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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A study was initiated to determine whether alveolar macrophages from patients with collagen vascular diseases but free of pulmonary symptoms were spontaneously activated and whether they released various mediators related to the pathogenesis of pulmonary fibrosis. Alveolar macrophages obtained by bronchoalveolar lavage from 32 patients with proved collagen vascular disease but no evidence of lung disease were compared with those from 10 patients with collagen vascular disease with interstitial lung disease (CVD-ILD) and from 10 healthy controls. The total number of alveolar macrophages did not differ between patients with collagen vascular disease and controls but were substantially increased in the CVD-ILD group. Alveolar macrophages from 31 of the 32 patients with collagen vascular disease and from all 10 in the CVD-ILD group had at least one criterion of activation. Neutrophil chemotactic activity was detected in supernatants from alveolar macrophage culture in 23 of the 32 patients with collagen vascular disease and from nine of the 10 in the CVD-ILD group; fibronectin secretion by alveolar macrophages was increased in 12 of the 32 patients with collagen vascular disease and in nine of the 10 in the CVD-ILD group. Furthermore, alveolar macrophages from 20 of the 32 patients with collagen vascular disease and four of the 10 CVD-ILD patients spontaneously released increased amounts of superoxide anion. Thus alveolar macrophages were spontaneously activated in a high proportion of patients with collagen vascular disease.
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PMID:Activated alveolar macrophages in subclinical pulmonary inflammation in collagen vascular diseases. 283 61

Idiopathic pulmonary fibrosis (IPF) and pulmonary fibrosis associated with a collagen vascular disorder (PF-CVD) are chronic inflammatory lung disorders which may be characterized in various subgroups of patients by increased numbers of macrophages, neutrophils, lymphocytes, and/or eosinophils. Previous studies have suggested that the cell populations recovered with bronchoalveolar lavage (BAL) may be important in predicting disease progression and response to therapy. We evaluated this hypothesis in 27 patients by determining if the cell populations recovered with BAL differed between patients who improved, remained stable, or worsened in their pulmonary functions (as defined by at least a 15 percent change in forced vital capacity) over a six-month observation period. The findings suggested that BAL eosinophilia may be a marker of progressive lung disease in patients with IPF and PF-CVD.
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PMID:Prognostic role of eosinophils in pulmonary fibrosis. 359 49

We evaluated the pulmonary circulatory effects of emphysema produced by papain in 6 dogs that received 4 weekly treatments of papain administered by aerosolization. A control group of 6 dogs received saline treatments. Hemodynamic measurements were performed during normoxic and hypoxic (10% oxygen) ventilation approximately 3 and 6 months after treatment. Baseline hemodynamic parameters were comparable in both groups and were unchanged at 6 months in the control group. In the papain-treated group at 6 months, there were significant reductions in PaO2 (95 +/- 4 to 79 +/- 3 mm Hg, p less than 0.005) and increases in mean pulmonary artery pressure (12 +/- 1 to 18 +/- 3 mm Hg, p less than 0.05) and pulmonary vascular resistance (2.0 +/- 0.3 to 6.2 +/- 2.1 units, p less than 0.01). Morphometric evaluation demonstrated increased medial thickness in muscular pulmonary arteries less than 50 mu in size. Morphologic evaluation confirmed the presence of panlobular emphysema with papain administration. We conclude that chronic emphysema produced in dogs by aerosol administration of papain results in elevated pulmonary artery pressure, which is characterized pathologically by medial hypertrophy of small pulmonary arteries. This model appears suitable for further study of the pathogenesis of pulmonary vascular disease associated with chronic lung disease.
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PMID:Pulmonary vascular structural and functional changes in papain-induced emphysema in dogs. 363 41

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.
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PMID:Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases. 365 23

Since March 1981, 19 patients have undergone heart-lung transplantation for end-stage pulmonary vascular disease, with 14 long-term survivors. In five of the survivors, obstructive airway disease has developed with the superimposition of a progressive restrictive ventilatory defect in three of them. None of these five patients showed a tendency for spontaneous improvement of flow rates. Biopsy and postmortem material was available in four of the five patients and showed obliterative bronchiolitis (OB) in three. A fourth patient showed clinical and physiologic data consistent with obliterative bronchitis, but histologic material was not available. Obstructive lung disease without restrictive features developed in a fifth patient, but no histologic evidence of OB was found at transbronchial biopsy. In addition to OB, recurrent lung infections were found in all patients, significant pleural fibrosis in two patients, and bronchiectasis in one patient. Despite these long-term sequelae of human heart-lung transplantation, ten of the 14 surviving patients are leading relatively normal lives.
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PMID:Post-transplant obliterative bronchiolitis and other late lung sequelae in human heart-lung transplantation. 643 51

Wasted ventilation fraction (Vd/Vt) normally declines substantially during exercise in persons without lung disease. Failure of Vd/Vt to decrease during exercise has been reported to be one of the earliest abnormalities in patients with dyspnea caused by pulmonary vaso-occlusive disease, suggesting that measurement of Vd/Vt at rest and during exercise are useful in the diagnosis of pulmonary vascular disorders. We studied pulmonary hemodynamic and Vd/Vt responses to exercise in 11 patients in the supine position with suspected pulmonary vascular involvement caused by progressive systemic sclerosis, systemic lupus erythematosus, or recurrent pulmonary emboli, 10 of whom had dyspnea at rest and/or on exertion. In contrast to previous reports of no change or an increase in Vd/Vt during exercise in patients with pulmonary vascular disease, we found Vd/Vt to decrease significantly during exercise in 8 of 9 patients in whom mean pulmonary artery pressures were abnormally elevated at rest and/or during exercise. Our findings suggest that normal responses of Vd/Vt to exercise do not exclude hemodynamically significant pulmonary vaso-occlusive disease.
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PMID:Lack of sensitivity of measurements of Vd/Vt at rest and during exercise in detection of hemodynamically significant pulmonary vascular abnormalities in collagen vascular disease. 723 73

Induction of indoleamine 2,3-dioxygenase (IDO), an enzyme expressed by mononuclear phagocytes and some fibroblast cell lines in response to interferon-gamma, leads to enhanced degradation of tryptophan to kynurenine. Because inflammatory lung diseases are generally associated with activation of pulmonary macrophages, we investigated tryptophan metabolism in patients with interstitial lung disease by measuring circulating levels of tryptophan and kynurenine in peripheral blood and by measuring the IDO activity of bronchoalveolar cells. IDO activities were increased for bronchoalveolar lavage (BAL) cells obtained from patients with interstitial lung disease (115.4 +/- 30.4, n = 37) when compared with BAL cells from normal subjects (15.2 +/- 7.4, n = 14; p < 0.05), and messenger RNA for IDO was present in BAL cells from patients with interstitial disease but was not present in BAL cells from normal volunteer subjects. Patients with inflammatory lung disease also had decreased tryptophan and increased kynurenine concentrations in serum. The ratio of serum tryptophan levels to serum kynurenine levels was significantly depressed for patients with idiopathic pulmonary fibrosis (18.4 +/- 1.7, n = 29; p < 0.0001), patients with fibrosing alveolitis associated with collagen vascular disease (13.1 +/- 1.6, n = 18; p < 0.0001), or patients with sarcoidosis (21.0 +/- 1.1, n = 50; p < 0.0001), as compared with the ratio for normal subjects (31.8 +/- 2.3, n = 18). Patients with fibrotic disease had the highest levels of BAL cell IDO activity, and patients with collagen vascular disease associated fibrosing alveolitis had the most depressed levels of serum tryptophan and the greatest elevations in serum kynurenine. Measurement of tryptophan and kynurenine concentrations in serum may provide a useful measure of disease activity in chronic inflammatory parenchymal lung diseases such as sarcoidosis and idiopathic pulmonary fibrosis.
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PMID:Tryptophan metabolism in chronic inflammatory lung disease. 749 May 12

Between November 1983 and September 1992, The Toronto Lung Transplant Program performed 131 lung transplantations in 122 recipients; 53 single lung transplantations and 78 double lung transplantations. Forty-five patients died, 25 (47%) in the single lung transplantation and 20 (25%) in the double lung transplantation groups. We retrospectively reviewed the hospital charts of all deceased recipients and the postmortem reports of the 35 patients (20 single lung transplantations and 15 double lung transplantations) who had autopsies. Preoperative single lung transplantation diagnoses included pulmonary fibrosis, (n = 17) obstructive disease (n = 6) and vascular disease (n = 2). Preoperative diagnosis of double lung transplantation included pulmonary fibrosis (n = 2), obstructive disease (n = 6), septic lung disease (n = 9), and vascular disease (n = 3). The most common cause of death in single lung transplantation was infection. Five patients died of bronchiolitis obliterans, and five more had bronchiolitis obliterans lesions present at autopsy that were not a direct cause of death. Diagnosis of primary disease was made in 23 of 25 single lung transplantations antemortem and 2 of 25 at autopsy. Autopsy diagnoses were disseminated Aspergillus and cytomegalovirus infection. In double lung transplantations, infection was also the primary cause of death; in three other patients, airway dehiscence preceded infection. Bronchiolitis obliterans was the second most common cause of death and was also present in four patients dying of infection. All double lung transplantation diagnoses were made antemortem. We concluded that infection and then bronchiolitis obliterans are the primary causes of death after lung transplantation. Although infection is a major cause both early and late after transplantation, bronchiolitis obliterans is an important factor in transplantation only late after the operation.
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PMID:Causes of death in lung transplant recipients. 780 15

Solid-organ transplantation has flourished during the last decade, with transplantation of heart and lungs becoming available to patients with end-stage cardiac or pulmonary diseases. The first lung transplant was performed in 1963 on a 58-year-old man with bronchogenic carcinoma. He survived for 18 days. During the next two decades, approximately 40 lung transplant procedures were attempted without success. These early attempts at lung transplantation were unsuccessful because of the development of lung rejection, anastomotic complications, or infection in the transplant recipients. In the early 1980s, human heart-lung transplantation was successfully performed for the treatment of pulmonary vascular disease. After this procedure, single-lung transplantation for the treatment of end-stage interstitial lung disease and obstructive lung disease was developed. More recently, the technique of double-lung transplantation has come into existence. This article reviews various aspects of lung transplantation, including immunosuppression, lung graft preservation, the various surgical techniques and types of lung transplant procedures available, recipient and donor selection criteria, and postoperative care of the transplant recipient. In addition, infectious and noninfectious complications seen in this particular patient population, including acute and chronic rejection, will be discussed.
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PMID:Lung transplantation. 828 76

Worldwide almost no epidemiologic data are available on the prevalence or incidence of interstitial lung diseases (ILD) in the general population. Therefore, a registration programme of ILD-prevalence was organised by the VRGT (Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding), among about 100 Flemish pneumologists since 1990. Most categories of the classification by Crystal et al. (1) were included and the diagnostic criteria (histology, laboratory tests, clinic, radiology) were registered. The present paper presents the results of 1992-1994: twenty pneumologists had forwarded the summary files of 237 patients to the central office in 1992 (n = 68), 1993 (n = 90) and 1994 (n = 79). The diagnoses that were most frequently made were: sarcoidosis in 27%, idiopathic pulmonary fibrosis in 20%, hypersensitivity pneumonitis in 14% (of which 68% by birds) and collagen-vascular disease in 10% (of which 54% in rheumatoid arthritis). Less frequent causes were eosinophilic pneumonia (4%), inhalation of inorganic material (4%, anthracosilicosis being excluded), histiocytosis X (3%), drugs (3%), angiitis and granulomatosis (2%), pulmonary hemosiderosis (1%), lymphocytic infiltrative lung disease (1%) and lymphangioleiomyomatosis (1%). The order of relative frequencies of the different categories of diseases was the same in the 3 registration years. In 9% of the patients the diagnosis was confined to "undefined fibrosis". The diagnosis was confirmed by histology in 63% of the cases. The overall male-female ratio was nearly one, with, however, a male preponderance in hypersensitivity pneumonitis (22/12), UIP(8/3) and "undefined fibrosis" (14/7).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Epidemiology of interstitial lung disease (ILD) in flanders: registration by pneumologists in 1992-1994. Working group on ILD, VRGT. Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding. 853 25


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