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Histological Transformation to Small Cell Carcinoma of an Adenosquamous Carcinoma of the Lung With Epidermal Growth Factor Receptor Mutation in Exons 20 and 21 After Treatment With Erlotinib: Case Report 厄洛替尼治疗后表皮生长因子受体20和21外显子突变的肺腺鳞癌组织学转化为小细胞癌:病例报告
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-08-01 DOI: 10.1177/1179548419872993
L. Fernández-Trujillo, Laura Tapia, Marcela Vallejo, M. Aguirre, J. Lores, L. Sua
Lung carcinoma currently represents 1 of the leading causes of death from cancer worldwide and regionally. The molecular identification of sensitive mutations of targeted treatment have changed the strategies of pharmacologic management in non-small cell lung carcinoma. However, mechanisms of resistance have been described, among them the change of histological type to small cell carcinoma. We present the case of a 46-year-old male patient, non-smoker, with a clinical history of a mass in the upper lobe of the right lung and an initial histological diagnosis of adenosquamous carcinoma of the lung, with the presence of mutations for epidermal growth factor receptor (EGFR) in exons 20 (S768I) and 21 (L858R). He received treatment with tyrosine kinase inhibitor (Erlotinib) with good clinical and radiological response. However, 1 year after the start of the medication, he consulted for a progressive onset of constitutional symptoms and respiratory symptoms, with radiographic worsening and new biopsy with a diagnosis of adenosquamous carcinoma with the adenocarcinoma component transformed to small cell carcinoma, with persistence of EGFR mutation. We describe the clinical, radiological, and laboratory characteristics as well as the outcome of this case. To conclude, among the mechanisms of resistance described to the treatment with tyrosine kinase inhibitors in patients with carcinomas with mutated EGFR, the transformation to small cell carcinoma besides being infrequent is particular, requiring a different diagnostic and therapeutic approach.
肺癌目前是全世界和区域癌症死亡的主要原因之一。靶向治疗敏感突变的分子鉴定改变了非小细胞肺癌的药物治疗策略。然而,耐药机制已被描述,其中包括组织学类型向小细胞癌的转变。我们报告一例46岁男性患者,非吸烟者,有右肺上叶肿块的临床病史,最初的组织学诊断为肺腺鳞癌,表皮生长因子受体(EGFR)外显子20 (S768I)和21 (L858R)存在突变。他接受了酪氨酸激酶抑制剂(厄洛替尼)治疗,临床和放射学反应良好。然而,在开始用药1年后,他就诊时发现身体症状和呼吸系统症状进行性发作,影像学恶化,新的活检诊断为腺鳞癌,腺癌成分转化为小细胞癌,并持续存在EGFR突变。我们描述了临床,放射学和实验室的特点,以及这个病例的结果。综上所述,在EGFR突变的癌症患者对酪氨酸激酶抑制剂治疗的耐药机制中,向小细胞癌的转化除了罕见之外是特殊的,需要不同的诊断和治疗方法。
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引用次数: 1
Streptococcus pneumoniae as a Cause of Community-Acquired Pneumonia in Indian Adolescents and Adults: A Systematic Review and Meta-Analysis. 肺炎链球菌作为印度青少年和成人社区获得性肺炎的病因:系统综述和荟萃分析。
IF 1 Q4 RESPIRATORY SYSTEM Pub Date : 2019-07-31 eCollection Date: 2019-01-01 DOI: 10.1177/1179548419862790
Canna J Ghia, Raja Dhar, Parvaiz A Koul, Gautam Rambhad, Mark A Fletcher

Background: Streptococcus pneumoniae is one of the primary cause of community-acquired pneumonia (CAP) worldwide. However, scant data are available on the prevalence of etiological organisms for CAP in adolescent and adult Indian population.

Objective: We performed a systematic review and meta-analysis to determine the contribution of S. pneumoniae in the causation of CAP in Indian patients aged 12 years or above.

Methodology: We performed a systematic search of both indexed and non-indexed publications using PubMed, databases of National Institute of Science Communication and Information Resources (NISCAIR), Annotated Bibliography of Indian Medicine (ABIM), Google Scholar, and hand search including cross-references using key terms 'community acquired pneumonia AND India'. All studies, published between January 1990 and January 2017, that evaluated Indian patients aged above 12 years with a confirmed diagnosis of CAP were eligible for inclusion. Our search retrieved a total of 182 studies, of which only 17 and 12 qualified for inclusion in the systematic review of all etiological organisms, and meta-analysis of S. pneumonia, respectively.

Results: A total of 1435 patients met the inclusion criteria. The pooled proportion of patients with S. pneumoniae infection was 19% (95% confidence interval [CI]: 12%-26%; I2 = 94.5% where I2 represents heterogeneity, P < .01). Other major etiological agents are Mycoplasma pneumoniae (15.5% [1.1%-35.5%]), Klebsiella pneumoniae (10.5% [1.6%-24.0%]), and Legionella pneumophila (7.3% [2.5%-23.8%]).

Conclusions: Analysis found approximately a one-fifth proportion of adult Indian patients of CAP with S. pneumoniae infection, suggesting it as a leading organism for causing CAP compared with other etiological organisms.

背景:肺炎链球菌是世界范围内社区获得性肺炎(CAP)的主要病因之一。然而,关于青少年和成年印度人群中CAP病原菌的流行率,缺乏可用的数据。目的:我们进行了一项系统综述和荟萃分析,以确定肺炎链球菌在12岁印度患者CAP病因中的作用 年或以上。方法:我们使用PubMed、国家科学传播与信息资源研究所(NISCAIR)数据库、印度医学注释书目(ABIM)、谷歌学者对索引和非索引出版物进行了系统搜索,并使用关键词“社区获得性肺炎与印度”进行了手部搜索,包括交叉参考。1990年1月至2017年1月期间发表的所有研究都对12岁以上的印度患者进行了评估 确诊CAP的年数符合入选条件。我们的检索共检索到182项研究,其中分别只有17项和12项符合纳入所有病原微生物的系统综述和肺炎链球菌的荟萃分析。结果:共有1435名患者符合入选标准。肺炎链球菌感染患者的合并比例为19%(95%置信区间[CI]:12%-26%;I2 = 94.5%,其中I2表示异质性,P 肺炎支原体(15.5%[1.1%-35.5%])、肺炎克雷伯菌(10.5%[1.6%-24.0%])和嗜肺军团菌(7.3%[2.5%-23.8%])。
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引用次数: 0
Role of Deleterious Rare Alleles for Acute-Onset Diffuse Interstitial Lung Disease in Collagen Diseases 急性弥漫性间质性肺疾病中有害稀有等位基因的作用
IF 2 Q4 RESPIRATORY SYSTEM Pub Date : 2019-07-01 DOI: 10.1177/1179548419866443
H. Furukawa, S. Oka, K. Shimada, A. Hashimoto, A. Komiya, T. Matsui, S. Tohma
Objective: Acute-onset diffuse interstitial lung disease (AoDILD) includes acute exacerbation of interstitial lung disease (ILD), drug-induced ILD, and Pneumocystis pneumonia in collagen diseases patients. As AoDILD causes a poor prognosis in collagen disease patients, the pathogenesis of AoDILD should be investigated. Exome sequencing studies revealed that rare variants were detected to be causative in some diseases. Recently reported upregulated genes in acute exacerbation of idiopathic pulmonary fibrosis could provide candidate genes for restricted exome analysis of AoDILD in collagen disease. Here, we investigated rare variants in the coding and boundary regions of these candidate genes in AoDILD. Methods: Deleterious rare variants in the coding and boundary regions of the candidate genes were analyzed by exome sequencing and the deleterious rare allele frequencies in AoDILD were compared with those of controls. Results: A significant association was detected for deleterious rare alleles in NPL (P = .0044, Pc = .0399, odds ratio [OR] = 10.05, 95% confidence interval [CI] = 3.01-33.55). A deleterious rare allele frequency in the 9 candidate genes (P = .0011, OR = 7.17, 95% CI = 2.80-18.33) was also increased in AoDILD in multigene panel analysis. The Krebs von den Lungen–6 (KL-6) levels in AoDILD patients with deleterious rare alleles were tended to be lower than those without (P = .0168, Pc = .1509). Conclusions: The deleterious rare alleles in NPL were associated with AoDILD. In addition, the deleterious rare allele frequency in the 9 candidate genes was also increased in AoDILD. The deleterious rare alleles might contribute to the pathogenesis of AoDILD.
目的:急性发作弥漫性间质性肺疾病(AoDILD)包括胶原蛋白疾病患者间质性肺疾病(ILD)急性加重、药物性ILD和肺囊虫性肺炎。由于AoDILD在胶原蛋白疾病患者中预后较差,因此需要进一步研究AoDILD的发病机制。外显子组测序研究显示,在某些疾病中检测到罕见变异是致病的。最近报道的特发性肺纤维化急性加重中的上调基因可以为胶原疾病中AoDILD的限制性外显子组分析提供候选基因。在这里,我们研究了AoDILD中这些候选基因的编码和边界区域的罕见变异。方法:采用外显子组测序方法分析候选基因编码区和边界区有害稀有等位基因的变异,并与对照进行比较。结果:在NPL中检测到有害稀有等位基因显著相关(P =。0044, Pc =。0399,优势比[OR] = 10.05, 95%可信区间[CI] = 3.01-33.55)。9个候选基因的有害稀有等位基因频率(P =。0011, OR = 7.17, 95% CI = 2.80-18.33)在多基因面板分析中AoDILD也增加。携带有害稀有等位基因的AoDILD患者KL-6水平倾向于低于不携带有害稀有等位基因的患者(P =;0168, Pc = .1509)。结论:NPL中有害稀有等位基因与aodld相关。此外,9个候选基因的有害稀有等位基因频率也在AoDILD中增加。这些有害的稀有等位基因可能与aodld的发病机制有关。
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引用次数: 1
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。
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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
期刊
Clinical Medicine Insights-Circulatory Respiratory and Pulmonary Medicine
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