• Bulletin of the Korean Chemical Society
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• v.35 no.9
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• pp.2781-2786
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• 2014

Human ${\alpha}_1$-antitrypsin (${\alpha}_1$-AT) is a natural inhibitor of neutrophil elastases and has several dozens of genetic variants. Most of the deficient genetic variants of human ${\alpha}_1$-AT are unstable and cause pulmonary emphysema. However, the most clinically significant variant, Z-type ${\alpha}_1$-AT, exhibits retarded protein folding that leads to accumulation of folding intermediates. These aggregate within the endoplasmic reticulum (ER) of hepatocytes, subsequently causing liver cirrhosis as well as emphysema. Here, we studied the role of an ER folding assistant protein Cpr5p on Z-type ${\alpha}_1$-AT folding. Cpr5p was induced > 2-fold in Z-type ${\alpha}_1$-AT-expressing yeast cells compared with the wild type. Knockout of CPR5 exacerbated cytotoxicity of Z-type ${\alpha}_1$-AT, and re-introduction of CPR5 rescued the knockout cells from aggravated cytotoxicity caused by the ${\alpha}_1$-AT variant. Furthermore, Cpr5p co-immunoprecipitated with Z-type ${\alpha}_1$-AT and facilitated its protein folding. Our results suggest that protein-folding diseases may be suppressed by folding assistant proteins at the site of causal protein biosynthesis.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1976.06a
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• pp.90.4-90
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• 1976

Foreign bodies of the esophagus are frequently seen in otolaryngological fields and there are numerous reports about the cases of esophageal foreign bodies in the literatures. It is well known and agreed to most of the authors that the most common subjects of foreign bodies in the esophagus are coins in young children. The authors recently experienced a rare case of foreign body in the first narrowing of the esophagus in a l-year-old female who visited with the complaints of fever, dysphagia and imspiratory dyspnea for 5 days, complicating subcutaneous emphysema, periesophageal abscess, mediastinal emphysema, mediastinitis and lung atelectasis causing death.

• Tuberculosis and Respiratory Diseases
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• v.46 no.2
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• pp.260-265
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• 1999

The first case of allergic bronchopulmonary aspergillosis(ABPA) was reported by Hinson, et al. in 1952. This was followed by a number of significant description of the disorder. Although typical ABP A initially presents with asthma, fleeting pulmonary infiltrates, and marked eosinophilia, there are many other ways in which the disease may be first manifested. Common radiologic findings in ABP A include pulmonary infiltrates, atelectasis, emphysema, fibrosis, lobar shrinkage with hilar elevation, cavitation, pneumothorax, aspergilloma and central bronchiectasis. We experienced a case of allergic bronchopulmonary aspergillosis presenting rare radiologic finding of bilateral pulmonary masses in chest radiography. With oral corticosteroid treatment, the size of both pulmonary masses was decreased significantly and his asthmatic symptoms were improved.

• Journal of Chest Surgery
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• v.32 no.1
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• pp.38-42
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• 1999

Background: In neonatal respiratory distress syndrome patients, various types of pulmonary air leak contributes to elevate morbity and mortality. Although early surgical interventions can provide better results in several cases, whole clinical outcomes are poor. This study was designed to investigate the clinical aspects of pulmonary air leak in the neonatal respiratory distress syndrome patients and major contributing factors to mortality. Material and Method : We retrospectively evaluated 48 cases of pulmonary air leak in the neonatal respiratory distress syndrome patients from September 1994 to May 1997. Result: There were 15 cases of primary and 33 cases of secondary pulmonary air leakages. The prominent manifestations were pure interstitial emphysema in 19 cases(39.9%) and combined pneumothorax in 19 cases(39.9%). In clinical aspects, normal birth weight was dominant(83.4%), the onset occurred within 24 hours was in 28 cases(58.8%). The pulmonary diseases were meconium aspiration syndrome(25.2%) and hyaline membrane disease(33.2%). The overall hospital mortality was 25.2%, and the majority were hyaline membrane diseases. Conclusion: Although the overral mortality rate of these diseases were high, more detailed studies about immediate treatement, perinatal prevention, intensive care to geriatric problems were needed individually to improve outcomes.

• Tuberculosis and Respiratory Diseases
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• v.71 no.3
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• pp.202-209
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• 2011

Background: A tuberculous destroyed lung is sequelae of pulmonary tuberculosis and causes various respiratory symptoms and pulmonary dysfunction. The patients with a tuberculous destroyed lung account for a significant portion of those with chronic lung disease in Korea. However, few reports can be found in the literature. We investigated the computed tomography (CT) findings in a tuberculous destroyed lung and the correlation with lung function. Methods: A retrospective analysis was carried out for 44 patients who were diagnosed with a tuberculous destroyed lung at the Keimyung University Dongsan Hospital between January 2004 and December 2009. Results: A chest CT scan showed various thoracic sequelae of tuberculosis. In lung parenchymal lesions, there were cicatrization atelectasis in 37 cases (84.1%) and emphysema in 13 cases. Bronchiectasis (n=39, 88.6%) was most commonly found in airway lesions. The mean number of destroyed bronchopulmonary segments was 7.7 (range, 4~14). The most common injured segment was the apicoposterior segment of the left upper lobe (n=36, 81.8%). In the pulmonary function test, obstructive ventilatory defects were observed in 31 cases (70.5%), followed by a mixed (n=7) and restrictive ventilatory defect (n=5). The number of destroyed bronchopulmonary segments showed a significant negative correlation with forced vital capacity (FVC), % predicted (r=-0.379, p=0.001) and forced expiratory volume in one second ($FEV_1$), % predicted (r=-0.349, p=0.020). After adjustment for age and smoking status (pack-years), the number of destroyed segments also showed a significant negative correlation with FVC, % predicted (B=-0.070, p=0.014) and $FEV_1$, % predicted (B=-0.050, p=0.022). Conclusion: Tuberculous destroyed lungs commonly showed obstructive ventilatory defects, possibly due to bronchiectasis and emphysema. There was negative correlation between the extent of destruction and lung function.

• Korean Journal of Radiology
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• v.19 no.6
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• pp.1187-1195
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• 2018

Objective: To compare correlations between pulmonary function test (PFT) results and different reconstruction algorithms and to suggest the optimal reconstruction protocol for computed tomography (CT) quantification of low lung attenuation areas and airways in healthy individuals. Materials and Methods: A total of 259 subjects with normal PFT and chest CT results were included. CT scans were reconstructed using filtered back projection, hybrid-iterative reconstruction, and model-based IR (MIR). For quantitative analysis, the emphysema index (EI) and wall area percentage (WA%) were determined. Subgroup analysis according to smoking history was also performed. Results: The EIs of all the reconstruction algorithms correlated significantly with the forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) (all p < 0.001). The EI of MIR showed the strongest correlation with FEV1/FVC (r = -0.437). WA% showed a significant correlation with FEV1 in all the reconstruction algorithms (all p < 0.05) correlated significantly with FEV1/FVC for MIR only (p < 0.001). The WA% of MIR showed the strongest correlations with FEV1 (r = -0.205) and FEV1/FVC (r = -0.250). In subgroup analysis, the EI of MIR had the strongest correlation with PFT in both eversmoker and never-smoker subgroups, although there was no significant difference in the EI between the reconstruction algorithms. WA% of MIR showed a significantly thinner airway thickness than the other algorithms ($49.7{\pm}7.6$ in ever-smokers and $49.5{\pm}7.5$ in never-smokers, all p < 0.001), and also showed the strongest correlation with PFT in both ever-smoker and never-smoker subgroups. Conclusion: CT quantification of low lung attenuation areas and airways by means of MIR showed the strongest correlation with PFT results among the algorithms used, in normal subjects.

• Tuberculosis and Respiratory Diseases
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• v.71 no.2
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• pp.126-133
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• 2011

Unilateral pulmonary artery hypoplasia (UPAH) is a rare disease in adults and is frequently accompanied by a congenital cardiac anomaly at a young age. The diagnosis is usually based on computed tomography (CT), angiography, and magnetic resonance imaging (MRI). However, no reports are available on retrograde flow in patients with UPAH. We describe a 68-year-old man with isolated UPAH and retrograde blood flow. He was admitted for dyspnea on exertion for the past 23 years. His diagnosis was delayed, as his symptoms and signs mimicked his underlying pulmonary diseases, such as emphysema and previous tuberculous pleurisy sequelae. A discrepancy was detected between the results of a ventilation-perfusion scan and the CT image. This was resolved by MRI, which showed retrograde blood flow from the right to the left pulmonary artery. Using MRI, we diagnosed this patient with isolated pulmonary artery hypoplasia and retrograde flow.

• Tuberculosis and Respiratory Diseases
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• v.46 no.4
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• pp.489-499
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• 1999

Background: The patient with bronchiectasis may have obstructive ventilatory impairment combined with mild restrictive ventilatory impairment due to fibrosis of surrounding lung parenchyme and pleural adhesions caused by chronic recurrent pulmonary infections. Since hyperinflation or emphysematous change can be occured in bronchiectasis, pulmonary functions such as lung volumes and diffusing capacity may also vary with associated emphysema. Methods: For the evaluation of lung volumes and diffusing capacity in bronchiectasis with respect to the anatomic types and severity of bronchiectasis, a total of 40 cases comprising 24 cases of tubular, and 16 cystic type of bronchiectasis were analyzed retrospectively. Correlation between lung functions and extent of bronchiectasis or associated emphysema detected in HRCT were also evaluated. Results: Vital capacity(VC) tended to decrease in cystic type than in tubular type. As the severity of bronchiectasis became serious, the VC were significantly reduced, whereas the total lung capacity(TLC), residual volume(RV) and its ratio to the total lung capacity(RV/TLC) had no significant difference. Lung clearance index(LCI) was significantly increased in cystic type than in tubular type, whereas the slope of phase III in single breath nitrogen curve($\triangle$N2/L) was not significantly changed regard to the type and severity of bronchiectasis. DLCO and DLCO/VA reflecting diffusing capacity were significantly decreased in cystic type and also as the severity of bronchiectasis became serious. The correlation coefficient of VC, DLCO and LCI with the extent of bronchiectasis were -0.322, -0.339 and 0.487, respectively, whereas other parameters were not significantly correlated with the extent of bronchiectasis. VC and DLCO correlated negatively with the extent of emphysema while RV, RV/TLC, LCI and $\triangle$N2/L correlated positively. Conclusion: These findings suggest that the reduction of VC and diffusing capacity or uneven distribution of inspired gas in bronchiectasis are related to both the extent of bronchiectasis and associated emphysema while increased residual volume be related to the extent of associated emphysema alone.

• Tuberculosis and Respiratory Diseases
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• v.50 no.6
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• pp.693-703
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• 2001

Background : Smoking is the most important and consistent determinant of the development and progression of COPD(Ed Note : Define COPD). The fact that cigarette smokers develop a different type of COPD, chronic bronchitis and emphysema, with different clinical and pathological aspects, suggests that the development of COPD has a relationship with other smoking-associated factors beyond just a simple smoking history. The aim of this was to analyze the smoking habits and history of patients with COPD and to evaluate the development of different types of COPD according to patient's smoking habits. Method : To evaluate the differences in the smoking patterns of patients with chronic bronchitis and emphysema, a pulmonary function test was conducted, and the smoking history and patterns was obtained through a smoking history questionnaire by a direct personal interview from 333 male cigarette smokers diagnosed with COPD, in the Yeungnam university medical center(190 patients diagnosed with chronic bronchitis, 143 patients diagnosed with emphysema). Result : The patients with emphysema smoked earlier and had a higher smoking history(ie, more packyears, more total amounts of smoked cigarette, and more deep inhalation and longer duration of plain cigarette exposure) than those with chronic bronchitis. The depth of inhalation was also significantly higher in the emphysema patients after taking into account age, cumulative cigarette consumption and the type of cigarette smoked. Conclusion : Emphysema was more associated with the increasing degree of inhalation as assessed by the depth of inhalation. A high alveolar smoke exposure may be a significant risk factor for the development of emphysema.

• Korean Journal of Radiology
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• v.21 no.9
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• pp.1104-1113
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• 2020

Objective: To assess the regional ventilation in patients with asthma-chronic obstructive pulmonary disease (COPD) overlap syndrome (ACOS) using xenon-ventilation dual-energy CT (DECT), and to compare it to that in patients with COPD. Materials and Methods: Twenty-one patients with ACOS and 46 patients with COPD underwent xenon-ventilation DECT. The ventilation abnormalities were visually determined to be 1) peripheral wedge/diffuse defect, 2) diffuse heterogeneous defect, 3) lobar/segmental/subsegmental defect, and 4) no defect on xenon-ventilation maps. Emphysema index (EI), airway wall thickness (Pi10), and mean ventilation values in the whole lung, peripheral lung, and central lung areas were quantified and compared between the two groups using the Student's t test. Results: Most patients with ACOS showed the peripheral wedge/diffuse defect (n = 14, 66.7%), whereas patients with COPD commonly showed the diffuse heterogeneous defect and lobar/segmental/subsegmental defect (n = 21, 45.7% and n = 20, 43.5%, respectively). The prevalence of ventilation defect patterns showed significant intergroup differences (p < 0.001). The quantified ventilation values in the peripheral lung areas were significantly lower in patients with ACOS than in patients with COPD (p = 0.045). The quantified Pi10 was significantly higher in patients with ACOS than in patients with COPD (p = 0.041); however, EI was not significantly different between the two groups. Conclusion: The ventilation abnormalities on the visual and quantitative assessments of xenon-ventilation DECT differed between patients with ACOS and patients with COPD. Xenon-ventilation DECT may demonstrate the different physiologic changes of pulmonary ventilation in patients with ACOS and COPD.