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)

Is an acute bronchial, obstructive disease of the infant caused mainly by the respiratory syncytial virus. It appears epidemically preceded by infections of the upper respiratory ducts, followed by coughing, dysnea, expiratory sibilants, suprasternal and subcostal during inspiration and radiologic evidences of choneking. In the differential diagnosis the physician must consider pulmonary dysgenesis, diaphragmatic hernia, congenital lobar emphysema, congenital cardiopathy, pneumothorax, obstruction due to foreign body, asthmatic crisis and fibrocystic disease. Fundamentally, two diagnoses should be discarded: 1) dyspenic bacterial bronchopneumonic syndrome; 2) prime infection T. B. bronchopneumonia with bronchiolitic syndrome.
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PMID:[Bronchiolitis]. 742 29

1. The frequency of heart and lung transplantation has increased dramatically over time. The number of heart transplants increased 170% between 1968 and 1981. Exponential increases began in 1982 and increased through 1990, as evidenced by a 1,835% growth in the number of procedures performed during those years. In more recent years, heart transplant numbers have leveled off, with only an 8% increase in the last 3 years. Lung transplant procedures have grown significantly in the short period of time between 1987 (n = 18) and 1993 (n = 669). 2. Since the beginning of the ISHLT Registry in 1968 the number of thoracic transplant programs has increased 7,767%, from 3 to 236. 3. The most frequently reported indications for thoracic transplantation include: coronary artery disease (43.4%) for heart, cystic fibrosis (39.5%) for double-lung, emphysema/COPD (40.2%) for single-lung and primary pulmonary hypertension (38.3%) for heart-lung. 4. The majority of heart transplant recipients are at least 18 years old (89.5%), male (78.2%), and White (83.8%). The majority of lung transplant recipients are at least 18 years old (93.7%), female (53.2%), and White (91.3%). 5. One-year survival over time has almost doubled for all types of thoracic transplantation, with increases from 47.7% in 1968-79 to 81.6% in 1993 for heart; 35.3% in 1987 to 67.1% in 1993 for lung; and 40% in the early 1980s to 73% in 1993 for heart-lung. 6. Long-term 10-year survival rates were 33.5% for heart and 5.6% for heart-lung transplant recipients. Five-year survival for lung recipients was 37%. 7. Male-to-male donor-to-recipient gender-match heart transplant patients exhibited slightly higher survival (4-5%) than other match pairs from one to 5 years posttransplant. Female-to-male combinations in lung transplant recipients exhibited slightly higher survival (4-8%) at 3 years than other match pairs. 8. There was no significant difference in 5-year survival for donor hearts procured from local, intraregional, or interregional sources. Lungs procured from interregional sources exhibited an 8-10% advantage over local and intraregionally procured organs at 3 years posttransplant.
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PMID:Thoracic organ transplants in the United States: a report from the UNOS/ISHLT Scientific Registry for Organ Transplants. United Network for Organ Sharing. International Society for Heart and Lung Transplantation. 754 66

Progress over the last 40 years has greatly reduced morbidity and mortality in the constantly changing field of thoracic surgery. The first part of this review focuses on current indications and limitations in lung surgery. Technical procedures for pneumonectomy, lobectomy, bronchial resection and conservative surgery are well established. Although major respiratory or cardiac failure still limit indications bronchogenic cancer extension is no longer a contraindication. Exeresis after 70 years of age is not an exception. Surgery for non-small cell lung cancer has given promising results with a 5-year survival rate of 60-80% for patients in stage I and II. For stage III, two recent comparative studies have demonstrated the effectiveness of preoperative adjuvant chemotherapy which should logically be proposed with or without radiotherapy in patients with resectable tumours. Surgical removal of lung metastases and mesotheliomas has also made considerable progress. Unfortunately, except for therapeutic trials, exeresis of small cell lung cancer does not provide any beneficial effect and cannot be proposed. Indications for surgery in patients with chronic obstructive pulmonary disease however has been quite successful and now goes beyond classical exeresis of large compressive bullae. In many situations patients with diffuse emphysema can benefit from surgical reduction in lung volume before proposing transplantation. Lung transplantation is indicated for pulmonary fibrosis, pulmonary vascular disease and obstructive lung pulmonary disease with an overall survival rate of 50% at 5 years and 43% at 6 years. The rate of successful bilateral lung transplantation for cystic fibrosis remains to be determined.
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PMID:[New techniques in thoracic surgery. I]. 756 9

Air travel can cause severe respiratory decompensation in a patient with advanced lung disease due to high altitude hypoxemia. We report our experience in flying 21 patients with advanced lung disease to a medical center remote from Israel for lung transplantation or pulmonary thromboendarterectomy (PTE). All patients had severe lung disease with marked hypoxemia (PaO2, 40 to 59) and 16 had significant pulmonary hypertension. Nine patients (with emphysema and pulmonary fibrosis) required single lung transplant, four (with cystic fibrosis and emphysema) required double-lung transplant, six (with primary or secondary pulmonary hypertension) required heart-lung transplant, and two (with major vessel pulmonary thrombosis) required PTE. All patients were flown by commercial aircraft to centers located 2,634 to 13,181 km away from Israel. Length of flight was between 4 and 21 h. Patients were given oxygen supplementation during the flight and were monitored by portable oximeters. All but three patients were hemodynamically stable and 19 of them were escorted by physicians. All but one hemodynamically unstable patient who died on board arrived safely at their destinations. We conclude that with careful preparation, sufficient oxygen supply, oximetric monitoring, and medical escort, almost any patient with severe lung disease can travel by air to any necessary destination.
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PMID:The safety of air transportation of patients with advanced lung disease. Experience with 21 patients requiring lung transplantation or pulmonary thromboendarterectomy. 758 32

Heart-lung transplantation is a complex, expensive, and resource-intensive procedure. It is performed more often where there is coexistence of end-stage pulmonary disease with advanced cardiac disease that is not secondary to pulmonary hypertension (e.g., some forms of congenital heart disease, some situations with Eisenmenger's syndrome, and possibly the coexistence of end-stage heart and end-stage lung disease). Available data indicate that some disease states are associated with a higher probability of successful outcome after HLT than are others. Objectively validated and reliable patient selection criteria cannot be obtained from the current medical literature. Expert opinion provided to OHTA contained varying degrees of detail and uniformity with respect to patient selection (Tables 11 and 12). While most agree that "irreversible cardiopulmonary disease" is an indication for this procedure, there are no published data that have provided details as to how patients so designated were selected as candidates for HLT. For example, for chronic obstructive pulmonary disease (emphysema), neither expert opinion nor published studies of HLT provided data indicating the stage of the disease at which a decision to transplant is reasonable and objectively justifiable. A similar lack of detail for cystic fibrosis, bronchiectasis, pulmonary fibrosis, sarcoidosis, asbestosis, and other diseases was noted. Thus, there are insufficient data regarding transplant recipients to permit the development of objective criteria for a threshold for HLT in the various disease states. The circumstances are more problematic regarding suggested contraindications for HLT. While objective survival rates obtained from registry data indicated that survival bore little relationship to recipient age, various opinions provided to OHTA specified a maximum age for HLT candidates, ranging from under 45 years to 60 years. Adverse factors and contraindications, such as "other significant disease," "recurrent pulmonary emboli," "hepatic disease," "infection," and "insulin-dependent diabetes" were nonspecific and unsupported by published, objective evidence. More ambiguous contraindications included "psychiatric illness," "poor family support structure," "history of noncompliance with medical regimens," and lack of "emotional stability." While psychiatric, psychologic, or sociologic evaluations of potential HLT recipients undeniably have relevance to patient selection, use of such criteria should be supported at least by evidence of generally consistent application across the transplant community. Of note was the recommended requirement that the recipient possess "adequate financial resources," which implies that the benefits of HLT are not expected to be equitably provided to the public.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Institutional and patient criteria for heart-lung transplantation. 771 18

Human lung transplantation was successfully performed in the early eighties and is now an option for patients with endstage lung disease, which is associated with poor survival. Most frequent indications for lung transplantation are emphysema, cystic fibrosis, fibrosing alveolitis, primary pulmonary hypertension and Eisenmenger's syndrome. Single lung transplantation (SLT) is most often performed in emphysema, fibrosing alveolitis and other diseases which are not associated with chronic infection of the lung. Double lung transplantation was recently replaced by the technique of sequential single lung or bilateral lung transplantation (BLT). Cardiopulmonary bypass can often be avoided and problems of the airway anastomosis are less frequent using BLT. Main indications for this procedure are cystic fibrosis, bronchiectasis and primary pulmonary hypertension (PPH). In PPH often only SLT is performed. Cor pulmonale is reversible following SLT or BLT even if the heart is not replaced. Combined heart-lung transplantation (HLT) is reserved for some cases of Eisenmenger's syndrome and few centers still prefer HLT in patients with cystic fibrosis. Patients are usually accepted for transplantation when they are considered to have life expectancy of 12 to 24 months. Quality of life and physical working capacity are severely decreased and patients suffer dyspnea NYHA grade III or IV. Most of the patients are hypoxic and need continuous oxygen therapy. Hypercapnia is also a negative predictive factor for survival without transplantation. In PPH cardiac index of less than 2 litres/m2 is associated with poor outcome. Not only absolute values for FEV1 and pO2 have to be considered in finding the best moment for assessment for transplantation but the clinical course of the disease during previous months and years also has to be taken into account. Contraindications to transplantation include acute infection, concomitant diseases of other organs, bronchial carcinoma and psychiatric disorders if noncompliance is likely. To achieve good results after lung transplantation, proper donor and recipient selection, experienced surgery and careful postoperative management are essential. Complications must be diagnosed early to provide effective treatment. Most complications occur within the first months after surgery. Early complications include primary organ failure, pleural bleeding, problems at the site of the airway anastomosis, infection and acute rejection. Acute rejection is common but can be treated successfully if diagnosed early.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Lung transplantation]. 778 72

Forty children (aged 1 to 18 years, 27 female and 13 male) have undergone heart-lung (21), double lung (17), and single lung (2) transplant procedures at our center from 1985 through April 1994. The indications for transplantation have been diverse, primary pulmonary hypertension (10), cystic fibrosis (11), congenital heart disease (10), arteriovenous malformation (3), emphysema (1), graft-versus-host disease (1), rheumatoid lung (1), cardiomyopathy (1), desquamative interstitial pneumonitis (1), and Proteus syndrome (1). The actuarial 1-year survival was 73% (mean follow-up 2 years). One-year actuarial survival for disease groups ranged from 60% for cystic fibrosis to 90% for congenital heart disease. We have identified six issues critical to the patient and programatic survival of pediatric lung transplantation. Our experience and management strategies in these areas are reviewed. Cytomegalovirus: Cytomegalovirus disease developed in six of eight patients with cytomegalovirus mismatching (donor +/recipient-) and in seven of 32 patients who survived more than 30 days (23%). All but cytomegalovirus donor -/recipient- patients were treated with ganciclovir for 4 weeks after transplantation. Obliterative bronchiolitis: Obliterative bronchiolitis developed in seven of 32 (25%) patients who survived more than 30 days. Obliterative bronchiolitis was manifest within the first posttransplantation year as a rapid decline in small airway function. Aggressive augmentation of immunosuppression has been used with little success. Posttransplantation lymphoproliferative disease: Posttransplantation lymphoproliferative disease developed in five of 32 (15%) patients who survived more than 30 days developed. One patient died (17% mortality) despite retransplantation. In four patients the disease resolved with reduction in immunosuppression alone, and one required the addition of interferon alfa. Cystic fibrosis: We have changed our management strategies to avoid triple drug immunosuppression, perioperative blood and bronchial cultures, aggressive antimicrobial therapy, and exclusion of patients with panresistant organisms; this has resulted in elimination of infectious mortalities thus far in the pediatric cystic fibrosis group. Airways: In 21 heart-lung recipients with tracheal anastomoses we have had no airway complications. The double and single lung transplant recipients accounted for 34 bronchial and one tracheal anastomoses. Three (9%) bronchial stenoses developed. Two were treated with silicone stents and one with balloon dilation.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Critical issues in pediatric lung transplantation. 781 8

We assessed the long-term results of our experience with 109 patients with end-stage cardiopulmonary disease who underwent primary combined heart-lung transplantation at Stanford University Medical Center between March 1981 and January 1994. Average recipient age was 31 +/- 10 years (mean +/- standard deviation) median, 31 years; range, 1 month to 52 years. Recipient diagnoses included primary pulmonary hypertension (31%), Eisenmenger's syndrome (39%), complex congenital heart disease (8%), cystic fibrosis (14%), bronchiectasis (2%), and emphysema (3%). Immunosuppression was with cyclosporine and a tapering regimen of corticosteroids. In 1986 azathioprine was added, and since 1987 induction therapy with OKT3 has been employed. Actuarial survival rates at 1, 5, and 10 years were 68% +/- 4.6%, 43% +/- 5.4%, and 23% +/- 8.1%, respectively (mean +/- 1 standard error of the mean). Fourteen deaths occurred in the hospital for an operative mortality rate of 12.8% +/- 3.3%, and 61 deaths occurred overall. Causes of death included hemorrhage (five patients), infection (21), rejection (one), nonspecific pulmonary failure (four), graft coronary artery disease (six), and obliterative bronchiolitis (eight). Infection, rejection, and obliterative bronchiolitis were the major complications. Only 20% +/- 3.9% of patients were free from any infection 3 months after transplantation. Heart and lung rejection commonly occurred asynchronously; actuarial estimates of freedom from isolated lung rejection at 1 and 5 years were 47% +/- 5.2% and 40% +/- 5.6%, respectively. For simultaneous heart and lung rejection these estimates were 87% +/- 3.5% and 86% +/- 3.8%, and for isolated heart rejection 63% +/- 5.1% and 51% +/- 6.4%, respectively. Although graft coronary artery disease developed less frequently than in patients after isolated heart transplantation (90% +/- 4.6% of patients were free of graft coronary artery disease at 5 years), obliterative bronchiolitis remains a major long-term complication and cause of morbidity and mortality. Actuarial estimates of freedom from obliterative bronchiolitis at 1, 5, and 10 years were 71% +/- 5.1%, 51% +/- 6.1%, and 42% +/- 7.8%, respectively. These results show satisfactory early and medium-term outcome after combined heart-lung transplantation but also underscore that much progress is needed in controlling infection, rejection, and obliterative bronchiolitis, all of which remain as major impediments to long-term survival.
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PMID:Long-term results of combined heart-lung transplantation: the Stanford experience. 786 27

Lung transplantation is a successful alternative treatment for a variety of end-stage lung diseases. The first 20 lung transplants performed in Louisiana between November 1990 and July 1994 are reported from Ochsner Foundation Hospital. Transplant procedures included 1 heart-lung, 11 bilateral sequential lung, and 8 single-lung transplants in 8 males and 11 females (1 retransplantation). The average age was 38 years (range 7-60), and the median waiting time was 34.5 days (range 1-329). Indications for transplant included emphysema, pulmonary fibrosis, pulmonary hypertension, cystic fibrosis, bronchiectasis, and bronchiolitis obliterans. Overall 1-year and 3-year survival were 65.0% and 58.5%, respectively. Infection was the major cause of morbidity and mortality. Rejection episodes were observed but treated successfully in all 20 patients. Lung transplantation has proved to be a successful treatment for a variety of severely limiting and terminal pulmonary conditions for patients in our state.
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PMID:Lung transplantation in Louisiana: report of the first twenty lung transplants performed in the state. 789 Oct 1

Neutrophil elastase (NE) is the most important protease of the human lung; if not inhibited NE is able to attack nearly all structural proteins and components of the immune system of the lung. In the normal human lung this is prevented by an excess of protease inhibitors. Based on quantitative and kinetic analyses alpha 1-antitrypsin (AAT) is the most important protease inhibitor of the human lung followed by the Secretory Leukoprotease Inhibitor (SLPI). As protease inhibitors may be inactivated by proteases and reactive oxygen metabolites released from polymorphonuclear cells, it is not surprising that some lung diseases--in particular lung emphysema caused by AAT deficiency, cystic fibrosis and ARDS--are characterized by an imbalance between proteases and protease inhibitors. Because uninhibited NE seems to play an important role in the pathogenesis of these diseases, it is obvious to use protease inhibitors as drugs. Up to now in most studies in men AAT purified from human plasma has been used: worldwide a substantial number of patients with lung emphysema caused by AAT deficiency is treated continuously; in addition AAT was aerosolized to patients with cystic fibrosis in a short term study with results suggesting that this therapy is efficient at least on a biochemical basis. We performed in vitro and in vivo animal studies with the recombinant form of SLPI (rSLPI). In these experiments we found that aerosolized rSLPI a) is not altered in its form or function in the first 13 h following aerosol; b) has a half-tie of 12 h in the lung; c) reaches the interstitium of the lung in intact form.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Therapy of lung diseases with antiproteases]. 791 Apr 1


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