Dissertations / Theses on the topic 'Lymphangioleiomyomatosis'
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Chang, William Y. C. "Metalloproteinases in lymphangioleiomyomatosis." Thesis, University of Nottingham, 2014. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.664270.
Full textNG, Ho Yin. "Cellular Characteristics of Lymphangioleiomyomatosis." Thesis, The University of Sydney, 2013. http://hdl.handle.net/2123/9510.
Full textKinder, Brent W. M. D. "Clinical Predictors of Survival in Lymphangioleiomyomatosis." University of Cincinnati / OhioLINK, 2011. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1298041138.
Full textHerranz, Ors Carmen 1992. "Towards improving lymphangioleiomyomatosis care : a study of biomarkers and therapies." Doctoral thesis, Universitat Pompeu Fabra, 2019. http://hdl.handle.net/10803/668273.
Full textLAM es un trastorno pulmonar raro, destructivo y metastásico. Esta enfermedad está causada por células que tienen mutaciones en los genes TSC1/2 cuyo origen sigue siendo desconocido. El tratamiento de LAM consiste en la inhibición de mTOR con sirolimus. Sin embargo, esta terapia presenta una tolerabilidad variable. El diagnóstico de LAM y su monitorización puede ser un desafío ya que los síntomas son muy heterogéneos. En esta tesis, nuestro objetivo era mejorar la atención de LAM a través de la identificación de biomarcadores y la exploración de nuevos enfoques terapéuticos. Paralelamente, también estudiamos el origen de la enfermedad. En cuanto a los biomarcadores, el principal metabolito derivado de la histamina MIAA estaba aumentado en el plasma de pacientes de LAM. Estudios combinados de tejidos pulmonares y modelos celulares mostraron un aumento en el metabolismo de las monoaminas y en la vía de señalización de la histamina. Además, la inhibición de estas dos rutas redujo la tumorogénesis de modelos LAM de ratón. Los resultados de esta tesis podrían ayudar a mejorar el diagnóstico, la monitorización y el tratamiento de las pacientes de LAM.
Contini, Paola <1975>. "Effect of estrogen suppression on lung function in pulmonary Lymphangioleiomyomatosis." Doctoral thesis, Alma Mater Studiorum - Università di Bologna, 2012. http://amsdottorato.unibo.it/4497/1/contini_paola_tesi.pdf.
Full textContini, Paola <1975>. "Effect of estrogen suppression on lung function in pulmonary Lymphangioleiomyomatosis." Doctoral thesis, Alma Mater Studiorum - Università di Bologna, 2012. http://amsdottorato.unibo.it/4497/.
Full textDelaney, Sean Phillip. "Modeling and Therapeutic Development for the Tuberous Sclerosis Related Neoplasm Lymphangioleiomyomatosis." Thesis, Université d'Ottawa / University of Ottawa, 2019. http://hdl.handle.net/10393/39810.
Full textVafamand, Shahpar. "Knowledge, Attitude, Lifestyle Practices, and Quality of Life in Sporadic Lymphangioleiomyomatosis Patients." ScholarWorks, 2014. http://scholarworks.waldenu.edu/dissertations/208.
Full textDavies, David Mark. "mTOR inhibition as a therapeutic strategy in tuberous sclerosis or sporadic lymphangioleiomyomatosis." Thesis, Cardiff University, 2011. http://orca.cf.ac.uk/54431/.
Full textBoustany, Sarah. "Mechanisms of Airway Remodelling." Thesis, The University of Sydney, 2008. http://hdl.handle.net/2123/3577.
Full textBoustany, Sarah. "Mechanisms of Airway Remodelling." University of Sydney, 2008. http://hdl.handle.net/2123/3577.
Full textAsthma is an inflammatory disease characterised by tissue remodelling. A prominent feature of this remodelling is an increase in the number and size of the blood vessels- formed from pre-existing capillaries – angiogenesis (Siddiqui et al., 2007; Wilson, 2003). This is triggered by many different endogenous angiogenic stimulators such as vascular endothelial growth factor (VEGF), and inhibited by endogenous angiogenic inhibitors such as tumstatin. Tumstatin is the non-collagenous domain (NC1) of the collagen IV α3 chain which, when cleaved, inhibits endothelial cell proliferation and induces apoptosis. Experiments described in this thesis have for the first time demonstrated the absence of tumstatin in the airways of individuals with asthma and lymphangioleiomyomatosis (LAM) as well as the functional responses to tumstatin as an angiogenic inhibitor, both in vitro and in vivo, in the airway. Although tumstatin was absent from the airways of asthmatic and LAM individuals it was present in the airways of individuals with no airways disease, chronic obstructive pulmonary disease, bronchiectasis and cystic fibrosis. No significant difference was seen in the levels of the Goodpasture Binding Protein (GPBP), a phosphorylating protein responsible for the alternate folding of tumstatin, between asthmatic, LAM and individuals with no airways disease. The αvβ3 integrin, reported to be necessary for the activity of tumstatin, as well as the individual αv and β3 sub-units were shown to be equally expressed in the airways of all patient groups. Co-localisation of tumstatin, VEGF and the αvβ3 integrin was seen in the disease free airways, however, a different pattern of VEGF and the αvβ3 integrin expression was observed in asthmatic and LAM airways with minimal co-localisation. Tumstatin was detected in serum and bronchoalveolar lavage fluid (BAL-f) samples from asthmatics and individuals with no airway disease, however there was no significant difference in the level of expression between the two groups. It was demonstrated that the tumstatin detected in the serum and BAL-f samples from asthmatics and individuals with no airway disease was part of the whole collagen IV α3 chain and not in its free and potentially active form. The ability of recombinant tumstatin to inhibit tube formation and proliferation of primary pulmonary endothelial cells was demonstrated for the first time. Further, the functional response of tumstatin was demonstrated in vivo in a mouse model of allergic airway disease. Tumstatin inhibited angiogenesis in the airway and decreased airway hyperresponsiveness. Whether there is potential for tumstatin, or a derivative thereof, to be of therapeutic value in airways diseases in which angiogenesis is a component should be the subject of future studies.
Gupta, Nishant. "The NHLBI Lymphangioleiomyomatosis (LAM) Registry: Longitudinal Analysis to Determine the Natural History of LAM." University of Cincinnati / OhioLINK, 2017. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1504879473662385.
Full textHaylen, Denise Clare. "Women's experiences of living with a rare disease, lymphangioleiomyomatosis (LAM): A life history study." Thesis, The University of Sydney, 2015. http://hdl.handle.net/2123/15491.
Full textDongre, Arundhati. "Modelling lymphangioleiomyomatosis (LAM) using two-dimensional and three-dimensional in vitro co-culture systems." Thesis, University of Nottingham, 2018. http://eprints.nottingham.ac.uk/50037/.
Full textMahi, Naim. "Connectivity Analysis of Single-cell RNA-seq Derived Transcriptional Signatures." University of Cincinnati / OhioLINK, 2020. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1613748441148963.
Full textLu, Yiyang. "Exploring Rapamycin-induced Pro-survival Pathways in Tuberous Sclerosis Complex and the Development of Alternative Therapies." University of Cincinnati / OhioLINK, 2020. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1613752713277464.
Full textTERRANEO, SILVIA. "WOMEN WITH LYMPANGIOLEIOMYOMATOSIS: FROM RESPIRATORY FUNCTION TO SERUM BIOMARKERS ANALYSIS. PHENOTYPING OF A RARE DISEASE." Doctoral thesis, Università degli Studi di Milano, 2019. http://hdl.handle.net/2434/628877.
Full textChulam, Carolina Salim Gonçalves Freitas. "Avaliação de hipertensão pulmonar em pacientes com linfangioleiomiomatose." Universidade de São Paulo, 2017. http://www.teses.usp.br/teses/disponiveis/5/5150/tde-24082017-133256/.
Full textIntroduction: Lymphangioleiomyomatosis (LAM) is associated with pulmonary hypertension (PH) and is included in group 5 of the current classification (unknown multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The objectives of the study were to evaluate the prevalence and characteristics of PH in patients with LAM at different stages of evolution, as well as to compare the clinical and functional characteristics of the 6-minute walk test (6MWT) and the quality of life of patients with and without PH. Methodology: One hundred and five patients with LAM underwent echocardiogram, pulmonary function test (PFT) and 6MWT. Patients with suspected PH on the echocardiogram, defined as the presence of estimated systolic pulmonary arterial pressure (PsAP) above 35 mmHg, or PFT showing carbon monoxide diffusion (DLco) below 40% of the predicted value, were submitted to right cardiac catheterization to confirm the diagnosis of PH. Results: Eight patients (7.6%) had PH confirmed in right cardiac catheterization, six patients (5.7%) had a pre-capillary pattern and two patients (1.9%) had a post capillary pattern. Only one patient (1%) presented mean pulmonary artery pressure (PAPm) above 35 mmHg. Patients with PH had lower FEV1 and DLco in PFP and greater oxygen desaturation and dyspnea intensity during the 6MWT compared to those without PH. In 63% of patients with confirmed PH, right heart catheterization was performed because of the DLco result. Conclusions: The prevalence of PH is low in patients with LAM. Pulmonary hypertension is commonly mild and is significantly associated with pulmonary parenchymal involvement. The measure DLco has improved the identification of PH in patients with LAM
Orpianesi, E. "STUDIO DELLE CITOCHINE E DELLE METALLOPROTEASI NELLA LINFANGIOLEIOMIOMATOSI E NELLA SCLEROSI TUBEROSA." Doctoral thesis, Università degli Studi di Milano, 2014. http://hdl.handle.net/2434/230542.
Full textJunior, Pedro Medeiros. "Avaliação da função pulmonar, sono, exercício e qualidade de vida em pacientes com linfangioleiomiomatose pulmonar." Universidade de São Paulo, 2008. http://www.teses.usp.br/teses/disponiveis/5/5150/tde-24112008-110954/.
Full textPulmonary lymphangioleiomyomatosis (LAM) is a progressive cistic lung disease of unknown etiology and no established medical treatment. It basically occurs in child bearing age women and its mean survival is about 10 years in some case series. LAM is caractherized by pulmonary function deterioration: obstructive pattern on espirometry and progressive impairment on DLCO capacity which results in rest dyspnea and hypoxemia in advanced cases. Exercise is an important factor that causes hypoxemia in this set of patients and correlates with progressive functional impaiment. Sleep is also another important physiologic moment when cardiorespiratory system may be stressed in these patients, leading to chronic hypoxemia though the years. This phenomenum may be responsible by cor pulmonale observed in advanced stages of LAM. In order to study hemoglobin saturation by oxygen during sleep and its correlation with lung function test (LFT), six minute walk test (6MWT), ecocardiography and SF-36 quality of life variables, 21 consecutive patients with LAM from Hospital das Clínicas University of São Paulo Medical School were evaluated from march 2005 to december 2007. All patients performed full polysomnography (PSG), lung function test, 6MWT and answered Berlim, Epworth and SF-36 quality of life questionnaire. Mean age at beginning of symptoms was 38 8 years, 41 10 years at diagnosis and 45 11 years by the time of the protocol. Dyspnea during routine daily activities was the most frequent symptom (20 / 21). LFT yielded mild obstruction (% FEV1 = 72%; FEV1/FVC = 0,68) and moderate impairment in DLCO (48%). Fourteen patients presented desaturation with SpO2 lower than 90% on PSG. Mean time with SpO2 lower than 90% was 2 hours and 25 minutes (mainly during REM and S3 and S4). Median apnea-hipopnea index (AHI) was 1,7 (normal). Pearson and Spearman correlations yielded that the main variables related to desaturation below 90% on PSG with p < 0.01 were: Desaturation on TC6M rs = 0,57 Spearman %FEV1 r = -0,80 Pearson %FVC r = -0,80 Pearson %DLCO rs = -0,64 Spearman After that, functional variables were stratified by SpO2 < 90% during PSG. Wilcoxon test showed that besides all 4 previous variables RV/TLC (p=0.04) was also statistically different between patients who did and did not desaturate. SF-36 yielded impairments in all domains, particularly in emotional aspects among patients who desaturated on PSG. Heart ecocardiography detected pulmonary hypertension in 5 patients. Our study found out for the first time that patients with LAM desaturate during sleep. Although pulmonary function variables are related to sleep desaturation, desaturation occured even in patients with mild or no impairments in lung function. 6MWT desaturation ,but not distance walked, correlated to sleep desaturation. SF-36 analysis yielded impairments in all domains of quality of life, particularly in emotional aspects in patients who desaturated.
Araujo, Mariana Sponholz. "Avaliação do impacto de um programa de reabilitação pulmonar na capacidade de exercício em portadoras de linfangioleiomiomatose." Universidade de São Paulo, 2015. http://www.teses.usp.br/teses/disponiveis/5/5150/tde-24082015-113343/.
Full textIntroduction: Lymphangioleiomyomatosis (LAM) is a low-grade neoplasm, which is frequently associated with reduced exercise capacity, secondary to multiple factors including gas exchange impairment, ventilatory limitation and dynamic hyperinflation (DH). Pulmonary rehabilitation (PR) has proven benefits in many chronic pulmonary diseases but it was not evaluated in LAM. Objectives: To evaluate the impact of a PR program in women with LAM, when compared to a control group, in the following parameters: exercise capacity (primary outcome), DH, dyspnea, daily physical activity, quality of life, anxiety and depression, lung function and muscle strength. Methods: A non-randomized controlled clinical trial that included 21 LAM patients in the PR group and 19 in the control group. The PR program lasted 3 months, comprising 24 sessions of 1 hour (30 minutes of aerobic exercise and 30 minutes of muscle strength training). The initial evaluation included a maximum incremental cardiopulmonary exercise test (CPET). The following variables were assessed before and after PR or observation (control group): exercise capacity using the tolerable limit duration (Tlim) in constant work rate (CWR) exercise testing; walking distance and oxygen desaturation (six-minute walk test, 6MWT), dyspnea (Modified Medical Research Council Dyspnea Scale -mMRC; Basal Dyspnea Index - BDI, and Transitional Dyspnea Index -TDI); daily physical activity (pedometer); health-related quality of life (St George\'s Respiratory Questionnaire, SGRQ); anxiety and depression (Hospital Anxiety and Depression Scale, HADS); pulmonary function tests (PFT) and muscle strength (one-repetition maximum, 1RM). Results: There was no difference in baseline characteristics between the PR and control groups related to age (45 ± 11 vs. 40 ± 9 years, p = 0.12), FEV1 (74 ± 30 vs. 70 ± 27% pred, p = 0.67), DLCO (67 ± 33 vs. 64 ± 30% pred, p = 0.79), maximum work rate (77 ± 33 vs. 76 ± 35 W, p = 0.93) and peak O2 (17 ± 5 vs. 16 ± 4 ml/ kg/ min; p = 0.52) in incremental CPET. The PR group had a significant improvement when compared to the control group in (expressed in median [interquartile range]): Tlim (169 s [2 - 303 s] vs. -33 s [-129 - 39 s], p = 0.001) and O2 (11% [2 - 26%] vs. -2% [-7 - 5% pred], p = 0.001) in CWR exercice testing, mMRC (0 [-1 - 0] vs. 0 [0 - 1], p< 0.001), ( TDI (3 [2 - 3] vs.0 [-2 - 0], p< 0.001), daily steps (752 [-694 - 1814] vs. -138 [-830 - 208], p= 0.02), SGRQ (-8 [-16 - 2] vs. 2 [-4 - 5], p = 0.002), walking distance in 6MWT (median 59 m [13 - 81] vs. 20 [-12 - 30], p = 0.002) and 1 RM for all muscle groups trained (ex. quadriceps 39% [20 - 70%] vs. 4% [0 - 17%], p <0.001). There was a trend towards improvement in depression symptoms and total HADS. DH, desaturation during exercise, anxiety symptoms and PFT did not improve after PR. There was a moderate correlation between increased Tlim and variations of mMRC, peak O2 in CWR exercise testing, Borg dyspnea isotime and Borg leg discomfort isotime. Conclusions: PR is associated with improvements in exercise capacity, dyspnea, daily physical activity, health-related quality of life and muscle strength in patients with LAM. The main mechanism suggested is peripheral muscles adaptation
BECETTE, VERONIQUE. "La lymphangioleiomyomatose pulmonaire diffuse : a propos d'un cas, revue de la litterature." Lille 2, 1989. http://www.theses.fr/1989LIL2M323.
Full textPreisler, Katharina. "Pulmonale Lymphangioleiomyomatose morphologische und immunhistochemische Untersuchungen zum Konzept des "Melano-Myoperizyten" /." [S.l.] : [s.n.], 2003. http://deposit.ddb.de/cgi-bin/dokserv?idn=970378327.
Full textHeinrich, Henriette. "Zellzyklusregulation in der pulmonalen Lymphangioleiomyomatose im Vergleich zu glatter, ortsständiger Muskulatur." Diss., lmu, 2010. http://nbn-resolving.de/urn:nbn:de:bvb:19-127083.
Full textMIGNOT, THEVENET FLORENCE. "La lymphangioleiomyomatose pulmonaire : transplantation pulmonaire et traitement hormonal ; a propos d'un cas ; revue de la litterature." Lyon 1, 1994. http://www.theses.fr/1994LYO1M222.
Full textRabbe, Géraldine. "La lymphangioléiomyomatose pulmonaire : à propos d'un cas. Revue de la littérature." Bordeaux 2, 1999. http://www.theses.fr/1999BOR2M141.
Full textHoffmann, Anna Lena. "Effekt von Sirolimus im Vergleich zu einer Therapie mit Hormonpräparaten auf die Lungenfunktion bei Patientinnen mit pulmonaler Lymphangioleiomyomatose." Diss., Ludwig-Maximilians-Universität München, 2014. http://nbn-resolving.de/urn:nbn:de:bvb:19-181905.
Full textChiaramonte, E. "ALTERAZIONI ISTOPATOLOGICHE INDOTTE DA CELLULE UMANE LAM/TSC IN TOPI NUDI: UN MODELLO DI LINFANGIOLEIOMIOMATOSI. REVERSIONE DEL DANNO CON ANTICORPO ANTI-EGFR." Doctoral thesis, Università degli Studi di Milano, 2012. http://hdl.handle.net/2434/171330.
Full textHoffmann, Anna Lena [Verfasser], and Jürgen [Akademischer Betreuer] Behr. "Effekt von Sirolimus im Vergleich zu einer Therapie mit Hormonpräparaten auf die Lungenfunktion bei Patientinnen mit pulmonaler Lymphangioleiomyomatose / Anna Lena Hoffmann. Betreuer: Jürgen Behr." München : Universitätsbibliothek der Ludwig-Maximilians-Universität, 2014. http://d-nb.info/1072376326/34.
Full textKovalenko, Andrii. "Therapeutic targeting of DGKA-mediated macropinocytosis in lymphangioleiomyomatosis." Thesis, 2020. https://hdl.handle.net/2144/41149.
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Pollock, Bar-Ziv Stacey. "The lung transplant experiences of women with pulmonary lymphangioleiomyomatosis." 2005. http://link.library.utoronto.ca/eir/EIRdetail.cfm?Resources__ID=371014&T=F.
Full textPreisler, Katharina [Verfasser]. "Pulmonale Lymphangioleiomyomatose : morphologische und immunhistochemische Untersuchungen zum Konzept des "Melano-Myoperizyten" / vorgelegt von Katharina Preisler." 2003. http://d-nb.info/970378327/34.
Full textHeinrich, Henriette [Verfasser]. "Zellzyklusregulation in der pulmonalen Lymphangioleiomyomatose im Vergleich zu glatter, ortsständiger Muskulatur / vorgelegt von Henriette Sophie Heinrich." 2010. http://d-nb.info/1011185296/34.
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