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Thinking outside the box: a review of gastrointestinal symptoms and complications in cystic fibrosis. 跳出框框思考:囊性纤维化胃肠道症状和并发症综述。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-07-01 Epub Date: 2023-06-26 DOI: 10.1080/17476348.2023.2228194
Alexander Yule, Darren Sills, Sherie Smith, Robin Spiller, Alan R Smyth

Introduction: Gastrointestinal (GI)-related symptoms, complications, and comorbidities in cystic fibrosis (CF) are common and research to reduce their burden is a priority for the CF community. To enable future research, this review aimed to summarize the range of GI symptoms, complications and comorbidities seen in CF, the underlying pathophysiology, and treatments.

Areas covered: This was a rapid systematic review undertaken using the recommendations from the Cochrane Rapid Reviews Methods Group. We searched databases including PubMed, Embase, Medline and the Cochrane database and identified those studies reporting GI-related symptoms, complications, or comorbidities in CF or their treatment. Our searches identified 2,930 studies and a total 119 studies met our inclusion criteria. Where a prevalence could be determined, GI symptoms were reported in 33.7% of study participants. The range of symptoms reported was broad and the highest median prevalence included flatulence (43.5%), bloating and abdominal distension (36%), and fatty stool (36%). Meconium ileus was reported in 12% and distal intestinal obstruction syndrome in 8.5.

Expert opinion: GI-related symptoms, complications, and comorbidities in CF are common. More consistent characterization and recording of these symptoms in clinical studies may help achieve the priority of reducing the burden of GI disease in CF.

引言:囊性纤维化(CF)的胃肠道(GI)相关症状、并发症和合并症很常见,减少其负担的研究是CF社区的优先事项。为了便于未来的研究,这篇综述旨在总结CF中出现的胃肠道症状、并发症和合并症的范围、潜在的病理生理学和治疗方法。涵盖的领域:这是一项使用Cochrane快速审查方法小组的建议进行的快速系统审查。我们搜索了包括PubMed、Embase、Medline和Cochrane数据库在内的数据库,并确定了那些报告CF或其治疗中胃肠道相关症状、并发症或合并症的研究。我们的检索确定了2930项研究,共有119项研究符合我们的纳入标准。在可以确定患病率的情况下,33.7%的研究参与者报告了胃肠道症状。报告的症状范围很广,最高的中位患病率包括胀气(43.5%)、腹胀和腹胀(36%)以及脂肪粪便(36%)。12%的患者报告了胎粪性肠梗阻,8.5.报告了远端肠梗阻综合征。专家意见:CF中常见的胃肠道相关症状、并发症和合并症。在临床研究中对这些症状进行更一致的表征和记录可能有助于优先减轻CF中胃肠道疾病的负担。
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引用次数: 0
Assessing accuracy of testing and diagnosis in cystic fibrosis. 评估囊性纤维化检测和诊断的准确性。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-05-01 DOI: 10.1080/17476348.2023.2213438
Malina Barillaro, Tanja Gonska

Introduction: Next to evaluating for defects in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, diagnostic guidelines for cystic fibrosis (CF) include CFTR function tests. The primarily used sweat test and genetics generally produce straightforward CF diagnoses. However, a widened CF disease spectrum and large number of CFTR gene variants with unknown or varying clinical consequences shift reliance on CFTR functional tests to assess for CF or CFTR-related disease. Recently, CFTR functional tests are used to record efficiency of CFTR modulator drugs.

Areas covered: This review provides background and accuracy of the currently used CFTR functional tests, including the sweat test, nasal potential difference (NPD), and intestinal current measurements (ICM). We summarize published evidence addressing technical and biological reasons for test variability and test result in relation to CF-associated symptoms.

Expert opinion: The CFTR functional tests demonstrate high accuracy despite biological and technical variability. Data is scarce for ICM. Each test identifies CF from non-CF but show lower accuracy for individuals not fitting the classic CF diagnostic criteria. Adherence to standardized protocols is critical to improve test accuracy across different centers. Lastly, instead of relying on the single test results, diagnostic assessment should be based on integrating multiple functional and genetic test results.

简介:除了评估囊性纤维化跨膜传导调节因子(CFTR)基因缺陷外,囊性纤维化(CF)的诊断指南还包括CFTR功能测试。主要使用的汗液测试和遗传学通常可以直接诊断CF。然而,CF疾病范围的扩大和大量临床后果未知或变化的CFTR基因变异,使得评估CF或CFTR相关疾病依赖于CFTR功能测试。近年来,CFTR功能试验被用于记录CFTR调节药物的疗效。涵盖领域:本综述提供了目前使用的CFTR功能测试的背景和准确性,包括汗液测试、鼻电位差(NPD)和肠电流测量(ICM)。我们总结了与cf相关症状相关的检测变异和检测结果的技术和生物学原因的已发表证据。专家意见:尽管存在生物和技术差异,CFTR功能测试仍显示出较高的准确性。ICM的数据很少。每个测试都能识别CF和非CF,但对于不符合经典CF诊断标准的个体,准确率较低。遵守标准化协议对于提高不同中心的测试准确性至关重要。最后,诊断评估不应依赖单一的检测结果,而应基于多种功能和基因检测结果的综合。
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引用次数: 0
Management of pulmonary arterial hypertension during pregnancy. 妊娠期肺动脉高压的处理。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-05-01 DOI: 10.1080/17476348.2023.2210838
Kaushiga Krishnathasan, Andrew Constantine, Isma Rafiq, Ana Barradas Pires, Hannah Douglas, Laura C Price, Konstantinos Dimopoulos

Introduction: Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure >20 mmHg and pulmonary vascular resistance >2 Wood Units (WU) on right-heart catheterization. Pregnancy is generally contraindicated in PAH, it is associated with high maternal mortality. Despite current recommendations, the number of women with PAH wishing to become pregnant is increasing. Specialist care is essential for preconception counseling, and the management of pregnancy and delivery in such patients.

Areas covered: We cover the physiology of pregnancy, and its effects on the cardiovascular system in PAH. We also discuss optimal management based on available evidence and guidance.

Expert opinion: Pregnancy should be avoided in most patients with PAH. Counseling on appropriate contraception should be offered routinely. Education of women with childbearing potential is essential and should start at the time of diagnosis of PAH, or the time of transition from pediatric to adult services in patients developing PAH in childhood. Women wishing to become pregnant should receive individualized risk assessment and optimization of PAH therapies via a dedicated specialist pre-pregnancy counseling service, to minimize risk and improve outcomes. Pregnant PAH patients should receive expert multidisciplinary management in a PH center, including close monitoring and early initiation of therapies.

肺动脉高压(PAH)定义为右心导管时平均肺动脉压>20 mmHg,肺血管阻力>2 Wood Units (WU)。妊娠一般禁忌症在多环芳烃,它是与高产妇死亡率。尽管有目前的建议,但希望怀孕的多环芳烃妇女人数正在增加。专科护理是必不可少的孕前咨询,并在这类患者的妊娠和分娩管理。涵盖领域:我们涵盖了怀孕的生理,以及它对多环芳烃的心血管系统的影响。我们还讨论了基于现有证据和指导的最佳管理。专家意见:多数PAH患者应避免妊娠。应定期提供适当避孕的咨询。对有生育潜力的妇女进行教育是必要的,应该在诊断出多环芳烃时开始,或者在儿童期多环芳烃患者从儿科服务过渡到成人服务时开始。希望怀孕的妇女应该通过专门的孕前咨询服务接受个性化的风险评估和PAH治疗的优化,以最大限度地降低风险并改善结果。妊娠PAH患者应在PH中心接受专家多学科管理,包括密切监测和早期开始治疗。
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引用次数: 0
Convalescent plasma therapy in COVID-19: Unravelling the data using the principles of antibody therapy. COVID-19恢复期血浆治疗:利用抗体治疗原理揭示数据
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-05-01 DOI: 10.1080/17476348.2023.2208349
Arturo Casadevall, Michael J Joyner, Liise-Anne Pirofski, Jonathon W Senefeld, Shmuel Shoham, David Sullivan, Nigel Paneth, Daniele Focosi

Introduction: When the COVID-19 pandemic struck no specific therapies were available and many turned to COVID-19 convalescent plasma (CCP), a form of antibody therapy. The literature provides mixed evidence for CCP efficacy.

Areas covered: PubMed was searched using the words COVID-19 and convalescent plasma and individual study designs were evaluated for adherence to the three principles of antibody therapy, i.e. that plasma 1) contain specific antibody; 2) have enough specific antibody to mediate a biological effect; and 3) be administered early in the course of disease. Using this approach, a diverse and seemingly contradictory collection of clinical findings was distilled into a consistent picture whereby CCP was effective when used according to the above principles of antibody therapy. In addition, CCP therapy in immunocompromised patients is useful at any time in the course of disease.

Expert opinion: CCP is safe and effective when used appropriately. Today, most of humanity has some immunity to SARS-CoV-2 from vaccines and infection, which has lessened the need for CCP in the general population. However, COVID-19 in immunocompromised patients is a major therapeutic challenge, and with the deauthorization of all SARS-CoV-2-spike protein-directed monoclonal antibodies, CCP is the only antibody therapy available for this population.

当COVID-19大流行袭来时,没有特定的治疗方法,许多人转向COVID-19恢复期血浆(CCP),这是一种抗体治疗形式。文献对CCP的疗效提供了不同的证据。涵盖领域:使用COVID-19和恢复期血浆搜索PubMed,并评估个体研究设计是否符合抗体治疗的三项原则,即血浆1)含有特异性抗体;2)有足够的特异性抗体介导生物效应;3)在病程早期给药。使用这种方法,多种多样且看似矛盾的临床发现被提炼成一致的图像,即根据上述抗体治疗原则使用CCP是有效的。此外,免疫功能低下患者的CCP治疗在病程中的任何时候都是有用的。专家意见:如果使用得当,CCP是安全有效的。今天,大多数人通过疫苗和感染对SARS-CoV-2有一定的免疫力,这减少了普通人群对CCP的需求。然而,在免疫功能低下的患者中,COVID-19是一个主要的治疗挑战,随着所有sars - cov -2刺突蛋白定向单克隆抗体的取消授权,CCP是这一人群唯一可用的抗体治疗。
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引用次数: 4
New paradigm in the treatment of myositis-associated interstitial lung disease. 肌炎相关间质性肺疾病治疗的新模式。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-05-01 DOI: 10.1080/17476348.2023.2215433
Takahisa Gono, Masataka Kuwana

Introduction: Interstitial lung disease (ILD) is the leading cause of mortality in idiopathic inflammatory myopathies or myositis. Clinical characteristics, including the course of ILD, rate of progression, radiological and pathohistological morphologies, extent and distribution of inflammation and fibrosis, responses to treatment, recurrence rate, and prognosis, are highly variable among myositis patients. A standard practice for ILD management in myositis patients has not yet been established.

Areas covered: Recent studies have demonstrated the stratification of patients with myositis-associated ILD into more homogeneous groups based on the disease behavior and myositis-specific autoantibody (MSA) profile, leading to better prognoses and prevention of the burden of organ damage. This review introduces a new paradigm in the management of myositis-associated ILD based on research findings from relevant literature selected by a search of PubMed as of January 2023, as well as expert opinions.

Expert opinion: Managing strategies for myositis-associated ILD are being established to stratify patients based on the severity of ILD and the prediction of prognosis based on the disease behavior and MSA profile. The development of a precision medicine treatment approach will provide benefits to all relevant communities.

简介:间质性肺疾病(ILD)是特发性炎性肌病或肌炎死亡的主要原因。临床特征,包括ILD的病程、进展速度、放射学和病理组织学形态、炎症和纤维化的程度和分布、对治疗的反应、复发率和预后,在肌炎患者中是高度不同的。肌炎患者ILD管理的标准实践尚未建立。涵盖领域:最近的研究表明,基于疾病行为和肌炎特异性自身抗体(MSA)谱,将肌炎相关ILD患者分层为更均匀的组,可导致更好的预后和预防器官损害负担。这篇综述介绍了一种管理肌炎相关ILD的新模式,该模式基于PubMed截至2023年1月检索的相关文献的研究结果以及专家意见。专家意见:正在制定肌炎相关ILD的管理策略,根据ILD的严重程度对患者进行分层,并根据疾病行为和MSA概况预测预后。精准医学治疗方法的发展将为所有相关社区带来好处。
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引用次数: 0
Understanding persistent breathlessness: impact on patients and optimal approaches to symptomatic reduction - an overview. 理解持续性呼吸困难:对患者的影响和减轻症状的最佳方法-概述。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-05-01 DOI: 10.1080/17476348.2023.2208348
Diana H Ferreira, Slavica Kochovska, Vanessa N Brunelli, David C Currow

Introduction: Persistent breathlessness (breathlessness persisting despite optimal treatment for the underlying condition and resulting in disability) is a prevalent syndrome associated with chronic and life-limiting conditions. Improving the clinical recognition and assessment of persistent breathlessness is essential to ensure people are provided with the best treatment for optimal symptom control.

Areas covered: This overview focuses on the impact of persistent breathlessness on patients, carers and the health system. It highlights the importance of identifying persistent breathlessness in clinical consultations, suggests steps to recognize this syndrome and discusses the evidence for non-pharmacological and pharmacological treatments in this context. Future research directions are also suggested.

Expert opinion: Persistent breathlessness is often invisible because 1) people may not engage with the health system and 2) both clinicians and patients are reluctant to discuss breathlessness in clinical consultations. Improving the recognition and assessment of this syndrome is critical to facilitate meaningful conversations between patients and clinicians and ensure patient-centered care. Non-pharmacological strategies are key to improving symptom management and health outcomes. Regular, low-dose, sustained-release morphine may help further reduce breathlessness in people who remain symptomatic despite disease-specific and non-pharmacological therapies.

简介:持续性呼吸困难(尽管对基础疾病进行了最佳治疗,但仍持续呼吸困难并导致残疾)是一种与慢性和限制性疾病相关的普遍综合征。改善对持续性呼吸困难的临床识别和评估对于确保为患者提供最佳治疗以实现最佳症状控制至关重要。涵盖领域:本概述侧重于持续性呼吸困难对患者、护理人员和卫生系统的影响。它强调了在临床咨询中识别持续性呼吸困难的重要性,提出了识别这种综合征的步骤,并讨论了在这种情况下非药物和药物治疗的证据。并提出了今后的研究方向。专家意见:持续的呼吸困难通常是看不见的,因为1)人们可能不参与卫生系统,2)临床医生和患者都不愿意在临床咨询中讨论呼吸困难。提高对这种综合征的认识和评估对于促进患者和临床医生之间有意义的对话以及确保以患者为中心的护理至关重要。非药物策略是改善症状管理和健康结果的关键。常规的、低剂量的、持续释放的吗啡可能有助于进一步减少那些尽管有疾病特异性和非药物治疗仍有症状的人的呼吸困难。
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引用次数: 0
Impact of aging on immune function in the pathogenesis of pulmonary diseases: potential for therapeutic targets. 衰老对肺部疾病发病机制中免疫功能的影响:潜在的治疗目标。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-05-01 Epub Date: 2023-04-26 DOI: 10.1080/17476348.2023.2205127
Sadiya Bi Shaikh, Chiara Goracci, Ariel Tjitropranoto, Irfan Rahman

Introduction: Several immunological alterations that occur during pulmonary diseases often mimic alterations observed in the aged lung. From the molecular perspective, pulmonary diseases and aging partake in familiar mechanisms associated with significant dysregulation of the immune systems. Here, we summarized the findings of how aging alters immunity to respiratory conditions to identify age-impacted pathways and mechanisms that contribute to the development of pulmonary diseases.

Areas covered: The current review examines the impact of age-related molecular alterations in the aged immune system during various lung diseases, such as COPD, IPF, Asthma, and alongside many others that could possibly improve on current therapeutic interventions. Moreover, our increased understanding of this phenomenon may play a primary role in shaping immunomodulatory strategies to boost outcomes in the elderly. Here, the authors present new insights into the context of lung-related diseases and describe the alterations in the functioning of immune cells during various pulmonary conditions altered with age.

Expert opinion: The expert opinion provided the concepts on how aging alters immunity during pulmonary conditions, and suggests the associated mechanisms during the development of lung diseases. As a result, it becomes important to comprehend the complex mechanism of aging in the immune lung system.

前言在肺部疾病期间发生的一些免疫学改变往往与在老年肺部观察到的改变相似。从分子角度来看,肺部疾病和衰老都与免疫系统严重失调有关。在此,我们总结了衰老如何改变呼吸系统疾病免疫力的研究结果,以确定受年龄影响的途径和机制,这些途径和机制有助于肺部疾病的发展:本综述探讨了在慢性阻塞性肺疾病、IPF、哮喘等各种肺部疾病中,与年龄相关的分子改变对老年免疫系统的影响,以及可能改善当前治疗干预措施的其他因素。此外,我们对这一现象的进一步了解可能会在制定免疫调节策略以提高老年人的治疗效果方面发挥重要作用。在此,作者提出了对肺部相关疾病的新见解,并描述了在各种肺部疾病中,免疫细胞的功能随着年龄的增长而发生的变化:专家意见:该专家意见提供了关于衰老如何在肺部疾病期间改变免疫力的概念,并提出了肺部疾病发生的相关机制。因此,理解肺部免疫系统衰老的复杂机制变得非常重要。
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引用次数: 0
Predicting the successful application of high-flow nasal oxygen cannula in patients with COVID-19 respiratory failure: a retrospective analysis. 预测高流量鼻吸氧插管在COVID-19呼吸衰竭患者中的成功应用:回顾性分析
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-04-01 DOI: 10.1080/17476348.2023.2199157
Ping-Tsung Yu, Chao-Hsien Chen, Chieh-Jen Wang, Kuan-Chih Kuo, Jou-Chun Wu, Hsin-Pei Chung, Yen-Ting Chen, Yen-Hsiang Tang, Wen-Kuei Chang, Chang-Yi Lin, Chien-Liang Wu
ABSTRACT Background The right time of high-flow nasal cannulas (HFNCs) application in COVID-19 patients with acute respiratory failure remains uncertain. Research design and methods In this retrospective study, COVID-19-infected adult patients with hypoxemic respiratory failure were enrolled. Their baseline epidemiological data and respiratory failure related parameters, including the Ventilation in COVID-19 Estimation (VICE), and the ratio of oxygen saturation (ROX index), were recorded. The primary outcome measured was the 28-day mortality. Results A total of 69 patients were enrolled. Fifty-four (78%) patients who intubated and received invasive mechanical ventilatory (MV) support on day 1 were enrolled in the MV group. The remaining fifteen (22%) patients received HFNC initially (HFNC group), in which, ten (66%) patients were not intubated during hospitalization were belong to HFNC-success group and five (33%) of these patients were intubated later due to disease progression were attributed to HFNC-failure group. Compared with those in the MV group, those in the HFNC group had a lower mortality rate (6.7% vs. 40.7%, p = 0.0138). There were no differences in baseline characteristics among the two groups; however, the HFNC group had a lower VICE score (0.105 [0.049–0.269] vs. 0.260 [0.126–0.693], p = 0.0092) and higher ROX index (5.3 [5.1–10.7] vs. 4.3 [3.9–4.9], p = 0.0007) than the MV group. The ROX index was higher in the HFNC success group immediately before (p = 0.0136) and up to 12 hours of HFNC therapy than in the HFNC failure group. Conclusions Early intubation may be considered in patients with a higher VICE score or a lower ROX index. The ROX score during HFNCs use can provide an early warning sign of treatment failure. Further investigations are warranted to confirm these results. Plain Language Summary High flow nasal cannulas (HFNCs) were widely used in patients with COVID-19 infection related hypoxemic respiratory failure. However, there were concerns about its failure and related delayed intubation may be associated with a higher mortality rate. This retrospective study revealed patients with higher baseline disease severity and higher VICE scores may be treated with primary invasive mechanical ventilation. On the contrary, if their baseline VICE score is low and ROX index is high, HFNCs treatment might be safely applied initially. The trends of serial ROX index values during HFNC use could be a reliable periscope to predict the HFNC therapy outcome, therefore avoided delayed intubation.
背景:新冠肺炎合并急性呼吸衰竭患者应用高流量鼻插管的时机尚不确定。研究设计与方法:本研究以低氧性呼吸衰竭的covid -19感染成人患者为研究对象。记录患者的基线流行病学资料和呼吸衰竭相关参数,包括COVID-19通气率(VICE)和血氧饱和度(ROX指数)。测量的主要结果是28天死亡率。结果:共纳入69例患者。54例(78%)患者在第1天插管并接受有创机械通气(MV)支持,被纳入MV组。其余15例(22%)患者最初接受HFNC (HFNC组),其中10例(66%)患者住院期间未插管,属于HFNC成功组,其中5例(33%)患者因疾病进展后插管,属于HFNC失败组。HFNC组死亡率较MV组低(6.7% vs. 40.7%, p = 0.0138)。两组患者的基线特征无差异;而HFNC组的VICE评分(0.105[0.049-0.269]比0.260 [0.126-0.693],p = 0.0092)低于MV组,ROX指数(5.3[5.1-10.7]比4.3 [3.9-4.9],p = 0.0007)高于MV组。HFNC治疗成功组的ROX指数在治疗前(p = 0.0136)和治疗12小时时均高于HFNC治疗失败组。结论:对于VICE评分较高或ROX指数较低的患者,可考虑早期插管。hfnc使用期间的ROX评分可以提供治疗失败的早期预警信号。有必要进一步调查以证实这些结果。
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引用次数: 0
The role of precision medicine in bronchiectasis: emerging data and clinical implications. 精准医学在支气管扩张中的作用:新数据和临床意义。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-04-01 DOI: 10.1080/17476348.2023.2205125
Grace Oscullo, David de la Rosa, Marta Garcia Clemente, Rosa Giron, Rafael Golpe, Luis Máiz, Miguel Angel Martinez-Garcia

Introduction: Bronchiectasis is a very heterogeneous disease. This heterogeneity has several consequences: severity cannot be measured by a single variable, so multidimensional scores have been developed to capture it more broadly. Some groups of patients with similar clinical characteristics or prognoses (clinical phenotypes), and even similar inflammatory profiles (endotypes), have been identified, and these have been shown to require a more specific treatment.

Areas covered: We comment on this 'stratified' model of medicine as an intermediate step toward the application of the usual concepts on which precision medicine is based (such as cellular, molecular or genetic biomarkers, treatable traits and individual clinical fingerprinting), whereby each subject presents certain specific characteristics and receives individualized treatment.

Expert opinion: True precision or personalized medicine is based on concepts that have not yet been fully achieved in bronchiectasis, although some authors are already beginning to adapt them to this disease in terms of pulmonary and extrapulmonary etiologies, clinical fingerprinting (specific to each individual), cellular biomarkers such as neutrophils and eosinophils (in peripheral blood) and molecular biomarkers such as neutrophil elastase. In therapeutic terms, the future is promising, and some molecules with significant antibiotic and anti-inflammatory properties are being developed.

支气管扩张是一种异质性疾病。这种异质性有几个后果:严重程度不能用单一变量来衡量,所以多维评分已经被开发出来,以更广泛地捕捉它。一些患者组具有相似的临床特征或预后(临床表型),甚至相似的炎症特征(内源性),已被确定,这些已被证明需要更具体的治疗。涵盖领域:我们评论这种“分层”医学模型,将其作为应用精准医学所基于的常规概念的中间步骤(如细胞、分子或基因生物标志物、可治疗特征和个体临床指纹),每个主题都呈现出某些特定特征并接受个性化治疗。专家意见:真正的精准或个性化医疗是基于支气管扩张尚未完全实现的概念,尽管一些作者已经开始根据肺和肺外病因、临床指纹(针对每个个体)、细胞生物标志物(如外周血中的中性粒细胞和嗜酸性粒细胞)和分子生物标志物(如中性粒细胞弹性酶)使其适应这种疾病。在治疗方面,未来是有希望的,一些具有显著抗生素和抗炎特性的分子正在开发中。
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引用次数: 0
Challenges in the diagnosis of idiopathic pulmonary fibrosis: the importance of a multidisciplinary approach. 特发性肺纤维化诊断的挑战:多学科方法的重要性。
IF 3.9 3区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2023-04-01 DOI: 10.1080/17476348.2023.2199156
Alessia Comes, Giacomo Sgalla, Simone Ielo, Tonia Magrì, L Richeldi

Introduction: The diagnosis of Idiopathic pulmonary fibrosis (IPF) requires the careful exclusion of secondary causes of interstitial lung disease (ILD), and the collaboration among different specialists is considered paramount to establish a diagnosis with high diagnostic confidence. The multidisciplinary discussion (MDD) has assumed an increasing importance over the years in the different phases of the IPF diagnostic work-up.

Areas covered: The role of MDD in the diagnosis and management of IPF will be described. Practical insights will be provided into how and when to perform MDD based on the available scientific evidence. Current limitations and future perspectives will be discussed.

Expert opinion: In the absence of high diagnostic confidence, agreement between different specialists during MDD is recognized as a surrogate indicator of diagnostic accuracy. Often, despite a lengthy evaluation, the diagnosis remains unclassifiable in a significant percentage of patients. MDD therefore appears to be pivotal in attaining an accurate diagnosis of ILDs. The discussion among different specialists can also include other specialists, such as rheumatologists and thoracic surgeons, in addition to the core group of pulmonologists, radiologists, and pathologists. Such discussions can allow greater diagnostic accuracy and have important effects on management, pharmacologic therapies, and prognosis.

特发性肺纤维化(IPF)的诊断需要仔细排除间质性肺疾病(ILD)的继发原因,不同专家之间的合作对于建立高诊断可信度的诊断至关重要。多年来,在IPF诊断工作的不同阶段,多学科讨论(MDD)的重要性日益增加。涵盖领域:将描述重度抑郁症在IPF诊断和管理中的作用。将根据现有的科学证据提供关于如何以及何时执行MDD的实际见解。将讨论当前的局限性和未来的前景。专家意见:在缺乏高诊断可信度的情况下,MDD期间不同专家之间的一致性被认为是诊断准确性的替代指标。通常,尽管经过长时间的评估,很大一部分患者的诊断仍然无法分类。因此,MDD似乎是获得ild准确诊断的关键。不同专家之间的讨论也可以包括其他专家,如风湿病学家和胸外科医生,除了肺科医生、放射科医生和病理学家的核心小组。这样的讨论可以提高诊断的准确性,并对管理、药物治疗和预后有重要影响。
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引用次数: 0
期刊
Expert Review of Respiratory Medicine
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