Tracheobronchomalacia

气管支气管软化症
  • 文章类型: Case Reports
    气管软化是一种情况,其中气管壁异常柔软,并且在呼吸努力增加时容易塌陷。气道软化症可以表现为像喉软化症这样的节段性疾病,气管软化和支气管软化,或作为弥漫性条件,如气管支气管软化(TBM)。与长段先天性气管狭窄不同,手术可能是首选治疗方法,长段TBM的管理仍然存在争议。
    Tracheomalacia is a condition where the tracheal wall is abnormally soft and prone to collapse during increased respiratory efforts. Airway malacia can manifest as segmental conditions like laryngomalacia, tracheomalacia and bronchomalacia, or as diffuse conditions such as tracheobronchomalacia (TBM). Unlike long-segment congenital tracheal stenosis, where surgery may be the preferred treatment, the management of long-segment TBM remains controversial.
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  • 文章类型: Journal Article
    Tracheobronchial stenosis, particularly central airway stenosis, which frequently results in severe complications such as lung damage, occurs in patients with tracheobronchial tuberculosis (TBTB).
    To analyze the clinical characteristics of patients with central airway stenosis due to tuberculosis (CASTB).
    Retrospective analysis was performed on the clinical features, radiological features, bronchoscopic features and treatment of 157 patients who were diagnosed with CASTB in two tertiary hospitals in Chongqing, China, from May 2020 to May 2022.
    CASTB mostly occurs in young patients and females. Patients with CASTB exhibited different symptoms repeatedly during the disease, especially varying degrees of dyspnea, prompting many patients to undergo bronchoscopic intervention and even surgery. Patients with cicatricial strictures constituted the highest proportion of the TBTB subtype with CASTB and 35.7% of the patients with CASTB were found to have tracheobronchomalacia (TBM) under bronchoscopy. CASTB and TBM mainly involved the left main bronchus. Patients with lower levels of education had higher rates of TBM. Patients with TBM manifested shortness of breath more frequently than patients without TBM. Patients with TBTB who had undergone bronchoscopic interventions have a higher rate of TBM.
    Despite mostly adequate anti-tuberculosis chemotherapy, patients with TBTB can present with CASTB involving severe scarring stenosis, bronchial occlusion, tracheobronchomalacia and even destroyed lung.
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  • 文章类型: Journal Article
    UNASSIGNED:评估高分辨率计算机断层扫描(HRCT)异常与支气管肺发育不良(BPD)临床表型之间的关系。
    未经评估:回顾,单中心研究于2013年至2020年在复旦大学附属儿童医院进行。这项研究包括在妊娠≤32周时出生的早产儿,这些早产儿被诊断为BPD,并且在月经后40至50周龄(PMA)之间有HRCT。根据两类肺部病变的七种类型对来自六个肺叶的HRCT图像进行评分:过度通气病变和实质病变。高通气评分(HS)包括衰减降低的评分,马赛克衰减,和bulla/bleb,而实质评分(PS)包括线性病变,合并,支气管壁增厚,还有支气管扩张.将所有七个分数进行汇总以创建总分(TS)。采用单向ANOVA测试或Kruskal-Wallis测试比较HRCT评分与BPD严重程度和临床表型。采用Spearman相关分析评价HRCT评分与临床表型的相关性。
    未经评估:共81例病例纳入研究。BPD更严重的病例有更高的TS(p=0.01),HS(p=0.02),PS(p=0.02),马赛克衰减分数(p=0.03),bulla/Bleb评分(p=0.03),和线性密度评分(p=0.01)。TS(r=0.28),PS(r=0.35),线密度(r=0.34),合并(r=0.24)与肺动脉高压(PH)相关。然而,有或没有气管支气管软化症(TBM)的患者之间无HRCT评分显着差异。合并肺实质疾病的BPD患者,PH,TBM的TS和HS最高。
    未经证实:在我们的研究中,HRCT评分与BPD严重程度和PH相关。HS可能是评估BPD严重程度的有用工具,而线密度和固结可能有助于预测PH。
    UNASSIGNED: To assess the relationship between high-resolution computed tomography (HRCT) abnormalities and clinical phenotypes of bronchopulmonary dysplasia (BPD).
    UNASSIGNED: A retrospective, single-center study was carried out at the Children\'s Hospital of Fudan University between 2013 and 2020. Preterm infants born at ≤ 32 weeks\' gestation who were diagnosed with BPD and had HRCT between 40 and 50 weeks postmenstrual age (PMA)were included in the study. HRCT images from six pulmonary lobes were scored based on seven types of pulmonary lesions from two categories: hyperaeration lesions and parenchymal lesions. The hyperaeration score (HS) included scores of decreased attenuation, mosaic attenuation, and bulla/bleb, while the parenchymal score (PS) included those of linear lesion, consolidation, bronchial wall thickening, and bronchiectasis. All seven scores were summed up to create the total score (TS). One-way ANOVA testing or Kruskal-Wallis testing was adopted for the comparison of HRCT scores with BPD severity and clinical phenotypes. The correlation between HRCT scores and clinical phenotypes was evaluated by Spearman\'s correlation analysis.
    UNASSIGNED: A total of 81 cases were included in the study. Cases with more severe BPD had a higher TS (p = 0.01), HS (p = 0.02), PS (p = 0.02), mosaic attenuation score (p = 0.03), bulla/Bleb score (p = 0.03), and linear density score (p = 0.01). TS (r = 0.28), PS (r = 0.35), linear density (r = 0.34), and consolidation (r = 0.24) were correlated with pulmonary hypertension (PH). However, no HRCT score was significantly different between the patients with or without tracheobronchomalacia (TBM). BPD patients with a combination of lung parenchymal disease, PH, and TBM had the highest TS and HS.
    UNASSIGNED: HRCT scores correlated with BPD severity and PH in our study. HS might be a useful tool in the assessment of BPD severity while linear densities and consolidation might be helpful in predicting PH.
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  • 文章类型: Journal Article
    背景:跳步飞神(TBFS)公式,广泛用于中医(TCM),能增强慢性阻塞性肺疾病(COPD)患者肺肾气虚型急性加重的疗效,减少急性加重的频率。根据中医理论和长期的实践观察,TBFS已成为COPD相关气管支气管软化症(TBM)的有效治疗方法。
    目的:基于小窝蛋白1-p38MAPK信号和细胞凋亡研究TBFS配方治疗COPD相关TBM的机制。
    方法:吸烟联合气管脂多糖注射制备大鼠COPD模型。分离COPD大鼠气管或支气管软骨细胞,培养,并用10ng/mLIL-1β处理24小时,以建立COPD相关TBM模型。正常大鼠给予TBFS制备含药血清,和CCK8测定用于筛选最佳含药血清浓度和SB203580剂量。TBFS含药血清和SB203580分别处理为对照,模型,含药血清,阻滞剂,含药血清联合阻滞剂组。流式细胞术和CCK8测定用于检测细胞凋亡和增殖活性。使用甲苯胺蓝染色和免疫组织化学分析软骨细胞蛋白聚糖和II型胶原含量。蛋白质印迹法检测caveolin1,p-p38MAPK的表达,TNF-α,IL-1β,MMP-13,Bax,和Bcl-2蛋白。定量PCR检测小窝蛋白1、p38MAPK的表达,IL-1β,MMP-13,Bax,Bcl-2和miR-140-5p。
    结果:COPD大鼠支气管软骨细胞的分离和鉴定表明,10ng/mLIL-1β可以产生稳定的COPD相关TBM模型。通过CCK8方法筛选,第四代支气管软骨细胞被确定为最佳细胞,5μMSB203580和5%低剂量含药血清是最佳干预剂量。各组实验软骨细胞分别处理48h。甲苯胺蓝染色和免疫组织化学分析显示,SB203580和TBFS含药血清联合SB203580可有效增加软骨细胞降解后的蛋白聚糖和II型胶原含量。用SB203580和TBFS含药血清联合SB203580处理的细胞的流式细胞术显示细胞凋亡显着减少,细胞增殖活性增强。蛋白质印迹和qPCR分析显示,含有TBFS药物的血清,SB203580和TBFS含药血清联合SB203580有效抑制小窝蛋白1、p-p38MAPK的表达,MMP-13,IL-1β,TNF-α,和Bax蛋白同时促进Bcl-2蛋白表达。用TBFS含药血清和SB203580治疗可有效抑制MMP-13,p38MAPK的表达,小窝蛋白1和Bax基因,并促进Bcl-2和miR-140-5p基因的表达。
    结论:浓度为10ng/mL的IL-1β可以产生稳定的COPD相关TBM细胞模型。TBFS可以提高蛋白多糖和II型胶原的含量,增加细胞活性,减少软骨细胞凋亡。TBFS的作用可能与抑制Caveolin1-p38MAPK信号通路中关键信号分子Caveolin1和p-p38MAPK表达的机制有关,从而降低下游效应产物MMP-13,IL-1β的表达,和TNF-α,同时抑制凋亡基因Bax的表达,提高Bcl-2和miR-140-5p基因的表达。
    BACKGROUND: The Tiao-bu-fei-shen (TBFS) formula, extensively used in Traditional Chinese Medicine (TCM), can enhance therapeutic efficacy and reduce the frequency of acute exacerbations of lung-kidney Qi deficiency in patients with chronic obstructive pulmonary disease (COPD). According to both TCM theory and long-term observation of practice, TBFS has become an effective treatment for COPD-associated tracheobronchomalacia (TBM).
    OBJECTIVE: To investigate the mechanism of the TBFS formula in treating COPD-associated TBM based on caveolin 1-p38 MAPK signaling and apoptosis.
    METHODS: A rat COPD model was prepared by exposure to smoking combined with tracheal lipopolysaccharide injection. The trachea or bronchus chondrocytes from COPD rats were isolated, cultured, and treated with 10 ng/mL IL-1β for 24 h to develop a model of COPD-associated TBM. Normal rats were administered TBFS to prepare drug-containing serum, and CCK8 assays were used to screen the optimal drug-containing serum concentration and SB203580 dose. TBFS drug-containing serum and SB203580 were processed separately for the control, model, drug-containing serum, blocker, and drug-containing serum combined with blocker groups. Flow cytometry and CCK8 assays were used to detect apoptosis and proliferative activity. Toluidine blue staining and immunohistochemistry were used to analyze the chondrocyte proteoglycan and type II collagen content. Western blotting was used to detect the expression of caveolin 1, p-p38 MAPK, TNF-α, IL-1β, MMP-13, Bax, and Bcl-2 proteins. Quantitative PCR was used to detect the expression of caveolin 1, p38 MAPK, IL-1β, MMP-13, Bax, Bcl-2, and miR-140-5p.
    RESULTS: The isolation and identification of bronchial chondrocytes from COPD rats revealed that 10 ng/mL IL-1β can produce a stable COPD-associated TBM model. Screened via the CCK8 method, fourth-generation bronchial chondrocytes were determined as the optimal cells, and 5 μM SB203580 and 5% low-dose drug-containing serum were the optimal intervention doses. The experimental chondrocytes of each group were treated separately for 48 h. Toluidine blue staining and immunohistochemical analysis revealed that TBFS drug-containing serum, SB203580, and TBFS drug-containing serum combined with SB203580 can effectively increase the proteoglycan and type II collagen content after chondrocyte degradation. Flow cytometry of cells treated with SB203580 and TBFS drug-containing serum combined with SB203580 revealed significantly reduced cell apoptosis and enhanced cell proliferation activity. Western blot and qPCR analyses revealed that the TBFS drug-containing serum, SB203580, and TBFS drug-containing serum combined with SB203580 effectively inhibit the expression of caveolin 1, p-p38 MAPK, MMP-13, IL-1β, TNF-α, and Bax proteins while promoting Bcl -2 protein expression. Treatment with TBFS drug-containing serum and SB203580 effectively inhibited the expression of MMP-13, p38 MAPK, caveolin 1, and Bax genes, and promoted the expression of Bcl-2 and miR-140-5p genes.
    CONCLUSIONS: A concentration of 10 ng/mL of IL-1β can generate a stable COPD-associated TBM cell model. TBFS can improve the proteoglycan and type II collagen content, increase cell activity, and reduce the amount of chondrocyte apoptosis. The role of TBFS may be related to mechanisms of inhibiting the expression of the key signaling molecules caveolin 1 and p-p38 MAPK in the caveolin 1-p38 MAPK signaling pathway, thereby reducing the expression of the downstream effector products MMP-13, IL-1β, and TNF-α, while inhibiting the expression of the apoptotic gene Bax and improving the expression of Bcl-2 and miR-140-5p genes.
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  • 文章类型: Journal Article
    气管支气管软化症(TBM)通常表现为喘息。重新评估TBM在儿童持续喘息中的作用至关重要。
    我们选择了在2009年1月至2019年7月期间经支气管镜检查诊断为TBM并接受支气管镜复查的持续性喘息或慢性咳嗽的儿童。收集并回顾性分析临床和支气管镜检查数据。为了进行统计分析,我们使用了Kaplan-Meier方法,Kruskal-Wallis测试,和Fisher精确检验。
    共纳入79例患者(男57例,女22例)。首次诊断TBM的中位年龄为7个月(四分位间[IQR]4-11个月)。首次喘息发作的中位年龄为4(IQR3-7)个月。在两次支气管镜检查之间的时间间隔内,50例(63.3%)软化病变消退,14例患者(17.7%)有所改善,11例患者(13.9%)无变化,4例患者(5.1%)病情加重。37例患者的软化病变在2岁之前解决。在50名患者中,22例患者(44.0%)在支气管镜检查评估之间报告了3次或更多的喘息,22例患者中有13例(59.1%)有特应性或过敏性疾病家族史,最终诊断为支气管哮喘。
    在持续喘息的儿童中,TBM的作用应该重新评估,尤其是那些有特应性或过敏性疾病家族史的人,支气管哮喘应及早考虑。
    Tracheobronchomalacia (TBM) is often manifested as wheezing. Reassessing the role of TBM in persistent wheezing in children is essential.
    We selected children who were diagnosed with TBM by bronchoscopy and who underwent bronchoscopic reexamination for persistent wheezing or chronic cough between January 2009 and July 2019. The clinical and bronchoscopy data were collected and retrospectively reviewed. For statistical analysis, we used the Kaplan-Meier method, Kruskal-Wallis test, and Fisher exact test.
    A total of 79 patients (57 males and 22 females) were included. The median age of the first TBM diagnosis was 7 (interquartile [IQR] 4-11) months. The median age of the first wheezing episode was 4 (IQR 3-7) months. During the time interval between the two bronchoscopies, malacia lesions resolved in 50 patients (63.3%), improvement was seen in 14 patients (17.7%), no change was observed in 11 patients (13.9%), and the condition was aggravated in 4 patients (5.1%). The malacia lesions in 37 patients resolved before 2 years of age. Among the 50 resolved patients, 22 patients (44.0%) reported wheezing three times or more between bronchoscopy evaluations, and 13 of these 22 patients (59.1%) with atopy or family history of allergic diseases were ultimately diagnosed with bronchial asthma.
    In children with persistent wheezing, the role of TBM should be reassessed, especially in those with atopy or family history of allergic diseases, and bronchial asthma should be considered early.
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  • 文章类型: Case Reports
    Relapsing polychondritis is an immune disorder of unknown etiology involving multiple systems that is characterized by persistent inflammation and destruction of cartilage, including the ears, nose, costal, joint, and airways. Airway involvement caused by relapsing polychondritis is common, and tracheobronchomalacia is the most serious complication, which is life-threatening. Currently, the exact mechanism of relapsing polychondritis with tracheobronchomalacia is unknown. Although glucocorticoids and immunosuppressive agents are administered, failures often occur. Currently, bronchoscopy-guided intervention therapy used in tracheobronchomalacia caused by chronic obstructive pulmonary disease or other etiology has gradually increased, but bronchoscopy-guided intervention therapy with extracorporeal membrane oxygenation assist used in tracheobronchomalacia caused by relapsing polychondritis has not been reported. Here, we report a case of relapsing polychondritis with severe tracheobronchomalacia. Although drug therapy was provided and airway stent implantation was performed, the tracheal stenosis was further aggravated. Because conventional anesthesia and mechanical ventilation cannot meet the needs of bronchoscopy-guided intervention therapy or guarantee sufficient safety. The intervention treatment was performed with the support of extracorporeal membrane oxygenation, which was successfully completed without obvious complications. The symptoms were significantly improved, and the patient was discharged uneventfully.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    本文报道了1例3月龄先天性气管软化患儿的诊疗过程。患儿为低出生体重儿,曾因“出生气促1 h”于2018年8月21日入当地妇幼保健院新生儿科,后因“先天性食道闭锁”转诊至深圳市儿童医院普外科,于8月23日在全身麻醉下行食管气管瘘修补+食管吻合术。术后患儿出现呼吸困难和撤呼吸机困难,纤维支气管镜检查提示重度气管软化。经抗感染、抗反流及呼吸支持治疗2个月后,患儿症状缓解,遂出院。之后患儿因“反复出现憋气、紫绀”多次住院治疗。本次因再次出现“憋气、紫绀”于11月30日入院。查纤维支气管镜和CT可见隆突处上端气管重度软化,气道最狭窄处仅为0.88 mm。多学科组讨论建议,患儿在全身麻醉和体外循环辅助下行气管支架置入术。12月14日,患儿行气管支架置入手术。术后第2天拔出气管插管,患儿呼吸平顺,未再有窒息发作。气管支架于术后8个月被取出。术后随访,患儿未出现呼吸困难,气管软化支架扩张端恢复正常,未扩张端依旧塌陷,但不影响呼吸。.
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  • 文章类型: Case Reports
    背景:刚性支气管镜检查已被证明是诊断和治疗中央气道阻塞(CAO)的几种原因的绝佳工具。已经在患有CAO的儿童和成人中进行了硅胶支气管臂支架置入术的侵入性治疗,在以往的研究中获得了令人满意的结果。然而,很少有关于通过硬质支气管镜支架置入治疗中央气道阻塞的婴儿的报道。这种技术对小儿胸外科医生来说仍然是一个挑战,儿科介入肺科医师,和努力治疗气道阻塞疾病的耳鼻喉科医生。
    方法:4例患者在出生后一段时间内主诉呼吸困难。
    方法:3例患者被诊断为气管支气管软化症,和气管食管瘘.
    方法:4例患者通过硬支气管镜接受硅胶支架治疗。
    结果:硅支架足以改善气管道的阻塞。所有患者均随访6个月以上。3名患者可以正常呼吸;只有1名患者支架迁移。
    结论:对于CAO患儿,通过硬质支气管镜进行有创硅胶气管支气管支架置入术是一种可行的选择。根据我们的经验,选择合适的尺寸是支架置入成功的关键因素。
    BACKGROUND: Rigid bronchoscopy has been proven to be an excellent tool for the diagnosis and management of several causes of central airway obstruction (CAO). The invasive treatment of silicone bronchobrachial stenting has been performed in children and adults with CAO, and satisfying results were obtained in previous studies. However, there are few reports on infants with central airway obstruction treated with stenting via rigid bronchoscopy. This technique remains a challenge to pediatric thoracic surgeons, pediatric interventional pulmonologists, and otolaryngologists who struggle to treat airway obstruction disease.
    METHODS: Four patients were presented to our hospital with complaints of dyspnea for a period of time after their birth.
    METHODS: Three patients were diagnosed as tracheobronchomalacia, and tracheoesophageal fistula.
    METHODS: Four patients were treated with silicone stenting through rigid bronchoscopy.
    RESULTS: Silicon stent was adequate for improving the obstruction of the tracheal tract. All the patients were followed-up longer than 6 months. Three patients could breathe normally; the stent migrated in only 1 patient.
    CONCLUSIONS: Invasive silicone tracheobronchial stenting via rigid bronchoscopy is a viable option for infants with CAO. Choosing an appropriate size is a critical factor for success of stenting according to our experience.
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  • 文章类型: Case Reports
    气管支气管软化症(TBM)是一种罕见的疾病,其特征是在呼气期间中央气道过度塌陷。很少报道吸入糜烂剂的潜在后果和治疗过程。据我们所知,这是由盐酸吸入引起的TBM的首次报道,通过插入多个气道支架成功治疗。一名45岁的女性在落入盐酸池后出现呼吸困难和咽部不适。患者通过多个支架插入成功治疗。支架置入后15个月,临床症状得到迅速改善。吸入化学损伤可能导致TBM,而多气道支架置入对此类特殊TBM显示出良好的治疗效果。
    Tracheobronchomalacia (TBM) is a rare disease characterized by excessive collapsibility of the central airways during expiration. Potential consequences and treatment courses of the aspiration of erosive agents are seldom reported. To our knowledge, this is the first report of TBM caused by hydrochloric acid inhalation, which was successfully treated by multiple airway stents insertion. A 45-year-old female presented with dyspnea and pharynx discomfort after falling into a hydrochloric acid pool. The patient was successfully treated with multiple stents insertion. In 15 months after the stents insertion, the clinical symptoms have been rapidly improved. Inhaled chemical damage may lead to TBM, while multiple airway stents insertion showed a good therapeutic effect for such special TBM.
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