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)

A population (n = 526), consisting of employees with COPD, was compared with 2 control populations for the prevalence of Pi-phenotypes. In the patient group, the proportions of ZZ, SZ and MZ were significantly elevated. Among the patient population a prospective study was carried out to evaluate the role of alpha 1-antitrypsin deficiency as a cofactor in COPD. Severity of disease was estimated by standard pulmonary function tests, X-ray signs for emphysema and clinical assessment. Patients with ZZ, SZ and MZ were significantly worse than their MM partners. An influence of MS cannot be rejected. Phenotyping of all patients with COPD is advocated. Screening can also be by determining the ratio of alpha 1-antitrypsin and acid alpha 1-glycoprotein concentrations, which allowed detection of all ZZ, SZ, MZ and about 60% of the MS patients.
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PMID:Heterozygosity in the Pi-system as a pathogenetic cofactor in chronic obstructive pulmonary disease (COPD). 387 84

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

Monodisperse aerosol particles can be used to assess noninvasively intrapulmonary airspace dimensions. Since emphysematic changes in the peripheral lung are difficult to detect with most of the common lung function tests, aerosol-derived airway morphometry was used to assess the peripheral airspace dimensions (EAD800) in 25 patients with COPD and in 36 healthy volunteers. Spirometric and body plethysmographic measurements were performed in all patients. In ten patients, high-resolution CT-derived mean lung density (MLD) was additionally assessed. In healthy subjects, EAD800 was 0.39 +/- 0.05 mm. In patients, EAD800 was significantly increased (0.82 +/- 0.33 mm). In a subset of nine patients with severe alpha 1-antitrypsin deficiency and clinically severe emphysema, EAD800 was even larger (1.14 +/- 0.32 mm). In patients, EAD800 correlated with MLD (r = 0.82), diffusion capacity (DCO) (r = 0.78), and FEV1 (r = -0.75). Since MLD is considered a valid indicator for lung emphysema, the close correlation between EAD800 and MLD suggests that EAD800 reflects enlarged peripheral airspace dimensions in patients with emphysema.
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PMID:Peripheral airspace dimensions in patients with COPD. 755 76

A mathematical simulation of the maximum expiratory flow-volume (MEFV) curve was developed using a lumped parameter model. The model uses a theoretical approximation of an activation function representing the lung's pressure-volume relationship during maximally forced expiration. The waveforms obtained by the model were compared to the flow-volume curves recorded from normal subjects and for patients with small airways disease, asthma, and emphysema. We were able to reproduce the flow-volume curves using the model and calculate new parameters that reflect the dependency of airways resistance on expired volume during FVS manoeuvre. These new parameters are based on the entire information presented in the flow-volume curve and on the reduction in flow at all lung volumes. We also calculated the mean slope of the resistance-expired volume curves obtained from the model by fitting a straight line to the curve. Using representative data for normal and COPD patients different mean slopes of 0.095, 0.13, 0.49 and 1.44 litre-1 were obtained for normal subject, small airways disease, asthma and emphysema patients, respectively. The model-based parameters may be applicable to human studies. However, further studies in large groups of patients are required to better define the true predictive value of the new indices described for the diagnosis of COPD.
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PMID:Maximum expiratory flow-volume curve: mathematical model and experimental results. 767 Jun 92

An enormous pulmonary cyst (phi approx. 20 cm) in a 72-year-old male patient with large bullous pulmonary emphysema caused compression of the right lung and the mediastinum with consecutive, O2-dependent dyspnoea at rest. The symptoms did not improve under conservative therapy of an accompanying COPD. Thus, in spite of two heart attacks in the previous history, an operation with bullectomy was indicated. A thoracotomy had to be avoided because of the very high cardiac risk. In the present case, a minimally invasive procedure enabled the complete cyst resection with a smooth postoperative course and an excellent functional therapeutic result.
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PMID:[Minimally invasive surgery of large bullous pulmonary emphysema]. 775 71

For patients with COPD, FEV1 has been shown to be the single most important factor to influence survival. Patients with alpha 1-antitrypsin deficiency (alpha 1ATD) develop emphysema at a young age, especially if they smoke, and the purpose of this study was to calculate survival with respect to the initial FEV1 and smoking history. For this study, patients with Pi-type ZZ were analyzed. FEV1 was divided in four groups: below 25%, 25 to 49%, 50 to 74%, and more than 75% of predicted. A total of 347 patients with 107 deaths were available for analysis. Three patients were lost to follow-up, and 17 had received a lung transplant. There was no significant difference in survival between males and females. The median survival time for patients with an initial FEV1 below 25% was 6.3 yr (95% CI 5.2-8.0). For the FEV1 groups 25 to 49% and 50 to 74%, median survival times were: 10.5 (9.2-11.9) and 14.2 (12.4-infinity) yr, respectively. The median survival for the above 75% group was not calculable. For patients with an initial FEV1 less than 50%, smokers had a poorer survival rate than patients who quit smoking during the study period (p < 0.01). We conclude that for patients with severe alpha 1ATD, FEV1 is a very strong predictor of survival regardless of sex. Patients who quit smoking had a better survival rate than patients who continued to smoke.
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PMID:Survival in relation to lung function and smoking cessation in patients with severe hereditary alpha 1-antitrypsin deficiency. 784 93

1. In 1992, there were 2,171 heart, 48 heart-lung, and 535 lung transplants performed in the United States. The number of lung transplants increased by about 32% over 1991, whereas heart transplants only increased by about 2% over 1991. The number of heart-lung transplants decreased in 1992. 2. The number of programs performing heart and lung transplants has continued to increase: about 16% in heart and about 280% in lung, since 1988. 3. The most frequently reported primary indications for transplant were: coronary artery disease (44%) and cardiomyopathy (41%) in heart; cystic fibrosis (40%) in double lungs; emphysema/COPD (35%) in single lungs; and congenital/Eisenmenger's syndrome (36%) in heart-lung. 4. Between 1988 and 1992, the following groups showed significant increases for heart and lung transplants: pediatric transplants, non-White recipients, non-White donors, older donors, and local utilization. 5. For the entire period covered by this report, overall one-year patient survival rates were: heart 82.3%, single lung 70.1%, double lung 66.4%, and heart-lung 58.7%. There has been little change in one-year heart transplant survival rates during this time. Survival rates for lung and heart-lung transplants increased dramatically between 1988 and 1990, but have declined slightly since then.
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PMID:Thoracic organ transplants in the United States from October 1987 through December 1992: a report from the UNOS Scientific Registry for Organ Transplants. 791 70

Current concepts of pathomechanisms leading to acquired emphysema suggest that alveolar macrophages (AM) activated by cigarette smoking may cause an elastase/antielastase imbalance localized to the microenvironment formed by phagocytes and lung tissue. A functional cell assay was used to evaluate the cell-associated elastinolytic activity of AM. AM were obtained by bronchoalveolar lavage from patients with emphysema and from patients with non obstructive chronic pulmonary diseases (non-COPD) and cultured under serum-free conditions in direct contact with 3H-labeled elastin particles. Elastinolytic activity was calculated from the released radioactivity in culture supernatants and expressed as micrograms of 3H-elastin degraded x 10(-5) AM x 72 h-1. AM of patients with emphysema had significantly higher elastinolytic activity compared to that of non-COPD patients (median: 10.8 versus 4.1 micrograms; P < 0.01). Further differentiation of patients revealed the lowest median activity in sarcoidosis (2.3 micrograms). In respect to smoking habits there was a major difference between smokers with emphysema degraded more than twice the amount of elastin than smokers in the non-COPD group (median:11 versus 3.9 micrograms, P = 0.01). From these data we conclude that AM-derived elsatinolytic proteases may be involved in the destruction of lung elastin, which is thought to be the key event occurring in the pathogenesis of pulmonary emphysema.
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PMID:Elastinolytic activity of alveolar macrophages in smoking-associated pulmonary emphysema. 804 73

We studied the effects of inhaled anticholinergic (oxitropium bromide, 0.2 mg) and beta-adrenergic (fenoterol 0.4 mg) drugs on respiratory function and arterial blood gases in fourteen male patients with pulmonary emphysema (age 63 +/- 8 years; FEV1/FVC 1.41 +/- 0.73 L) using a three day, randomized, double-blind placebo-controlled design. Each day, spirometry and arterial blood gas analyses were performed before and 45 min after inhalation. Both fenoterol and oxitropium bromide significantly improved FEV1 by 21% and 16%, respectively, although there was no significant difference between the two drugs. However, the mean value of PaO2 significantly decreased from 74.5 +/- 2.6 to 69.3 +/- 2.7 Torr with fenoterol, but not with oxitropium and placebo. Change of PaO2 with fenoterol was significantly correlated with %FRC, but not with FEV1 nor change of FEV1. We therefore conclude that an anticholinergic inhalant bronchodilator is more favorable for patients with COPD than a beta-adrenergic agent.
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PMID:[Effects of inhaled beta-adrenergic and anticholinergic drugs on respiratory function and arterial blood gases in patients with pulmonary emphysema]. 809 80

A retrospective study to investigate the prevalence and severity of impairment of the lung diffusing capacity for carbon monoxide (DCO) in patients with lung carcinoma was done using a case-control matched design. A group of 70 patients with the histologic diagnosis of lung carcinoma was matched on a one-to-one basis with a group of patients with chronic bronchitis and/or pulmonary emphysema (COPD); the matching was done based on the FEV1 within 10 percent of each other for all pairs. The groups of lung carcinoma and COPD had similar history of smoking consumption and pulmonary function, except that the Dco was reduced in the carcinoma group (mean +/- SD: 56.4 +/- 12.8 percent of predicted) compared with the control group (64.4 +/- 20.1 percent of predicted; p = 0.0027). Among the carcinoma group, only one patient had a Dco above 75 percent of the predicted value, while in the control group, 22 patients had a Dco above 75 percent of predicted values. It appears that Dco is more frequently reduced in patients with lung carcinoma than in a group of patients with similar FEV1 and smoking history who have chronic bronchitis and/or emphysema but had no evidence of lung carcinoma. We speculate that the reduction of Dco in patients with lung carcinoma could be due to the concurrent presence of pulmonary emphysema.
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PMID:Reduction of lung diffusion for carbon monoxide in patients with lung carcinoma. 813 54


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