Disabil Rehabil Assist Technol. 2025 Feb 10:1-8. doi: 10.1080/17483107.2025.2463548. Online ahead of print.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs. Rehabilitation is crucial in managing respiratory symptoms. Telerehabilitation, which provides remote rehabilitation services via digital platforms, gained importance during the COVID-19 pandemic. Despite its growing use, there is little clarity on the available tools and devices for CF telerehabilitation.

OBJECTIVE: This scoping review aims to map the existing tools and devices used in telerehabilitation for pediatric and adult CF patients.

METHODS: The review was conducted following the Joanna Briggs Institute methodology, using the PRISMA-ScR checklist. Comprehensive searches were performed across seven databases, alongside grey literature. Studies involving CF patients and telerehabilitation interventions were included. Data were extracted and analyzed both numerically and thematically.

RESULTS: Eighteen studies were included, involving 622 CF patients. The review identified 10 platforms, seven telemonitoring devices, and three rehabilitation devices. Most studies focused on motor rehabilitation with individual, synchronous sessions. Commonly used platforms included Zoom, Skype, and Google Meet, while only three studies used platforms specifically designed for CF.

CONCLUSIONS: Telerehabilitation for CF is in its early stages and less developed than for other conditions. There is a need for dedicated platforms and devices that address CF patients' specific motor and respiratory needs. Future efforts should focus on developing these tools to improve patient engagement and outcomes.

PMID:39928374 | DOI:10.1080/17483107.2025.2463548

Chron Respir Dis. 2025 Jan-Dec;22:14799731241310897. doi: 10.1177/14799731241310897.

ABSTRACT

OBJECTIVES: To assess real-world survival and healthcare resource utilization (HCRU) in US patients with non-cystic fibrosis bronchiectasis (NCFBE).

METHODS: This retrospective analysis, using data from the STATinMED RWD Insights database from Jan 2015-Oct 2022, included adults with NCFBE (from Jan 2015-Oct 2021) and non-NCFBE comparators (from Jan 2015-Aug 2020); baseline characteristics were balanced by inverse probability treatment weighting. Outcomes included survival through end of study. HCRU was assessed over 12 months.

RESULTS: 117,718 patients with NCFBE and 306,678 comparators were included. Patients with NCFBE had a 77% higher risk of death than comparators (hazard ratio [HR] 1.77 [95% CI 1.74-1.80]). Risk of death was higher among patients aged ≥65 years (vs 18-34 years; HR 11.03 [95% CI 10.36-11.74]), among Black patients (vs White; HR 1.53 [95% CI 1.50-1.55]), and among patients with comorbid COPD (HR 1.42 [95% CI 1.40-1.44]). Patients with NCFBE incurred higher all-cause and respiratory-related HCRU than comparators for outpatient office, outpatient hospital, emergency department (ED), inpatient and respiratory-related pulmonologist visits (all p < .0001); HCRU increased with exacerbations.

CONCLUSIONS: Patients with NCFBE have high mortality burden and incur high HCRU, both of which are further increased with exacerbations. Prevention and delay of exacerbations are key areas for improvement of disease management.

PMID:39925084 | DOI:10.1177/14799731241310897

Comput Biol Med. 2025 Feb 7;187:109748. doi: 10.1016/j.compbiomed.2025.109748. Online ahead of print.

ABSTRACT

BACKGROUND: Lung disease remains a leading cause of morbidity and mortality in individuals with cystic fibrosis (CF). Despite significant advances, the complex molecular mechanisms underlying CF-related airway pathology are not fully understood. Building upon previous single-cell transcriptomics studies in CF patients and healthy controls, this study employs enhanced analytical methodologies to deepen our understanding of CF-associated gene expression.

METHODS: We employed advanced single-cell transcriptomics techniques, integrating data from multiple sources and implementing rigorous normalization and mapping strategies using a comprehensive lung reference panel. These sophisticated methods were designed to enhance the accuracy and depth of our analysis, with a focus on elucidating differential gene expression and characterizing co-expression network dynamics associated with cystic fibrosis (CF).

RESULTS: Our analysis uncovered novel genes and regulatory networks that had not been previously associated with CF airway disease. These findings highlight new potential therapeutic targets that could be exploited to develop more effective interventions for managing CF-related lung conditions.

CONCLUSION: This study provides critical insights into the molecular landscape of CF airway disease, offering new avenues for targeted therapeutic strategies. By identifying key genes and networks involved in CF pathogenesis, our research contributes to the broader efforts to improve the prognosis and quality of life for patients with CF. These discoveries pave the way for future studies aimed at translating these findings into clinical practice.

PMID:39921941 | DOI:10.1016/j.compbiomed.2025.109748

Respir Med. 2025 Feb 5:107982. doi: 10.1016/j.rmed.2025.107982. Online ahead of print.

ABSTRACT

BACKGROUND/RATIONALE: Lung transplant (LTx) candidates with cystic fibrosis (CF) have ventilatory and musculoskeletal limitations and benefit from pulmonary rehabilitation (PR). Their training response has not been well characterized. The study aims to: 1) characterize the effect of outpatient PR and 2) evaluate the clinical characteristics associated with their PR response.

METHODS: Single-center retrospective cohort study of CF LTx candidates (July 2009-June 2019) with available pre-transplant exercise data, who participated in PR 2 to 3 times/week until transplantation. Demographics, CF-related characteristics, aerobic and muscle training volumes, and six-minute walk distance (6MWD) were characterized using descriptive statistics, paired t-tests and Spearman correlations to describe relationships between CF-related characteristics and training volumes.

RESULTS: In 86 CF LTx candidates (32±10 years, 49% males, FEV1: 23±5%; listing 6MWD 421±89 meters), the median PR time was 87 days (24-36 sessions). 78% had at least one exacerbation and 55% required hospitalization. 88% used supplemental oxygen and 37% required home non-invasive ventilation. Treadmill speed (1.7±0.5 mph); biceps (50 IQR [40-70] lbs*reps) and quadriceps (30 IQR [30-40] lbs*reps) training volumes improved with PR (p< 0.05), whereas 6MWD remained unchanged. The presence of ≥ 1 respiratory exacerbation was associated with a lower progression in treadmill speed [-0.36 mph 95%CI (-0.67 to -0.04), p=0.028].

CONCLUSION: CF LTx candidates participating in PR increased treadmill speed and muscle training volumes, with preservation of 6MWD. Respiratory exacerbations were prevalent and important determinants of aerobic training.

PMID:39921065 | DOI:10.1016/j.rmed.2025.107982

J Cyst Fibros. 2025 Feb 6:S1569-1993(25)00046-3. doi: 10.1016/j.jcf.2025.01.013. Online ahead of print.

ABSTRACT

BACKGROUND: Pulmonary infections with multidrug-resistant nontuberculous mycobacteria (NTM), particularly Mycobacterium abscessus (MAB), are increasingly more prevalent in individuals with lung disease such as cystic fibrosis and are extremely difficult to treat. Protracted antibiotic therapies consist of multidrug regimens that last for months to years. Despite these intense protocols, failure rates are high with 50%-60% of patients not achieving a sustained culture-negative status. A major contributor to the difficult medical management of NTM infections is formation of pulmonary aggregate MAB biofilms which protect the resident bacteria from antimicrobials and host immune effectors. Thereby, novel and more effective approaches to combat recalcitrant NTM infections are urgently needed.

METHODS: We developed an epitope-targeted monoclonal antibody-based technology to rapidly disrupt biofilms and release resident bacteria into a transient yet highly vulnerable phenotype that is significantly more sensitive to killing by both antibiotics and host innate immune effectors (e.g., PMNs and antimicrobial peptides). Herein, we tested this technology in a pre-clinical murine lung infection model to determine whether this treatment would mediate clearance of MAB from the lungs and speed return to homeostasis.

RESULTS: As early as 48 h after a single treatment, bacterial loads were reduced to below the level of detection and histopathologic analysis showed markedly decreased inflammation and rapid eradication of aggregate biofilms compared to controls.

CONCLUSIONS: These new data add to those from multiple prior published studies which show the significant efficacy of this novel therapeutic approach to resolve recalcitrant bacterial biofilm diseases, now potentially including those induced by NTM.

PMID:39919951 | DOI:10.1016/j.jcf.2025.01.013

J Cyst Fibros. 2025 Feb 6:S1569-1993(25)00048-7. doi: 10.1016/j.jcf.2025.01.015. Online ahead of print.

ABSTRACT

BACKGROUND: Elucidating the molecular and cellular effects caused by CFTR variants is crucial to understand Cystic Fibrosis (CF) disease pathophysiology, but also to predict disease severity, to provide genetic counselling, and to determine the most adequate therapeutic strategy for people with CF (pwCF). While the current CFTR modulator drugs (CFTRm) are approved mainly for pwCF with the most prevalent variant, p.Phe508del, pwCF carrying rare and/or uncharacterized CFTR variants are not eligible. However, previous studies have shown that such rare variants can be rescued by the approved CFTRm, suggesting clinical benefit for those pwCF. Here, we characterized the rare and non-eligible p.Ile148Asn CFTR variant found in Portuguese pwCF, regarding CFTR processing, traffic and function, and response to existing CFTRm.

METHODS: We used the forskolin-induced swelling (FIS) assay in intestinal organoids (IOs) from 2 CF individuals carrying p.Ile148Asn in heterozygosity with p.Phe508del and p.Gly542Ter, respectively. Additionally, a Cystic Fibrosis Bronchial Epithelial (CFBE) cell line expressing p.Ile148Asn-CFTR was generated to study the molecular defect of this variant individually.

RESULTS: Our results show that p.Ile148Asn is a CF-causing variant, impairing both CFTR plasma membrane (PM) traffic and function, albeit partially. Moreover, p.Ile148Asn-CFTR can be rescued by approved CFTRm in CFBE cells and IOs, suggesting potential clinical benefit for these individuals.

CONCLUSION: The work emphasizes the importance of testing CFTRm for rare variants not included in the drug label. It also shows that the 'theranostic' approach using IOs from pwCF, which captures the genetic background of each individual, complements theratyping in cell lines that focuses only on CFTR variants.

PMID:39919950 | DOI:10.1016/j.jcf.2025.01.015

Dev Cell. 2025 Feb 4:S1534-5807(25)00031-0. doi: 10.1016/j.devcel.2025.01.011. Online ahead of print.

ABSTRACT

Cellular stresses, particularly endoplasmic reticulum (ER) stress induced by ER-to-Golgi transport blockade, trigger Golgi-independent secretion of cytosolic and transmembrane proteins. However, the molecular mechanisms underlying this unconventional protein secretion (UPS) remain largely elusive. Here, we report that an ER tubulovesicular structure (ER tubular body [ER-TB]), shaped by the tubular ER-phagy receptors ATL3 and RTN3L, plays an important role in stress-induced UPS of transmembrane proteins such as cystic fibrosis transmembrane conductance regulator (CFTR) and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike protein. Correlative light-electron microscopy analyses demonstrate the formation of ER-TB under UPS-inducing conditions in HEK293 and HeLa cells. Individual gene knockdowns of ATL3 and RTN3 inhibit ER-TB formation and the UPS of trafficking-deficient ΔF508-CFTR. Combined supplementation of ATL3 and RTN3L induces ER-TB formation and UPS. ATL3 also participates in the SARS-CoV-2-associated convoluted membrane formation and Golgi-independent trafficking of SARS-CoV-2 spike protein. These findings suggest that ER-TB serves a common function in mediating stress-induced UPS, which participates in various physiological and pathophysiological processes.

PMID:39919755 | DOI:10.1016/j.devcel.2025.01.011

Am J Respir Crit Care Med. 2025 Feb 7. doi: 10.1164/rccm.202407-1474OC. Online ahead of print.

ABSTRACT

RATIONALE: The mucoid phenotype of Staphylococcus aureus is caused by adaptation. Excessive biofilm formation associated with a protective effect for mucoid S. aureus was observed in isolates from respiratory samples of people with cystic fibrosis (pwCF). However, there is little knowledge about the prevalence of mucoid S. aureus in pwCF and a potential association with CF lung disease.

METHODS: A prospective multicenter study was conducted (cross-sectional and longitudinal). Specimens and case report forms were sent to the central study laboratory for characterization of S. aureus and analysis of clinical parameters.

MEASUREMENTS AND MAIN RESULTS: Cross-sectional study: In 41 of 451 S. aureus-positive pwCF (9.1%) from 13 CF-centers, mucoid S. aureus was cultured. Longitudinal study: The distribution of CFTR genotypes, the number of pwCF with highly effective modulator therapy and co-infection with Pseudomonas aeruginosa were equivalent in the mucoid (35 pwCF) versus the control group (only non-mucoid S. aureus, 36 pwCF). While lung function did not differ between groups as a whole, a subgroup analysis revealed significantly worse lung function for female pwCF with mucoid S. aureus as well as for pwCF if P. aeruginosa co-infection was excluded.

CONCLUSIONS: In the era of highly effective modulator therapy, worse lung function was associated with female and P. aeruginosa-negative pwCF with mucoid S. aureus compared to pwCF with only non-mucoid S. aureus. Therefore, appropriate culture conditions should be established to detect mucoid S. aureus. Further investigations are needed to elucidate the relationship between mucoid S. aureus and CF lung disease.

PMID:39918841 | DOI:10.1164/rccm.202407-1474OC

Chronic Obstr Pulm Dis. 2025 Feb 5. doi: 10.15326/jcopdf.2024.0582. Online ahead of print.

ABSTRACT

BACKGROUND: Pseudomonas aeruginosa is an important pathogen in patients with chronic respiratory diseases. It can colonize the airway and could have prognostic value in bronchiectasis and cystic fibrosis. Its role in chronic obstructive pulmonary disease (COPD) is less well defined.

METHODS: A prospective study was conducted in Hong Kong to investigate the possible association between Pseudomonas aeruginosa colonization and acute exacerbation of COPD (AECOPD) risks.

RESULTS: Among 327 Chinese patients with COPD included, 33 (10.1%) of the patients had Pseudomonas aeruginosa colonization. Patients with or without Pseudomonas aeruginosa colonization had similar background characteristics. Patients with Pseudomonas aeruginosa colonization had increased risks of moderate to severe AECOPD, severe AECOPD and pneumonia with adjusted odds ratio (aOR) of 3.15 (95% CI 1.05 - 9.48, p = 0.042), 2.59 (95% CI 1.01 - 6.64, p = 0.048) and 4.19 (95% CI 1.40 - 12.54, p = 0.011) respectively. Patients with Pseudomonas aeruginosa colonization also had increased annual frequency of moderate to severe AECOPD, median 0 [0 - 0.93] in the non-Pseudomonas aeruginosa colonization group and 1.35 [0 - 3.39] in the Pseudomonas aeruginosa colonization group, with a p-value of 0.005 in multi-variate linear regression.

CONCLUSION: Pseudomonas aeruginosa colonization is a potential independent risk factor for moderate to severe AECOPD and pneumonia among patients with COPD without co-existing bronchiectasis.

PMID:39912873 | DOI:10.15326/jcopdf.2024.0582

J Clin Sleep Med. 2025 Feb 6. doi: 10.5664/jcsm.11600. Online ahead of print.

NO ABSTRACT

PMID:39912229 | DOI:10.5664/jcsm.11600

Pediatr Int. 2025 Jan-Dec;67(1):e15882. doi: 10.1111/ped.15882.

NO ABSTRACT

PMID:39911093 | DOI:10.1111/ped.15882

Curr Microbiol. 2025 Feb 5;82(3):118. doi: 10.1007/s00284-025-04091-7.

ABSTRACT

Cyclic dimeric guanosine monophosphate (c-di-GMP) plays a vital role within the nucleotide signaling network of bacteria, participating in various biological processes such as biofilm formation and toxin production, among others. Substantial evidence demonstrates its critical involvement in the progression of chronic infections. Treating chronic infections seems critical, and there is a worldwide quest for drugs that target pathogens' unique and complex virulence-associated signaling networks. c-di-GMP is a promising therapeutic target by serving as a distinct virulence factor, solving problems associated with drug resistance, biofilm dispersion, and its related septicemia complications. c-di-GMP levels act as checkpoints for several biofilm-associated molecular pathways, viz., Gac/Rsm, BrlR, and SagS signaling systems. C-di-GMP is also engaged in the Wsp chemosensory pathway responsible for rugose small colony variants observed in cystic fibrosis-related lung infections. Considering all factors, c-di-GMP serves as a pivotal hub in the intricate cascade of biofilm regulation. By overseeing QS systems, exopolysaccharide synthesis, and antibiotic resistance pathways in chronic infections, it emerges as a linchpin for effective drug development strategies against biofilm-related ailments. This underscores the significance of understanding the multifaceted signaling networks. c-di-GMP's role is highlighted in this review as a concealed virulence component in various bacterial pathogens, suggesting that medications targeting it could hold promise in treating chronic disorders associated with biofilms.

PMID:39909925 | DOI:10.1007/s00284-025-04091-7

J Cyst Fibros. 2025 Feb 5:S1569-1993(25)00051-7. doi: 10.1016/j.jcf.2025.02.002. Online ahead of print.

ABSTRACT

OBJECTIVE: Access to highly effective modulator therapies (HEMT) in resource-limited countries is limited by prohibitive cost and restrictive patents. We report the clinical outcomes of a cost-reduction strategy in South Africa (SA), where generic elexacaftor/tezacaftor/ivacaftor (gETI) was pharmacokinetically enhanced with clarithromycin (gETI/c) for people with CF (pwCF) eligible for HEMT.

METHODS: A multi-center observational study from December 2021 to May 2024. Analysis of variance (ANOVA) and linear mixed effects analyses were conducted to describe and compare change in sweat chloride (SC), FEV1pp, BMI (m/kg2) and adverse events (AE) over 18-months follow-up for different gETI dose categories: a) standard, full or b) modulator sparing dose (gETI/c at 25-50 % recommended dose, twice/thrice weekly).

RESULTS: 70/413 (17 %) eligible pwCF [median age 27 years (range 6-52); 68 (97 %) with ≥ one copy F508del] received gETI with standard (n = 38) or modulator-sparing doses (n = 32); 29 changed dosing regimens across the study period. The overall mean (SD) reduction in SC after 1-month of treatment was -52.9 (16.9) mmol/L (p < 0.001), with no evidence of difference between dose groups (p = 0.2). Overall mean (SD) FEV1pp and BMI increased at 1-month by 14.9 (95 % CI 11.49-18.40) and 0.84 (95 % CI 0.16-1.49), respectively. Improvements in FEV1pp and BMI were sustained throughout follow-up, with no evidence of difference between dosing groups. No serious AEs were reported.

CONCLUSION: Our experience with gETI is similar to real-world reports using the originator product. Boosting ETI with CYP3A-inhibitors is a safe and effective strategy to increase access to ETI in settings where access to HEMT is restricted.

PMID:39909761 | DOI:10.1016/j.jcf.2025.02.002

Microbiol Spectr. 2025 Feb 5:e0301024. doi: 10.1128/spectrum.03010-24. Online ahead of print.

ABSTRACT

Pseudomonas aeruginosa is a bacterial pathogen that is a major cause of lung infections in cystic fibrosis (CF) and other patients. Isolates of P. aeruginosa from CF patients commonly carry filamentous phages (Pf phages), which constitute a family of temperate phages known to be related to biofilm production and antibiotic sequestration. In this study, we identified 12 new Pf phage genomes in a collection of clinical isolates of P. aeruginosa from CF patients. Study of the anti-phage defense systems in the bacterial isolates revealed the presence of 89 such systems, of which eight were encoded in the Pf phage genomes. Finally, although a weak relation between resistance to phage infection and the number of anti-phage defense systems was detected, it was observed that the phage resistance was related to the presence of Pf phages and the anti-phage defense systems encoded in these phages.IMPORTANCEBacteria harbor a wide range of defense mechanisms to avoid phage infections that hamper the application of phage therapy because they can lead to the rapid acquisition of phage resistance. In this study, eight anti-phage defense systems were found in the genome of 12 Pf phages that were presents in 56% of the CF isolates of P. aeruginosa. The high prevalence of these phages underlines the importance of our findings about newly discovered filamentous phages and the role of these phages in resistance to phage infections. Thus, the knowledge of the anti-defense system in the Pf phage genomes could be useful in assessing the possible application of phage therapy to treat an infectious disease.

PMID:39907445 | DOI:10.1128/spectrum.03010-24

Respir Investig. 2025 Feb 3;63(2):224-225. doi: 10.1016/j.resinv.2025.01.002. Online ahead of print.

NO ABSTRACT

PMID:39904248 | DOI:10.1016/j.resinv.2025.01.002

PLoS Pathog. 2025 Feb 4;21(2):e1012784. doi: 10.1371/journal.ppat.1012784. Online ahead of print.

ABSTRACT

Aspergillus fumigatus (Af) is a major mould pathogen found ubiquitously in the air. It commonly infects the airways of people with cystic fibrosis (CF) leading to Aspergillus bronchitis or allergic bronchopulmonary aspergillosis. Resident alveolar macrophages and recruited neutrophils are important first lines of defence for clearance of Af in the lung. However, their contribution to the inflammatory phenotype in CF during Af infection is not well understood. Here, utilising CFTR deficient mice we describe a hyperinflammatory phenotype in both acute and allergic murine models of pulmonary aspergillosis. We show that during aspergillosis, CFTR deficiency leads to increased alveolar macrophage death and persistent inflammation of the airways in CF, accompanied by impaired fungal control. Utilising CFTR deficient murine cells and primary human CF cells we show that at a cellular level there is increased activation of NFκB and NFAT in response to Af which, as in in vivo models, is associated with increased cell death and reduced fungal control. Taken together, these studies indicate that CFTR deficiency promotes increased activation of inflammatory pathways, the induction of macrophage cell death and reduced fungal control contributing to the hyper-inflammatory of pulmonary aspergillosis phenotypes in CF.

PMID:39903773 | DOI:10.1371/journal.ppat.1012784

Pediatr Pulmonol. 2024 Jun;59(6):1661-1676. doi: 10.1002/ppul.26970. Epub 2024 Apr 12.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is a life-shortening multisystem genetic disease. Although progressive pulmonary disease is the predominant cause of morbidity and mortality, improvements in treatment for CF-related lung disease, with associated increase in longevity, have increased the prevalence of extrapulmonary manifestations1.

METHODS: To discuss these issues, a multidisciplinary meeting of international leaders and experts in the field was convened in November 2021 at the Shaping Initiatives and Future Trends Symposium with the goal of highlighting shifting management paradigms in CF. The main topics covered were: (1) nutrition and obesity, (2) exocrine pancreas, (3) CF-related diabetes, (4) CF liver disease, (5) CF-related bone disease, and (6) post-lung transplant care. This document summarizes the proceedings, highlighting the key priorities and important research questions that were discussed.

RESULTS: Improved life expectancy, the advent of cystic fibrosis transmembrane conductance regulator modulators, and the increasing appreciation of the heterogeneity or spectrum of disease are leading to a shift in management for patients with cystic fibrosis. Care should be individualized to ensure that increased longevity is accompanied by improved extra-pulmonary care and reduced morbidity.

PMID:39903130 | DOI:10.1002/ppul.26970

Health Qual Life Outcomes. 2025 Feb 4;23(1):10. doi: 10.1186/s12955-025-02338-2.

ABSTRACT

BACKGROUND: Measuring the quality of life in patients with cystic fibrosis is important, both in terms of assessing the implementation of new therapies and monitoring their effects, as well as the ongoing evaluation of patients' condition. The objective of this study is to present tools for measuring the quality of life in adult patients with cystic fibrosis, along with their characteristics and measurement properties.

METHODS: The systematic review was performed according to the PRISMA guidelines based on a previously prepared research protocol (PROSPERO: CRD42023491030). Searches were performed in Medline (via PubMed), Embase (via OVID), and Cochrane Library databases. In addition, manual searches of bibliographies from the studies included in the analysis and grey literature were performed. Quality assessment of the included studies was performed according to the guidelines of COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN).

RESULTS: The systematic search identified 3,359 studies, of which 26 met the inclusion criteria for the analysis. Two publications were additionally included as a result of the manual search. A total of 16 tools for measuring the quality of life in adults with cystic fibrosis were identified, the measurement properties of which were presented in the included studies. Among these tools, the Cystic Fibrosis Questionnaire-Revised (CFQ-R) and the Cystic Fibrosis Quality of Life Questionnaire (CFQoL) were most frequently analyzed. There were also other new, promising tools.

CONCLUSION: Most studies reported acceptable measurement properties of tools used to measure quality of life in adult patients with cystic fibrosis. In many cases, however, significant limitations were observed related to the lack of comprehensive analysis of the factor structure and other aspects related to validation and responsiveness. There have also been problems with the reliability of some tool scales (including the CFQ-R 14+). The small number of studies makes it difficult to present clear conclusions regarding the usefulness of existing tools. In turn, new tools that may be used in economic analyses (CFQ-R-8 dimensions) or in individualized assessment of quality of life using a mobile application (Q-Life) seem promising. However, further research on large patient populations is necessary to analyze the measurement properties of all tools.

PMID:39901267 | DOI:10.1186/s12955-025-02338-2

BMC Nurs. 2025 Feb 3;24(1):127. doi: 10.1186/s12912-025-02774-x.

ABSTRACT

BACKGROUND: Cystic Fibrosis (CF) significantly affects the respiratory system, often requiring lung transplantation in advanced stages. This life-saving procedure presents substantial challenges and uncertainties. While there is existing research on support and information needs post-lung transplant from various perspectives, this study aims to specifically address the unique experiences and challenges faced by individuals with CF during both the pre-transplant and post-transplant periods.

METHODS: Twenty-three lung-transplanted individuals with CF participated in this exploratory qualitative study. Data was collected through individual semi-structured interviews and analyzed using inductive content analysis.

RESULTS: Participants faced physical and mental challenges, including fatigue, depression, and anxiety. The waiting period involved isolation, dependence on family, and guilt. Post-transplant, they dealt with relief but also severe pain and adjusted to a new identity. Participants highlighted the importance of taking immunosuppressive medications as prescribed, even though the regimen was complicated and these medications had side effects. Participants stressed the need for earlier and more open dialogue with healthcare professionals and better emotional preparation for the transplant process, including preparedness for pain and previously inadequately addressed concerns such as depression and anxiety.

CONCLUSIONS: This study underscores the significant physical and emotional challenges individuals with CF face during lung transplantation, highlighting the need for comprehensive, person-centered care. Psychological support, effective post-transplant pain management, and early palliative care may be beneficial approaches to improve the patient experience. Nurses can play a pivotal role in this process by ensuring clear communication, managing pain, educating patients on immunosuppressive regimens, and advocating for holistic care.

PMID:39901222 | DOI:10.1186/s12912-025-02774-x

Semin Respir Crit Care Med. 2025 Feb 3. doi: 10.1055/a-2531-1018. Online ahead of print.

ABSTRACT

Sleep disorders that involve circadian rhythm disruption and sleep-disordered breathing (SDB) such as obstructive sleep apnea (OSA) are closely linked to respiratory infections. SDB leads to a proinflammatory state due to intermittent hypoxia, sleep fragmentation, increased oxidative stress, and elevation of inflammatory mediators such as tumor necrosis factor (TNF), interleukin-6 (IL-6), and C-reactive protein (CRP). Furthermore, inflammatory mediator levels correlate with SDB severity, especially in people with OSA. Nocturnal microaspiration, gastroesophageal reflux, and associated comorbidities (e.g. obesity) increase the risk of community-acquired pneumonia, viral infections such as SARS-CoV-2, respiratory complications, and death. OSA has been associated with post-COVID syndrome. It also increases the risk of postoperative complications in both adults and children. Circadian rhythm disorders such as insomnia predispose to immune disorders and increase the risk of infection. Chronic conditions such as bronchiectasis, with or without concomitant cystic fibrosis, can lead to structural sleep changes and increase the risk of OSA due to chronic cough, arousals, aspirations, hypoxia, upper airway edema, and overexpression of proinflammatory cytokines. The protective effect of treatment for sleep disorders against respiratory infection is currently unknown. However, in people presenting with respiratory infection, it is important to test for SDB to prevent complications.

PMID:39900109 | DOI:10.1055/a-2531-1018