Congenital lobar emphysema: evaluation and long-term follow-up of thirty cases at a single center

Congenital lobar emphysema (CLE) is characterized by overdistension and air-trapping in the affected lobe, and is one of the causes of infantile respiratory distress. In this report, we review our 27 years of experience with 30 CLE patients. Patients' medical records were evaluated with regard to age, clinical presentation, diagnostic methods, associated diseases, treatment, histopathologic findings, and final clinical and laboratory findings at the end of a long-term period. The mean age of 30 patients (18 male) at diagnosis was 4.9 +/- 6.7 months (range, 2 days-2.5 years). Tachypnea, dyspnea, cough, cyanosis, wheezing, hoarseness, and decreased breath sounds on the affected side were the main symptoms and clinical findings. On chest X-rays, emphysema was seen in all patients; shift/herniation to the opposite lung, atelectasis, and pneumothorax were observed in 16, 5, and 2 cases, respectively. Computerized tomography of the thorax was performed in 16 cases and revealed emphysema at affected lobe/lobes in all, a shift/herniation to the opposite side in 12 cases, and atelectasis of neighbor lobe/lobes in 7 cases. All 8 patients who had perfusion scintigraphy showed reduced perfusion in the affected lobe. Narrowed and flaccid bronchi were detected in one patient by using flexible bronchoscopy. Blood gas analysis was performed in 11 patients, and hypoxia and hypercarbia were revealed in 9 and 7 of these patients, respectively. The most common affected lobe was the left upper lobe (57%), followed by the right upper lobe (30%) and right middle lobe (27%). Two lobes were involved in 4 patients. Associated abnormalities were observed in 5 patients. Twenty-one patients underwent lobectomy; 9 were followed conservatively. Ages at diagnosis were significantly younger in surgically treated patients. Emphysema was detected in all pathological specimens, with an additional bronchial cartilage deficiency in 2 patients. In the surgically treated group, 2 patients died and 2 patients were lost to follow-up. In the conservatively treated group, one patient was lost to follow-up. Mean follow-up duration of all patients was 63.2 +/- 56.2 months (range, 1-209 months). At follow-up visits, all patients were doing well. In surgically treated patients, chest X-rays were normal (9 cases), or showed hyperlucency on the operated side (6 cases) or chronic changes in the operation area (2 cases). Hyperexpansion in the affected lobe was found to be reduced in all cases in the conservatively treated group.     

Assessment of bronchial wall thickening on posteroanterior chest radiographs in acute asthma.

A central bronchus that is readily visible end-on in approximately 50% of normal frontal chest radiographs is the bronchus to the anterior segment of either upper lobe. Bronchial wall thickening, or "cuffing," is considered to be a radiographic sign of an asthmatic exacerbation and is cited as a useful sign in a number of leading textbooks; however, to the authors' knowledge, no prior chest radiographic study has quantitatively assessed this specific sign in a population of asthmatics suffering an acute exacerbation. Posterior chest radiographs were reviewed retrospectively for 51 nonasthmatic, nonsmoking control subjects and for 45 adult asthmatic subjects during an acute exacerbation of moderate to severe asthma. Readers were blinded as to whether the radiograph was from an asthmatic or control subject. If visible end-on, the bronchus to the anterior segment of either upper lobe was assessed by measuring the diameter of the lumen and the thickness of the bronchial wall. At least one clearly defined bronchus to the anterior segment of an upper lobe was visible end-on in 22 patients (43%) in the control group and in 21 patients (47%) in the asthma group (p = NS). Mean wall thickness was 0.7 +/- 0.1 mm in the control group and 0.8 +/- 0.1 mm in the asthma group (p = 0.04). Lumen/wall thickness was 3.1 +/- 0.2 (SEM) in the control group and 2.5 +/- 0.2 in the asthma group (p = 0.055). The presence of bronchial wall thickness does not reliably distinguish radiographs of acutely asthmatic from normal individuals.     

电磁导航支气管镜联合径向超声对肺外周病变的诊断价值

目的 探讨电磁导航支气管镜(ENB)联合径向超声(EBUS)对肺外周病变的诊断价值.方法 纳入2018年1月至2019年7月就诊唐都医院呼吸科的肺外周病变32例,筛除最终诊断不明确的和未探及活检的6例,对比入选26例诊断率及确诊病例临床特征分层关系和未确诊病例临床特征.结果 ENB+EBUS确诊17例,诊断率65.4％...     

Bronchoscopic thermal vapour ablation therapy in the management of heterogeneous emphysema

The need for a less invasive procedure than surgical lung volume reduction that can produce consistent improvements with reduced morbidity remains a medical goal in patients with emphysema. We sought to determine the effect of bronchoscopic thermal vapour ablation (BTVA) on lung volumes and outcomes in patients with emphysema. 44 patients with upper lobe-predominant emphysema were treated unilaterally with BTVA. Entry criteria included: age 40-75 yrs, forced expiratory volume in 1 s (FEV(1)) 15-45% predicted, previous pulmonary rehabilitation and a heterogeneity index (tissue/air ratio of lower lobe/upper lobe) from high-resolution computed tomography (HRCT) ≥ 1.2. Changes in FEV(1), St George's Respiratory Questionnaire (SGRQ), 6-min walk distance (6 MWD), modified Medical Research Council (mMRC) dyspnoea score, and hyperinflation were measured at baseline, and 3 and 6 months post-BTVA. At 6 months, mean ± SE FEV(1) improved by 141 ± 26 mL (p<0.001) and residual volume was reduced by 406 ± 113 mL (p<0.0001). SGRQ total score improved by 14.0 ± 2.4 points (p<0.001), with 73% improving by ≥ 4 points. Improvements were observed in 6 MWD (46.5 ± 10.6 m) and mMRC dyspnoea score (0.9 ± 0.2) (p<0.001 for both). Lower respiratory events (n=11) were the most common adverse event and occurred most often during the initial 30 days. BTVA therapy results in clinically relevant improvements in lung function, quality of life and exercise tolerance in upper lobe predominant emphysema.     

Bronchogenic cyst: an uncommon cause of congenital lobar emphysema

We report a case of a 1-month-old boy who has developed respiratory distress. Chest X-ray and CT scan showed over distension of the left upper lobe and a mediastinal shift in favour of congenital lobar emphysema (CLE) of the left upper lobe. One month after uneventful lobectomy, he was readmitted at hospital for another episode of respiratory distress. Chest radiography revealed relapse of compressive emphysema in the remaining left lobe. Gastro oesophageal transit and MRI were performed, which have shown a mediastinal cystic mass. Accordingly, the patient underwent thoracotomy. Surgical examination found a subcarinal bronchogenic cyst which compressed the main left bronchus, causing the CLE of both upper and lower left lobes. Histological examination of removed cyst confirmed these data. Authors discuss causes of diagnostic delay.     

Computerized tomography in the evaluation of allergic bronchopulmonary aspergillosis

Allergic bronchopulmonary aspergillosis (ABPA) is a destructive disease of the lung characteristically associated with central bronchiectasis. This study was designed to determine if high-resolution computerized tomography (CT) could be used to define bronchiectasis in patients with suspected disease. Sixteen patients with asthma were studied. All demonstrated immediate cutaneous reactivity to common aeroallergens including Aspergillus fumigatus. Eight patients had clinical and immunologic evidence of ABPA. Six to 12 high-resolution CT sections (1.5 mm) were obtained at 1- to 2-cm intervals from the aortic knob to the dome of the diaphragm. All radiographs were evaluated separately by two readers in a blinded fashion and graded as to the presence and type of bronchial dilatation and bronchial wall thickening. Bronchial dilatation was seen in 41% of lung lobes in the ABPA group compared to 15% in the non-ABPA asthmatic control group. Upper lobe involvement and bronchial wall thickening was common to both groups. The presence of bronchiectasis in the control group may indicate that asthma is a more destructive lung disease than is currently appreciated. High-resolution CT of the chest has been shown in previous studies to have a sensitivity and specificity approaching that of bronchography, and the current findings support its use in the detection of bronchiectasis in patients with asthma suspected of having ABPA.     

Assessment of Bronchial Wall Thickening on Posteroanterior Chest Radiographs in Acute Asthma

A central bronchus that is readily visible end-on in approximately 50% of normal frontal chest radiographs is the bronchus to the anterior segment of either upper lobe. Bronchial wall thickening, or “cuffing,” is considered to be a radiographic sign of an asthmatic exacerbation and is cited as a useful sign in a number of leading textbooks; however, to the authors’ knowledge, no prior chest radiographic study has quantitatively assessed this specific sign in a population of asthmatics suffering an acute exacerbation. Posterior chest radiographs were reviewed retrospectively for 51 nonasthmatic, nonsmoking control subjects and for 45 adult asthmatic subjects during an acute exacerbation of moderate to severe asthma. Readers were blinded as to whether the radiograph was from an asthmatic or control subject. If visible end-on, the bronchus to the anterior segment of either upper lobe was assessed by measuring the diameter of the lumen and the thickness of the bronchial wall. At least one clearly defined bronchus to the anterior segment of an upper lobe was visible end-on in 22 patients (43%) in the control group and in 21 patients (47%) in the asthma group (p = NS). Mean wall thickness was 0.7 ± 0.1 mm in the control group and 0.8 ± 0.1 mm in the asthma group (p = 0.04). Lumen/wall thickness was 3.1 ± 0.2 (SEM) in the control group and 2.5 ± 0.2 in the asthma group (p = 0.055). The presence of bronchial wall thickness does not reliably distinguish radiographs of acutely asthmatic from normal individuals.     

34例支气管袖状肺叶切除治疗中心型肺癌的临床分析

目的:探讨支气管袖状肺叶切除术治疗34例中心型肺癌的临床疗效。方法:我科自2003年1月至2010年12月,进行了34例支气管袖状肺叶切除术,右肺上叶袖状切除14例,右中下肺叶袖状切除4例,左肺上叶袖状切除10例,左肺下叶袖状切除6例。结果:全组无死亡病例,并发症肺部感染14.7%(5/34),心律失常11.8%(4/34)。结论:支气管袖状肺叶除术提高了中心型肺癌的手术切除率,最大限度保留健康肺组织,提高了肺癌的治愈率和长期生存率,且不合并严重术后并发症。     

Selective lobar blockade using an arndt endobronchial blocker in 2 patients with respiratory compromise who underwent lung resection

Selective lobar blockade is an alternative to one-lung ventilation in thoracic surgery. We present 2 cases of lung resection with severe respiratory compromise. The first patient had previously undergone a left lower lobectomy and 2 atypical resections in the left and right upper lobes and was scheduled for a right lower lobectomy. The second patient presented chronic obstructive pulmonary disease with forced vital capacity of 1200 mL (26% of predicted value) and forced expiratory volume in 1 second of 820 mL (25% of predicted value) and was scheduled for an atypical resection of the left upper lobe with pleural abrasion. Selective lobar blockade was achieved in both cases using an Arndt endobronchial blocker. Ventilation during the operation was sufficient. Surgery was uneventful in both cases and lobar collapse was satisfactory.     

Evaluation of lung lobar ventilation dynamics with magnetic resonance imaging: a comparison of interstitial pneumonia patients with normal subjects]

Magnetic resonance imaging (MRI) was utilized to study lung lobar dynamic ventilation in 11 patients with interstitial pneumonia (IP) and 10 non-smoking men. The IP patients included 7 with interstitial lung disease associated with collagen vascular disease, 3 with idiopathic interstitial pneumonia, and 1 with lung cancer who was excluded from statistical analysis. We calculated lung lobar volumes in each phase from each dynamic image and constructed time-volume curves(TVCs). Lung lobar volume rates(%), fluctuation rates(%), lobar fluctuation rate/total lung fluctuation rate (%), and time lag (sec.) for the IP patients and normal subjects were calculated and compared. In the former, the mean volume rate for the right upper lobe was larger (p < 0.01) than that in normal subjects. The mean volume rate for the left lower lobe in the IP patients was smaller(p < 0.01) than that in the normal subjects. In IP patients, peak TVC for the right middle lobe appeared later (p < 0.01) than that in normal subjects. Although the fluctuation rates and fluctuation rate/total lung fluctuation rate for the lower lobes tended to be higher than those for the upper and middle lobes in normal subjects, this tendency was not distinct in IP patients. The quantitative evaluation of pulmonary ventilation dynamics with MRI may be a useful noninvasive technique for the assessment of lung lobar ventilation in patients with IP.     

Reperfusion after a two-hour period of pulmonary artery occlusion causes pulmonary necrosis.

The role of reperfusion injury in the progression to necrosis in pulmonary embolism was evaluated. To simulate this condition, we used a technique that enables occlusion and reopening of the pulmonary arterial branch supplying the right upper lobe in conscious rats. The rats were divided into five groups: the occlusion group (n = 12), in which the pulmonary artery (PA) branch was occluded without reperfusion; the reperfusion group (n = 12), in which the PA branch was reopened after a 2-h period of occlusion; and the reperfusion-SOD (n = 9), reperfusion-IM (n = 8), or reperfusion-IA-SOD (n = 6) groups, in which superoxide dismutase (SOD), indomethacin (IM), or inactivated SOD (IA-SOD), respectively, was administered during reperfusion. The lungs were removed 24 h after the PA occlusion, and histologic examination was performed. In the occlusion group, the alveolar structure of the right upper lobe was well preserved, and there was no erythrocyte or leukocyte accumulation. The only significant changes compared with the control lobe was the appearance of wavy internal elastic lamina of the PA and slight neutrophil adherence to the endothelial cells. In contrast, the right upper lobe of the reperfusion group disclosed numerous foci of hemorrhagic necrosis, with disrupted alveoli and leukocyte accumulation in all cases. With SOD treatment, the changes compatible with hemorrhagic necrosis were attenuated to the level of the control lobes. However, neither IM nor IA-SOD decreased these changes.(ABSTRACT TRUNCATED AT 250 WORDS)     

Regional distribution of macroscopic lung disease in cystic fibrosis

We used macroscopic morphometry and multivariate analysis (Hotelling's T2) to quantitate the extent of chronic lung disease and its regional distribution in formalin-inflated right lungs obtained at autopsy from 17 young adults with cystic fibrosis (CF) and 5 without CF as control subjects. Lungs were point-counted, by lobe, for 6 anatomic variables: parenchyma, nonparenchyma, bronchi, vessels, cysts, and emphysema, and the results were expressed for each as volume proportion (Vv). Considering all 6 variables simultaneously, CF lungs differed significantly from control lungs (p = 0.003). For individual variables, Vv bronchus was significantly elevated and Vv parenchyma was significantly reduced in the CF group. Within the CF lungs, there was a significant difference between upper and lower lobes when all variables were considered together (p = 0.001). For individual variables, Vv parenchyma and Vv vessels were significantly less, and Vv bronchus was significantly greater in the upper compared with the lower lobe. There was no difference in the upper versus lower lobe Vv for any variable in the control group. These results indicate that lung disease and remodeling are irregularly distributed in CF and that the upper lobe is disproportionately severely involved.     

Histopathologic variability in usual and nonspecific interstitial pneumonias.

Findings of surgical lung biopsy (SLB) are important in categorizing patients with idiopathic interstitial pneumonia (IIP). We investigated whether histologic variability would be evident in SLB specimens from multiple lobes in patients with IIP. SLBs from 168 patients, 109 of whom had multiple lobes biopsied, were reviewed by three pathologists. A diagnosis was assigned to each lobe. A different diagnosis was found between lobes in 26% of the patients. Patients with usual interstitial pneumonia (UIP) in all lobes were categorized as concordant for UIP (n = 51) and those with UIP in at least one lobe were categorized as discordant for UIP (n = 28). Patients with nonspecific interstitial pneumonia (NSIP) in all lobes were categorized as having fibrotic (n = 25) or cellular NSIP (n = 5). No consistent distribution of lobar histology was noted. Patients concordant for UIP were older (63 +/- 9 [mean +/- SD] yr; p < 0.05 as compared with all other groups) than those discordant for UIP (57 +/- 12 yr) or with fibrotic NSIP (56 +/- 11 yr) or cellular NSIP (50 +/- 9 yr). Semiquantitative high-resolution computed tomography demonstrated a varied profusion of fibrosis (p < 0.05 for all group comparisons), with more fibrosis in concordant UIP (2.13 +/- 0.62) than in discordant UIP (1.42 +/- 0.73), fibrotic NSIP (0.83 +/- 0.58), or cellular NSIP (0.44 +/- 0.42). Survival was better for patients with NSIP than for those in both UIP groups (p < 0.001), although survival in the two UIP groups was comparable (p = 0.16). Lobar histologic variability is frequent in patients with IIP, patients with a histologic pattern of UIP in any lobe should be classified as having UIP.     

Lung structure abnormalities, but normal lung function in pediatric bronchiectasis

BACKGROUND: Bronchiectasis is not considered to be uncommon in children anymore. The relationship between pulmonary function and severity of bronchiectasis is still controversial. STUDY OBJECTIVES: To assess the extent and severity of bronchiectasis through high-resolution CT (HRCT) scan score, and to correlate it with clinical, microbiological, and functional data. PATIENTS AND METHODS: Forty-three white children with HRCT-diagnosed bronchiectasis were studied. Bronchiectasis extent, bronchial wall thickening severity, and bronchial wall dilatation severity were evaluated using the Reiff score. Clinical, microbiological, and spirometry results were related to total HRCT scan score and to subscores as well. RESULTS: The percentages of affected lobes were as follows: right lower lobe, 65%; middle lobe, 56%; left lower lobe, 51%; right upper lobe, 37%; lingula, 30%; and left upper lobe, 30% (chi(2) = 18.4; p = 0.002). The mean (+/- SEM) HRCT score was 20 +/- 2.6. Total score or subscores of bronchiectasis extent, bronchial wall thickening severity, and bronchial wall dilatation severity were not significantly related to FEV(1) and FVC. Seventy-four percent of patients had asthma. The age at the onset of cough correlated with age at the time of the HRCT scan (p = 0.004) and with the presence of asthma (p = 0.01). Positive findings of deep throat or sputum cultures were found more frequently in atopic patients (p = 0.02) and asthmatic (p < 0.01) patients, and in children who were < 2 years of age at the onset of cough (p < 0.01). CONCLUSIONS: Normal lung function may coexist with HRCT scan abnormalities and does not exclude damage to the bronchial structure. Pulmonary function is not an accurate method for assessing the severity of lung disease in children with bronchiectasis.     

The elastic properties of lobes of excised human lungs.

Static pressure-volume curves were obtained from the upper and lower lobes of 5 excised human lungs. Comparison of the upper and lower lobe pressure-volume curves showed a small but significant difference. The upper lobes contained a greater percentage of total lobar capacity above approximately 80 per cent of the lung volume. It would thus appear that the difference in lobar elastic properties could influence ventilation distribution in normal humans.     

耐多药肺结核并发糖尿病患者胸部CT扫描特征分析

目的 分析耐多药肺结核并发糖尿病患者与未并发糖尿病患者的胸部CT表现,以提高临床认识。方法 通过《首都医科大学附属北京胸科医院病案电子化管理系统》,搜集2012—2017年经改良罗氏培养基痰培养及绝对浓度法药物敏感性试验(简称“药敏试验”)确诊的耐多药肺结核且符合排除标准的660例患者资料,按照是否并发糖尿病将患者分为并发糖尿病组(159例,并发组)、未并发糖尿病组(501例,未并发组)。采用数字表法从两组患者中分别随机抽取60例患者进行对照研究,由2名影像科经验丰富医师在不知患者是否并发糖尿病的情况下重新阅读CT片,以记录胸部CT表现及病变分布范围。采用SPSS 17.0软件进行统计学分析,计数资料采用卡方检验或Fisher精确概率检验,计量资料采用非参数检验方法中的曼-惠特妮U检验,以P<0.05为差异有统计学意义。结果 并发组患者中的段或叶性实变[48.3%(29/60)]、大结节[26.7%(16/60)]、任何形式空洞[93.3%(56/60)]、胸腔积液[40.0%(24/60)]的发生率明显高于未并发组患者[分别为28.3%(17/60)、11.7%(7/60)、76.7%(46/60)、20.0%(12/60)](χ ²=5.08,P=0.024;χ ²=4.36,P=0.037;χ ²=6.54,P=0.011;χ ²=5.71,P=0.017),而支气管扩张[45.0%(27/60)]的发生率明显低于未并发组[70.0%(42/60)](χ ²=7.67,P=0.006);且并发组患者中的段性或叶性实变累及叶数[(0.78±0.96)个]、大结节累及肺叶数[(0.48±0.96)个]和任何形式空洞累及叶数[(2.05±1.43)个]明显多于未并发组[分别为(0.38±0.72)个、(0.13±0.39)个、(1.58±1.43)个](U=1380.50,P=0.011;U=1511.00,P=0.027;U=1432.00,P=0.046),而支气管扩张累及肺叶数[(0.97±1.37)个]明显少于未并发组[(1.58±1.57)个](U=1335.50,P=0.010)。结论 耐多药肺结核并发糖尿病患者CT扫描出现段以上肺实变的频率及分布范围、出现大结节和空洞的频率及累及范围、并发胸腔积液的频率均较未并发糖尿病患者高;而支气管扩张多见于未并发糖尿病患者。     

不同剂型糖皮质激素对支气管哮喘合并糖尿病患者临床效果与胰岛β细胞功能的影响

目的探讨不同剂型糖皮质激素对支气管哮喘合并糖尿病患者临床效果和胰岛β细胞功能以及血糖水平的影响。方法采用该院2019年8月—2020年8月接收的84例支气管哮喘合并糖尿病患者作为研究对象,按照数字表评分法将患者划为对照组和研究组,每组42例。对照组患者使用口服糖皮质激素进行治疗,研究组患者使用吸入性糖皮质激素进行治疗,比较两组患者治疗后的临床效果。结果研究组患者临床治疗有效率为97.62%,空腹血糖(7.01±0.01)mmol/L、餐后2 h血糖(7.87±0.30)mmol/L、糖化血红蛋白(7.05±0.27)%、胰岛素抵抗指数(2.82±0.77)、MI指数(4.00±0.66)、胰岛β细胞功能(3.56±0.70),各项数据与对照组进行比较差异有统计学意义(χ²=4.480,P=0.034;t=17.979、28.923、24.187、8.270、10.183、9.155,P<0.001)。结论为支气管哮喘合并糖尿病患者使用吸入性糖皮质激素治疗效果更佳。     

Application of lobe-selective bronchial blockade against airway bleeding

A 48-year-old man underwent resection of a right upper lobe necrotic tumor and part of his chest wall. Lobe-selective bronchial blockade of bleeding from the right upper lobe was achieved by combining a left-side double-lumen endotracheal tube with a bronchial blocker placed at the right intermediate bronchus. The bleeding right upper lobe was isolated, and the other lobes were protected from blood contamination during the lobectomy procedure.     

Compensatory increases in residual lobar volume following lung resection]

To investigate compensatory increases in residual lobar volume after lobectomy and pneumonectomy, we measured lung lobar volumes on the basis of pre- and postoperative computed tomographic (CT) images obtained on 40 patients (11 right upper, 7 right lower, 10 left upper, 8 left lower lobectomies and 4 left pneumonectomies). A personal computer image processing program was utilized to calculate lung lobar volumes from sequential CT images. Decreases in whole lung volume after lung resection averaged from 7.4% to 9.5% of preoperative whole lung volume in the lobectomy patients, and 30% in the patients who underwent left pneumonectomy. Those values were much smaller than the volumes of resected lobe, as measured on the basis of preoperative CT images. Increased residual lobar volume after lung resection averaged from 11% to 15% of preoperative whole lung volume in both the lobectomy and left pneumonectomy patient groups. Residual lobes compensated for approximately 60% of the resected lobar volume in the lobectomy patients, but only about 30% of resected lung volume in the left pneumonectomy patients. Increases in residual lobar volume tended to be larger in patients who underwent upper lobectomies, and on the operative side in patients other than those who underwent left lower lobectomies. We concluded that compensatory increases in residual lobar volume should be taken into full consideration when making predictions about postoperative pulmonary function.