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Search Results: 1 - 10 of 5972 matches for " Pulmonary Fibrosis "
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Use of a mathematical model of autopoietic maintenance of pulmonary alveolar integrity to show a possible relationship between epithelial-mesenchymal transition and the genesis of emphysema and fibrosis  [PDF]
Kyongyob Min, Keita Hosoi, Yoshinori Kinoshita, Satoshi Hara, Hiroyuki Degami, Tetsuo Takada, Takahiko Nakamura
Open Journal of Molecular and Integrative Physiology (OJMIP) , 2011, DOI: 10.4236/ojmip.2011.12004
Abstract: Rationale: There is accumulating evidence that a group of stem/progenitor cells (SPCs) maintain alveolar epithelial integrity. Pulmonary emphysema is characterized by the histological finding of the loss of alveolar epithelial integrity along with corresponding bronchiolar fibrosis. Objectives: Based on the concept of autopoiesis (the capacity to produce oneself), we proposed a mathematical model in the maintenance of alveolar epithelial integrity as related to the genesis of pulmonary emphysema and fibrosis. Methods: A tessellation automaton model was used to describe the autopoietic dynamics of the bronchiolo-alveolar epithelial surface. The alveolar septal volume en-closed by the epithelial surface is a distributed system of discrete elements, which move by random walk in the manner of Brownian motion. Assuming that the numbers of components and events in the automaton are large, an approximate theoretical treatment in terms of differential equations is possible, allowing a set of partial differential equations to be produced. Results: 1) Assuming the loss of progenitor cells through the epithelial-mesenchymal transition (EMT), a sharp bifurcation between two qualitatively distinct regions of the phase space (one that is repaired completely, and another that has disappeared entirely) clearly appeared. 2) Thus, from the system of discrete and spatial partial differential equations, we obtained a system of ordinary differential equations in equilibrium conditions that defined a close relationship between the degree of emphysema, the density of alveolar septal fibroblasts, and the mean concentration of SPCs. Conclusions: A mathematical model of the autopoietic maintenance of the alveolar epithelial surface suggested a close relationship between alveolar emphysema and fibrosis and EMT in lungs affected by chronic obstructive pulmonary disease.
Increased survivin expression contributes to apoptosis-resistance in IPF fibroblasts  [PDF]
Thomas H. Sisson, Toby M. Maher, Iyabode O. Ajayi, Jessie E. King, Peter D.R. Higgins, Adam J. Booth, Rommel L. Sagana, Steven K. Huang, Eric S. White, Bethany B. Moore, Jeffrey C. Horowitz
Advances in Bioscience and Biotechnology (ABB) , 2012, DOI: 10.4236/abb.2012.326085
Abstract: Fibroblasts perform critical functions during the normal host response to tissue injury, but the inappropriate accumulation and persistent activation of these cells results in the development of tissue fibrosis. The mechanisms accounting for the aberrant accumulation of fibroblasts during fibrotic repair are poorly understood, although evidence supports a role for fibroblast resistance to apoptosis as a contributing factor. We have shown that TGF-β1 and endothelin-1 (ET-1), soluble mediators implicated in fibrogenesis, promote fibroblast resistance to apoptosis. Moreover, we recently found that ET-1 induced apoptosis resistance in normal lung fibroblasts through the upregulation of survivin, a member of the Inhibitor of Apoptosis (IAP) protein family. In the current study, we sought to determine the role of survivin in the apoptosis resistance of primary fibroblasts isolated from the lungs of patients with Idiopathic Pulmonary Fibrosis (IPF), a fibrotic lung disease of unclear etiology for which there is no definitive therapy. First, we examined survivin expression in lung tissue from patients with IPF and found that there is robust expression in the fibroblasts residing within fibroblastic foci (the “active” lesions in IPF which correlate with mortality). Next, we show that survivin expression is increased in fibroblasts isolated from IPF lung tissue compared to cells from normal lung tissue. Consistent with a role in fibrogenesis, we demonstrate that TGF-β1 increases survivin expression in normal lung fibroblasts. Finally, we show that inhibition of survivin enhances susceptibility of a subset of IPF fibroblasts to apoptosis. Collectively, these findings suggest that increased survivin expression represents one mechanism contributing an apoptosis-resistant phenotype in IPF fibroblasts.
Anterior Mediastinal Fat Changes in Idiopathic Pulmonary Fibrosis: A Preliminary Study  [PDF]
Wafaa Ali Hassan, Eman Abo-Elhamd
Open Journal of Respiratory Diseases (OJRD) , 2014, DOI: 10.4236/ojrd.2014.41003
Abstract:
Background: The mediastinum is composed primarily of fatty tissue that is surrounded by the lungs bilaterally.
There is a lack in the published literature in studying changes in mediastinal fat in idiopathic pulmonary fibrosis
(IPF). The purpose of this study was to determine whether the shape and dimensions of the anterior mediastinal fat in patients with IPF are different from that of a normal control group and to correlate the changes with disease severity. Design and Setting: This prospective case control study was done at the chest department of Assiut University Hospital on IPF patients from May 2010-September 2012. A questionnaire containing questions such as age, sex, clinical findings, high resolution computerized tomography (HRCT) score and pulmonary function tests (PFTs) was filled for patients and normal controls. Results: The IPF retrosternal AP dimension was significantly shorter (p = 0.03) and the transverse dimension was longer (p = 0.001) than that in the normal control group. The convex shape of the anterior mediastinum was predictive of IPF (p = 0.001), whereas concave shape was predictive of normal controls (p = 0.001). The change in anteroposterior diameter (AP) and transverse diameters showed significant correlation with the changes in FVC, DLCO and HRCT score. Conclusions: IPF patients had reduced retrosternal AP and increased transverse dimensions than those of the controls with convex shape of their anterior mediastinal fat. Changes in anterior mediastinal fat dimensions are correlated with lower FVC, DLCO and higher HRCT score. A larger sample size, better multicenteric study is needed to confirm the results of this study.
Telomerase in pulmonary fibrosis. A link to alveolar cell apoptosis and differentiation
Argyris Tzouvelekis,Andreas Karameris,Evangelos Tsiambas,Anastasios Koutsopoulos
Pneumon , 2010,
Abstract: SUMMARY. Introduction: Telomerase is crucial for extended life span and differentiation and is linked to immortality. Therefore, its role may be crucial in the pathogenesis of pulmonary fibrosis. Our objective was to implicate telomerase in the pathogenesis of idiopathic fibrotic lung disease. Patients and Methods: Assessment of telomerase activity and expression was carried out using TRAP detection kit and qRT-PCR. Experimental procedure was enhanced by a series of immunostainings and fluorescence in situ hybridization analysis in tissue microarrays constructed with tissue samples from patients with idiopathic pulmonary fibrosis (IPF) and cryptogenic organizing pneumonia (COP). Results: We demonstrated significant downregulation of telomerase expression and activity in patients with fibrotic lung disease compared to controls. Immunolocalization studies coupled by FISH analysis revealed the presence of two subpopulations of type II AECs based on their telomerase expression levels: telomerase positive type II AECs, mainly overlying areas of active fibrosis and telomerase negative type II AECs, mainly localized in areas of established fibrosis. Conclusions: Downregulation of telomerase expression and activity in IPF may indicate a causal relationship between low telomerase expression and disease pathogenesis. The duality phenomenon in telomerase expression suggests that telomerase may regulate the fate of AECs towards either an apoptotic or a mesenchymal phenotype contributing directly to fibrosis. Pneumon 2010, 23(3):207-239.
NEUMOTóRAX ESPONTáNEO ASOCIADO A FIBROSIS PULMONAR EN UN PACIENTE CON NEUROFIBROMATOSIS TIPO 2
Alcalá-Cerra1,Gabriel; Moscote-Salazar,Luis Rafael; Lozano-Tagua,Carlos Fernando; Sabogal-Barrios,Rubén;
Revista Facultad de Medicina de la Universidad Nacional de Colombia , 2010,
Abstract: pulmonary involvement in patients with neurofibromatosis has been repetitively reported as a very rare complication in type 1 variety. it is characterized by pulmonary intersticial disease, pulmonary fibrosis and bullaes, the last with high risk of rupture. we described a case of spontaneous pneumothorax in a patient with type 2 neurofibromatosis, as consequence of pulmonary fibrotic changes. to our knowledge this association had not been reported.
Influência do biofármaco DNA-hsp65 na les?o pulmonar induzida por bleomicina
Padua, Adriana Ignacio de;Silva, Célio Lopes;Ramos, Simone Gusm?o;Faccioli, Lúcia Helena;Martinez, José Ant?nio Baddini;
Jornal Brasileiro de Pneumologia , 2008, DOI: 10.1590/S1806-37132008001100002
Abstract: objective: to evaluate the effects of immunization with a dna-hsp65 vaccine in an experimental model of pulmonary fibrosis. methods: a total of 120 male c57bl/6 mice were distributed into four groups: ss, injected with saline (placebo) and then receiving intratracheal (it) instillation of saline; sb, injected with saline (placebo) and then receiving it instillation of bleomycin; pb, treated with plasmid only, without bacterial genome, and then receiving it instillation of bleomycin; and bb, treated with the vaccine and then receiving it instillation of bleomycin. bleomycin was instilled 15 days after the last immunization, and the animals were killed six weeks thereafter. the left and right lungs were removed, the former for morphological analysis and the latter for hydroxyproline measurements. results: the proportion of deaths within the first 48 h after the it instillation (deaths attributed to the surgical procedure) was higher in the sb group than in the ss group (57.7% vs. 11.1%). the mean area of pulmonary interstitial septa was greater in the sb and pb groups (53.1 ± 8.6% and 53.6±9.3%, respectively) than in the ss and bb groups (32.9 ± 2.7% and 34.3 ± 6.1%, respectively). the mean area of interstitial septa stained by picrosirius was greater in the sb, pb and bb groups than in the ss group (8.2 ± 4.9%, 7.2 ± 4.2% and 6.6 ± 4.1%, respectively, vs. 2.0±1.4%). the total hydroxyproline content in the lung was significantly lower in the ss group (104.9 ± 20.9 pg/lung) than in the other groups (sb: 160.4 ± 47.8 pg/lung; pb: 170.0 ± 72.0 pg/lung; and bb: 162.5 ± 39.7 pg/lung). conclusions: immunization with the dna-hsp65 vaccine reduced the deposition of noncollagen matrix in a model of bleomycin-induced lung lesion.
Mitomicina C endovesical y fibrosis pulmonar
Janeiro Pais,José Manuel; Pastor Casas Agudo,Vicente; López Garcia,Daniel; González Dacal,Juan; Lamas Meilán,Cipriano; González Martín,Marcelino;
Actas Urológicas Espa?olas , 2009, DOI: 10.4321/S0210-48062009000700017
Abstract: the mitomycin c is a chemotherapeutic agent used in several types of carcinomas. in the superficial vesical carcinoma comes using since more than a quarter century in the form of endovesical instillations. it is a drug relatively safe, although there have been described adverse effects related to its systemic administration as myelosuppression, anaemia, kidney toxicity and less frequently pulmonary fibrosis. in this article we presented the case of a patient who suffers a respiratory illness compatible with interstitial lung disease that develops a respiratory severe insufficiency finishing with the death, after the administration of endovesical mitomycin c, being this adverse effect exceptional for this route of administration.
Mortality caused by idiopathic pulmonary fibrosis in the State of Rio Grande do Sul (Brazil)
Fortuna, Fabrício Piccoli;Perin, Christiano;Cunha, Leticia;Moreira, José da Silva;Rubin, Adalberto Sperb;
Jornal de Pneumologia , 2003, DOI: 10.1590/S0102-35862003000300002
Abstract: epidemiologic data on idiopathic pulmonary fibrosis are relatively scarce, and its real incidence and prevalence are unknown. recent studies suggest that mortality due to idiopathic pulmonary fibrosis is rising in developed countries. objective: to describe mortality caused by idiopathic pulmonary fibrosis in the state of rio grande do sul (rs), brazil, from 1970 to 2000, analyzing its trend and comparing it with that from other countries. method: prevalence study, using data from the brazilian institute of geography and statistics (ibge), analyzing death certificates in which idiopathic pulmonary fibrosis was stated as the ultimate cause of death. results: annual mortality rate adjusted to the population was 0.22/100,000 people in the 1970?s, 0.3/100,000 people in the 1980?s, and 0.48/100,000 people in the 1990?s. total mortality raised 36% from 1970 to 1980, and 73% from 1980 to 1990. the mortality rate adjusted to the population raised 36% and 60% during the same periods. the rise in both total and adjusted mortality from ipf was statistically significant (p < 0.05). the mean mortality rate per 100,000 inhabitants between 1996 and 1998, however, was 0.683, corresponding to a 70% increase when compared to the previous three-year period, which was 0.4 (p = 0.0002), probably reflecting coding practices. conclusion: there was a significant increase in ipf mortality in rs from 1970 to 2000, partly due to changes in coding practices. this increase is in conformity with observations in other countries, although mortality rates in rs are considerably lower.
Achados histológicos e sobrevida na fibrose pulmonar idiopática
Coletta, Ester Nei Aparecida Martins;Pereira, Carlos Alberto de Castro;Ferreira, Rimarcs Gomes;Rubin, Adalberto Sperb;Villela, Lucimara Sonja;Malheiros, Tatiana;Stávale, Jo?o Norberto;
Jornal de Pneumologia , 2003, DOI: 10.1590/S0102-35862003000600009
Abstract: background: idiopathic pulmonary fibrosis was recently redefined as usual interstitial pneumonia of unknown etiology. consequently, the prognostic value of histological findings needs to be reassessed. objective: to correlate clinical, functional and histological findings with survival in patients with idiopathic pulmonary fibrosis. method: patients (n = 51; mean age: 66 ± 8 years; gender: 21 females/30 males) were evaluated. of the 51, 26 were smokers or ex-smokers. duration of symptoms, forced vital capacity and smoking habits were recorded. all patients presented usual interstitial pneumonia verified through histology. degree of honeycombing, established fibrosis, desquamation, cellularity, myointimal thickening of blood vessels and number of fibroblastic foci were graded according to the semiquantitative method. results: median duration of symptoms was 12 months and initial forced vital capacity was 72 ± 21%. cox multivariate analysis revealed that survival correlated inversely and significantly (p < 0.05) with duration of symptoms and fibroblastic foci score, as well as with myointimal thickening of blood vessels. limited numbers of fibroblastic foci, as well as myointimal thickening involving less than 50% of blood vessels, were predictive of greater survival. no correlation with survival was found for gender, age, forced vital capacity, inflammation or degree of cellularity. conclusion: semiquantitative analysis of lung biopsies yields relevant prognostic information regarding patients with usual interstitial pneumonia.
Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos
Fernández Casares,Marcelo; González,Alejandra; Caputo,Flavia; Bottinelli,Yanina; Nastavi,Patricia; Zamboni,Marcelo;
Medicina (Buenos Aires) , 2012,
Abstract: the most frequently observed pulmonary complications of vasculitis (aav) with anti-neutrophil cytoplasmic positive antibodies (anca) are alveolar hemorrhage, granulomas and airway stenosis. in recent years, some reports have been published that show the association of vasculitis with pulmonary fibrosis (pf), suggesting that it may be another complication of aav. we report and describe here two cases with such association, and their clinical, tomographic and immunological characteristics. given that in the association between pf and aav, as reported in the last years, pf could be the first manifestation of aav, the search for anca in patients with pf may be necessary, as a cause of it and for the possible subsequent development of vasculitis.
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