| 1. |
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| 2. |
- Davies, Julia R., et al.
(författare)
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Identification of MUC5B, MUC5AC and small amounts of MUC2 mucins in cystic fibrosis airway secretions
- 1999
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Ingår i: Biochemical Journal. - 0264-6021. ; 344:2, s. 321-330
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Tidskriftsartikel (refereegranskat)abstract
- To investigate the genetic identities of the mucins secreted in cystic fibrosis (CF) airways, sputum was collected from five individuals. Samples were separated into gel and sol phases by high-speed centrifugation and the gel phase was extracted in 6 M guanidinium chloride. The 'insoluble' residue remaining after extraction of the gel phase was brought into solution by reduction/alkylation. Density-gradient centrifugation in CsCl revealed polydisperse distributions of sialic acid-containing mucins in the gel phase, insoluble residue and sol phase fractions and the degree of variation between the different individuals was low. Antibodies recognizing MUC5AC and MUC5B identified these mucins in each of the fractions. MUC2, however, was present only as a component of the insoluble residue from the gel which accounted for less than 4% by mass of the total mucins, MUC5B and MUC5AC from the gel phase were large oligomeric species composed of disulphide-bond linked subunits and MUC5B was present as two populations with different charge densities which are likely to correspond to MUC5B 'glycoforms'. The sol phase contained, in addition to MUC5AC and MUC5B mainly smaller mucins which did not react with the antisera and which were probably degraded. MUC5AC appeared to be enriched in the sol, suggesting that this mucin may be more susceptible to proteolytic degradation than MUC5B. The mucins present in sputum remained broadly similar during acute exacerbation and following antibiotic treatment, although the relative amount of an acidic MUC5B glycoform was decreased during infection.
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| 3. |
- Dennersten, Ulrika, et al.
(författare)
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Lung function in the aging Swedish cystic fibrosis population.
- 2009
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Ingår i: Respiratory medicine. - : Elsevier BV. - 1532-3064 .- 0954-6111. ; 103:7, s. 1076-82
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Tidskriftsartikel (refereegranskat)abstract
- Swedish Cystic Fibrosis (CF) care follows international guidelines in general. The only difference in our CF care package since 25 years is the physiotherapy regimen. Airway clearance therapy has since the early 1980s, from the day of diagnosis, been based upon daily physical exercise in conjunction with techniques for transporting and evacuating mucus. Postural Drainage+/-percussion and vibration has not been used in any age. The aim of this study was to evaluate our CF care package. Lung function data from the start of the study and with a 3-year interval were collected in the entire Swedish CF population > or =7 years old. Data were analysed for the age groups 7-17 and > or =18 years of age. Change of lung function over the study period was calculated. The impact of chronic Pseudomonas aeruginosa (Pa) colonisation and basal FEV(1)%p was also evaluated in a linear mixed model. Data from 99% of the country-wide CF population were available at inclusion. Mean FEV(1)%p was 90+/-21 vs 73+/-26 in the different age groups and mean VC%p was 94+/-18 vs 91+/-20. Forty percent of the adult group was > or =30 years old. Overall, 41% were chronically Pa colonised. Mean annual FEV(1)%p rate of decline was 0.77 and 0.64 in the different age groups. Lung function among Swedish CF patients is good and annual rate of decline low, even in an old cohort. The large proportion of adult patients emphasises future demands on CF care.
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| 4. |
- Fagevik Olsén, Monika, 1964, et al.
(författare)
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Long COVID - respiratory symptoms in non-hospitalised subjects - a cross-sectional study
- 2023
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Ingår i: European Journal of Physiotherapy. - : Informa UK Limited. - 2167-9169 .- 2167-9177. ; 25:5, s. 283-290
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Tidskriftsartikel (refereegranskat)abstract
- Background: The aim of this study was to describe and analyse the variety of respiratory appearances in Long COVID subjects who were not hospitalised during the acute phase of the infection. Methods: A consecutive series of 60 subjects participated (x) over bar 10.8 months (SD 4.5) after the acute phase of the infection. Respiratory function was tested concerning lung volumes, expiratory flow, muscle strength, physical capacity including concurrent oxygen saturation, chest expansion, lung sounds, pain and breathing pattern. Differences between those with or without positive test and duration of symptoms more or less than 6 months were analysed with T-test, Chi-square test and Fisher's exact test. Results: Decreased forced vital capacity was found in 6/60 (10%), and forced expiratory volume in 1 s and 7/60 (12%), low maximal inspiratory pressure in 38/58 (54%) and low maximal expiratory pressure in 10/58 (17%). Decreased physical capacity was registered in 36/52 (69%), and thoracic expansion in 26/46 (56%). Pathologic lung sounds had 15/58 (26%) and six patients desaturated during the test of physical capacity. A majority (36/58, 67%) presented pain in the ribcage. All but three patients (95%) showed a dysfunctional breathing pattern in sitting and standing. Only poor and fair correlations were found between age, duration and level of physical capacity compared to spirometry, respiratory muscle strength and thoracic expansion. Conclusion: Abnormal breathing pattern and respiratory movements as well as pain, and reduced lung volumes, flow, respiratory muscle strength, physical capacity and thoracic expansion may be involved in Long COVID. The breathing symptoms should therefore be looked for in a wider picture beyond spirometry and oximetry.
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| 5. |
- Fagevik Olsén, Monika, 1964, et al.
(författare)
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Positive expiratory pressure : common clinical applications and physiological effects
- 2015
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Ingår i: Respiratory Medicine. - : Elsevier BV. - 0954-6111 .- 1532-3064. ; 109:3, s. 297-307
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Forskningsöversikt (refereegranskat)abstract
- Breathing out against resistance, in order to achieve positive expiratory pressure (PEP), is applied by many patient groups. Pursed lips breathing and a variety of devices can be used to create the resistance giving the increased expiratory pressure. Effects on pulmonary outcomes have been discussed in several publications, but the expected underlying physiology of the effect is seldom discussed.The aim of this article is to describe the purpose, performance, clinical application and underlying physiology of PEP when it is used to increase lung volumes, decrease hyperinflation or improve airway clearance.In clinical practice, the instruction how to use an expiratory resistance is of major importance since it varies. Different breathing patterns during PEP increase or reduce expiratory flow, result in movement of EPP centrally or peripherally and can increase or decrease lung volume. It is therefore necessary to give the right instructions to obtain the desired effects. As the different PEP techniques are being used by diverse patient groups it is not possible to give standard instructions. Based on the information given in this article the instructions have to be adjusted to give the optimal effect. There is no consensus regarding optimal treatment frequency and number of cycles included in each treatment session and must also be individualized.In future research, more precise descriptions are needed about physiological aims and specific instructions of how the treatments have been performed to assure as good treatment quality as possible and to be able to evaluate and compare treatment effects. (c) 2014 Elsevier Ltd. All rights reserved.
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| 6. |
- Fagevik Olsén, Monika, 1964, et al.
(författare)
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Technical Aspects of Devices and Equipment for Positive Expiratory Pressure With and Without Oscillation
- 2021
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Ingår i: Respiratory care. - : Daedalus Enterprises, Inc.. - 0020-1324 .- 1943-3654. ; 66:5, s. 862-877
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Forskningsöversikt (refereegranskat)abstract
- BACKGROUND: Breathing exercises with positive expiratory pressure (PEP) and oscillating PEP are common treatments for patients with respiratory impairments. There are several trials evaluating the clinical effects of a variety of commercially available and self-made devices. There is a lack of evaluation concerning technical aspects and construction of the devices. The aims of this review were to describe and compare technical aspects of devices and equipment used for PEP and oscillating PEP as a basis for clinical decisions regarding prescriptions.METHODS: In this systematic review, we included trials evaluating different technical aspects of devices and equipment for PEP and oscillating PEP until June 2019. The literature search was performed in PubMed, CINAHL, Cochrane Library, Embase and PEDro.RESULTS: The literature search resulted in 812 studies, which, after being read by 2 independent reviewers, were reduced to 21 trials that matched the inclusion criteria. The achieved PEP is dependent on the given resistance or achieved expiratory flow through the devices and their separate parts. Oscillation frequency in oscillating PEP devices affects the pressure and oscillation amplitude and flow. For some devices, the device's position also has an impact on the outcome. There are similarities and differences among all of the devices, and the equipment components are not interchangeable without changing the achieved PEP levels.CONCLUSIONS: Many devices are available to provide PEP and oscillating PEP treatment. These devices differ substantially in design as well as in performance. When using PEP devices, it is important to understand how all parts of the devices affect outcomes. An increased understanding of how PEP is produced for the spontaneously breathing patient is important to achieve desired treatment effects.
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| 7. |
- Fagevik Olsén, Monika, 1964, et al.
(författare)
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Variations in respiratory and functional symptoms at four months after hospitalisation due to COVID-19: a cross-sectional study
- 2024
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Ingår i: BMC PULMONARY MEDICINE. - 1471-2466. ; 24:1
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Tidskriftsartikel (refereegranskat)abstract
- Background Much remains unknown about complex respiratory symptoms after COVID-19. Here we aimed to describe and analyse patients' various respiratory symptoms 4 months after discharge from hospitalisation for COVID-19, focusing on sex, previous pulmonary disease, and prolonged mechanical ventilation. Methods This cross-sectional study involved five hospitals and included 52 patients with self-assessed respiratory dysfunction at 4 months after discharge from hospitalisation for severe COVID-19. Their average age was 63 years, 38% were women, 15 had a previous diagnosed pulmonary disease, and 29 were current or previous smokers. Additionally, 31 had required intensive care-among whom 21 were intubated and 11 needed mechanical ventilation for >= 20 days. Respiratory function was tested concerning lung volumes, expiratory flow, muscle strength, physical capacity (including concurrent oxygen saturation), thoracic expansion, and respiratory movements. Results Among 52 patients, 47 (90%) had one or several objectively measured respiratory function abnormalities. Decreased thoracic expansion was observed in 32 patients (62%), abnormal respiratory movements in 30 (58%), decreased vital capacity in 21 (40%), low physical function in 13 (26%), and desaturation during the test in 9 (17%). Respiratory inspiratory muscle strength was more commonly diminished than expiratory strength (27% vs. 8%). We did not observe differences between men and women, or between patients with versus without diagnosed pulmonary disease, except that those with pulmonary disease had significantly lower physical capacity assessed with 6MWD (70% vs. 88% predicted, p = 0.013). Compared to those who did not, patients who required >= 20 days of mechanical ventilation performed similarly on most tests, except that all thoracic breathing movements were significantly smaller (p < 0.05). The numbers and combinations of abnormal findings varied widely, without clear patterns. Conclusion Patients with remaining respiratory symptoms 4 months after discharge from hospitalization due to COVID-19 may suffer from various abnormal breathing functions, and dysfunctional breathing that is not detected using traditional measurements. These patients may benefit from multidimensional measuring of breathing movement, thoracic expansion, and respiratory muscle strength, along with traditional measurements, to assess their symptoms and enable prescription of optimal treatment interventions and rehabilitation.
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| 8. |
- Jovic, Sandra, et al.
(författare)
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The neutrophil-recruiting chemokine GCP-2/CXCL6 is expressed in cystic fibrosis airways and retains its functional properties after binding to extracellular DNA.
- 2016
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Ingår i: Mucosal Immunology. - : Elsevier BV. - 1933-0219. ; 9:1, s. 112-123
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Tidskriftsartikel (refereegranskat)abstract
- Infections in cystic fibrosis (CF), often involving Pseudomonas aeruginosa, result from a dysregulated airway immunity where one hallmark is the accumulation of necrotic and apoptotic immune cells, in particular neutrophils. In addition, neutrophils actively release DNA, forming neutrophil extracellular traps (NETs) that contain antimicrobial proteins. Altogether, free DNA in complex with actin accumulates in the airway lumen, resulting in highly viscous sputum that provides an anionic matrix, binding cationic antimicrobial proteins. In this study, granulocyte chemotactic protein 2 (GCP-2)/CXCL6, a neutrophil-activating chemokine with bactericidal properties, was detected in the airway epithelium of CF patients and was also present in azurophilic and specific granules of neutrophils. Elastase of neutrophils, but not of P. aeruginosa, completely degraded CXCL6 (chemokine (C-X-C motif) ligand 6). In addition, CXCL6 colocalized with extracellular DNA in both CF sputa and in in vitro-formed NETs. In vitro, CXCL6 bound DNA with a KD of 2,500 nM. Interestingly, both the bactericidal and the receptor-activating properties of CXCL6 (against neutrophils) remained largely unaffected in the presence of DNA. However, the chemotactic properties of CXCL6 were reduced by the presence of DNA. Taken together, CXCL6 is expressed in CF, retaining its functional properties even after binding to the anionic scaffold that extracellular DNA provides in CF.Mucosal Immunology advance online publication, 20 May 2015; doi:10.1038/mi.2015.43.
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| 9. |
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| 10. |
- Lannefors, Louise
(författare)
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Cystic Fibrosis - long term results of a treatment package including preventive physical exercise
- 2010
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Cystic Fibrosis (CF) is a severe hereditary disorder leading to progressive deterioration of lung function and death. This thesis describes the demography of CF in Sweden, its treatment, with special reference to physiotherapy, and its effects on lung function and peak working capacity (PWC). The study is based on Swedish CF patients and evaluates our current CF treatment package, which differs from those in use in other countries in that it includes preventive physical exercise from the day of diagnosis as an essential part of physiotherapy. Airway clearance therapy based upon physical exercise has been used since the early 1980s, when it replaced the previous airway clearance technique, which was postural drainage with percussion and vibrations. Study I used inhaled radioactive aerosols to compare the effect on mucus mobilisation of three different airway clearance techniques, of which one was based upon physical exercise. There were no significant differences between the techniques. We found increased mucus mobilisation in the dependent lung during postural drainage in the lateral position. Ventilation has a central role in mucus mobilisation and we felt that it was justifiable to continue with physical exercise as the basis for airway clearance therapy. Study II evaluated the demography of the Swedish CF population, its mortality rate and social situation. Survival rate for CF patients in Sweden is good, the median age of the population is high, the age at death is high, and the mortality rate is low. 75% of all patients who have finished school are in full or part time work, and 22% of all adults have at least one child. Studies III and IV investigate the physical condition of patients ≥7 years of age. They include summaries of lung function and PWC and their rates of change in different sub-groups over a 3 year period. Lung function and PWC were good in all age groups with no gender difference and with teenagers at least equally good as other age groups. The rate of decline in lung function and PWC was low. The proportion chronically colonised with Pseudomonas aeruginosa was low, but associated with worse lung function and PWC, and a more rapid rate of decline. These studies show that the Swedish treatment package, which includes preventive physical exercise, is good in an international perspective. It was not however possible to evaluate whether there is a mechanism that make PWC influence Pseudomonas colonisation. Further long term studies are desired.
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| 11. |
- Lannefors, Louise, et al.
(författare)
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Demographic transition of the Swedish cystic fibrosis community--results of modern care.
- 2002
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Ingår i: Respiratory Medicine. - : Elsevier BV. - 1532-3064 .- 0954-6111. ; 96:9, s. 681-685
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Tidskriftsartikel (refereegranskat)abstract
- Assessing the results of modern cystic fibrosis (CF)-care and estimating the future population and its demography is important to evaluate the treatment regimens and to calculate the future needs of health-care resources.This paper updates previous incidence calculations. It assesses the results of modern CF-care in terms of survival and changing demography in Sweden. The incidence of CF in Sweden was calculated as 1/5600 live-births. Of the CF-population alive in 1999, 45% were > or = 18 years old. The mean annual mortality rate since 1991 was 0.9% (+/-0.4) and the median age at death 26 years (range 0-72). Of those born > or = 1991, 95% were estimated to survive their 25th birthday. The incidence of CF in Sweden is low. Modern CF-care in Sweden shows good results. The CF-population is growing rapidly and the adult part of the population will soon be larger than the paediatric. Continuously adapted resources are required to assure the future treatment quality especially for the growing adult CF-population.
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| 12. |
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| 13. |
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| 14. |
- Sinderholm Sposato, Niklas, 1977, et al.
(författare)
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Effectiveness of manual therapy interventions in cystic fibrosis care: a pilot study
- 2024
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Ingår i: JOURNAL OF BODYWORK AND MOVEMENT THERAPIES. - 1360-8592 .- 1532-9283. ; 39, s. 323-329
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Tidskriftsartikel (refereegranskat)abstract
- Background: Cystic fibrosis (CF) is a severe genetic condition that affects multiple organ systems and imposes a substantial treatment burden. Regarding the lungs and airways, the progressive pathophysiological changes place a significant strain on the musculoskeletal components of the respiratory system for people with CF. This pilot study investigated the effectiveness of manual therapy interventions (MTIs) on thoracic mobility, respiratory muscle strength, lung function, and musculoskeletal pain. Method: A study with a pretest-posttest design was conducted with 15 eligible people with CF at the Sahlgrenska University Hospital CF Centre. After an initial set of diagnostic tests at baseline, the participants underwent eight weekly 30-min MTIs. The MTIs included passive joint mobilisation and soft tissue manipulation of primary and secondary anatomical areas of the musculoskeletal respiratory system. On the day of the final intervention, the baseline measurements were repeated. Results: Trends of increased thoracic mobility were observed following the intervention, with a statistically significant increase in respiratory muscle strength. No change in lung function was observed. Musculoskeletal pain before and after the intervention showed a significant decrease in tender points, and all participants reported positive experiences with MTIs. Conclusion: MTIs may improve thoracic mobility, alleviate pain, and enhance respiratory muscle strength in people with CF. Further research is needed to confirm their potential role as a CF physiotherapy supplement. Clinical trial id: NCT04696198.
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| 15. |
- Sinderholm Sposato, Niklas, 1977, et al.
(författare)
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Musculoskeletal aspects of respiratory function in cystic fibrosis: a cross-sectional comparative study
- 2024
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Ingår i: EUROPEAN CLINICAL RESPIRATORY JOURNAL. - 2001-8525. ; 11:1
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundRespiration is an intricate interaction between visceral and musculoskeletal structures. In cystic fibrosis (CF), the airways and lungs are subject to progressive obstruction and destruction. However, knowledge about the musculoskeletal aspects of respiratory function and symptoms is still limited in this patient group.MethodsIn a cross-sectional comparative study, 21 adults with CF enrolled at the Gothenburg CF Centre were matched with 42 healthy controls. The two groups were examined and compared in terms of thoracic mobility, respiratory muscle strength, lung function, and musculoskeletal pain in accordance with a predefined protocol.ResultsSignificant differences were observed between the groups in the number of tender points, thoracic excursion, forced vital capacity (FVC), and forced expiratory volume (FEV). The CF group also demonstrated a tendency toward reduced function in other measurements, although these were not statistically significant.ConclusionThis cross-sectional study revealed that people with CF have reduced thoracic mobility and an increased prevalence of muscular tender points, alongside decreased lung function, compared to healthy controls. These findings stress the need for greater emphasis on the often-overlooked musculoskeletal aspects of CF care, especially as people with CF are living longer and may require more musculoskeletal health support.
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| 16. |
- Sinderholm Sposato, Niklas, 1977, et al.
(författare)
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Thoracic configuration in patients ageing with cystic fibrosis: A retrospective single-blinded cohort study.
- 2023
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Ingår i: Respiratory physiology & neurobiology. - : Elsevier BV. - 1569-9048 .- 1878-1519. ; 307
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Tidskriftsartikel (refereegranskat)abstract
- In patients with cystic fibrosis (CF), thoracic morphology and its role in respiratory function is conditioned by anthropometric factors, as well as by pathological changes. While the lungs are continuously monitored, examinations of potential thoracic cage adaptations to the disease are rare. Hence, the aim of this study was to investigate thoracic configuration, and its correlation to spirometry measures over time.In total, 344 high-resolution computed tomography (HRCT) examinations from 90 patients were assessed and analysed. Those results were subsequently related to spirometry measurements performed within the same period.The cohort displayed no homogenous change in thoracic configuration over time, and correlation between thoracic area and spirometry variables could not be supported statistically.Although the current study included a larger cohort of patients with CF compared to previous studies on thoracic morphology, no patient group-specific changes in thoracic configuration were revealed. Furthermore, no correlations between structural findings and functional respiratory measurements were found.
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