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  • 1
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    Online Resource
    Cystic fibrosis and transition to adult healthcare
    Springer Science and Business Media LLC ; 2021
    In:  Der Pneumologe Vol. 18, No. 2 ( 2021-03), p. 97-103
    Details
    In: Der Pneumologe, Springer Science and Business Media LLC, Vol. 18, No. 2 ( 2021-03), p. 97-103
    Type of Medium: Online Resource
    ISSN: 1613-5636 , 1613-6055
    URL: Article
    DOI: 10.1007/s10405-020-00367-w
    Language: German
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2021
    detail.hit.zdb_id: 3120281-0
    detail.hit.zdb_id: 2163983-8
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  • 2
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    Real-life impact of highly effective CFTR modulator therapy in children with cystic fibrosis
    Frontiers Media SA ; 2023
    In:  Frontiers in Pharmacology Vol. 14 ( 2023-5-9)
    Details
    In: Frontiers in Pharmacology, Frontiers Media SA, Vol. 14 ( 2023-5-9)
    Abstract: Introduction: Recently, cystic fibrosis transmembrane regulator modulator therapy with elexacaftor/tezacaftor/ivacaftor has become available for children with cystic fibrosis (CF) carrying at least one F508del mutation. Objective: To assess the intermediate term effects of elexacaftor/tezacaftor/ivacaftor in children with cystic fibrosis in a real-world setting. Methods: We performed a retrospective analysis of records of children with cystic fibrosis, who started elexacaftor/tezacaftor/ivacaftor between 8/2020 and 10/2022. Pulmonary function tests, nutritional status, sweat chloride and laboratory data were assessed before, 3 and 6 months after the start of elexacaftor/tezacaftor/ivacaftor respectively. Results: Elexacaftor/tezacaftor/ivacaftor was started in 22 children 6–11 years and in 24 children 12–17 years. Twenty-seven (59%) patients were homozygous for F508del (F/F) and 23 (50%) patients were transitioned from ivacaftor/lumacaftor (IVA/LUM) or tezacaftor/ivacaftor (TEZ/IVA) to elexacaftor/tezacaftor/ivacaftor. Overall, mean sweat chloride concentration decreased by 59.3 mmol/L (95% confidence interval: −65.0 to −53.7 mmol/L, p & lt; 0.0001) under elexacaftor/tezacaftor/ivacaftor. Sweat chloride concentration also decreased significantly after transition from IVA/LUM or TEZ/IVA to elexacaftor/tezacaftor/ivacaftor (−47.8 mmol/l; 95% confidence interval: −57.6 to −37.8 mmol/l, n = 14, p & lt; 0.0001). Sweat chloride reduction was more marked in children with the F/F than in those with the F/MF genotype (69.4 vs 45.9 mmol/L, p & lt; 0.0001). At 3 months follow-up, body-mass-index-z-score increased by 0.31 (95% CI, 0.2–0.42, p & lt; 0.0001) with no further increase at 6 months. BMI-for-age-z-score was more markedly improved in the older group. Overall pulmonary function (percent predicted FEV 1 ) at 3 months follow-up increased by 11.4% (95% CI: 8.0–14.9, p & lt; 0.0001) with no further significant change after 6 months. No significant differences were noted between the age groups. Children with the F/MF genotype had a greater benefit regarding nutritional status and pulmonary function tests than those with the F/F genotype. Adverse events led to elexacaftor/tezacaftor/ivacaftor dose reduction in three cases and a temporary interruption of therapy in four cases. Conclusion: In a real-world setting, elexacaftor/tezacaftor/ivacaftor therapy had beneficial clinical effects and a good safety profile in eligible children with cystic fibrosis comparable to previously published data from controlled clinical trials. The positive impact on pulmonary function tests and nutritional status seen after 3 months of elexacaftor/tezacaftor/ivacaftor therapy was sustained at 6 months follow-up.
    Type of Medium: Online Resource
    ISSN: 1663-9812
    URL: Article
    DOI: 10.3389/fphar.2023.1176815
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2023
    detail.hit.zdb_id: 2587355-6
    SSG: 15,3
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  • 3
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    Effects of a Long-Term Monitored Exercise Program on Aerobic Fitness in a Small Group of Children with Cystic Fibrosis
    MDPI AG ; 2022
    In:  International Journal of Environmental Research and Public Health Vol. 19, No. 13 ( 2022-06-28), p. 7923-
    Details
    In: International Journal of Environmental Research and Public Health, MDPI AG, Vol. 19, No. 13 ( 2022-06-28), p. 7923-
    Abstract: Background: The aim of this study was to investigate the effects of a monitored exercise program on aerobic fitness in children with cystic fibrosis (CF). Methods: Six children (2f/4m) with ages ranging from 6 to 14 years (11.3 ± 3.3 years.) and a mean ppFEV1 102.5 ± 13.5% pred. participated in the partially monitored 12-month exercise program. VO2peak and Wpeak were used as parameters of aerobic fitness. Incremental Cardio-Pulmonary Exercise Tests (CPETs) were performed before the program began (T1), after 6 months (T3) of monitoring, and after a further 6 months (T4) without monitoring. Habitual physical activity (HPA) was assessed with accelerometry. Results: The values of VO2peak and Wpeak improved slightly from T1 to T3 (p 〉 0.05), without a further increase after monitoring was stopped (T4). However, the VO2peak and Wpeak values were higher after monitoring was stopped compared to at T1. The exercise program with and without monitoring (p 〉 0.05) had no or only a slight effect on the FEV1 values, steps/day, and the intensity of HPA. Conclusions: Monitoring seems to facilitate the achievement of beneficial effects on physical fitness in CF children. For that reason, continuous individual exercise monitoring programs that involve close contact with an exercise therapist should be provided to maintain long-term motivation and participation in physical activities and sport activities during leisure time.
    Type of Medium: Online Resource
    ISSN: 1660-4601
    URL: Article
    DOI: 10.3390/ijerph19137923
    Language: English
    Publisher: MDPI AG
    Publication Date: 2022
    detail.hit.zdb_id: 2175195-X
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  • 4
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    Table_1.DOCX
    Frontiers Media SA ; 2021
    In:  Frontiers in Microbiology Vol. 12 ( 2021-7-12)
    Details
    In: Frontiers in Microbiology, Frontiers Media SA, Vol. 12 ( 2021-7-12)
    Abstract: Mycobacterium abscessus complex (MABC) infection has a devastating impact on the course of cystic fibrosis (CF) and non-CF lung disease. Diagnosis of MABC pulmonary disease is challenging, and current diagnostic approaches lack accuracy, especially in CF. In this study, we aimed to establish an MABC-specific interferon-γ release assay to detect host immune responses to MABC and improve diagnostics of MABC infection by the detection of antigen-specific T cells. Four species-specific proteins of MABC were overexpressed in an Escherichia coli expression system. Purified proteins were used to stimulate peripheral blood mononuclear cells of study subjects in an ELISpot assay. Interferon-γ response of 12 subjects with established diagnosis of MABC infection (10 CF and two non-CF) was compared with 35 controls (22 CF and 13 non-CF) distributed to three control groups, 17 CF subjects without NTM infection, nine subjects with NTM infection other than MABC, and nine subjects with tuberculosis. Cellular in vitro responses in the MABC group were stronger than in the control groups, especially toward the protein MAB_0405c (39 vs. 4 spots per 300,000 PBMC, p = 0.004; data represent mean values) in all patients and also in the subgroup of CF subjects (39 spots vs. 1 spot, p = 0.003). Receiver operating characteristic curve analysis indicated that spot numbers of at least 20 were highly predictive of MABC infection (all patients: area under curve 0.773, sensitivity 58%, and specificity 94%; CF patients: area under curve 0.818, sensitivity 60%, and specificity 100%). In conclusion, we identified MAB_0405c as a protein that may stimulate MABC-specific interferon-γ secretion and may add to the diagnosis of MABC infection in affected patients.
    Type of Medium: Online Resource
    ISSN: 1664-302X
    URL: Article
    URL: Article
    URL: Article
    URL: Article
    URL: Article
    DOI: 10.3389/fmicb.2021.692395
    DOI: 10.3389/fmicb.2021.692395.s001
    DOI: 10.3389/fmicb.2021.692395.s002
    DOI: 10.3389/fmicb.2021.692395.s003
    DOI: 10.3389/fmicb.2021.692395.s004
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2021
    detail.hit.zdb_id: 2587354-4
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  • 5
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    Association between habitual physical activity (HPA) and sleep quality in patients with cystic fibrosis
    AME Publishing Company ; 2020
    In:  Journal of Thoracic Disease Vol. 12, No. S2 ( 2020-10), p. S264-S265
    Details
    In: Journal of Thoracic Disease, AME Publishing Company, Vol. 12, No. S2 ( 2020-10), p. S264-S265
    Type of Medium: Online Resource
    ISSN: 2072-1439 , 2077-6624
    URL: Journal
    URL: Article
    DOI: 10.21037/jtd
    DOI: 10.21037/jtd-cus-2020-010-ab
    Language: Unknown
    Publisher: AME Publishing Company
    Publication Date: 2020
    detail.hit.zdb_id: 2573571-8
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  • 6
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    Association between habitual physical activity (HPA) and sleep quality in patients with cystic fibrosis
    Springer Science and Business Media LLC ; 2021
    In:  Sleep and Breathing Vol. 25, No. 2 ( 2021-06), p. 609-615
    Details
    In: Sleep and Breathing, Springer Science and Business Media LLC, Vol. 25, No. 2 ( 2021-06), p. 609-615
    Abstract: Sleep disturbances and poor sleep quality are known to be present in patients with CF. Regular physical activity plays an important role in the treatment of CF patients due to its positive influence on progression of disease and quality of life. The aim of this work is to create a home-based sleep and activity profile and to investigate the influence of habitual physical activity (HPA) on sleep quality in children, adolescents, and adults with CF. Methods A total of 109 CF patients (64 male, mean age 22.7 ± 12.0 years; mean ppFEV1 63.0 ± 26.7) were equipped with an actigraph for a home-based collection of data on sleep and activity over 4 weeks. Results Age, FEV1, and BMI affect sleep and activity in CF patients. Especially younger age and higher FEV1 show a great influence on certain aspects of sleep (SE, TST, TIB, WASO, # of awakenings) and activity and its different intensities. General HPA does not affect sleep, but there is a strong correlation between times spent in vigorous to very vigorous intensities and better sleep quality. Conclusion Besides younger age and higher FEV1, daily activity in higher intensities influences sleeping behavior of CF patients in a positive way. Patients with poor sleep quality and sleep disturbances possibly benefit from an intensification of physical activity in the home environment. Trail registration number: 14–6117-BO (University Duisburg-Essen) and NCT 03518697 (clinical trials).
    Type of Medium: Online Resource
    ISSN: 1520-9512 , 1522-1709
    URL: Article
    DOI: 10.1007/s11325-020-02130-0
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2021
    detail.hit.zdb_id: 2072363-5
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  • 7
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    Health-Related and Motor Performance-Related Fitness and Physical Activity Among Youth With Cystic Fibrosis
    SAGE Publications ; 2021
    In:  Perceptual and Motor Skills Vol. 128, No. 5 ( 2021-10), p. 2097-2116
    Details
    In: Perceptual and Motor Skills, SAGE Publications, Vol. 128, No. 5 ( 2021-10), p. 2097-2116
    Abstract: Little is known about motor competence and the longitudinal development of motor performance among youth with cystic fibrosis (CF). In this study, we assessed aspects of motor performance in different age groups of young patients with CF and compared them with a healthy reference group of same aged children. We also examined the development of motor performance among different age groups of these children with CF, using The Deutscher Motorik Test (DMT) to assess attributes of health-related and motor performance-related fitness. We used an incremental ergometer cycle test to determine maximal exercise capacity (expressed as peak workload). We evaluated and recorded habitual physical activity (PA) as measured by the number of steps per day and the time spent in different PA intensities (expressed in metabolic equivalents). In total, 31 children and adolescents with CF agreed to participate (13 girls,18 boys) aged 6–17 years ( M = 11.3, SD =3.3 years); they had a mean one second forced expiratory volume (expressed as a percentage of predicted value [% pred] ) of 87.2% ( SD = 22.3%). We found their values of health-related and motor performance-related fitness to be significantly lower ( p  〈  0.05) than those of their healthy peer participants. In contrast to the reference group, participants with CF up to 14 years of age showed a linear improvement in these values and in their PA, followed by a plateau or even a nonsignificant decrease after age 14. These findings have important implications for the development and prescription of exercise programs for children with CF. Besides aerobic and strength exercises, we recommend that neuromuscular training be integrated into exercise programs to improve the coordinative abilities of youth with CF. More attention should be paid to vulnerable older adolescents to ensure their long-term motivation to maintain exercise participation.
    Type of Medium: Online Resource
    ISSN: 0031-5125 , 1558-688X
    URL: Article
    DOI: 10.1177/00315125211036415
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2021
    detail.hit.zdb_id: 2066876-4
    SSG: 5,2
    SSG: 7,11
    SSG: 31
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  • 8
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    Barriers for Sports and Exercise Participation and Corresponding Barrier Management in Cystic Fibrosis
    MDPI AG ; 2022
    In:  International Journal of Environmental Research and Public Health Vol. 19, No. 20 ( 2022-10-13), p. 13150-
    Details
    In: International Journal of Environmental Research and Public Health, MDPI AG, Vol. 19, No. 20 ( 2022-10-13), p. 13150-
    Abstract: Background: Nowadays physical activity (PA)/exercise is an important component of cystic fibrosis (CF) therapy. The aim of the study was to assess the barriers to PA and the barrier management and to explore the effect of supervision on the barriers and barrier management during an exercise program. Methods: In total, 88 people with CF (pwCF) of the ages 6 to 50 years old (mean 24.2 ± 7.9 yrs) participated in the partially supervised 12-month exercise program and filled in a structured and validated questionnaire about barriers to sports and barrier management at baseline. Additionally, 23 pwCF filled in the questionnaire after 6 months and 12 months. The items were clustered into physical and psychosocial barriers and into preventive counter strategies and situational counter strategies and analyzed at baseline and over time. Results: Physical barriers were more relevant than psychosocial barriers and no trend could be seen in the situational and preventive counter strategies. When divided in subgroups, the less active pwCF ( 〈 7500 steps/day), more active pwCF ( 〉 7500 steps/day), physical barriers, and psychosocial barriers showed no significant differences. However physical barriers showed a tendency to have a higher value in the less active group compared to the more active group (p 〉 0.05). Stratified by age or FEV1%pred between the subgroups, no differences could be seen regarding barriers and counter strategies. Conclusions: Physical barriers seemed to have a higher priority when it comes to not participating in PA/exercise. Supervision over 6 months during an exercise program did not show a beneficial effect on barriers and barrier management. Besides the motivational aspect of sport counselling, the volitional aspect seemed to be more important to incorporate more PA into daily life. Individual barriers and their concrete counter strategies should be discussed with the patient with CF. Sport counselling is needed permanently and should be part of the CF routine care.
    Type of Medium: Online Resource
    ISSN: 1660-4601
    URL: Article
    DOI: 10.3390/ijerph192013150
    Language: English
    Publisher: MDPI AG
    Publication Date: 2022
    detail.hit.zdb_id: 2175195-X
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  • 9
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    Changes in Clinical Markers During A Short-Term Transfer Program of Adult Cystic Fibrosis Patients from Pediatric to Adult Care
    Bentham Science Publishers Ltd. ; 2019
    In:  The Open Respiratory Medicine Journal Vol. 13, No. 1 ( 2019-06-30), p. 11-18
    Details
    In: The Open Respiratory Medicine Journal, Bentham Science Publishers Ltd., Vol. 13, No. 1 ( 2019-06-30), p. 11-18
    Abstract: Transition from child-oriented to adult-oriented health care in Cystic Fibrosis (CF) has become more important over recent decades as the survival of people with this disease has increased. The transition process usually begins in adolescence, with full transfer completed in early adulthood. Objective: This study investigated the impact of a short-term transfer program on clinical markers in an adult CF cohort still being managed by pediatricians. Methods: Clinically relevant data from the year before (T-1), the time of Transfer (T) and the year after the transfer (T+1) were analysed retrospectively. Results: 39 patients (median age 29.0 years; 64% male) were transferred between February and December 2016. Lung function had declined significantly in the year before transfer (in % predicted: Forced Expiratory Volume in 1 second (FEV), 62.8 vs. 57.7, p 〈 0.05; Forced Vital Capacity (FVC), 79.9 vs. 71.1, p 〈 0.05), but remained stable in the year after transfer (in % predicted: FEV: 56.3; FVC 68.2). BMI was stable over the whole observational period. There was no relevant change in chronic lung infection with P. aeruginosa , Methicillin-Resistant Staphylococcus aureus (MRSA) and Burkholderia sp. during the observation period. The number of patient contacts increased significantly in the year after versus the year before transfer (inpatient: 1.51 vs. 2.51, p 〈 0.05; outpatient: 2.67 vs. 3.41, p 〈 0.05). Conclusions: Our data show that, within the framework of a structured transfer process, it is possible to transfer a large number of adult CF patients, outside a classic transition program, from a pediatric to an adult CF center in a short period of time, without any relevant changes in clinical markers and, stability.
    Type of Medium: Online Resource
    ISSN: 1874-3064
    URL: Article
    DOI: 10.2174/1874306401913010011
    Language: English
    Publisher: Bentham Science Publishers Ltd.
    Publication Date: 2019
    detail.hit.zdb_id: 2395996-4
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  • 10
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    Effects of a long‐term exercise program on motor performance in children and adolescents with CF
    Wiley ; 2020
    In:  Pediatric Pulmonology Vol. 55, No. 12 ( 2020-12), p. 3371-3380
    Details
    In: Pediatric Pulmonology, Wiley, Vol. 55, No. 12 ( 2020-12), p. 3371-3380
    Abstract: The aim of this study was to examine motor performance and trainability in youths with cystic fibrosis (CF). Methods Twenty‐two children and adolescents (11 f/11 m), age range 6–17 years (11.3 ± 3.3 years), mean FEV1 91.0 ± 21.7% pred.finished the partially monitored 12‐months exercise program. Patients performed the Deutsche Motorik Test (DMT) to assess flexibility, balance, strength, power and totalmotor performance. An incremental ergometer cycle test was used to assess maximal exercise capacity (Wpeak). All tests were performed before (T1), after 6 months of monitored exercise training (T3) and another 6 months without monitoring (T4). Results Motor Competence in total and test‐items of the DMT (except foreward bend) improved to T3 ( p   〈  .05). No further improvement could be observed after the end of the monitoring (T3). However, the values remained stable at the improved level (T4). Girls scored lower in test items depending on strength/power but scored higher in balancing compared to boys ( p   〉  .05). Wpeak and FEV1 were not influenced by the training program. From T3 to T4 a slight decrease was observed ( p  ≤ .05). Conclusions The findings demonstrate benefits of an individualizedmonitored long‐term exercise intervention on motor performance in CF with improvements of test‐tasks to predicted normal. Monitoringseems to be a facilitator in maintaining motivation toward physical activity as no further increase in motor performance was observed after stopping supervision. The results suggest that an individually tailored monitoredregular exercise program should include all aspects of physical fitness with a variety of movement experiences.
    Type of Medium: Online Resource
    ISSN: 8755-6863 , 1099-0496
    URL: Issue
    URL: Article
    DOI: 10.1002/ppul.v55.12
    DOI: 10.1002/ppul.25064
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 1491904-7
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