had significantly increased liver stiffness. There was a statistically significant negative correlation between liver stiffness and vitamin D levels and FEV1% predicted respectively. Children with abnormal glucose tolerance had increased liver stiffness compared to their normal glucose tolerant counterparts. Conclusion: Measuring liver stiffness by 2D SWE is a reliable addition to CFLD screening with data comparable to the more conventional ultrasound investigation. Increased liver stiffness is associated with lower vitamin D levels, lower FEV1% predicted and abnormal glucose tolerance.
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| 29. |
- Hillman, Magnus, et al.
(författare)
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Reduced levels of active GLP-1 in patients with cystic fibrosis with and without diabetes mellitus.
- 2012
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 11:2, s. 144-149
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Tidskriftsartikel (refereegranskat)abstract
- Glucagon like peptide 1 (GLP-1) is an incretin hormone released as a bioactive peptide from intestinal L-cells in response to eating. It acts on target cells and exerts several functions as stimulating insulin and inhibiting glucagon. It is quickly deactivated by the serine protease dipeptidyl peptidase IV (DPP-IV) as an important regulatory mechanism. GLP-1 analogues are used as antidiabetic drugs in patients with type 2 diabetes. We served patients with cystic fibrosis (CF, n=29), cystic fibrosis related diabetes (CFRD, n=19) and healthy controls (n=18) a standardized breakfast (23g protein, 25g fat and 76g carbohydrates) after an overnight fasting. Blood samples were collected before meal as well as 15, 30, 45 and 60min after the meal in tubes prefilled with a DPP-IV inhibitor. The aim of the study was to compare levels of GLP-1 in patients with CF, CFRD and in healthy controls. We found that active GLP-1 was significantly decreased in patients with CF and CFRD compared to in healthy controls (p<0.01). However, levels in patients with CFRD tended to be lower but were not significantly lower than in patients with CF without diabetes (p=0.06). Total GLP-1 did not differ between the groups, which points to that the inactive form of GLP-1 is more pronounced in CF patients. The endogenous insulin production (measured by C-peptide) was significantly lower in patients with CFRD as expected. However, levels in non-diabetic CF patients did not differ from the controls. We suggest that the decreased levels of GLP-1 could affect the progression toward CFRD and that more studies need to be performed in order to evaluate a possible treatment with GLP-1 analogues in CF-patients.
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| 30. |
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| 31. |
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| 32. |
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| 33. |
- Knudsen, Per Kristian, et al.
(författare)
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Differences in prevalence and treatment of Pseudomonas aeruginosa in cystic fibrosis centres in Denmark, Norway and Sweden
- 2009
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 8:2, s. 135-142
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Chronic 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? METHODS: 989 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. RESULTS: In 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. CONCLUSIONS: It 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.
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| 34. |
- Kondori, Nahid, 1967, et al.
(författare)
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Development of IgG antibodies to Exophiala dermatitidis is associated with inflammatory responses in patients with cystic fibrosis.
- 2014
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Ingår i: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. - : Elsevier BV. - 1873-5010. ; 13:4, s. 391-399
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Tidskriftsartikel (refereegranskat)abstract
- The clinical importance of airway colonisation by the fungus Exophiala dermatitidis in patients with cystic fibrosis (CF) is unclear. We have previously shown that E. dermatitidis frequently colonises the airways of patients with CF. The aims of the present study were to determine whether patients who are colonised by E. dermatitidis have detectable fungal antigens in the circulation, develop anti-fungal antibodies, and show signs of inflammation and impaired respiratory function.
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| 35. |
- Kongstad, Thomas, et al.
(författare)
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Improved air trapping evaluation in chest computed tomography in children with cystic fibrosis using real-time spirometric monitoring and biofeedback.
- 2013
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Ingår i: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. - : Elsevier BV. - 1873-5010. ; 12:6, s. 559-566
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Tidskriftsartikel (refereegranskat)abstract
- The quality of chest Computed Tomography (CT) images in children is dependent upon a sufficient breath hold during CT scanning. This study evaluates the influence of spirometric breath hold monitoring with biofeedback software on inspiratory and expiratory chest CT lung density measures, and on trapped air (TA) scoring in children with cystic fibrosis (CF). This is important because TA is an important component of early and progressive CF lung disease.
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| 36. |
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| 37. |
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| 38. |
- Naehrlich, L., et al.
(författare)
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Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020
- 2021
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993. ; 20:4, s. 566-577
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Tidskriftsartikel (refereegranskat)abstract
- Background: Viral infections can cause significant morbidity in cystic fibrosis (CF). The current Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic could therefore have a serious impact on the health of people with CF (pwCF). Methods: We used the 38-country European Cystic Fibrosis Society Patient Registry (ECFSPR) to collect case data about pwCF and SARS-CoV-2 infection. Results: Up to 30 June 2020, 16 countries reported 130 SARS-CoV-2 cases in people with CF, yielding an incidence of 2.70/10 0 0 pwCF. Incidence was higher in lung-transplanted patients (n = 23) versus non transplanted patients (n = 107) (8.43 versus 2.36 cases/10 0 0). Incidence was higher in pwCF versus the age-matched general population in the age groups < 15, 15-24, and 25-49 years (p < 0.001), with similar trends for pwCF with and without lung transplant. Compared to the general population, pwCF (regardless of transplantation status) had significantly higher rates of admission to hospital for all age groups with available data, and higher rates of intensive care, although not statistically significant. Most pwCF recovered (96.2%), however 5 died, of whom 3 were lung transplant recipients. The case fatality rate for pwCF (3.85%, 95% CI: 1.26-8.75) was non-significantly lower than that of the general population (7.46%; p = 0.133). Conclusions: SARS-CoV-2 infection can result in severe illness and death for pwCF, even for younger patients and especially for lung transplant recipients. PwCF should continue to shield from infection and should be prioritized for vaccination. (c) 2021 The Authors. Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. This is an open access article under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/ )
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| 39. |
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| 40. |
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| 41. |
- Pressler, Tacjana, et al.
(författare)
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Diagnostic significance of measurements of specific IgG antibodies to Pseudomonas aeruginosa by three different serological methods
- 2009
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 8:1, s. 37-42
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Tidskriftsartikel (refereegranskat)abstract
- The aim of the study was to evaluate three serological methods for their ability to identify CF patients in different infection status especially those at risk of developing chronic Pseudomonas aeruginosa (Pa) infection. Methods: Two ELISA methods: exotoxin A (ExoA) and CF-IgG-ELISA (CF-IgG) and Crossed Immunoelectrophoresis (CIE) were used for measurement of Pa-antibodies in sera from 791 Scandinavian CF patients. Results: 381 patients were cultured negative for Pa in the year before study start, 129 patients were intermittently colonized and 281 patients were chronically infected. The sensitivity of the investigated assays was 96%, 93% and 97%, specificity 89%, 89% and 83% for CIE, ExoA and CF-IgG respectively. The negative predictive value was for CIE 97%, for ExoA 95% and for CF-IgG 98% and positive predictive values 87%, 86% and 80%. Out of the 381 patients cultured negative for Pa, 11 changed status to chronically infected. Twenty-four out of the 129 patients intermittently colonized became chronically infected. The antibody levels in this latter group of patients were significantly higher already at the study start and increased significantly during the study period (p < 0.05). Elevated levels of specific anti-Pseudomonal antibodies showed to be the risk factor for developing chronic P. aeruginosa infection (OR 4.9 and OR 2.7, p < 0.05 for CF-IgG and ExoA). Conclusion: All three serological assays were equally informative. The very high sensitivity of the assays made it possible to characterize patients with different infection status. Elevated levels of specific anti-Pseudomonas antibodies showed to be the risk factor for developing chronic Pa infection. Due to the specificity of the tests, antibiotic treatment based on serology might be considered in selected cases. There is a window of opportunity for suppression and eradication of initial P aeruginosa infection making measurement of specific anti-Pseudomonas antibodies helpful. (c) 2008 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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| 42. |
- Qvist, Tavs, et al.
(författare)
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Epidemiology of nontuberculous mycobacteria among patients with cystic fibrosis in Scandinavia
- 2015
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 14:1, s. 46-52
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Tidskriftsartikel (refereegranskat)abstract
- Background: Nontuberculous mycobacteria (NTM) are an emerging threat to cystic fibrosis (CF) patients but their epidemiology is not well described. Methods: In this retrospective observational study we identified all Scandinavian CF patients with a positive NTM culture from airway secretions from 2000 to the end of 2012 and used national CF databases to describe microbiological and clinical characteristics. Results: During the 13-year period 157 (11%) CF patients were culture positive for NTM at least once. Mycobacterium abscessus complex (MABSC) (45%) and Mycobacterium avium complex (MAC) (32%) were the predominant species with geographical differences in distribution. Younger patients were more prone to MABSC (p < 0.01). Despite treatment, less than one-third of MABSC patients with repeated positive cultures cleared their infection and a quarter had a lung transplant or died. Conclusion: NTM are significant CF pathogens and are becoming. more prevalent in Scandinavia. MABSC and MAC appear to target distinct patient groups. Having multiple positive cultures despite treatment conveys a poor outcome. (C) 2014 The Authors. Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.
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| 43. |
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| 44. |
- Svedberg, Marcus, 1975, et al.
(författare)
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Risk factors for progression of structural lung disease in school-age children with cystic fibrosis
- 2020
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 19:6, s. 910-916
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Tidskriftsartikel (refereegranskat)abstract
- Background: Computed tomography (CT) is used to monitor progression of structural lung disease (SLD) in children with cystic fibrosis (CF). Our goals were to identify the risk factors for the annual progression of SLD and the impacts of airway pathogens on SLD. Method: Seventy-five school-aged children diagnosed with CF underwent 200 CT scans at Gothenburg CF Centre in the period 2003–2015. SLD was evaluated with a quantitative scoring system. Mixed models were used to calculate the yearly progression rates of SLD and FEV1 and to analyse the effects of common airway pathogens in CF. Results: The yearly mean progression (95% CI) rates for total disease (%Dis), bronchiectasis (%Be), and FEV1 were 0.62 (0.38–0.86), 0.43 (0.28–0.58) and −0.16 (−0.18–0.13), respectively. Adjusting for airway pathogens, the yearly mean progression rates for %Dis, %Be and FEV1 were 0.23 (−0.04–0.51), 0.12 (0.00–0.25), and −0.12 (−0.16–0.08), respectively. A single infection with P aeruginosa was associated with significant increase in lung damage, assessed as %Dis (p = 0.044) and%Be (p = 0.0047), but not in FEV1 (p = 0.96). At age of 7 years, there was a good correlation between the extent of SLD and subsequent progression of %Dis (r = 0.63, p = 0.0042) and %Be (r = 0.74, p = 0.0057) while there was no significant correlation between the FEV1 and the rate of decline of FEV1 (r = −0.22, p = 0.12). Conclusion: Intermittent respiratory infections with P aeruginosa were associated with significant SLD but no change in FEV1. More SLD at the age of 7 years signals a higher progression rate of SLD subsequently.
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| 45. |
- Tanner, Lloyd, et al.
(författare)
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Citrullination of extracellular histone H3.1 reduces antibacterial activity and exacerbates its proteolytic degradation
- 2021
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 20:2, s. 346-355
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Cystic fibrosis (CF), involves excessive airway accumulation of neutrophils, often in parallel with severe infection caused by Pseudomonas aeruginosa. Free histones are known to possess bactericidal properties, but the degree of antibacterial activity exerted on specific lung-based pathogens is largely unknown. Neutrophils have a high content of peptidyl deiminase 4 (PADI4), which citrullinate cationic peptidyl-arginines. In histone H3.1, several positions in the NH2-terminal tail are subject to citrullination.METHODS: Full-length and segmented histone subunit H3.1 was investigated for bactericidal activity towards P. aeruginosa (strain PAO1). PADI4-induced citrullination of histone H3.1 was assessed for antibacterial activity towards P. aeruginosa. Next, the effect of neutrophil elastase (NE)-mediated proteolysis of histone H3.1 was investigated. Finally, PADI4, H3.1, and citrullinated H3.1 were examined in healthy control and CF patient lung tissues.RESULTS: Full-length histone H3.1 and sections of the histone H3.1 tail, displayed bactericidal activity towards P. aeruginosa. These antibacterial effects were reduced following citrullination by PADI4 or proteolysis by NE. Interestingly, citrullination of histone H3.1 exacerbated NE-mediated degradation. In CF lung tissue, citrullinated histone H3.1 and PADI4 immunoreactivity was abundant. Degraded histone H3.1 was detected in the sputum of CF patients but was absent in the sputum of healthy controls.CONCLUSIONS: Citrullination impairs the antibacterial activity of histone H3.1 and exacerbates its proteolytic degradation by NE. Citrullination is likely to play an important role during resolution of acute inflammation. However, in chronic inflammation akin to CF, citrullination may dampen host defense and promote pathogen survival, as exemplified by P. aeruginosa.
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| 46. |
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| 47. |
- Wine, J. J., et al.
(författare)
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Progress in understanding mucus abnormalities in cystic fibrosis airways
- 2018
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993. ; 17:2
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Tidskriftsartikel (refereegranskat)abstract
- Normal airways below the carina maintain an essentially sterile environment via a multi-pronged innate defence system that includes mucus clearance via mucociliary clearance and cough, multiple antimicrobials and cellular components including macrophages and neutrophils. In cystic fibrosis (CF), loss of CFTR function compromises these defences, and with present standard of care virtually all people with CF eventually develop mucus accumulation, plugging and chronic infections. This review focuses on how mucus is affected by CFTR loss. (C) 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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| 48. |
- Nordrum, Lene, 1974
(författare)
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Exploring spontaneous-event marking though parallel corpora: Translating English ergative intransitive constructions into Norwegian and Swedish
- 2015
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Ingår i: Languages in Contrast. - : John Benjamins Publishing Company. - 1387-6759 .- 1569-9897. ; 15:2, s. 230-250
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Tidskriftsartikel (refereegranskat)abstract
- English ergative verbs in one-participant constructions such as ‘the door opens’ often correspond to reflexive verbs or -S forms in Norwegian and Swedish. Following Kemmer (1993), such constructions profile spontaneous-event meaning and belong to the middle domain. This study uses corpus methodology to explore spontaneous-event marking in Norwegian and Swedish from the point of view of Kemmer’s suggested two-cycle development path for middle marking in the Scandinavian languages. The results suggest that there are differences in the middle systems in the Scandinavian languages to the effect that Norwegian has completed Kemmer’s two cycles, whereas Swedish has not. Further, there is tentative indication that ergative verbs are on the increase in both Norwegian and Swedish for certain Subjects and in discipline-specific contexts, possibly due to influence from English.
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