Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:6.2.1.7 (BAL)
1,977 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is well known that silica exposure leads in an experimental model to the development of an acute fibrotic process. In human beings two main observations have already been done: (1) silica exposure is frequently associated with the development of connective tissue disease (CTD), especially progressive systemic sclerosis; (2) 10 to 20% patients with CTD developed pulmonary fibrosis. In this context we report 26 cases of coal miners who presented with clinical, radiological, biological and functional characteristics mimicking idiopathic pulmonary fibrosis (IPF), with or without associated coal worker's pneumoconiosis (CWP). All were men; mean age was 68 +/- 9.2 years. Twenty-three were smokers. Duration of exposure was 28.8 +/- 9.1 years. All the patients had dyspnea (stage III, IV in the NHYA classification) and diffuse crackles. Eleven out of 26 had finger clubbing. Computed tomography showed honeycombing (23 cases), and/or ground glass opacities (6 cases) with bronchiectasis (3 cases) predominant in the lower lobes; 19 had radiological signs of CWP, micronodules (n = 16) and nodules (n = 3) predominant in the upper lobes. BAL exhibited an increased % of neutrophils (11.9 +/- 16.1%). Lung function demonstrated a restrictive pattern (TLC = 73 +/- 15.6% and VC = 80 +/- 18% of predicted values) associated with a decreased DLCO (51.8 +/- 23.6% of predicted values) and hypoxemia (at rest = 66.5 +/- 11.2 mmHg, upon effort = 56 +/- 12 mmHg). Lung biopsies were performed in four cases and demonstrated interstitial fibrosis of intraalveolar septum with an accumulation of immune and inflammatory cells similar to the one described in IPF. The association between IPF and silica exposure with or without associated CWP points out the problem of legal recognition of idiopathic-like pulmonary fibrosis as a complication of the occupational exposure of coal workers.
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PMID:["Primary" diffuse interstitial fibrosis in coal miners: a new entity? Study Group on Interstitial Pathology of the Society of Thoracic Pathology of the North]. 941 11

It has been shown that interleukin 8 (IL-8) is increased in bronchoalveolar lavage fluid (BALF) of patients with idiopathic pulmonary fibrosis (IPF) and there is increasing evidence that it is involved in the pathogenesis of this disease. To date, no data are available as to whether IL-8 is elevated in sera of IPF patients. We obtained sera from 42 patients with IPF and 20 healthy controls at time of BAL. From 20 of 42 patients with IPF and 12 of 20 controls BALF was available, enabling us to measure IL-8 in serum and BALF of the same time point. IL-8 was significantly elevated in serum (54.7 +/- 7.5 pg/ml, p < 0.0001) and BALF (715.7 +/- 112.4 pg/ml, p < 0.0001) of patients with IPF compared with controls (IL-8 in serum, 5.2 +/- 0.8 pg/ml; IL-8 in BALF, 67.3 +/- 9.7 pg/ml). We observed a significant positive correlation between IL-8 levels in BALF and percentage of BALF neutrophils (p < 0.001) and between serum IL-8 and BALF IL-8 levels (p < 0.005) in patients with IPF. Consequently, the serum IL-8 level correlated positively with the percentage of BAL neutrophils (p < 0.01), indicating that it may reflect the degree of neutrophilic alveolitis in IPF. Furthermore, the serum IL-8 level showed a negative correlation with important indicators of impairment of lung function (DL(CO), TLC, VC) and PaO2. In conclusion, we were able to demonstrate that the degree of neutrophilic alveolitis in IPF is reflected by increased serum levels of IL-8 and we suggest that the serological assessment of IL-8 may provide a useful parameter for clinicians in monitoring patients with IPF.
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PMID:Serum level of interleukin 8 is elevated in idiopathic pulmonary fibrosis and indicates disease activity. 951 88

Neutrophil alveolitis is a hallmark of cryptogenic fibrosing alveolitis (CFA), known for its poor prognosis. Corticosteroids, as the remedy of choice, are ineffective in a majority of patients. More and more evidence indicates that pentoxifylline (POF) could be an effective therapeutic alternative. Furthermore, colchicine has been proposed for therapy of CFA for many years now. We conducted an experimental study comparing the efficacy of these drugs in preventing neutrophil alveolitis in vivo. Alveolitis was induced in male rats by intratracheal instillation of bleomycin. Treatment consisted of daily injections of POF i.p., colchicine i.p., or prednisolone i.m. After 8 days the animals were sacrificed and body weights, cell differentials in BAL, amount of proliferating interstitial cells as determined by KI-67 staining of lung tissue, and collagen concentrations in lungs were determined. Bleomycin instillation was followed by a significant weight loss in the animals, a neutrophil alveolitis in BAL and an increased amount of proliferating cells in lung interstitium. POF significantly inhibited any of the parameters named, whereas prednisolone and colchicine had little effect. Data cannot be applied directly in human disease. There are however many similarities between CFA and bleomycin-induced lung injury and alveolitis. We conclude that POF is an effective inhibitor of neutrophil alveolitis, whereas neither colchicine nor prednisolone exerted significant influence in our model. We suggest POF effects should be further investigated regarding anti-inflammatory and anti-fibrotic properties.
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PMID:Comparative study on effects of pentoxifylline, prednisolone and colchicine in experimental alveolitis. 987 83

In interstitial pulmonary diseases investigations are conducted to find markers of the activity of the interstitial processes so that noninvasive monitoring of the disease might be possible. In 188 patients divided into 9 groups: 42 with active sarcoidosis, 24 with inactive sarcoidosis, 16 with active sarcoidosis treated with steroids and 22 with inactive sarcoidosis after corticotherapy, 17 with avian fanciers' lung exposed to the antigen, 16 with avian fanciers' lung after a year interval in exposure to the antigen, 20 with advanced and 13 with moderate idiopathic pulmonary fibrosis, and 18 healthy persons the BAL was performed. In the BALF concentrations of protein and phospholipids were assayed by colorimetric method. The results indicate usefulness of the studied biochemical parameters in BALF in evaluation of the activity of interstitial pulmonary diseases. Significant differences were found between the results in the active group of patients compared to the control group and to the inactive forms of interstitial pulmonary diseases. Particularly valuable is phospholipids to protein concentration ratio in BALF.
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PMID:[Extracellular components of bronchoalveolar lavage fluid (BALF) as a marker of the activity of interstitial pulmonary diseases. III: Phospholipids to protein concentration ratio]. 1037 48

As a result of several studies with different animal models there is evidence that the concentration of AP in BAL is produced in the pneumocyte II and that an increase of AP in the BAL is a marker of tissue damage. By measuring AP in the BAL of patients with interstitial lung diseases we investigated its potential role as a diagnostic tool. To detect plasma leakage we also measured the concentration of albumin in the BAL. We studied 85 patients with following diagnoses: Sarcoidosis in 34 patients (Stage 1/2/3 14/7/13), idiopathic pulmonary fibrosis (IPF) in 14, bronchiolitis obliterans with organizing pneumonia (BOOP) in 7, hypersensitivity pneumonitis (HP) in 6. The control group consisted in 24 patients (13 nonsmokers, 11 smokers). In IPF and BOOP we observed significantly higher concentrations of AP than in controls and sarcoidosis (42.4 +/- 36.6 and 35.6 +/- 16 vs. 15.8 +/- 12.7 and 15.0 +/- 9.8 U/l, p < 0.05, ANOVA). Compared with controls in sarcoidosis higher concentrations of albumin (5.7 +/- 4 vs. 13.2 +/- 10 mg/dl, p < 0.05, ANOVA) and a lower AP/albumin-ratio (3.6 +/- 3.0 vs. 1.3 +/- 0.9 U/10 mg, p < 0.05, ANOVA) were seen. This result is an argument against plasma leakage as the source of AP in BAL. There were no differences in AP and albumin between the different stages of sarcoidosis and between smokers and nonsmokers in the control group. We conclude, that there are different concentrations of AP and albumin in BAL in different interstitial lung diseases. Compared with controls we observed higher concentrations of AP and an AP/albumin-ratio in the normal range in IPF, a normal concentration of AP and a lowered AP/albumin-ratio in sarcoidosis.
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PMID:[Concentration of alkaline phosphatase (AP) and AP/albumin ratio in bronchoalveolar lavage (BAL) for the diagnosis of interstitial lung diseases]. 1107 22

A 73-year-old woman suffering from dyspnea on effort and chronic cough was admitted to our hospital. Chest computed tomography disclosed ground-glass opacities, irregular linear opacities and honeycombing distributed predominantly in the subpleural area. The serum levels of SP-D and KL-6 rose to 889 ng/ml, 1,755 U/ml, respectively. These findings indicated idiopathic pulmonary fibrosis. However, the number of lymphocytes and the CD4/CD8 ratio in the BAL fluid were elevated. Transbronchial lung biopsy specimens demonstrated alveolitis with granuloma formation. The evidence that she had lived in a house with a heavy fungal growth and that tests of precipitation in response to Penicillium corylophilum were positive confirmations of a diagnosis of chronic hypersensitivity pneumonia.
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PMID:[A case of chronic hypersensitivity pneumonia with elevation of serum SP-D and KL-6]. 1192 22

Fibrosing alveolitis associated with systemic sclerosis (FASSc) has a better prognosis than idiopathic pulmonary fibrosis. In view of recent reports that idiopathic nonspecific interstitial pneumonia (NSIP) has a better prognosis than idiopathic usual interstitial pneumonia (UIP), we classified histologic appearances of surgical lung biopsies performed in 80 patients with FASSc. NSIP (n = 62, 77.5%), subcategorized as cellular NSIP (n = 15) and fibrotic NSIP (n = 47) was much more prevalent than UIP (n = 6), end-stage lung disease (ESL, n = 6), or other patterns (n = 6). There were 25 deaths (NSIP 16/62, 26%; UIP/ESL 6/12, 50%). Five-year survival differed little between NSIP (91%) and UIP/ESL (82%); mortality was associated with lower initial carbon monoxide diffusing capacity (DL(CO)) and FVC levels (p = 0.004 and p = 0.007, respectively). Survival and serial FVC and DL(CO) trends did not differ between cellular and fibrotic NSIP. Increased mortality in NSIP was associated with lower initial DL(CO) levels (p = 0.04), higher BAL eosinophil levels (p = 0.03), and deterioration in DL(CO) levels during the next 3 years (p < 0.005). We conclude that NSIP is the histopathologic pattern in most patients with FASSc. However, outcome is linked more strongly to disease severity at presentation and serial DL(CO) trends than to histopathologic findings.
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PMID:Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. 1207 54

Idiopathic pulmonary fibrosis (IPF) is an inflammatory lung disease characterized by the accumulation of inflammatory cells and deposition of collagen, resulting in lung remodelling. High numbers of T cells are present in bronchoalveolar lavage fluid (BALF) of IPF patients, although the characteristics of these cells are yet to be determined. To elucidate the pathogenic mechanisms of IPF, we analysed the T cell receptor (TCR) of BALF lymphocytes in three patients with IPF and three healthy subjects as control. TCR repertoire of BALF lymphocytes and T cell clonality were examined by family PCR and Southern blot analysis, and single-strand conformation polymorphism (SSCP), respectively. We observed that the TCR repertoire in the lung was heterogeneous, both in the control subjects and three patients with IPF. SSCP analysis demonstrated an increase in the number of accumulated T cell clones in BALF of two of the three patients, but not in the healthy subject. Furthermore, junctional sequence analysis showed the presence of conserved amino acid motifs (ETGRSG, LAxG, QGQ, GxQP, GRxG, VAR, PGT, GTI, GGT, TGR, LxLxQ, SGQ) in the TCR-CDR 3 region of BAL lymphocytes in patients with IPF, whereas only two amino acid motifs (VTTG, GGE) were found in the control. Our findings suggest that T cells in BALF of patients with IPF expand oligoclonally in the lung, suggesting antigen stimulation of these cells.
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PMID:Conserved CDR 3 region of T cell receptor BV gene in lymphocytes from bronchoalveolar lavage fluid of patients with idiopathic pulmonary fibrosis. 1210 34

The objective of this study was to evaluate the mechanisms of colchicine action in pulmonary fibrosis. The study included 10 patients with pulmonary fibrosis (idiopathic pulmonary fibrosis 5, asbestosis 4, and scleroderma 1) who had been admitted to Bellevue Hospital Center, a tertiary care public hospital in New York City. We administered colchicine 0.6 mg orally for 12 weeks to patients with pulmonary fibrosis. Symptoms, high resolution CT scans, pulmonary function tests, and bronchoalveolar lavage parameters were compared prior to and after treatment. Results showed declines in dyspnea index, selective improvement in several CT scans, but no statistically significant change in BAL cells, cytokines, fibronectin, or hydroxyproline. However, there was a decline in hydroxyproline in the BAL fluid in 8/10 patients. We concluded that colchicine has a mild antifibrotic effect which may be in inhibiting collagen formation since there was no effect on the inflammation that accompanies fibrosis.
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PMID:Mechanisms of colchicine effect in the treatment of asbestosis and idiopathic pulmonary fibrosis. 1217 1

The pulmonary granulomatous diseases may be staged using clinical examination, pulmonary function tests, ⁶⁷Ga scans, chest X-rays, BAL and serum ACE levels; furthermore, these disorders are clearly associated to changes in lymphocyte subpopulations, CD4+/CD8+ ratio and surface receptors; in particular, T cell activation characterizes early alveolitis phase, while activated macrophages and related cytokines prevail in granulomata and fibrosis development. In this study, we dosed the serum and blood concentrations of IL-6 (a well-known pro-inflammatory cytokine), sIL-2R (marker of T-cell activation), TNF-alpha and IFN-gamma (associated with the granuloma development), in patients affected by active or inactive sarcoidosis, primary tuberculosis, idiopathic pulmonary fibrosis and healthy control subjects, using the ELISA method. Cytokines assay showed significant changes only in subjects with primary tuberculosis and active sarcoidosis; infact, primary tuberculosis was characterized by high values of IL-6 and IFN-gamma both in peripheral blood and in BAL, with high values of sIL-2R in BAL; patients with active sarcoidosis showed high levels of IFN-gamma and TNF-alpha both in BAL and in peripheral blood, associated to an increase of serum sIL-2R levels. Our data confirm that the compared assay of these cytokines in peripheral blood and BAL specimens, may be useful to diagnose and to assess the disease activity in pulmonary granulomatous diseases; in particular, the levels of sIL-2R are a marker of the alveolitis phase, while TNF-alpha and IL-6 levels discriminate patients with sarcoidosis or tuberculosis granulomata, respectively.
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PMID:Cytokines assay in peripheral blood and bronchoalveolar lavage in the diagnosis and staging of pulmonary granulomatous diseases. 1265 92


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