Respiratory Physiology & Neurobiology最新文献

{"title":"Respiratory physiological exploration during self-induced cognitive trance","authors":"","doi":"10.1016/j.resp.2024.104301","DOIUrl":"10.1016/j.resp.2024.104301","url":null,"abstract":"<div><h3>Background and methods</h3><p>Little is known about self-induced cognitive trance (SICT) on respiratory function. The aims of this prospective, single-center, non-randomized, open-label study of healthy volunteers, were to characterize spirometry changes during SICT, confirm the safety of this technique, and investigate the potential clinical benefits of SICT.</p></div><div><h3>Results</h3><p>Nine people participated. There were no significant difference in FEV1 FVC or FEF 25–75 before, during, and after SICT. There were significant improvements in grip strength during SICT (+2.2 kg/5.7 %, p&lt;0.05) and in self-efficacy score related to physical activity at the end of the trance. One participant had a significant worsening of FEV1 during SICT in the context of a recent upper airway infection.</p></div><div><h3>Conclusion</h3><p>SICT does not significantly modify spirometry data in healthy volunteers and can improve self-efficacy related to physical activity. SICT should probably be performed with caution during upper airway infections.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104301"},"PeriodicalIF":1.9,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141601454","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Combination of acute intermittent hypoxia and intermittent transcutaneous electrical stimulation in obstructive sleep apnea: a randomized controlled crossover trial","authors":"Shiqian Zha ,&nbsp;Xu Liu ,&nbsp;Hao Chen,&nbsp;Yueying Hao,&nbsp;Jingyi Zhang,&nbsp;Qingfeng Zhang,&nbsp;Ke Hu","doi":"10.1016/j.resp.2024.104298","DOIUrl":"10.1016/j.resp.2024.104298","url":null,"abstract":"<div><p>Intermittent hypoxia (IH) and intermittent transcutaneous electrical stimulation (ITES) might benefit patients with obstructive sleep apnea (OSA). However, the therapeutic value of combined IH and ITES in OSA is unknown. In this prospective, randomized, controlled crossover study, normoxia (air exposure for 50 min before sleep and sham stimulation for 6 h during sleep), IH (5 repeats of 5 min 10–12 % O₂ alternating with 5 min air for 50 min, and sham stimulation for 6 h), ITES (air exposure for 50 min and 6 repeats of 30 min transcutaneous electrical stimulation alternating with 30 min of sham stimulation for 6 h), and IH&amp;ITES (10–12 % O₂ alternating with air for 50 min and transcutaneous electrical stimulation alternating with sham stimulation for 6 h) were administered to patients with OSA over four single-night sessions. The primary endpoint was difference in OSA severity between the interventions according to apnea-hypopnea index (AHI) and oxygen desaturation index (ODI). The efficacy was response to IH, ITES, IH&amp;ITES defined as a ≥50 % reduction in AHI compared with normoxia. Twenty participants (17 male, 3 female) completed the trial. The median (IQR) AHI decreased from 14.5 (10.8, 17.5) events/h with normoxia to 6.9 (3.9, 14.8) events/h with IH (p=0.020), 5.7 (3.4, 9.1) events/h with ITES (p=0.001), and 3.5 (1.8, 6.4) events/h with IH&amp;ITES (p=0.001). AHI was significantly different between IH and IH&amp;ITES (p=0.042) but not between ITES and IH&amp;ITES (p=0.850). For mild-moderate OSA (n=17), IH, ITES, and IH&amp;ITES had a significant effect on AHI (p=0.013, p=0.001, p=0.001, respectively) compared with normoxia, but there were no differences in post hoc pairwise comparisons between intervention groups. No serious adverse events were observed. In conclusion, IH, ITES, and IH&amp;ITES significantly reduced OSA severity. IH&amp;ITES showed better efficacy in mild-moderate OSA than IH and was comparable to ITES. Our data do not support recommending IH&amp;ITES over ITES for OSA.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104298"},"PeriodicalIF":1.9,"publicationDate":"2024-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141402904","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prediction of cough effectiveness in amyotrophic lateral sclerosis patients assessed by ultrasuond of the diaphragm during the cough expiration phase","authors":"Fausta Viccaro ,&nbsp;Altea Lecci ,&nbsp;Valentina Baccolini ,&nbsp;Antonio Sciurti ,&nbsp;Daniel Piamonti ,&nbsp;Maurizio Inghilleri ,&nbsp;Letizia D’Antoni ,&nbsp;Paolo Palange","doi":"10.1016/j.resp.2024.104299","DOIUrl":"10.1016/j.resp.2024.104299","url":null,"abstract":"<div><p>Assessing cough effectiveness, using Cough Peak Flow, is crucial for patients with Neuromuscular Diseases, such as Amyotrophic Lateral Sclerosis. Impaired cough function can contribute to respiratory decline and failure. The goal of the study is to determine the correlation between diaphragmatic excursion and cough expiratory phase, potentially utilizing ultrasonographic indices to estimate Cough Peak Flow in these patients. Twenty-two patients were enrolled in this study. The upward displacement of the diaphragm was measured with ultrasonography during voluntary cough expiration and Cough Peak Flow was simultaneously measured. A multivariable linear regression model was built to quantify the association between Cough Peak Flow and diaphragm expiratory excursion. There is significative relationship between Cough Peak Flow and diaphragm excursion with a Pearson’s r coefficient of 0.86 observed in the patients group. Multiple linear regression analysis for Cough Peak Flow (Adjusted R² = 0.86) revealed significant associations between Cough Peak Flow and expiratory excursion (adjusted <em>β</em>-coefficient: 64.78, 95 %, CI: 51.50–78.07, p&lt;0.001) and sex (adjusted <em>β</em>-coefficient: −69.06; 95 % CI: −109.98 to −28.15, p=0.001). Our results predict the cough effectiveness by using M-mode diaphragmatic sonography with a potentially significant impact on therapeutic choices.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104299"},"PeriodicalIF":1.9,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1569904824000922/pdfft?md5=ca15cc3930a2dd725812018d3a01ad8e&pid=1-s2.0-S1569904824000922-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141327732","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The influence of different spontaneous breathing trials on regional ventilation distribution in patients with prolonged mechanical ventilation","authors":"Pu Wang ,&nbsp;Mei-Yun Chang ,&nbsp;Hai-Yen Hsia ,&nbsp;Meng Dai ,&nbsp;Yifan Liu ,&nbsp;Yeong-Long Hsu ,&nbsp;Feng Fu ,&nbsp;Zhanqi Zhao","doi":"10.1016/j.resp.2024.104296","DOIUrl":"10.1016/j.resp.2024.104296","url":null,"abstract":"<div><h3>Objective</h3><p>This study aimed to explore the influence of different spontaneous breathing trials (SBTs) on regional ventilation distribution in patients with prolonged mechanical ventilation (PMV).</p></div><div><h3>Methods</h3><p>A total of 24 patients with PMV were analyzed retrospectively. They received three different SBT modes which are automatic tube compensation (ATC), continuous positive airway pressure (CPAP), and T-piece (TP), over three days, and every SBT lasted two hours. Electrical impedance tomography (EIT) was used to monitor the SBT process and five-minute EIT data from five periods (pre-SBT which is t0, at the beginning and the end of the first hour SBT are t1 and t2, at the beginning and the end of the second hour SBT are t3 and t4) were analyzed.</p></div><div><h3>Results</h3><p>In all PMV patients, the temporal skew of aeration (TSA) values at t3 were significantly different in three SBTs (ATC: 18.18±22.97; CPAP: 20.42±17.01; TP:11.26±11.79; p=0.05). In the weaning success group, TSA (t1) values were significantly different too (ATC: 11.11±13.88; CPAP: 19.09±15.77; TP: 9.09±12.74; p=0.04). In the weaning failure group, TSA (t4) values were significantly different in three SBTs (ATC: 36.67±18.46; CPAP: 15.38±11.69; TP: 17.65±17.93; p=0.04). The patient’s inspiratory effort (Global flow index at t1) in patients with weaning failure under CPAP (3.51±4.31) was significantly higher than that in the ATC (1.15±1.47) and TP (0.89±1.28). The SBT mode with the best ventilation uniformity may be the one that activates the respiratory muscles the most which may be the optimal SBT. The SBT mode of most uniform ventilation distribution settings varies from patient to patient.</p></div><div><h3>Conclusion</h3><p>The regional ventilation distribution was different for each individual, making the SBT with the best ventilation distribution of patients need to be personalized. EIT is a tool that can be considered for real-time assessment.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104296"},"PeriodicalIF":1.9,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1569904824000892/pdfft?md5=0902ba6f2c42c3392f8cd3abbb81d5c2&pid=1-s2.0-S1569904824000892-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141327733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sex- and age-adjusted reference values for dynamic inspiratory constraints during incremental cycle ergometry","authors":"Abed A. Hijleh ,&nbsp;Danilo C. Berton ,&nbsp;Igor Neder-Serafini ,&nbsp;Matthew James ,&nbsp;Sandra Vincent ,&nbsp;Nicolle Domnik ,&nbsp;Devin Phillips ,&nbsp;Denis E. O'Donnell ,&nbsp;J. Alberto Neder","doi":"10.1016/j.resp.2024.104297","DOIUrl":"10.1016/j.resp.2024.104297","url":null,"abstract":"<div><p>Activity-related dyspnea in chronic lung disease is centrally related to dynamic (_dyn) inspiratory constraints to tidal volume expansion. Lack of reference values for exertional inspiratory reserve (IR) has limited the yield of cardiopulmonary exercise testing in exposing the underpinnings of this disabling symptom. One hundred fifty apparently healthy subjects (82 males) aged 40–85 underwent incremental cycle ergometry. Based on exercise inspiratory capacity (IC_dyn), we generated centile-based reference values for the following metrics of IR as a function of absolute ventilation: IR_dyn1 ([1-(tidal volume/IC_dyn)] x 100) and IR_dyn2 ([1-(end-inspiratory lung volume/total lung capacity] x 100). IR_dyn1 and IR_dyn2 standards were typically lower in females and older subjects (<em>p</em>&lt;0.05 for sex and age versus ventilation interactions). Low IR_dyn1 and IR_dyn2 significantly predicted the burden of exertional dyspnea in both sexes (<em>p</em>&lt;0.01). Using these sex and age-adjusted limits of reference, the clinician can adequately judge the presence and severity of abnormally low inspiratory reserves in dyspneic subjects undergoing cardiopulmonary exercise testing.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104297"},"PeriodicalIF":1.9,"publicationDate":"2024-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141318113","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dexamethasone weakens the respiratory effects of pro-inflammatory cytokine TNF-α in rat","authors":"Nina Pavlovna Aleksandrova,&nbsp;Galina Anatolevna Danilova","doi":"10.1016/j.resp.2024.104284","DOIUrl":"10.1016/j.resp.2024.104284","url":null,"abstract":"<div><p>The goal of the current study was to identify the role of the glucocorticoids in the respiratory effects of proinflammatory cytokines. For this purpose intravenous injections of TNF-α were used in anesthetized spontaneously breathing rats before and after pretreatment of dexamethasone, a synthetic steroid with predominant glucocorticoid activity. Dexamethasone was injected intraperitoneally at a dose of 1 mg/kg. TNF-α was administrated into the tail vein at a dose of 40 mg/kg. We found that dexamethasone pretreatment eliminated the cytokine-induced increase in pulmonary ventilation and decrease in the hypoxic ventilatory response. Dexamethasone had a pronounced rapid effect on the respiratory activity of TNF-α as early as 30 minutes after administration. Therefore, we assume that this mechanism of action of dexamethasone was non-genomic, associated with the blocking of secondary mediators of the cytokine response.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104284"},"PeriodicalIF":2.3,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141186812","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ventilatory efficiency in long-term dyspnoeic patients following COVID-19 pneumonia","authors":"Daniel Piamonti ,&nbsp;Luigi Panza ,&nbsp;Roberto Flore ,&nbsp;Valentina Baccolini ,&nbsp;Daniela Pellegrino ,&nbsp;Arianna Sanna ,&nbsp;Altea Lecci ,&nbsp;Giulia Lo Muzio ,&nbsp;Dario Angelone ,&nbsp;Flavio Marco Mirabelli ,&nbsp;Matteo Morviducci ,&nbsp;Paolo Onorati ,&nbsp;Emanuele Messina ,&nbsp;Valeria Panebianco ,&nbsp;Carlo Catalano ,&nbsp;Matteo Bonini ,&nbsp;Paolo Palange","doi":"10.1016/j.resp.2024.104285","DOIUrl":"10.1016/j.resp.2024.104285","url":null,"abstract":"<div><h3>Background</h3><p>Long COVID is defined as persistency of symptoms, such as exertional dyspnea, twelve weeks after recovery from SARS-CoV-2 infection.</p></div><div><h3>Objectives</h3><p>To investigate ventilatory efficiency by the use of cardiopulmonary exercise testing (CPET) in patients with exertional dyspnea despite normal basal spirometry after 18 (T₁₈) and 36 months (T₃₆) from COVID-19 pneumonia.</p></div><div><h3>Methods</h3><p>One hundred patients with moderate-critical COVID-19 were prospectively enrolled in our Long COVID program. Medical history, physical examination and lung high-resolution computed tomography (HRCT) were obtained at hospitalization (T₀), 3 (T₃) and 15 months (T₁₅). All HRCTs were revised using a semi-quantitative CT severity score (CSS). Pulmonary function tests were obtained at T₃ and T₁₅. CPET was performed in a subset of patients with residual dyspnea (mMRC ≥ 1), at T₁₈ and at T₃₆.</p></div><div><h3>Results</h3><p>Remarkably, at CPET, ventilatory efficiency was reduced both at T₁₈ (V’_E/V’CO₂ slope = 31.4±3.9 SD) and T₃₆ (V’_E/V’CO₂ slope = 31.28±3.70 SD). Furthermore, we identified positive correlations between V’_E/V’CO₂ slope at T₁₈ and T₃₆ and both percentage of involvement and CSS at HRCT at T₀, T₃ and T₁₅. Also, negative linear correlations were found between V’_E/V’CO₂ slope at T₁₈ and T₃₆ and DL_CO at T₃ and T₁₅.</p></div><div><h3>Conclusions</h3><p>At eighteen months from COVID-19 pneumonia, 20 % of subjects still complains of exertional dyspnea. At CPET this may be explained by persistently reduced ventilatory efficiency, possibly related to the degree of lung parenchymal involvement in the acute phase of infection, likely reflecting a damage in the pulmonary circulation.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104285"},"PeriodicalIF":2.3,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1569904824000788/pdfft?md5=dbf6465847a6d1c57f4e75bbaa5702a4&pid=1-s2.0-S1569904824000788-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of oxidative stress and neuroinflammatory mediators in the pathogenesis of high-altitude cerebral edema in rats","authors":"Ruzanna A. Shushanyan ,&nbsp;Nikolay V. Avtandilyan ,&nbsp;Anna V. Grigoryan ,&nbsp;Anna F. Karapetyan","doi":"10.1016/j.resp.2024.104286","DOIUrl":"10.1016/j.resp.2024.104286","url":null,"abstract":"<div><p>High-altitude environments present extreme conditions characterized by low barometric pressure and oxygen deficiency, which can disrupt brain functioning and cause edema formation. The objective of the present study is to investigate several biomolecule expressions and their role in the development of High Altitude Cerebral Edema in a rat model. Specifically, the study focuses on analyzing the changes in total arginase, nitric oxide, and lipid peroxidation (MDA) levels in the brain following acute hypobaric hypoxic exposure (7620 m, SO₂=8.1 %, for 24 h) along with the histopathological assessment. The histological examination revealed increased TNF-α activity, and an elevated number of mast cells in the brain, mainly in the hippocampus and cerebral cortex. The research findings demonstrated that acute hypobaric hypoxic causes increased levels of apoptotic cells, shrinkage, and swelling of neurons, accompanied by the formation of protein aggregation in the brain parenchyma. Additionally, the level of nitric oxide and MDA was found to have increased (p&lt;0.0001), however, the level of arginase decreased indicating active lipid peroxidation and redox imbalance in the brain. This study provides insights into the pathogenesis of HACE by evaluating some biomolecules that play a pivotal role in the inflammatory response and the redox landscape in the brain. The findings could have significant implications for understanding the neuronal dysfunction and the pathological mechanisms underlying HACE development.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"327 ","pages":"Article 104286"},"PeriodicalIF":2.3,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141200638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Estimation of differential pathlength factor from NIRS measurement in skeletal muscle","authors":"B. Koirala ,&nbsp;A. Concas ,&nbsp;A. Cincotti ,&nbsp;Yi Sun ,&nbsp;A. Hernández ,&nbsp;M.L. Goodwin ,&nbsp;L.B. Gladden ,&nbsp;N. Lai","doi":"10.1016/j.resp.2024.104283","DOIUrl":"10.1016/j.resp.2024.104283","url":null,"abstract":"<div><p>The utilization of continuous wave (CW) near-infrared spectroscopy (NIRS) device to measure non-invasively muscle oxygenation in healthy and disease states is limited by the uncertainties related to the differential path length factor (<span><math><mi>DPF</mi></math></span>). <span><math><mi>DPF</mi></math></span> value is required to quantify oxygenated and deoxygenated heme groups’ concentration changes from measurement of optical densities by NIRS. An integrated approach that combines animal and computational models of oxygen transport and utilization was used to estimate the <span><math><mi>DPF</mi></math></span> value <em>in situ</em>. The canine model of muscle oxidative metabolism allowed measurement of both venous oxygen content and tissue oxygenation by CW NIRS under different oxygen delivery conditions. The experimental data obtained from the animal model were integrated in a computational model of O₂ transport and utilization and combined with Beer-Lambert law to estimate <span><math><mi>DPF</mi></math></span> value in contracting skeletal muscle. A 2.1 value was found for <span><math><mi>DPF</mi></math></span> by fitting the mathematical model to the experimental data obtained in contracting muscle (T3) (Med.Sci.Sports.Exerc.48(10):2013–2020,2016). With the estimated value of <span><math><mi>DPF</mi></math></span>, model simulations well predicted the optical density measured by NIRS on the same animal model but with different blood flow, arterial oxygen contents and contraction rate (J.Appl.Physiol.108:1169–1176, 2010 and 112:9–19,2013) and demonstrated the robustness of the approach proposed in estimating <span><math><mi>DPF</mi></math></span> value. The approach used can overcome the semi-quantitative nature of the NIRS and estimate non-invasively <span><math><mi>DPF</mi></math></span> to obtain an accurate concentration change of oxygenated and deoxygenated hemo groups by CW NIRS measurements in contracting skeletal muscle under different oxygen delivery and contraction rate.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"326 ","pages":"Article 104283"},"PeriodicalIF":2.3,"publicationDate":"2024-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1569904824000764/pdfft?md5=98270b0a35e7743d357af86b35803e3e&pid=1-s2.0-S1569904824000764-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141093999","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Respiratory characterization of a humanized Duchenne muscular dystrophy mouse model","authors":"Angela L. Roger ,&nbsp;Debolina D. Biswas ,&nbsp;Meredith L. Huston ,&nbsp;Davina Le ,&nbsp;Aidan M. Bailey ,&nbsp;Logan A. Pucci ,&nbsp;Yihan Shi ,&nbsp;Jacqueline Robinson-Hamm ,&nbsp;Charles A. Gersbach ,&nbsp;Mai K. ElMallah","doi":"10.1016/j.resp.2024.104282","DOIUrl":"10.1016/j.resp.2024.104282","url":null,"abstract":"<div><p>Duchenne muscular dystrophy (DMD) is the most common X-linked disease. DMD is caused by a lack of dystrophin, a critical structural protein in striated muscle. Dystrophin deficiency leads to inflammation, fibrosis, and muscle atrophy. Boys with DMD have progressive muscle weakness within the diaphragm that results in respiratory failure in the 2nd or 3rd decade of life. The most common DMD mouse model – the <em>mdx</em> mouse – is not sufficient for evaluating genetic medicines that specifically target the human <em>DMD</em> (h<em>DMD</em>) gene sequence. Therefore, a novel transgenic mouse carrying the h<em>DMD</em> gene with an exon 52 deletion was created (h<em>DMD</em>Δ52;<em>mdx</em>). We characterized the respiratory function and pathology in this model using whole body plethysmography, histology, and immunohistochemistry. At 6-months-old, h<em>DMD</em>Δ52;<em>mdx</em> mice have reduced maximal respiration, neuromuscular junction pathology, and fibrosis throughout the diaphragm, which worsens at 12-months-old. In conclusion, the h<em>DMD</em>Δ52;<em>mdx</em> exhibits moderate respiratory pathology, and serves as a relevant animal model to study the impact of novel genetic therapies, including gene editing, on respiratory function.</p></div>","PeriodicalId":20961,"journal":{"name":"Respiratory Physiology & Neurobiology","volume":"326 ","pages":"Article 104282"},"PeriodicalIF":2.3,"publicationDate":"2024-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141088329","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}