Chronic Respiratory Disease最新文献

{"title":"Independent effect of the triglyceride-glucose index on all-cause mortality in critically ill patients with chronic obstructive pulmonary disease and asthma: A retrospective cohort study.","authors":"Wen-Qiang Zhou, Xin Song, Wei-Hua Dong, Zhi Chen","doi":"10.1177/14799731241245424","DOIUrl":"https://doi.org/10.1177/14799731241245424","url":null,"abstract":"<p><strong>Background: </strong>The triglyceride-glucose (TyG) index serves as a reliable proxy for insulin resistance (IR). IR has been linked to heightened incidence, prevalence, or severity of chronic obstructive pulmonary disease (COPD) and asthma. Prior research indicates that critically ill patients are prone to developing IR. Nevertheless, few studies have delved into the correlation between IR and all-cause mortality in critically ill patients with COPD and asthma. Therefore, the aim of this study is to explore the association between the TyG index and all-cause mortality in patients with COPD and asthma, with the goal of assessing the impact of IR on the prognosis of this patient population.</p><p><strong>Methods: </strong>This is a retrospective study, and all data are from the Medical Information Mart for Intensive Care IV (MIMIC-IV) critical care database. This study included 684 ICU patients with COPD and asthma and divided them into quartiles based on TyG index levels. The primary outcomes of this study were all-cause mortality during follow-up, encompassing mortality at 30 days, 90 days, and 180 days. The Kaplan-Meier analysis was used to compare all-cause mortality among the above four groups. Cox proportional hazards analyses were performed to examine the association between TyG index and all-cause mortality in critically ill patients with COPD and asthma. Restricted cubic spline analysis was used to assess potential nonlinear association between the TyG index and the primary outcome.</p><p><strong>Results: </strong>A total of 684 patients (53.9% female) were included. The 90-days all-cause mortality rate and 180-days all-cause mortality were 11.7% and 12.3%, respectively. Kaplan-Meier analysis revealed a significant association between the TyG index and both 90-days all-cause mortality (log-rank <i>p</i> = .039) and 180-days all-cause mortality (log-rank <i>p</i> = .017). Cox proportional hazards analysis revealed a significant association between the TyG index and 90-days all-cause mortality in both the unadjusted model (HR, 1.30 [95% CI 1.08-1.57] <i>p</i> = .005) and the model adjusted for age, gender, and diabetes (HR, 1.38 [95% CI 1.15-1.67] <i>p</i> < .001). Similarly, the TyG index was associated with 180-days all-cause mortality in the unadjusted model (HR, 1.30 [95% CI 1.09-1.56] <i>p</i> = .004) and the model adjusted for age, sex, and diabetes (HR, 1.38 [95% CI 1.15-1.66] <i>p</i> < .001). The restricted cubic splines (RCS) regression model indicated a significant nonlinear association between the TyG index and both 90-days and 180-days all-cause mortality. Specifically, TyG index >4.8 was associated with an increased risk of mortality at both 90 days and 180 days.</p><p><strong>Conclusions: </strong>In summary, our results extend the utility of the TyG index to critically ill patients with COPD and asthma. Our study shows that the TyG index is a potential predictor of all-cause mortality in critically ill patients wi","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241245424"},"PeriodicalIF":4.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11015761/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140851619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of a respiratory and neurological rehabilitation treatment plan in post Covid-19 affected university students. Randomized clinical study.","authors":"Zacarías Sánchez Milá, David Rodríguez Sanz, Ana Martín Nieto, Ana Jiménez Lobo, Manuel Ramos Hernández, Angélica Campón Chekroun, Raúl Frutos Llanes, José Manuel Barragán Casas, Jorge Velázquez Saornil","doi":"10.1177/14799731241255967","DOIUrl":"10.1177/14799731241255967","url":null,"abstract":"<p><strong>Background: </strong>COVID-19 demonstrated the possibility of neurological complications such as loss of sense of smell and taste, together with respiratory problems. Respiratory training and rehabilitation of neurological sequelae are essential to improve respiratory function and thus quality of life, and the aim of this study is to evaluate the efficacy of a pulmonary and neurological rehabilitation program.</p><p><strong>Objectives: </strong>To apply a treatment to reduce dyspnea, increase exertional capacity, increase vital capacity and respiratory muscle strength, together with an increase in olfactory and gustatory sensitivity in post-SARS-CoV-2 patients.</p><p><strong>Methods: </strong>A randomised controlled experimental study was conducted in 220 patients with a medical diagnosis of COVID-19 and more than 5 months of evolution, dyspnoea or perceived fatigue, including olfactory and gustatory perception problems, of whom 200 patients completed the study. 100 patients were randomly assigned to the intervention group, consisting of an inspiratory training treatment plan (Powerbreathe Plus®) combined with aerobic exercise and olfactory gustatory treatment for 31 days, and 100 patients to the control group, for 31 days without any type of therapy.</p><p><strong>Results: </strong>The study was conducted in post-Covid-19 patients for 5 months. Two hundred patients were divided into an intervention group (<i>n</i> = 100) and a control group (<i>n</i> = 100). The comparison between the groups showed significant differences in spirometric variables; forced vital capacity (<i>p</i> < .001; Eta2 (0.439); Mean: 0,6135), the ratio between both FEV1/FVC (<i>p</i> < 0.01; Eta2 (0.728); Mean:9,313), peak inspiratory pressure (<i>p</i> < 0.01; Eta2 (0.906); Mean:4,526); changes were observed in dyspnoea measured with the modified Borg scale (<i>p</i> < 0.01; Eta2 (0.811); Mean:1,481) and the modified Medical Research Council scale (<i>p</i> < 0.01; Eta2 (0.881); Mean: 0.777); finally, changes were found in neurological variables, in the questions of the Singapore Smell and Taste Questionnaire, How was your sense of smell after treatment? (<i>p</i> < 0.01; Eta2 (0.813); Mean: 1,721) and How is your sense of taste after treatment? (<i>p</i> < 0.01; Eta2 (0.898); Mean: 1,088).</p><p><strong>Conclusion: </strong>The implementation of a respiratory rehabilitation treatment plan with the Powerbreathe Plus® device, aerobic exercise and neurorehabilitation with olfactory and gustatory training, is a therapeutic option against respiratory and neurological sequelae in patients who have suffered such sequelae due to the SARS-CoV-2 virus. Clinicaltrials.gov: NCT05195099. First posted 18/01/2022; Last Update Posted 29/06/2022.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241255967"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11100389/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140944354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fidelity of a home-based pulmonary rehabilitation program in people with COPD referred from primary care.","authors":"Simone Dal Corso, Anne E Holland, Johnson George, Michael J Abramson, Grant Russell, Nick Zwar, Billie Bonevski, Jaycie Perryman, Narelle S Cox","doi":"10.1177/14799731241307247","DOIUrl":"10.1177/14799731241307247","url":null,"abstract":"<p><p><b>Purpose:</b> Pulmonary rehabilitation (PR) is highly effective but underutilised. Pathways to home-based PR (HBPR) from general practice could improve utilisation, but program fidelity in this setting is unknown. This study aimed to explore the fidelity of HBPR in people referred from general practice. <b>Methods:</b> Secondary analysis of intervention-group data from two-arm cluster RCT (RADICALS-interdisciplinary intervention for people with COPD including smoking cessation support, home medicine reviews and 8-weeks HBPR). HBPR fidelity assessed by the extent to which exercise training was prescribed according to protocol. Completion of HBPR and contributing factors were determined. <b>Results:</b> 107 participants (68% of intervention group) were referred to HBPR, with <i>n</i> = 75 (70%) commencing the program (mean age 68 years, FEV₁ 65% predicted, median mMRC 1). Aerobic training was prescribed according to protocol for 74% of participants in week one, and on average 89% of participants in weeks 2-8. Resistance training was prescribed according to protocol for 98% and 88% of participants (Week 1 and Weeks 2-8, respectively). Rehabilitation completers (<i>n</i> = 57, 76%) were 26 times more likely to have attended the Week 2 phone call (95% CI 2-352). Clinically meaningful improvements were achieved in health-related quality of life (SGRQ) and health status (CAT) following rehabilitation. <b>Conclusion:</b> PR program fidelity can be maintained when delivering HBPR to people with COPD referred directly from general practice. Early engagement with PR may be key to supporting rehabilitation completion.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241307247"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11618893/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142779437","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The impact on thirty day readmissions for patients hospitalized for acute exacerbations of chronic obstructive pulmonary disease admitted to an observation unit versus an inpatient medical unit: A retrospective observational study.","authors":"Jacob Bell, Steven Lim, Takahisa Mikami, Jeeyune Bahk, Stephen Argiro, David Steiger","doi":"10.1177/14799731241242490","DOIUrl":"10.1177/14799731241242490","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to evaluate the utility of an Observation Unit (OU) in management of acute exacerbations of chronic obstructive pulmonary disease (AECOPD) and to identify the clinical characteristics of patients readmitted within 30-days for AECOPD following index admission to the OU or inpatient floor from the OU.</p><p><strong>Methods: </strong>This is a retrospective observational study of patients admitted from January to December 2017 for AECOPD to an OU in an urban-based tertiary care hospital. Primary outcome was rate of 30-day readmission after admission for AECOPD for patients discharged from the OU versus inpatient service after failing OU management. Regression analyses were used to define risk factors.</p><p><strong>Results: </strong>163 OU encounters from 92 unique patients were included. There was a lower readmission rate (33%) for patients converted from OU to inpatient care versus patients readmitted after direct discharge from the OU (44%). Patients with 30-day readmissions were more likely to be undomiciled, with history of congestive heart failure (CHF), pulmonary embolism (PE), or had previous admissions for AECOPD. Patients with >6 annual OU visits for AECOPD had higher rates of substance abuse, psychiatric diagnosis, and prior PE; when these patients were excluded, the 30-day readmission rate decreased to 13.5%.</p><p><strong>Conclusion: </strong>Patients admitted for AECOPD with a history of PE, CHF, prior AECOPD admissions, and socioeconomic deprivation are at higher risk of readmission and should be prioritized for direct inpatient admission. Further prospective studies should be conducted to determine the clinical impact of this approach on readmission rates.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241242490"},"PeriodicalIF":4.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10981268/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140305102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical implications of frailty assessed in hospitalized patients with acute-exacerbation of interstitial lung disease.","authors":"Marine Van Hollebeke, Karan Chohan, Colin J Adams, Jolene H Fisher, Shane Shapera, Lee Fidler, Ewan C Goligher, Tereza Martinu, Lisa Wickerson, Sunita Mathur, Lianne G Singer, W Darlene Reid, Dmitry Rozenberg","doi":"10.1177/14799731241240786","DOIUrl":"10.1177/14799731241240786","url":null,"abstract":"<p><strong>Background: </strong>Approximately 50% of patients with interstitial lung disease (ILD) experience frailty, which remains unexplored in acute exacerbations of ILD (AE-ILD). A better understanding may help with prognostication and resource planning. We evaluated the association of frailty with clinical characteristics, physical function, hospital outcomes, and post-AE-ILD recovery.</p><p><strong>Methods: </strong>Retrospective cohort study of AE-ILD patients (01/2015-10/2019) with frailty (proportion ≥0.25) on a 30-item cumulative-deficits index. Frail and non-frail patients were compared for pre- and post-hospitalization clinical characteristics, adjusted for age, sex, and ILD diagnosis. One-year mortality, considering transplantation as a competing risk, was analysed adjusting for age, frailty, and Charlson Comorbidity Index (CCI).</p><p><strong>Results: </strong>89 AE-ILD patients were admitted (median: 67 years, 63% idiopathic pulmonary fibrosis). 31 were frail, which was associated with older age, greater CCI, lower 6-min walk distance, and decreased independence pre-hospitalization. Frail patients had more major complications (32% vs 10%, <i>p</i> = .01) and required more multidisciplinary support during hospitalization. Frailty was not associated with 1-year mortality (HR: 0.97, 95%CI: [0.45-2.10]) factoring transplantation as a competing risk.</p><p><strong>Conclusions: </strong>Frailty was associated with reduced exercise capacity, increased comorbidities and hospital complications. Identifying frailty may highlight those requiring additional multidisciplinary support, but further study is needed to explore whether frailty is modifiable with AE-ILD.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241240786"},"PeriodicalIF":4.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10958799/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140183872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between illness perception and adherence to inhaler therapy in elderly Chinese patients with chronic obstructive pulmonary disease.","authors":"You-Ran Liu, Yan Wang, Jie Wang, Xi Wang","doi":"10.1177/14799731241286837","DOIUrl":"10.1177/14799731241286837","url":null,"abstract":"<p><p><b>Background:</b> Despite the fact that inhaled medications serve as the foundation of chronic obstructive pulmonary disease (COPD) treatment, patient adherence to inhaler therapy remains low, significantly impacting health outcomes in disease management. The Common Sense Model of Self-Regulation suggests that illness perception plays a crucial role in individual behavior. Nevertheless, the relationship between illness perception and inhaler adherence, as well as the underlying mechanisms, remains unclear in the elderly Chinese COPD population. <b>Objective:</b> This study aimed to explore the correlation between dimensions of illness perception and adherence to inhaler therapy in elderly Chinese patients with COPD. <b>Methods:</b> A cross-sectional study was conducted by recruiting 305 participants (mean age: 70.96 years; 69.8% male) using convenience sampling from a tertiary hospital in Anhui, China. The Chinese versions of the Test of Adherence to Inhalers (TAI) and Brief Illness Perception Questionnaire (B-IPQ) were used to evaluate adherence to inhalation and perception of their illness in patients with COPD. Binary logistic regression analyses were used to explore the relationship between inhaler adherence and illness perception in patients with COPD. <b>Results:</b> 84.3% of participants showed poor adherence, and the mean (standard deviation) B-IPQ total score was 44.87 (6.36). The results indicated an essential correlation between illness perception and inhaler adherence. Specifically, personal control (AOR = 2.149, <i>p</i> < 0.001), treatment control (AOR = 1.743, <i>p</i> < 0.001), comprehension (AOR = 5.739, <i>p</i> < 0.001) and emotions (AOR = 1.946, <i>p</i> < 0.001) within illness perception emerged as significant positive predictors for inhaler adherence among patients with COPD. <b>Conclusion:</b> This study suggests that clinical practitioners should monitor the illness perception of patients with COPD and develop targeted intervention measures to improve patient adherence to inhaler therapy.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241286837"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11425765/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142307239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Factors influencing patient-provider communication about subjective cognitive decline in people with COPD: Insights from a national survey.","authors":"Michael Stellefson, Min Qi Wang, Olivia Campbell","doi":"10.1177/14799731241268338","DOIUrl":"10.1177/14799731241268338","url":null,"abstract":"<p><p><b>Objective:</b> While there is a growing body of evidence indicating a relationship between COPD and cognitive impairment, there is a gap in evidence regarding discussions of cognitive symptoms in healthcare settings. This study investigated the extent to which individuals with Chronic Obstructive Pulmonary Disease (COPD) and Subjective Cognitive Decline (SCD) self-reported confusion or memory loss with healthcare professionals. <b>Methods:</b> A secondary analysis of 2019 BRFSS data of US adults aged 45+ with COPD (<i>N</i> = 107,204), using logistic regression to explore associations between socio-demographic and health-related indicators with discussion of cognitive symptoms with healthcare professionals. <b>Results:</b> Less than half (45.88%) of individuals reporting SCD discussed their cognitive symptoms with their healthcare provider. In the adjusted model, unemployed (AOR = 2.92, 95% CI: 1.70-5.02, <i>p</i> < .005), retired (AOR = 3.16, 95% CI: 1.37-7.30, <i>p</i> < .01), and current smokers (AOR = 1.73, 95% CI: 1.02-2.93, <i>p</i> < .05) were more likely to discuss cognitive decline with a healthcare professional than their counterparts. In contrast, males (AOR = 0.53, 95% CI: 0.32-0.86, <i>p</i> < .05) and binge drinkers (AOR = 0.49, 95% CI: 0.30-0.79, <i>p</i> < .01) were significantly less likely to do so when compared to their counterparts. <b>Discussion:</b> The study highlighted significant disparities in the likelihood of individuals with COPD discussing cognitive symptoms based on socio-demographic and health risk behaviors. <b>Conclusion:</b> Addressing gender disparities, occupational status, and personal health risks is crucial for improving patient-provider communication about SCD among adults with COPD.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241268338"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11292718/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141859200","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The effects of inspiratory muscle training on biomarkers of muscle damage in recovered COVID-19 patients after weaning from mechanical ventilation.","authors":"Muneeb Iqbal, Kumail Hassan, Edward Bliss, Eliza J Whiteside, Ben Hoffman, Dean E Mills","doi":"10.1177/14799731241289423","DOIUrl":"10.1177/14799731241289423","url":null,"abstract":"<p><p><b>Background:</b> COVID-19 patients experience respiratory muscle damage, leading to reduced respiratory function and functional capacity often requiring mechanical ventilation which further increases susceptibility to muscle weakness. Inspiratory muscle training (IMT) may help mitigate this damage and improve respiratory function and functional capacity. <b>Methods:</b> We studied the effects of IMT on muscle damage biomarkers, respiratory function, and functional capacity in COVID-19 recovered young adults, successfully weaned from mechanical ventilation. Participants were randomly allocated to either an IMT (<i>n</i> = 11) or control (CON; <i>n</i> = 11) intervention for 4 weeks. The IMT group performed 30 dynamic inspiratory efforts twice daily, at 50% of their maximal inspiratory mouth pressure (P_Mmax) while the CON group performed 60 inspiratory efforts at 10% of <i>p</i>_Mmax daily. Serum was collected at baseline, week two, and week four to measure creatine kinase muscle-type (CKM), fast skeletal troponin-I (sTnI) and slow sTnI. <b>Results:</b> Time × group interaction effects were observed for CKM and slow sTnI, but not for fast sTnI. Both were lower at two and 4 weeks for the IMT compared to the CON group, respectively. Time × group interaction effects were observed for forced expiratory volume in 1s, forced vital capacity, P_Mmax and right- and left-hand grip strength. These were higher for the IMT compared to the CON group. <b>Conclusion:</b> Four weeks of IMT decreased muscle damage biomarkers and increased respiratory function and grip strength in recovered COVID-19 patients after weaning from mechanical ventilation.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241289423"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457248/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142371133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Respiratory impairments in patients suffering from Fabry disease - A cross-sectional study.","authors":"Huma Ahmed, Vibeke Backer, Grigoris Effraimidis, Åse Krogh Rasmussen, Caroline Michaela Kistorp, Ulla Feldt-Rasmussen","doi":"10.1177/14799731231221821","DOIUrl":"10.1177/14799731231221821","url":null,"abstract":"<p><strong>Background: </strong>The inherited X-linked disorder, Fabry disease, is caused by deficient lysosomal enzyme α-galactosidase A, with progressive accumulation of globotriaosylceramide in multiple organs including the upper and lower airways.</p><p><strong>Objectives: </strong>To assess pulmonary function at the time of the first pulmonary function test (PFT) performed among the National Danish Fabry cohort and define the prevalence of affected lung function variables.</p><p><strong>Materials and method: </strong>A cross-sectional retrospective cohort study of 86 adult patients enrolled in one or both international patient registry databases for Fabry disease, <i>Fabry Registry</i> or <i>FollowME</i> with at least one PFT. The Mainz Severity Score Index (MSSI) was calculated to determine the disease severity. Lung function variables were examined by multivariate regression adjusted for important variables for developing airway illness.</p><p><strong>Results: </strong>Seventeen patients (20%) showed obstructive airflow limitation and 7 (8%) a restrictive lung deficiency. Smoking status (<i>p</i> = .016) and MSSI (<i>p</i> < .001) were associated with increasing obstructive airway limitation.</p><p><strong>Conclusion: </strong>The prevalence of affected lung function among the National Danish Fabry cohort was 28%. Patients with classic gene variants frequently developed a decrease in lung function regardless of their smoking status, with significant relationship with disease severity.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731231221821"},"PeriodicalIF":4.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10858660/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139706271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Antifibrotic drug treatment of patients with idiopathic pulmonary fibrosis in Sweden: A registry-based observational study.","authors":"Lisa Carlson, Dimitrios Kalafatis, Ida Pesonen, Jesper M Magnusson, Magnus Skold","doi":"10.1177/14799731241299443","DOIUrl":"10.1177/14799731241299443","url":null,"abstract":"<p><strong>Objectives: </strong>Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis of the lung parenchyma, resulting in respiratory failure. This study analysed differences in patient characteristics and antifibrotic treatment strategies during the first years after IPF diagnosis.</p><p><strong>Methods: </strong>Data from patients with IPF was extracted from the Swedish IPF registry. Patients were defined as treated (either as fully- or reduced treated) or non-treated with antifibrotic drugs. Differences in clinical parameters and side effects were defined.</p><p><strong>Results: </strong>Among 532 patients, 371 received treatment with antifibrotic drugs. Treated patients were younger, had worse lung function, higher body mass index (BMI), higher Gender-Age-Physiology stage, and were more often on oxygen treatment. Non-treated patients displayed a stable BMI, whereas patients treated with antifibrotics declined in BMI during follow-up. More than half (56%) of treated patients had reduced antifibrotic treatment. Sixty per cent reported side effects, with diarrhoea, nausea, and skin rash as the most common.</p><p><strong>Conclusions: </strong>Patients prescribed antifibrotic treatment had more advanced disease compared to patients not prescribed antifibrotics. A considerable proportion of the patients had reduced treatment, probably due to more side effects in this group. This indicates that individuals starting treatment at IPF diagnosis are considered to be in greater need of antifibrotic drug treatment by the prescriber, compared to individuals with less severe disease.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"21 ","pages":"14799731241299443"},"PeriodicalIF":3.5,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11558727/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616010","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}