Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The pathogenesis of primary pulmonary hypertension is still unclear. The case of a 68-yr-old female patient who complained of recurrent dizzy spells and collapses over a period of 6 weeks and died of global cardiac failure is presented. Autopsy revealed severe pulmonary hypertension, slight chronic bronchitis, and bronchiolitis as well as intra-alveolar accumulation of macrophages. Chlamydiae were detected within the pulmonary arteries and in intramural and intra-alveolar macrophages by immunofluorescence, confocal laser scanning microscopy, scanning and transmission electron microscopy. Nested-polymerase chain reaction (PCR) and nonradioactive deoxyribonucleic acid (DNA) hybridization of PCR products from pulmonary arteries revealed Chlamydia pneumoniae DNA. Chlamydia pneumoniae has already been detected in atherosclerosis and in pulmonary emphysema. It can induce proliferation of smooth muscle cells. Chlamydia pneumoniae might be relevant in aggravation of primary pulmonary hypertension and might perhaps be a trigger factor in some cases.
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PMID:Detection of Chlamydia pneumoniae in unexplained pulmonary hypertension. 1184 18

Vascular endothelial growth factor (VEGF) plays a central role in the life and death of pulmonary vascular endothelial cells. Treatment of neonatal or adult rats with a VEGF receptor blocker destroys lung capillaries by inducing endothelial cell apoptosis and causes emphysema. Human lung tissue samples from patients with endstage emphysema have decreased levels of VEGF messenger RNA and protein and have decreased expression of kinase insert domain-containing receptor (VEGF receptor II). These decreases are associated with a high rate of alveolar septal cell apoptosis, indicating perhaps that decreased VEGF and kinase insert domain-containing receptor expression impairs endothelial cell survival in emphysematous lungs. Combination of VEGF receptor blockade with chronic hypoxia (3-wk exposure) results in obliteration of small precapillary pulmonary arteries by proliferating endothelial cells, severe pulmonary hypertension, and death caused by right-side heart failure. We propose that 1) VEGF receptor blockade causes endothelial cell apoptosis, 2) hypoxic vasoconstriction (shear stress) selects apoptosis-resistant endothelial cells that proliferate and obliterate the lumen, and 3) the vascular remodeling observed is relevant to the structural alterations that characterize severe pulmonary hypertension (including primary pulmonary hypertension) in humans. The endovascular cell growth in human disease and in our model exhibits some similarities with neoplastic cell growth. Chemotherapy strategies can now be employed in the animal model in an attempt to treat established vascular-obliterative lung disease.
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PMID:Janus face of vascular endothelial growth factor: the obligatory survival factor for lung vascular endothelium controls precapillary artery remodeling in severe pulmonary hypertension. 1200 44

Based on data reported to the UNOS/ISHLT Thoracic and International Registry for Thoracic Organ Transplantation: 1. The number of heart transplant operations performed in the United States decreased between 1998-1999 and 17 (1%) more procedures were performed in 1999 (2,181) than in 2000 (2,198). Sixty-nine more lung transplants (an 8% increase) were reported in 2000 than in 1999. 2. Coronary artery disease and cardiomyopathy were the most frequently cited indications for heart transplantation in the US and have been reported at similar rates during the past 10 years. Combined, these diagnoses account for approximately 85% of all heart transplants. In 2000, half of all lung transplants were performed for emphysema/COPD or alpha-1 antitrypsin deficiency. The most frequently reported diagnoses for thoracic transplantation outside the US were: cardiomyopathy (49%) for heart, cystic fibrosis (30%) for double lung, emphysema/COPD (34%) for single lung and primary pulmonary hypertension (21%) for heart-lung transplants. 3. US heart transplant recipients were predominately male (76%), aged 50-64 (51%) and white (81%). US lung transplant recipients were also predominately between ages 50-64 (47%) and white (90%), but unlike heart recipients were more likely to be female (51%). No meaningful variance from the US recipient demographic profile was noted for the non-US recipients during the same time period. 4. Pediatric recipients (< 18 years of age) received 11% of the reported heart transplants and 6% of the reported lung transplants in the US. 5. Among US thoracic transplant recipients during 1999, the one-year survival rates were 84% for heart, 59% for heart-lung and 77% for lung. The 5-year survival rates for transplants performed during 1995 were: 71% for heart, 56% for heart-lung and 44% for lung transplants. 6. The long-term patient survival rates were: 23% at 19 years for heart, 16% at 11 years for lung and 23% at 14 years for heart-lung recipients. 7. During the first year after transplantation, 66% of heart recipients and 44% of lung recipients did not require rehospitalization. Among those recipients who were rehospitalized, the major cause was infection.
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PMID:Worldwide thoracic organ transplantation: a report from the UNOS/ISHLT international registry for thoracic organ transplantation. 1221 89

Lung transplantation has evolved to a standard treatment modality for patients suffering from end-stage lung diseases and it provides very good short- and satisfactory long-term survival. Accepted indications for lung transplantation include chronic obstructive pulmonary disease (COPD) and other causes of emphysema (i.e., alpha-1-antitrypsin deficiency), parenchymal diseases (i.e., idiopathic pulmonary fibrosis), genetic disorders such as cystic fibrosis, vascular diseases (i.e., primary pulmonary hypertension), chronic infectious diseases (i.e., bronchiectasis), as well as rare indications such as lymphangioleiomyomatosis or sarcoidosis. Presented article reviews the current strategies in the treatment of lung transplant recipients, surgical techniques, limitations of and effects of lung transplantation.
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PMID:[Lung transplantation--present status worldwide and in the Czech Republic]. 1474 18

Delivery of genes to the pulmonary vascular endothelium is a rational approach for the investigation and potential therapy of pulmonary vascular diseases. Furthermore, in view of the exposure of this vascular bed to the entire cardiac output, this technique could be used as an efficient basis to achieve systemic delivery of secreted factors. The attraction of direct gene delivery to endothelium for the therapy of vascular disease has been especially heightened in the last couple of years in view of the new discoveries concerning the genetic basis of primary pulmonary hypertension (PPH). In brief, mutations in the bone morphogenetic protein receptor type 2 (BMPR2, a member of the transforming growth factor-beta [TGF-beta] family of receptors) gene have been found in many patients with familial PPH. Subsequent in vitro studies have confirmed an association between BMPR2 mutations and abnormal proliferative responses in pulmonary endothelial and smooth-muscle cells (2). Other TGF-beta signaling pathways may also be involved in this process, and the mechanisms involved may also have relevance for the more common cases of pulmonary vascular disease secondarily associated with chronic airways obstruction, connective tissue diseases, and perhaps HIV infection. Additionally, new evidence is emerging concerning the role of the vasculature in the pathogenesis of emphysema.
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PMID:Delivery of DNA to pulmonary endothelium using adenoviral vectors. 1497 May 86

The purpose of this study was to explore the relationship between diagnosis and the cost-effectiveness and cost-utility of lung transplantation. A microsimulation model was used, based on empirical data from the Dutch lung transplantation program, collected between 1991 and 1999. We assessed life-years, quality-adjusted life-years, and costs with and without transplantation for the diagnostic categories alfa-1 antitrypsin deficiency, COPD/emphysema, bronchiectasis, primary and secondary pulmonary hypertension, cystic fibrosis, and pulmonary fibrosis. Alfa-1 antitrypsin deficiency and bronchiectasis had the highest survival gain. Secondary pulmonary hypertension and pulmonary fibrosis had the lowest survival gain and the lowest gain of quality-adjusted life-years. As compared with COPD/emphysema, alfa-1 antitrypsin deficiency, bronchiectasis, and CF had 25%, 40% and 19% more favorable cost-effectiveness ratios, respectively. Cost-utility ratios varied less, with values of -7%, -14% and -11% for alfa-1 antitrypsin deficiency, bronchiectasis, and primary pulmonary hypertension, respectively, compared with COPD. In conclusion, our model suggests that there is considerable variation in cost-effectiveness and, to a lesser degree, in cost-utility between the different diagnostic categories. These variations are the result of differences in survival and in quality of life with and without lung transplantation.
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PMID:Cost-effectiveness of lung transplantation in relation to type of end-stage pulmonary disease. 1519 75

Between February 1983 and July 1987, twelve patients underwent heart-lung transplantation at the University of Cape Town and the University of Munich. The patients included eight men and four women, whose ages ranged from 15 to 49 years (mean, 27 years). The underlying pathologic condition was idiopathic primary pulmonary hypertension in five cases, Eisenmenger's syndrome in four cases, idiopathic pulmonary fibrosis in one case, diffuse fibrosing alveolitis in one case, and chronic emphysema in one case. The immunosuppressive regimen consisted of cyclosporine A, azathioprine, and rabbit antithymocyte globulin (RATG) during the first 2 postoperative weeks; RATG was subsequently replaced by methylprednisolone. Pulmonary rejection frequently occurred in the absence of cardiac rejection; in one case, however, this situation was reversed. Two patients required retransplantation, which was undertaken for caseating pulmonary tuberculosis with obliterative bronchiolitis after 1 year in one case and for early pulmonary insufficiency after 2 days in the other case. There were no operative deaths, but three early deaths occurred, owing to respiratory insufficiency of unknown origin (10 days postoperatively), multiorgan failure (10 days postoperatively), and acute liver dystrophy (11 days postoperatively). Five weeks after operation, a fourth patient died of multi-organ failure. There were five late deaths, all of which resulted from infectious complications. Three patients, including one who underwent retransplantation, remain alive and well, 10 to 36 months postoperatively.
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PMID:Initial experience with heart and lung transplantation. 1522 70

Heart-lung transplantation (HLT), followed by single lung transplantation (SLT) and subsequently bilateral lung transplantation (BLT) have been developed as treatments for patients with end-stage pulmonary diseases. Initially, SLT was limited to idiopathic pulmonary fibrosis (IPF) cases and thought to be contraindicated not only for infectious diseases, but also for non-infectious diseases, including pulmonary emphysema (PE) and primary pulmonary hypertension (PPH), based on physiologic points of view. However, SLT is now widely performed for those non-infectious diseases and most of the recipients return to a normal active life. It is quite possible that BLT is superior to SLT in terms of pulmonary function, and it has been reported that BLT is better for PE and PPH patients in regards to perioperative course, postoperative exercise capacity, and long-term survival. For those situations and because of the present scarcity of donor organs, SLT must be utilized for selected non-infectious diseases for which it is safe and effective. When a single lung is replaced for IPF, PE, and PPH recipients, different physiologic situations are produced postoperatively, the understanding of which is extremely important to achieve good results, not only in the perioperative but also in the long term.
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PMID:Physiologic aspects in human lung transplantation. 1590 Feb 36

During the last decade, activity in lung transplantation increased rapidly to a plateau of approximately 900 transplants per year in the United States, and it is unlikely to increase further unless the supply of usable donor organs can be expanded. However, the demand for lung transplantation has continued to rise, and the median waiting time for lung transplantation in the United States is approaching 2 years. The main indications for lung transplantation have been chronic obstructive pulmonary disease, alpha1-antitrypsin deficiency emphysema, cystic fibrosis, idiopathic pulmonary fibrosis, primary pulmonary hypertension, and Eisenmenger's syndrome. Overall, survival after lung transplantation has been good, with 1-, 3-, and 5-year survival rates of 75, 58, and 44%, respectively, and the results have been improving. Chronic rejection and infection have been the main causes of medium-term mortality, and the high prevalence of chronic rejection remains a barrier to better outcomes. Quality of life has been enhanced by lung transplantation, but the posttransplantation immunosuppressive regimen has been associated with considerable morbidity.
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PMID:Lung and heart-lung transplantation: overview of results. 1608 94

Idiopathic pulmonary fibrosis (IPF) is typically a fatal disease that fails to respond to medical therapy. For these patients lung transplantation offers the promise of improved quality and duration of life. The initial reports of successful transplantation for IPF date back to the mid-1980s although recent data suggest IPF patients make up nearly 20% of all single lung transplants. The survival rates following lung transplantation for IPF are estimated at 67% for 1 year, 52% for 3 years, and 35% for 5 years. Mortality rates following lung transplantation for IPF are higher than emphysema, but are generally comparable to primary pulmonary hypertension. Given the relatively high mortality following transplant the decision of when to transplant a patient is of paramount importance. Although individual patients differ, generalizations predicting a poor prognosis include the diagnosis of usual interstitial pneumonia (UIP), a forced vital capacity or total lung capacity of less than 65% predicted, a diffusion capacity for carbon monoxide (DL(CO)) less than 45% of predicted, and the presence of extensive honeycombing on high-resolution computed tomographic scans. The presence of any of these features should prompt the patient and physician to consider lung transplantation as a potential therapeutic modality. Patients with interstitial lung disease related to collagen vascular diseases or other causes need to be considered on an individual basis; their prognosis is usually better than patients with IPF, and the potential for systemic involvement may preclude listing for lung transplantation.
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PMID:Timing of lung transplantation for patients with fibrotic lung diseases. 1608 98


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