Respiratory System Abnormalities

呼吸系统异常
  • 文章类型: Journal Article
    先天性肺异常(CLA)是指一组罕见的畸形,通常在产前或儿童早期发现。然而,相当比例的病例直到成年后才被发现,或者偶然发现或表现为反复呼吸道感染或肺出血的症状。虽然大多数CLA在CT和MRI上有特征性的影像学表现,由于在成年人中很少遇到它们,因此它们仍然是诊断挑战。放射科医师经常在建议诊断和指导适当的管理策略方面发挥关键作用。特征成像模式的识别对于准确诊断至关重要。作者检查了成人CLA患者的影像学表现和临床表现,重点关注有支气管肺受累的患者以及合并有支气管肺和血管异常的患者。讨论的实体包括支气管囊肿,支气管闭锁,先天性肺叶过度膨胀,先天性肺气道畸形,肺动脉近端中断,支气管肺隔离症,低遗传肺综合征,胎盘转移肺,和混合病变。讨论并说明了这些患者可能出现的常见并发症。©RSNA,2024补充材料可用于本文。
    Congenital lung anomaly (CLA) refers to a rare group of malformations that are typically identified prenatally or in early childhood. However, a significant proportion of cases evade detection until adulthood and either are incidentally discovered or manifest with symptoms of recurrent respiratory infection or pulmonary hemorrhage. While most CLAs have characteristic imaging findings at CT and MRI, they remain a diagnostic challenge due to the infrequency with which they are encountered in adults. Radiologists frequently play a pivotal role in suggesting the diagnosis and guiding appropriate management strategies, and recognition of characteristic imaging patterns is crucial for accurate diagnosis. The authors examine the imaging appearances and clinical manifestations in adult patients with CLA, with a focus on patients who have bronchopulmonary involvement and those with combined bronchopulmonary and vascular anomalies. Entities discussed include bronchogenic cyst, bronchial atresia, congenital lobar overinflation, congenital pulmonary airway malformation, proximal interruption of the pulmonary artery, bronchopulmonary sequestration, hypogenetic lung syndrome, placental transmogrification of the lung, and hybrid lesions. Common complications that may arise in these patients are discussed and illustrated. ©RSNA, 2024 Supplemental material is available for this article.
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  • 文章类型: Journal Article
    目的:在产前诊断为先天性肺畸形(CLM)的无症状儿童中,产后胸部X线(CXR)的临床意义尚不确定。我们评估了这些儿童产后使用CXR的理由。
    方法:我们纳入了通过胸部计算机断层扫描血管造影或组织病理学分析证实的出生时无症状的CLM患者,接受了常规的产后CXR,并参与了我们的标准护理前瞻性结构化纵向随访计划。排除有主要相关疾病的儿童。主要结果是4周龄和6月龄时CXR结果对症状发展的阳性和阴性预测值(PPV和NPV)。其次,我们试图确定CXR结果是否与在初次观察性住院期间或产后住院时间延长期间接受额外诊断相关.
    结果:在121名患者中,35例(29%)CXR未见异常,23例(19%)非特异性异常,和可能的CLM在63(52%)。CXR在4周和6个月时与症状发展相关的PPV分别为0.05和0.25。相应的NPV分别为0.96和0.91。在初始观察性住院期间,CXR发现与接受进一步诊断之间存在关联(p=.047)。其他诊断结果不影响临床管理。CXR结果与初始住院时间延长无关(p=0.40)。
    结论:在产前诊断为CLM的无症状患者中,产后CXR的常规做法可以省略,因为CXR结果不影响后续的临床管理。
    OBJECTIVE: The clinical implications of a postnatal chest X-ray (CXR) in asymptomatic children with a prenatally diagnosed congenital lung malformation (CLM) are uncertain. We assessed the justification for the postnatal use of CXR in these children.
    METHODS: We included patients with CLM confirmed through chest computed tomography angiography or histopathological analysis who were asymptomatic at birth, underwent routine postnatal CXR, and participated in our standard of care prospective structured longitudinal follow-up program. Children with major associated morbidities were excluded. Primary outcomes were the positive and negative predictive values (PPV and NPV) of CXR findings for symptom development at 4 weeks and 6 months of age. Secondarily, we sought to establish whether CXR findings were associated with undergoing additional diagnostics during the initial observational hospital stay or prolonged postnatal hospital admission.
    RESULTS: Among 121 included patients, CXR showed no abnormalities in 35 (29%), nonspecific abnormalities in 23 (19%), and probable CLM in 63 (52%). The PPV of CXR in relation to symptom development at 4 weeks and 6 months was 0.05 and 0.25, respectively. Corresponding NPVs were 0.96 and 0.91. An association was identified between CXR findings and undergoing further diagnostics during the initial observational hospital stay (p = .047). Additional diagnostic findings did not influence clinical management. CXR findings were not associated with prolonged initial hospital stay (p = .40).
    CONCLUSIONS: The routine practice of postnatal CXR in asymptomatic patients with prenatally diagnosed CLM can be omitted, as CXR findings do not influence subsequent clinical management.
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  • 文章类型: Journal Article
    目的:我们旨在确定无症状儿童下呼吸道感染(LRTI)的发生率,观察性治疗的先天性肺异常(CLA)与接受手术治疗的有症状患者不同.第二,我们试图比较接受手术的患者的LRTI切除前后的发生率.
    方法:这项回顾性队列研究包括1999年至2021年出生的经CT扫描证实患有CLA的患者,这些患者被纳入前瞻性纵向随访计划。使用发病率比(IRR)比较手术和观察管理的患者在1、2、5、8和12年的LRTI发病率。通过IRR评估接受CLA相关手术的患者切除前后LRTI发生率的差异。
    结果:在217名患者中,81人(37%)接受了手术,136人(63%)接受了观察性管理。手术组和观察组之间的LRTI发病率在任何随访时刻均无显著差异。在接受CLA相关手术的儿童中,切除前LRTI发病率明显高于切除后LRTI发病率(IRR为3.57,95%置信区间:[2.00;6.33],p<.001)。
    结论:我们无法证明接受手术和观察治疗的CLA患者在整个儿童期的LRTI发病率存在差异。我们建议在多学科环境中讨论CLA患者的LRTI病例,使用额外的诊断,如胸部X光检查,以筛查CLA参与,能够对病灶的手术切除做出深思熟虑的决定。
    OBJECTIVE: We aimed to determine if the incidence of lower respiratory tract infections (LRTI) among children with asymptomatic, observationally managed congenital lung abnormalities (CLA) differed from that of symptomatic patients who underwent surgery. Second, we sought to compare the pre- and post-resection incidence of LRTI in patients who underwent surgery.
    METHODS: This retrospective cohort study included patients born between 1999 and 2021 with CLA confirmed by CT scan who were enrolled in a prospective longitudinal follow-up program. The LRTI incidence rates at 1, 2, 5, 8, and 12 years were compared between surgically and observationally managed patients using incidence rate ratios (IRR). Differences in pre- and post-resection LRTI incidence rates among patients who underwent CLA-related surgery were assessed through IRR.
    RESULTS: Among 217 included patients, 81 (37%) had undergone surgery and 136 (63%) had been observationally managed. The LRTI incidence rates did not significantly differ at any follow-up moment between the surgical and observational groups. Among the children who underwent CLA-related surgery, the pre-resection LRTI incidence rates were significantly higher than the post-resection LRTI incidence rates (IRR of 3.57, 95% confidence interval: [2.00; 6.33], p < .001).
    CONCLUSIONS: We could not demonstrate differences in LRTI incidence throughout childhood between patients with surgically and observationally managed CLA. We recommend discussing cases of LRTI in patients with CLA in a multidisciplinary setting, using additional diagnostics such as chest X-ray to screen for CLA involvement, enabling a well-considered decision on surgical resection of the lesion.
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  • DOI:
    文章类型: Journal Article
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  • 文章类型: Journal Article
    肺基底段(ABLL)的异常全身动脉供应是咯血的罕见原因。ABLL可能会因为大咯血而变得复杂,左向右分流和感染引起的心力衰竭。我们描述了在妊娠晚期出现这种情况的情况。计算机断层扫描胸部血管造影证实了诊断。需要多学科方法来确定治疗方法,最终包括妊娠36周和4天的选择性剖腹产,随后经动脉栓塞9天产后。
    Anomalous systemic arterial supply to the basal segment of the lung (ABLL) is a rare cause of haemoptysis. ABLL may be complicated by massive haemoptysis, heart failure due to left-to-right shunt and infection. We describe a case of this condition presenting in the third trimester of pregnancy. Computed tomography chest angiogram confirmed the diagnosis. A multidisciplinary approach was necessary to determine treatment, which ultimately consisted of elective caesarean section at 36 weeks and 4 days\' gestation, followed by transarterial embolisation 9 days postpartum.
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  • 文章类型: English Abstract
    METHODS: Congenital pulmonary malformations (CPM) are rare and can be associated with high morbidity. Clinical presentation, diagnostic procedures, imaging, and therapy of CPM are discussed.
    RESULTS: Today, most CPM can be diagnosed prenatally by ultrasound. Postnatally, respiratory symptoms up to respiratory failure and recurrent lower respiratory tract infection are typical findings. Due to low diagnostic accuracy of chest x‑ray in CPM, all children with prenatal diagnosis of CPM or postnatally suspected CPM should undergo cross-sectional imaging.
    CONCLUSIONS: Based on imaging alone, the various subtypes of CPM cannot be definitively differentiated, which is why histological confirmation remains the gold standard. Surgical resection is the standard of care with minimally invasive procedures increasingly being employed. In certain situations, a watch-and-wait approach is possible.
    UNASSIGNED: LEISTUNGSFäHIGKEIT: Kongenitale Lungenfehlbildungen (KL) sind selten und können mit einer hohen Morbidität einhergehen. Klinik, diagnostisches Vorgehen, Bildmorphologie und Therapie werden diskutiert.
    UNASSIGNED: Die meisten kongenitalen Lungenfehlbildungen werden heutzutage pränatal durch Ultraschall diagnostiziert. Postnatal sind respiratorische Symptome bis zum respiratorischen Versagen und rezidivierende Infektionen typisch. Wegen der geringen diagnostischen Aussagekraft der konventionellen Röntgenuntersuchung sollten alle Kinder mit pränatal diagnostizierter oder postnatal vermuteter kongenitaler Lungenfehlbildung eine Schnittbildgebung des Thorax erhalten. EMPFEHLUNG FüR DIE PRAXIS: Anhand der Bildgebung können die verschiedenen Unterformen der kongenitalen Lungenfehlbildungen nicht mit Sicherheit abgegrenzt werden, so dass eine histologische Diagnosesicherung der Goldstandard ist. Kongenitale Lungenfehlbildungen werden meistens reseziert, wobei zunehmend minimal-invasive Verfahren zum Einsatz kommen. Auch ein Watch-and-wait-Ansatz ist unter bestimmten Voraussetzungen vertretbar.
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  • DOI:
    文章类型: Journal Article
    目的:评估婴儿肺功能检查的作用(潮汐呼吸流量循环,TBFVL)在患有气道异常的儿童中,并将如此获得的TBFVL与支气管镜检查结果相关联。
    方法:在这项前瞻性队列研究中,我们纳入了0~2岁有气道异常的儿童,并进行了TBFVL和支气管镜检查.主要结局指标是喉软化症中TBFVL的图形模式。次要结果指标是各种气道异常和对照中的TBFVL结果类型。
    结果:在53名注册儿童中,28例(52.3%)患有喉软化症。模式3(吸气肢体颤动)是喉软化症最常见的TBFVL模式。在TBFVL参数中,与对照组相比,孤立性喉软化症儿童的吸气时间与呼气时间(Ti/Te)和tPTEF/tE的比率显着升高。在六个月的随访中,TBFVL模式1(正常)成为最常见的模式。
    结论:一种特殊类型的气道异常可能具有TBFVL的特征性图形模式,TBFVL模式可能表明随访中气道异常的改善。
    OBJECTIVE: To evaluate the role infant pulmonary function tests (Tidal Breathing Flow Volume Loops, TBFVL) in children with airway anomalies and to correlate the TBFVL so obtained with bronchoscopy findings.
    METHODS: In this prospective cohort study, we enrolled children aged 0-2 years with airway anomalies and performed TBFVL and bronchoscopy. The primary outcome measure was graphic pattern of TBFVL in laryngomalacia. Secondary outcome measures were types of TBFVL results in various airway anomalies and controls.
    RESULTS: Out of 53 children enrolled, 28 (52.3%) had laryngomalacia. Pattern 3 (fluttering of inspiratory limb) was commonest TBFVL pattern in laryngomalacia. Among TBFVL parameters, the ratio of inspiratory time to expiratory time (Ti/Te) and tPTEF/tE was significantly high in children with isolated laryngomalacia compared to controls. At six months of follow-up, TBFVL pattern 1 (normal) became the commonest pattern.
    CONCLUSIONS: A particular type of airway anomaly may have a characteristic graphic pattern in TBFVL and TBFVL pattern may indicate improvement in airway anomalies in follow-up.
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  • 文章类型: Journal Article
    背景:多年来,先天性肺畸形(CLM)的治疗在小儿胸外科仍是一个有争议的话题.意大利小儿外科学会进行了一项全国性调查,以研究中心之间当前的管理差异,试图定义先天性肺畸形儿童的国家指南和标准化方法。
    方法:经过国家协会的批准,设计了一项包括35个产后管理项目的电子调查,专注于外科手术,麻醉学,放射学和肺炎方面。这项调查是联系所有进行胸外科手术的儿科手术单位进行的。
    结果:39个儿科手术单位(97.5%)参与了这项研究。13个中心(33.3%)被归类为高容量(A组),而26个中心(66.7%)是低容量(B组)。观察到诊断成像方案的差异,与B组相比,A组进行的CT扫描较少(p=0.012)。手术指征有利于两组无症状CLM和肺隔离的手术方法。而等待观察方法对于先天性大叶气肿很常见。无症状CLM的手术时机差异显著,大多数高容量中心对小于12个月的患者进行手术(p=0.02)。胸腔镜检查是大多数中心无症状CLM的首选方法,而大多数中心未进行术后长期随访.
    结论:胸腔镜手术在无症状CLM患者中似乎是一致的,在有症状的儿童中似乎是可变的。手术时机和术前影像学评估缺乏统一性已被确定为建立CLM通用国家护理模式的关键领域。
    BACKGROUND: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations.
    METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery.
    RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers.
    CONCLUSIONS: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.
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  • 文章类型: Review
    背景:当患者尽管接受常规治疗仍继续咳嗽时,包括草药和/或针灸的东亚传统医学(EATM)已被频繁使用。以前对EATM治疗慢性咳嗽的系统评价主要是在草药上进行的,针对导致咳嗽的患者。在临床实践中,EATM干预措施不仅限于草药,考虑到慢性咳嗽通常是由两种或多种疾病或非特异性原因引起的,全面的调查是临床相关的。我们检查了EATM治疗慢性咳嗽的研究现状。
    方法:基于Arksey和O\'Malley\的范围审查方法框架,总共有六个英语,中文,韩语,和日本电子数据库在2022年8月进行了搜索。包括针对慢性咳嗽患者(无论其病因如何)的EATM的任何临床研究。
    结果:在纳入的474项研究中,研究设计主要为随机对照试验(72.4%),人口平均分布在儿童和成人之间。大多数研究中没有报告咳嗽的原因(56.1%)。咳嗽的常见原因是上呼吸道咳嗽综合征和呼吸道感染(9.5%,each),其次是混合原因(7.6%),非特异性病因(5.9%),和胃食管反流病(4.0%)。EATM平均进行了19.1天,最常见的是中草药(80.6%)。常规药物经常用作对照(81.2%)。对于结果,总有效率是最常用的(94.3%),其次是咳嗽严重程度(53.8%)。EATM治疗在大多数研究中显示出积极的结果。
    结论:在未来的EATM研究中,有必要明确慢性咳嗽的病因或报告该研究针对非特异性慢性咳嗽.此外,应进行高质量的研究,评估EATM与安慰剂对照治疗的疗效,使用经过验证的评估工具。
    BACKGROUND: When patients continue to experience cough despite conventional treatment, East Asian traditional medicine (EATM) including herbal medicine and/or acupuncture has been frequently used. Previous systematic reviews of EATM treatment for chronic cough have been conducted mainly on herbal medicine, targeting patients with conditions that cause cough. In clinical practice, EATM interventions are not limited to herbal medicine, and considering that chronic cough is often caused by two or more conditions or unspecific causes, a comprehensive investigation is clinically relevant. We examined the current research status of EATM for chronic cough.
    METHODS: Based on Arksey and O\'Malley\'s scoping review methodological framework, a total of six English, Chinese, Korean, and Japanese electronic databases were searched on August 2022. Any clinical studies on EATM targeting chronic cough patients (regardless of their cause) were included.
    RESULTS: Among 474 included studies, the study designs were mainly randomized controlled trials (72.4%), and the population was evenly distributed between children and adults. The cause of cough was not reported in most studies (56.1%). The common cause of cough was upper airway cough syndrome and post-respiratory infection (9.5%, each), followed by mixed cause (7.6%), nonspecific cause (5.9%), and gastroesophageal reflux disease (4.0%). EATM was conducted for a mean of 19.1 days, and herbal medicine was the most common (80.6%). Conventional medication was frequently used as a control (81.2%). For outcomes, the total effective rate was the most frequently utilized (94.3%), followed by cough severity (53.8%). EATM treatment showed positive outcomes in most studies.
    CONCLUSIONS: In future EATM studies, it is necessary to either specify the cause of chronic cough or to report that the study was targeting nonspecific chronic cough. In addition, high-quality studies assessing the efficacy of EATM with placebo control treatment should be conducted, using validated evaluation tools.
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  • 文章类型: English Abstract
    Approximately 10% of the population suffer from a cough lasting longer than 8 weeks. Compared to acute cough, which usually occurs in the context of banal respiratory tract infections, the differential diagnoses of chronic cough require an increased use of diagnostic tests and thus a structured, evidence-based approach according to current international guidelines. A targeted history (smoking status, medication, previous diseases) and ENT status are always followed by chest x‑ray and pulmonary function tests before extended diagnostics. In the case of angiotensin-converting enzyme (ACE) inhibitor use and unremarkable physical examination, a drug discontinuation test can be carried out first. In case of inconspicuous findings, a disease entity that can be treated empirically such as upper airway cough syndrome is most likely. If the cough remains unexplained, cough suppression techniques, physiotherapy or speech therapy should be sought before off-label-use of medication.
    UNASSIGNED: Etwa 10 % der Bevölkerung leiden unter einem länger als 8 Wochen anhaltenden Husten. Im Vergleich zum akuten Husten, der meist im Rahmen von banalen Atemwegsinfekten auftritt, erfordern die Differenzialdiagnosen des chronischen Hustens einen erhöhten Einsatz von Diagnostik und somit ein strukturiertes, evidenzbasiertes Vorgehen gemäß aktuellen internationalen Leitlinien. Nach gezielter Anamnese (Raucherstatus, Medikation, Vorerkrankungen) und HNO-Status folgen immer eine Röntgenaufnahme des Thorax und Lungenfunktionsdiagnostik vor der erweiterten Diagnostik. Bei Angiotensin-Converting-Enzym(ACE)-Hemmer-Einnahme und unauffälliger körperlicher Untersuchung ist ein vorgeschalteter Auslassversuch möglich. Unauffällige Befunde machen probatorisch zu behandelnde Krankheitsbilder wie z. B. Upper-Airway-Cough-Syndrom wahrscheinlich. Bleibt der Husten dennoch ungeklärt, sind Hustenunterdrückungstechniken, physiotherapeutische oder logopädische Verfahren vor einer „Off-Label-Use“ Medikation anzustreben.
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