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)

Histiocytosis X (HX), also referred as Langerhans cell granulomatosis is a disorder characterized by the presence of destructive granulomas containing Langerhans cells, lymphocytes, eosinophils and fibroblastes in the involved organs. Three presentations are commonly observed: 1) nonproductive cough or effort dyspnea, 2) spontaneous pneumothorax 3) incidental pulmonary infiltrates on chest X-ray in asymptomatic patients. HRCT may be helpful in the initial diagnosis of pulmonary HX. HRCT scans show nodules, cysts and estimate the extent of disease. But the final diagnosis of histiocytosis X requires the histologic demonstration of specific histiocytosis X cell in biopsy specimens of the lung. The aim of this study was to define the importance of the detection of Langerhans cells in bronchoalveolar lavage fluid (BALF) for the diagnosis of HX. The searched cells express a specific CD1 antigen, recognized by the monoclonal antibody OKT-6. In our study the demonstration of more than 5% of CD1 positive cells was defined to confirm HX. We have studied the BALF in 21 patients with suspected histiocytosis X. In BALF of 4 patients more than 5% of CD1 positive cells were found. In 1 of them HX was confirmed with open lung biopsy. Two patients displayed 5% of CD1 positive cells. The final diagnosis of the first patient was hypersensitivity pneumonitis and of the second one was bronchitis chronica. In 5 patients out of 15 patients in whom less than 5% of CD1 positive cells were found histiocytosis X was histologically proven. In other 10 patients the following disorders were histologically recognised: pulmonary emphysema 3 cases, pneumoconiosis-3, LMA-BOOP-1, sarcoidosis-1 and pleuritis eosinophilica-1. The estimation of Langerhans cells in BALF can be a useful method among the diagnostic procedures for histiocytosis X. It is necessary to remember that demonstration of less than 5% of CD1 positive cells do not exclude histiocytosis X.
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PMID:[Usefulness of CD1 expression on surfaces of cells in bronchoalveolar fluid for diagnosis of histiocytosis X--our experience]. 1064 82

Chronic inflammation in many pulmonary diseases, such as sarcoidosis and IPF, lead to end-stage lung disease and fibrosis. In other diseases, such as chronic thromboembolic disease and emphysema, long-term complications can result in pulmonary hypertension and cor pulmonale. Therapeutic options for end-stage lung disease are quite limited. One possible solution is lung transplantation. Although fraught with potential serious complications, including infection, rejection, and death, lung transplantation may offer overall improvement in mortality rates and quality of life.
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PMID:Update in pulmonary medicine. 1180 59

Endothelial adherence and migration of leukocytes into tissue is mediated by different sets of adhesion molecules. The expression of these sets might not only preselect the types of leukocytes that enter the inflammatory sites, but also activate these leukocytes, induce adherence to epithelial cells, and cause the release of cytokines. Atopic asthma, extrinsic allergic alveolitis, and sarcoidosis as examples of immunologic lung diseases were investigated for the expression of adhesion molecules. Bronchial biopsies in chronic obstructive lung disease (COPD) and resected lung tissue of juvenile emphysema were chosen for controls. Immunohistochemistry was done on sections from bronchial and transbronchial biopsies and on smears from bronchoalveolar lavage cells. In all three types of immune disorders, lymphocytes expressed the integrins alpha4/beta1 (VLA4) and ICAM3, whereas lymphocytes in COPD bronchitis and in emphysema controls were unreactive. Eosinophils in atopic asthma bronchitis in contrast to COPD bronchitis also expressed both VLA4 and ICAM3. The expression of VCAM1 on endothelial cells was only seen in atopic asthma and was related to disease activity. The expression of other adhesion molecules was nonspecific. Expression of VCAM1 on endothelial cells and its ligand VLA4 on lymphocytes and eosinophils seems to be a specific event in atopic asthma. Expression of VLA4 and ICAM3 on lymphocytes, however, might be a specific event in all three immune reactions.
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PMID:Expression of adhesion molecules in allergic lung diseases. 1196 48

Vascular endothelial growth factor (VEGF) plays multifunctional roles in both the development of vasculature and the maintenance of vascular function. A decrease in VEGF reduces angiogenesis and induces apoptosis of vascular endothelial cells. Inhibition of the VEGF receptor causes endothelial cell apoptosis and emphysema. We postulated that VEGF concentrations might be reduced in patients with chronic lung diseases. The level of VEGF was evaluated by enzyme-liked immunosorbent assay in bronchoalveolar lavage fluid (BALF) from normal smokers, nonsmoking volunteers, idiopathic pulmonary fibrosis, pulmonary fibrosis associated with a connective tissue disease, and sarcoidosis. The isoforms of VEGF in BALF were determined by high-performance liquid chromatography. VEGF in nonsmoking volunteers was detectable at a high concentration. In contrast, VEGF in most of the normal smokers was below the detectable limit. The VEGF found in nonsmoking volunteers BALF was VEGF165. VEGF was significantly decreased in idiopathic pulmonary fibrosis, pulmonary fibrosis associated with a connective tissue disease, and sarcoidosis compared with nonsmoking volunteers. The smoking patients showed a further decrease in VEGF. These data suggest that the decrease in VEGF in smokers and patients with chronic lung diseases may reduce angiogenesis and induce apoptosis of vascular endothelial cells.
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PMID:Decreased level of vascular endothelial growth factor in bronchoalveolar lavage fluid of normal smokers and patients with pulmonary fibrosis. 1215 75

The aim of this pictorial essay is to illustrate the distribution of normal findings and various lung diseases on coronal reconstructions as compared with cross-sectional high-resolution CT images. The volumetric CT images were obtained at 120 kVp, 200 mA/rotation, pitch 6:1, and a high-spatial-frequency reconstruction algorithm. The scans were performed using 2.5-mm collimation and reconstructed at 1.25-mm intervals. Coronal reconstructions were obtained at 2.5-mm slice thickness and 2.5-mm intervals. The pictorial includes images of normal anatomy, emphysema, idiopathic pulmonary fibrosis, Langerhans cell histiocytosis, sarcoidosis, bronchiolitis obliterans organizing pneumonia, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, lymphocytic interstitial pneumonia, pulmonary edema, tuberculosis, cytomegalovirus pneumonia, bronchiectasis, and panbronchiolitis.
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PMID:Multidetector spiral high-resolution computed tomography of the lungs: distribution of findings on coronal image reconstructions. 1236 68

Sarcoidosis is defined as a multisystem disease of unknown genesis that affects in particular the middle-aged. In 80% of the cases, the acute form is associated with pulmonary manifestations, and in the majority of patients clears up--even in the absence of treatment--with no sequelae. The chronic version is characterized by the development of multiple pulmonary granulomas and progressive fibrosis. Subsequently, emphysema, cor pulmonale and right heart insufficiency develops. Almost 50% of chronic sarcoidosis patients have cardiac involvement with arrhythmias, heart block and the clinical picture of a cardiomyopathy, which in many cases leads to death--acutely (sudden cardiac death) or over the longer term. In manifest disease, treatment with steroids should be initiated and continued over a year. Conduction disorders require the implantation of a pacemaker.
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PMID:[Heart failure, skin manifestations, enlarged hilar lymph nodes. Is it sarcoidosis?]. 1465 79

Pulmonary surfactant, a complex of lipids and proteins, maintains alveolar integrity and participates in the control of host defense and inflammation in the lung. Surfactant proteins A, B, C, and D are important components of surfactant that play diverse roles in the surface tension reducing as well as host defense and inflammation control functions of surfactant. Hyperoxia or exposure of cells/tissues to elevated levels of oxygen occurs when high levels of oxygen are used to treat a variety of pulmonary disorders that include respiratory distress syndrome of premature infants, emphysema, sarcoidosis, end-stage lung diseases, and others. The lung serves as a primary target organ in hyperoxia, and hyperoxic lung injury is characterized by pulmonary edema, inflammation, and respiratory failure. Hyperoxic lung injury is associated with significant changes in the expression of surfactant proteins that likely serves as an adaptive response to elevated oxygen levels. In most animal species studied, hyperoxia increases the tissue expression of surfactant protein mRNAs. A limited number of studies have indicated that the increased tissue expression of surfactant protein mRNAs is associated with increased levels of surfactant proteins in the bronchoalveolar lavage.
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PMID:Regulation of surfactant protein gene expression by hyperoxia in the lung. 1471 50

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

To elucidate the biological significance of selectin for idiopathic pulmonary fibrosis, we titrated the serum soluble E-selectin. From 31 cases of idiopathic pulmonary fibrosis patients without signs or symptoms of infection, the serum was obtained and the concentration was titrated by enzyme-linked immunosorbent assay. The serum soluble E-selectin titer was significantly higher than that of healthy controls. However, significant elevation was not observed in the sera from the patients with other pulmonary diseases, such as pulmonary emphysema, sarcoidosis, or bronchiectasis. In the patients with idiopathic pulmonary fibrosis, the number of white blood cells, C-reactive protein or lactate dehydrogenase activity did not show a significant relationship with the soluble E-selectin titer. About 16 out of the 31 idiopathic fibrosis patients, the serum surfactant apoprotein-A titer, which is a parameter of the disease activity of idiopathic pulmonary fibrosis, was also tested. The surfactant apoprotein-A titer was loosely correlated with the soluble E-selectin titer. These observations suggest that E-selectin may be relevant to the pathogenesis of idiopathic pulmonary fibrosis, and it may be a novel clinical parameter for idiopathic pulmonary fibrosis.
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PMID:Increased level of soluble E-selectin in the serum from patients with idiopathic pulmonary fibrosis. 1507 24

Central bronchi and pulmonary arteries are surrounded and enveloped by a strong connective-tissue sheath termed the peribronchovascular interstitium, extending from the level of the pulmonary hila into the peripheral lung. Thickening of the peribronchovascular interstitium can be seen in a wide variety of diseases. The CT appearance can be smooth, nodular, or irregular depending on the underlying cause. Many of the diseases affecting the peribronchovascular interstitium are entities that show a predilection for lymphatic routes, such as sarcoidosis, pulmonary lymphangitic carcinomatosis, silicosis, and lymphoproliferative disorders. There are other entities that mainly affect the peribronchovascular interstitium without a predominant perilymphatic distribution, such as hydrostatic pulmonary edema, cryptogenic organizing pneumonia, Kaposi's sarcoma, interstitial pulmonary emphysema, and interstitial hemorrhage. Although there is an overlap in radiologic features, some CT findings are useful in differentiating among these entities. When CT shows mainly peribronchovascular abnormality, the differential diagnosis is considerably reduced, and it is generally possible to reach the correct diagnosis by considering the clinical context. We illustrate the CT findings and pathologic correlation for a number of different disorders that mainly affect the peribronchovascular interstitium.
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PMID:Diseases affecting the peribronchovascular interstitium: CT findings and pathologic correlation. 1575 80


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