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Night-to-night variability in obstructive sleep apnoea: when might a multi-night measurement be helpful? 阻塞性睡眠呼吸暂停的夜间变异性:多夜测量何时可能有用?
Pub Date : 2025-01-23 DOI: 10.1080/17476348.2025.2457370
Esther Irene Schwarz
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引用次数: 0
Towards the integrated care of COPD, asthma and bronchiectasis: description and objectives of a treatable trait-based complex obstructive airway disease unit. 实现慢性阻塞性肺病、哮喘和支气管扩张综合治疗:基于可治疗性状的复杂阻塞性气道疾病单元的描述和目标。
Pub Date : 2025-01-22 DOI: 10.1080/17476348.2025.2453652
Borja G Cosio, Alexandre Palou, Meritxell López, Ruth Engonga, Jose Luis Valera, Nuria Toledo-Pons

Introduction: Expert management of Complex Obstructive Airway Diseases (COAD) requires knowledge, resources, and skills that are commonly shared in the management of the different conditions usually included in the acronym, namely asthma, bronchiectasis, and Chronic Obstructive Pulmonary Disease (COPD). We discuss the basis to shift the paradigm of single-disease management into a holistic approach and describe its potential benefits.

Areas covered: The prevalence and significance of the overlap between the different conditions is reviewed. Literature research on the topic of treatable traits in airway diseases is analyzed, with special emphasis in the role of an expert nurse and the multidisciplinary team approach for the management of asthma, bronchiectasis, and COPD. Finally, we describe the experience and organization of a COAD unit addressing desirable clinical outcomes and patient-related outcome measures.

Expert opinion: The division between different airway diseases generates confusion when the diseases present features common to various airway conditions. We describe here how a holistic approach of the airway disease process based on treatable traits regardless the diagnostic label reverts in a more efficient use of resources and better clinical outcomes. The role of an expert respiratory nurse and a multidisciplinary team are key areas for improvement.

简介:复杂阻塞性气道疾病(COAD)的专家管理需要在管理通常包含在首字母缩略词中的不同疾病(即哮喘、支气管扩张和慢性阻塞性肺疾病(COPD))方面共享的知识、资源和技能。我们讨论了将单一疾病管理范式转变为整体方法的基础,并描述了其潜在的好处。所涵盖的领域:审查了不同条件之间重叠的普遍性和重要性。对气道疾病可治疗特征的文献检索进行了分析,特别强调了专家护士的作用和多学科团队方法在哮喘、支气管扩张和慢性阻塞性肺病管理中的作用。最后,我们描述了COAD单元的经验和组织,解决了理想的临床结果和患者相关的结果措施。专家意见:当疾病表现出各种气道疾病的共同特征时,不同气道疾病之间的区分会产生混淆。我们在这里描述了如何基于可治疗特征的气道疾病过程的整体方法,而不考虑诊断标签,以更有效地利用资源和更好的临床结果。专家呼吸护理和多学科团队的作用是改进的关键领域。
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引用次数: 0
Multifaceted pulmonary manifestations of amyloidosis: state-of-the-art update. 淀粉样变的多面肺部表现:最新进展。
Pub Date : 2025-01-22 DOI: 10.1080/17476348.2025.2457374
Antonios Charokopos, Misbah Baqir, Anja C Roden, Jay H Ryu, Teng Moua

Introduction: Amyloidosis, a polymeric deposition disease classified according to protein subtype, may have varied pulmonary manifestations. Its anatomic-radiologic phenotypes include nodular, cystic, alveolar-septal, and tracheobronchial forms. Clinical presentation may range from asymptomatic parenchymal nodules to respiratory failure from diffuse parenchymal infiltration or diaphragmatic deposition.

Areas covered: In this review, we systematically describe the molecular subtypes of amyloidosis and their clinical and radiologic findings in the lungs as well as key extrapulmonary organ systems. We detail novel treatment approaches to systemic amyloidosis. We also discuss prognostic elements for each subtype. We identify key clinical scenarios where reaching a precise diagnosis can be complicated, and we offer insights on the varied presentations of pulmonary amyloidosis.

Expert opinion: Pulmonary amyloidosis is often difficult to diagnose as it may mimic other conditions, including fibrotic interstitial lung diseases and neoplasms, or can co-exist with certain connective tissue diseases. Despite some early artificial intelligence screening tools, improved familiarity among clinicians can aid in the more accurate and timely diagnosis of this multidimensional clinical entity. We additionally believe that multidisciplinary clinical pathwaysto diagnose and/or treat pulmonary amyloidosis have the potential to improve awareness, decrease diagnostic delay, and further elucidate knowledge on this multifaceted disease.

淀粉样变性是一种根据蛋白质亚型分类的聚合沉积疾病,可有多种肺部表现。其解剖-放射学表型包括结节型、囊性、肺泡-间隔型和气管支气管型。临床表现可从无症状实质结节到弥漫性实质浸润或横膈膜沉积引起的呼吸衰竭。涵盖的领域:在这篇综述中,我们系统地描述了淀粉样变性的分子亚型及其在肺和关键肺外器官系统中的临床和放射学表现。我们详细介绍了系统性淀粉样变性的新治疗方法。我们还讨论了每个亚型的预后因素。我们确定了达到精确诊断可能很复杂的关键临床情况,并提供了对肺淀粉样变性各种表现的见解。专家意见:肺淀粉样变通常难以诊断,因为它可能与其他疾病相似,包括纤维化间质性肺疾病和肿瘤,或可与某些结缔组织疾病共存。尽管有一些早期的人工智能筛查工具,但临床医生之间熟悉程度的提高可以帮助更准确、更及时地诊断这种多维临床实体。我们也相信,诊断和/或治疗肺淀粉样变性的多学科临床途径有可能提高认识,减少诊断延误,并进一步阐明对这种多面疾病的认识。
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引用次数: 0
The use of noninvasive positive pressure ventilation for severe asthma: a systematic review and meta-analysis of randomized controlled trials with trial sequential analysis. 使用无创正压通气治疗严重哮喘:随机对照试验的系统回顾和荟萃分析。
Pub Date : 2025-01-19 DOI: 10.1080/17476348.2025.2454947
Luigi La Via, Giuseppe Cuttone, Giovanni Misseri, Massimiliano Sorbello, Federico Pappalardo, Antonino Maniaci, Gilberto Duarte-Medrano, Natalia Nuño-Lámbarri, Christian Zanza, Cesare Gregoretti

Introduction: To evaluate the effectiveness of noninvasive positive pressure ventilation (NPPV) versus standard therapy in severe asthma exacerbations through meta-analysis.

Methods: Nine randomized controlled trials (344 patients) were analyzed from inception to August 2024. Primary outcomes included respiratory rate, forced expiratory volume in first second (FEV1), and oxygen saturation (SpO2). Random effect models and trial sequential analyses were employed.

Results: NPPV demonstrated significant reduction in respiratory rate versus standard therapy (mean difference [MD] -3.97, 95% CI -7.32 to -0.61, p = 0.02), though with high heterogeneity (I2 = 87%). FEV1 showed significant improvement with NPPV (MD 15.56%, 95% CI 6.86 to 24.26, p < 0.001) based on two studies. SpO2 showed no significant improvement (MD 0.62%, 95% CI -0.14 to 1.37, p = 0.11). No differences were found between pediatric and adult populations. Trial sequential analyses indicated insufficient evidence for definitive conclusions regarding respiratory rate and SpO2 improvements.

Conclusions: While NPPV may benefit severe asthma patients, particularly in reducing respiratory rate and improving FEV1, current evidence is insufficient for recommending routine clinical use. Larger randomized controlled trials are needed to establish NPPV's effectiveness in severe asthma exacerbation treatment.

Protocol registration: registration ID CRD42024580051.

前言:通过荟萃分析评估无创正压通气(NPPV)与标准治疗在严重哮喘加重中的有效性。方法:对9项随机对照试验(344例患者)进行分析。主要结局包括呼吸频率、第一秒用力呼气量(FEV1)和血氧饱和度(SpO2)。采用随机效应模型和试验序列分析。结果:与标准治疗相比,NPPV显示呼吸频率显著降低(平均差异[MD] -3.97, 95% CI -7.32至-0.61,p = 0.02),尽管异质性很高(I2 = 87%)。NPPV组FEV1明显改善(MD 15.56%, 95% CI 6.86 ~ 24.26, p p = 0.11)。在儿童和成人人群中没有发现差异。试验序列分析表明,没有足够的证据得出关于呼吸速率和SpO2改善的明确结论。结论:虽然NPPV可能对严重哮喘患者有益,特别是在降低呼吸频率和改善FEV1方面,但目前的证据不足以推荐常规临床应用。需要更大规模的随机对照试验来确定NPPV在严重哮喘加重治疗中的有效性。协议注册:注册号CRD42024580051。
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引用次数: 0
Challenges of symptom management in interstitial lung disease: dyspnea, cough, and fatigue. 间质性肺疾病症状管理的挑战:呼吸困难、咳嗽和疲劳。
Pub Date : 2025-01-15 DOI: 10.1080/17476348.2025.2453657
Amy Pascoe, Anne E Holland, Natasha Smallwood

Introduction: Interstitial lung disease (ILD) is a broad group of conditions characterized by fibrosis of the lung parenchyma. Idiopathic pulmonary fibrosis (IPF) is the most common subvariant. IPF is marked by considerable symptom burden of dyspnea, cough and fatigue that is often refractory to optimal disease-directed treatment.

Areas covered: In this narrative review, we searched MEDLINE for articles related to the current evidence regarding management of chronic dyspnea, cough, and fatigue as three of the most prevalent and distressing symptoms associated with IPF and other ILDs. Each symptom shares common features of multi-factorial etiology and a lack of safe and effective pharmacological therapies. Both corticosteroids and opioids have been utilized in this context, yet there is insufficient evidence of therapeutic benefit and considerable risk of harms. Whilst some may benefit from symptom-directed pharmacological management, usage must be carefully monitored. Use of non-pharmacological strategies, such as breathing techniques and speech therapy represent low risk and low-cost option, yet broader validation of these therapies' effectiveness is needed.

Expert opinion: Symptom management in IPF and other ILDs requires an iterative and individualized approach. Leveraging the expertise of multidisciplinary teams within an integrated care setting is an important opportunity to maximize health outcomes.

间质性肺病(ILD)是一大类以肺实质纤维化为特征的疾病。特发性肺纤维化(IPF)是最常见的亚型。IPF的显著特征是呼吸困难、咳嗽和疲劳,这些症状往往难以进行最佳的疾病定向治疗。涵盖领域:在这篇叙述性综述中,我们在MEDLINE检索了与慢性呼吸困难、咳嗽和疲劳这三种与IPF和其他ild相关的最普遍和最令人痛苦的症状的管理相关的文章。每种症状都具有多因素病因和缺乏安全有效的药物治疗的共同特征。皮质类固醇和阿片类药物均已在此背景下使用,但没有足够的证据表明其治疗益处和相当大的危害风险。虽然有些人可能受益于针对症状的药理学管理,但必须仔细监测使用情况。使用非药物策略,如呼吸技术和语言治疗是低风险和低成本的选择,但需要对这些疗法的有效性进行更广泛的验证。专家意见:IPF和其他ild的症状管理需要反复和个性化的方法。在综合护理环境中利用多学科团队的专业知识是最大限度提高健康结果的重要机会。
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引用次数: 0
Unveiling the potential of lung transplantation for situs inversus. 揭示逆位肺移植的潜力。
Pub Date : 2025-01-01 Epub Date: 2024-12-26 DOI: 10.1080/17476348.2024.2447513
Yudai Miyashita, Taisuke Kaiho, Chitaru Kurihara

Introduction: Situs inversus is a rare congenital condition where the organs in the chest and abdomen are reversed, thus complicating surgeries such as lung transplantation. Kartagener syndrome (KS), associated with situs inversus, includes chronic sinusitis and bronchiectasis, which can progress to end-stage lung disease requiring transplantation. This review discusses the unique surgical considerations, technical challenges, and outcomes of lung transplantation in patients with situs inversus, particularly KS.

Areas covered: The review highlights anatomical and physiological challenges in lung transplantation due to reversed organ positioning, requiring customized surgical approaches and intraoperative modifications. Preoperative imaging, anesthesia adjustments, and tailored surgical techniques are crucial for successful transplantation. Postoperative care focuses on managing complications such as primary graft dysfunction, infections, and anastomotic issues. Literature on survival rates, chronic lung allograft dysfunction, and quality of life is analyzed, indicating outcomes comparable to other lung transplant recipients.

Expert opinion: Despite significant challenges, lung transplantation in patients with situs inversus and KS is feasible with outcomes similar to traditional cases. Advances in imaging, surgical planning, and minimally invasive techniques offer promise for improved outcomes. Ongoing research, collaboration, and ethical considerations are essential to optimizing care and expand treatment possibilities for this high-risk patient population.

简介:位置倒置是一种罕见的先天性疾病,它是指胸腔和腹部的器官倒置,从而使肺移植等手术复杂化。Kartagener综合征(KS),与倒位相关,包括慢性鼻窦炎和支气管扩张,可发展为需要移植的终末期肺部疾病。这篇综述讨论了独特的手术注意事项、技术挑战和逆位肺移植患者的结果,特别是KS。涉及领域:该综述强调了由于器官定位逆转导致的肺移植解剖学和生理学上的挑战,需要定制手术入路和术中修改。术前成像、麻醉调整和量身定制的手术技术是移植成功的关键。术后护理的重点是处理并发症,如原发性移植物功能障碍、感染和吻合口问题。对生存率、慢性同种异体肺移植功能障碍和生活质量的文献进行分析,表明结果与其他肺移植受者相当。专家意见:尽管面临着巨大的挑战,但在位置反转和KS患者中进行肺移植是可行的,其结果与传统病例相似。影像学、手术计划和微创技术的进步为改善预后提供了希望。正在进行的研究、合作和伦理考虑对于优化这一高危患者群体的护理和扩大治疗可能性至关重要。
{"title":"Unveiling the potential of lung transplantation for situs inversus.","authors":"Yudai Miyashita, Taisuke Kaiho, Chitaru Kurihara","doi":"10.1080/17476348.2024.2447513","DOIUrl":"10.1080/17476348.2024.2447513","url":null,"abstract":"<p><strong>Introduction: </strong>Situs inversus is a rare congenital condition where the organs in the chest and abdomen are reversed, thus complicating surgeries such as lung transplantation. Kartagener syndrome (KS), associated with situs inversus, includes chronic sinusitis and bronchiectasis, which can progress to end-stage lung disease requiring transplantation. This review discusses the unique surgical considerations, technical challenges, and outcomes of lung transplantation in patients with situs inversus, particularly KS.</p><p><strong>Areas covered: </strong>The review highlights anatomical and physiological challenges in lung transplantation due to reversed organ positioning, requiring customized surgical approaches and intraoperative modifications. Preoperative imaging, anesthesia adjustments, and tailored surgical techniques are crucial for successful transplantation. Postoperative care focuses on managing complications such as primary graft dysfunction, infections, and anastomotic issues. Literature on survival rates, chronic lung allograft dysfunction, and quality of life is analyzed, indicating outcomes comparable to other lung transplant recipients.</p><p><strong>Expert opinion: </strong>Despite significant challenges, lung transplantation in patients with situs inversus and KS is feasible with outcomes similar to traditional cases. Advances in imaging, surgical planning, and minimally invasive techniques offer promise for improved outcomes. Ongoing research, collaboration, and ethical considerations are essential to optimizing care and expand treatment possibilities for this high-risk patient population.</p>","PeriodicalId":94007,"journal":{"name":"Expert review of respiratory medicine","volume":" ","pages":"43-54"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142883764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Interactions between epithelial mesenchymal plasticity, barrier dysfunction and innate immune pathways shape the genesis of allergic airway disease. 上皮间充质可塑性、屏障功能障碍和先天免疫途径之间的相互作用形成了过敏性气道疾病的发生。
Pub Date : 2025-01-01 Epub Date: 2025-01-06 DOI: 10.1080/17476348.2024.2449079
Allan R Brasier

Introduction: In genetically predisposed individuals, exposure to aeroallergens and infections from RNA viruses shape epithelial barrier function, leading to Allergic Asthma (AA). Here, activated pattern recognition receptors (PRRs) in lower airway sentinel cells signal epithelial injury-repair pathways leading to cell-state changes [epithelial mesenchymal plasticity (EMP)], barrier disruption and sensitization.

Areas covered: 1. Characteristics of sentinel epithelial cells of the bronchoalveolar junction, 2. The effect of aeroallergens on epithelial PRRs, 3. Role of tight junctions (TJs) in barrier function and how aeroallergens disrupt their function, 4. Induction of mucosal TGF autocrine loops activating type-2 innate lymphoid cells (ICL2s) leading to Th2 polarization, 5. How respiratory syncytial virus (RSV) directs goblet cell hyperplasia, and 6. Coupling of endoplasmic reticulum (ER) stress to metabolic adaptations and effects on basal lamina remodeling.

Expert opinion: When aeroallergens or viral infections activate innate immunity in sentinel cells of the bronchoalveolar junction, normal barrier function is disrupted, promoting chronic inflammation and Th2 responses. An improved mechanistic understanding of how activated PRRs induce EMP couples with TJ disruption, metabolic reprogramming and ECM deposition provides new biologically validated targets to restore barrier function, reduce sensitization, and remodeling in AA.

在遗传易感个体中,暴露于空气过敏原和RNA病毒感染会影响上皮屏障功能,导致过敏性哮喘(AA)。在这里,下气道前哨细胞中激活的模式识别受体(PRRs)向上皮损伤修复途径发出信号,导致细胞状态改变[上皮间充质可塑性(epithelial mesenchymal plasticity, EMP)]、屏障破坏和致敏。覆盖范围:1;支气管肺泡交界处前哨上皮细胞的特征2. 空气过敏原对上皮PRRs的影响3. 紧密连接(TJs)在屏障功能中的作用及空气过敏原如何破坏其功能4. 诱导粘膜TGF β自分泌环激活2型先天淋巴样细胞(ICL2s)导致Th2极化;5. 呼吸道合胞病毒(RSV)如何引导杯状细胞增生6. 内质网应激与代谢适应的耦合及其对基底膜重塑的影响。专家意见:当空气过敏原或病毒感染激活支气管肺泡连接处前哨细胞的先天免疫时,正常屏障功能被破坏,促进慢性炎症和Th2反应。对活化的PRRs如何诱导EMP与TJ破坏、代谢重编程和ECM沉积耦合的机制的进一步了解,为恢复屏障功能、减少AA的致敏和重塑提供了新的生物学验证靶点。
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引用次数: 0
Current perspectives on the rehabilitation of COPD patients with comorbidities. COPD合并合并症患者的康复治疗现状
Pub Date : 2025-01-01 Epub Date: 2025-01-16 DOI: 10.1080/17476348.2025.2452441
Audrey Borghi-Silva, Patrícia Faria Camargo, Flávia Cristina Rossi Caruso, Cássia da Luz Goulart, Renata Trimer, Aldair Darlan Santos-Araújo, Izadora Moraes Dourado, Andrea Lúcia Gonçalves da Silva

Introduction: Chronic obstructive pulmonary disease (COPD) is frequently accompanied by a variety of comorbidities, complicating management and rehabilitation efforts. Understanding this interplay is crucial for optimizing patient outcomes.

Areas covered: This review, based on the MEDLINE, Embase and Cochrane Library databases, summarizes the main research on the rehabilitation of patients with COPD, with an emphasis on relevant comorbidities, such as cardiovascular diseases, pulmonary hypertension, lung cancer, metabolic, musculoskeletal, and gastrointestinal disorders. anxiety/depression and cognitive disorders. The study highlights the importance of pre-participation assessments, ongoing monitoring and personalized rehabilitation programs. A review includes a comprehensive literature search to assess the scientific evidence on these interventions and their impact.

Expert opinion: The integration of cardiorespiratory rehabilitation program is essential for improving physical capacity and quality of life in COPD patients with comorbidities. While existing studies highlight positive outcomes, challenges such as interdisciplinary collaboration and access to rehabilitation services remain. Future strategies must prioritize personalized and integrated approaches programs combining pharmacological optimization and a close monitoring during cardiopulmonary rehabilitation to significantly reduce hospital readmissions and mortality, even in patients with complex multimorbidities. Continued research is necessary to refine rehabilitation protocols and better understand the complexities of managing COPD alongside cardiac conditions.

慢性阻塞性肺疾病(COPD)经常伴有各种合并症,使治疗和康复工作复杂化。了解这种相互作用对于优化患者预后至关重要。涵盖领域:本综述基于MEDLINE、Embase和Cochrane图书馆数据库,总结了COPD患者康复的主要研究,重点关注相关合并症,如心血管疾病、肺动脉高血压、肺癌、代谢、肌肉骨骼和胃肠道疾病。焦虑/抑郁和认知障碍。该研究强调了参与前评估、持续监测和个性化康复计划的重要性。综述包括全面的文献检索,以评估这些干预措施及其影响的科学证据。专家意见:整合心肺康复计划对于改善COPD合并合并症患者的身体能力和生活质量至关重要。虽然现有的研究强调了积极的结果,但跨学科合作和获得康复服务等挑战仍然存在。未来的策略必须优先考虑个性化和综合方法,将药物优化和心肺康复期间的密切监测相结合,以显著减少再入院率和死亡率,即使是患有复杂的多种疾病的患者。有必要继续研究以完善康复方案,并更好地了解慢性阻塞性肺病与心脏病同时治疗的复杂性。
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引用次数: 0
Addressing the need for better management of pleural infections in low- to middle-income countries. 解决中低收入国家更好地管理胸膜感染的需要。
Pub Date : 2025-01-01 Epub Date: 2025-01-14 DOI: 10.1080/17476348.2025.2453117
Jane A Shaw, Coenraad F N Koegelenberg
{"title":"Addressing the need for better management of pleural infections in low- to middle-income countries.","authors":"Jane A Shaw, Coenraad F N Koegelenberg","doi":"10.1080/17476348.2025.2453117","DOIUrl":"10.1080/17476348.2025.2453117","url":null,"abstract":"","PeriodicalId":94007,"journal":{"name":"Expert review of respiratory medicine","volume":" ","pages":"7-10"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142967631","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Respiratory manifestations of sickle cell disease in children: a comprehensive review for the pediatrician. 儿童镰状细胞病的呼吸表现:儿科医生的综合综述。
Pub Date : 2025-01-01 Epub Date: 2025-01-20 DOI: 10.1080/17476348.2025.2451960
Lisa Grigoli, Maria Marocchi, Laura Venditto, Michele Piazza, Laura Tenero, Giorgio Piacentini, Marco Zaffanello, Giuliana Ferrante

Introduction: Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by the production of sickle hemoglobin, leading to red blood cells sickling and hemolysis in hypoxic conditions. The resulting acute and chronic endothelial inflammation leads to chronic organ damage. Respiratory manifestations in SCD usually start from childhood and represent the leading causes of morbidity and mortality. Nevertheless, they are generally poorly addressed or recognized later in life, often contributing to a more severe course and complications.

Areas covered: This narrative review aims to outline the significant acute and chronic respiratory manifestations in children with SCD, focusing on prevention and clinical management. Compelling issues that need to be addressed in the future are also discussed. We searched the PubMed database for original papers written in English. Age restrictions were set for children (birth to 18 years). No limitations were set for the date and study country.

Expert opinion: Early detection and treatment of respiratory manifestations in SCD should be central to follow-up with patients affected by SCD. Nonetheless, studies are lacking, especially in pediatric age, and there is still no consensus on their management. Further research is strongly needed to accomplish universally accepted guidelines to guarantee patients the best care possible.

简介:镰状细胞病(SCD)是一种遗传性血红蛋白病,其特征是镰状血红蛋白的产生,在缺氧条件下导致红细胞镰状和溶血。由此产生的急性和慢性内皮炎症导致慢性器官损伤。SCD的呼吸道表现通常始于儿童时期,是发病率和死亡率的主要原因。然而,这些问题在以后的生活中通常没有得到很好的解决或认识,往往导致更严重的病程和并发症。涵盖领域:本综述旨在概述SCD儿童的急性和慢性呼吸症状,重点是预防和临床管理。还讨论了未来需要解决的紧迫问题。我们在PubMed数据库中搜索用英语写的原始论文。儿童(出生至18岁)有年龄限制。没有对日期和研究国家设定限制。专家意见:早期发现和治疗SCD的呼吸症状应该是SCD患者随访的核心。然而,缺乏研究,特别是在儿科年龄,对其处理仍然没有共识。迫切需要进一步的研究来完成普遍接受的指导方针,以保证患者尽可能得到最好的护理。
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引用次数: 0
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Expert review of respiratory medicine
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