Pub Date : 2019-06-19eCollection Date: 2019-01-01DOI: 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.
{"title":"The Impact of a Newly Established Multidisciplinary Team on the Interventional Treatment of Patients With Emphysema.","authors":"Vasileios Kouritas, Richard Milton, Emmanouel Kefaloyannis, Kostas Papagiannopoulos, Allesandro Brunelli, Doytchin Dimov, Sishik Karthik, Andrew Hardy, Peter Tcherveniakov, Nilanjan Chaudhuri","doi":"10.1177/1179548419852063","DOIUrl":"https://doi.org/10.1177/1179548419852063","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Objectives: </strong>To investigate the impact of a newly established dedicated emphysema MDT meeting on the interventional treatment of such patients.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>More LVRS and less EBV insertions were performed in group A (<i>P </i>=<i> </i>.009). In group B, the interventions were performed sooner than in group A (<i>P </i>=<i> </i>.003). Postoperative overall morbidity and length of in-hospital stay were similar in the 2 groups (<i>P </i>=<i> </i>.918 and .758, respectively). Improvement of breathing ability was reported in more patients from group A (<i>P </i>=<i> </i>.012). In group B, the total number of re-interventions was higher (<i>P </i>=<i> </i>.001) and the time to re-intervention had the tendency to be less (<i>P </i>=<i> </i>.069). Survival was similar between the 2 groups (<i>P </i>=<i> </i>.884). Intervention without discussion at the MDT and EBV as initial intervention was an independent predictor of re-intervention.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"13 ","pages":"1179548419852063"},"PeriodicalIF":2.0,"publicationDate":"2019-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548419852063","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37385320","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}
Pub Date : 2019-05-30eCollection Date: 2019-01-01DOI: 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.
{"title":"An Exploration Into Knowledge, Attitudes, and Beliefs Towards Risky Health Behaviours in a Paediatric Cystic Fibrosis Population.","authors":"Rebecca Keyte, Helen Egan, Michail Mantzios","doi":"10.1177/1179548419849427","DOIUrl":"10.1177/1179548419849427","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"13 ","pages":"1179548419849427"},"PeriodicalIF":2.0,"publicationDate":"2019-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548419849427","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37338553","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}
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.
{"title":"Respiratory Fungal Diseases in Adult Patients With Cystic Fibrosis.","authors":"Emanuele Delfino, Filippo Del Puente, Federica Briano, Chiara Sepulcri, Daniele Roberto Giacobbe","doi":"10.1177/1179548419849939","DOIUrl":"https://doi.org/10.1177/1179548419849939","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"13 ","pages":"1179548419849939"},"PeriodicalIF":2.0,"publicationDate":"2019-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548419849939","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37339184","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}
Pub Date : 2019-04-12eCollection Date: 2019-01-01DOI: 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.
{"title":"Aztreonam Lysine Inhalation Solution in Cystic Fibrosis.","authors":"Elizabeth Claire Elson, Joel Mermis, Deepika Polineni, Christopher M Oermann","doi":"10.1177/1179548419842822","DOIUrl":"https://doi.org/10.1177/1179548419842822","url":null,"abstract":"<p><p>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 <i>Pseudomonas aeruginosa</i> (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 <i>Burkholderia cepacia</i>, 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 <i>P. aeruginosa</i> and long-term suppressive therapy of chronic endobronchial infection among people with cystic fibrosis.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"13 ","pages":"1179548419842822"},"PeriodicalIF":2.0,"publicationDate":"2019-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548419842822","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37180429","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}
Pub Date : 2019-03-29eCollection Date: 2019-01-01DOI: 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, Courtney M Wheatley, Sarah E Baker, Elizabeth R Peitzman, Erik H Van Iterson, Theresa A Laguna, Wayne J Morgan, Eric M Snyder","doi":"10.1177/1179548419835788","DOIUrl":"https://doi.org/10.1177/1179548419835788","url":null,"abstract":"<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","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}
Pub Date : 2019-03-12eCollection Date: 2019-01-01DOI: 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.
{"title":"Retrospective Analysis of Medication Utilization and Clinical Outcomes in Patients With Idiopathic Pulmonary Fibrosis Treated With Nintedanib or Pirfenidone.","authors":"Anastasia Y Ipatova, Pamela H Koerner, Richard T Miller, Francis Staskon, Melanie Radi","doi":"10.1177/1179548419834922","DOIUrl":"https://doi.org/10.1177/1179548419834922","url":null,"abstract":"<p><p>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<sup>®</sup>). 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.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"13 ","pages":"1179548419834922"},"PeriodicalIF":2.0,"publicationDate":"2019-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548419834922","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37071812","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}
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.
{"title":"An Unusual Mass of Posterior Mediastinum: A Case of Retrotracheal Parathyroid Adenoma Presenting With Primary Hyperparathyroidism.","authors":"Sani Rabiou, Boubacar Efared, Sani Aminou, Hicham Harmouchi, Kassim Sidibé, Marouane Lakranbi, Yassine Ouadnouni, Mohamed Smahi","doi":"10.1177/1179548418811840","DOIUrl":"https://doi.org/10.1177/1179548418811840","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":44269,"journal":{"name":"Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine","volume":"12 ","pages":"1179548418811840"},"PeriodicalIF":2.0,"publicationDate":"2018-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1179548418811840","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36719677","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}
Pub Date : 2018-09-18eCollection Date: 2018-01-01DOI: 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).
{"title":"Obesity and Weaning from Mechanical Ventilation-An Exploratory Study.","authors":"Ogugua Ndili Obi, Mark Mazer, Charles Bangley, Zuheir Kassabo, Khalid Saadah, Wayne Trainor, Kenneth Stephens, Patricia L Rice, 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}
Pub Date : 2018-08-30eCollection Date: 2018-01-01DOI: 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, Hannan Dogar, Zhang Chao, Leandra Wallace, Kevin Henegar, Nashreen Anderson, 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}
Pub Date : 2018-08-19eCollection Date: 2018-01-01DOI: 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, Dominic Keating, David M Kaye, Tom Kotsimbos, 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}