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Evaluation of screening tools for primary ciliary dyskinesia in Egypt: single center study. 埃及原发性睫状肌运动障碍筛查工具评估:单中心研究。
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-07-03 DOI: 10.5826/mrm.2024.966
Amr G Elbanna, Walaa Shoman, Moushira A R Elheneidy, Ihab Elsawy, Ahmad Kantar, Nader Fasseeh

Background: Primary ciliary dyskinesia (PCD) is a chronic respiratory illness that places significant strain on the healthcare system due to the complexity and expense of its diagnosis and treatment methods. The diagnostic process typically requires skilled technicians and an assortment of intricate, costly, and time-consuming approaches. Implementing screening tools can enhance efficiency by focusing the diagnostic process on those strongly suspected of having PCD. Tools such as the PCD Rule (PICADAR), North America Criteria Defined Clinical Features (NA-CDCF), the Clinical Index Score (CI), and the newly proposed CInew13 could potentially serve as useful screening tools. This study aims to examine the effectiveness of these tools individually, compare their performance against each other, and assess their results relative to prior research.

Methods: We conducted a diagnostic accuracy test on 83 Egyptian patients referred to Alexandria University Children's Hospital for potential PCD diagnosis between January 2015 and December 2022. The scores obtained from the screening tools were calculated and assessed.

Results: Of the initial group, 10 patients were ruled out because they fit other diagnostic parameters. Forty-three cases received a confirmed diagnosis, while 30 did not. Notably, the confirmed cases consistently scored higher on our screening tools than those that remained unconfirmed (p <.001, for all tested scores). We used receiver operating characteristic curves to assess and compare the effectiveness of each tool. The NA-CDCF had the smallest area under curve 0.736 (95% confiedence interval 0.619-0.832); in contrast, the CI score had the largest 0.898 (95% confidence interval 0.808-0.957).

Conclusion: All the tools tested were effective in identifying suitable patients for PCD testing at statistically significant levels. However, the PICADAR and NA-CDCF scores' performance did not significantly differ in the current study. The CI and CInew13 scores, on the other hand, outperformed both.

背景:原发性睫状肌运动障碍(PCD)是一种慢性呼吸系统疾病,由于其诊断和治疗方法的复杂性和昂贵性,给医疗系统带来了巨大的压力。诊断过程通常需要熟练的技术人员和各种复杂、昂贵、耗时的方法。使用筛查工具可将诊断过程集中在那些被强烈怀疑患有 PCD 的患者身上,从而提高效率。PCD 规则 (PICADAR)、北美临床特征定义标准 (NA-CDCF)、临床指数评分 (CI) 和新提出的 CInew13 等工具都有可能成为有用的筛查工具。本研究旨在检查这些工具各自的有效性,比较它们之间的表现,并评估它们相对于先前研究的结果:我们对 2015 年 1 月至 2022 年 12 月期间转诊至亚历山大大学儿童医院接受潜在 PCD 诊断的 83 名埃及患者进行了诊断准确性测试。我们计算并评估了筛查工具的得分:在初始组中,10 名患者因符合其他诊断参数而被排除。43例得到确诊,30例未得到确诊。值得注意的是,在我们的筛查工具中,确诊病例的得分始终高于仍未确诊的病例(P所有测试工具都能有效地识别出适合进行 PCD 检测的患者,且具有显著的统计学意义。然而,在本研究中,PICADAR 和 NA-CDCF 分数的表现并无显著差异。而 CI 和 CInew13 分数的表现则优于两者。
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引用次数: 0
Lung ultrasound in respiratory therapy: a global reflective survey. 呼吸治疗中的肺部超声:全球反思性调查。
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-07-02 DOI: 10.5826/mrm.2024.980
Chris Sara Mathew, Edwin Dias, Jithin Kalathikudiyil Sreedharan, Mohammed Al Ahmari, Lisa Trujillo, Andrew West, Manjush Karthika

Background: Lung ultrasound (LUS) is a non-invasive point of care diagnostic tool used to assess the presence and severity of various lung disorders for more than two decades. Within the healthcare professionals are the respiratory therapists (RTs) who play a vital role in managing ventilated and other patients requiring respiratory support, but the incorporation of LUS into their scope of practice has not been well highlighted. This international cross-sectional survey was specifically designed to evaluate the knowledge, attitude, and practice of RTs with respect to LUS.

Methods: This observational cross-sectional study was conducted among RTs from different parts of the world using a questionnaire-based study tool. 514 RTs responded to all the questions and were considered for statistical analysis. Descriptive statistics, Analysis of variance, Fisher's exact, Chi-square, Bonferroni post-hoc analysis, and Binomial logistic regression analyses were performed to identify the significance of the data.

Results: From 22 countries, 514 RTs responded to the survey, with the major share from the middle eastern countries. Out of the 514 responders, 44.9% of the responders were in the age group of 23-30 years; 67.1% were bachelor's degree holders; and 40.9% of participants had more than 10 years of experience. The knowledge-based questions revealed that RTs with higher experience and academic qualification provided more positive responses. While in the attitude-related domain it is observed that standardized training in LUS helps them to enhance the current practice and to add LUS to the academic curriculum of respiratory therapy schools; however there remains barriers to practice LUS based on their responses. The practice-based questions revealed that RTs expect some additional seminars/workshops/webinars to be done on LUS frequently. More than half of the participants are found to be knowledgeable with a positive attitude and working towards the inclusion of LUS in respiratory therapy profession.

Conclusion: It is concluded that respiratory therapists have a positive attribute towards the inclusion of lung ultrasound in their clinical practice. Providing more structured training for professional respiratory therapists and including lung ultrasound modules in the respiratory therapy school curriculum may facilitate mastering their diagnostic skills, thereby expanding the scope of practice.

背景:肺部超声波(LUS)是一种非侵入性的护理点诊断工具,用于评估各种肺部疾病的存在和严重程度已有二十多年的历史。医护人员中的呼吸治疗师(RT)在管理呼吸机患者和其他需要呼吸支持的患者方面发挥着至关重要的作用,但将肺部超声纳入其工作范围的问题却没有得到很好的强调。这项国际横断面调查旨在评估 RTs 对 LUS 的认识、态度和实践:这项观察性横断面研究使用基于问卷的研究工具,对来自世界各地的 RT 进行了调查。514 名 RT 回答了所有问题,并被纳入统计分析。研究采用了描述性统计、方差分析、费雪精确法、Chi-square、Bonferroni 事后分析和二项式逻辑回归分析来确定数据的显著性:来自 22 个国家的 514 名 RTs 回答了调查,其中大部分来自中东国家。在 514 名答复者中,44.9% 的答复者年龄在 23-30 岁之间;67.1% 的答复者拥有学士学位;40.9% 的答复者拥有 10 年以上的工作经验。基于知识的问题显示,经验和学历较高的 RT 提供了更积极的回答。在态度相关领域,他们认为 LUS 标准化培训有助于他们提高目前的实践水平,并将 LUS 纳入呼吸治疗学校的学术课程;然而,根据他们的回答,在实践 LUS 方面仍然存在障碍。基于实践的问题显示,呼吸治疗师希望能经常举办一些额外的 LUS 研讨会/讲习班/网络研讨会。半数以上的参与者对 LUS 抱有积极的态度,并努力将 LUS 纳入呼吸治疗专业:结论:呼吸治疗师对将肺部超声纳入其临床实践持积极态度。为专业呼吸治疗师提供更多有组织的培训,并将肺部超声模块纳入呼吸治疗学校的课程,可促进其掌握诊断技能,从而扩大实践范围。
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引用次数: 0
Post-COVID conditions response: a collaborative approach to -establishing multidisciplinary clinics in Ecuador. COVID 后情况应对:在厄瓜多尔建立多学科诊所的合作方法。
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-07-01 DOI: 10.5826/mrm.2024.974
Paola Yépez, Vanessa Noboa, Mary Bolgiano, Alejandra Mafla, Evelyn Caballero, Bhakti Hansoti, Michelle Grunauer

Introduction: Worldwide, 3.7% (144.7 million) of people diagnosed with COVID-19 developed Post-COVID Conditions (PCC). Therefore, creating and implementing multidisciplinary rehabilitation clinics is important to address the needs of patients and improve overall recovery. This study was made possible with support from the United States Agency for International Development funded RISE program, under the terms of the cooperative agreement 7200AA19CA00003.

Methods: This case study was conducted in Ecuador and describes the creation and implementation of 21 PCC rehabilitation clinics in primary healthcare centers and secondary level hospitals in 7 provinces across the country. Data was gathered for the identification of partnering health facilities and needs, for the evaluation of knowledge enhancement in health professionals after a specific training program, and for the measurement of key performance indicators. This article emphasizes the organization, educational strategies, and implementation of rehabilitation programs tailored specifically for the management of Post-COVID Conditions in Ecuador.

Results: The implementation of PCC rehabilitation clinics involved a collaborative effort between the Ministry of Public Health (MOPH), the private sector and a non-governmental organization (Jhpiego). Twenty-one health facilities from the primary and secondary level of care were selected, and PCC rehabilitation implemented in 7 provinces of Ecuador. Additionally, 133 health providers were trained and a total of 13,846 patients treated, among whom 859 had a diagnosis of PCC. Medical doctors outperformed nurses in both pre- and post-tests scores. However, all healthcare professionals demonstrated comparable improvement in knowledge acquisition. Rehabilitation manuals were developed and adopted by the MOPH, rehabilitation equipment was donated and a mobile application, "RESPIRA", was developed and disseminated free of charge.

Conclusion: The establishment of PCC rehabilitation clinics in Ecuador was successful in identifying patients in need of early rehabilitation. The insights of this study can serve as a guide for the development of similar initiatives in other countries. Tailored courses are essential to address disparities and ensure comprehensive skill development and promote equitable healthcare delivery.

导言:在全球范围内,3.7%(1.447 亿)确诊为 COVID-19 的患者出现了 COVID 后遗症 (PCC)。因此,建立和实施多学科康复诊所对于满足患者需求和改善整体康复非常重要。根据合作协议 7200AA19CA00003 的条款,本研究得到了美国国际开发署资助的 RISE 计划的支持:本案例研究在厄瓜多尔进行,介绍了在全国 7 个省的初级医疗保健中心和二级医院创建和实施 21 个 PCC 康复诊所的情况。收集的数据用于确定合作的医疗机构和需求、评估医疗专业人员在接受特定培训项目后的知识提升情况,以及衡量关键绩效指标。这篇文章强调了厄瓜多尔专门为后COVID病症管理而定制的康复计划的组织、教育策略和实施情况:结果:在公共卫生部(MOPH)、私营部门和非政府组织(Jhpiego)的共同努力下,PCC 康复诊所得以实施。从一级和二级医疗机构中选择了 21 家医疗机构,并在厄瓜多尔的 7 个省实施了 PCC 康复项目。此外,133 名医疗服务提供者接受了培训,共有 13,846 名患者接受了治疗,其中 859 人确诊为 PCC 患者。医生在测试前和测试后的成绩均优于护士。不过,所有医护人员在知识掌握方面的进步都不相上下。澳门卫生部编写并采用了康复手册,捐赠了康复设备,开发并免费推广了移动应用程序 "RESPIRA":结论:厄瓜多尔 PCC 康复诊所的建立成功地识别了需要早期康复的患者。这项研究的启示可为其他国家制定类似举措提供指导。量身定制的课程对于解决差异、确保全面的技能发展和促进公平的医疗保健服务至关重要。
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引用次数: 0
Pharmacological treatment in Idiopathic Pulmonary Fibrosis: current issues and future perspectives 特发性肺纤维化的药物治疗:当前问题与未来展望。
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-06-13 DOI: 10.5826/mrm.2024.982
Carlo Vancheri, Enrico Sciacca, Giuseppe Muscato, Lucia Spicuzza, Mary Fruciano, Elisa Gili, Gianluca Sambataro, Stefano Palmucci, Alessandro Libra

Idiopathic pulmonary fibrosis (IPF) represents a fibrotic interstitial lung disease characterized by uncertain etiology and poor prognosis. Over the years, the path to effective treatments has been marked by a series of advances and setbacks. The introduction of approved antifibrotic drugs, pirfenidone and nintedanib, marked a pivotal moment in the management of IPF. However, despite these advances, these drugs are not curative, although they can slow the natural progression of the disease. The history of drug therapy for IPF goes together with the increased understanding of the pathogenic mechanisms underlying the disease. Based on that, current research efforts continue to explore new therapies, possible personalized treatment strategies, drug combinations, and potential biomarkers for diagnosis and prognosis. In this review, we outline the route that led to the discover of the first effective therapies, ongoing clinical trials, and future directions in the search for more effective treatments.

特发性肺纤维化(IPF)是一种纤维化间质性肺病,病因不明,预后不良。多年来,有效治疗的道路上经历了一系列的进步和挫折。抗纤维化药物吡非尼酮(pirfenidone)和宁替达尼(nintedanib)的问世标志着 IPF 治疗的关键时刻。然而,尽管取得了这些进展,但这些药物并不能治愈疾病,尽管它们可以延缓疾病的自然进展。药物治疗 IPF 的历史与人们对该疾病致病机制认识的不断深入密不可分。在此基础上,目前的研究工作继续探索新的疗法、可能的个性化治疗策略、药物组合以及用于诊断和预后的潜在生物标志物。在这篇综述中,我们将概述导致发现首批有效疗法的途径、正在进行的临床试验以及寻找更有效疗法的未来方向。
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引用次数: 0
Effect of Home-Based Pulmonary Rehabilitation on Pulmonary Fibrosis. 居家肺康复对肺纤维化的影响
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-06-05 DOI: 10.5826/mrm.2024.950
Rashmita Saha, Vijay Pratap Singh, Stephen Rajan Samuel, Vishak Acharya K, Preetam Rajgopal Acharya, K Vijaya Kumar

Background: Pulmonary fibrosis is a chronic, progressive lung condition that involves lung tissue scarring and thickening. The effects of home-based pulmonary rehabilitation (PR) in post-covid pulmonary fibrosis (PCPF) and other forms of fibrosis together have not been evaluated. This study aims to evaluate the effectiveness of home-based pulmonary rehabilitation on pulmonary function, functional capacity, and health-related quality of life in people with pulmonary fibrosis (post-COVID pulmonary fibrosis, pulmonary fibrosis secondary to pulmonary tuberculosis (TB), pulmonary fibrosis secondary to interstitial lung disease (ILD), pulmonary fibrosis secondary to bronchiectasis).

Methods: A single-group pretest-posttest experimental study was performed after recruiting 98 pulmonary fibrosis subjects from K.M.C hospitals. After being screened for the inclusion and exclusion criteria, 45 subjects were analyzed, and 6 subjects were lost to follow-up.  A home-based pulmonary rehabilitation program was carried out for 8 weeks (warm-up, stretching exercises, aerobic exercise, strength training for upper limb and lower limb, breathing exercises mainly involved; others: energy saving techniques, controlled coughing techniques, dyspnea relieving positions). The program was supervised via weekly phone calls. Pulmonary function (Pulmonary function test), exercise capacity (6-minute walk test), dyspnea (modified Borg scale), and health-related quality of life (SF-36) were evaluated before and after the intervention. During the enrollment and after the 6-minute walk test, saturation of peripheral oxygen (SPO2) level was also evaluated pre-intervention and after the 8-weeks program.

Results: Pulmonary function [FVC(L) t = -12.52, p<0.05; FEV1(L) t = -2.56, p<0.05; FEV1/FVC t = 7.98, p<0.05 and DLCO (ml/min/mmHg) t = -5.13, p<0.05], 6MWD [MD 88.66; p<0.05] and HRQOL measured by SF-36 scores (p<0.05) were improved significantly. Both the baseline SPO2 level before the 6MWT [MD 1.07, p<0.05] and the SPO2 level after the 6MWT [MD 1.16, p<0.05] showed a significant improvement. The rating of perceived exertion(dyspnea) [MD 1.30, p<0.05] was reduced significantly after the 8-week program.

Conclusion: Our study shows that home-based pulmonary rehabilitation is an effective option for improving lung function and physical functional capacity by reducing dyspnea perception and improving the saturation of peripheral oxygen (SPO2) level, and enhancing the quality of life in people with pulmonary fibrosis.

背景:肺纤维化是一种慢性进行性肺部疾病,涉及肺组织瘢痕和增厚。目前尚未评估家庭肺康复(PR)对卵巢癌后肺纤维化(PCPF)和其他形式的肺纤维化的影响。本研究旨在评估家庭肺康复对肺纤维化患者(COVID后肺纤维化、肺结核(TB)继发的肺纤维化、间质性肺病(ILD)继发的肺纤维化、支气管扩张继发的肺纤维化)的肺功能、功能能力和健康相关生活质量的影响:方法:从 K.M.C 医院招募 98 名肺纤维化受试者,进行单组前测-后测实验研究。根据纳入和排除标准进行筛选后,对 45 名受试者进行了分析,6 名受试者失去了随访机会。 受试者接受了为期 8 周的家庭肺康复计划(热身运动、伸展运动、有氧运动、上肢和下肢力量训练,主要涉及呼吸运动;其他:节能技巧、控制性咳嗽技巧、呼吸困难缓解体位)。该计划每周通过电话进行监督。干预前后对肺功能(肺功能测试)、运动能力(6 分钟步行测试)、呼吸困难(改良博格量表)和健康相关生活质量(SF-36)进行了评估。在入学时和 6 分钟步行测试后,还对干预前和 8 周计划后的外周血氧饱和度(SPO2)水平进行了评估:结果:肺功能[FVC(L) t = -12.52, p结论:我们的研究表明,通过减少呼吸困难感和提高外周血氧饱和度(SPO2)水平来改善肺功能和身体机能,并提高肺纤维化患者的生活质量,家庭肺康复是一种有效的选择。
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引用次数: 0
mRNA vaccines protect from the lung microvasculature injury and the capillary blood volume loss occurring in SARS-CoV-2 paucisymptomatic infections. mRNA 疫苗可防止 SARS-CoV-2 无症状感染时出现的肺微血管损伤和毛细血管血容量减少。
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-06-03 DOI: 10.5826/mrm.2024.973
Roberto W Dal Negro, Paola Turco, Massimiliano Povero

Introduction: The reduction of lung capillary blood volume (Vc) had been identified as the microvascular injury mostly underlying the respiratory Long-COVID syndrome following post-COVID-19 pneumonia. The same kind of injury have been recently also found in several individuals after milder paucisymptomatic SARS-CoV-2 infections. Though current guidelines strongly recommend vac-cination, studies aimed to investigate the in vivo protection of anti-SARS-CoV-2 vaccines on lung microvascular targets still are missing to our best knowledge.

Aim: to assess the protection of mRNA vaccines from the reduction of lung capillary blood volume (Vc) caused by pauci-symptomatic SARS.CoV-2 infections in vaccinated compared to unvaccinated individuals.

Methods: Non-smoking individuals with recent paucisymptomatic SARS-CoV-2 infection were divided into vaccinated and unvaccinated groups. Lung function parameters, including single-breath diffusing capacity and microvascular blood volume, were compared between groups.

Results: fifty vaccinated and twenty-five unvaccinated well-matched individuals were studied. Differently than usual lung function parameters, only the single-breath simultaneous assessment of sDLCO, sDLNO/sDLCO ratio and Vc allowed to identify the occurrence of the lung microvascular injury with high sensitivity and specificity (p<0.001).

Conclusion: mRNA vaccines proved to exert a high protection from the loss of lung capillary blood volume (Vc) induced by SARS.CoV-2 paucisymptomatic infections (p<0.001). The availability of this non-invasive investigational model should be regarded as a very helpful tool for assessing and comparing in vivo the protective effect of mRNA vaccines on the human microvascular structures of the deep lung.

简介肺毛细血管血容量(Vc)的减少已被确定为 COVID-19 后肺炎后呼吸系统 Long-COVID 综合征的主要微血管损伤。最近,在一些感染 SARS-CoV-2 后症状较轻的人身上也发现了同样的损伤。目的:与未接种疫苗的人相比,评估 mRNA 疫苗在接种疫苗的人与无症状的 SARS.CoV-2 感染引起的肺毛细血管血容量(Vc)减少方面的保护作用:方法: 将近期感染无症状 SARS-CoV-2 的非吸烟者分为接种疫苗组和未接种疫苗组。结果:研究了 50 名接种疫苗和 25 名未接种疫苗的匹配良好的个体。与通常的肺功能参数不同,只有单次呼吸同时评估 sDLCO、sDLNO/sDLCO 比值和 Vc 才能以较高的灵敏度和特异性(p 结论:事实证明,mRNA 疫苗对 SARS.CoV-2 引起的肺毛细血管血容量(Vc)损失具有较高的保护作用(p
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引用次数: 0
Characteristics of culture-negative subclinical pulmonary tuberculosis: a single-center observation. 培养阴性亚临床肺结核的特征:单中心观察。
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-05-15 DOI: 10.5826/mrm.2024.955
Supakorn Chansaengpetch, Rathachai Kaewlai, Tirathat Virojskulchai, Apinut Jaroonpipatkul, Nitipatana Chierakul, Nisa Muangman, Trongtum Tongdee, Wiwatana Tanomkiat, Krisna Dissaneevate, Sitthiphon Bunman, Ruchira Ruangchira-Urai, Wanwisa Dejnirattisai, Narongpon Dumavibhat

Background: Little is known about culture-negative subclinical pulmonary tuberculosis (TB), and its diagnosis remains challenging. Therefore, this study aimed to identify the characteristics and the extent of disease associated with culture-negative subclinical pulmonary TB.

Methods: This retrospective cohort study was conducted on immunocompetent individuals with subclinical pulmonary TB at a university hospital in Thailand from January 2014 to December 2019. Subclinical pulmonary TB was diagnosed based on the presence of radiographic abnormalities consistent with TB in the absence of TB symptoms. All subjects demonstrated significant improvement or resolution of radiographic abnormalities following the completion of treatment. At least two negative sputum cultures were needed to fulfill the definition of culture-negative pulmonary TB. Data were analyzed using univariate and multiple logistic regression analyses to determine the characteristics of those with culture-negative subclinical pulmonary TB compared to culture-positive ones.

Results: Out of the 106 individuals identified with subclinical pulmonary TB, 84 met the criteria for inclusion in the analysis. The study found lower radiographic extent and increasing age were key attributes of culture-negative subclinical pulmonary TB. The odds ratios (95% confidence interval) were 7.18 (1.76 to 29.35) and 1.07 (1.01 to 1.13), respectively. They tend to have lower rates of bilateral involvement in both chest x-ray (8.5% vs. 32.0%, p=0.006) and computed tomography (15.4% vs. 42.9%, p=0.035). However, no other specific radiographic findings were identified.

Conclusions: People with culture-negative subclinical pulmonary TB were likely to have less radiographic -severity, reflecting early disease. Nevertheless, no radiographic patterns, except for unilaterality, were related to culture-negative subclinical pulmonary TB.

背景:人们对培养阴性亚临床肺结核(TB)知之甚少,其诊断仍具有挑战性。因此,本研究旨在确定与培养阴性亚临床肺结核相关的特征和疾病程度:这项回顾性队列研究于 2014 年 1 月至 2019 年 12 月期间在泰国一所大学医院对患有亚临床肺结核的免疫功能正常者进行了研究。亚临床肺结核的诊断依据是在无肺结核症状的情况下出现与肺结核一致的影像学异常。完成治疗后,所有受试者的影像学异常均有明显改善或消失。至少有两次痰培养阴性才符合培养阴性肺结核的定义。我们使用单变量和多元逻辑回归分析法对数据进行了分析,以确定培养阴性亚临床肺结核患者与培养阳性亚临床肺结核患者的特征:在106名亚临床肺结核患者中,84人符合纳入分析的标准。研究发现,较低的影像学范围和年龄的增加是培养阴性亚临床肺结核的主要特征。几率比(95% 置信区间)分别为 7.18(1.76 至 29.35)和 1.07(1.01 至 1.13)。他们在胸部 X 光(8.5% 对 32.0%,P=0.006)和计算机断层扫描(15.4% 对 42.9%,P=0.035)中双侧受累的比例往往较低。然而,没有发现其他特殊的放射学结果:结论:培养阴性的亚临床肺结核患者的影像学严重程度可能较低,这反映了疾病的早期。尽管如此,除了单侧肺结核外,没有任何放射学模式与培养阴性亚临床肺结核有关。
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引用次数: 0
Effective treatment with oral Salbutamol on late onset respiratory impairment in a DOK7 Congenital Myasthenia Syndrome: a case report. 口服沙丁胺醇有效治疗 DOK7 先天性肌无力综合征晚期呼吸障碍:病例报告。
IF 2 Q3 RESPIRATORY SYSTEM Pub Date : 2024-05-15 DOI: 10.5826/mrm.2024.942
Corina Tomsa, Fausta Viccaro, Luigi Panza, Letizia D'Antoni, Paolo Palange

Introduction: DOK7 gene deficiency is a neuromuscular disease with an alteration in post-synaptic neuromuscular junction, leading to progressive respiratory impairment. Although, the therapy is not standardized, adrenergic agonists are suggested as first-line treatment.  Case presentation: Our patient had an ambiguous late childhood-onset and had a generalized muscle weakness free of respiratory symptoms during the early phase of the disease. Subsequently, when the respiratory muscle and the diaphragm involvement was impaired, a substantial loss of respiratory function with hypopneas and severe desaturation was detected. It was noteworthy the striking respiratory beneficial impact of oral salbutamol in the resolution of symptoms and functional impairments, leading to a remarkable respiratory improvement and a better quality of life.  Conclusion: Oral salbutamol treatment combined to a timely clinical recognition led to an outstanding respiratory improvement.

简介DOK7 基因缺乏症是一种神经肌肉疾病,突触后神经肌肉接头发生改变,导致进行性呼吸障碍。虽然治疗方法尚未标准化,但肾上腺素能激动剂被建议作为一线治疗药物。 病例介绍:我们的患者在儿童晚期发病,病程早期全身肌肉无力,无呼吸道症状。随后,当呼吸肌和膈肌受累受损时,发现呼吸功能大幅减退,并伴有低通气和严重饱和度降低。值得注意的是,口服沙丁胺醇在缓解症状和功能障碍方面对呼吸系统有显著的益处,从而显著改善了呼吸系统,提高了生活质量。 结论口服沙丁胺醇治疗与及时的临床识别相结合,可显著改善患者的呼吸状况。
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引用次数: 0
Knowledge and perceptions regarding pulmonary rehabilitation amongst Ecuadorian physicians following COVID-19 outbreak. COVID-19 爆发后厄瓜多尔医生对肺康复的认识和看法。
IF 2.3 Q3 RESPIRATORY SYSTEM Pub Date : 2024-03-15 eCollection Date: 2024-01-01 DOI: 10.5826/mrm.2024.919
Miguel Felix, Emanuel Vanegas, Azza Sarfraz, Zouina Sarfraz, Genesis Camacho, Alanna Barrios-Ruiz, Jack Michel, Zeynep Yukselen, Arjola Agolli, Derly Madeleiny Andrade Molina, Pilar Cejudo, Karla Robles-Velasco, María José Farfán Bajaña, Juan Carlos Calderón, Arturo Cortes-Telles, Ivan Cherrez-Ojeda

Background: Pulmonary rehabilitation is already an established technique for patients with chronic respiratory disease, aimed at improving breathlessness, exercise capacity, health status, and well-being. The aim of this study was to assess the knowledge and perceptions about pulmonary rehabilitation post-COVID-19 infection among Ecuadorian physicians.

Methods: We conducted a cross-sectional online survey-based study using a 27-item questionnaire to assess the knowledge about specific topics related to pulmonary rehabilitation. The sample comprised Ecuadorian physicians who were currently enrolled to an active medical practice that included care to COVID-19 patients. Descriptive statistics were applied for demographic variables of interest. A chi-square goodness of fit test was used to determine whether the observed frequencies of each of the answers per query were within or outside of the expected frequencies by chance.

Results: In total, 295 participants answered the survey, out of which 57.3% were general practitioners. Most agreed that COVID-19 infected patients must be followed-up with some measurement of respiratory function (81.4%, p=0.000), but only 18.3% (n=54, p=0.000) were aware of specific guidelines related to rehabilitation. 93.6% (n=276, p=0.000) considered that pulmonary rehabilitation provides a benefit, of any kind, to patients with past COVID-19 infection.

Conclusions: Most physicians considered pulmonary rehabilitation beneficial following COVID-19. However, there is uncertainty on how to adequately follow up patients, complementary tests, and specific guidelines outlining rehabilitative interventions.

背景:肺康复已经是一项针对慢性呼吸系统疾病患者的成熟技术,旨在改善呼吸困难、运动能力、健康状况和幸福感。本研究旨在评估厄瓜多尔医生对 COVID-19 感染后肺康复的了解和看法:我们进行了一项基于在线调查的横断面研究,使用 27 个项目的问卷来评估与肺康复相关的特定主题的知识。样本包括厄瓜多尔医生,他们目前都在积极开展医疗实践,其中包括对 COVID-19 患者的护理。对相关人口统计学变量进行了描述性统计。采用卡方拟合优度检验来确定每个问题的答案的观察频率是否在预期频率之内或之外:共有 295 名参与者回答了调查,其中 57.3% 为全科医生。大多数人同意,COVID-19 感染者必须通过测量呼吸功能进行随访(81.4%,p=0.000),但只有 18.3%(n=54,p=0.000)的人了解与康复相关的具体指南。93.6%(n=276,p=0.000)的医生认为肺康复治疗对既往感染过COVID-19的患者有任何益处:结论:大多数医生认为,COVID-19 感染后进行肺康复治疗是有益的。结论:大多数医生认为,COVID-19 感染后进行肺康复治疗是有益的,但在如何对患者进行充分随访、辅助检查以及概述康复干预措施的具体指南方面还存在不确定性。
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引用次数: 0
Kyphoscoliosis complicating asthma with fixed airway obstruction. 伴有固定气道阻塞的哮喘并发脊柱后凸。
IF 2.3 Q3 RESPIRATORY SYSTEM Pub Date : 2024-01-29 eCollection Date: 2024-02-06 DOI: 10.5826/mrm.2024.937
Sarah Bonello, Yanika Farrugia, Theresa Mallia, Nicoletta Maniscalco, Martin Balzan

Introduction: Kyphoscoliosis is present in up to 2% of the juvenile population and can have deleterious effects on respiratory mechanics, leading to chronic respiratory failure later on in adult life.

Case presentation: Hereby we describe a 53-year-old patient with severe uncontrolled asthma who presented with chronic hypercapnic respiratory failure. During her medical workup, she was noted to have several comorbidities leading to her respiratory failure. The patient had radiological evidence of bronchiectasis with recurrent episodes of infection, and a severe deformity of the spine due to Kyphoscoliosis. Probably the kyphotic component of this deformity had worsened due to a long history of oral steroid use leading to severe osteoporosis and consequent vertebral compression fractures reaching a Cobb angle of 73 degrees. This was probably caused by the patient's non-compliance with inhaler therapy and an excessive reliance on oral steroid use. Her respiratory failure was treated with domiciliary noninvasive positive pressure ventilation and 24-hour oxygen therapy and her symptoms improved.

Conclusion: A multidisciplinary approach across different specialities is necessary when managing such a patient with kyphoscoliosis, bronchiectasis, asthma with airflow limitation with respiratory failure.

导言:多达 2% 的青少年存在脊柱后凸,会对呼吸力学产生有害影响,导致成年后出现慢性呼吸衰竭:我们在此描述一名 53 岁的患者,她患有严重的哮喘,且病情未得到控制,并出现慢性高碳酸血症呼吸衰竭。在体检过程中,我们发现她患有多种导致呼吸衰竭的并发症。该患者有支气管扩张的放射学证据,并伴有反复感染,脊柱因脊柱后凸而严重变形。可能是由于长期口服类固醇导致严重骨质疏松,进而造成椎体压缩性骨折,导致脊柱畸形加重,Cobb 角达到 73 度。这可能是由于患者不遵从吸入器治疗和过度依赖口服类固醇造成的。她的呼吸衰竭得到了家用无创正压通气和 24 小时氧疗的治疗,症状也得到了改善:在治疗这类患有脊柱侧弯、支气管扩张、气流受限并伴有呼吸衰竭的哮喘患者时,有必要采用跨学科的方法。
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引用次数: 0
期刊
Multidisciplinary Respiratory Medicine
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