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)

16 patients (nine men, seven women; mean age 45 [23-62] years) with end-stage pulmonary disease underwent lung transplantation, 14 unilateral, three bilateral (one bilateral retransplantation). The diagnoses were: emphysema (n = 5), idiopathic pulmonary fibrosis (n = 5), emphysema in alpha 1-antitrypsin deficiency (n = 1), mucoviscidosis (n = 1), drug-induced fibrosis (n = 1), chronic lung rejection after heart-lung transplantation (n = 1), secondary pulmonary hypertension with persistent ductus arteriosus (n = 1), sarcoidosis (n = 1) and chronic transplant failure after unilateral transplantation (n = 1). Eleven patients survived long-term (mean follow-up period 247 [8-585] days). Ten of these patients have been independent of additional oxygen after an average of 8 months postoperatively, eight are physically active according to their age. Three patients died of reperfusion damage intraoperatively or in the early postoperative period. The two other patients died from septicaemia 5 1/2 and 7 months postoperatively. It is concluded that lung transplantation in end-stage pulmonary disease provides the only therapeutic means with any expectation of physical rehabilitation.
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PMID:[Isolated lung transplantation. Analysis of 17 consecutive operations. Munich Lung Transplant Group]. 815 68

Lung- and heart-lung transplantation has become a serious therapeutic option for terminally ill patients with chronic pulmonary failure. Due to the still rather high risk of acute rejection, severe pulmonary or systemic infection, and the relatively demanding postoperative care, this treatment can only be considered for patients where inspite exhausted conservative options for therapy quality of life and prognosis are not acceptable. Not only due to lack of donors single-lung transplantation has developed to be the favorite treatment for patients with interstitial lung disease (idiopathic, sarcoidosis, asbestosis etc.) and even for pulmonary hypertension unless irreversible damage to the myocardium is present. Double-lung transplantation is the treatment of choice for patients with emphysema and cystic fibrosis. Heart-lung transplantation is mainly limited to patients with a high degree of right ventricular or valve dysfunction as in Eisenmenger-Syndrome. Midterm clinical results have evolved to be very satisfactory while on the longterm basis chronic transplant dysfunction not responding to any kind of therapy causes a lot of concern. One- and two-year survival rates are 85 and 82% resp. in our program and thus are in the same range as those after heart transplantation.
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PMID:[Lung and heart-lung transplantation: indications, complications and prognosis]. 818 61

Between February 1988 and January 1992, 61 patients have undergone bilateral lung transplantations (42 heart-lung and 19 double-lung) in Bordeaux. The underlying diseases were primary or secondary hypertension (20), emphysema (22), or other diseases including cystic fibrosis, pulmonary fibrosis, silicosis, and sarcoidosis (19). Actuarial survival for double-lung and heart-lung transplant recipients was 66% and 72% at 1 year and 57% and 53% at 3 years, respectively. Forty-two patients were still alive 6 months after operation, and we studied their pulmonary function at the short and long term. All parameters except arterial carbon dioxide tension had improved dramatically at 6 months (p < 0.0001). Vital capacity, forced expiratory volume in 1 second, and forced expiratory flow rate between 25% and 75% of vital capacity were at 79% +/- 3%, 92% +/- 5%, and 105% +/- 8% of the predicted values, respectively. Arterial oxygen tension was 88 +/- 3 mm Hg. Nine months after operation, a slight decrease in forced expiratory volume in 1 second and forced expiratory flow rate between 25% and 75% of vital capacity appeared but values remained more than 75% predicted. This was related to the occurrence of obliterative bronchiolitis in 6 patients (14%). At 9 months, flow rates and oxygen tension of these 6 patients were highly different from those of patients free of obliterative bronchiolitis (p < 0.0002 for flow rates and p < 0.01 for oxygen tension). Only 1 patient required retransplantation. The others are living an almost normal life. Our results are discussed in view of the published reports on single-lung transplantation. Short-term results of bilateral lung transplantation are thus excellent and maintained on a long-term basis. Therefore, in our opinion, bilateral lung transplantation is the therapy of choice for pulmonary hypertension and emphysema.
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PMID:Long-term functional results after bilateral lung transplantation. Bordeaux Lung and Heart-Lung Transplant Group. 832 78

High-resolution computed tomography (CT) of the lung provides detailed visualization of the lung parenchyma. The technique involves the use of thin-section axial tomography. High-resolution CT can facilitate the diagnosis of certain pulmonary processes. It is useful in differentiating similar patterns of abnormalities seen on chest radiographs, such as those seen in lymphangitic carcinomatosis and sarcoidosis, and in delineating the extent of co-morbid lung diseases, such as emphysema and asbestosis. In some chronic processes such as idiopathic pulmonary fibrosis, high-resolution CT can be used for biopsy of areas of active inflammation, thus increasing the diagnostic yield. As clinicians and radiologists become more familiar with high-resolution CT, it is likely that its role in the diagnosis and assessment of lung disease will increase.
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PMID:High-resolution CT of the lungs. 836 98

Seven cases of single lung transplantation are reported. The recipients were all below 60 years of age and severely disabled with end-stage lung disease. Transplantation was performed according to ABO blood group compatibility and negative lymphocytotoxic cross-match between donor and recipient irrespective of HLA mismatch. Recipients' diagnoses were sarcoidosis (3), alfa-1 antitrypsin deficiency (3), and idiopathic emphysema (1). Mean recipient age was 48 +/- 2.4 years (range 45-52). Donor age was 29.7 +/- 5.6 years (range 16-49). The immunosuppressive regimen included cyclosporin A, azathioprine, steroids and rabbit antithymocyte globulin. Excellent graft function was achieved. Six patients survived the postoperative period and are alive 4-18 months posttransplant. One patient died after the operation due to pneumonia with respiratory distress syndrome. Graft function was also monitored by transbronchial biopsy, and 57 biopsy procedures were performed without fatal complications. Acute cellular rejection was seen in 16 biopsy specimens from 5 recipients (grade 1 and 2 rejection in 14, grade 3 rejection in 2). Neither severe rejection with septal necrosis (grade 4) nor obliterative bronchiolitis was seen. The rejection rate was 0.03 episodes per patient/month. In contrast to other reports, episodes of cellular rejection occurred throughout the observation period, and were not mainly limited to the first 4 months posttransplant. Graft vascular occlusive disease or chronic vascular rejection was found in 6 biopsy specimens from one recipient. Five patients experienced 7 episodes of cytomegalovirus infection. The cytomegalovirus infection rate was 0.01 episodes per patient/month. The incidence of infection was significantly lower compared to previous studies of rejection in other lung graft combinations. Both infections and rejection episodes may contribute to the development of obliterative bronchiolitis. Almost one third of the specimens (30%) showed lymphocytic bronchitis without perivascular inflammation. The absence of perivascular infiltrates and exclusion of infectious agents leaves in question the aetiology of this inflammation. The lymphocytic bronchitis could be ischaemic, related to aspiration, or represent recurrent sarcoidosis, or, in fact, express bronchial rejection. All biopsy specimens regarded as rejection with cellular infiltrates in the lung parenchyma also showed a lymphocytic bronchitis. The impact of HLA mismatch on cellular and vascular rejection is unclear. Transbronchial biopsy is a reasonably safe and reliable method in the diagnosis of rejection and infection in single lung transplantation.
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PMID:Single lung transplantation. Morphological surveillance by transbronchial biopsy. 839 61

A 36-year-old woman presented with respiratory insufficiency due to cystic sarcoidosis. She had been previously treated with multiple courses of prednisone without improvement. Enlarging blebs involving both lower lobes impaired the function of the relatively spared upper lobes. Bilateral lower lobectomies were performed in one step via median sternotomy without complications, with prompt subjective and objective improvement of her respiratory status. One-stage bilateral upper-lobe bullectomy for bullous emphysema has been previously reported, but to our knowledge this is the first performance of one-stage bilateral lower-lobe bullectomies for cystic sarcoidosis. The immediate benefits were evident; long-term results will depend on the course of the underlying disease.
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PMID:Bullectomies for bullous sarcoidosis. 844 49

Lung volumes and breathing patterns used during speech differ from those of quiet respiration and may be expected to vary with different types of lung disease. To test this possibility, 41 patients with asthma, emphysema, or sarcoidosis and 16 healthy subjects completed a speech protocol. Volumes, times, and flow rates were recorded during conversation and during a counting task. A total of 16 measured variables were derived for each breath and analyzed statistically. Alterations in speech breathing were disease and task specific. Discriminant function analysis applied to data from either speech task could correctly classify subjects with more than 50% accuracy, showing that different patterns were significantly disease specific. Compared with healthy subjects during conversation, all patients averaged a more rapid respiratory rate and increased the proportion of time spent on inspiration (Ti/Ttot). During counting, patient groups showed a variety of patterns, most commonly subordinating metabolic need to communication drive and sounding more breathless to observers. Regression analysis was used to determine how strongly changes in measured speech variables related to degree of physiologic impairment. The effect of severity of disease on speech production is distinguishable from the effect of the diagnostic category.
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PMID:Speech breathing in patients with lung disease. 848 31

High-resolution CT (HRCT) scans were performed on 156 patients, using a bone-reconstruction algorithm, 1.5 mm sections at 4 cm intervals from apex to base of the lungs and a 512 x 512 matrix. The patients either appeared to have a pathologic condition on chest film, or they presented positive clinical symptoms--i.e., cough, dyspnea, fever--and questionable/negative chest films. Since HRCT is capable of showing the secondary lobule, we employed it to study both its anatomy and the alterations that can modify its normal morphology--i.e., thickening of interlobular septa, reticular pattern, nodular pattern, high-density areas, sub-pleural lines, honeycomb pattern. HRCT findings in secondary lobules, airways, and pleura were examined. They were: lymphangitic spread of carcinoma, pulmonary fibrosis, sarcoidosis, pneumoconiosis, interstitial edema, inflammatory disorders, bronchiectasis, emphysema, and bullae. Even though some limitations still exist due to the non-specificity of HRCT findings, the latter is the best method currently available to recognize and locate interstitial conditions and, sometimes, to make a diagnosis--e.g., of lymphangitic spread of carcinoma, interstitial edema, fibrosis, emphysema, bronchiectasis. Moreover, HRCT can accurately locate pathologic areas for lung biopsy and can be used instead of chest radiographs in the follow-up.
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PMID:[High-resolution x-ray computed tomography in the study of the pulmonary parenchyma. Personal experience]. 850 18

We describe a rare case of bullous emphysema occurring in a young male with sarcoidosis. The patient had progressive pulmonary symptoms over 14 years. Chest radiology showed bilateral bullae predominantly of both upper and the left lower lobes and mild bronchiectasis. Pulmonary function tests revealed mild restrictive disease and a severe reduction in diffusion capacity. Loss of alveolar surface area due to bullous disease contributes to functional and physiological impairment of lung function in sarcoidosis.
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PMID:Bullous emphysema occurring in pulmonary sarcoidosis. 856 98

In patients with emphysema the integrity of the extracellular matrix (connective tissue skeleton) is compromised. In this study we analyzed glycosaminoglycans, which are main constituents of this matrix, in urines from patients with chronic obstructive pulmonary disease (COPD)/emphysema. Glycosaminoglycans (GAGs) were purified by anion exchange chromatography and quantified using the 1,9-dimethylmethylene blue assay. Heparan sulfate (HS) was assayed using three different chemical methods: digestion with heparitinase or with nitrous acid and by use of an adapted 1,9-dimethylmethylene blue assay. A specific epitope on the HS molecule, defined by the monoclonal antibody JM403, was determined using an inhibition enzyme immunoassay. In patients with COPD total urinary glycosaminoglycan and HS content were not altered. The JM403 epitope of HS, however, was greatly decreased in patients (0.6 versus 4.1 units/mg creatinine for control subjects, p < 0.0001). A similar pattern was observed when patients with bronchial carcinoma with and without emphysema were compared (0.4 versus 2.4 units/mg creatinine respectively, p < 0.0005). Patients with sarcoidosis did not show a decreased epitope content. These results indicate a structural change or an altered processing of the HS molecule in patients with emphysema. Taking into consideration the importance of HS for the stability of the alveolar extracellular matrix, this change may be associated with the pathogenesis of emphysema.
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PMID:Altered composition of urinary heparan sulfate in patients with COPD. 888 91


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