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European Respiratory Review : An... Jun 2023Pulmonary rehabilitation has established a status of evidence-based therapy for patients with symptomatic COPD in the stable phase and after acute exacerbations.... (Review)
Review
Pulmonary rehabilitation has established a status of evidence-based therapy for patients with symptomatic COPD in the stable phase and after acute exacerbations. Rehabilitation should have the possibility of including different disciplines and be offered in several formats and lines of healthcare. This review focusses on the cornerstone intervention, exercise training, and how training interventions can be adapted to the limitations of patients. These adaptations may lead to altered cardiovascular or muscular training effects and/or may improve movement efficiency. Optimising pharmacotherapy (not the focus of this review) and oxygen supplements, whole-body low- and high-intensity training or interval training, and resistance (or neuromuscular electrical stimulation) training are important training modalities for these patients in order to accommodate cardiovascular and ventilatory impairments. Inspiratory muscle training and whole-body vibration may also be worthwhile interventions in selected patients. Patients with stable but symptomatic COPD, those who have suffered exacerbations and patients waiting for or who have received lung volume reduction or lung transplantation are good candidates. The future surely holds promise to further personalise exercise training interventions and to tailor the format of rehabilitation to the individual patient's needs and preferences.
Topics: Humans; Pulmonary Disease, Chronic Obstructive; Lung; Exercise Therapy; Exercise; Exercise Tolerance
PubMed: 37286219
DOI: 10.1183/16000617.0222-2022 -
Transplant International : Official... 2024
Topics: Humans; Kidney; Kidney Transplantation; Publishing
PubMed: 38389709
DOI: 10.3389/ti.2024.12711 -
Journal of Cystic Fibrosis : Official... Jul 2023Phase 3 clinical trials showed elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was safe and efficacious in people with cystic fibrosis (CF) with ≥1 F508del-CFTR allele....
BACKGROUND
Phase 3 clinical trials showed elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was safe and efficacious in people with cystic fibrosis (CF) with ≥1 F508del-CFTR allele. To assess long-term effects of ELX/TEZ/IVA under real-world conditions of use, a 5-year observational registry-based study is being conducted. We report interim results from the first 2 years of follow-up.
METHODS
The study included people with CF in the US Cystic Fibrosis Foundation Patient Registry (CFFPR) who initiated ELX/TEZ/IVA between October 2019 and December 2020. Pulmonary exacerbations (PEx), percent predicted forced expiratory volume in 1 second (ppFEV), hospitalizations, bacterial pathogens, body mass index (BMI), CF complications and comorbidities, and liver function tests (LFTs) after treatment initiation were compared with the 5-year pre-treatment period. Death and lung transplantation were assessed relative to 2019 CFFPR data.
RESULTS
16,116 people with CF were included (mean treatment duration 20.4 months). Among those with 5 years of pre-treatment data, mean PEx/patient/year declined to 0.18 (95% CI: 0.17, 0.19) in Years 1 and 2 post-treatment from 0.86 (95% CI: 0.83, 0.88) in the baseline year (79% reduction), after a continued increase observed pre-treatment. Similarly, a decline in mean hospitalizations/patient/year was observed in Year 1 that was sustained in Year 2 (74% reduction from baseline year). The mean absolute change in ppFEV from baseline was +8.2 percentage points (95% CI: 8.0, 8.4) in Year 1 and +8.9 percentage points (95% CI: 8.7, 9.1) in Year 2, after a continued decline observed pre-treatment. Positive bacterial cultures decreased for all evaluated pathogens, and mean BMI increased by 1.6 kg/m (95% CI: 1.5, 1.6) by Year 2. No new safety concerns were identified based on evaluation of CF complications, comorbidities, and LFTs. The annualized rates of death (0.47% [95% CI: 0.39, 0.55]) and lung transplantation (0.16% [95% CI: 0.12, 0.22]) were considerably lower than reported in 2019 (1.65% and 1.08%, respectively).
CONCLUSIONS
ELX/TEZ/IVA treatment was associated with sustained improvements in lung function, reduced frequency of PEx and all-cause hospitalization, increased BMI, and lower prevalence of positive bacterial cultures. Additionally, there was a 72% lower rate of death and 85% lower rate of lung transplantation relative to the year before ELX/TEZ/IVA availability. These results, from the largest cohort of ELX/TEZ/IVA-treated people to date, extend our understanding of the broad clinical benefits of ELX/TEZ/IVA.
Topics: Humans; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Aminophenols; Benzodioxoles; Registries; Mutation; Chloride Channel Agonists
PubMed: 36963986
DOI: 10.1016/j.jcf.2023.03.002 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Jan 2024Lung transplantation is a well-established treatment for children facing advanced lung disease and pulmonary vascular disorders. However, organ shortage remains highest... (Review)
Review
Lung transplantation is a well-established treatment for children facing advanced lung disease and pulmonary vascular disorders. However, organ shortage remains highest in children. For fitting the small chest of children, transplantation of downsized adult lungs, lobes, or even segments were successfully established. The worldwide median survival after pediatric lung transplantation is currently 5.7 years, while under consideration of age, underlying disease, and peri- and posttransplant center experience, median survival of more than 10 years is reported. Timing of referral for transplantation, ischemia-reperfusion injury, primary graft dysfunction, and acute and chronic rejection after transplantation remain the main challenges.
PubMed: 38584780
DOI: 10.5606/tgkdc.dergisi.2024.25806