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Role of lung maintenance program in the heterogeneity of lung destruction in emphysema.

Centrilobular emphysema caused by chronic cigarette smoking is a heterogeneous disease with a predominance of upper lobe involvement. It is presumed that this heterogeneity indicates a particular susceptibility to cigarette smoke or the fact that the inhaled smoke distributes preferentially to upper lung zones. The less involved areas might therefore retain the capacity for lung regeneration and gain of pulmonary function in terminally ill patients. We propose that the interplay between molecular and cellular switches involved in the lung response to environmental injuries determines the heterogeneous pattern of emphysema due to cigarette smoke. Regional activation of alveolar destruction by apoptosis and oxidative stress coupled with regional failure of defense mechanisms may account for the irregular pattern of lung destruction in cigarette smoke-induced emphysema. Protection afforded by the key antioxidant transcription factor Nrf-2 and the antiproteolytic and antiapoptotic actions of alpha(1)-antitrypsin is central to maintain lung homeostasis and lung structure. As the lung is injured by environmental pollutants, including cigarette smoke, molecular sensors of cellular stress, such as the mTOR/protein translation regulator RTP-801, may engage both inflammation and alveolar cell apoptosis. As injury prevails during the course of this chronic disease, it leads to a more homogeneous pattern of lung disease.

Apoptosis↗

Functional levels of alpha 2-macroglobulin in plasma in relation to emphysema.

In most studies, concentrations of alpha 2-macroglobulin are determined by immunological techniques. In this study, the amidolytic activity of porcine pancreatic elastase complexed with alpha 2-macroglobulin was measured using an elastase-specific substrate, succinyl-trialanyl-p-nitroanilide. The activities of plasmas from 47 emphysema cases were compared with 39 normal subjects. The age ranges of both groups were from 50 to 84 years. The mean activity of bound elastase in emphysema cases was 2.48 +/- 0.03 kU/l of plasma. The mean for normal subjects was 1.48 +/- 0.11 kU/l of plasma. The difference was very significant (2P less than 0.001). All except 2 of the emphysema cases had smoked. The same results were obtained when only people who had smoked for 25 years or more were included in the analysis. All the plasma samples of people included in the study were assayed by an immunological method for absolute level (in g/l) of alpha 1-proteinase inhibitor. The levels of alpha 1-proteinase inhibitor for all the persons studied fell within the normal range for MM-phenotypes (2 to 4 g/l).

Aged↗

Surgical management of infants with congenital lobar emphysema and concomitant congenital heart disease.

OBJECTIVE: Congenital lobar emphysema (CLE) is an uncommon cause of infantile respiratory distress. It is diagnosed on the basis of evidence of lobar overaeration, mediastinal shift, and compression of the adjacent lobe. Concomitant congenital heart disease (CHD) and CLE is not uncommon. In the literature a 12% to 20% concomitance rate is given. The optimal treatment of respiratory symptoms associated with CLE and CHD is not clear; however, there has been a great deal of progress in the treatment of CLE and CHD. The aim of this study was to evaluate a clinical experience with and long-term follow-up of the surgical treatment of 13 patients with concomitant CLE and CHD. MATERIAL AND METHODS: We reviewed the cases of 13 patients with concomitant CLE and CHD. The medical records were evaluated with reference to age, type of CHD, pulmonary artery pressure, clinical symptoms, and results of surgical management. RESULTS: One patient died. This patient had ventricular septal defect (VSD) and left upper lobe emphysema in the postoperative period. The remaining patients undergoing follow-up were clinically well at the final evaluation. Postoperative thoracic computed tomography revealed complete spontaneous regression of emphysema 3 months after division of ductus arteriosus in 1 patient. Pulmonary hypertensive episode was seen in 3 patients after the early postoperative period. Five of the patients were discharged with bronchodilator treatment after surgery. Six patients needed positive inotropic support. Among the patients with pulmonary hypertension and those with VSD who had undergone cardiopulmonary bypass, we found a greater need for inotropic support, a higher risk of postoperative infection, and a longer intubation period. Echocardiography in the late postoperative revealed decreased pulmonary artery diameter and pressure; myocardial performance was normal. Results of blood gas analyses revealed increased oxygen saturation and decreased partial pressure of carbon dioxide. Normal exercise activity was found in all patients. DISCUSSION: The presence of CHD, especially in infants with unusual respiratory distress symptoms, should be kept in mind, and echocardiography and/or cardiac catheterization should be considered in the diagnosis. In patients with high pulmonary artery pressure, palliative or corrective surgery for CHD in addition to lobectomy can be considered. We believe that for lesions without high pulmonary artery pressure, such as small atrial septal defect and patent foramen ovale, clinical follow-up is sufficient treatment after lobectomy. If the cause of CLE is compression of large ductus arteriosus, only division of the patent ductus arteriosus may be considered before lobectomy and clinical and radiologic follow-up. The cardiac lesion should be assessed as to severity and ease of management. A corrective procedure can be carried out at lobectomy. Because of the technical ease with which the cardiac operation can be performed at the time of lobectomy, we suggest that in addition to lobectomy, operative treatment of cardiac lesions be performed.

Child, Preschool↗

Low serum alpha-1-antitrypsin level as a contributory factor of combined emphysema in silicosis.

In order to evaluate low serum alpha-1-antitrypsin level as a contributory factor of combined emphysema in silicotic patients, serum alpha-1-antitrypsin analysis was carried out in 80 patients with silicosis. Low serum alpha-1-antitrypsin level was found in 5 patients. Large opacities were observed roentgenologically in only 1 out of these 5 cases in contrast to 31 of the other 75 cases. Also the suggestive findings for the emphysema were showed in 4 of these 5 cases while such findings were found on their chest X-ray films in only 43 of the other 75 cases. FEV 1.0% below 50 were calculated in 3 of the 5 cases, on the contrary in 22 of the 75 cases. RV above 50% predicted value was showed by all the 5 low antitrypsin patients, in contrast to only 25 of other 74 cases. The silicotic patients with low serum alpha-1-antitrypsin concentration are most likely to have an association with a high incidence of complicated emphysema.

Adult↗

Experimental pulmonary emphysema in Syrian hamsters.

Syrian hamsters were exposed to the aerosol of a 5% solution of papain in the presence of cysteine and ethylenediamine-tetraacetic acid. Pulmonary emphysema in hamsters was observed after exposure to papain for two to three hours. There was no change with time in the severity of emphysema from the second through the fourth weeks after exposure to papain. Pathogenesis of pulmonary emphysema was discussed in relation to proteolysis of glycosaminoglycan-protein complex.

Animals↗

Pathogenesis and management of virus infection-induced exacerbation of senile bronchial asthma and chronic pulmonary emphysema.

The number of senile patients with therapy resistant bronchial asthma, chronic pulmonary emphysema increases due to the habit of smoking and increased number of older people, and these inflammatory pulmonary diseases are the leading causes of death worldwide. Rhinoviruses cause the majority of common colds, and provoke exacerbations of bronchial asthma and chronic pulmonary emphysema. Here, I review the pathogenesis and management of rhinovirus infection-induced exacerbation of senile bronchial asthma and chronic pulmonary emphysema.

Aged↗

The role of free radicals and neutrophil elastase in development of pulmonary emphysema.

Extracellular proteolysis is hypothesized to be the major cause of pulmonary emphysema and oxygen-derived free radicals and neutrophil elastase are thought to play an important role in its pathogenesis. In this study, peripheral polymorphonuclear leukocytes (PMNs) obtained from 16 patients with emphysema generated a significantly larger amount of superoxide and elastase activity than those obtained from normal controls. A significant correlation was observed between elastase activity and superoxide release. In addition, the superoxide release showed a negative correlation with the disease duration. The superoxide release appeared to correlated with a decline of FEV1.0 over the course of several years in 8 patients. It seems likely that activated PMNs play an important role in the development of pulmonary emphysema.

Aged↗

Effects of muscle relaxation therapy using specially designed plates in patients with pulmonary emphysema.

It has been suggested that respiratory muscle dysfunction plays a major role in the development of acute ventilatory failure in patients with chronic obstructive pulmonary disease (COPD). In this study, we devised a respiratory muscle relaxation maneuver using wedge-shaped wooden plates, with which pressure was exerted on the intercostal and accessory respiratory muscles by hand, or by tapping with a wooden hammer, for 15-20 minutes twice a day. The muscle relaxation maneuver was performed in 5 moderate to severe pulmonary emphysema patients for 4 weeks and in 7 emphysema patients for 6 weeks, and the effects on pulmonary function were examined. After the therapy, inspiratory capacity (IC) and vital capacity (VC) in both the 4 weeks-and 6 weeks-treated groups, and the forced expiratory volume in 1 second (FEV 1.0) in the 6 weeks-treated group were significantly increased, and CO2 retention had also improved. Daily peak expiratory flow (PEF) showed significant increases from 2 weeks after the initiation of the therapy. These results suggest that the respiratory muscle relaxation maneuver is effective for improving the pulmonary function of pulmonary emphysema patients.

Aged↗

Usefulness of the double-wall sign in detecting pneumothorax in patients with giant bullous emphysema.

OBJECTIVE: We describe a new sign improving detection of pneumothorax in patients with giant bullous emphysema: air surrounding both sides of the bulla wall (the intrathoracic equivalent of the double-wall sign of pneumoperitoneum). We report the radiographic and CT appearances of the double-wall sign in seven patients with giant bullous emphysema, four of whom had pneumothorax. CONCLUSION: Recognizing the double-wall sign of pneumothorax should aid in the triage of patients with giant bullous emphysema.

Adult↗

[The role of oxidative stress in the pathogenesis of pulmonary emphysema].

OXIDATIVE PULMONARY DAMAGE: The pathogenesis of pulmonary emphysema is incompletely understood. Nearly 90% of all patients with chronic obstructive pulmonary diseases are smokers. Cigarette smoke is a rich source of oxidants. Oxidative stress increases oxidant generation, which cannot be neutralized with antioxidant defense mechanisms. Lipids, proteins and deoxyribonucleic acid are components of the cell that are most sensitive to oxidative damage. Oxygen radicals can modify amino acid side chains, form protein aggregates, cleave peptide bonds, and make proteins more susceptible to proteolytic degradation. It has been shown that neutrophils have a principal effector role in pulmonary tissue damage. Neutrophil elastase can damage air spaces by degrading elastin, and a variety of extracellular membrane proteins, proteoglycans, and glycoproteins. Neutrophil elastase can also stimulate inflammation by increasing interleukin-8 synthesis. Additionally, neutrophil elastase can activate or inactivate inhibitors of neutrophil collagenase, and secretory leukoprotease proteinase inhibitor. Apart from neutrophils, oxidative stress causes activation of other phagocytes and severe inflammatory response ensues. LIPID PEROXILATION AND PULMONARY EMPHYSEMA: Except protein oxidation and lipid peroxidation, oxidants may disturb signal transmission in the cells, as well as normal cell membrane function and function of organelles. Modified structure of deoxyribonucleic acid may cause mutations, which in absence of repairation enzyme activity lead to cell injury. IRON AND OXIDATIVE STRESS: Iron metabolism is also important in the development of pulmonary emphysema due to its role in production of some oxidants.

Humans↗

[Subcutaneous emphysema as a complication of tooth extraction].

Subcutaneous emphysema is a rare complication in dentistry, which may lead to diagnostic errors and inadequate therapy. A 17 year old female patient, in whom the separation of tooth roots was performed by the use of air-powered drill during the extraction of the first right lower molar, is presented in this paper. During the intervention, swelling of the right half of the face and the lower eyelid suddenly occurred, accompanied with simultaneous feeling of choking, and pressure in the neck and chest. Because of the suspicion of the allergic reaction, the patient was administered antihistaminic agent, together with parenteral corticosteroid, and was sent to an institution where she was treated as an in-patient. During hospitalization, subcutaneous emphysema of the face and neck was diagnosed by physical and x-ray examination. The emphysema completely disappeared after the use of oxygen and antibiotics.

Adolescent↗

Quality of life before and after lung transplantation in patients with emphysema versus other indications.

Whether lung transplantation improves Health-related Quality of Life in patients with emphysema and other end-stage lung diseases before and after lung transplantation was examined. Between 1992 and 1999, 23 patients with emphysema and 19 patients with other indications completed self-administered questionnaires before lung transplantation, and at 4, 7, 13, and 25 mo. after transplantation. The questionnaire included the Nottingham Health Profile, the State-Trait Anxiety Inventory, the Self-rating Depression Scale, the Index of Well-being, the self-report Karnofsky Index, and four respiratory-specific questions. Neither before nor after transplantation were significant differences found on most dimensions of Health-related Quality of Life between patients with emphysema and other indications. Before transplantation, both groups report major restrictions on the dimensions Energy and Mobility of the Nottingham Health Profile, low experienced well-being, depressive symptoms, and high dyspnea. About 4 mo. after transplantation, most Health-related Quality of Life measures improved significantly in both groups. These improvements were maintained in the following 21 mo.

Activities of Daily Living↗

5-Hydroxytryptamine uptake by lungs of hamsters with pulmonary emphysema.

Elastase-induced emphysema in hamsters was used as a model to determine whether this type of chronic lung injury affected the metabolic function of pulmonary endothelium. 5-Hydroxytryptamine (5-HT) uptake was studied in isolated perfused lungs of hamsters from a control group and from those with emphysema of various severity. After measuring 5-HT uptake, we determined the mean linear intercept and internal surface area for each lung. The concentration of 5-HT used in the perfusate ranged from the level in the resting hamster to supraphysiologic levels. The results reveal that elastase-induced emphysema does not affect 5-HT uptake by the isolated perfused hamster lung despite significant loss of alveolar surface area.

Animals↗

A case of localized persistent interstitial pulmonary emphysema.

Interstitial pulmonary emphysema is a well-documented complication of assisted mechanical ventilation in premature infants with respiratory distress syndrome. Localized persistent interstitial pulmonary emphysema (LPIPE) confined to a single lobe was incidentally presented in a 4-day-old female infant. This patient was a normal full-term baby with no respiratory distress symptom and no experience of assisted mechanical ventilation. Chest radiograph showed radiolucent area in right lower lobe zone, which needed differential diagnosis from other congenital lesions such as congenital cystic adenomatoid malformation and congenital lobar emphysema. CT scan showed irregular-shaped air cystic spaces and pathologically, cystic walls primarily consisted of compressed lung parenchyma and loose connective tissue intermittently lined by multinucleated foreign body giant cells.

Diagnosis, Differential↗

Lung transplantation in patients with pulmonary emphysema.

Lung transplantation is a viable option for patients with chronic obstructive pulmonary disease (COPD), and emphysema is the most common indication to undergo lung transplantation. A total of seven lung and one heart-lung transplantations were performed between July 1996 and June 2004 at the Yongdong Severance Hospital, and herein, three emphysema patients who underwent single lung transplantations are reviewed. There were 2 males and 1 female, with a mean age of 50 years (35, 57 and 58 years). They all underwent an operation, without cardiopulmonary bypass, and there was no operative mortality. The mean survival was 12 months (4 months, 15 months and 17 months) and all succumbed to death due to activation of pulmonary tuberculosis, post-transplantation lymphoproliferative disease and cytomegalovirus (CMV) gastritis associated with asphyxia. Infection was the most common postoperative complication, resulting in longer hospital stays, higher medical expenses and shorter survival rates, necessitating aggressive prophylactic management. The accumulation of experience, modifications to operative procedures and perioperative care may lead to improved early and long-term survival in patients with emphysema undergoing single or bilateral lung transplantations.

Adult↗

Endoscopic ultrasonographic appearance of gastric emphysema.

Emphysematous gastritis (or phlegmonous gastritis) and gastric emphysema (or gastric pneumatosis) are variations of conditions associated with the presence of intramural air in the stomach. The presence of air in the gastric wall is a very rare clinical condition, associated with bacterial infection, increased intragastric pressure from gastric outlet obstruction, gastric mucosal disruption or air dissection from the mediastinum. In adults, this can occur in the setting of instrumentation-related injury, gastric outlet obstruction by gastric, duodenal or pancreatic malignancies or bowel ischemia. Here we describe a case of gastric emphysema related to repeated biliary stenting and partial duodenal obstruction in a patient with inoperable periampullary cancer, and provide the first description of the endoscopic ultrasonographic findings of gastric emphysema in the literature. In our case, endoscopic ultrasound showed a band of bright echogenicity arising from the submucosa layer, representing air in the gastric wall.

Aged↗

Emphysema in nonsmokers: alpha 1-antitrypsin deficiency and other causes.

Nonsmokers with signs of emphysema at an earlier age than is typical for emphysema deserve a workup for one of the less common causes of emphysema, which include alpha 1-antitrypsin deficiency, connective tissue diseases, hypocomplementemic urticarial vasculitis syndrome, intravenous drug use, human immunodeficiency virus infection, and several rare metabolic disorders.

Age of Onset↗

Lung volume reduction surgery for emphysema: out on a limb without a NETT.

Lung volume reduction surgery (LVRS) has recently been rediscovered and offers the potential of improving the quality of life of patients with advanced emphysema. In this article, we discuss the historical and contemporary versions of LVRS. Although initial enthusiasm has been substantial, existing data seem insufficient to demonstrate the safety and efficacy of the procedure in comparison with conventional medical therapy. Fundamental questions remain regarding the long-term effects of an operation versus medical therapy, the optimal selection criteria, the best measures of efficacy, the mechanisms of improvement, the cost-effectiveness of the procedure, and the optimal surgical technique. Until such questions are answered, advising patients about the best management their emphysema will be difficult. The National Emphysema Treatment Trial will address many of these issues and should be embraced by both health-care providers and patients.

Forced Expiratory Volume↗