Therapeutic Advances in Respiratory Disease最新文献

{"title":"New era, new GOALs: cardiovascular screening and lipid management in cystic fibrosis.","authors":"Katherine A Despotes, Agathe S Ceppe, Jennifer L Goralski, Scott H Donaldson","doi":"10.1177/17534666251317200","DOIUrl":"10.1177/17534666251317200","url":null,"abstract":"<p><strong>Background: </strong>Cardiovascular disease (CVD) risks are increasing in people with cystic fibrosis (pwCF). While cholesterol levels were historically low in pwCF, higher levels after initiating highly effective modulator therapy (HEMT) have been reported. Mechanisms are unclear and there is little guidance on screening.</p><p><strong>Objectives: </strong>To evaluate serum lipid changes at multiple timepoints after ivacaftor initiation, and to assess current screening practices for CVD risk factors among CF providers.</p><p><strong>Design: </strong>This was a post-hoc correlative analysis of prospectively collected clinical data and serum samples from the GOAL cohort study. Cross-sectional survey methodology was also employed.</p><p><strong>Methods: </strong>We evaluated serum lipids (total cholesterol (TC), low-density lipoprotein (LDL), and high-density lipoprotein (HDL)) at baseline, 3- and 18 months after ivacaftor initiation using samples from the GOAL study biorepository. We also surveyed CF providers across the United States on their CVD risk screening practices.</p><p><strong>Results: </strong>Fifty GOAL participants' samples were analyzed. Using the repeated measures model, TC significantly varied by visit (<i>p</i> = 0.004), driven by a significant increase from baseline at 3 months (mean difference 9.4 mg/dL). This difference diminished by 18 months. BMI was a significant covariate for TC. No significant differences by visit were detected in LDL or HDL. Seventy-five respondents participated in the survey (response rate 5.6%; 41 adult providers, 18 pediatric providers, and 10 providers caring for both) with 67% reporting no lipid screening policy existed in their center. In the past year, 29% of adult providers prescribed lipid-lowering therapy, 54% started anti-hypertensive medications, and 48% initiated ischemic cardiac evaluations for pwCF.</p><p><strong>Conclusion: </strong>TC significantly increased within 3 months of initiating ivacaftor, but subsequently diminished toward baseline by 18 months. Lipid screening practices among CF providers were variable and providers are increasingly being confronted with managing CVD risk factors. Partnering with primary care providers is likely to become increasingly important in CF care models.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251317200"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11786282/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143075525","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":"Early, integrated palliative care for people with chronic respiratory disease: lessons learnt from lung cancer.","authors":"Anne M Walker, Donald R Sullivan, Phan Nguyen, Anne E Holland, Natasha Smallwood","doi":"10.1177/17534666241305497","DOIUrl":"https://doi.org/10.1177/17534666241305497","url":null,"abstract":"<p><p>Lung cancer and chronic non-malignant respiratory disease cause pervasive, multifactorial suffering for patients and informal carers alike. Palliative care aims to reduce suffering and improve quality of life for patients and their families. An established evidence base exists that has demonstrated the essential role of specialist palliative care for people with lung cancer. Emerging evidence supports similar benefits among people with chronic respiratory disease. Many lessons can be learnt from lung cancer care, particularly as the model of care delivery has transformed over recent decades due to major advances in the diagnostic pathway and the development of new treatments. This narrative review aims to summarize the evidence for specialist palliative care in lung cancer and chronic respiratory disease, by highlighting seven key lessons from lung cancer care that can inform the development of proactive, integrated models of palliative care among those with chronic respiratory disease. These seven lessons emphasize (1) managing challenging symptoms; (2) the efficacy of specialist palliative care; (3) the importance of providing specialist palliative care integrated with disease-directed care according to patients' needs not prognosis; (4) the need for new models of collaborative palliative care, (5) which are culturally appropriate and (6) able to evolve with changes in disease-directed care. Finally, we discuss (7) some of the critical research gaps that persist and reduce implementation in practice.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666241305497"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143374274","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The impact of quantitative platform on candidacy for bronchoscopic lung volume reduction: a multi-center retrospective cohort study.","authors":"Max Wayne, Suchitra Pilli, Hee Jae Choi, Nathaniel Moulton, Praveen Chenna, Allen Cole Burks, Alexander Chen","doi":"10.1177/17534666251314724","DOIUrl":"10.1177/17534666251314724","url":null,"abstract":"<p><strong>Background: </strong>Bronchoscopic lung volume reduction (BLVR) can be an effective treatment for highly selected patients with severe emphysema but only half of carefully selected patients derive clinical benefit. Two commercially available platforms exist to help determine candidacy for BLVR via quantitative analysis of computed tomography (CT) scans.</p><p><strong>Objectives: </strong>To determine if the two commercially available quantitative platforms identified the same patient population that may benefit from BLVR.</p><p><strong>Design: </strong>A multicenter, retrospective cohort study.</p><p><strong>Methods: </strong>Consecutive patients referred for BLVR between January 1, 2022 and March 31, 2023 at three medical centers in the United States with the same CT scan submitted for quantitative analysis to two commercially available platforms to determine BLVR candidacy were analyzed. The primary outcome of interest was whether quantitative analysis provided different recommendations for individual patients. The recommendation to proceed with BLVR was based on a prespecified algorithm using criteria established in clinical trials for each quantitative platform, respectively.</p><p><strong>Results: </strong>A total of 83 patients referred for BLVR across three centers were included; patients were a median 67 years old, had a median post bronchodilator FEV1 of 30% predicted (IQR: 25, 38), a median residual volume of 220% predicted (IQR: 185, 268), and 29 (34.9%) received endobronchial valves. A total of 26 patients (31.3%) received different recommendations from the two quantitative platforms.</p><p><strong>Conclusion: </strong>In this cohort of patients evaluated for BLVR across multiple medical centers, nearly a third of patients received different recommendations based on the platform utilized for valve assessment. This suggests that the selection process for BLVR may warrant refinement.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251314724"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11783484/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143068062","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 albumin corrected anion gap and in-hospital mortality in critically ill patients with chronic obstructive pulmonary disease.","authors":"Mohan Giri, Anju Puri, Lan Huang, Shuliang Guo","doi":"10.1177/17534666251315352","DOIUrl":"10.1177/17534666251315352","url":null,"abstract":"<p><strong>Background: </strong>The relationship between albumin-corrected anion gap (ACAG) and in-hospital mortality in critically ill patients with COPD remains unclear.</p><p><strong>Objective: </strong>This study investigated the association between ACAG levels and the risk of in-hospital mortality in critically ill patients with COPD.</p><p><strong>Design: </strong>A retrospective cohort study.</p><p><strong>Methods: </strong>This study uses data from the Medical Information Mart for Intensive Care (MIMIC-IV) database. The receiver operating characteristic (ROC) curve was used to determine the optimal threshold for ACAG, and participants were divided into two categories based on this threshold. The primary outcome was in-hospital mortality. We employed univariable and multivariable logistic regression analyses and Kaplan-Meier (KM) survival curves to assess the relationship between ACAG and the risk of in-hospital mortality. Moreover, subgroup analyses were conducted.</p><p><strong>Results: </strong>A total of 2121 patients (54.7% male) were enrolled in the study. The in-hospital mortality rate was 18.9%. In patients with elevated ACAG levels, the in-hospital mortality rate was significantly higher than in those with lower ACAG levels (27.7% vs 11.3%, <i>p</i> < 0.001). Multivariate logistic regression analysis indicated that even after mitigating for potential confounders, patients in the high ACAG group had significantly greater odds of in-hospital mortality across all models (Model I: OR = 3.000, 95% CI: 2.383-3.777, <i>p</i> < 0.001; Model II: OR = 3.021, 95% CI: 2.397-3.808, <i>p</i> < 0.001; Model III: OR = 1.916, 95% CI: 1.458-2.519, <i>p</i> < 0.001). Patients with elevated ACAG levels have more than twice the risk of in-hospital mortality compared to those with lower levels (hazard ratio (HR): 2.1277, 95% CI: 1.7490-2.5884).</p><p><strong>Conclusion: </strong>This study demonstrates that elevated ACAG levels are strongly associated with an increased risk of in-hospital mortality in critically ill COPD patients, suggesting that ACAG could serve as a potential predictor of adverse outcomes in this patient population.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251315352"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11770725/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143047321","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":"Sleep and breathing in children with Joubert syndrome and a review of other rare congenital hindbrain malformations.","authors":"Jia-Der Ju-Wang, Jennifer C Dempsey, Cristian Zhang, Daniel Doherty, Manisha Witmans, Mary Anne Tablizo, Maida Lynn Chen","doi":"10.1177/17534666241308405","DOIUrl":"https://doi.org/10.1177/17534666241308405","url":null,"abstract":"<p><strong>Background: </strong>Joubert syndrome (JS) is an autosomal recessive disorder with a distinctive mid-hindbrain malformation known as the \"molar tooth sign\" which involves the breathing control center and its connections with other structures. Literature has reported significant respiratory abnormalities which included hyperpnea interspersed with apneic episodes during wakefulness. Larger-scale studies looking at polysomnographic findings or subjective reports of sleep problems in this population have not yet been published.</p><p><strong>Objectives: </strong>The primary objectives were (1) compare a large group of children with JS and their unaffected siblings for caregiver-reported sleep difficulties. Secondary objectives were (1) present new polysomnography (PSG) data on our JS cohort; (2) review sleep disordered breathing (SDB) in other rare congenital hindbrain anatomic abnormalities.</p><p><strong>Design: </strong>We conducted a cross-sectional study on a cohort of 109 families affected by JS.</p><p><strong>Methods: </strong>Pediatric Sleep Questionnaire (PSQ) and the Children's Sleep Habits Questionnaire (CSHQ) along with general medical health information focused on respiratory and sleep problems were mailed to all patients and families. Caregivers were asked to complete the survey for both children with JS and unaffected siblings, if any. Baseline diagnostic PSG was retrospectively reviewed for those with available studies, and the sleep parameters were compared to a referent cohort.</p><p><strong>Results: </strong>Study participants with JS were older than their unaffected siblings (<i>p</i> = 0.02). Genetic mutations were available for 41 out of 118 individuals, with the most common mutation being MKS3 (31.4%). Patients with JS had higher scores in the PSQ compared to their unaffected siblings (<i>p</i> < 0.001). PSG data showed severe SDB with apnea-hypopnea index (AHI) of 23 ± 15 events/h in patients with JS. Events were primarily obstructive (obstructive AHI 18 ± 15 events/h vs central AHI 4 ± 4 events/h). Abnormal sleep architecture with increased arousal indices, decreased efficiency, and more time awake and in light sleep or wakefulness when compared to the referent data.</p><p><strong>Conclusion: </strong>SDB is common and severe in patients with JS, and the significantly greater obstructive component reported in this cohort makes it necessary to perform complete PSG studies to address or prevent clinical manifestations in this at-risk population. PSQ could represent a viable method to screen for SDB in JS.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666241308405"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11748088/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143056043","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":"Potential of phosphodiesterase 4B inhibition in the treatment of progressive pulmonary fibrosis.","authors":"Rebecca Keith, Anoop M Nambiar","doi":"10.1177/17534666241309795","DOIUrl":"10.1177/17534666241309795","url":null,"abstract":"<p><p>Idiopathic pulmonary fibrosis (IPF) is often regarded as the archetypal progressive fibrosing interstitial lung disease (ILD). The term \"progressive pulmonary fibrosis\" (PPF) generally describes progressive lung fibrosis in an individual with an ILD other than IPF. Both IPF and PPF are associated with loss of lung function, worsening dyspnea and quality of life, and premature death. Current treatments slow the decline in lung function but have side effects that may deter the initiation or continuation of treatment. There remains a high unmet need for additional therapies that can be used alone or in combination with current therapies to preserve lung function in patients with IPF and PPF. Phosphodiesterase-4 (PDE4) is an enzyme involved in the regulation of inflammatory processes. Pre-clinical studies have shown that preferential inhibition of PDE4B has anti-inflammatory and antifibrotic effects and a lower potential for gastrointestinal adverse events than pan-PDE4 inhibition. The preferential PDE4B inhibitor nerandomilast demonstrated efficacy in preserving lung function in a phase II trial in patients with IPF and is under investigation in phase III trials as a treatment for IPF and PPF.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666241309795"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11694302/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915713","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":"A narrative review of proactive palliative care models for people with COPD.","authors":"Amy Pascoe, Xinye Chen, Natasha Smallwood","doi":"10.1177/17534666241310987","DOIUrl":"https://doi.org/10.1177/17534666241310987","url":null,"abstract":"<p><p>Chronic obstructive pulmonary disease (COPD) refers to a group of lung diseases that are distinct in underlying aetiology but share a common disease course of persistent and progressive airflow restriction. People living with COPD, as well as the people who care for them, frequently have severe and unmet physical and psychosocial needs, including breathlessness, fatigue, cough, anxiety and depression. Early proactive palliative care is well placed to address these needs, yet it is frequently under-utilised in this group. This narrative review aimed to identify core components of palliative care and examine how existing models of care are implemented to better understand which models can best serve the needs of people with COPD. Symptom palliation, advance care planning, and support for caregivers emerged as the common components underpinning both generalist and specialist models of palliative care. Models of proactive palliative care were diverse in terms of where and how care was delivered as well as which health professionals were involved. Five key models of palliative care were identified: (1) multi-disciplinary integrated services, (2) nurse-led care, (3) hospice and residential aged care, (4) home-based care, and (5) telemonitoring and telehealth. Each model describes a diverse set of interventions and many of these share common elements, including the normalisation of palliative principles within routine care and the provision of diverse delivery settings to accommodate individual preferences and needs. Successful palliative care models must be practical, accessible and innovative to respond to individuals' complex and evolving needs, foster multi-disciplinary collaboration and input and optimally utilise local healthcare resources.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666241310987"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143374264","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A real-world study to evaluate effectiveness of mepolizumab in treating severe asthma in Taiwan (REMIT).","authors":"Shih-Lung Cheng, Shu-Min Lin, Chung-Kan Peng, Ming-Cheng Chan, Sheng-Yeh Shen, Ping-Hung Kuo, Chien-Hao Lai, Chou-Chin Lan, Chung-Yu Chen, Ching-Hsiung Lin, Kuang-Ming Liao, Po-Hao Feng, Jiin-Torng Wu, Yu-Feng Wei, Xiaomeng Xu, Rafael Alfonso-Christancho, Tina Lai, Aldo Navarro, Dominique Milea, Diahn-Warng Perng","doi":"10.1177/17534666241308406","DOIUrl":"10.1177/17534666241308406","url":null,"abstract":"<p><strong>Background: </strong>REMIT is the first real-world study of mepolizumab effectiveness in patients with severe asthma (SA) in Taiwan.</p><p><strong>Objectives: </strong>The primary objective evaluated changes in clinically significant exacerbations (CSEs; defined as use of oral corticosteroids (OCS) or emergency department (ED) visits and/or hospitalizations) in the 12 months pre- and post-mepolizumab treatment. Secondary objectives assessed changes in the number of CSEs requiring ED visits/hospitalizations and daily maintenance OCS (mOCS) dosage 12 months pre- and post-mepolizumab treatment. Three- and four-component clinical remissions were analyzed based on OCS-free, exacerbation-free, and asthma control (± stability in lung function).</p><p><strong>Design: </strong>REMIT was a retrospective, observational, self-controlled study analyzing patients in Taiwan with SA who were newly prescribed subcutaneous mepolizumab 100 mg Q4W.</p><p><strong>Methods: </strong>Data were extracted from records of 15 medical centers in Taiwan for patients indexed between November 1, 2018 and October 31, 2020.</p><p><strong>Results: </strong>A total of 170 patients were included: mean age at index date, 58.7 years; 53.5% female; 100% Chinese; 7.1% with chronic rhinosinusitis with nasal polyps, 1.8% with eosinophilic granulomatosis with polyangiitis, 1.2% with hypereosinophilic syndrome; and 55.7% with blood eosinophil count >300/µL. Pre-treatment, 71.2% had ⩾2 exacerbations, and 28.7% were on mOCS; 75.3% had no prior biologic treatment, and 24.7% had switched from other biologics. Most patients (80.0%) completed ⩾10 mepolizumab doses. Following the first mepolizumab administration (index date), CSEs reduced by 46.0% (rate ratio (RR): 0.545, 95% confidence interval (CI): 0.418-0.710; <i>p</i> < 0.0001) in the 12 months post-index. Exacerbations requiring ED visits/hospitalization reduced by 46.9% (RR: 0.531, 95% CI: 0.349-0.808; <i>p</i> = 0.0031). Median mOCS dose reduced by 100% by end of study and 81.8% of patients discontinued mOCS post-treatment. After 1 year of mepolizumab treatment, 28% and 23% patients achieved three- and four-component clinical remission, respectively.</p><p><strong>Conclusion: </strong>Mepolizumab use in a patient population in Taiwan with SA significantly reduced CSEs and mOCS use in routine clinical practice.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666241308406"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11744627/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143011950","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":"Real-world therapeutic performance of pirfenidone for connective tissue disease-associated interstitial lung diseases.","authors":"Xueting Yuan, Chen Yu, Shengyun Liu, Qiang Shu, Xinwang Duan, Lin Tang, Liying Peng, Shuang Zhou, Chanyuan Wu, Jiuliang Zhao, Dong Xu, Lan Song, Hui Huang, Mengtao Li, Yanhong Wang, Qian Wang, Xiaofeng Zeng","doi":"10.1177/17534666241292507","DOIUrl":"10.1177/17534666241292507","url":null,"abstract":"<p><strong>Background: </strong>Pirfenidone (PFD) is commonly applied for antifibrotic treatment in patients with idiopathic pulmonary fibrosis but has rarely been studied in cases with connective tissue disease-associated interstitial lung diseases (CTD-ILDs).</p><p><strong>Objectives: </strong>We aimed to examine the efficacy of PFD in patients with CTD-ILD based on real-world data.</p><p><strong>Design: </strong>A retrospective cohort study.</p><p><strong>Methods: </strong>This study assessed the clinical features of CTD-ILD patients with or without a 6-month PFD treatment. A linear mixed effects model was employed to evaluate the effectiveness of PFD in alleviating lung function changes. Differences in response to PFD were analyzed based on CTD subtype, imaging classification, and pattern of pulmonary function at baseline.</p><p><strong>Results: </strong>A total of 289 patients with CTD-ILD were included, with 155 (53.6%) receiving PFD treatment and the remaining constituting the control group. Patients with the usual interstitial pneumonia (UIP) pattern were more likely to receive PFD treatment, and a relatively lower proportion of cases in the PFD group received immunosuppressive therapies compared to the control group (<i>p</i> < 0.05). At the 6-month follow-up, patients in the PFD group demonstrated a more significant improvement in forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLCO) (ΔFVC%: 2.9% vs 0.45%, <i>p</i> = 0.009; ΔDLCO%: 1.9% vs -1.1%, <i>p</i> = 0.004). In the linear mixed model analysis, there was a statistically significant group-time interaction between FVC% and DLCO% changes over time (FVC%: β = 4.52, <i>p</i> < 0.001; DLCO%: β = 4.13, <i>p</i> = 0.003). Furthermore, subgroup analysis indicated that pirfenidone may have superior therapeutic effects in patients with systemic sclerosis (SSc)-associated ILD, non-UIP pattern, and restrictive pattern of lung function at baseline.</p><p><strong>Conclusion: </strong>This study provided real-world data demonstrating the effectiveness of PFD in terms of lung function improvement in patients with CTD-ILD.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"18 ","pages":"17534666241292507"},"PeriodicalIF":4.3,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11544659/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142605139","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":"Video-assisted thoracoscopic surgery for non-cystic fibrosis bronchiectasis in children.","authors":"Fengxia Ding, Zhengxia Pan, Chun Wu, Hongbo Li, Yonggang Li, Yong An, Jiangtao Dai, Gang Wang, Bo Liu","doi":"10.1177/17534666241228159","DOIUrl":"10.1177/17534666241228159","url":null,"abstract":"<p><strong>Background: </strong>Pediatric bronchiectasis is a common respiratory disease in children. The use of video-assisted thoracoscopic surgery (VATS) for its treatment remains controversial.</p><p><strong>Objectives: </strong>The objective of our study was to compare and analyze the clinical efficacy of thoracoscopic surgery and thoracotomy in the treatment of pediatric bronchiectasis and summarize the surgical treatment experience of VATS in children with bronchiectasis.</p><p><strong>Design: </strong>Retrospective single-center cohort study.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on the clinical data of 46 pediatric patients who underwent surgery with bronchiectasis at the Children's Hospital of Chongqing Medical University from May 2015 to May 2023. The patients were divided into two groups: the VATS group (25 cases) and the thoracotomy group (21 cases). Comparative analysis was performed on various parameters including basic clinical data, surgical methods, operation time, intraoperative blood loss, transfusion status, postoperative pain, postoperative mechanical ventilation time, chest tube drainage time, length of hospital stay, incidence of complications, and follow-up information.</p><p><strong>Results: </strong>There were no statistically significant differences between the two groups of patients in terms of age, weight, gender, etiology, duration of symptoms, site of onset, and comorbidities (<i>p</i> > 0.05). The operation time in the VATS group was longer than that in the thoracotomy group (<i>p</i> < 0.001). However, the VATS group had better outcomes in terms of intraoperative blood loss, transfusion status, postoperative pain, postoperative mechanical ventilation time, chest tube drainage time, and length of hospital stay (<i>p</i> < 0.05). The incidence of postoperative complications in the VATS group was lower than that in the thoracotomy group, although the difference was not statistically significant (<i>p</i> = 0.152). Follow-up data showed no statistically significant difference in the surgical treatment outcomes between the two groups (<i>p</i> = 0.493).</p><p><strong>Conclusion: </strong>The incidence of complications and mortality in surgical treatment of bronchiectasis is acceptable. Compared with thoracotomy surgery, VATS has advantages such as smaller trauma, less pain, faster recovery, and fewer complications. For suitable pediatric patients with bronchiectasis, VATS is a safe and effective surgical method.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"18 ","pages":"17534666241228159"},"PeriodicalIF":4.3,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10851711/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139703509","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}