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The Impact of a Newly Established Multidisciplinary Team on the Interventional Treatment of Patients With Emphysema. 新成立的多学科团队对肺气肿患者介入治疗的影响。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-06-19 eCollection Date: 2019-01-01 DOI: 10.1177/1179548419852063
Vasileios Kouritas, Richard Milton, Emmanouel Kefaloyannis, Kostas Papagiannopoulos, Allesandro Brunelli, Doytchin Dimov, Sishik Karthik, Andrew Hardy, Peter Tcherveniakov, Nilanjan Chaudhuri

Background: The emphysema interventional treatment involves mainly lung volume reduction surgery (LVRS) and endobronchial valve (EBV) implantation. Few institutes discuss these cases at a dedicated emphysema multidisciplinary team (MDT) meeting.

Objectives: To investigate the impact of a newly established dedicated emphysema MDT meeting on the interventional treatment of such patients.

Methods: During a study period of 4 years, the outcome of 44 patients who underwent intervention according to the proposal of the emphysema MDT (group A) was compared with the outcome of 44 propensity score matched patients (group B) treated without the emphysema MDT proposal.

Results: More LVRS and less EBV insertions were performed in group A (P =.009). In group B, the interventions were performed sooner than in group A (P =.003). Postoperative overall morbidity and length of in-hospital stay were similar in the 2 groups (P =.918 and .758, respectively). Improvement of breathing ability was reported in more patients from group A (P =.012). In group B, the total number of re-interventions was higher (P =.001) and the time to re-intervention had the tendency to be less (P =.069). Survival was similar between the 2 groups (P =.884). Intervention without discussion at the MDT and EBV as initial intervention was an independent predictor of re-intervention.

Conclusions: Interventional treatment for patients with chronic obstructive pulmonary disease (COPD) after discussion at a dedicated MDT involved more LVRS performed, required fewer interventions for their disease, and had longer re-intervention-free intervals and better breathing improvement.

背景:肺气肿的介入治疗主要包括肺减容手术(LVRS)和支气管内瓣膜(EBV)植入术。很少有机构在专门的肺气肿多学科小组(MDT)会议上讨论这些病例。目的:探讨新成立的肺气肿MDT专题会议对此类患者介入治疗的影响。方法:在为期4年的研究期间,将44例根据肺气肿MDT建议进行干预的患者(a组)与44例倾向评分匹配的未接受肺气肿MDT建议的患者(B组)的结果进行比较。结果:A组LVRS较多,EBV插入较少(P = 0.009)。B组干预时间早于A组(P = 0.003)。两组患者术后总发病率和住院时间相似(P =。分别为918和0.758)。A组患者呼吸能力改善较多(P = 0.012)。B组患者再干预总次数较高(P = .001),再干预时间有减少趋势(P = .069)。两组患者生存率相近(P = .884)。在MDT和EBV中不进行讨论的干预作为初始干预是再次干预的独立预测因子。结论:在专门的MDT讨论后,慢性阻塞性肺疾病(COPD)患者的介入治疗涉及更多的LVRS,对其疾病需要更少的干预,并且有更长的再干预间隔和更好的呼吸改善。
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引用次数: 4
An Exploration Into Knowledge, Attitudes, and Beliefs Towards Risky Health Behaviours in a Paediatric Cystic Fibrosis Population. 探讨儿童囊性纤维化人群对危险健康行为的知识、态度和信念。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-05-30 eCollection Date: 2019-01-01 DOI: 10.1177/1179548419849427
Rebecca Keyte, Helen Egan, Michail Mantzios

Risky behaviours are prevalent within the cystic fibrosis (CF) population; however, there is a lack of research which has investigated risky behaviour engagement among adolescents with CF, with reasons for initiation currently being unknown, as no qualitative studies have been conducted. This research therefore examines knowledge, attitudes, and beliefs towards risky behaviours at an age commonly associated with initiation. Ten paediatric participants were recruited. Thematic analysis illustrated several psychological factors associated with risky behaviours. A desire for normalcy was evident, with this been associated with a desire to engage in normalised risky behaviours. Evidence of a life-orientated illness perspective was also prevalent, with participants believing that many individuals engage in risky behaviours for fun. Overall, there was a reported lack of knowledge on consequences of risky behaviours, with many participants not being informed of these by health care professionals (HCPs). This research provides insight into an area of CF paediatric care which could be improved on, with the provision of awareness regarding risky behaviours not being embedded within paediatric CF care. Consequently, this research demonstrates the need for interventions to be integrated into paediatric CF care for the prevention and reduction of risky behaviours.

危险行为在囊性纤维化(CF)人群中普遍存在;然而,由于没有进行定性研究,因此缺乏对CF青少年危险行为参与的调查,目前尚不清楚开始的原因。因此,这项研究考察了在一个通常与启蒙有关的年龄对危险行为的知识、态度和信念。招募了10名儿科参与者。专题分析说明了与危险行为相关的几个心理因素。对正常状态的渴望是显而易见的,这与从事正常风险行为的愿望有关。以生活为导向的疾病观点的证据也很普遍,参与者认为许多人为了好玩而从事危险行为。总的来说,据报道,人们对危险行为的后果缺乏了解,许多参与者没有被卫生保健专业人员告知这些情况。这项研究深入了解了CF儿科护理的一个领域,该领域可以改进,对儿童CF护理中未包含的危险行为的认识。因此,这项研究表明,有必要将干预措施纳入儿科CF护理,以预防和减少危险行为。
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引用次数: 15
Respiratory Fungal Diseases in Adult Patients With Cystic Fibrosis. 成人囊性纤维化患者的呼吸道真菌疾病
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-05-22 eCollection Date: 2019-01-01 DOI: 10.1177/1179548419849939
Emanuele Delfino, Filippo Del Puente, Federica Briano, Chiara Sepulcri, Daniele Roberto Giacobbe

Clinical manifestations of respiratory fungal diseases in adult cystic fibrosis (CF) patients are very heterogeneous, ranging from asymptomatic colonization to chronic infections, allergic disorders, or invasive diseases in immunosuppressed CF patients after lung transplantation. In this narrative review, mainly addressed to clinicians without expertise in CF who may nonetheless encounter adult CF patients presenting with acute and chronic respiratory syndromes, we briefly summarize the most representative clinical aspects of respiratory fungal diseases in adult CF patients.

成人囊性纤维化(CF)患者呼吸道真菌疾病的临床表现非常异质性,从无症状定植到肺移植后免疫抑制CF患者的慢性感染、过敏性疾病或侵袭性疾病。在这篇叙述性综述中,主要针对没有CF专业知识的临床医生,他们可能会遇到患有急性和慢性呼吸综合征的成人CF患者,我们简要总结了成人CF患者呼吸道真菌疾病最具代表性的临床方面。
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引用次数: 16
Aztreonam Lysine Inhalation Solution in Cystic Fibrosis. 氨曲南赖氨酸吸入液在囊性纤维化中的作用。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-04-12 eCollection Date: 2019-01-01 DOI: 10.1177/1179548419842822
Elizabeth Claire Elson, Joel Mermis, Deepika Polineni, Christopher M Oermann

Patients with cystic fibrosis (CF) develop pulmonary disease secondary to airway infection and dysregulated inflammation. Therapeutic innovations such as nebulized antimicrobial therapy targeting specific pathogens have resulted in improvements in quality of life and life expectancy. Aztreonam lysine for inhalation (AZLI) solution was initially approved to improve respiratory symptoms in CF patients with Pseudomonas aeruginosa (PA) in 2010 by the Food and Drug Administration. Since then, research broadening labeling and clinical application has been developed. In this review, we analyze published and ongoing research regarding AZLI therapy in CF. A search of the Cochrane Database of Systematic Reviews and the PubMed and ClinicalTrials.gov databases was conducted to identify publications about AZLI. Three pre-approval studies were identified and assessed. Two are Phase 3, placebo-controlled trials, assessing a variety of safety and efficacy endpoints, leading to FDA approval. The third is an open-label extension of the two previous trials. An additional seven post-approval, completed trials were identified and are included in this review. They represent a variety of study designs including safety and efficacy in patients with mild lung disease and young patients, an active comparator trial vs inhaled tobramycin, an eradication study, a study among patients with Burkholderia cepacia, and a study assessing continuous alternating antibiotic therapy. Finally, five ongoing clinical trials are discussed. Overall, studies demonstrated that inhaled aztreonam is a safe and effective antimicrobial treatment for the eradication of newly acquired P. aeruginosa and long-term suppressive therapy of chronic endobronchial infection among people with cystic fibrosis.

囊性纤维化(CF)患者继发于气道感染和炎症失调的肺部疾病。治疗创新,如针对特定病原体的雾化抗菌治疗,已导致生活质量和预期寿命的改善。吸入氨曲南赖氨酸(AZLI)溶液最初于2010年被美国食品和药物管理局(fda)批准用于改善铜绿假单胞菌(PA) CF患者的呼吸道症状。从那时起,扩大标签和临床应用的研究得到了发展。在这篇综述中,我们分析了已经发表的和正在进行的关于AZLI治疗CF的研究。我们检索了Cochrane系统评价数据库、PubMed和ClinicalTrials.gov数据库,以确定关于AZLI的出版物。确定并评估了三项预批准研究。其中两个是第三阶段,安慰剂对照试验,评估各种安全性和有效性终点,最终获得FDA批准。第三个试验是前两个试验的开放标签扩展。另外7项批准后完成的试验被确定并纳入本综述。它们代表了各种各样的研究设计,包括轻度肺病患者和年轻患者的安全性和有效性,一项与吸入妥布霉素的活性比较试验,一项根除研究,一项洋葱伯克霍尔德菌患者研究,以及一项评估持续交替抗生素治疗的研究。最后,讨论了五个正在进行的临床试验。综上所述,研究表明,吸入氨曲南对于根除新获得的铜绿假单胞菌和囊性纤维化患者慢性支气管内感染是一种安全有效的抗菌治疗方法。
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引用次数: 9
Cystic Fibrosis Transmembrane Conductance Regulator Genotype, Not Circulating Catecholamines, Influences Cardiovascular Function in Patients with Cystic Fibrosis. 囊性纤维化跨膜传导调节基因型,而不是循环儿茶酚胺,影响囊性纤维化患者的心血管功能。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-03-29 eCollection Date: 2019-01-01 DOI: 10.1177/1179548419835788
Alexander L Bisch, Courtney M Wheatley, Sarah E Baker, Elizabeth R Peitzman, Erik H Van Iterson, Theresa A Laguna, Wayne J Morgan, Eric M Snyder
<p><strong>Background: </strong>Cystic fibrosis (CF) is a genetic disease affecting multiple organ systems of the body and is characterized by mutation in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR). Previous work has shown that a single dose of aβ-agonist increases cardiac output (Q) and stroke volume (SV) and decreases systemic vascular resistance (SVR) in healthy subjects. This effect is attenuated in patients with CF; however, the mechanism is unknown. Potential explanations for this decreased cardiovascular response to a β-agonist in CF include inherent cardiovascular deficits secondary to the CFTR mutation, receptor desensitization from prolonged β-agonist use as part of clinical care, or inhibited drug delivery to the bloodstream due to mucus buildup in the lungs. This study sought to determine the effects of endogenous epinephrine (EPI) and norepinephrine (NE) on cardiovascular function in CF and to evaluate the relationship between cardiovascular function and CFTR F508del mutation.</p><p><strong>Methods: </strong>A total of 19 patients with CF and 31 healthy control subjects completed an assessment of Q (C<sub>2</sub>H<sub>2</sub> rebreathing), SV (calculated from Q and heart rate [HR]), Q and SV indexed to body surface area (BSA, QI, and SVI, respectively), SVR (through assessment of Q and mean arterial blood pressure [MAP]), and HR (from 12-lead electrocardiogram [ECG]) at rest along with plasma measures of EPI and NE. We compared subjects by variables of cardiovascular function relative to EPI and NE, and also based on genetic variants of the F508del mutation (homozygous deletion for F508del, heterozygous deletion for F508del, or no deletion of F508del).</p><p><strong>Results: </strong>Cystic fibrosis patients demonstrated significantly lower BSA (CF = 1.71 ± 0.05 m<sup>2</sup> vs healthy = 1.84 ± 0.04 m<sup>2</sup>, <i>P</i> = .03) and SVI (CF = 30.6 ± 2.5 mL/beat/m<sup>2</sup> vs healthy = 39.9 ± 2.5 mL/beat/m<sup>2</sup>, <i>P</i> = .02) when compared with healthy subjects. Cystic fibrosis patients also demonstrated lower Q (CF = 4.58 ± 0.36 L/min vs healthy = 5.71 ± 0.32 L/min, <i>P</i> = .03) and SV (CF = 54 ± 5.5 mL/beat vs healthy = 73.3 ± 4.5 mL/beat, <i>P</i> = .01), and a higher HR (CF = 93.2 ± 3.9 bpm vs healthy = 80.5 ± 2.7 bpm, <i>P</i> < .01) and SVR (CF = 2082 ± 156 dynes*s/cm<sup>-5</sup> vs healthy = 1616 ± 74 dynes*s/cm<sup>-5</sup>, <i>P</i> = .01) compared with healthy subjects. Furthermore, CF patients demonstrated a lower SV (<i>P</i> < .01) corrected for NE when compared with healthy subjects. No significant differences were seen in HR or Q relative to NE, or SVR relative to EPI. Differences were seen in SV (F<sub>(2,14)</sub> = 7.982, <i>P</i> < .01) and SV index (F<sub>(2,14)</sub> = 2.913, <i>P</i> = .08) when patients with CF were stratified according to F508del mutation (number of deletions).</p><p><strong>Conclusions: </strong>Individuals with CF have lower cardiac an
背景:囊性纤维化(CF)是一种影响人体多器官系统的遗传性疾病,其特征是囊性纤维化跨膜传导调节因子(CFTR)基因编码突变。先前的研究表明,在健康受试者中,单剂量的aβ激动剂可增加心输出量(Q)和脑卒中量(SV),并降低全身血管阻力(SVR)。这种效应在CF患者中减弱;然而,其机制尚不清楚。CF患者对β-激动剂的心血管反应降低的潜在解释包括CFTR突变引起的固有心血管缺陷,长期使用β-激动剂作为临床护理的一部分导致受体脱敏,或由于肺部粘液积聚而抑制药物向血液的输送。本研究旨在确定内源性肾上腺素(EPI)和去甲肾上腺素(NE)对CF患者心血管功能的影响,并评估CFTR F508del突变与心血管功能的关系。方法:19例CF患者和31例健康对照者分别完成静止时Q (C2H2再呼吸)、SV(由Q和心率[HR]计算)、Q和SV与体表面积(分别为BSA、QI和SVI)、SVR(通过Q和平均动脉血压[MAP]评估)和HR(12导联心电图[ECG])的评估,并测量血浆EPI和NE。我们通过与EPI和NE相关的心血管功能变量,以及F508del突变的遗传变异(F508del纯合缺失、F508del杂合缺失或F508del无缺失)对受试者进行了比较。结果:囊性纤维化患者BSA (CF = 1.71±0.05 m2 vs健康者= 1.84±0.04 m2, P = 0.03)和SVI (CF = 30.6±2.5 mL/beat/m2 vs健康者= 39.9±2.5 mL/beat/m2, P = 0.02)显著低于健康者。囊性纤维化患者还演示了低Q (CF = 4.58±0.36 L / min vs健康= 5.71±0.32 L / min, P = 03)和SV (CF = 54±5.5 mL /击败vs健康= 73.3±4.5毫升/打,P = . 01),和更高的人力资源(CF bpm vs健康= 80.5 = 93.2±3.9±2.7 bpm, P < . 01)和SVR (CF = 2082±156达因* s / cm-5 vs健康= 1616±74达因* s / cm-5, P = . 01)与健康受试者相比。此外,与健康受试者相比,CF患者经NE校正后的SV更低(P < 0.01)。HR或Q相对于NE, SVR相对于EPI无显著差异。CF患者按F508del突变(缺失数)分层时,SV (F(2,14) = 7.982, P < 0.01)和SV指数(F(2,14) = 2.913, P = 0.08)有差异。结论:CF患者静息时心脏和外周血流动力学参数较低。此外,这些结果表明心血管功能的损害可能是F508del CFTR基因型的结果,而不是受体脱敏或抑制药物传递的结果。
{"title":"Cystic Fibrosis Transmembrane Conductance Regulator Genotype, Not Circulating Catecholamines, Influences Cardiovascular Function in Patients with Cystic Fibrosis.","authors":"Alexander L Bisch,&nbsp;Courtney M Wheatley,&nbsp;Sarah E Baker,&nbsp;Elizabeth R Peitzman,&nbsp;Erik H Van Iterson,&nbsp;Theresa A Laguna,&nbsp;Wayne J Morgan,&nbsp;Eric M Snyder","doi":"10.1177/1179548419835788","DOIUrl":"https://doi.org/10.1177/1179548419835788","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Cystic fibrosis (CF) is a genetic disease affecting multiple organ systems of the body and is characterized by mutation in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR). Previous work has shown that a single dose of aβ-agonist increases cardiac output (Q) and stroke volume (SV) and decreases systemic vascular resistance (SVR) in healthy subjects. This effect is attenuated in patients with CF; however, the mechanism is unknown. Potential explanations for this decreased cardiovascular response to a β-agonist in CF include inherent cardiovascular deficits secondary to the CFTR mutation, receptor desensitization from prolonged β-agonist use as part of clinical care, or inhibited drug delivery to the bloodstream due to mucus buildup in the lungs. This study sought to determine the effects of endogenous epinephrine (EPI) and norepinephrine (NE) on cardiovascular function in CF and to evaluate the relationship between cardiovascular function and CFTR F508del mutation.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;A total of 19 patients with CF and 31 healthy control subjects completed an assessment of Q (C&lt;sub&gt;2&lt;/sub&gt;H&lt;sub&gt;2&lt;/sub&gt; rebreathing), SV (calculated from Q and heart rate [HR]), Q and SV indexed to body surface area (BSA, QI, and SVI, respectively), SVR (through assessment of Q and mean arterial blood pressure [MAP]), and HR (from 12-lead electrocardiogram [ECG]) at rest along with plasma measures of EPI and NE. We compared subjects by variables of cardiovascular function relative to EPI and NE, and also based on genetic variants of the F508del mutation (homozygous deletion for F508del, heterozygous deletion for F508del, or no deletion of F508del).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Cystic fibrosis patients demonstrated significantly lower BSA (CF = 1.71 ± 0.05 m&lt;sup&gt;2&lt;/sup&gt; vs healthy = 1.84 ± 0.04 m&lt;sup&gt;2&lt;/sup&gt;, &lt;i&gt;P&lt;/i&gt; = .03) and SVI (CF = 30.6 ± 2.5 mL/beat/m&lt;sup&gt;2&lt;/sup&gt; vs healthy = 39.9 ± 2.5 mL/beat/m&lt;sup&gt;2&lt;/sup&gt;, &lt;i&gt;P&lt;/i&gt; = .02) when compared with healthy subjects. Cystic fibrosis patients also demonstrated lower Q (CF = 4.58 ± 0.36 L/min vs healthy = 5.71 ± 0.32 L/min, &lt;i&gt;P&lt;/i&gt; = .03) and SV (CF = 54 ± 5.5 mL/beat vs healthy = 73.3 ± 4.5 mL/beat, &lt;i&gt;P&lt;/i&gt; = .01), and a higher HR (CF = 93.2 ± 3.9 bpm vs healthy = 80.5 ± 2.7 bpm, &lt;i&gt;P&lt;/i&gt; &lt; .01) and SVR (CF = 2082 ± 156 dynes*s/cm&lt;sup&gt;-5&lt;/sup&gt; vs healthy = 1616 ± 74 dynes*s/cm&lt;sup&gt;-5&lt;/sup&gt;, &lt;i&gt;P&lt;/i&gt; = .01) compared with healthy subjects. Furthermore, CF patients demonstrated a lower SV (&lt;i&gt;P&lt;/i&gt; &lt; .01) corrected for NE when compared with healthy subjects. No significant differences were seen in HR or Q relative to NE, or SVR relative to EPI. Differences were seen in SV (F&lt;sub&gt;(2,14)&lt;/sub&gt; = 7.982, &lt;i&gt;P&lt;/i&gt; &lt; .01) and SV index (F&lt;sub&gt;(2,14)&lt;/sub&gt; = 2.913, &lt;i&gt;P&lt;/i&gt; = .08) when patients with CF were stratified according to F508del mutation (number of deletions).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Individuals with CF have lower cardiac an","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"13 ","pages":"1179548419835788"},"PeriodicalIF":2.0,"publicationDate":"2019-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548419835788","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37291239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
Retrospective Analysis of Medication Utilization and Clinical Outcomes in Patients With Idiopathic Pulmonary Fibrosis Treated With Nintedanib or Pirfenidone. 尼达尼布或吡非尼酮治疗特发性肺纤维化患者的药物使用和临床结果回顾性分析。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-03-12 eCollection Date: 2019-01-01 DOI: 10.1177/1179548419834922
Anastasia Y Ipatova, Pamela H Koerner, Richard T Miller, Francis Staskon, Melanie Radi

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease which results in thickening and scarring of the interstitial tissue. As the only 2 Food and Drug Administration (FDA)-approved medications on the market, it is valuable to compare the impact of nintedanib and pirfenidone on clinical outcomes. Records of patients who started nintedanib or pirfenidone between calendar years 2015 and 2016 at a national specialty pharmacy were retrospectively reviewed. Data collection was derived from patient management applications and statistical data analysis was completed in SAS (SAS Institute Inc®). The nintedanib population contained 2605 patients and of the population completing clinical assessment surveys (n = 1343), 46% of respondents (n = 612) reported no adverse events, with the remaining 54% reporting at least 1 adverse event. Average proportion of days covered (PDC) was 84.2% (SD = 17.0). Average final monthly copay for this group was $235. The pirfenidone population had 1322 patients, and of the surveyed population (n = 764), 58% of respondents (n = 445) reported no adverse events, with the remaining 42% reporting at least 1 adverse event. Average PDC was 83.4% (SD = 17.3). Average final monthly copay for this group was $339. Outcomes in the studied IPF population were similar for nintedanib and pirfenidone.

特发性肺纤维化(IPF)是一种慢性进行性肺疾病,导致间质组织增厚和瘢痕形成。作为市场上仅有的两种FDA批准的药物,比较尼达尼布和吡非尼酮对临床结果的影响是有价值的。回顾性回顾了2015年至2016年在国家专业药房开始使用尼达尼布或吡非尼酮的患者记录。数据收集来自患者管理应用程序,统计数据分析在SAS (SAS Institute Inc®)中完成。尼达尼布人群包含2605名患者,在完成临床评估调查的人群中(n = 1343), 46%的受访者(n = 612)报告没有不良事件,其余54%报告至少1个不良事件。平均覆盖天数比例(PDC)为84.2% (SD = 17.0)。这一群体的平均每月共付额为235美元。吡非尼酮人群有1322例患者,在调查人群(n = 764)中,58%的受访者(n = 445)报告没有不良事件,其余42%报告至少1次不良事件。平均PDC值为83.4% (SD = 17.3)。这一群体的平均每月共付额为339美元。在研究的IPF人群中,尼达尼布和吡非尼酮的结果相似。
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引用次数: 7
An Unusual Mass of Posterior Mediastinum: A Case of Retrotracheal Parathyroid Adenoma Presenting With Primary Hyperparathyroidism. 后纵隔异常肿块:气管后甲状旁腺腺瘤1例,表现为原发性甲状旁腺功能亢进。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2018-11-16 eCollection Date: 2018-01-01 DOI: 10.1177/1179548418811840
Sani Rabiou, Boubacar Efared, Sani Aminou, Hicham Harmouchi, Kassim Sidibé, Marouane Lakranbi, Yassine Ouadnouni, Mohamed Smahi

Although parathyroid ectopy in the mediastinum has been the subject of several publications, its location in the posterior mediastinum is very rarely reported. We report a case of a 69-year-old patient who presented with clinical symptoms of malignant hypercalcemia due to a retrotracheal mediastinal parathyroid adenoma. The surgical excision leads to a quick normalisation of the phosphocalcic balance with improvement of the clinical symptoms. Ectopic hypersecreting parathyroid adenoma with life-threatening hypercalcemia should prompt radiological assessment and appropriate surgical management to prevent further clinical complications.

虽然甲状旁腺异位在纵隔已经是一些出版物的主题,它的位置在纵隔后是非常罕见的报道。我们报告一个69岁的病人,因气管后纵隔甲状旁腺瘤而出现恶性高钙血症的临床症状。手术切除后,随着临床症状的改善,磷钙平衡迅速恢复正常。异位高分泌性甲状旁腺腺瘤伴危及生命的高钙血症应及时进行放射学评估和适当的手术处理,以防止进一步的临床并发症。
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引用次数: 4
Obesity and Weaning from Mechanical Ventilation-An Exploratory Study. 肥胖与机械通气脱机的探索性研究。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2018-09-18 eCollection Date: 2018-01-01 DOI: 10.1177/1179548418801004
Ogugua Ndili Obi, Mark Mazer, Charles Bangley, Zuheir Kassabo, Khalid Saadah, Wayne Trainor, Kenneth Stephens, Patricia L Rice, Robert Shaw

Introduction: Obesity is associated with increased risk of hypercapnic respiratory failure, prolonged duration on mechanical ventilation, and extended weaning periods.

Objective: Pilot study to determine whether morbidly obese adult tracheotomized subjects (body mass index [BMI] ⩾ 40) can be more efficiently weaned from the ventilator by optimizing their positive end-expiratory pressure (PEEP) using either an esophageal balloon or the best achieved static effective compliance.

Methods: We randomly assigned 25 morbidly obese adult tracheotomized subjects (median [interquartile range] BMI 53.4 [26.4]; range 40.4-113.8) to 1 of 2 methods of setting PEEP; using either titration guided by esophageal balloon to overcome negative transpulmonary pressure (Ptp) (goal Ptp 0-5 cmH2O) (ESO group) or titration to maximize static effective lung compliance (Cstat group). Our outcomes of interest were number of subjects weaned by day 30 and time to wean.

Results: At day 30, there was no significant difference in percentage of subjects weaned. 8/13 subjects (62%) in the ESO Group were weaned vs. 9/12(75%) in the Cstat Group (P = 0.67). Among the 17 subjects who weaned, median time to ventilator liberation was significantly shorter in the ESO group: 3.5 days vs Cstat group 14 days (P = .01). Optimal PEEP in the ESO and Cstat groups was similar (ESO mean ± SD = 26.5 ± 5.7 cmH2O and Cstat 24.2 ± 7 cmH2O (P = .38).

Conclusions: Optimization of PEEP using esophageal balloon to achieve positive transpulmonary pressure did not change the proportion of patients weaned. Among patients who weaned, use of the esophageal balloon resulted in faster liberation from mechanical ventilation. There were no adverse consequences of the high PEEP (mean 25.4; range 13-37 cmH2O) used in our study. The study was approved by the Institutional Review Board at our institution (UMCIRB#10-0343) and registered with clinicaltrials.gov (NCT02323009).

肥胖与高碳酸血症性呼吸衰竭的风险增加、机械通气时间延长和脱机时间延长有关。目的:初步研究确定病态肥胖成人气管切开术受试者(体重指数[BMI]大于或等于40)是否可以通过使用食管球囊或最佳实现的静态有效依从性优化其呼气末正压(PEEP)来更有效地脱离呼吸机。方法:我们随机分配25名病态肥胖成人气管切开术受试者(中位数[四分位数间距]BMI为53.4 [26.4];范围40.4-113.8)至2种设置PEEP方法中的一种;使用食管球囊引导下的滴定来克服负肺压(Ptp)(目标Ptp 0-5 cmH2O) (ESO组)或滴定以最大化静态有效肺顺应性(Cstat组)。我们感兴趣的结果是在第30天断奶的受试者数量和断奶时间。结果:在第30天,断奶的受试者百分比无显著差异。ESO组有8/13例(62%)断奶,而Cstat组有9/12例(75%)断奶(P = 0.67)。在17名受试者中,ESO组的中位呼吸机解放时间显著缩短:3.5天,而Cstat组为14天(P = 0.01)。ESO组和Cstat组的最佳PEEP相似(ESO平均值±SD = 26.5±5.7 cmH2O, Cstat为24.2±7 cmH2O (P = 0.38)。结论:优化食管球囊实现正肺压的PEEP并没有改变断奶患者的比例。在断奶的患者中,使用食管球囊可以更快地从机械通气中解脱出来。高PEEP无不良后果(平均25.4;范围13-37 cmH2O)在我们的研究中使用。该研究获得了我们机构审查委员会(UMCIRB#10-0343)的批准,并在clinicaltrials.gov上注册(NCT02323009)。
{"title":"Obesity and Weaning from Mechanical Ventilation-An Exploratory Study.","authors":"Ogugua Ndili Obi,&nbsp;Mark Mazer,&nbsp;Charles Bangley,&nbsp;Zuheir Kassabo,&nbsp;Khalid Saadah,&nbsp;Wayne Trainor,&nbsp;Kenneth Stephens,&nbsp;Patricia L Rice,&nbsp;Robert Shaw","doi":"10.1177/1179548418801004","DOIUrl":"https://doi.org/10.1177/1179548418801004","url":null,"abstract":"<p><strong>Introduction: </strong>Obesity is associated with increased risk of hypercapnic respiratory failure, prolonged duration on mechanical ventilation, and extended weaning periods.</p><p><strong>Objective: </strong>Pilot study to determine whether morbidly obese adult tracheotomized subjects (body mass index [BMI] ⩾ 40) can be more efficiently weaned from the ventilator by optimizing their positive end-expiratory pressure (PEEP) using either an esophageal balloon or the best achieved static effective compliance.</p><p><strong>Methods: </strong>We randomly assigned 25 morbidly obese adult tracheotomized subjects (median [interquartile range] BMI 53.4 [26.4]; range 40.4-113.8) to 1 of 2 methods of setting PEEP; using either titration guided by esophageal balloon to overcome negative transpulmonary pressure (Ptp) (goal Ptp 0-5 cmH<sub>2</sub>O) (ESO group) or titration to maximize static effective lung compliance (Cstat group). Our outcomes of interest were number of subjects weaned by day 30 and time to wean.</p><p><strong>Results: </strong>At day 30, there was no significant difference in percentage of subjects weaned. 8/13 subjects (62%) in the ESO Group were weaned vs. 9/12(75%) in the Cstat Group (<i>P</i> = 0.67). Among the 17 subjects who weaned, median time to ventilator liberation was significantly shorter in the ESO group: 3.5 days vs Cstat group 14 days (<i>P</i> = .01). Optimal PEEP in the ESO and Cstat groups was similar (ESO mean ± SD = 26.5 ± 5.7 cmH<sub>2</sub>O and Cstat 24.2 ± 7 cmH<sub>2</sub>O (<i>P</i> = .38).</p><p><strong>Conclusions: </strong>Optimization of PEEP using esophageal balloon to achieve positive transpulmonary pressure did not change the proportion of patients weaned. Among patients who weaned, use of the esophageal balloon resulted in faster liberation from mechanical ventilation. There were no adverse consequences of the high PEEP (mean 25.4; range 13-37 cmH<sub>2</sub>O) used in our study. The study was approved by the Institutional Review Board at our institution (UMCIRB#10-0343) and registered with clinicaltrials.gov (NCT02323009).</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"12 ","pages":"1179548418801004"},"PeriodicalIF":2.0,"publicationDate":"2018-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548418801004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36518325","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 10
The Effect of Exercise on Oxygen Content in Anemic Patients With Chronic Obstructive Pulmonary Disease. 运动对慢性阻塞性肺疾病贫血患者氧含量的影响。
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2018-08-30 eCollection Date: 2018-01-01 DOI: 10.1177/1179548418796483
Ahmet Sinan Copur, Hannan Dogar, Zhang Chao, Leandra Wallace, Kevin Henegar, Nashreen Anderson, Ashok Fulambarker

Background: Anemia is reported in one-third of the patients with chronic obstructive pulmonary disease (COPD). Anemia, by decreasing oxygen content, can be a contributing factor for hypoxemia. We determined to find whether anemia causes more prominent hypoxia by decreasing the total oxygen content after exercise in anemic patients with COPD.

Methods: Stable moderate-to-severe COPD patients with and without anemia were recruited. Arterial blood gas analyses were performed on room air before and after a 6-minute walking test (6MWT). Walking distance, oxygen saturation, and heart rate were recorded in each case before and after the 6MWT. Pulmonary function test measurements and other data were obtained from the chart. The mean and standard deviations were calculated for continuous variables. The independent t-test and Kruskal-Wallis test were performed for numerical covariate and univariate analyses. The paired t-test was used for the analyses of data before and after exercise.

Results: A total of 24 male patients were included in the study; 12 of which were anemic. The oxygen content was decreased in the anemic group (15.22 ± 1.28 vs 15.07 ± 1.22) after exercise, but it was not significant. In the non-anemic group, no oxygen content decrease was observed after exercise (18.83 ± 1.41 vs 18.9 ± 1.37). Interestingly, the Spo2, but not Sao2, was significantly lower after exercise in anemic patients with COPD (93.46% ± 5.06% vs 88.20% ± 6.35% before and after exercise, respectively).

Conclusions: Anemia does not cause more prominent hypoxemia after exercise in patients with COPD. However, the recorded Spo2 levels were significantly lower after exercise in the anemic patients with COPD.

背景:三分之一的慢性阻塞性肺疾病(COPD)患者存在贫血。由于含氧量减少,贫血可能是低氧血症的一个促成因素。我们决定通过降低COPD贫血患者运动后的总氧含量来发现贫血是否会引起更突出的缺氧。方法:招募伴有或不伴有贫血的稳定的中重度COPD患者。在6分钟步行试验(6MWT)前后对室内空气进行动脉血气分析。记录6MWT前后的步行距离、血氧饱和度、心率。肺功能测试测量和其他数据从图表中获得。计算连续变量的均值和标准差。对数值协变量和单变量分析进行独立t检验和Kruskal-Wallis检验。运动前后数据分析采用配对t检验。结果:共纳入24例男性患者;其中12个是贫血的。贫血组运动后氧含量降低(15.22±1.28 vs 15.07±1.22),但差异不显著。非贫血组运动后氧含量无明显下降(18.83±1.41 vs 18.9±1.37)。有趣的是,COPD贫血患者运动后Spo2明显降低(运动前后分别为93.46%±5.06%和88.20%±6.35%),Sao2无明显降低。结论:COPD患者运动后贫血并不会引起更明显的低氧血症。然而,COPD贫血患者运动后Spo2水平明显降低。
{"title":"The Effect of Exercise on Oxygen Content in Anemic Patients With Chronic Obstructive Pulmonary Disease.","authors":"Ahmet Sinan Copur,&nbsp;Hannan Dogar,&nbsp;Zhang Chao,&nbsp;Leandra Wallace,&nbsp;Kevin Henegar,&nbsp;Nashreen Anderson,&nbsp;Ashok Fulambarker","doi":"10.1177/1179548418796483","DOIUrl":"https://doi.org/10.1177/1179548418796483","url":null,"abstract":"<p><strong>Background: </strong>Anemia is reported in one-third of the patients with chronic obstructive pulmonary disease (COPD). Anemia, by decreasing oxygen content, can be a contributing factor for hypoxemia. We determined to find whether anemia causes more prominent hypoxia by decreasing the total oxygen content after exercise in anemic patients with COPD.</p><p><strong>Methods: </strong>Stable moderate-to-severe COPD patients with and without anemia were recruited. Arterial blood gas analyses were performed on room air before and after a 6-minute walking test (6MWT). Walking distance, oxygen saturation, and heart rate were recorded in each case before and after the 6MWT. Pulmonary function test measurements and other data were obtained from the chart. The mean and standard deviations were calculated for continuous variables. The independent <i>t</i>-test and Kruskal-Wallis test were performed for numerical covariate and univariate analyses. The paired <i>t</i>-test was used for the analyses of data before and after exercise.</p><p><strong>Results: </strong>A total of 24 male patients were included in the study; 12 of which were anemic. The oxygen content was decreased in the anemic group (15.22 ± 1.28 vs 15.07 ± 1.22) after exercise, but it was not significant. In the non-anemic group, no oxygen content decrease was observed after exercise (18.83 ± 1.41 vs 18.9 ± 1.37). Interestingly, the Spo<sub>2</sub>, but not Sao<sub>2</sub>, was significantly lower after exercise in anemic patients with COPD (93.46% ± 5.06% vs 88.20% ± 6.35% before and after exercise, respectively).</p><p><strong>Conclusions: </strong>Anemia does not cause more prominent hypoxemia after exercise in patients with COPD. However, the recorded Spo<sub>2</sub> levels were significantly lower after exercise in the anemic patients with COPD.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"12 ","pages":"1179548418796483"},"PeriodicalIF":2.0,"publicationDate":"2018-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548418796483","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36463120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Subclinical Left Ventricular Dysfunction is Influenced by Genotype Severity in Patients with Cystic Fibrosis. 基因型严重程度对囊性纤维化患者亚临床左心室功能障碍的影响
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2018-08-19 eCollection Date: 2018-01-01 DOI: 10.1177/1179548418794154
Michael Pallin, Dominic Keating, David M Kaye, Tom Kotsimbos, John W Wilson

Background and objective: Over 2000 genotypes in the cystic fibrosis (CF) gene have been described. These genotypic differences result in variable clinical manifestations of CF, with severity of disease dependent on CF transmembrane conductance (CFTR) protein function. CFTR is widely distributed in nucleated cells, including cardiac myocytes, but the effect of genotype on cardiac function is not known.

Methods: This retrospective review of echocardiographic data is from a single adult CF centre between 2000 and 2015. Patients were cohorted based on the functional classification of genotype. 'Severe' patients had both CF genes from functional classification groups 1-3; 'mild' patients had one or no gene from these groups, or in the event of the second gene being unknown were pancreatic sufficient.

Results: Genotype and echocardiography were recorded during the inclusion period in 100 patients, 79 of whom were classified as having severe genotypes. Although the severe group were younger they had a lower fractional shortening (33.66 ± 6.6 vs 36.9 ± 6.3, P < .05), left atrial area (14.9 ± 3.6 versus 18.0 ± 4.2 cm2; P < .01) and volume (39.9 ± 18.7 versus 51.0 ± 18.7 mL; P < .05) and showed a trend to lower left ventricular ejection fraction.

Conclusions: This study is the first to show that in CF, severity of genotype (functional classification) is associated with cardiac impairment. Patients with severe CF genotype and cardiac dysfunction should be identified to evaluate cardiac response to gene-modifying treatments prior to consideration for lung transplantation.

背景与目的:囊性纤维化(CF)基因有2000多种基因型。这些基因型差异导致CF的临床表现不同,疾病的严重程度取决于CF跨膜传导(CFTR)蛋白的功能。CFTR广泛分布于有核细胞,包括心肌细胞,但基因型对心功能的影响尚不清楚。方法:回顾性分析2000年至2015年间单个成人CF中心的超声心动图数据。根据基因型的功能分类对患者进行分组。“重度”患者具有功能分类组1-3的两种CF基因;“轻度”患者有一个或没有来自这些群体的基因,或者在第二个基因未知的情况下,胰腺足够。结果:100例患者在纳入期间记录了基因型和超声心动图,其中79例为重度基因型。重度组较年轻,缩短分数较低(33.66±6.6 vs 36.9±6.3,P < 0.05);结论:本研究首次表明,CF中基因型(功能分类)的严重程度与心脏损害相关。有严重CF基因型和心功能障碍的患者在考虑进行肺移植之前,应确定以评估心脏对基因修饰治疗的反应。
{"title":"Subclinical Left Ventricular Dysfunction is Influenced by Genotype Severity in Patients with Cystic Fibrosis.","authors":"Michael Pallin,&nbsp;Dominic Keating,&nbsp;David M Kaye,&nbsp;Tom Kotsimbos,&nbsp;John W Wilson","doi":"10.1177/1179548418794154","DOIUrl":"https://doi.org/10.1177/1179548418794154","url":null,"abstract":"<p><strong>Background and objective: </strong>Over 2000 genotypes in the cystic fibrosis (CF) gene have been described. These genotypic differences result in variable clinical manifestations of CF, with severity of disease dependent on CF transmembrane conductance (CFTR) protein function. CFTR is widely distributed in nucleated cells, including cardiac myocytes, but the effect of genotype on cardiac function is not known.</p><p><strong>Methods: </strong>This retrospective review of echocardiographic data is from a single adult CF centre between 2000 and 2015. Patients were cohorted based on the functional classification of genotype. 'Severe' patients had both CF genes from functional classification groups 1-3; 'mild' patients had one or no gene from these groups, or in the event of the second gene being unknown were pancreatic sufficient.</p><p><strong>Results: </strong>Genotype and echocardiography were recorded during the inclusion period in 100 patients, 79 of whom were classified as having severe genotypes. Although the severe group were younger they had a lower fractional shortening (33.66 ± 6.6 vs 36.9 ± 6.3, <i>P</i> < .05), left atrial area (14.9 ± 3.6 versus 18.0 ± 4.2 cm<sup>2</sup>; <i>P</i> < .01) and volume (39.9 ± 18.7 versus 51.0 ± 18.7 mL; <i>P</i> < .05) and showed a trend to lower left ventricular ejection fraction.</p><p><strong>Conclusions: </strong>This study is the first to show that in CF, severity of genotype (functional classification) is associated with cardiac impairment. Patients with severe CF genotype and cardiac dysfunction should be identified to evaluate cardiac response to gene-modifying treatments prior to consideration for lung transplantation.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"12 ","pages":"1179548418794154"},"PeriodicalIF":2.0,"publicationDate":"2018-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548418794154","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36431408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 9
期刊
Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine
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