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1

Enes, Giovana da Silva Tavares 1982. "Fibrose cística = estreitando laços maternos = Cystic fibrosis : strengthening maternal ties." [s.n.], 2012. http://repositorio.unicamp.br/jspui/handle/REPOSIP/308361.

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Orientador: Antonio Fernando Ribeiro<br>Dissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciências Médicas<br>Made available in DSpace on 2018-08-20T12:30:23Z (GMT). No. of bitstreams: 1 Enes_GiovanadaSilvaTavares_M.pdf: 802222 bytes, checksum: 877dccb29eaf785982eed2f02beb39c6 (MD5) Previous issue date: 2012<br>Resumo: A Fibrose Cística é uma doença autossômica recessiva, sistêmica, hereditária, crônica e progressiva e pode levar à morte. São características da doença as secreções mucosas espessas e viscosas que obstrui os ductos das glândulas exócrinas e contribuem pa
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2

Yi, Hao. "The Effect of Metformin on Inflammatory and Fibrotic Responses in Renal Fibrosis." Thesis, The University of Sydney, 2019. https://hdl.handle.net/2123/21855.

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Chronic kidney disease (CKD) is a worldwide public health problem, with adverse outcomes of kidney failure, cardiovascular disease, and premature death. It is well recognised that renal fibrosis is the unifying pathology in almost all forms of progressive CKD. To date, kidney transplantation and dialysis are the only options for the management of end-stage kidney disease, which results in a significant burden on the health system. Hence innovative strategies are needed to both prevent and treat CKD. It is well recognised that inflammatory pathways play a central role in the progression of CKD
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Correia, Cyntia Arivabeni de Araujo. "Estudo dos genes TNF alfa, ADIPOQ e STATH entre portadores de fibrose cistica." [s.n.], 2009. http://repositorio.unicamp.br/jspui/handle/REPOSIP/308583.

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Orientador: Carmen Silvia Bertuzzo<br>Tese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciencias Medicas<br>Made available in DSpace on 2018-08-13T02:33:23Z (GMT). No. of bitstreams: 1 Correia_CyntiaArivabenideAraujo_D.pdf: 1775265 bytes, checksum: 99ecfece81f00d7833282ae41bae5731 (MD5) Previous issue date: 2009<br>Resumo: A Fibrose Cística (FC) possui uma grande variabilidade de expressão fenotípica, o que significa que crianças com o mesmo genótipo podem diferir quanto à sua apresentação. A proteína defeituosa formada é chamada CFTR (proteína reguladora da conductância iôn
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4

Chadwick, Helen Kay. "Cognitive function in cystic fibrosis and cystic fibrosis related diabetes (CFRD)." Thesis, University of Leeds, 2016. http://etheses.whiterose.ac.uk/16912/.

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Cystic fibrosis (CF) is a complex multisystem disease caused by a gene mutation of a protein called the CF Transmembrane Conductance Regulator (CFTR). Glucose tolerance abnormalities are common in CF and the prevalence of CF related diabetes (CFRD), which shares clinical characteristics with type 1 (T1DM) and type 2 diabetes (T2DM), increases with age. Impaired glucose tolerance (IGT), T1DM and T2DM are associated with cognitive impairment relative to healthy controls. The overall aim of this thesis was to examine cognitive function in CF. Study 1 investigated the impact of CF on cognitive fun
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Karlas, Thomas, Joachim Berger, Nikita Garnov, et al. "Estimating steatosis and fibrosis." Universitätsbibliothek Leipzig, 2015. http://nbn-resolving.de/urn:nbn:de:bsz:15-qucosa-169692.

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6

Katre, Ashwini A. "Ozone and lung fibrosis." Thesis, Birmingham, Ala. : University of Alabama at Birmingham, 2009. https://www.mhsl.uab.edu/dt/2009m/katre.pdf.

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7

Kahre, Tiina. "Cystic fibrosis in Estonia /." Online version, 2004. http://dspace.utlib.ee/dspace/bitstream/10062/577/5/KahrePhD.pdf.

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8

Dwyer, Tiffany Jane. "Exercise in cystic fibrosis." Thesis, The University of Sydney, 2010. http://hdl.handle.net/2123/6349.

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Exercise and physical activity have many benefits for adults with cystic fibrosis (CF), including the potential to aid mucus clearance, improve lung function, exercise capacity and quality of life. Despite the recommendations from consensus documents for CF adults to engage in regular physical activity, exercise participation amongst this population is often very low. No in-depth study has been undertaken to explore the determinants of exercise participation for this group and no studies have examined the benefits of habitual physical activity on the health status and quality of life of CF adu
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Dwyer, Tiffany Jane. "Exercise in cystic fibrosis." Discipline of Physiotherapy, Faculty of Health Sciences, University of Sydney, 2010. http://hdl.handle.net/2123/6349.

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Doctor of Philosophy (PhD)<br>Exercise and physical activity have many benefits for adults with cystic fibrosis (CF), including the potential to aid mucus clearance, improve lung function, exercise capacity and quality of life. Despite the recommendations from consensus documents for CF adults to engage in regular physical activity, exercise participation amongst this population is often very low. No in-depth study has been undertaken to explore the determinants of exercise participation for this group and no studies have examined the benefits of habitual physical activity on the health status
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10

Utley, Courtney, and Kristen L. McHenry. "Advances in Cystic Fibrosis." Digital Commons @ East Tennessee State University, 2016. https://dc.etsu.edu/etsu-works/2546.

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The purpose of this review was to identify the history of and advances in cystic fibrosis (CF). New treatment plans, medication developments, and a historical perspective of airway clearance therapy (ACT) will be presented. The importance of treatment compliance and time management in the care of cystic fibrosis patients will also be discussed. Furthermore, the development of cystic fibrosis clinics and the pivotal role they play in the treatment of the disease will be addressed. Lastly, a brief discussion concerning the need for and process of lung transplantation will be reported.
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Álvarez, Fernández Antonio. "Fibrosis Quística: caracterización y particularidades de la edad adulta." Doctoral thesis, Universitat Autònoma de Barcelona, 2021. http://hdl.handle.net/10803/673281.

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Aquesta tesi doctoral analitza les particularitats de la fibrosi quística (FQ) de l'adult mitjançant les dades clíniques, analítiques, radiològiques, funcionals, microbiològiques, genètics i de seguiment, recollides entre gener de 1983 i desembre de 2019 en 349 pacients amb FQ confirmada , controlats en la Unitat d'Adults de FQ de l'Hospital Vall d'Hebron. A més d'estudiar la població global, es van realitzar estudis de: diferències entre els pacients diagnosticats a la infància i en l'edat adulta; relació entre genotip i fenotip; variants deep intró; sub/infertilitat femenina i evolució d
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Mekus, Frauke. "Cystic fibrosis as a genetically complex disease Cystische Fibrose: eine genetisch komplexe Erkrankung /." [S.l.] : [s.n.], 2000. http://deposit.ddb.de/cgi-bin/dokserv?idn=959242287.

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13

Nyabam, Samuel. "Understanding the link between transglutaminase and the induction of fibrosis in cystic fibrosis (CF)." Thesis, Aston University, 2015. http://publications.aston.ac.uk/25336/.

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The emerging role of the multifunctional enzyme, Transglutaminase 2 (TG2) in Cystic Fibrosis (CF) has been linked to its increased expression and intracellular transamidating activity. However, a full understanding of the molecular mechanisms involved still remains unclear despite numerous studies that have attempted to delineate this process. These mechanisms include the NFκB and TGFβ1 pathway amongst others. This study reveals for the first time that the development of fibrosis in CF is due to a TG2-driven epithelial to mesenchymal transition (EMT) via a mechanism involving the activation of
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Pasinetti, Nadia. "Wound healing signals mediated by Rho/ROCK activation in response to radiotherapy and consequences fot treatmeny of late damage within normal tissues." Phd thesis, Université Paris Sud - Paris XI, 2012. http://tel.archives-ouvertes.fr/tel-00714360.

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Radiotherapy is the second most important treatment modality after surgery in the treatment of cancer. Recent technical advancements, such as intensity-modulated radiation therapy (IMRT) or image-guided radiation therapy (IGRT), combined with new targeted drugs have significant promise for therapeutic outcome. However radiation treatment could result in disabling normal tissue injury and in the development of progressive fibrosis in a subset of sensitive patients and in long-term cancer survivors. The main feature of tissue fibrosis is excessive accumulation of abnormal and cross-linked collag
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15

Hamama, Saad. "Radiation-induced fibrosis : Characterization of the anti-fibrotic mechanisms displayed by pentoxifylline/vitamin E." Thesis, Paris 11, 2012. http://www.theses.fr/2012PA11T071.

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La fibrose radio-induite est une complication sévère et tardive de la radiothérapie. Plusieurs études cliniques ont montré que la combinaison pentoxifylline-vitamine E est un traitement sûr et efficace contre la fibrose. Cependant, les mécanismes moléculaires de son efficacité restent inexplorés. Nous avons montré l’efficacité de la combinaison pentoxifylline-vitamine E dans l’entéropathie radique dans une faisabilité clinique. En parallèle, en utilisant un modèle unique, in vitro, de cellules musculaires lisses intestinales primaires isolées des personnes atteintes de l’entéropathie radique,
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Dobson, Lee. "Glucose tolerance in cystic fibrosis." Thesis, University of Exeter, 2004. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.403679.

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17

Hurley, Matthew. "Lung infection in cystic fibrosis." Thesis, University of Nottingham, 2016. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.716679.

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Cystic fibrosis (CF) is characterised by viscid secretions that, in the lungs, pre-dispose to infection. Many people with CF experience 'pulmonary exacerbations' accompanied by intermittent deteriorations in lung function. Pulmonary exacerbations are associated with a more rapid decline in lung health over time and are associated with infection. Early infection managed aggressively may be successfully eradicated. With successive infections and cumulative lung damage, chronic infection is established. Chronic infection leads to progressive decline in lung function with associated reductions in
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Downey, D. G. "Airways inflammation in cystic fibrosis." Thesis, Queen's University Belfast, 2002. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.269047.

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19

Evans, Katharine Sarah Emily. "Cystic Fibrosis and the eye." Thesis, Cardiff University, 2009. http://orca.cf.ac.uk/54848/.

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Cystic Fibrosis (CF) results from the defective function of CF Transmembrane Conductance Regulator (CFTR), an ion channel which facilitates epithelial chloride secretion. Previous observations of dry eye and abnormal visual function in CF subjects have been considered secondary manifestations due to associated vitamin A deficiency (VAD) and CF-related diabetes (CFRD). However, CFTR is fundamentally present in the corneal, conjunctival and retinal pigment epithelium and the corneal endothelium. The hypothesis for this thesis was that abnormal chloride secretion in CF causes reduced basal tear s
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Wright, Adam. "The macrophage in cystic fibrosis." Thesis, University of Leicester, 2007. http://hdl.handle.net/2381/8783.

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Background: Cystic fibrosis (CF) is caused by absent/defective CF transmembrane conductance regulator protein (CFTR). CF is characterized by thick airway mucus, chronic infection and neutrophil inflammation leading to respiratory failure. I analysed airway macrophages (MΦs) and their expression of pattern recognition receptors (PRRs) in CF, since these cells are crucial to airway immune defence and they can orchestrate inflammation. I also performed transcript analysis of CF monocyte-derived MΦs (MDMs). Methods: Sputum was induced in CF paediatric and adult cohorts. Phenotype and function of C
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Rao, Satish Ramakrishna. "Blood monocytes in cystic fibrosis." Thesis, University of Leicester, 2009. http://hdl.handle.net/2381/7345.

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Background: Neutrophilic inflammation causes lung damage in cystic fibrosis (CF). Recent data from animal models and other chronic pulmonary inflammatory conditions suggest that the monocytes/macrophages may be an important driver of airway inflammation. CF may be associated with increased airway levels of chemoattractants for monocytes and resulting expansion of CD14++ small macrophages. I sought to assess the levels of monocyte chemoattractants CCL2 and CX3CL1 in the blood and airways of CF patients, and expression of their respective receptors CCR2 and CX3CR1 on monocytes. In a pilot study,
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22

Li, Shuo. "Murine gammaherpesvirus mediated splenic fibrosis." Thesis, University of Edinburgh, 2012. http://hdl.handle.net/1842/8156.

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Infection of IFNγ receptor knockout (IFNγ R-/-) mice with murine gammaherpesvirus-68 (MHV-68) results in fibrosis in the lung, spleen, liver and lymph nodes. In the spleen, pathology involves an increase in the number of latently infected B cells that corresponds with a Th2 biased immune response, in which germinal centres become walled off and fibrosis dominates the splenic architecture. Remarkably, the spleen recovers from this pathology, and the starting point for this process is a loss of latently infected B cells. The aim of this project is to gain further understanding of the control of
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O'Rawe, Angela Marie. "Energy balance in cystic fibrosis." Thesis, Queen's University Belfast, 1994. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.261933.

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24

McCloskey, Margaret. "Energy balance in cystic fibrosis." Thesis, Queen's University Belfast, 1999. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.287209.

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25

Smith, David L. "Nocturnal hypoxaemia in cystic fibrosis." Thesis, University of Southampton, 1994. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.296267.

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26

Corbett, Christopher. "Novel markers of liver fibrosis." Thesis, University of Birmingham, 2015. http://etheses.bham.ac.uk//id/eprint/5603/.

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With chronic liver disease rising, the need to stage of liver disease and fibrosis accurately is paramount as it helps guide therapy and informs prognosis. Liver biopsy is a flawed gold standard, associated with morbidity and mortality. Application of simple non-invasive tests to assess fibrosis could provide a safe way of identifying patients in greatest need of intervention and of monitoring response to therapy. I have shown in this thesis that transient elastography is an excellent tool for ruling out significant fibrosis in patients with chronic liver disease. It is easy to learn and succe
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Hiscox, Rachel Joy. "The retina in cystic fibrosis." Thesis, Cardiff University, 2013. http://orca.cf.ac.uk/59738/.

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Cystic fibrosis (CF) is caused by defective function of CF Transmembrane Conductance Regulator (CFTR), an epithelial ion channel that facilitates chloride secretion. Previous research has identified impaired dark adaptation (DA) in CF, which has been attributed to concomitant vitamin A deficiency or CF-related diabetes (CFRD). However, CFTR has been localised to the retinal pigment epithelium (RPE) and it is proposed that abnormal DA could be a primary manifestation of CF. DA is similarly impaired in individuals with type 1 and 2 diabetes and is thought to be caused by retinal hypoxia as oxyge
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McIlwaine, Patricia Margaret. "Airway clearance in cystic fibrosis." Thesis, Ulster University, 2014. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.625501.

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This thesis overviews and highlights a body of work performed at the BC Children's Hospital, Vancouver, Canada between 1986 and 2013, and involving travel to Belgium, Denmark and the United Kingdom. It is based on five articles published by McIlwaine on research and development in the use of various airway clearance techniques for the treatment of cystic fibrosis, namely: Autogenic Drainage; Positive Expiratory Pressure; Oscillating Positive Expiratory Pressure; and High Frequency Chest Wall Oscillation. The thesis comprises of seven chapters, including the introduction. Chapter 2 presents the
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Ward, Andrew. "A cystic fibrosis infection monitor." Thesis, University of Strathclyde, 2015. http://oleg.lib.strath.ac.uk:80/R/?func=dbin-jump-full&object_id=26047.

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Abu, Helal Raghd. "Endothelial Cell CEACAM1 Regulates Fibrosis." Ohio University / OhioLINK, 2009. http://rave.ohiolink.edu/etdc/view?acc_num=ohiou1619725200062184.

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31

Abu, Helal Raghd. "Endothelial Cell CEACAM1 Regulates Fibrosis." Ohio University / OhioLINK, 2021. http://rave.ohiolink.edu/etdc/view?acc_num=ohiou1619725200062184.

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32

Patel, Vimal. "Myocardial fibrosis in hypertrophic cardiomyopathy." Thesis, University College London (University of London), 2018. http://discovery.ucl.ac.uk/10046437/.

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Hypertrophic cardiomyopathy (HCM) is characterised by myocardial hypertrophy, fibrosis and abnormal vascular pathology and is usually caused by mutations in sarcomeric protein genes. Histological studies and in vivo imaging with cardiac magnetic resonance imaging (CMRI) have shown that myocardial fibrosis is an important entity that contributes to disease progression. However, little is known about the regulation of genes involved in collagen synthesis and metabolism, the pathways that contribute to the development of myocardial fibrosis and whether this is an early pathological process which
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Vitko, Megan Sue. "Intestinal Dysfunction in Cystic Fibrosis." Case Western Reserve University School of Graduate Studies / OhioLINK, 2016. http://rave.ohiolink.edu/etdc/view?acc_num=case1459248266.

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34

Bizzell, Laurie. "Maternal Stress and Cystic Fibrosis." Thesis, University of North Texas, 1996. https://digital.library.unt.edu/ark:/67531/metadc278693/.

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The purpose of the current study was to examine the relationship between parent and child factors for mothers of children diagnosed with cystic fibrosis to predict mother's psychological distress. Mothers were surveyed to identify measurement models in areas of Child and Parental characteristics and a Full Causal Model of Maternal distress. Factors related to Child Characteristics include general parental stressors and cystic fibrosis specific parental stressors. Factors related to Parental Characteristics include the mother's sense of parental competence and self-esteem. Additional factors re
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Putman, Melissa. "Cystic Fibrosis Related Bone Disease." Thesis, Harvard University, 2015. http://nrs.harvard.edu/urn-3:HUL.InstRepos:17613728.

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Over the past several decades, life expectancy for patients with cystic fibrosis (CF) has increased significantly. As patients live longer, other nonpulmonary co-morbidities related to CF have become increasingly prevalent, including CF-related bone disease. Because CF related bone disease has only recently emerged as a clinical problem, and the underlying bone alterations and pathogenesis of this condition have not been established. This thesis explores the underlying bone micro-architecture and strength alterations found in adults with CF using state-of-the-art bone imaging techniques and e
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Zibadi, Sherma. "Metabolic Syndrome-Induced Cardiac Fibrosis." Diss., The University of Arizona, 2009. http://hdl.handle.net/10150/195321.

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Recent studies support the association between metabolic syndrome (MetS), a cluster of cardiovascular risk factors, and diastolic dysfunction. Disproportionate collagen accumulation, particularly cross-linking of collagen, plays a key role in translating interstitial fibrosis into mechanical chamber stiffness and diastolic dysfunction. Characteristic changes in the expression and activity of myocardial lysyl oxidase (LOX), a matrix modifying enzyme that catalyzes cross-linked collagen, are unclear in MetS. We established a diet-induced MetS model to study diastolic dysfunction by treating male
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Dentini, Priscila. "Complexo Burkholderia cepacia em pacientes com fibrose cística em um Centro de Referência no Brasil = identificação, prevalência e importância clínica." [s.n.], 2010. http://repositorio.unicamp.br/jspui/handle/REPOSIP/309992.

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Orientador: José Dirceu Ribeiro<br>Dissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciências Médicas<br>Made available in DSpace on 2018-08-16T17:48:38Z (GMT). No. of bitstreams: 1 Dentini_Priscila_M.pdf: 10675938 bytes, checksum: afc138751a1f11e6991384ca9bbee25c (MD5) Previous issue date: 2010<br>Resumo: Objetivos: Determinar a prevalência da colonização/infecção pelo Complexo Burkholderia cepacia (CBc) e os respectivos genomovares em pacientes com fibrose cística (FC) e comparar os indicadores clínicos, nutricionais, tomográficos e funcionais de gravidade da lesão p
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Fernandes, Flávia Ferreira. "ELF (Enhanced Liver Fibrosis) como marcador não invasivo de fibrose hepáticana hepatite C crônica." Universidade do Estado do Rio de Janeiro, 2014. http://www.bdtd.uerj.br/tde_busca/arquivo.php?codArquivo=8773.

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A fibrose hepática é o aspecto mais relevante e o mais importante determinante de morbimortalidade na hepatite C crônica (HCC). Historicamente, a biópsia hepática é o método de referência para avaliação da fibrose causada pela HCC, apesar de apresentar limitações. O estudo de marcadores não invasivos, que possam obviar a necessidade da biópsia, é uma área de constante interesse na hepatologia. Idealmente, a avaliação da fibrose hepática deveria ser acurada, simples, prontamente disponível, de baixo custo e informar sobre o prognóstico da patologia. Os marcadores não invasivos mais estudados s
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Meziani, Lydia. "Study of Interaction Between the Inflammatory Response and Radiation-Induced Fibrosis." Thesis, Paris 11, 2015. http://www.theses.fr/2015PA11T041.

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La fibrose radio-induite (FRI) est une complication tardive de la radiothérapie souvent associée à une réponse inflammatoire chronique et à un infiltrat de macrophages. Aujourd’hui, les macrophages sont pressentis comme des médiateurs cellulaires important dans le processus de fibrose mais leur rôle n’a jamais été étudié dans le contexte de la FRI. Dans une précédente étude nous avions montré que l’irradiation (IR) induit une polarisation M1 des macrophages cardiaques après irradiation de souris ApoE-/- et est associée un score de fibrose élevé, ce qui suggérait que la polarisation des macroph
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Durham, Dixie Lea. "Survey of adult cystic fibrosis patients and parents of cystic fibrosis patients on nutrition education." [Boise, Idaho] : Boise State University, 2009. http://scholarworks.boisestate.edu/td/8/.

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Kaarteenaho-Wiik, R. (Riitta). "Tenascin expression and distribution in pulmonary and pleural fibrotic disorders." Doctoral thesis, University of Oulu, 1999. http://urn.fi/urn:isbn:9514253051.

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Abstract Fibrotic pulmonary and pleural disorders represent a group of intrathoracic disorders with different etiologies and prognoses. A prominent part of both pulmonary and pleural fibrotic disorders remains etiologically unknown. An essential feature for all these disorders is an increase and disarray of many extracellular matrix proteins which take part in the remodeling of the fibrotic tissue. Further, the injury in pulmonary as well as in pleural fibrosis occurs often at the border between the epithelial or mesothelial and the mesenchymal cells breaking the epithelial basement membrane.
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Liu, Yi-Chia. "Understanding chronic inflammatory diseases in the human lung : the cystic fibrosis and idiopathic pulmonary fibrosis paradigms." Thesis, University of Nottingham, 2014. http://eprints.nottingham.ac.uk/27807/.

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The chronic infection of the cystic fibrosis (CF) lung with Pseudomonas aeruginosa strongly correlates with critical outcomes. Pseudomonas alkyl-quinolone signal (PQS) is a diffusible cell-density dependent signal controlling the production of virulence determinants. The PQS amount in the CF lung was proportionate to P. aeruginosa colonisation and PQS molecules have been demonstrated to inhibit pro-inflammatory signalling. However, how PQS influence the recognition of P. aeruginosa by the human lung is unknown. The contribution of PQS to the interaction of P. aeruginosa with human bronchial ep
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DaSilva, Santos Iara Karlla. "Impacto de la inflamación y fibrosis en la función del injerto renal." Doctoral thesis, Universitat Autònoma de Barcelona, 2018. http://hdl.handle.net/10803/666855.

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A pesar de los grandes avances en el campo del trasplante renal (TR), los resultados a largo plazo aún no son óptimos. Varios estudios han demostrado que diversos factores como la inflamación precoz y/o la presencia de fibrosis intersticial (FI) están asociados a un peor pronóstico del injerto, pero todavía se trata de un tema controvertido. En este estudio analizamos el estado inflamatorio (Banff, macrófagos CD68+, fenotipos de macrófagos M1-M2) y la expresión génica de múltiples factores relacionados con la inflamación y FI (TGF-β1, metaloproteinasas, proteínas de matriz extracelular, entre
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Pat, Betty Kila. "Signal transduction pathways in renal fibrosis /." St. Lucia, Qld, 2003. http://www.library.uq.edu.au/pdfserve.php?image=thesisabs/absthe17739.pdf.

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Ball, Lindsay Clare. "Cystic fibrosis and vitamin D supplementation." Thesis, Birmingham, Ala. : University of Alabama at Birmingham, 2010. https://www.mhsl.uab.edu/dt/2010m/ball.pdf.

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46

Andersson, Charlotte. "Towards Pharmacological Treatment of Cystic Fibrosis." Doctoral thesis, Uppsala University, Department of Medical Cell Biology, 2002. http://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-2634.

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<p>S-nitrosogluthatione is an endogenous substance, present at decreased levels in the lungs of CF patients and was recently found to induce mature CFTR in airway epithelial CF cell lines. We show that S-nitrosoglutathione in physiological concentrations increases the presence of ΔF508 CFTR in the cell membrane and induces cAMP dependent chloride transport in cystic fibrosis airway epithelial cells. The properties of S-nitrosoglutathione include other potential benefits for the CF patient and make this agent an interesting candidate for pharmacological treatment of CF that needs to be further
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47

Renzoni, Elisabetta Augusta. "Fibroblast gene expression in pulmonary fibrosis." Thesis, Imperial College London, 2008. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.490948.

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Background: Idiopathic pulmonary fibrosis (IPF) has a median survival since diagnosis of2-3 years. Interstitial lung disease associated with systemic sclerosis (SSc-ILD) is characterised by a better survival than IPF. Lung fibroblasts are the main cell type responsible for the excessive extracellular matrix synthesis in lung fibrosis. The pathogenesis offibrosis in both conditions is largely unknown, and it is unclear to what extent it differs between the two. Hypothesis: The gene expression profile of fibroblasts from IPF and SSc-ILD differs from control lung fibroblasts; significant differen
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48

Dunlevy, Fiona Kathleen Carol. "Protease-antiprotease imbalance in cystic fibrosis." Thesis, Queen's University Belfast, 2008. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.491992.

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Affects over 8000 patients In the UK. Persistent neutrophilic inflammation is associated with high levels of airway NE. DX-890 is a smallprotein inhibitor ofNE developed by Dyax, USA for use in CF. This project investigated the ex-vivo effects ofDX-890 on human sputum, neutrophils and epithelial cells, to help determine the potential ofDX-890 as a drug for CF. The Ki ofDX-890 was measured to be 11.12 pM. ICso values measured in pure NE and CF sol were similar, demonstrating that DX-890 retained activity in CF sol. Thickened dehydrated CF mucus may prevent access ofDX-890 to NE and reduce effic
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49

Govan, John R. W. "Pseudomonas, alginate biosynthesis and cystic fibrosis." Thesis, University of Edinburgh, 1994. http://hdl.handle.net/1842/28137.

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50

Winbanks, Catherine, and winbanks@unimelb edu au. "Novel Aspects of Renal Tubulointerstitial Fibrosis." RMIT University. Medical Sciences, 2007. http://adt.lib.rmit.edu.au/adt/public/adt-VIT20080617.143850.

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Tubulointerstitial fibrosis is the key histological predictor of the progression of declining renal function and the final common pathway of progressive kidney disease, regardless of aetiology. Despite its significance, there are currently no treatments available to abrogate this process and those that suffer with this burden eventually succumb to renal failure. Tubulointerstitial fibrosis is largely mediated by fibroblasts and myofibroblasts present in the interstitium. In response to injury, activated fibroblasts differentiate into myofibroblasts which serves as a histological hallmark of
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