Airi Hasegawa, Kazuya Tone, Yuri Baba, Zenya Saito, Takuya Akutsu, Takaaki Kitayama, Shunsuke Inaki, Mina Gochi, Takashi Yaguchi, Koichi Makimura, Masamichi Takagi, Jun Araya
{"title":"Nodular-bronchiectatic pattern in pulmonary nocardiosis: Immune status and treatment outcomes in a multicenter retrospective study.","authors":"Airi Hasegawa, Kazuya Tone, Yuri Baba, Zenya Saito, Takuya Akutsu, Takaaki Kitayama, Shunsuke Inaki, Mina Gochi, Takashi Yaguchi, Koichi Makimura, Masamichi Takagi, Jun Araya","doi":"10.1016/j.rmed.2024.107922","DOIUrl":"10.1016/j.rmed.2024.107922","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary nocardiosis is a rare opportunistic infection, with approximately 15 % of patients being immunocompetent. The isolation rate of Nocardia spp. has recently increased, indicating rising clinical concern. This study aimed to summarize computed tomography (CT) findings, evaluate treatment outcomes, and improve disease recognition.</p><p><strong>Methods: </strong>We retrospectively analyzed 12 pulmonary nocardiosis cases recorded over 10 years in three hospitals, excluding two unclear cases. All pathogens were detected on smears and isolated from respiratory specimens.</p><p><strong>Results: </strong>The mean age was 73.0 ± 12.9 years, with 9 men and 6 smokers. Among the included patients, 8 had underlying pulmonary diseases, 4 had non-pulmonary diseases, and 3 were on steroids. The most common species were N. cyriacigeorgica and N. nova. CT findings included cavitary, consolidation/infiltrative, and nodular-bronchiectatic (NB) patterns. The NB pattern, particularly common in immunocompetent patients, was associated with significantly better treatment outcomes than non-NB patterns (Fisher's exact test, p = 0.0476). All isolates were susceptible to trimethoprim/sulfamethoxazole. Eight patients recovered with appropriate antimicrobial therapy, while two patients died.</p><p><strong>Conclusions: </strong>Pulmonary nocardiosis presented three CT patterns, with the NB pattern being the most frequent and showing favorable treatment outcomes, especially in immunocompetent patients. While Nocardia spp. can develop in patients with preexisting bronchiectasis, it may also independently cause bronchiectasis. Nocardia infection should be considered as a differential diagnosis in patients exhibiting the NB pattern, resembling that of nontuberculous mycobacterial pulmonary diseases.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"107922"},"PeriodicalIF":3.5,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142897245","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}
Arsal Tharwani, Omar Minai, Youlan Rao, Hyoshin Kim, Gustavo A Heresi
{"title":"The minimally important difference in the six-minute walk test predicts clinical worsening in pulmonary arterial hypertension.","authors":"Arsal Tharwani, Omar Minai, Youlan Rao, Hyoshin Kim, Gustavo A Heresi","doi":"10.1016/j.rmed.2024.107919","DOIUrl":"10.1016/j.rmed.2024.107919","url":null,"abstract":"<p><strong>Background: </strong>The minimally important difference (MID) in the 6-min walk test (6MWT) for pulmonary arterial hypertension (PAH) is estimated to be 33 m using distributional and anchor-based methods. Quality of life was used as the anchor. Here, we sought to determine whether the MID is predictive of clinical worsening.</p><p><strong>Methods: </strong>This was a post hoc analysis of the pivotal clinical trial of tadalafil in PAH (n = 405) and its extension phase (n = 161). The 6MWT was determined at the end of the placebo-controlled phase of 16 weeks and dichotomized as < 33 or ≥33 m. Primary outcome was clinical worsening ascertained at 16 weeks and at 68 weeks of follow up. Cox proportional hazard analysis was used to determine the association between 6MWT and clinical worsening.</p><p><strong>Results: </strong>Mean age for patients in the pivotal trial of tadalafil was 54 years old ( ± 15.5 yrs). There were 317 (78.3 %) female patients and 61 % with idiopathic PAH. 53 % of the patients were on background bosentan therapy. A 6MWT <33 m was associated with an increased risk of clinical worsening at 16 and 68 weeks. These results were unchanged after adjusting for age, sex, background therapy with bosentan, and tadalafil dose. There were no PAH hospitalizations during short-term and long-term follow up in patients achieving a 6MWT ≥33 m CONCLUSIONS: The 6MWT MID of 33 m is predictive of short- and long-term clinical worsening. These results further validate 33 m as a relevant MID for the 6MWT.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"107919"},"PeriodicalIF":3.5,"publicationDate":"2024-12-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142882769","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}
Kabadi Alisha A, Yang Jenny Z, Fernandes Timothy M
{"title":"Stroke Volume Augmentation Improves with PH-Targeted Therapy in Patients with Exercise-Induced Pulmonary Hypertension.","authors":"Kabadi Alisha A, Yang Jenny Z, Fernandes Timothy M","doi":"10.1016/j.rmed.2024.107921","DOIUrl":"https://doi.org/10.1016/j.rmed.2024.107921","url":null,"abstract":"<p><p>Exercise-induced pulmonary arterial hypertension (EiPH) is often treated with off-label use of pulmonary arterial hypertension-targeted therapy (PH-targeted therapy). Most measures of PAH severity are normal in patients with EiPH, posing challenges in evaluating for physiological improvement in patients treated for EiPH. In this study, we used non-invasive cardiopulmonary exercise testing (CPET) to assess for objective improvement in patients treated for EiPH. Fourteen patients were included in the study, diagnosed with EiPH by invasive CPET with simultaneous right heart catheterization and subsequently treated with PH-targeted therapy. Follow-up non-invasive CPET was performed to evaluate oxygen consumption (VO<sub>2</sub>, L/min) and oxygen pulse (O<sub>2</sub> pulse, ml/beat), which was defined by the ratio of VO<sub>2</sub> to heart rate (beats/min). ). Adequate stroke volume augmentation was a ratio of O<sub>2</sub> pulse at anaerobic threshold (O<sub>2</sub> pulse<sub>AT</sub>) to O<sub>2</sub> pulse at rest (O<sub>2</sub> pulse<sub>R</sub>) of >2.6. The results showed a significant improvement in O<sub>2</sub> pulse and VO<sub>2</sub> at anaerobic threshold after treatment with PH-targeted therapy. In addition, O<sub>2</sub> Pulse<sub>AT</sub>:O2 Pulse<sub>R</sub> significantly improved. Patients treated for EiPH demonstrated improvement in both stroke volume augmentation and VO<sub>2</sub> measured on non-invasive CPET, supporting the use of this test to assess for objective improvement in exercise capacity for patients treated for EiPH. Structured Abstract RATIONALE: Exercise-induced pulmonary arterial hypertension (EiPH) is a clinical entity characterized by exertional dyspnea. Cardiopulmonary exercise testing (CPET) can evaluate exertional limitations in EiPH patients and assess response to therapy. We describe a series of patients treated for EiPH and monitored with non-invasive CPET.</p><p><strong>Methods: </strong>case series of 14 patients with EiPH treated with PH-targeted therapy. EiPH was defined by a slope of the mean pulmonary artery pressure (mPAP) vs cardiac output of >3 mmHg/L/min with a resting mPAP of <20mmHg and pulmonary vascular resistance <3 Woods units. Oxygen pulse (O<sub>2</sub> pulse, ml/beat), a surrogate of stroke volume (SV), was measured by the ratio of oxygen consumption (VO<sub>2</sub>, L/min) to heart rate (beats/min). Adequate SV augmentation was a ratio of O<sub>2</sub> pulse at anaerobic threshold (O<sub>2</sub> pulse<sub>AT</sub>) to O<sub>2</sub> pulse at rest (O<sub>2</sub> pulse<sub>R</sub>) of >2.6.</p><p><strong>Results: </strong>Patients were majority female (86%) with a mean age of 59.1.±12.2 years, and 57% had connective tissue diseases. Patients took PH-targeted therapy (endothelin receptor antagonist (n=7), phosphodiesterase-5 inhibitor (n=5) or both (n=2)) for a median exposure of 150 days. There was no change in O<sub>2</sub> pulse<sub>R</sub> after treatment. However, there was a sign","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"107921"},"PeriodicalIF":3.5,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142882767","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":"State-of-the-art narrative review: Mounier-Kuhn syndrome and tracheobronchomegaly.","authors":"Shivang Sharma, Stephen J Kuperberg","doi":"10.1016/j.rmed.2024.107914","DOIUrl":"10.1016/j.rmed.2024.107914","url":null,"abstract":"<p><p>Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly is an uncommon disease of the central airways. It is characterized by pathological dilatation of the trachea and main bronchi and inevitably leads to recurrent respiratory infections, bronchiectasis, hospitalizations, and results in considerable morbidity and mortality. Despite numerous case reports, there is a shortage of evidence on clinical outcomes and limited data on interventions, thus presenting a significant gap in the literature. Fortunately, new strategies and increasing clinical experience have improved the clinical approach, diagnostic workup, classification, and management of MKS. Articles in English, Spanish, and French were searched from databases, including Pubmed, Google Scholar, Medline, and SCOPUS, using the terms \"Mounier-Kuhn syndrome,\" \"Tracheomegaly,\" \"Tracheobronchomegaly,\" and \"Bronchomegaly,\" without date restrictions. A total of 360 articles with the aforementioned syntax were indexed on Pubmed. This state-of-the-art review attempts to fill a void in the current literature by summarizing the current scientific knowledge and highlighting novel interventional strategies in the management of Mounier Kuhn Syndrome.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"107914"},"PeriodicalIF":3.5,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876487","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}
Navneet Chadha , Felicity C. Blackstock , Sheree Smith , Pat G. Camp , Clarice Tang
{"title":"Characteristics of rehabilitation programs for chronic respiratory diseases in Asia: A scoping review","authors":"Navneet Chadha , Felicity C. Blackstock , Sheree Smith , Pat G. Camp , Clarice Tang","doi":"10.1016/j.rmed.2024.107885","DOIUrl":"10.1016/j.rmed.2024.107885","url":null,"abstract":"<div><div>The rates of chronic respiratory disease (CRD) is rising in Asia. Pulmonary rehabilitation (PR) has been shown to be a highly efficacious intervention for people with CRD. While PR models are well established in Western countries, environmental, cultural and societal factors may influence how rehabilitation programs for people with CRD are conducted in Asia. This review aims to identify the characteristics of rehabilitation programs for people with CRD within Asia and identify differences between these rehabilitation programs to the recently updated American Thoracic Society (ATS) PR guidelines. Utilising the PRISMA scoping review guidelines, five databases– CINAHL, Medline, Embase, Web of Science and Health and Medical Collection were searched from inception until 13th December 2023. A total of 137 studies (n = 19,128) were included in the review. As many as 113 studies (83 %) included aerobic exercises as part of rehabilitation, only 90 studies (66 %) included resistance training. Thirty-nine studies included interventions such as Tai Chi, Qigong and Yoga. Comparing to the 2023 ATS PR guidelines, only 22 % of the included studies evaluated a rehabilitation program that was consistent with the guidelines. Improvement in exercise capacity (76 %) and quality of life (QOL) (73 %) were the most frequent outcomes used to evaluate program efficacy. The results suggest that models of rehabilitation varied greatly within the Asia region, with some more heavily adapted to suit the local context as compared to others. Further consideration on how to balance adaptation of PR with fidelity of the intervention needs to be taken.</div></div>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":"236 ","pages":"Article 107885"},"PeriodicalIF":3.5,"publicationDate":"2024-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142721529","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}
Karsten Keller , Ingo Sagoschen , Volker H. Schmitt , Visvakanth Sivanathan , Ioannis T. Farmakis , Omar Hahad , Sebastian Koelmel , Frank P. Schmidt , Christine Espinola-Klein , Stavros Konstantinides , Thomas Münzel , Philipp Lurz , Lukas Hobohm
{"title":"Risk factors for intensive care admission in childhood patients with COVID-19 - Results of the German nationwide inpatient sample","authors":"Karsten Keller , Ingo Sagoschen , Volker H. Schmitt , Visvakanth Sivanathan , Ioannis T. Farmakis , Omar Hahad , Sebastian Koelmel , Frank P. Schmidt , Christine Espinola-Klein , Stavros Konstantinides , Thomas Münzel , Philipp Lurz , Lukas Hobohm","doi":"10.1016/j.rmed.2024.107880","DOIUrl":"10.1016/j.rmed.2024.107880","url":null,"abstract":"<div><h3>Background</h3><div>COVID-19 pandemic research efforts have mainly focused on adults, but data in paediatric populations are sparse.</div></div><div><h3>Methods</h3><div>We used the German nationwide-inpatient-sample analysing all hospitalized children ≤18 years with confirmed COVID-19-diagnosis in Germany during the year 2020, stratified for intensive care unit (ICU) admission.</div></div><div><h3>Results</h3><div>Overall, 3360 hospitalization-cases of children ≤18 years with COVID-19-infection were diagnosed in Germany 2020 (median age 7.0 [IQR 0.0–15.0] years, 49.8 % female); among them 4.3 % were admitted on an ICU. In-hospital death occurred in five patients with and three without ICU admission (3.5 % vs. 0.1 %, P < 0.001) and ICU admission was independently associated with increased case-fatality (OR 21.573 [95%CI 4.191–111.044], P < 0.001).</div><div>Obesity (OR 3.419 [95%CI 1.300–8.993], P = 0.013), diabetes mellitus (OR 6.929 [95%CI 3.327–14.432], P < 0.001), pneumonia (OR 7.373 [95%CI 4.823–11.271], P < 0.001), acute respiratory distress syndrome (ARDS) (OR 48.058 [95%CI 11.689–197.588], P < 0.001) and multisystem inflammatory syndrome caused by COVID-19 (OR 9.573 [95%CI 3.036–30.191], P < 0.001), heart failure (OR 64.509 [95%CI 24.462–170.121], P < 0.001), myocarditis (OR 4.682 [95%CI 1.278–17.149], P = 0.020), acute and/or chronic kidney failure (OR 7.946 [95%CI 3.606–17.508], P < 0.001), cancer (OR 5.220 [95%CI 2.599–10.485], P < 0.001) and liver diseases (OR 5.501 [95%CI 2.177–13.899], P < 0.001) were associated with an ICU admission.</div></div><div><h3>Conclusion</h3><div>Proportion of hospitalized paediatric COVID-19-patients admitted on ICU in Germany was low with 4.3 % accompanied by 3.5 % case-fatality rate. Independent factors for ICU admission comprised cardio-vascular risk factors, comorbidities, and complications of COVID-19.</div></div>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":"236 ","pages":"Article 107880"},"PeriodicalIF":3.5,"publicationDate":"2024-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142716934","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 associated with uncontrolled severe asthma in the biologic era","authors":"Hideki Yasui , Kyohei Oishi , Fumiya Nihashi , Kazuki Furuhashi , Tomoyuki Fujisawa , Yusuke Inoue , Masato Karayama , Hironao Hozumi , Yuzo Suzuki , Noriyuki Enomoto , Suguru Kojima , Mitsuru Niwa , Masanori Harada , Masato Kato , Dai Hashimoto , Koshi Yokomura , Naoki Koshimizu , Mikio Toyoshima , Masahiro Shirai , Toshihiro Shirai , Takafumi Suda","doi":"10.1016/j.rmed.2024.107881","DOIUrl":"10.1016/j.rmed.2024.107881","url":null,"abstract":"<div><h3>Background</h3><div>Despite the development of biologics for severe asthma, individuals with uncontrolled status persist, posing a significant social problem. This multicenter prospective study aimed to identify factors associated with the uncontrolled status of patients with severe asthma in the biologic era assessed using the Asthma Control Questionnaire (ACQ).</div></div><div><h3>Methods</h3><div>Subjects with severe asthma diagnosed by respiratory specialists were enrolled from 11 hospitals. Clinical data and questionnaires were collected. We compared controlled (ACQ-5 <1.5) with uncontrolled severe asthma (ACQ-5 ≥1.5) and assessed factors linked to uncontrolled severe asthma using logistic regression analysis.</div></div><div><h3>Results</h3><div>One hundred fifty-four patients were analyzed (median age, 66 years; 62.3 % female; 52.6 % administered biologics). Among them, 56 patients (36.4 %) had uncontrolled severe asthma (ACQ-5 ≥1.5). The uncontrolled group had more frequent exacerbations (≥2 times in the previous year) and elevated blood neutrophil counts compared with the controlled group. Factors associated with uncontrolled status were analyzed in the overall population, with patients stratified into two groups: those receiving biologics and those not receiving biologics. Multivariate analysis revealed that frequent exacerbations and elevated blood neutrophil counts were associated with uncontrolled status in the overall population and in patients without biologics, whereas elevated blood neutrophil counts were significantly associated with uncontrolled status in patients receiving biologics.</div></div><div><h3>Conclusion</h3><div>Elevated blood neutrophil counts and frequent exacerbations were independently associated with uncontrolled severe asthma. Specifically, elevated blood neutrophil counts were a significant factor related to uncontrolled status irrespective of biologics, suggesting their potential utility as a biomarker in the biologic era.</div></div>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":"236 ","pages":"Article 107881"},"PeriodicalIF":3.5,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142695625","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}
Connett Gj , Maguire S , Larcombe Tc , Scanlan N , Shinde Ss , Muthukumarana T , Bevan A , Keogh Rh , Legg Jp
{"title":"Real-world impact of Elexacaftor-Tezacaftor-Ivacaftor treatment in young people with Cystic Fibrosis: A longitudinal study","authors":"Connett Gj , Maguire S , Larcombe Tc , Scanlan N , Shinde Ss , Muthukumarana T , Bevan A , Keogh Rh , Legg Jp","doi":"10.1016/j.rmed.2024.107882","DOIUrl":"10.1016/j.rmed.2024.107882","url":null,"abstract":"<div><h3>Background</h3><div>Elexacaftor, Tezacaftor, Ivacaftor (ETI) became available in the UK in August 2020 to treat people with Cystic Fibrosis (CF) aged >12 years. We report a real-world study of clinical outcomes in young people treated with ETI at our CF centre within the first two years of its availability.</div></div><div><h3>Methods</h3><div>Participants aged 12–17 were identified within our clinic, with demographic data supplemented by the UK CF registry. Comprehensive outcome data spanning two years pre- and two years post-initiation of CFTR modulators were compiled from various local sources, including patient records, medication delivery logs, and clinical notes.</div></div><div><h3>Results</h3><div>Of the 62 patients started on ETI (32 male, mean age 13.3 years), most (76 %) were homozygous for the F508del mutation. Three discontinuations occurred: one pregnancy, two related to side effects. Adherence was high (Proportion of Days covered >90 % both years). Following ETI initiation there was a significant increase in mean FEV1% (+11.7 units; 95 % CI 7.4–15.6), sustained throughout the two-year treatment period. There was no association between baseline lung function and the degree of improvement or rate of decline post-treatment. Improvements were similar for all treatable genotypes. BMI z-score increased by 0.25 units after four months of treatment, returning to baseline by 24 months. Intravenous antibiotic use decreased by 88 % (median IV days/year reduced from 32 to 4 days, p < 0.01).</div></div><div><h3>Conclusions</h3><div>ETI use in adolescents in a real-world setting led to sustained improvements in health outcomes, consistent with those seen in open trial extension studies.</div></div>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":"236 ","pages":"Article 107882"},"PeriodicalIF":3.5,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142710920","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}
Ada E.M. Bloem , Hanneke M. Dolk , Anne E. Wind , Joanne J. van der Vis , Maarten J. Kampen , Jan W.H. Custers , Martijn A. Spruit , Marcel Veltkamp
{"title":"Prognostic value of the 6-min walk test derived attributes in patients with idiopathic pulmonary fibrosis","authors":"Ada E.M. Bloem , Hanneke M. Dolk , Anne E. Wind , Joanne J. van der Vis , Maarten J. Kampen , Jan W.H. Custers , Martijn A. Spruit , Marcel Veltkamp","doi":"10.1016/j.rmed.2024.107862","DOIUrl":"10.1016/j.rmed.2024.107862","url":null,"abstract":"<div><h3>Introduction</h3><div>Idiopathic pulmonary fibrosis (IPF) is a fatal progressive fibrosing lung disease. A decreased 6-min walk distance (6MWD) and exercise-induced oxygen desaturation measured during the 6-min walk test (6MWT), are known predictors of mortality in patients with IPF. However, the use of antifibrotic drugs showed a survival benefit in IPF. Therefore, this study aimed to evaluate to what extend 6MWT-derived attributes are associated with two-year survival when antifibrotic drugs were introduced as part of standard IPF-care.</div></div><div><h3>Methods</h3><div>This real-world data-study included patients with IPF with a 6MWT between 2015 and 2020, and used composite outcome: mortality or lung transplantation within 2 years of follow-up. Data were collected systematically, including demographics, pulmonary function tests, comorbidities, medications and 6MWT-derived attributes. The prediction attributes of 6MWT were studied with a Cox Proportional-Hazards model and Kaplan-Meier survival curves. The best discriminating attribute to predict mortality was added to the prediction model Gender-Age-Physiology (GAP).</div></div><div><h3>Results</h3><div>In 216 patients, 2-year transplant-free survival cut-off points were identified for the 6MWD (≥413 m), 6MWD %predicted (≥83 %), SpO<sub>2</sub>-nadir (≥86 %) and distance-saturation-product (≥374 m%), with the best discriminative value for SpO<sub>2</sub>-nadir (area under the curve: 0.761). 2-Year survival percentage of patients with SpO<sub>2</sub>-nadir below or above threshold (86 %) was 37.1 % and 80.0 %, respectively. Exercise-induced oxygen desaturation added to the GAP model showed an improvement in its predictive power.</div></div><div><h3>Conclusion</h3><div>Patients with IPF who have an exercise-induced oxygen desaturation have worse prognosis. Addition of SpO<sub>2</sub>-nadir to the GAP model seems promising for use in clinical care of IPF patients.</div></div>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":"236 ","pages":"Article 107862"},"PeriodicalIF":3.5,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142688676","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":"Three-year outcome of a very young severe uncontrolled preschool wheezers cohort, a real-life study","authors":"Hortense Petat, Christophe Marguet","doi":"10.1016/j.rmed.2024.107875","DOIUrl":"10.1016/j.rmed.2024.107875","url":null,"abstract":"<div><div>Preschool wheeze is a public health issue. Disease control can be difficult to obtain in this population, in which no biologic therapy is indicated. We studied the evolution of severe preschool wheezers in real-life and identified the factors leading to no-control.</div><div>We conducted a retrospective study at our tertiary asthma center. Each child under 3 years of age with severe, uncontrolled preschool wheeze was admitted to a pediatric day hospital for further investigations. We collected the results of clinical, biological and radiological exams, and follow-up data at 1 (Y<sub>+1</sub>), 2 (Y<sub>+2</sub>) and 3 years (Y<sub>+3</sub>).</div><div>We included 135 patients; 63 (47 %) were still being followed at Y<sub>+3</sub>; 53 % were discontinued due to disease control. The median age at inclusion was 12 months. 29 % of patients followed up still had severe uncontrolled wheezing at Y<sub>+3</sub>. Eosinophils greater than 0.23G/L (p = 0.03) and a first case of bronchiolitis before the age of 2 months (p = 0.01) were factors in uncontrolled wheezing at Y<sub>1</sub>. Tobacco exposure was a factor associated with uncontrolled wheezing at Y<sub>+2</sub> (p < 0.001). A first case of bronchiolitis before the age of 2 months (p = 0.007), male sex (p < 0.001) and a familial history of atopy (p = 0.05) were factors in uncontrolled disease at Y<sub>+3</sub>.</div><div>We report a real-life study, with a very young population and very severe wheezing. Our therapeutic approach is original, enabling us to study the evolution of “therapeutic pressure” in the early years of this frequent disease, the pathophysiology of which is still poorly understood.</div></div>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":"235 ","pages":"Article 107875"},"PeriodicalIF":3.5,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142693128","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}