Update on COPD and Cystic Fibrosis

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Pulmonary".

Deadline for manuscript submissions: closed (10 March 2024) | Viewed by 10199

Special Issue Editor


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Guest Editor
Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA
Interests: inflammatory lung diseases; aging; fibroblast growth factor signaling; klotho; cystic fibrosis; chronic obstructive pulmonary disease; pulmonary vascular remodeling; fibroblast growth factor 23
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Special Issue Information

Dear Colleagues,

Chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF) are two chronic inflammatory lung diseases, which seem to be very different from each other.

COPD, known as a chronic airway disease strongly associated with smoking, is currently the third leading cause of death, with a high socioeconomical burden due to the number of people affected, which is more than 65 million. Despite the availability of therapies mainly addressing symptoms, there is lack of access for many patients, and more research is needed to help to prevent the development of respiratory symptoms, better characterize COPD itself, provide equal access for patients, and finally identify novel therapies.

CF research has experienced a revolution over the last decade due to the development of therapies including the highly effective modulator therapy (HEMT) leading to a substantial increase in life expectancy and quality of life for people with CF (pwCF). Unfortunately, we have still not found a cure due to missing 10% of CFTR mutations, which do not respond to HEMT and pwCF with existing CF-related disease, who are now aging and potentially developing more and new comorbidities.

The aim of this Special Issue of Medicina is to showcase new advances in the field of cystic fibrosis and COPD research. This Special Issue is open to basic research, clinical and translational studies, case reports, perspectives, reviews, and systematic reviews and meta-analyses.

The aim of this Special Issue will be to deliver new insights into the evolving field of CF and COPD research. Manuscripts focused on the following topics are strongly encouraged.

  • Novel therapeutic strategies in CF and COPD;
  • Chronic inflammation and infections in CF and COPD;
  • Disparity research in COPD;
  • Aging in COPD and CF;
  • Access to care for patients with CF and COPD.

Dr. Stefanie Krick
Guest Editor

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Keywords

  • COPD
  • cystic fibrosis
  • inflammation
  • airway disease
  • aging

Published Papers (6 papers)

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Research

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10 pages, 1571 KiB  
Article
Respiratory Muscle Training and Its Impact on Balance and Gait in Patients with Severe COPD
by Crisan Alexandru Florian, Pescaru Camelia Corina, Maritescu Adelina, Carunta Vlad, Oancea Cristian and Vastag Emanuela
Medicina 2024, 60(2), 257; https://doi.org/10.3390/medicina60020257 - 01 Feb 2024
Viewed by 897
Abstract
Background and Objectives: Improving extrapulmonary symptoms in COPD through respiratory muscle training can help alleviate the burden of respiratory symptoms, reduce fatigue, and improve exercise capacity in patients with COPD. This, in turn, can enhance physical activity, balance, and gait, ultimately improving [...] Read more.
Background and Objectives: Improving extrapulmonary symptoms in COPD through respiratory muscle training can help alleviate the burden of respiratory symptoms, reduce fatigue, and improve exercise capacity in patients with COPD. This, in turn, can enhance physical activity, balance, and gait, ultimately improving the overall quality of life for individuals with COPD. This study aimed to investigate the effects of respiratory muscle training on balance and gait in patients with moderate to severe COPD. Materials and Methods: We included 65 patients with moderate to severe COPD randomly assigned to either the pulmonary rehabilitation protocol group (PR) or the pulmonary rehabilitation and inspiratory muscle training group (PR + IMT) for three weeks. Patients performed a spirometry, maximal inspiratory and expiratory pressure (MIP/MEP), 6 min walking test (6MWT), activities-specific balance confidence (ABC) scale questionnaire, Berg Balance Scale (BBS), timed up and go test (TUG), and single-leg stance test (SLS). Results: Rehabilitation had a notable impact on MIP in Group 2 (PR + IMT), with a highly significant difference between pre- and post-rehabilitation distributions (p < 0.0001). At the same time, Group 1 (PR-only) showed no significant changes (p = 0.27). In Group 1 (Control), pre- and post-rehabilitation comparisons reveal slight non-significant changes for SLS EO (p = 0.16), ABC (p = 0.07), TUG (p = 0.06), and BBS (p = 0.13). In contrast, in Group 2 (Cases), there are significant improvements in all variables after rehabilitation compared to the pre-rehabilitation values: SLS EO (p < 0.0001), ABC (p < 0.0001), TUG (p < 0.0001), and BBS (p < 0.0001). Conclusions: Our research demonstrated that respiratory muscle training significantly positively impacts balance and gait performance among patients with moderate to severe COPD compared to a control group. Full article
(This article belongs to the Special Issue Update on COPD and Cystic Fibrosis)
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9 pages, 1320 KiB  
Article
The Benefit of Hydrogen Gas as an Adjunctive Therapy for Chronic Obstructive Pulmonary Disease
by Shih-Feng Liu, Chin-Ling Li, Hui-Ching Lee, Hui-Chuan Chang, Jui-Fang Liu and Ho-Chang Kuo
Medicina 2024, 60(2), 245; https://doi.org/10.3390/medicina60020245 - 30 Jan 2024
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Abstract
Background and Objectives: Recent studies suggest that hydrogen gas possesses anti-inflammatory, antioxidant, and anti-apoptotic properties. This study aimed to explore the therapeutic potential of hydrogen gas and assess its safety and tolerability in individuals with chronic obstructive pulmonary disease (COPD). Materials and Methods: [...] Read more.
Background and Objectives: Recent studies suggest that hydrogen gas possesses anti-inflammatory, antioxidant, and anti-apoptotic properties. This study aimed to explore the therapeutic potential of hydrogen gas and assess its safety and tolerability in individuals with chronic obstructive pulmonary disease (COPD). Materials and Methods: Enrolled COPD patients received standard treatments along with additional hydrogen inhalation for 30 min in the morning, afternoon, and evening over a 30-day period. The assessment included changes in the COPD Assessment Test (CAT), the modified Medical Research Council (mMRC) Dyspnea Scale, lung function, sleep quality, inflammation markers, and oxidative stress markers before and after hydrogen inhalation. Results: Six patients participated in this study. Patients 2, 3, 4, 5, and 6 demonstrated improvements in CAT scores following hydrogen gas intervention, with patients 2, 4, 5, and 6 also showing improvements in mMRC scores. Statistically, this study revealed significant improvements in CAT [15.5 (10.5–19.75) vs. 8.5 (3–13.5); p = 0.043] and mMRC scores [2.5 (1–4) vs. 2 (0–3.25); p = 0.046] before and after intervention, respectively. However, no significant differences were observed in lung function, DLCO, sleep quality, and 6 MWT before and after hydrogen therapy. CBC examination showed a significant difference in platelet count before and after treatment [247 (209.75–298.75) vs. 260 (232.75–314.5); p = 0.043], respectively, while other blood tests, inflammation markers, and oxidative stress markers did not exhibit significant differences before and after hydrogen therapy. All patients experienced no obvious side-effects. Conclusions: Adjuvant therapy with hydrogen gas demonstrated symptom improvements in specific COPD patients, and no significant adverse effects were observed in any of the patients. Hydrogen gas may also exert a modulatory effect on platelet count. Full article
(This article belongs to the Special Issue Update on COPD and Cystic Fibrosis)
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11 pages, 2662 KiB  
Article
Fibroblast Growth Factor 23 Signaling Does Not Increase Inflammation from Pseudomonas aeruginosa Infection in the Cystic Fibrosis Bronchial Epithelium
by Meghan June Hirsch, Emma Lea Matthews, Seth Bollenbecker, Molly Easter, Megan R. Kiedrowski, Jarrod W. Barnes and Stefanie Krick
Medicina 2023, 59(9), 1635; https://doi.org/10.3390/medicina59091635 - 09 Sep 2023
Cited by 1 | Viewed by 1521
Abstract
Background and Objectives: Chronic inflammation due to Pseudomonas aeruginosa (PA) infection in people with cystic fibrosis (CF) remains a concerning issue in the wake of modulator therapy initiation. Given the perpetuating cycle of colonization, infection, chronic inflammation, and recurrent injury to the [...] Read more.
Background and Objectives: Chronic inflammation due to Pseudomonas aeruginosa (PA) infection in people with cystic fibrosis (CF) remains a concerning issue in the wake of modulator therapy initiation. Given the perpetuating cycle of colonization, infection, chronic inflammation, and recurrent injury to the lung, there are increases in the risk for mortality in the CF population. We have previously shown that fibroblast growth factor (FGF) 23 can exaggerate transforming growth factor (TGF) beta-mediated bronchial inflammation in CF. Our study aims to shed light on whether FGF23 signaling also plays a role in PA infection of the CF bronchial epithelium. Materials and Methods: CF bronchial epithelial cells were pretreated with FGF23 or inhibitors for FGF receptors (FGFR) and then infected with different PA isolates. After infection, immunoblot analyses were performed on these samples to assess the levels of phosphorylated phospholipase C gamma (PLCγ), total PLCγ, phosphorylated extracellular signal-regulated kinase (ERK), and total ERK. Additionally, the expression of FGFRs and interleukins at the transcript level (RT-qPCR), as well as production of interleukin (IL)-6 and IL-8 at the protein level (ELISA) were determined. Results: Although there were decreases in isoform-specific FGFRs with increases in interleukins at the mRNA level as well as phosphorylated PLCγ and the production of IL-8 protein with PA infection, treatment with FGF23 or FGFR blockade did not alter downstream targets such as IL-6 and IL-8. Conclusions: FGF23 signaling does not seem to modulate the PA-mediated inflammatory response of the CF bronchial epithelium. Full article
(This article belongs to the Special Issue Update on COPD and Cystic Fibrosis)
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9 pages, 847 KiB  
Article
Comparison of BODE and ADO Indices in Predicting COPD-Related Medical Costs
by Chin-Ling Li, Hui-Chuan Chang, Ching-Wan Tseng, Yuh-Chyn Tsai, Jui-Fang Liu, Meng-Lin Tsai, Meng-Chih Lin and Shih-Feng Liu
Medicina 2023, 59(3), 577; https://doi.org/10.3390/medicina59030577 - 15 Mar 2023
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Abstract
Background and Objectives:The ADO (age, dyspnea, and airflow obstruction) and BODE (body mass index, airflow obstruction, dyspnea, and exercise capacity) indices are often used to evaluate the prognoses for chronic obstructive pulmonary disease(COPD); however, an index suitable for predicting medical costs has [...] Read more.
Background and Objectives:The ADO (age, dyspnea, and airflow obstruction) and BODE (body mass index, airflow obstruction, dyspnea, and exercise capacity) indices are often used to evaluate the prognoses for chronic obstructive pulmonary disease(COPD); however, an index suitable for predicting medical costs has yet to be developed. Materials and Methods: We investigated the BODE and ADO indices to predict medical costs and compare their predictive power. A total of 396 patients with COPD were retrospectively enrolled. Results: For hospitalization frequencies, BODE was R2 = 0.093 (p < 0.001), and ADO was R2 = 0.065 (p < 0.001); for hospitalization days, BODE was R2 = 0.128 (p < 0.001), and ADO was R2 = 0.071 (p < 0.001); for hospitalization expenses, BODE was R2 = 0.020 (p = 0.047), and ADO was R2 = 0.012 (p = 0.179). BODE and ADO did not differ significantly in the numbers of outpatient visits (BODE, R2 = 0.012, p = 0.179; ADO, R2 = 0.017, p = 0.082); outpatient medical expenses (BODE, R2 = 0.012, p = 0.208; ADO, R2 = 0.008, p = 0.364); and total medical costs (BODE, R2 = 0.018, p = 0.072; ADO, R2 = 0.016, p = 0.098). In conclusion, BODE and ADO indices were correlated with hospitalization frequency and hospitalization days. However, the BODE index exhibits slightly better predictive accuracy than the ADO index in these items. Full article
(This article belongs to the Special Issue Update on COPD and Cystic Fibrosis)
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Review

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12 pages, 325 KiB  
Review
Comorbid Cognitive Impairment in Chronic Obstructive Pulmonary Disease (COPD): Current Understanding, Risk Factors, Implications for Clinical Practice, and Suggested Interventions
by Rayan A. Siraj
Medicina 2023, 59(4), 732; https://doi.org/10.3390/medicina59040732 - 08 Apr 2023
Cited by 3 | Viewed by 2097
Abstract
Cognitive impairment is a common comorbidity in patients with COPD, significantly impacting health and clinical outcomes. Yet it remains under investigated and is largely overlooked. Although the exact cause of cognitive impairment in patients with COPD is still unclear, factors such as hypoxemia, [...] Read more.
Cognitive impairment is a common comorbidity in patients with COPD, significantly impacting health and clinical outcomes. Yet it remains under investigated and is largely overlooked. Although the exact cause of cognitive impairment in patients with COPD is still unclear, factors such as hypoxemia, vascular disease, smoking, exacerbation, and physical inactivity have been suggested. While international guidelines recommend identifying comorbidity in patients with COPD, such as cognitive impairment, cognitive assessment is not yet part of the routine assessment. Unidentified cognitive deficits in patients with COPD may have severe impacts on clinical management, resulting in an inability to maintain functional independence, poor self-management, and a greater dropout from pulmonary rehabilitation programs. There is a need to consider cognitive screening as a part of COPD assessment to promote early detection of cognitive impairment. Recognizing cognitive impairment early in the course of the illness allows the development of individualized interventions to meet patients’ needs and improve clinical outcomes. Pulmonary rehabilitation should be tailored to cognitively impaired patients with COPD to maximize the benefits and minimize the incompletion rate. Full article
(This article belongs to the Special Issue Update on COPD and Cystic Fibrosis)
11 pages, 898 KiB  
Review
The Relationship of Cholesterol Responses to Mitochondrial Dysfunction and Lung Inflammation in Chronic Obstructive Pulmonary Disease
by Bakr Jundi, Huma Ahmed, Joshua Reece and Patrick Geraghty
Medicina 2023, 59(2), 253; https://doi.org/10.3390/medicina59020253 - 28 Jan 2023
Cited by 2 | Viewed by 2236
Abstract
Hyperlipidemia is frequently reported in chronic obstructive pulmonary disease (COPD) patients and is linked to the progression of the disease and its comorbidities. Hypercholesterolemia leads to cholesterol accumulation in many cell types, especially immune cells, and some recent studies suggest that cholesterol impacts [...] Read more.
Hyperlipidemia is frequently reported in chronic obstructive pulmonary disease (COPD) patients and is linked to the progression of the disease and its comorbidities. Hypercholesterolemia leads to cholesterol accumulation in many cell types, especially immune cells, and some recent studies suggest that cholesterol impacts lung epithelial cells’ inflammatory responses and mitochondrial responses. Several studies also indicate that targeting cholesterol responses with either statins or liver X receptor (LXR) agonists may be plausible means of improving pulmonary outcomes. Equally, cholesterol metabolism and signaling are linked to mitochondrial dysfunction and inflammation attributed to COPD progression. Here, we review the current literature focusing on the impact of cigarette smoke on cholesterol levels, cholesterol efflux, and the influence of cholesterol on immune and mitochondrial responses within the lungs. Full article
(This article belongs to the Special Issue Update on COPD and Cystic Fibrosis)
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