Jia-Der Ju-Wang, Jennifer C Dempsey, Cristian Zhang, Daniel Doherty, Manisha Witmans, Mary Anne Tablizo, Maida Lynn Chen
{"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":"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}
Dominic L Sykes, Kayleigh Brindle, Rohan Menon, Simon P Hart, Jennifer Nielsen, Warren Jackson, John Gallagher, Elisabeth Kirton, Mengru Zhang, Alyn H Morice, Michael G Crooks
{"title":"Azithromycin and oesophageal motility in chronic respiratory disease: a feasibility study.","authors":"Dominic L Sykes, Kayleigh Brindle, Rohan Menon, Simon P Hart, Jennifer Nielsen, Warren Jackson, John Gallagher, Elisabeth Kirton, Mengru Zhang, Alyn H Morice, Michael G Crooks","doi":"10.1177/17534666251360065","DOIUrl":"https://doi.org/10.1177/17534666251360065","url":null,"abstract":"<p><strong>Background: </strong>The role of the gut-lung axis in respiratory disease is increasingly recognised. Much emphasis has been placed on gastro-oesophageal reflux disease; however, oesophageal dysmotility may also play a significant role. Azithromycin, a known prokinetic, has been shown to be of major benefit in a number of respiratory diseases, but the relationship between oesophageal function and the lung has not been examined.</p><p><strong>Objectives: </strong>We assessed the feasibility of performing continuous cough monitoring and repeated high-resolution oesophageal manometry (HROM) in patients with chronic respiratory disease.</p><p><strong>Design: </strong>We conducted an open-label, single-arm, feasibility trial.</p><p><strong>Methods: </strong>Azithromycin 250 mg once daily was given to patients with chronic respiratory disease who reported a chronic cough. All participants were monitored continually for at least 1 week prior to and 4 weeks after azithromycin with the Hyfe Cough Tracker. Participants also had HROM performed at two time-points, immediately before and 4 weeks after initiation of azithromycin. Feasibility outcomes pertaining to recruitment, data quality, and acceptability of trial processes were assessed. Exploratory outcome data for metrics of oesophageal function were also analysed.</p><p><strong>Results: </strong>A total of 30 participants (57% female, mean age 65.2 (SD = 11.3)) were recruited over a 10-month period, giving a recruitment rate of three patients per month in a single centre. A total of 87% (<i>n</i> = 26) of participants completed all three study visits. All pre-specified data quality outcomes met their 'green' traffic light stop-go criteria. HROM demonstrated that the majority (52%) of participants had abnormal oesophageal function, as defined by the Chicago Classification, at baseline. Changes in oesophageal function were not significantly associated with changes in objective or subjective cough measures, except for a weakly negative correlation with the Hull Airway Reflux Questionnaire score.</p><p><strong>Conclusion: </strong>A large-scale trial examining the effect of azithromycin on the relationship between oesophageal function and cough in respiratory disease is feasible and acceptable to patients.</p><p><strong>Trial registration: </strong>This trial was prospectively registered ClinicalTrials.gov ID: NCT05469555.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251360065"},"PeriodicalIF":3.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144745126","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":"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}
Zhengtong Qiao, Ziwei Kou, Jiazhen Zhang, Daozheng Lv, Xuefen Cui, Dongpan Li, Tao Jiang, Xinjuan Yu, Kai Liu
{"title":"Optimal intensity and type of lower limb aerobic training for patients with chronic obstructive pulmonary disease: a systematic review and network meta-analysis of RCTs.","authors":"Zhengtong Qiao, Ziwei Kou, Jiazhen Zhang, Daozheng Lv, Xuefen Cui, Dongpan Li, Tao Jiang, Xinjuan Yu, Kai Liu","doi":"10.1177/17534666251323190","DOIUrl":"10.1177/17534666251323190","url":null,"abstract":"<p><strong>Background: </strong>Lower limb aerobic exercise is the core component of pulmonary rehabilitation for chronic obstructive pulmonary disease (COPD) patients. The optimal intensity and type (e.g., interval or continuous) of exercise training remains to be determined.</p><p><strong>Objectives: </strong>We aimed to evaluate the optimal intensities and types of lower limb aerobic exercise in patients with COPD.</p><p><strong>Design: </strong>Systematic review and network meta-analysis of randomized controlled trials.</p><p><strong>Data sources and methods: </strong>The PubMed, Web of Science, Embase, and the Cochrane Central Register of Controlled Trials were searched for relevant data. The interventions were classified according to their intensity and type as high-intensity interval training (HIIT), high-intensity continuous training (HICT), moderate-intensity continuous training (MICT), and low-intensity continuous training (LICT). We assessed exercise capacity using peak work rate (Wpeak) and the 6-min walking test (6-MWT). Lung function was evaluated by measuring peak minute ventilation (VE) and the percentage of predicted FEV<sub>1</sub> (FEV<sub>1</sub>pred%). Dyspnea was assessed using the Modified Medical Research Council (mMRC) scale. Quality of life was measured with the Chronic Respiratory Questionnaire (CRQ).</p><p><strong>Results: </strong>Fifteen studies were identified (979 subjects). HIIT showed the greatest improvement in Wpeak, 6-MWT, VE, and mMRC compared to usual care (MD 18.48 (95% CI 12.35, 24.60), 67.73 (34.89, 100.57), 6.26 (2.81, 9.72), and -0.53 (-0.89, -0.17), respectively) and showed the improvement in CRQ (MD 10.80 (95% CI 1.65, 19.95)). MICT showed improvement in Wpeak and 6-MWT (MD 18.28 (95% CI 11.20, 25.22), 61.92 (28.34, 95.51)) similar to HICT (MD 16.08 (95% CI 8.19, 23.84), 64.64 (28.70, 100.57)) and showed the highest improvement in CRQ compared to usual care (MD 10.83 (95% CI 1.68, 19.98)). LICT significantly improved Wpeak compared to usual care (MD 13.47 (95% CI 4.77, 22.13)). The quality of evidence for outcomes varied from very low to moderate.</p><p><strong>Conclusion: </strong>HIIT and MICT might be optimal training approaches for patients with COPD. LICT exhibited limited clinical efficacy. While HICT was as effective as MICT, it caused more dyspnea.</p><p><strong>Trial registration: </strong>This systematic review and network meta-analysis was prospectively registered with PROSPERO (No. CRD 42024520134).</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251323190"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11907633/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626143","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":"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":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11807278/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ophir Freund, Tali Eviatar, Roni Meidan, Tamar Shalmon, Dana Stav, Tzlil Hershko, Tal Moshe Perluk, Ori Wand, Sonia Schneer, Yochai Adir, David Shitrit, Ori Elkayam, Amir Bar-Shai, Avraham Unterman
{"title":"Dynamics of interstitial lung disease following immunosuppressive treatment differ between antisynthetase syndrome and systemic sclerosis.","authors":"Ophir Freund, Tali Eviatar, Roni Meidan, Tamar Shalmon, Dana Stav, Tzlil Hershko, Tal Moshe Perluk, Ori Wand, Sonia Schneer, Yochai Adir, David Shitrit, Ori Elkayam, Amir Bar-Shai, Avraham Unterman","doi":"10.1177/17534666251336896","DOIUrl":"10.1177/17534666251336896","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease (ILD) is the main clinical feature of antisynthetase syndrome (ASS). In the absence of randomized controlled trials to guide therapy, treatment strategies are often extrapolated from other diseases, mainly systemic sclerosis (SSc).</p><p><strong>Objectives: </strong>Our aim was to evaluate the dynamics of ILD severity following immunosuppressive treatment (IST) in ASS compared to SSc.</p><p><strong>Design: </strong>A multicenter retrospective observational study.</p><p><strong>Methods: </strong>ASS (<i>n</i> = 22) and SSc (<i>n</i> = 32) subjects with ILD were included in the registries of three medical centers. All patients received ISTs. We analyzed changes in forced vital capacity (FVC) and diffusion capacity for carbon monoxide corrected for hemoglobin (DLCOc) after treatment initiation using linear mixed-effects models. Changes in high-resolution chest CT scans were analyzed by a radiologist blinded to clinical data.</p><p><strong>Results: </strong>The median (interquartile range) age was 66 (59-71), 72% were females, and 81% of IST included mycophenolate mofetil (MMF). Baseline demographics, comorbidities, and pulmonary functions were similar between the groups. Among the ASS group, the mixed-effects models showed significant improvements in FVC% (<i>F</i> = 11.3, <i>p</i> < 0.01) and DLCOc% (<i>F</i> = 7.1, <i>p</i> = 0.015) after treatment initiation over time, while in the SSc group, there were no significant changes in FVC% (<i>F</i> = 0.4, <i>p</i> = 0.551) and DLCOc% (<i>F</i> = 0.8, <i>p</i> = 0.384). Changes in FVC% and DLCOc% were higher in the ASS group compared with SSc (<i>p</i> = 0.017 and <i>p</i> < 0.01, respectively), which persisted after adjustment to steroid use and in a sub-analysis of patients with serial pre- and post-IST pulmonary functions. Both groups had improved total CT scores after IST, without changes in other radiologic scores.</p><p><strong>Conclusion: </strong>Immunosuppressive treatment, mostly with MMF, was associated with significant improvement of FVC% and DLCOc% in ASS, compared to their stabilization only in SSc. This should encourage future randomized controlled studies of MMF in ASS patients.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251336896"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12062639/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144049504","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}
Filipa Alexandra Baptista Peixoto Befecadu, Paola Gasche, Dan Adler, Ivan Guerreiro, Sophie Pautex, Lisa Hentsch
{"title":"Healthcare professionals' representation toward optimal palliative care provision for COPD patients: a cross-sectional survey.","authors":"Filipa Alexandra Baptista Peixoto Befecadu, Paola Gasche, Dan Adler, Ivan Guerreiro, Sophie Pautex, Lisa Hentsch","doi":"10.1177/17534666251341748","DOIUrl":"10.1177/17534666251341748","url":null,"abstract":"<p><strong>Background: </strong>Chronic obstructive pulmonary disease (COPD) is a condition causing chronic physical symptoms, psychological burdens, as well as social consequences. This contributes to a major decrease in quality of life (QoL). Palliative care (PC) is a person-centered approach intended to relieve physical, psychological, social, and spiritual suffering. Despite international practice guidelines, patients with COPD have limited access to PC, mostly during end-of-life (EoL) care. It is therefore important to explore healthcare professionals' (HPs) point of view about PC to improve access for COPD patients to PC.</p><p><strong>Objectives: </strong>This study aimed to describe the perceptions of HPs working with COPD patients in Switzerland in different settings on PC provision, implementation, access, and organization. Additionally, we aimed to identify gaps, barriers, training needs, and solutions for HPs related to PC needs.</p><p><strong>Design: </strong>This is a cross-sectional survey study with quantitative and open-ended questions.</p><p><strong>Methods: </strong>We used an electronic survey sent to HPs working with patients suffering from COPD in the inpatient, outpatient, and home-based settings.</p><p><strong>Results: </strong>A total of 56 out of 98 participants (57%) answered the questionnaire of which 41.1% were nurses. 47.2% of participants were uncertain about the good timing of addressing COPD patients to PC and did it after several acute exacerbations, during EoL, or at the request of the patient. 45.5% did not know the availability of a local specialized PC. Lack of skills/training was identified as one of the hindering factors to discuss EoL (42.9% <i>N</i> = 56).</p><p><strong>Conclusion: </strong>Despite recognizing the importance of PC, several barriers were identified, including a lack of knowledge about when to initiate a PC and limited utilization of tools for identifying PC needs. Multidisciplinary teamwork and the identification of a nurse coordinator could improve earlier referrals to PC and improve QoL for COPD patients.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251341748"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12120299/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144161117","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}
Van Luan Pham, Cam Phuong Pham, Thi Thai Hoa Nguyen, Tuan Khoi Nguyen, Minh Hai Nguyen, Thi Anh Thu Hoang, Tuan Anh Le, Dinh Thy Hao Vuong, Dac Nhan Tam Nguyen, Van Khiem Dang, Thi Oanh Nguyen, Hung Kien Do, Ha Thanh Vu, Thi Thuy Hang Nguyen, Van Thai Pham, Le Huy Trinh, Hoang Gia Nguyen, Cong Minh Truong, Tran Minh Chau Pham, Thi Bich Phuong Nguyen
{"title":"Excellent survival benefit achieved in patients with advanced-stage non-small cell lung cancer harboring the epidermal growth factor receptor-G719X mutation treated by afatinib: the real-world data from a multicenter study in Vietnam.","authors":"Van Luan Pham, Cam Phuong Pham, Thi Thai Hoa Nguyen, Tuan Khoi Nguyen, Minh Hai Nguyen, Thi Anh Thu Hoang, Tuan Anh Le, Dinh Thy Hao Vuong, Dac Nhan Tam Nguyen, Van Khiem Dang, Thi Oanh Nguyen, Hung Kien Do, Ha Thanh Vu, Thi Thuy Hang Nguyen, Van Thai Pham, Le Huy Trinh, Hoang Gia Nguyen, Cong Minh Truong, Tran Minh Chau Pham, Thi Bich Phuong Nguyen","doi":"10.1177/17534666251341747","DOIUrl":"10.1177/17534666251341747","url":null,"abstract":"<p><strong>Background: </strong>Afatinib is indicated for patients with advanced-stage non-small cell lung cancer (NSCLC) with epidermal growth factor receptor (EGFR) mutations, including uncommon mutations. However, the differences in survival benefits between patients with different types of EGFR mutations remain unclear.</p><p><strong>Objectives: </strong>This study aimed to compare the effectiveness of afatinib treatment in patients harboring the EGFR-G719X mutation with that in patients carrying other uncommon EGFR mutations.</p><p><strong>Design: </strong>This was a retrospective study.</p><p><strong>Methods: </strong>Ninety-two patients with locally advanced and metastatic NSCLC, of whom 49 patients with EGFR-G719X mutations that were both single and compound, and 43 patients harbored other uncommon EGFR mutations, who were treated with afatinib as first-line treatment. The patients were followed up and evaluated every 3 months or when there were symptoms of progressive disease. The endpoints were the objective response rate (ORR), time-to-treatment failure (TTF), and overall survival (OS).</p><p><strong>Results: </strong>The average ages of patients with the EGFR-G719X and uncommon EGFR mutations were 62.7 years and 63.1 years, respectively. There were no significant differences in sex or smoking history between the two groups. In total, 28.6% of patients with the G719X mutation and 23.3% of patients with other mutations had brain metastases. The ORR of patients with the G719X mutation was 79.6%, which was 10% higher than that of patients with other EGFR mutations. Patients harboring the EGFR-G719X mutation had median TTF and median OS periods of 19.3 months and 31.4 months, respectively, which were significantly higher than those of patients carrying other mutations at 11.2 months. Subgroup analysis showed that TTF and OS benefits were observed in female patients, patients without brain metastasis, and patients with good performance status who harbored the G719X mutation.</p><p><strong>Conclusion: </strong>Patients with the EGFR-G719X mutation achieve significantly better TTF and OS benefits than those with other uncommon EGFR mutations.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251341747"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12104603/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143591","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}
Daniel Baum, Monika Sombati, Lysann Rostock, Rahel Decker, Axel Rolle, Samer Etman, Dirk Koschel, Till Ploenes
{"title":"<i>FEV<sub>1</sub></i> and DL<sub><i>CO</i></sub> predicting general complications but not prolonged air leaks in pulmonary segmentectomy.","authors":"Daniel Baum, Monika Sombati, Lysann Rostock, Rahel Decker, Axel Rolle, Samer Etman, Dirk Koschel, Till Ploenes","doi":"10.1177/17534666251341777","DOIUrl":"10.1177/17534666251341777","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary segmentectomy is increasingly recognized as a viable alternative to lobectomy for early stage non-small-cell lung cancer (NSCLC), offering comparable oncological outcomes with potentially reduced morbidity. Identifying reliable predictors for postoperative complications and prolonged air leak (PAL) is crucial for optimizing patient selection. While multifactorial scoring systems exist, their complexity limits clinical utility and the predictive value of single factors, such as forced expiratory volume in 1s (<i>FEV<sub>1</sub></i>) and diffusing capacity for carbon monoxide (DL<sub><i>CO</i></sub>), remains underexplored.</p><p><strong>Objectives: </strong>This study aimed to evaluate the ability of preoperative <i>FEV<sub>1</sub></i> and DL<sub><i>CO</i></sub> to predict complications (Clavien-Dindo ⩾ 3a) and PAL in patients undergoing pulmonary segmentectomy.</p><p><strong>Design: </strong>A retrospective, single-center study compared outcomes between patients undergoing segmentectomy (<i>n</i> = 33) and lobectomy (<i>n</i> = 126) for NSCLC.</p><p><strong>Methods: </strong>Patient characteristics, complication rates, and PAL incidence were analyzed. Logistic regression and ROC curve analyses assessed the predictive accuracy of <i>FEV<sub>1</sub></i> and DL<sub><i>CO</i></sub> for complications and PAL.</p><p><strong>Results: </strong>Baseline characteristics, including <i>FEV<sub>1</sub></i> and DL<sub><i>CO</i></sub>, were comparable between the segmentectomy and lobectomy groups (<i>p</i> > 0.05). <i>FEV<sub>1</sub></i> was identified as a significant predictor of complications, with lower values associated with increased risk. DL<sub><i>CO</i></sub> exhibited an even stronger predictive value for complications in the segmentectomy cohort, with an AUC of 0.924, indicating excellent predictive accuracy. In contrast, neither <i>FEV<sub>1</sub></i> nor DL<sub><i>CO</i></sub> demonstrated significant predictive value for PAL, which occurred in 30% of segmentectomy and 20% of lobectomy patients (<i>p</i> > 0.05).</p><p><strong>Conclusion: </strong>Preoperative <i>FEV<sub>1</sub></i> and DL<sub><i>CO</i></sub> are valuable predictors of complications (Clavien-Dindo ⩾ 3a) in pulmonary segmentectomy, with DL<sub><i>CO</i></sub> showing high predictive accuracy. However, their inability to reliably predict PAL highlights the need for multifactorial models to enhance risk assessment. Despite the limited sample size, our findings align with larger studies and reinforce the clinical utility of <i>FEV<sub>1</sub></i> and DL<sub><i>CO</i></sub> for preoperative risk stratification in segmentectomy patients.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251341777"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12246529/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144584942","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":"Palliative care for chronic respiratory diseases in low- and middle-income countries: a narrative review.","authors":"Zeina Al Achkar, Toufic Chaaban","doi":"10.1177/17534666251318616","DOIUrl":"10.1177/17534666251318616","url":null,"abstract":"<p><p>Palliative care is essential for patients with chronic pulmonary diseases, especially in low- and middle-income countries (LMICs). Chronic respiratory diseases (CRDs), such as chronic obstructive pulmonary disease and interstitial lung diseases, cause significant morbidity and mortality globally, with a heavy burden in LMICs. Despite the need, access to palliative care in LMICs is limited, leading to inadequate symptom management and support. Palliative care benefits include improved quality of life, reduced healthcare costs, and increased patient and family satisfaction. However, barriers in LMICs, including limited resources, infrastructure, and trained providers, as well as cultural and regulatory challenges, hinder care delivery. Early integration of palliative care for patients with CRDs can enhance outcomes and reduce healthcare utilization, yet it remains underutilized in these regions. This review highlights the challenges and impact of palliative care for CRDs in these regions. Addressing these issues requires regulatory reforms, provider education, and investments in healthcare infrastructure. Solutions include national policies, training healthcare professionals, telemedicine, and research collaborations. Understanding and addressing barriers to palliative care in LMICs is crucial for improving care quality and outcomes for patients with CRDs.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251318616"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11831687/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143433824","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}