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Survival benefit of cardiopulmonary bypass support in bilateral lung transplantation for emphysema patients.

BACKGROUND: This study is designed to examine a possible association of cardiopulmonary bypass (CPB) support and outcome of lung transplantation in a well-balanced group of emphysema patients. METHODS: We performed a retrospective analysis of 62 consecutive primary bilateral lung transplantations for emphysema. Risk factors for their possible association with patient survival were analyzed by multivariate logistic regression. RESULTS: The use of CPB support was associated with improved survival (odds ratio=0.25; P=0.038). The actuarial survival at 1 year was 97% for patients treated with CPB and 77% for patients treated without CPB support. In 28 patients (45%), 2 human leukocyte antigen (HLA)-DR mismatches between donor and recipient occurred, whereas 34 patients had 0 or 1 HLA-DR mismatches. The use of CPB support in the group with two HLA-DR mismatches was associated with improved survival (odds ratio=0.06; P=0.020). This association was not present in the group with 0 or 1 HLA-DR mismatches. CONCLUSIONS: These results demonstrate a significant survival benefit of CPB support during bilateral lung transplantation in emphysema patients. The difference in survival benefit of CPB support between the patients with 0 or 1 HLA-DR mismatches and the patients with 2 HLA-DR mismatches indicates that the immunosuppressive effect of CPB support might be responsible for this survival benefit. The underlying immunological mechanism might be important in the future treatment of organ transplantation.

Cardiopulmonary Bypass↗

A surgical approach to treating emphysema: lung volume reduction surgery.

Chronic obstructive pulmonary disease (COPD) is the fourth leading cause of death in Americans. In addition, COPD significantly affects the quality of life of afflicted patients. Historically, the treatment for emphysema has been primarily medical and includes medications, smoking cessation, and pulmonary rehabilitation. Recently a surgical procedure, lung volume reduction surgery (LVRS) has been reintroduced as a treatment for end-stage lung disease, primarily emphysema. In LVRS, wedge resections are performed to reduce the size of the lungs and improve alveolar functioning. This article describes the evaluation of patients who undergo LVRS and the associated pathophysiology in addition to the surgical procedure, inclusion and exclusion criteria, pre- and postoperative care, potential complications, and preliminary data associated with the procedure at one facility. Although LVRS is not a procedure for all patients with emphysema, it may significantly improve the quality of life of selected patients.

Forced Expiratory Volume↗

Massive periorbital emphysema associated with laparoscopic nephrectomy.

A 63-year-old man presented with marked swelling and inability to open the left eye immediately after laparoscopic nephrectomy for a left renal mass. A diagnosis of periorbital emphysema was made, and within 3 days, the emphysema spontaneously improved. This case demonstrates that periorbital emphysema, a rare condition, can be a benign complication of laparoscopic nephrectomy.

Emphysema↗

Massive subcutaneous emphysema mimicking necrotizing fasciitis after dacryocystorhinostomy.

We present a case of massive subcutaneous emphysema mimicking necrotizing fasciitis after uncomplicated dacryocystorhinostomy surgery. Subcutaneous emphysema progressing down fascial planes of the head and neck after dacryocystorhinostomy has not been reported in the literature. Using the clinical presentation and radiographic imaging, we briefly review the underlying cause of subcutaneous emphysema after dacryocystorhinostomy.

Adult↗

Morphological quantification of emphysema in small human lung specimens: comparison of methods and relation with clinical data.

Small human lung specimens are frequently used for cell biological studies of the pathogenesis of emphysema. In general, lung function and other clinical parameters are used to establish the presence and severity of emphysema/chronic obstructive pulmonary disease without morphological analysis of the specimens under investigation. In this study we compared three morphological methods to analyze emphysema, and evaluated whether clinical data correlate with the morphological data of individual lung samples. A total of 306 lung specimens from resected lung(lobes) from 221 patients were inflated and characterized using three morphological parameters: the Destructive Index, the Mean Linear Intercept, and Section Assessment. Morphological data were related to each other, to lung function data, and to smoking behavior. Significant correlations (P < .001) were observed between Section Assessment and Destructive Index (r = 0.92), Mean Linear Intercept with Destructive Index (r = 0.69) and Mean Linear Intercept with Section Assessment (r = 0.65). Section Assessment, being much less time consuming than Mean Linear Intercept and Destructive Index, is the parameter of choice for initial analysis. Destructive Index is the most sensitive parameter. There was a significant (P < .001), but weak correlation for all three parameters with the diffusion capacity for CO (K(CO)) (Destructive Index: r = -0.28; Mean Linear Intercept: r = -0.34; Section Assessment: r = -0.32), and with FEV(1)/IVC (Destructive Index: r = -0.29; Mean Linear Intercept: r = -0.33; Section Assessment: r = -0.28), but not with other lung function parameters. A significant difference (P < .05) between (ex-) smokers and never-smokers was observed for Destructive Index and Section Assessment. It is concluded that the application of the three morphological parameters represents a useful method to characterize emphysematous lesions in a (semi-)quantitative manner in small human lung specimens, and that Section Assessment is a suitable and fast method for initial screening. The extent of emphysema of individual lung specimens should be established by means of morphometry, rather than lung function data.

Adult↗

Functional evaluation of emphysema using diffusion-weighted 3Helium-magnetic resonance imaging, high-resolution computed tomography, and lung function tests.

PURPOSE: To assess the emphysematous enlargement of distal airspaces and concomitant large and small airway disease using diffusion-weighted Helium-magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT), and lung function tests (LFT). METHODS: Seven patients were examined after single lung transplantation (LTx) and 1 before double LTx for various forms of emphysema. Five patients after double LTx served as controls. Patients were assessed by Helium-MRI (apparent diffusion coefficient [ADC]), HRCT (mean lung density [MLD], emphysema index [EI]), and LFT. RESULTS: Transplanted lungs: mean ADC = 0.17 cm/s, MLD = -848 H, EI = 22%. Emphysematous lungs: mean ADC = 0.33 cm/s, MLD = -922 H; EI = 54%. Good correlations were found between ADC and MLD (r = 0.6), EI (r = 0.8), intrathoracic gas volume (r = 0.7), forced expiratory volume in 1 second (r = 0.7), and forced expiratory flows (r = 0.7). In contrast, HRCT only provided moderate correlations with LFT (EI: r = 0.5; MLD: r [le] 0.4). CONCLUSION: In this initial study, He-MRI yield good correlations with HRCT and agrees better than HRCT with the functional characterization of emphysema regarding hyperinflation, large and small airway disease as provided by LFT.

Female↗

A patient of severe cervicofacial subcutaneous emphysema associated with Munchausen's syndrome.

Subcutaneous cervicofacial emphysema is an entity with the presence of air within the fascial planes of the head and neck, which results from various causes. A case of severe subcutaneous cervicofacial emphysema associated with Munchausen's syndrome is presented. Detailed diagnostic investigations to find out the organic cause of the condition were failed. Episodes of facial swelling were prevented by psychiatric support. Munchausen's syndrome should be kept in mind among the etiological factors of subcutaneous cervicofacial emphysema cases, in whom no organic causes can be found out.

Adult↗

Relief of tension subcutaneous emphysema using a large bore subcutaneous drain.

We report a case of subcutaneous emphysema following multiple attempts to cannulate both subclavian veins for pacemaker insertion. Initial bilateral pneumothoraces were complicated by subcutaneous emphysema which became so severe that respiration and cardiac output became seriously impaired. The insertion of a single large bore subcutaneous drain produced a dramatic improvement. The aetiology, complications and management of tension subcutaneous emphysema are discussed.

Aged↗

New principles for the treatment of diffuse pulmonary emphysema.

Emphysema is due to destruction of lung tissue but the main cause of the dyspnoea is a diminished elasticity of the lungs. Medical therapy, such as bronchodilation, corticosteroids, and treatment of intercurrent infections, can give temporary relief. Long-term oxygen treatment can improve and prolong life in patients with severe epmphysema. Lung transplantation is a final option in selected patients. Rehabilitation including exercise training will cause increase in maximal exercise tolerance and decrease of dyspnoea, and thereby an improvement of quality of life. In recent years surgical reduction of the lung volume has been reintroduced and in selected cases given dramatic results. The goal is to reduce the volume in both lungs with about 30%. Depending on the distribution of the emphysema, pieces of the upper or lower lobes will be removed. Sternotomy or bilateral thoracoscopy, using staplers, are the most common methods. Short-term results are good, with an improvement of FEV1 of 22-96%, improved arterial oxygen pressure, and a radical improvement of life quality. The impact on the daily life of the patient can be dramatic. The long-term results are still not well known, and many questions remain before volume reduction surgery can be regarded as an established form of treatment for emphysema, and randomized studies are badly needed.

Humans↗

Serum IgG and IgA antibodies to Chlamydia pneumoniae and severity of emphysema.

OBJECTIVE: Chronic Chlamydia pneumoniae infection has been identified serologically in patients with COPD. The aim of this study was to examine whether the severity of emphysema is related to elevated antibody titres against C. pneumoniae. METHODOLOGY: We measured antibody titres against C. pneumoniae using ELISA, and assessed the severity of emphysema by the percentage of low attenuation area (%LAA) using high resolution (HR) CT in patients with COPD and in non-smoking control subjects. RESULTS: The mean %LAA was 2.2% in non-smoking controls (n = 28) and 13.3% in COPD patients (n = 94). COPD patients with a high IgG antibody index to C. pneumoniae (> or =2.0, n = 42) had a significantly higher %LAA (16.8%) than those with a low IgG index (<2.0, n = 52) (10.6%, P = 0.01). In addition, COPD patients with a high IgA antibody index (> or =2.0, n = 46) had a significantly higher %LAA (15.9%) than those with a low IgA index (<2.0, n = 48) (10.9%, P = 0.048). COPD patients with a high IgA antibody index also had a significantly lower %DLco than that associated with a low IgA index (68.1% and 80.3%, respectively, P = 0.007). There were no significant differences in age, smoking index or FEV(1)/FVC between these groups. CONCLUSION: These results suggest that high antibody titres against C. pneumoniae are linked with the severity of emphysema on high resolution CT and decreased diffusing capacity to carbon monoxide.

Aged↗

Absence of alpha-1-antitrypsin (Pi Null Bellingham) and the early onset of emphysema.

BACKGROUND: Alpha-1-antitrypsin is the body's major inhibitor of human neutrophil elastase, a powerful proteolytic enzyme capable of degrading the common tissue components. There are over 70 genetic variants of alpha-1-antitrypsin, with the Z allele being of greatest clinical relevance. Individuals homozygous for this allele (approximately one in 2500 in Caucasians) have low serum alpha-1-antitrypsin levels (10-20% of normal) and are predisposed to emphysema, especially if they smoke. Much rarer are mutations which result in the complete or almost complete absence of alpha-1-antitrypsin in the serum. AIM: To determine the cause of complete absence of alpha-1-antitrypsin in a patient who at age 27 years had both emphysema and idiopathic cardiomyopathy. METHODS: Molecular biology techniques were used to sequence the alpha-1-antitrypsin gene. Allele specific amplification was used to show the presence of the mutations in other family members. RESULTS: Investigation showed that the proband was homozygous for the Pi Null Bellingham variant of alpha-1-antitrypsin due to the mutation Lys 217 (AAG) to Stop (TAG). His grandmother was heterozygous for Pi Null Bellingham and the additional rare variant P Lowell, Asp 256 (GAT) to Val (GTT), a variant that also results in alpha-1-antitrypsin deficiency. CONCLUSION: Patients with complete absence of alpha-1-antitrypsin develop premature emphysema not having smoked or after only minimal exposure, and much earlier than the more common Pi Z individuals who have the usual form of alpha-1-antitrypsin deficiency.

Adult↗

Protective role of glutathione on alpha 1 proteinase inhibitor inactivation by the myeloperoxidase system. Hypothetic study for therapeutic strategy in the management of smokers' emphysema.

In smoking subjects with obvious emphysema, the interaction between neutrophil-derived MPO and H2O2 produced by alveolar inflammatory cells (alveolar macrophages (AM) and polymorphonuclear neutrophils (PMN)) has the ability to spontaneously inactivate, in vitro, the alpha 1 proteinase inhibitor (alpha 1PI). This inactivation can induce a desequilibrium of the protease-antiprotease balance in the lungs. In this study, we investigated the ability of glutathione to protect alpha 1PI. In a cellular model of alpha 1PI inactivation mimicking the effects of alveolar inflammatory cells present in the lower respiratory tract of smoking patients with emphysema, we demonstrated that glutathione can protect alpha 1PI against the oxidative inactivation by these activated cells. This protection has been computed in a cellular experimentation (AM and MPO-system) with a 50% inhibitory concentration of 62 microM. Moreover, glutathione has an important inhibitory effect directly on H2O2 released by PMA-stimulated AM (IC50 = 30 microM) or PMA stimulated PMN (IC50 = 70 microM). The mechanism, which governs glutathione may be a result of a scavenging effect on H2O2 as demonstrated in a free cellular experiment. With this in vitro demonstrated effectiveness, glutathione as a therapeutic antioxidant, via the aerosol, has been proposed, in order to prevent tissue damage, inflicted by an excess of activated phagocytic cells, in some lung diseases such as smoking patients with emphysema.

Glutathione↗

Modern concepts in the management of emphysema in the aged and infirm.

The haphazard method of treating patients seriously ill with emphysema is gradually being replaced by modern, well organized cardiopulmonary therapeutic and rehabilitation programs. This evolution is the result of a further expansion in our knowledge of cardiopulmonary physiology and biochemistry; an additional factor is the recent public awareness of the seriousness of the problem, brought about by public educational programs. Emphysema is chiefly a disease of old age. It develops as a result of a degenerative process in which the alveolar walls become thinner and the lungs less elastic. Senile emphysema per se may not be disabling, but it is often associated with a severe chronic pulmonary disorder and thus can become the most distressing disease of old age, with shortness of breath even on such slight exertion as dressing or talking. Disturbances in cardiopulmonary physiology due to obstruction in the free flow of air and to superimposed infections require the use of all available procedures designed to obtain maximal pulmonary ventilation. The magnitude, the difficulty and the many controversial aspects of this problem are evident. The eventual solution will come gradually with continued interest and research.

Aerosols↗

Requirement for macrophage elastase for cigarette smoke-induced emphysema in mice.

To determine which proteinases are responsible for the lung destruction characteristic of pulmonary emphysema, macrophage elastase-deficient (MME-/-) mice were subjected to cigarette smoke. In contrast to wild-type mice, MME-/- mice did not have increased numbers of macrophages in their lungs and did not develop emphysema in response to long-term exposure to cigarette smoke. Smoke-exposed MME-/- mice that received monthly intratracheal instillations of monocyte chemoattractant protein-1 showed accumulation of alveolar macrophages but did not develop air space enlargement. Thus, macrophage elastase is probably sufficient for the development of emphysema that results from chronic inhalation of cigarette smoke.

Animals↗

Pulmonary interstitial emphysema.

Forty one of 210 preterm infants ventilated for respiratory distress syndrome in a three year period had radiological evidence of pulmonary interstitial emphysema. The development of this condition was significantly associated with malpositioning of the endotracheal tube in a main bronchus and the use of high peak pressure ventilation. Pulmonary interstitial emphysema was associated with a significant increase in the number of pneumothoraces, intraventricular haemorrhages, and the need for prolonged respiratory support, but did not increase mortality. Although in 12 infants in whom fast rate ventilation was used there was a significant reduction in the number of pneumothoraces, outcome was not altered in any other way. Fast rate ventilation may be of greater benefit if initiated before the development of pulmonary interstitial emphysema.

Gestational Age↗

Diagnosis of old anterior myocardial infarction in emphysema with poor R wave progression in anterior chest leads.

The electrocardiograms of patients with emphysema may suggest associated old anterior myocardial infarction. Sixteen patients with the physiological characteristics of emphysema were studied, who also showed poor R wave progression in the anterior chest leads, so that RV3 was less than or equal to 4 mm. A thallium-201 cardiac scan consistent with previous anterior (septal) myocardial infarction was present in seven patients. In these patients there was no significant increase in RV3 amplitude when recorded one interspace below the conventional site. In the nine patients with a thallium-201 cardiac scan negative for old anterior myocardial infarction, RV3 amplitude increased from 2.2 +/- 0.4 mm to 6.4 +/- 1.2 mm. Patients with or without associated old anterior myocardial infarction could be better diagnosed by consideration of RV3 amplitude as recorded from one interspace lower, as compared with conventional electrode placement. All five patients with RV3 (lower interspace) less than 2 mm had associated anterior infarction, and all seven patients with RV3 (lower interspace) greater than 3 mm did not. This simple manoeuvre is recommended in patients with emphysema and an electrocardiogram suggestive of old anterior myocardial infarction.

Adult↗

Relation between progressive massive fibrosis, emphysema, and pulmonary dysfunction in coalworkers' pneumoconiosis.

The correlations between progressive massive fibrosis (PMF), emphysema, and impairment of ventilation were studied in 108 dead coalminers, all of whom had suffered with complicated coalworkers' pneumoconiosis (radiological category B or C) during life. The findings indicated that both factors were contributing to impairment of ventilation in proportion to their size or extent, but these contributions were in the main independent of one another. A joint opinion on the types of emphysema found to be present in a random 50 out of the 108 cases was given by two pulmonary pathologists. They found that most of the emphysema was of the centrilobular variety, which appeared to be unrelated to the PMF. The distribution of the PMF throughout the lungs both radiologically and anatomically is shown.

Coal Mining↗

Emphysema and airway obstruction in non-smoking South African gold miners with long exposure to silica dust.

OBJECTIVE: Occupational exposure to silica dust is associated with significant impairment of lung function. The present study investigates which pathological changes in the lung are associated with impairment of lung function in silica dust exposed workers who were life-long non-smokers. METHODS: 242 South African white gold miners who were lifelong non-smokers and who had a necropsy at death were studied. The pathological features identified at necropsy were the degree and type of emphysema, the presence of airway disease, and the degree of silicosis in the lung parenchyma and pleura. These features were related to lung function tests done a few years before death, to type of impairment (obstructive or restrictive), and to cumulative silica dust exposure. RESULTS: The degree of emphysema found at necropsy was not associated with a statistically significant impairment of lung function or with dust exposure. The degree of silicosis in the lung parenchyma and the large airways disease (based on mucus gland hyperplasia) were associated with a statistically significant impairment of lung function. The large airway disease was, however, not positively associated with dust exposure or silicosis. In miners with a moderate or a higher degree of limitation of airflow the main findings were silicosis, heart disease, and obesity. The presence of small airways disease could not be established from the necropsy material. CONCLUSION: The results indicate that the level of exposure to silica dust to which these miners were exposed, without a confounding effect of tobacco smoking, is not associated with a degree of emphysema that would cause a statistically significant impairment of lung function. Silicosis of the lung parenchyma was associated with loss of lung function. Other factors that may play a part in impairment of lung function in these miners are obesity and heart disease.

Adult↗