Pulmonary Emphysema: Enlargement of air spaces distal to the TERMINAL BRONCHIOLES where gas-exchange normally takes place. This is usually due to destruction of the alveolar wall. Pulmonary emphysema can be classified by the location and distribution of the lesions.Emphysema: A pathological accumulation of air in tissues or organs.Pancreatic Elastase: A protease of broad specificity, obtained from dried pancreas. Molecular weight is approximately 25,000. The enzyme breaks down elastin, the specific protein of elastic fibers, and digests other proteins such as fibrin, hemoglobin, and albumin. EC 3.4.21.36.alpha 1-Antitrypsin Deficiency: Deficiency of the protease inhibitor ALPHA 1-ANTITRYPSIN that manifests primarily as PULMONARY EMPHYSEMA and LIVER CIRRHOSIS.Subcutaneous Emphysema: Presence of air or gas in the subcutaneous tissues of the body.alpha 1-Antitrypsin: Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.Lung: Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.Matrix Metalloproteinase 12: A secreted matrix metalloproteinase which is highly expressed by MACROPHAGES where it may play a role in INFLAMMATION and WOUND HEALING.Pulmonary Alveoli: Small polyhedral outpouchings along the walls of the alveolar sacs, alveolar ducts and terminal bronchioles through the walls of which gas exchange between alveolar air and pulmonary capillary blood takes place.Mediastinal Emphysema: Presence of air in the mediastinal tissues due to leakage of air from the tracheobronchial tree, usually as a result of trauma.Respiratory Function Tests: Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc.SmokeHelium: Helium. A noble gas with the atomic symbol He, atomic number 2, and atomic weight 4.003. It is a colorless, odorless, tasteless gas that is not combustible and does not support combustion. It was first detected in the sun and is now obtained from natural gas. Medically it is used as a diluent for other gases, being especially useful with oxygen in the treatment of certain cases of respiratory obstruction, and as a vehicle for general anesthetics. (Dorland, 27th ed)Pulmonary Disease, Chronic Obstructive: A disease of chronic diffuse irreversible airflow obstruction. Subcategories of COPD include CHRONIC BRONCHITIS and PULMONARY EMPHYSEMA.Pneumonectomy: The excision of lung tissue including partial or total lung lobectomy.Vasculitis, Leukocytoclastic, Cutaneous: Disorder characterized by a vasculitic syndrome associated with exposure to an antigen such as a drug, infectious agent, or other foreign or endogenous substance. Its pathophysiology includes immune complex deposition and a wide range of skin lesions. Hypersensitivity or allergy is present in some but not all cases.ElastinPanniculitis: General term for inflammation of adipose tissue, usually of the skin, characterized by reddened subcutaneous nodules.Smoking: Inhaling and exhaling the smoke of burning TOBACCO.Tomography, X-Ray Computed: Tomography using x-ray transmission and a computer algorithm to reconstruct the image.Bronchoalveolar Lavage Fluid: Washing liquid obtained from irrigation of the lung, including the BRONCHI and the PULMONARY ALVEOLI. It is generally used to assess biochemical, inflammatory, or infection status of the lung.Forced Expiratory Volume: Measure of the maximum amount of air that can be expelled in a given number of seconds during a FORCED VITAL CAPACITY determination . It is usually given as FEV followed by a subscript indicating the number of seconds over which the measurement is made, although it is sometimes given as a percentage of forced vital capacity.Pulmonary Fibrosis: A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.Lung Compliance: The capability of the LUNGS to distend under pressure as measured by pulmonary volume change per unit pressure change. While not a complete description of the pressure-volume properties of the lung, it is nevertheless useful in practice as a measure of the comparative stiffness of the lung. (From Best & Taylor's Physiological Basis of Medical Practice, 12th ed, p562)Pneumonia: Infection of the lung often accompanied by inflammation.Disease Models, Animal: Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.Lung Diseases: Pathological processes involving any part of the LUNG.Bronchi: The larger air passages of the lungs arising from the terminal bifurcation of the TRACHEA. They include the largest two primary bronchi which branch out into secondary bronchi, and tertiary bronchi which extend into BRONCHIOLES and PULMONARY ALVEOLI.Mice, Inbred C57BLTobacco: A plant genus of the family SOLANACEAE. Members contain NICOTINE and other biologically active chemicals; its dried leaves are used for SMOKING.Cough: A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation. It is a protective response that serves to clear the trachea, bronchi, and/or lungs of irritants and secretions, or to prevent aspiration of foreign materials into the lungs.Lung Diseases, Obstructive: Any disorder marked by obstruction of conducting airways of the lung. AIRWAY OBSTRUCTION may be acute, chronic, intermittent, or persistent.PubMed: A bibliographic database that includes MEDLINE as its primary subset. It is produced by the National Center for Biotechnology Information (NCBI), part of the NATIONAL LIBRARY OF MEDICINE. PubMed, which is searchable through NLM's Web site, also includes access to additional citations to selected life sciences journals not in MEDLINE, and links to other resources such as the full-text of articles at participating publishers' Web sites, NCBI's molecular biology databases, and PubMed Central.Periodicals as Topic: A publication issued at stated, more or less regular, intervals.BooksInternal Medicine: A medical specialty concerned with the diagnosis and treatment of diseases of the internal organ systems of adults.Evidence-Based Medicine: An approach of practicing medicine with the goal to improve and evaluate patient care. It requires the judicious integration of best research evidence with the patient's values to make decisions about medical care. This method is to help physicians make proper diagnosis, devise best testing plan, choose best treatment and methods of disease prevention, as well as develop guidelines for large groups of patients with the same disease. (from JAMA 296 (9), 2006)Weight Gain: Increase in BODY WEIGHT over existing weight.Air Sacs: Thin-walled sacs or spaces which function as a part of the respiratory system in birds, fishes, insects, and mammals.SkatoleVeterinary Medicine: The medical science concerned with the prevention, diagnosis, and treatment of diseases in animals.Hypertension, Pulmonary: Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.Syndrome: A characteristic symptom complex.Pulmonary Medicine: A subspecialty of internal medicine concerned with the study of the RESPIRATORY SYSTEM. It is especially concerned with diagnosis and treatment of diseases and defects of the lungs and bronchial tree.Dyspnea: Difficult or labored breathing.Spirometry: Measurement of volume of air inhaled or exhaled by the lung.Telepathology: Transmission and interpretation of tissue specimens via remote telecommunication, generally for the purpose of diagnosis or consultation but may also be used for continuing education.Urology: A surgical specialty concerned with the study, diagnosis, and treatment of diseases of the urinary tract in both sexes, and the genital tract in the male. Common urological problems include urinary obstruction, URINARY INCONTINENCE, infections, and UROGENITAL NEOPLASMS.Bronchitis, Chronic: A subcategory of CHRONIC OBSTRUCTIVE PULMONARY DISEASE. The disease is characterized by hypersecretion of mucus accompanied by a chronic (more than 3 months in 2 consecutive years) productive cough. Infectious agents are a major cause of chronic bronchitis.Bronchitis: Inflammation of the large airways in the lung including any part of the BRONCHI, from the PRIMARY BRONCHI to the TERTIARY BRONCHI.Nephrology: A subspecialty of internal medicine concerned with the anatomy, physiology, and pathology of the kidney.

Fragile lung in the Marfan syndrome. (1/1021)

Two cases of the Marfan syndrome presented with spontaneous pneumothorax. Both had chest radiographs showing bilateral bullae in the upper lung zones and pulmonary function tests consistent with mild emphysema. There were dereases in forced expiratory flow rates at low lung volumes, carbon monoxide transfer factor, and lung elastic recoil. It is suggested that pneumothorax and bullous emphysema in this syndrome are caused by a weakness in the pulmonary connective tissue framework.  (+info)

Detection of anti-cytokeratin 8 antibody in the serum of patients with cryptogenic fibrosing alveolitis and pulmonary fibrosis associated with collagen vascular disorders. (2/1021)

BACKGROUND: It has been suggested that the humoral immune system plays a role in the pathogenesis of cryptogenic fibrosing alveolitis (CFA). Although circulating autoantibodies to lung protein(s) have been suggested, none of the lung proteins have been characterised. The purpose of this study was to determine the antigen to which the serum from patients with pulmonary fibrosis reacted. METHODS: The anti-A549 cell antibody was characterised in a patient with CFA using Western immunoblotting and immunohistochemical staining of A549 cells. As we identified that one of the antibodies against A549 cells was anti-cytokeratin 8, the expression of mRNA of cytokeratin 8 in A549 cells was evaluated. In addition, we attempted to establish an enzyme linked immunosorbent assay to measure the levels of anti-cytokeratin 8 antibody in the serum of patients with CFA and pulmonary fibrosis associated with collagen vascular disorders (PF-CVD). RESULTS: Initially two anti-A549 cell antibodies were detected in the serum of patients with pulmonary fibrosis, one of which was characterised as anticytokeratin 8 antibody by Western immunoblotting. We were able to establish an ELISA to measure anti-cytokeratin 8 antibody and found significantly higher levels in patients with CFA and PF-CVD than in normal volunteers, patients with sarcoidosis, pneumonia, and pulmonary emphysema. CONCLUSIONS: One of the anti-A549 cell antibodies in the serum of patients with CFA was against cytokeratin 8. The serum levels of anti-cytokeratin 8 antibody were increased in patients with CFA and PF-CVD. These results suggest that anticytokeratin 8 antibody may be involved in the process of lung injury in pulmonary fibrosis.  (+info)

Improvements in thoracic movement following lung volume reduction surgery in patients with severe emphysema. (3/1021)

In twelve patients with severe emphysema who underwent lung volume reduction surgery (LVRS), we assessed the results of dyspnea scale, pulmonary function, 6-minute walk distance (6MD), and thoracic movement prior to and 6 months following LVRS. Postoperatively, forced expiratory volume (FEV1), maximum inspiratory mouth pressures (MIP), maximum expiratory mouth pressures (MEP), maximum voluntary ventilation (MVV), diffusing capacity for carbon monoxide (DLCO), partial pressure of oxygen (PaO2) and 6MD were significantly increased with the decrease in dyspnea scale and lung hyperinflation. Thoracic movement, as assessed by the bilateral lung area ratio of the mid-sagittal dimension of dynamic magnetic resonance imaging (MRI) at full inspiration to that at full expiration, was significantly increased. The improvement in thoracic movement was significantly correlated with the increases in FEV1, MVV, and MIP, and with the decrease in residual volume (RV), and with the improvement in the dyspnea scale. These findings suggest that LVRS is an effective procedure for improving not only the airflow limitation and gas exchange but also the thoracic movement in severe emphysema, and these improvements may contribute to an increase in exercise performance and relief of dyspnea.  (+info)

Physiological basis of improvement after lung volume reduction surgery for severe emphysema: where are we? (4/1021)

Lung volume reduction surgery has become an accepted therapeutic option to relieve the symptoms of selected patients with severe emphysema. In a majority of these patients, it causes objective as well as subjective functional improvement. A proper understanding of the physiological determinants underlying these beneficial effects appears very important in order to better select patients for the procedure that is currently largely carried out on an empirical basis. Lung volume reduction surgery has two distinct effects. Firstly, it causes an increased elastic recoil, which at least partially explains the enhanced maximal expiratory flow. Secondly, it is associated with a reduction of hyperinflation which allows for an increase in global inspiratory muscle strength and in diaphragmatic contribution to tidal volume as well as a decrease in the inspiratory elastic load imposed by the chest wall. Taken together, these effects result in a reduced work of breathing and in an enhanced maximal ventilation which both contribute to the increased exercise capacity and reduced dyspnoea after surgery. The improved lung recoil and the reduced hyperinflation after volume reduction surgery were the primary postulates upon which the usual selection criteria for the procedure were based. It is now likely that these are correct. Nevertheless, some patients do not benefit from lung volume reduction surgery and the current literature does not allow for a refinement of the selection process from a physiological point of view. The exact mechanisms underlying the improvement in lung recoil, lung mechanics, and respiratory muscle function remain incompletely understood. Moreover, the effects of lung volume reduction surgery on gas exchange and pulmonary haemodynamics still need to be more fully investigated. An analysis of the characteristics of patients who do not benefit from the procedure and the development of an animal model for lung volume reduction surgery would probably help address these important issues.  (+info)

Subjective differentiation of normal and pathological bronchi on thin-section CT: impact of observer training. (5/1021)

The effect of observer training on sensitivity, specificity and interobserver agreement in the differentiation between normal and pathological bronchi on computed tomography (CT) was studied. The wall thickness of bronchi with normal walls and with pathologically thickened walls were subjectively scored by three independent observers before and after a training period of 2 weeks. Sensitivity, specificity and interobserver agreement were calculated for reading sessions before and after training. Increase and decrease in agreement after training were determined. There was a statistically significant difference (p=0.001) between objectively measured wall thickness of normal and pathological bronchi, both for reference bronchi and for bronchi used for reading sessions. While training increased interobserver agreement, it had no effect on sensitivity (0.46 versus 0.44 after training) and specificity (0.71 versus 0.72 after training) in detecting pathological bronchi. Increased agreement after training was significantly (p=0.001) more frequent than decreased agreement. There is a discrepancy between the effect of training on interobserver agreement and on sensitivity and specificity in the subjective differentiation between normal and pathological bronchi. Interobserver agreement alone is not a reliable indicator of a beneficial effect of training in the evaluation of this parameter.  (+info)

Outcome of asthma: longitudinal changes in lung function. (6/1021)

Current knowledge about factors determining outcome of asthma is limited, but observations over the last few decades suggest that active asthma has a negative impact on the longitudinal changes in lung function. This review aims to give an overview of the present knowledge concerning longitudinal changes in lung function, including clinical markers for distinctly poor outcome with regard to lung function, in children and adults suffering from asthma. The majority of patients with asthma have a good prognosis. However, some patients with asthma, especially those with more severe disease, are at risk of impaired growth of lung function during childhood, a lower maximally attained level of lung function and excessive decline in lung function in adulthood, which may lead to life-threatening lung function impairment. Clinical markers of poorly controlled airway inflammation appear to have a negative impact on the longitudinal changes in lung function, and disease progression to nonreversible airflow obstruction may be observed in a minority of patients with asthma. Early intervention with anti-inflammatory therapy may improve the short-term outcome of asthma, but long-term controlled studies are clearly needed in order to verify whether or not treatment, especially with inhaled corticosteroids, according to the current international guidelines alters the natural history of asthma, i.e. disease progression with regard to changes in lung function and possible development of nonreversible airflow obstruction.  (+info)

Surgical aspects and techniques of lung volume reduction surgery for severe emphysema. (7/1021)

Lung volume reduction surgery (LVRS) has become an accepted procedure for palliative treatment of diffuse, nonbullous emphysema. Single or multiple peripheral segmental wedge resections of the most destroyed areas of the lungs are performed with the use of stapling devices, in order to decrease hyperinflation and restore diaphragmatic function. Median sternotomy, videoendoscopy or anterior muscle sparing thoracotomies have been used as surgical approaches. The functional improvement after bilateral resections exceed those after a unilateral approach. LVRS has demonstrated its potential as an alternative to transplantation, and with growing experience, the indications for the procedure have been widened. In selected patients with peripheral lung cancer who have been considered unsuitable for a surgical resection, the combination of both tumour resection and LVRS has successfully been performed. In contrast to LVRS, laser surgery of the emphysematous lung has been abandoned in most institutions.  (+info)

Emphysematous lesions, inflammation, and fibrosis in the lungs of transgenic mice overexpressing platelet-derived growth factor. (8/1021)

Because of its expression pattern and its potent effects on mesenchymal cells, platelet-derived growth factor (PDGF) has been implicated as an important factor in epithelial-mesenchymal cell interactions during normal lung development and in the pathogenesis of fibrotic lung disease. To further explore the role of PDGF in these processes, we have developed transgenic mice that express the PDGF-B gene from the lung-specific surfactant protein C (SPC) promoter. Adult SPC-PDGFB transgenic mice exhibited lung pathology characterized by enlarged airspaces, inflammation, and fibrosis. Emphysematous changes frequently occurred throughout the lung, but inflammation and fibrotic lesions were usually confined to focal areas. The severity of this phenotype varied significantly among individual mice within the same SPC-PDGFB transgenic lineage. A pathology similar to that observed in adult mice was noted in lungs from transgenic mice as young as 1 week of age. Neonatal transgenic mice exhibited enlarged saccules and thickened primary septa. Results of these studies indicated that overexpression of PDGF-B induced distinct abnormalities in the developing and adult lung and led to a complex phenotype that encompassed aspects of both emphysema and fibrotic lung disease.  (+info)

  • Functional and radiological findings have showed that these patients are suffering from severe breathlessness, but whose pulmonary functional tests revealed no signs of obstruction, normal static lung volumes, and depressed DLco, most with a history of smoking . (scirp.org)
  • The RePneu LVRC, a minimally invasive medical implant designed to treat the symptoms of severe emphysema, is intended to improve lung function, exercise ability and quality of life for this underserved patient population. (mdtmag.com)
  • There is no cure for severe emphysema, and patients have very few options available to relieve their symptoms," he said. (mdtmag.com)
  • We have seen significant clinical improvements in treated patients, and we believe this is an important treatment option for many patients with severe emphysema. (mdtmag.com)
  • The RENEW trial is enrolling patients throughout the US and Europe who have been clinically diagnosed with severe emphysema and are experiencing increasing difficulty with simple daily tasks despite optimal medical management. (mdtmag.com)
  • Though RePneu LVRC is investigational in the United States and not yet approved for commercial use by the FDA, the device has been used in Europe - in clinical trials and commercially - for the treatment of severe emphysema since 2008. (mdtmag.com)
  • This paper utilizes trial data to identify the predictors of HRQoL in patients with severe emphysema, and subsequently estimates the impact of a new treatment on HRQoL (measured by utilities). (uzh.ch)
  • Conclusions: Coils and SoC resulted in statistically significant improvements in HRQoL compared to SoC alone in patients with severe emphysema. (uzh.ch)
  • The 17 NETT clinical centers studied more than 1,200 patients who had severe emphysema. (nih.gov)
  • The NETT clarified the short-term and long-term risks and benefits of LVRS as a treatment for severe emphysema. (nih.gov)
  • A disorder marked by pulmonary inflation, primarily affecting the respiratory bronchioles and usually more severe in the upper lobes. (thefreedictionary.com)
  • Pulmonary emphysema is defined as the permanent enlargement of airspaces distal to the terminal bronchioles and the destruction of the alveolar walls. (medscape.com)
  • To interpret these results, we established a novel simulation model and found that not only enlargement of preexisting low-attenuation areas but also coalescence of adjoining low-attenuation areas due to alveolar wall destruction caused emphysema progression in patients with exacerbations. (nih.gov)
  • Pulmonary emphysema, a significant global health problem, is characterized by a loss of alveolar structures. (jci.org)
  • Because VEGF is a trophic factor required for the survival of endothelial cells and is abundantly expressed in the lung, we hypothesized that chronic blockade of VEGF receptors could induce alveolar cell apoptosis and emphysema. (jci.org)
  • These findings suggest that VEGF receptor signaling is required for maintenance of the alveolar structures and, further, that alveolar septal cell apoptosis contributes to the pathogenesis of emphysema. (jci.org)
  • Regional emphysema severity was quantified in each tissue sample using the mean linear intercept (Lm) between alveolar walls from micro-CT scans. (biomedcentral.com)
  • Researchers identified two characteristics-emphysema mostly in the upper lobes of the lungs and low exercise capacity-that helped predict which patients would benefit from surgery. (nih.gov)
  • Emphysema causes irreversible damage to delicate lung tissue, leading to shortness of breath and even feelings of suffocation that result in reduced quality of life. (mdtmag.com)
  • There's little as frightening and frustrating as the feeling of not getting enough air, and this shortness of breath, can turn normal activities into major obstacles for people with emphysema. (integrisok.com)
  • To identify whether the extent of arterial stiffness is associated with emphysema severity. (nih.gov)
  • We identified 127 genes whose expression levels were significantly associated with regional emphysema severity while controlling for gene expression differences between individuals. (biomedcentral.com)
  • Methods: Patient level HRQoL data from RENEW and the National Emphysema Treatment Trial (NETT) were combined and mapped to generic EuroQol 5-dimension health utility questionnaire (EQ-5D) values using a published algorithm. (uzh.ch)
  • How was the National Emphysema Treatment Trial conducted? (nih.gov)
  • With you not having smoked for 20 years, it is quite unlikely that your current dry cough is a sign of emphysema. (medhelp.org)
  • It usually involves the upper lobe and is the most common type of emphysema. (humpath.com)
  • The newly published findings from the largest study of bilateral lung volume reduction surgery (LVRS) to treat severe emphysema also confirm that patients with upper-lobe emphysema and poor exercise capacity before surgery are more likely to have improved survival compared to similar patients treated with medical therapy without surgery. (psychcentral.com)
  • The aim of this study was to elucidate the roles of MafB in the development of pulmonary emphysema. (ijbs.com)
  • Acute bovine pulmonary emphysema and edema (ABPEE) is one of the more common causes of acute respiratory distress in cattle, particularly adult beef cattle, and is characterized by sudden onset, minimal coughing, and a course that ends fatally or improves dramatically within a few days. (merckvetmanual.com)
  • The goal of treatment for people with pulmonary emphysema is to live more comfortably with the disease, control symptoms, and prevent the disease from getting worse, with minimal side effects. (ahealthyme.com)
  • The prevalence of pulmonary interstitial emphysema widely varies with the population studied. (wikipedia.org)
  • The term 'prevalence' of Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification usually refers to the estimated population of people who are managing Berry aneurysm, cirrhosis, pulmonary emphysema, and cerebral calcification at any given time. (rightdiagnosis.com)