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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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Fortin, Carol M. "Creatinine Clearance Estimation in Cystic Fibrosis Patients." The University of Arizona, 2006. http://hdl.handle.net/10150/624684.
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Class of 2006 AbstractObjectives: To develop a new equation to predict creatinine clearance specific for cystic fibrosis patients. Methods: A literature review was performed to capture data on the daily creatinine excretion in CF patients in relation to age, weight, height, and other physiologic variables. Nonlinear mixed effect modeling was then used to develop an equation to estimate creatinine clearance using individual covariates. The performance of the new equation developed was compared to the Cockcroft and Gault method in a CF population (age > 16 years). Results: A database of individual patient data from a previously published study of 19 patients was created. Significant covariates for model development included actual body weight, sex, and serum creatinine. The final candidate model was: 5.62× ABW0.67 CrCl = sCr(mg / dl) × 0.649( female) Conclusions: The results of the mean absolute error and root mean squared error calculations show that the new equation resulted in less bias and better precision than Cockcroft-Gault, Jeliffe I, and Jeliffe II based on the limited data available. However, these conclusions are limited in that the only evaluation data available was the same data that was used for model development.
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Rucker, Bianca M. G. "A sexual profile of adults with cystic fibrosis : the sexuality and sexual concerns of adults with cystic fibrosis." Thesis, University of British Columbia, 1987. http://hdl.handle.net/2429/26909.
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Only in recent years have diagnostic and therapeutic advances lengthened the life expectancy for patients who have cystic fibrosis sufficiently to allow some of them to live into adulthood. Health care professionals have been focusing on survival issues and are only recently beginning to look at quality of life issues, such as sexuality, of these patients. The purpose of the study was to create a sexual profile of adults with cystic fibrosis which would describe their sexuality and sexual concerns.
A questionnaire was developed and sent to all of the adult cystic fibrosis patients (19 years of age and older) in British Columbia (50 patients), all of whom attend the Shaughnessy Hospital Adult CF Clinic in Vancouver. The 62% response rate provided data for the sexual profile which indicated that 90% of the respondents were sexually active. Only a small number of subjects reported sexual difficulties in their relationships. Concerns about the impact of CF on their sexuality included: the effect of the potentially limited lifespan on their relationships, practical considerations such as fatigue and coughing during sexual activity, and poor body image.
A major issue for CF males is that most of them are infertile due to CF. How and when men should be told about this issue was an important question for the CF Clinic staff. Responses indicated that men thought they should find out from either the physician in the pediatric CF clinic or the physician in the adult CF clinic. Furthermore, 100% of the men suggested that this issue be discussed with males before the age of 19 years.
Limitations and recommendations of the research are discussed. A major recommendation is for physicians and other health care professionals in CF clinics to give patients the opportunity to discuss sexual issues.Education, Faculty of
Educational and Counselling Psychology, and Special Education (ECPS), Department of
Graduate
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Bhakta, Dharti, Kalyn Schmidt, Aubrey Silvester, Marcella Honkonen, and Hanna Phan. "Impact on Vitamin D Status in Cystic Fibrosis Patients After Implementation of 2012 Cystic Fibrosis Foundation Guidelines." The University of Arizona, 2015. http://hdl.handle.net/10150/614103.
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Class of 2015 AbstractObjectives: The primary objective of the study was to evaluate for change in vitamin D levels and regimens in cystic fibrosis (CF) patients following implementation of the 2012 Cystic Fibrosis Foundation (CFF) vitamin D guidelines. Secondary endpoints included clinician adherence to guideline recommendations for treatment and management of vitamin D deficiency. Methods: This retrospective chart review included CF patients with 25-hydroxy vitamin D (25(OH)D) levels from University of Arizona Medical Center (UAMC) between April 1, 2011-March 31, 2012 and July 1, 2012-June 30, 2013. Total 25(OH)D levels and vitamin D regimens were collected along with data on respiratory cultures, pulmonary function, and hospitalizations. Data were analyzed by Student’s T-tests and chi square analyses. Results: A total of 62 patients were included in the study. Mean 25(OH)D levels did not significantly differ between the study periods (28.9±10.5 ng/mL pre-guideline and 27.0±9.1 ng/mL post-guideline, p=0.158). Cholecalciferol use increased post-guideline (57.1%) versus pre-guideline (75.8%, p=0.027). Post-guideline cholecalciferol doses increased to 2836.5±2669.4 international units [IU] daily compared to 1518.0±912.0 IU daily pre-guideline (p<0.001). Clinician adherence to dose titration recommendations resulted in significant 25(OH)D level elevations (28.3±8.9 ng/mL versus 24.7±9.0, p=0.047). Conclusions: The prescribing pattern of clinicians significantly changed to reflect vitamin D regimens suggested by CFF guidelines. This finding suggests that had sufficient time been allowed following guideline implementation, a significant difference in 25(OH)D levels would have resulted. Additional research is needed concerning the effect of the guidelines on vitamin D status, clinical outcomes, and comorbidities.
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Rogers, Geraint Berian. "Molecular analysis of respiratory infections in cystic fibrosis patients." Thesis, King's College London (University of London), 2004. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.407194.
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Butler, Sarah Louise. "Pulmonary colonisation of patients with cystic fibrosis by Burkholderia cepacia." Thesis, University of Edinburgh, 1994. http://hdl.handle.net/1842/27646.
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This thesis describes studies investigating the biological properties of B. cepacia, in particular those which may contribute to the organism's role as an opportunist pathogen and considers the host-bacterium interaction in CF including factors involved in early colonisation and the host immune response to B. cepacia colonisation. Emphasis is placed on studies of a highly transmissible or epidemic strain isolated in Edinburgh in 1989 and responsible for colonisation of CF patients in other regional CF centres. B. cepacia adhesion to respiratory mucin was measured in an ELISA-based mucin adherence assay. The majority of B. cepacia strains did not adhere to purified respiratory mucin. Interestingly, the epidemic strain exhibited the greatest degree of mucin adherence. Adhesion of B. cepacia to buccal epithelial cells studied by fluorescent labelling and flow cytometry also showed that the epidemic strain demonstrated greatest adhesion. The humoral immune response in CF patients colonised with B. cepacia was investigated by ELISA, incorporating B. cepacia LPS and by immunoblotting against LPS, flagella and outer membrane protein antigens. Elevated levels of specific anti-B. cepacia IgG, IgA and IgM were observed in patients chronically colonised by B. cepacia, especially in those patients colonised by the epidemic strain. Detection of anti-B. cepacia antibodies may aid in early diagnosis of B. cepacia colonisation, but as yet does not appear to have prognostic value. Evidence presented in this thesis indicates that B. cepacia persists in the CF respiratory tract despite a specific humoral immune response and causes bacteraemia despite being serum sensitive. These factors together with the intractability of B. cepacia to antimicrobial therapy and the close taxonomic relationship with the highly virulent Burkholderia pseudomallei, allows speculation that the association B. cepacia with CF may involve a stage of intracellular growth for survival.
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Meloff, Liann Rachel. "Assessment of eating disorder symptomatology in patients with cystic fibrosis." Thesis, National Library of Canada = Bibliothèque nationale du Canada, 2001. http://www.collectionscanada.ca/obj/s4/f2/dsk3/ftp05/NQ64876.pdf.
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Al-Baba, Sami. "Diagnosis and typing of psendomonas aeruginosa from cystic fibrosis patients." Thesis, Manchester Metropolitan University, 2011. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.533358.
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Kinirons, Martin J. "Oral and dental changes in patients suffering from cystic fibrosis." Thesis, Queen's University Belfast, 1987. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.317090.
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Nelson, J. W. "Pulmonary colonisation of patients with cystic fibrosis by Pseudomonas aeruginosa." Thesis, University of Edinburgh, 1991. http://hdl.handle.net/1842/19187.
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Chronic respiratory colonisation by the adaptable opportunistic pathogen Pseudomonas aeruginosa is a major debilitating feature of the inherited disease cystic fibrosis (CF). This thesis considers various aspects of the pathogenesis of P.aeruginosa in CF, including the serological response to bacterial colonisation, and possible factors involved in early colonisation. Anti-P.aeruginosa lipopolysaccharide (LPS) antibodies in sera, saliva and sputa from patients with CF were measured by enzyme-linked immunosorbent assay (ELISA) incorporating either a polyvalent pseudomonas smooth LPS extract vaccine, or P.aeruginosa core, rough LPS. Elevated levels of anti-LPS IgG antibodies in serum, and IgA antibodies in saliva and sputum were demonstrated in patients chronically colonised by P.aeruginosa. Low levels of serum anti-LPS IgG antibodies were detected in some patients intermittently colonised by P.aeruginosa, but not in non-P.aeruginosa colonised patients. Anti-LPS IgA antibodies were detected in some of both intermittently and non-colonised patients. Immunoblot analysis of serum IgG and sputum IgA antibodies to P.aeruginosa LPS revealed a response directed towards O-antigenic LPS in the initial stages of pulmonary colonisation with non-mucoid P.aeruginosa and a response towards common core LPS during subsequent chronic infection with mucoid P.aeruginosa. Flagellar preparations from P.aeruginosa strains were characterised and used in ELISA and immunoblot studies to detect anti-P.aeruginosa flagellar antibodies in sera, saliva and sputum. Serum anti-flagellar IgG antibodies were detected, particularly in those CF patients intermittently or chronically colonised by P.aeruginosa. Antibodies to both type -a and -b flagella were detected; in some patients a pronounced antibody response to only one of the flagellar types was evident. Anti-P.aeruginosa LPS monoclonal antibodies (MAbs) were produced for use in a sandwich ELISA for the detection of P.aeruginosa in respiratory secretions of patients with CF. LPS defective mutants expressing only common core LPS were used to immunise mice for preparation of MAbs. Antibodies were screened in ELISA and the antigenic component(s) of LPS recognised by the most cross-reactive MAbs was checked by immunoblotting. Five IgG MAbs were characterised and found to recognise the core component of P.aeruginosa LPS. Two of the MAbs were particularly reactive against core LPS from all O-antigenic serotypes of P.aeruginosa and were included in the sandwich ELISA for detection of P.aeruginosa LPS. A biotin-streptavidin amplification system was used to increase assay sensitivity. The sensitivity of the assay was 0.1 ng/ml P.aeruginosa LPS; the assay was able to detect P.aeruginosa LPS in the respiratory secretions from patients with CF.
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MacDougall, J. H. "A study of Pseudomanas aeruginosa from adult cystic fibrosis patients." Thesis, Imperial College London, 1987. http://hdl.handle.net/10044/1/47182.
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Nixon, Lisette Sheena. "Neutrophil function in patients with cystic fibrosis and chronic pulmonary infection." Thesis, Cardiff University, 2003. http://orca.cf.ac.uk/54082/.
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The thesis investigates reasons for a failure of neutrophils to clear pulmonary bacterial infection in cystic fibrosis (CF). Patients with CF experience bacterial colonization of the lungs associated with progressive lung injury and poor prognosis. Neutrophil responsiveness in vitro was determined in patients with CF at different clinical states, and compared to healthy subjects. Neutrophils from the patients were able to phagocytose and kill Pseudomonas aeruginosa effectively, but the presence of sputum sol reduced intracellular killing. Superoxide generation and elastase release in response to fMLP were shown to be reduced in neutrophils from patients with an exacerbation of respiratory symptoms. This was not observed when the cells were stimulated with PMA, which acts intracellularly, rather than through cell surface receptors. There was no difference in the down-regulation of cell surface L-selectin and up-regulation of CD11b in response to fMLP suggesting no alteration in number or function of the fMLP receptors. There was increased adherence to nylon columns by neutrophils from patients with CF. Infection resulted in a greater proportion of band neutrophils, and this correlated with the reduced superoxide generation and elastase release, although it was not possible to separate the band forms to prove this conclusively. Alterations in circulating lipid and fatty acid composition inpatients could potentially affect neutrophil membrane composition and fluidity and therefore signal processing or release of products. The reason for the observed reduced responsiveness appears to be multifactorial. Band cell number post receptor signalling and/or receptor desensitization may result in the observed reduced responsiveness, which returns towards healthy subject levels after treatment of an exacerbation, is likely to be the result of chronic infection.
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Sherrard, Laura Jayne. "Characterisation of antibiotic resistance in Prevotella isolates from cystic fibrosis patients." Thesis, Queen's University Belfast, 2013. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.602795.
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Antibiotic treatment of lung infection has been a major contributor to increased life expectancy amongst cystic fibrosis (CF) patients in recent years. Diverse polymicrobial communities including bacteria from the genus, Prevotella, are detected in respiratory samples from CF patients. However, the role of Prevotella in disease progression and the effect of high antibiotic consumption on resistance in this genus are unknown. The primary aim of this study was to determine and compare antimicrobial susceptibility and mechanisms of resistance in Prevotella spp. isolated from CF and non-CF patients. All isolates were susceptible to chloramphenicol, meropenem and piperacillin/tazobactam while metronidazole resistance was rare. In contrast, resistance to amoxicillin, ceftazidime and tetracycline was apparent. CF isolates were more resistant to azithromycin, clindamycin and co-amoxiclav compared to non-CF isolates. Azithromycin resistance was also associated with current prescription of the antibiotic in CF patients. More than 50% of isolates weref3-lactamase positive with an association between β-lactamase production and presence of cfxAlcfxA2. Extended spectrum f3-lactamase (ESf3L) production was detected in 69% of isolates tested and was associated with resistance to ceftazidime and amoxicillin. Isolates positive for ermF and tetQ were more resistant to azithromycin/clindamycin and doxycycline/tetracycline, respectively with ermF detected more commonly in the CF isolates. The nim-type gene was rarely detected but a metronidazole resistant subpopulation with a stable phenotype could be selected for in a nim-positive isolate following exposure to a subinhibitory concentration of the antibiotic in vitro. This study profiles in vitro antibiotic susceptibility of Prevotella spp. in CF and demonstrates that meropenem, piperacillin/tazobactam, chloramphenicol and metronidazole are likely to be the most effective antibiotics if treatment is indicated
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Cawood, Abbie Louise. "Vitamin A metabolism, in healthy adults and patients with cystic fibrosis." Thesis, University of Southampton, 2003. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.274439.
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Ho, Ling-Pei. "Endogenous nitric oxide production in airways of patients with cystic fibrosis." Thesis, University of Glasgow, 1999. http://theses.gla.ac.uk/38961/.
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Nitric oxide (NO) is a highly reactive molecule with physiological and pathological roles in the airways. This thesis first investigated the production of NO in the airways of cystic fibrosis patients compared to normal and asthmatic subjects after validating a method to measure this which excluded known confounding factors. The first finding was that exhaled NO, against expectation were not increased in cystic fibrosis patients, even during infective exacerbations. I hypothesised that this could be due to lack of detection of NO (secondary to removal of NO by reaction with other reactive molecules and/or impedance of diffusion of this gaseous molecule into the airway) or lack of production (secondary to decreased expression of nitric oxide synthase II (NOS II), the gene thought to be responsible for NO production within an inflammatory setting). The thesis thus explored these possibilities by investigating a) exhaled NO levels in patients with bronchiectasis, a group of patients with similar suppurative airway disease to cystic fibrosis b) breath condensate levels of hydrogen peroxide, another highly reactive volatile molecule in cystic fibrosis patients compared to normal subjects c) expression of NOS II in cystic fibrosis and nominal primary epithelial cells and epithelial cell lines at baseline and in response to pro inflammatory stimuli and d) nitrite levels in breath condensate of CF patients. In order to perform the above, methods for measuring hydrogen peroxide and nitrite in breath condensate were developed. The correlation between the levels of these molecules and lung function and circulating leucocytes were also determined. The thesis found that like exhaled gaseous NO, hydrogen peroxide levels were not elevated in exhaled air of patients with CF. However nitrite levels were increased and this correlated with the levels of circulating leucocytes. The expression of NOS II were normal in primary cystic fibrosis epithelial cells and epithelial cell lines in vitro. Therefore, it is concluded that exhaled NO is not helpful as a marker of airways inflammation in chronic suppurative airway diseases of cystic fibrosis and bronchiectasis. This is likely to be due to lack of detection rather than production of NO from the epithelium. Breath condensate nitrite levels may be a more useful tool for measuring airways inflammation in these conditions and further developments in this area are suggested.
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Jannetta, Evelyn Elena. "Qualitative study of cystic fibrosis (CF) patients' expectations of gene therapy." Thesis, University of Edinburgh, 2009. http://hdl.handle.net/1842/8745.
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Introduction: Gene therapy is currently being developed for people with cystic fibrosis (CF), a life-threatening condition for which there is no cure. The UK CF Gene Therapy Consortium are preparing for a multi-dose gene therapy trial of sufficient duration that clinical benefit may be seen. Aims: The current study aimed to explore the expectations and beliefs of cystic fibrosis (CF) patients involved in the preparatory phase of the gene therapy trial (the Run-in study), from which participants will be selected for the multi-dose actual gene therapy trial. Method: Twelve participants (six with mild and six with moderate CF) were interviewed using a semi-structured interview. Interviews were recorded, transcribed verbatim and then analysed using a Constructivist Grounded Theory approach. Results: Since entering the Run-in study, half of the patients had increased their expectations of gene therapy being an effective future treatment. Most of the participants hoped to derive clinical benefit from the trial itself though half were unsure of what to expect. Whilst half of the participants expressed the hope of a future cure for CF, the remainder saw gene therapy only in terms of an improved treatment. Participants used several strategies to manage their expectations including not thinking too far ahead and trusting the research team. Discussion: The findings indicate that participants in the Run-in trial are generally eager to be involved in the gene therapy trial and have developed a strong sense of trust in the research team conducting the trials. The levels of optimism expressed for personal benefit from trial were higher than those from earlier studies. Some of the positive expectations were unlikely to be met by the gene therapy trial and participants risk disappointment. However other patients participated with apparently realistic expectations and it seems likely that some patients would have participated even without prospect for personal benefit. Possible areas of psychological support are discussed e.g. a standard clinical interview for all those not accepted for the gene therapy trial; screening for anxiety pre-, during and post-participation.
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Blair, Caroline. "Dysfunction in the families of anorexia nervosa and cystic fibrosis patients." Thesis, University of Edinburgh, 1994. http://hdl.handle.net/1842/20071.
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The present study compared the families of 27 Anorexia Nervosa patients with the families of 29 Cystic Fibrosis (CF) patients and a third group of 31 well controls. The individual child in each household was aged between 14 and 24. Assessment was carried out at baseline and at an eight month follow up. Data was collected via three modalities: (1) Self report questionnaires (2) the Camberwell Family which was rated for Expressed Emotion (EE) and (3) Observation of a family problem solving task which was rated for evidence of Minuchin's "psychosomatic family" dimensions. The self report measures of family functioning for the most part did not distinguish between the 3 groups. There were no differences between groups in levels of EE criticism. AN and CF households showed more emotional over-involvement (EOI) than did the well households and EOI correlated with severity of illness suggesting that this family characteristic is an illness related phenomenon. Mothers of both CF and AN patients showed more emotional disturbance than did mothers of well young people. There were more enmeshed and overprotective households in the AN group than in the CF and well groups. AN families were also less successful problem solvers than the other two groups giving partial support to Minuchin's conceptualization. The robust psychological health of CF patients found in previous research was replicated here and was in marked contract to the AN sufferers. No conclusive relationship was found between change in health status and change in household climate between baseline and follow up. This could in part be explained by design limitations. AN families were more likely to feel that they had been blamed for their child's illness than were CF families. A brief qualitative account of the AN and CF families in the study showed that they had had very different experiences of getting appropriate support.
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Sharma, Poonam. "Genome analysis of multidrug resistant bacteria from patients with cystic fibrosis." Thesis, Aix-Marseille, 2013. http://www.theses.fr/2013AIXM5096.
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La mucoviscidose est une maladie génétique autosomique causée par une mutation dans le gène CFTR (Cystic Fibrosis Transmembrane Conductance Regulator). Mon travail s’est décomposé en deux parties principales : d’une part j’ai réalisé une revue de la littérature sur l’analyse des génomes bactériens isolés de patients mucoviscidosiques comparativement aux génomes des mêmes espèces isolées dans d’autrescontextes et d’autre part j’ai analysé les génomes de trois espèces bactériennes (Microbacterium yannicii, Chryseobacterium oranimense et Haemophilus parahaemolyticus). L’analyse exhaustive des génomes bactériens issus de patients atteints de mucoviscidose a révélé une extraordinaire évolution de ces génomes en fonction du temps et des traitements reçus par ces patients qui témoigne de la capacité qu’ont ces bactéries à s’adapter à leur écosystème notamment par l’acquisition de nouveaux gènes par transfert latéral de gènes. Ce travail montre l’extraordinaire plasticité des génomes bactériens dans un milieu donné et à ce titre le poumon de patients atteints de mucoviscidose représente un modèle unique pour comprendre l’évolution des génomes bactériens. De plus, notre travail a permis d’identifier leurs mécanismes moléculaires de résistance aux antibiotiques. Les travaux à venir sur l’étude des métagénomes de prélèvements chez ces patients pourrait permettre de répondre à ces questions dans le futur. La découverte de nouvelles espèces et / ou émergentes va nous permettre d’avoir une image plus complète de la mucoviscidose qui pourrait conduire à une meilleure connaissance de la maladie et donc à une meilleure prise en charge thérapeutiqueCystic fibrosis is an autosomal genetic disorder caused by a mutation in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene. Pulmonary infection is the major problem faced by patients with cystic fibrosis. My work is divided into two main parts: first I made a review of the literature on the analysis of bacterial genomes isolated from CF patients compared to the genomes of the same species isolated in autrescontextes and other part I analyzed the genomes of three species of bacteria (Microbacterium yannicii, Chryseobacterium oranimense and Haemophilus parahaemolyticus). The comprehensive analysis of bacterial genomes from cystic fibrosis patients revealed an extraordinary evolution of these genomes with time and treatment received by these patients reflects the ability of these bacteria to adapt to their particular ecosystem the acquisition of new genes by lateral gene transfer. This work shows the extraordinary plasticity of bacterial genomes in a given environment and as the lungs of patients with cystic fibrosis represents a unique model for understanding the evolution of bacterial genomes. In addition, our work has identified their molecular mechanisms of resistance to antibiotics. Future work on the study of metagenomes sampling in these patients could help to answer these questions in the future. The discovery of new species and / or emerging will allow us to have a more complete picture of cystic fibrosis which could lead to a better understanding of the disease and thus a better therapeutic management
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Shur, Jagdeep. "Formulated muco-regulatory agents in the airways of patients with cystic fibrosis." Thesis, University of Portsmouth, 2006. https://researchportal.port.ac.uk/portal/en/theses/formulated-mucoregulatory-agents-in-the-airways-of-patients-with-cystic-fibrosis(f03c4296-92e0-45c6-b9f9-1c160bb43427).html.
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Cystic fibrosis (CF) is a lethal hereditary disorder characterised by the unregulated production of visoelastic mucus, which poses a barrier to the effective delivery of drugs to the CF lung. The barrier properties of CF sputum are thought to be due to the high DNA and actin content that together with mucins form a tangled network, held together by electrostatic charges, hydrogen bonds and van der Waals forces. CF sputum affects the deposition pattern of aerosols on the epithelial surface of the upper respiratory tract and thereby, preventing drug diffusion. To improve drug delivery in CF it is vital to reduce or remove this barrier. Recent studies have suggested that negatively charges species such as unfractionated heparin (UFH). a member of the glycosaminoglycan family, may posess mucoactive properties and shows promise as a potential therapy for treating patients with chronic obstructive pulmonmary disease (COPD) and CF. This study investigates the potential mucoactive properties of UFH, and describes the development of dry powder inhaler (DPI) formulations of UFH that can be administered to CF patients to treat airway obstruction. In this study, a novel barrier function assay was employed to investigate the diffusion of the corticosteroid dexamethasone through CF sputum and calf-thymus DNA. In addition, atomic force microscopy (AFM) and confocal laser scanning microscopy (CLSM) were used to investigate the ultrastructure of both whole CF sputum and calf-thymus DNA. The effect of charged oligsaccharides and glycosaminoglycans, as potential mucoactive agents, on the barrier and structural properties of CF sputum were also investigated. The feasibility of producing dry powders of UFH, which are suitable for inhalation from DPIs, using spray drying, was investigated. The effect of these powders on CF sputum rheology was also studied. Studies investigating the barrier properties of CF sputum, showed that sputum significantly (p < 0.05) reduced the diffusion of dexamethasone by 90% in comparison to diffusion through buffer. Following treatment with 1.0 and 10mg/ml UFH, the diffusion of dexamethasome through CF sputum was significantly (p < 0.05) increased. CLSM analysis of untreated CF sputum showed the pressence of highly polymerised fibrous bundles of DNA, which were observed to be interacting with F-actin present in the sputum. The fibrous bundles of DNA present in CF sputum were dissipated following treatment with UFH. Therefore, it was hypothesised that UFH may be exerting its mucoactive properties by targeting the interactions between DNA and other components present in CF sputum. Results suggest that the DNA component of CF sputum may also be responsible for the barrier properties of CF sputum. Indeed, it was observed that calf-thymus DNA (5mg/ml) significantly (p < 0.05) reduced the diffusion of dexamethasone. Treatment of calf-thymus DNA (5 mg/ml) with 10 mg/ml UFH, significantly (p < 0.05) increased the diffusion of dexamethasone. AFM analysis of calf-thymus DNA showed the presence of DNA networks that exhibited pore sizes of 0.38 ± 0.03 μm. Treatment of DNA (5 mg/ml) with 0.01 mg/ml UFH significantly (p < 0.05) increased the pore size of the DNA network to 0.78 ± 0.07 μm. Hence, these data suggest that UFH may possess mucoactive properties. Spray drying was successfully used to produce spherical micronised UFH particles, which were suitable for inhalation. The material, however, was cohesive (forming agglomerates) and hygroscopic, which compomised its physical stability. These properties may render a UFH DPI drug delivery system ineffective, as insufficient fine material would be liberated that would penetrate the deep lung. Co-spray drying UFH with L-leucine significantly reduced the cohesive properties of UFH and improved it physical stability characteristics. Rapid formulation screening studies using a conventional DPI suggested that co-spray dried UFH/leucine formulations are easily dispersed from the inhaler, with the liberation of a large proportion of fine material. In addition, both spray dried UFH and co-spray UFH/leucine significantly (p < 0.05) reduced the viscoelasticity of CF sputum. This work suggest that UFH possess mucoactive propoerties, and that the use of L-leucine may aid the development of dry powder inhlaer formulations of UFH, which can be administered to CF patients to treat airway obstruction.
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Sayoc, Emmanuel Castillo. "Use of hydro-acoustics in the treatment of patients with cystic fibrosis." Thesis, Georgia Institute of Technology, 1999. http://hdl.handle.net/1853/16940.
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Maeda, Yasunori. "Isolation and characterisation of viridans group streptococci from patients with cystic fibrosis." Thesis, University of Ulster, 2010. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.554337.
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Viridans group streptococci (VGS) are commensal bacteria inhabiting the human oral-nasopharynx, gastrointestinal and urogenital tracts. Recent studies have revealed various aspects of VGS, including possible pathogenesis in patients with cystic fibrosis (CF). This thesis aims to investigate molecular identification techniques for the VGS thus allowing the population structure of VGS to be determined in adult patients with cystic fibrosis, as well as their molecular characterisation in terms of fluoroquinolone and macrolide resistance. The thesis consists of 10 chapters, including a literature review and eight experimental chapters and general discussion. Chapter 1 provides an up-to-date review of the current literature involving the identification and characterisation of VGS organisms. The first experimental theme is the development of a molecular technique for the reliable speciation of members of the VGS into distinct species, as well as an exploration of other novel molecular markers, including the development of a novel PCR assay which uses clustered regulatory interspaced short palindromic repeats (CRlSPRs)-like sequence. Having established methods to identify these organisms, these techniques formed the foundation for a study describing the population structure of VGS in adults patients with CF. Possible pathogenic mechanisms and molecular epidemiology of the resulting VGS populations were examined, as well as the frequency of antibiotic resistance in VGS organisms. Emergence of antibiotic resistance in VGS is major concern as seen in other pathogenic bacteria. Antibiotic resistance in VGS was further characterised in terms of its specific.
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Enright, Stephanie. "The efficacy of inspiratory muscle training in adult patients with cystic fibrosis." Thesis, University of Salford, 2001. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.366006.
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Taylor, Rowena Frances Halstead. "Microbiological aspects of infection with 'Pseudomonas aeruginosa' in patients with cystic fibrosis." Thesis, University College London (University of London), 1996. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.321609.
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Ashish, Abdul. "Impact of Pseudomonas aeruginosa Liverpool epidemic strain (LES) on cystic fibrosis patients." Thesis, University of Liverpool, 2015. http://livrepository.liverpool.ac.uk/2026659/.
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Cystic Fibrosis (CF) is the commonest life limiting inherited disease illness in the western world. Over the last few decades there have been many advances in the diagnosis and management of this condition. Patients born with the disease now are living into their fourth decade, which is a statement to the progress made over time. Along with the progress there have been new challenges in the world of CF. In the last two decades there have been several studies reporting the presence of transmissible Pseudomonas Aeruginosa (Psa) strains in CF clinics worldwide. The first one to be reported in UK Liverpool paediatric clinic was later identified as the Liverpool Epidemic Strain (LES). Previous studies have demonstrated chronic infection with LES can result in accelerated fall in lung function, increased hospitalisation and antibiotic requirements. This thesis looks at the effect of chronic infection of adults with CF and its implications on healthcare institutions caring for such patients. In particular on an individual level i investigated the health related quality of life associated with patients chronically infected with LES and compared it those with unique or no Psa strains. I have demonstrated that chronic infection with LES strain significantly worsens health related quality of life compared to those with unique or no Psa strains. Patients infected by transmissible Psa strains had worse physical functioning, respiratory symptoms, treatment burden, vitality, role, health perception and emotion than those with unique Psa strains (p < 0.01), and significantly poorer physical functioning, respiratory symptoms, treatment burden, body image, weight, role, and emotion than those without any Psa infection (p < 0.05). To understand the susceptibility of LES strains to common anti-pseudomonal antibiotics I studied the antibiograms of patients infected with LES strain over a 5-year period and compared the change in susceptibility to those infected with unique Psa strain. LES exhibited significantly more resistant isolates in 2004 (p < 0.0001). There was an increase in antibiotic resistance in both LES and other Psa strains over time (p < 0.001). Cox proportional hazards analysis of both unmatched (n=125) and matched (n=56) patients in 2004 revealed that LES infected patients were more likely to develop antibiotic resistant isolates over time (hazard ratio 8.1, p < 0.001). Fewer LES isolates were classed as fully sensitive in both matched and unmatched groups at the end of study period (p < 0.001). I then looked at the phenotypic characteristics exhibited by LES strain in comparison with unique Psa strain during an infective episode to elucidate whether it is a specific character of LES assisting to survive in harsh CF environment. We analyzed sets of 40 sputum samples isolates from five CF patients each chronically infected with a different non-LES strain of P. aeruginosa. For each sample (two per patient), diversity was assessed by characterising nine phenotypic traits. All P. aeruginosa populations were highly diverse. The majority of phenotypic variation found was due to within-sample variation. I demonstrated that maintenance of diverse populations in the CF lung is a general feature of P. aeruginosa infections rather than a unique characteristic of LES. To assess the healthcare resource implication on institutions caring for such patients we carried out an economic analyses of healthcare utilisation of patients chronically infected with LES to those with unique Psa strain over a four-year period. Ascertainable costs were correlated in these two groups of patients. The mean cost per patient per year was higher for LES patients for inpatient care (£4393.37 v £1817, p=0.0006), outpatient attendance (£3764 v £2515.91, p=0.0035) and also hospital antibiotic therapy (£980 v £ 505, p=0.001). Regular prescription costs were similar in both groups. Overall, the healthcare cost of caring for an adult CF patient with LES chronic infection was significantly more (1.6 times) than that for a matched patient with unique Psa strain chronic infection. Finally, I looked the effects of segregation policies instituted at Liverpool adult Cf centre in limiting cross infection. Regular genotypic surveillance of sputum samples of all patients was instituted to monitor cross infection rates. This study elucidated the cross infection policies over a 7-year period and looked at the yearly number of patients with LES and other strains. There was a decline in the proportion of patients with LES (71% to 53%) and an increase in those with unique strains (23% to 31%) and without Psa infection (6% to 17%) over the study period. Areas of potential future research based on this thesis are also outlined.
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Wheatley, Courtney M. "Endogenous and Exogenous Regulation of Exhaled Ions in Patients with Cystic Fibrosis." Diss., The University of Arizona, 2013. http://hdl.handle.net/10150/293489.
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Exercise has become a vital component of the therapy regimen prescribed to cystic fibrosis (CF) patients due to its systemic benefits, such as increased sputum expectoration, attenuation of the expected 2-3% annual decline in pulmonary function, and extended life expectancy. However, exercise still is not viewed as being as beneficial as pharmacological treatments by many CF patients and can be intimidating. My aims in this study were two-fold; first, to determine the ideal exercise intensity for individuals with CF; and second, to determine if exercise at this ideal intensity could provide improvements in ion regulation in the lungs, which was measured using exhaled breath condensate (EBC) collection and nasal potential difference (NPD), that were comparable to one of their standard pharmacological therapies, albuterol. I hypothesized that with moderate intensity exercise, Na⁺ absorption would decrease from baseline due to Na⁺ channel inhibition, rather than increase or remain unchanged, as was expected with albuterol, and cause an even greater increase Cl- secretion compared to albuterol due to activation of both CF-dependent and independent Cl- efflux with exercise. CF (n=14) and healthy (n=16) subjects completed three visits, a baseline screening and two treatment visits. I collected EBC at baseline, 30- and 60-minutes post-albuterol administration on one visit, and at baseline and during three separate 15 min exercise bouts at low, moderate and high intensity on the other visit. Following the EBC collection, NPD was performed at 30- and 80-minutes post albuterol or following moderate and high intensity exercise. We also measured spirometry and diffusing capacity of the lungs for nitric oxide (DLNO) during each visit at the various time points. In CF subjects, moderate intensity exercise resulted in greater improvements in DLNO (39 ± 29vs.15 ± 22% change from baseline, exercise vs. albuterol respectively), similar levels of bronchodilation compared to 60-minutes post-albuterol administration, no change in Na⁺ absorption, and a four-fold increase in Cl- secretion. Our results suggest that moderate intensity exercise is the best dose for CF patients, and can provide comparable changes as its pharmacological counterpart albuterol, when compared over a short term duration.
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Coughlan, Mary Louise. "Comparison of cardiac output determinants in response to progressive upright and supine exercise in cystic fibrosis patients." Thesis, McGill University, 1989. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=59255.
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This study was designed to characterize the cardiac output (Q$ sb{ rm c}$) response to progressive submaximal upright (U) exercise in CF patients. Secondly, the Q$ sb{ rm c}$ adjustments were compared to those of similar supine (S) exercise, in an attempt to assess myocardial accommodation to the enhanced ventricular preload in the S posture. Q$ sb{ rm c}$ generally increased with exercise intensity in both U and S positions, although gr.IV plateaued at 50% VO$ sb2$max (S). Maximal stroke volume index (SI) was achieved at 50% VO$ sb2$max (U) in all groups, except gr.IV and at 30% VO$ sb2$max (S) in all groups. The change from U to S posture resulted in a significant (p $ le$.05) increase in SI at rest and for every submaximal exercise in gr.I, but not in CF patients, independent of disease severity eg(Rest:gr.I:27 $ pm$ 7(U) vs 39 $ pm$ 8(S); gr.II:24 $ pm$ 5vs28 $ pm$ 10; gr.III:18 $ pm$ 4 vs 22 $ pm$ 5; gr.IV:17 $ pm$ 4 vs 20 $ pm$ 6 ml/bt/m$ sp2$). These observations suggest a limitation in ventricular volume accommodation in CF patients which becomes apparent under the S exercise conditions.
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Syrmis, Melanie Wanda. "Respiratory disease in patients with cystic fibrosis : role and pathogenesis of Pseudomonas aeruginosa /." [St. Lucia, Qld.], 2005. http://www.library.uq.edu.au/pdfserve.php?image=thesisabs/absthe19064.pdf.
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Drury, Donna. "Vitamin D and K status and bone health in pediatric cystic fibrosis patients." Thesis, McGill University, 2006. http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=101116.
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The objective of this study was to investigate the extent to which vitamin D and K are associated with bone health in pediatric cystic fibrosis (CF) patients. We hypothesized that: (1) the prevalence of vitamin D and K deficiencies would be high despite routine vitamin therapy, (2) bone health would be reduced and (3) vitamin K and D status would be associated with bone health.Our results showed poor bone mineral mass in these CF children despite mild disease and good nutritional status. Neither vitamin K nor D was a predictor of bone health but weight and height Z-scores, fat-free mass, physical activity and lung function were all consistent predictors.
These results indicate that nutritional status as well as physical activity are key determinants of bone health in CF children and offer a unique opportunity in the prevention of CF-related bone disease. Further vitamin intervention research needs to be done in this population.
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O'Connor, B. S. "The use of Azithromycin in Cystic Fibrosis Patients not infected with Pseudomonas Aeruginosa." Thesis, Queen's University Belfast, 2010. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.527879.
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Micallef, Christianne. "Developing a model system for 'Staphylococcus aureus' respiratory infection in cystic fibrosis patients." Thesis, Kingston University, 2008. http://eprints.kingston.ac.uk/20405/.
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For the first time, an in vitro cystic fibrosis (CF) artificial sputum model (ASM) was found to support the growth and survival of a clinical epidemic strain of meticillin-resistant Staphylococcus aureus (EMRSAl6-252). Specific components, which included mucin. DNA and others, were removed from ASM and the physiological impact of this was fully explored using viable counts and light microscopy. As CF patients are known to develop cystic fibrosis-related diabetes or CFRD, glucose was added to ASM (GASM), to explore the physiological impact of glucose on the growth and survival MRSA252. Total RNA was extracted from the corresponding log phases of MRSA252 grown in brain heart infusion (BHI) as a laboratory control, as well as ASM and GASM. RNA was extracted in order to conduct microarray analysis. MRSA252 DNA was used as a control. RNA (from the samples) was labelled with Cy5 and control DNA was labelled with Cy3. Once labelled and amplified, the Cy5/Cy3 mixture was then purified and hybridised onto an array containing seven sequenced S. aureus : genomes (N315, Mu50, MW2, MRSA252, MSSA476, COL and NCTC8325).
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Doud, Melissa S. "A Multi-Faceted Diagnostic Approach to Lung Infections in Patients with Cystic Fibrosis." FIU Digital Commons, 2010. http://digitalcommons.fiu.edu/etd/166.
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One in 3,000 people in the US are born with cystic fibrosis (CF), a genetic disorder affecting the reproductive system, pancreas, and lungs. Lung disease caused by chronic bacterial and fungal infections is the leading cause of morbidity and mortality in CF. Identities of the microbes are traditionally determined by culturing followed by phenotypic and biochemical assays. It was first thought that the bacterial infections were caused by a select handful of bacteria such as S. aureus, H. influenzae, B. cenocepacia, and P. aeruginosa. With the advent of PCR and molecular techniques, the polymicrobial nature of the CF lung became evident. The CF lung contains numerous bacteria and the communities are diverse and unique to each patient. The total complexity of the bacterial infections is still being determined. In addition, only a few members of the fungal communities have been identified. Much of the fungal community composition is still a mystery. This dissertation addresses this gap in knowledge. A snap shot of CF sputa bacterial community was obtained using the length heterogeneity-PCR community profiling technique. The profiles show that south Florida CF patients have a unique, diverse, and dynamic bacterial community which changes over time. The identities of the bacteria and fungi present were determined using the state-of-the-art 454 sequencing. Sequencing results show that the CF lung microbiome contains commonly cultured pathogenic bacteria, organisms considered a part of the healthy core biome, and novel organisms. Understanding the dynamic changes of these identified microbes will ultimately lead to better therapeutical interventions. Early detection is key in reducing the lung damage caused by chronic infections. Thus, there is a need for accurate and sensitive diagnostic tests. This issue was addressed by designing a bacterial diagnostic tool targeted towards CF pathogens using SPR. By identifying the organisms associated with the CF lung and understanding their community interactions, patients can receive better treatment and live longer.
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Hill, Emily M. "Emerging Pathogens in Cystic Fibrosis Patients at Virginia Commonwealth University Medical Center (VCUMC)." VCU Scholars Compass, 2016. http://scholarscompass.vcu.edu/etd/4606.
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Cystic fibrosis (CF) is an autosomal recessive disorder affecting 70,000 individuals worldwide. This disease is characterized by the buildup of mucus in the airways leading to chronic lung infections resulting in pulmonary failure and death in 95% of CF patients. Routine surveillance of CF pathogens using traditional microbiology culture guides management and treatment of CF patients. Molecular profiling studies have revealed emerging pathogens that may play a role in CF lung disease by either directly causing infection or upregulating the virulence factors of classic CF pathogens, such as P. aeruginosa; however, routine CF culture protocols have not been modified to detect these organisms. The goal of this study was to expand the data relevant to the use of microbiology cultures for the management and treatment of CF patients at Virginia Commonwealth University Medical Center (VCUMC) by directly selecting for emerging CF pathogens in culture. This was accomplished by developing,optimizing, and implementing an agar to select for colistin-resistant non-fermenting Gram- negative rods (NF GNRS). In addition, McKay agar and anaerobic media were utilized to recover members of the Streptococcus anginosus group (SAG) and anaerobes in CF respiratory samples. The prevalences of SAG, anaerobes, and colistin-resistant NF GNRs recovered on study media from 75 adult and pediatric CF patients at VCUMC were 17.33%, 41.33%, and 4% respectively. Approximately 62% of patients culture-positive for SAG were also infected with P. aeruginosa and 53.8% of SAG recovered in culture were from CF patients experiencing PE. These findings further support the claim that interspecies interactions among emerging and classic CF pathogens may result in periods of clinical instability or PE. Twenty-eight of the 75 patients were culture-positive for Veillonella species, with the majority of samples collected during a period of surveillance. Four colistin-resistant NF GNRs were isolated on the study media alone. The selective nature of the study media prevented the mixed respiratory flora and classic CF pathogens from overgrowing and obscuring the growth of these colistin-resistant NF GNRs. The presence and role of emerging pathogens in the CF patient population at VCUMC warrants further investigation; therefore, the routine culture protocol needs to be revised to recover and select for those organisms thought to play a role in PE and lung function decline.
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Matier, Thomas Bailie. "Inflammatory potential of Prevotella species isolated from the airways of cystic fibrosis patients." Thesis, Queen's University Belfast, 2016. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.707827.
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The role of Prevotella, a Gram-negative anaerobe, frequently detected in the airways of patients with cystic fibrosis (CF), is not well understood. The aims of this study were to determine the lipopolysaccharide (LPS) chemotype of Prevotella spp. isolated from CF and non-CF patients and to characterise both the structure and inflammatory potential of Prevotella spp. LPS. Also, to ascertain the extent of capsular polysaccharide (CPS) production by these isolates and to investigate the role of Prevotella spp. CPS within the CF airways. SDS-PAGE demonstrated LPS consisting solely of a lipid A-core oligosaccharide in 33/36 (92%) CF isolates and 9/18 (50%) non-CF isolates. Structural elucidation of LPS revealed a highly negatively charged heptasaccharide containing hexoses, with the first two sugars of the core region highly phosphorylated. The lipid A moiety possessed the typical structure of the genus Prevotella lipid A and was also highly phosphorylated. The biological properties of P. denticola and P. intermedia LPS were examined in THP-1 cells over a 24-h period. The THP-1 cell peak response to Prevotella, E. coli, and P. aeruginosa LPS was within the same order of magnitude for IL-8, IL-6, and IL-1 (3. However, Prevotella LPS induced earlier up-regulation of gene expression and a more rapid pro-inflammatory response in THP-1 cells. Prevotella spp. isolates were screened for capsule production using eosin staining and brightfield microscopy. The majority of Prevotella spp. isolates (43/49; 87.8 %) were capsulated, with capsulated isolates significantly more resistant to complement mediated killing. Purified Prevotella spp. CPS induced a pro-inflammatory response in THP-1 monocytes, and also caused a significant decrease in the susceptibility of both Prevotella spp. and P. aeruginosa isolates to the cationic antimicrobial peptide polymyxin B. These findings indicate that Prevotella spp. isolates may have the potential to contribute to chronic inflammatory processes within the airways patients with CF.
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Terrell, Andrew S. "The effect of hydro-acoustic therapy on sputum production in patients with cystic fibrosis." Thesis, Georgia Institute of Technology, 2000. http://hdl.handle.net/1853/17076.
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Williamson, Emma Charlotte Mary. "Molecular approaches to fungal infections in immunocompromised patients." Thesis, University of Bristol, 2001. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.369153.
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Saynor, Zoe Louise. "Assessment and interpretation of aerobic exercise (dys)function in paediatric patients with cystic fibrosis." Thesis, University of Exeter, 2016. http://hdl.handle.net/10871/21875.
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The purpose of this thesis was to extend our understanding of the assessment and interpretation of aerobic exercise function of paediatric patients with cystic fibrosis (CF). The first investigation sought to establish (1) the validity of traditional criteria to verify maximal oxygen (V ̇O2max) during a maximal cardiopulmonary exercise test (CPET); and (2) the utility of supramaximal verification (Smax) to confirm V ̇O2max. Traditional criteria significantly underreported V ̇O2max, whilst Smax was shown to provide a valid measurement in this patient group. The reproducibility of this CPET protocol, over the short- (48 h) and medium- (4-6 weeks) term, was then established in study two. V ̇O2max was repeatedly determined with no learning effect over 48 h (typical error (TE): ∆150 mL; ∆9.3%) and 4-6 weeks (TE: ∆160 mL; ∆13.3%). Supplementary maximal and submaximal CPET parameters should be incorporated for a comprehensive evaluation of a patient, however they are characterised by greater variability over time. The influence of mild-to-moderate CF on aerobic exercise function and the matching of muscle O2 delivery-to-O2 utilisation during ramp incremental exercise to exhaustion were then examined in study three. Aerobic function was impaired in CF, indicated by very likely reduced fat-free mass normalised V ̇O2max (mean difference, ±90% CI: -7.9 mL∙kg-1∙min-1, ±6.1), very likely lower V ̇O2 gain (-1.44 mL∙min-1∙W-1, ±1.12) and a likely slower V ̇O2 mean response time (MRT) (11 s, ±13). Arterial oxygen saturation was lower in CF, supporting the notion that centrally mediated O2 delivery may be impaired during ramp incremental exercise. Although a faster rate of fractional O2 extraction would be expected in the face of reduced O2 delivery, this was not observed, suggesting additional impairment in O2 extraction and utilisation at the periphery in CF. The fourth study then demonstrated the clinical utility of CPET to assess the response to 12 weeks treatment with Ivacaftor, using a case-based design. Whilst one patient with relatively mild disease demonstrated no meaningful change in V ̇O2max, the second demonstrated a 30% improvement in V ̇O2max, due to increased O2 delivery and extraction. Furthermore, changes in aerobic function were detected earlier than spirometric indices of pulmonary function. This study demonstrated that CPET represents an important and comprehensive clinical assessment tool and its use as an outcome measure in the functional assessment of patients is encouraged. Study five investigated the V ̇O2 kinetics in this patient group. During moderate intensity cycling, the phase II V ̇O2 time constant (τ) (p = 0.84, effect size (ES) = 0.11) and overall MRT (p = 0.52, ES=0.33) were not slower in CF. However, both were slowed during very heavy intensity cycling (p = 0.02, ES = 1.28 and p = 0.01, ES = 1.40, respectively) in CF. Cardiac output and muscle deoxygenation dynamics were unaltered in CF, however, the arterial-venous O2 content difference (C(a-v ̅)O2) was reduced (p=0.03) during VH and ∆C(a-v ̅)O2 correlated with the phase II τ (r= -0.85; p=0.02) and MRT (r = -0.79; p=0.03) in CF. This study showed that impaired oxidative muscle metabolism in this group is exercise intensity-dependent and mechanistically linked to an intrinsic intramuscular impairment, which limits O2 extraction and utilisation. In conclusion, this thesis has provided guidelines for a valid and reproducible CPET protocol for children and adolescents with mild-to-moderate CF, demonstrated the utility of CPET as clinical outcome measure and furthered our understanding of the factors responsible for impaired aerobic exercise function in this patient group.
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Whitaker, Paul. "Investigation into non-immediate hypersensitivity reactions to intravenous antibiotics in patients with cystic fibrosis." Thesis, University of Leeds, 2012. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.613425.
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Antibiotic hypersensitivity represents a major clinical challenge in patients with cystic fibrosis (CF). Many patients have significant restrictions in antibiotic choice as a result of multiple previous hypersensitivity reactions; this often leads to suboptimal treatment. The majority of reactions are non-immediate and in similar cohorts of non-CF patients it has been demonstrated that they are T cell mediated. In this study we aimed to investigate the mechanism of hypersensitivity in a cohort of patients with CF and established hypersensitivity. As demonstrated in recent studies skin testing had low sensitivity in our cohort of patients. Only 13% of patients developed positive intradermal tests to piperacillin; no positives were seen with other beta-Iactams. In contrast, in-vitro Iymphocyte proliferation was seen in 68% of patients with piperacillin hypersensitivity. This is the first time drug specific Iymphocytes have been identified in patients with CF and supports the clinical viewpoint that the non-immediate reactions seen are T-cell mediated. Positive proliferation was also seen in patients with colistin hypersensitivity, including patients with neurological symptoms such as headache. Interestingly, the group of patients with neurological symptoms secondary to colistin also had positive drug specific IgG antibodies further supporting an immune mechanism. Whilst desensitisation is performed with some success at present it is not known whether any immune modulation takes place. The piperacillin model provides us with a useful tool to characterise the mechanisms of drug hypersensitivity and desensitisation. Prospective studies are needed to assess how drug sensitivity develops and whether clinical practice could be modified to reduce the risk.
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Dowey, Le Roy C. "The influence of β-carotene on the immune function in patients with cystic fibrosis." Thesis, University of Ulster, 2002. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.398969.
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Skrentny, Thomas, and Brittany Traylor. "Influence of Genetic Variation of the β2 Adrenergic Receptor in Patients with Cystic Fibrosis." The University of Arizona, 2010. http://hdl.handle.net/10150/623791.
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Class of 2010 AbstractOBJECTIVES: Cystic fibrosis (CF) is a disease that adversely affects the lung resulting in a reduction of lung diffusion. Stimulation of the β2 adrenergic receptors results in mucocilliary clearance, and therefore, lung diffusion. We sought to determine the influence of an inhaled β-‐agonist on the diffusing capacity of the lungs for carbon monoxide (DLCO), alveolar-‐capillary membrane conductance (DM), pulmonary capillary blood volume (Vc), and peripheral oxygen saturation (SaO2) in subjects with CF and compare the data to matched healthy subjects. METHODS: To determine this we recruited 20 healthy subjects and 18 subjects with CF (age=23±7 vs. 24±4years, ht=168±8 vs. 174±12cm. wt=64±16 vs. 70±13kg, BMI= 23±4 vs. 23±3kg/m2, FEV1= 72±27 vs. 92±12%pred., VO2peak = 45±25 vs. 99±24%pred., P<0.05 for FEV1 and VO2peak, mean±SD) for the study and measured DLCO, DM, Vc and SaO2 before and 30, 60, and 90 minutes following the administration of inhaled albuterol. RESULTS: Within the healthy subjects, there were no differences in DLCO, DM, Vc, DM/Vc at baseline or in response to albuterol according to genetic variation of the ADRB2 at amino acid 27. Within the CF group, the Glu27Glu/Gln27Glu group had higher DM/Vc when compared to the Gln27Gln group at baseline. Both genotype groups had a significant decline in Vc and a significant improvement in DM/Vc and SaO2 in response to albuterol, but not in DLCO or DM. CONCLUSIONS: These results suggest that there are differences in lung diffusion and peripheral SaO2 according to genetic variants of the ADRB2 at position 27 and could play a potential role in treatment options.
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Jones, Samantha. "Experiences of healthcare professionals and patients in paediatric cystic fibrosis : making and breaking bonds." Thesis, Bangor University, 2016. https://research.bangor.ac.uk/portal/en/theses/experiences-of-healthcare-professionals-and-patients-in-paediatric-cystic-fibrosis-making-and-breaking-bonds(6c218116-d52d-4596-bef5-e760caa854e9).html.
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This thesis explores the experience of Cystic Fibrosis (CF) in young people and healthcare professionals across three papers. Firstly, a systematic literature review explores the experiences of peer support for young people with CF, including peer support with CF peers, non-CF peers, impact of segregation, diagnosis disclosure, need for and purpose of peer support and parental and peer relationships. The need for social support in the lives of young people with CF was highlighted. The findings emphasised the need to continue addressing social needs of youth with CF, particularly finding ways to reduce the risk of cross-infection if youth choose to pursue friendships with CF peers. Limitations to the evidence base prevented reciprocal associations, bi-directional relationships and changes over time to be examined. Future research needs to consider the paucity of literature on peer support for young people with CF and potential avenues for future research are discussed. The second paper presents findings from an empirical study, qualitatively exploring the lived experiences of healthcare professionals (HCPs) working with children and adolescents with CF and the transition to adult services. This study was undertaken according to the principles of interpretative phenomenological analysis (IPA), with semi-structured interviews conducted with seven participants. Three superordinate themes emerged from the data, all dynamically intertwined and represent the interplay between both professional and (inter)-personal dynamics within CF care. Implications for clinical practice and future research are discussed. The third paper discusses implications for theory and clinical practice emerging from the first two papers. Discussion emphasises how this study examined an under-represented area and expands the opportunity to bring HCPs’ experiences and social needs of young people with CF to the foreground. This paper concludes with personal reflections on the research process and outcomes.
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Henriksson, Gert. "Clinical, immunological and olfactory aspects of sinusitis and nasal polyposis : with special reference to patients with cystic fibrosis /." Stockholm, 2004. http://diss.kib.ki.se/2004/91-7140-151-2/.
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Goldenberg, Rachel Brager. "Singing and Cystic Fibrosis| A collective case study on the effects of private voice lessons on the pulmonary function and quality of life of adult Cystic Fibrosis patients." Thesis, Shenandoah University, 2016. http://pqdtopen.proquest.com/#viewpdf?dispub=10000826.
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Cystic Fibrosis (CF) is a genetic disease that affects multiple systems including the respiratory tract. Thickened secretions in the airway must be removed to prevent deterioration of pulmonary function. Airway clearance techniques are based on physiological principles such as cephalad airflow, the combination of expiratory airflow with high frequency oscillation at the chest wall or oral cavity, and repetitive cough. Diaphragmatic breathing, respiratory muscle training, and voicing during therapy have also been shown to improve or maintain pulmonary function in CF patients. The physiology of airway clearance is similar to the physiology of singing, which involves controlled airflow resulting in pressure oscillations and vibration. Singers build awareness of their bodies and learn to manage their breath. It is also a relatively inexpensive and enjoyable activity that requires little equipment or space. Singing should not replace medically recommended therapies, but it may be a viable adjunct therapy.
In this concurrent mixed methods collective case study, the effects of nine private singing lessons on the pulmonary function, measured by FEV 1 and quality of life, measured by the Cystic Fibrosis Questionnaire-Revised (CFQ-R) of four CF patients were investigated. Participants also completed an exit questionnaire to determine their satisfaction with the lessons and impressions of the study. Lessons were taught by the same teacher and tailored to the needs of each participant. The teacher kept a journal of the proceedings of each lesson. To evaluate the efficacy of the lessons, the teacher assessed each participant before and after the lessons using a rubric and helped the participants to create modified phonetograms. Two single-factor analyses of variance (ANOVAs) were performed on the phonetograms to compare the pre and post voice lesson effects in terms of maximum and minimum sound pressure levels. Due to the small sample size, no further statistical analysis was performed, and the results of the study will be pilot data for future research.
Effects on FEV1 were inconclusive, but the teacher observed all participants coughed during singing and not during conversation, suggesting the mobilization of mucus. All participants improved in the domain of body image on the CFQ-R. This, combined with comments from participants on the exit questionnaire about gaining confidence, suggests an overall improvement in self-esteem resulting from the lessons. Most participants also improved in the domain of physical functioning. Two participants improved significantly in terms of maximum vocal intensity as indicated by their phonetograms. The teacher was well informed about CF but did not need to structure the musical aspects of the lessons in any specialized way, although an emphasis was placed on breathing and the allowance and encouragement of coughing from participants, which is deviant from the norm. All participants reported satisfaction with the treatment and wrote about breath control being one of the most important gains from the lessons. All but one reported they would continue lessons if given the chance. The results suggest singing lessons may provide some airway clearance and improve the quality of life of adult CF patients. Further investigation of this topic is warranted.
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Sans, Serramitjana Eulàlia. "Nanoencapsulated antimicrobials to fight Pseudomonas aeruginosa respiratory infections in cystic fibrosis patients: a promising strategy." Doctoral thesis, Universitat de Barcelona, 2017. http://hdl.handle.net/10803/461914.
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P. aeruginosa is one of the major opportunistic pathogen colonizing the respiratory tract of cystic fibrosis (CF) patients and causing chronic airways infection. Once P.aeruginosa established chronically in the CF lung, bacterial density increases and the microorganism switches to a mucoid form and to a stable biofilm mode of growth in which susceptibility to antimicrobials decreases. The high resistance of P.aeruginosa to multiple antimicrobials led to scenarios in which almost no treatment options are available. In this regard, the research on the introduction of less toxic antimicrobials as well as the use of pharmaceutical forms enabling dose reductions, longer administration intervals, and reduced systemic toxicity has been stimulated.
Therefore, the aim of this thesis was to develop nanoencapsulated colistin and tobramycin in lipid nanoparticles (SLN: Solid Lipid Nanoparticles and NLC: Nanostrucutured Lipid Carriers) and explore their antimicrobial activity versus free drug against P.aeruginosa clinical isolates from CF patients and to investigate the efficacy of these novel formulations in the eradication of biofilms, one of the most relevant mechanisms involved in persistence and in chronic infections.
ELABORATION AND CHARACTERIZATION
The main objective of the first part of this thesis was to elaborate and characterize lipid nanoparticles (SLN and NLC) as colistin and tobramycin carriers to treat P.aeruginosa lung infection.
The nanoparticles obtained displayed a 200–400 nm size, high drug encapsulation (79–94%) and a sustained drug release profile. The integrity of the nanoparticles was not affected by nebulization through a mesh vibrating nebulizer. Next, tobramycin-NLCs were able to overcome an artificial mucus barrier in the presence of mucolytic agents. Moreover, lipid nanoparticles loaded with both antimicrobials appeared to be less toxic than free drug in cell culture. Finally, an in vivo distribution experiment showed that nanoparticles spread homogenously through the lung and there was no migration of lipid nanoparticles to other organs, such as liver, spleen or kidneys.
STABILITY
The second essential point of this work concerns the stability of both types of lipid nanoparticles after freeze-drying.
The results showed that colistin-SLNs lost their antimicrobial activity at the third month; on the contrary, the antibacterial activity of colistin-NLCs was maintained throughout the study within an adequate range. In addition, colistin-NLCs exhibited suitable physic-chemical properties at 5 °C and 25 °C/60% relative humidity over one year. Altogether, colistin-NLCs proved to have better stability than colistin- SLNs.
The last part focuses on the study of the antimicrobial activity of SLN and NLC loaded with colistin and tobramycin against P.aeruginosa isolates from Sant Joan de Déu and Vall d’Hebrón hospitals CF patients.
Regarding the data documenting planktonic experiments, colistin nanoparticles had the same antimicrobial activity as free drug. The activity of tobramycin-loaded SLN was less than that of either tobramycin-loaded NLC or free tobramycin. However, in the relation to biofilms, nanoencapsulated antimicrobials were much more efficient than their free form. Moreover, the results showed the more rapid killing of P. aeruginosa bacterial biofilms by NLC-colistin than by free colistin. Nevertheless, the two formulations did not differ in terms of the final percentages of living and dead cells, which were higher in the inner than in the outer layers of the treated biofilms.
Since it seems clear than biofilms play a key role in respiratory infections in CF patients by P. aeruginosa, these formulations seem to us encouraging alternative to the currently available CF therapies.P.aeruginosa és un dels principals patògens oportunistes colonitzadors del tracte respiratori dels pacients amb fibrosi quística (FQ) causant una infecció crònica. Una vegada aquest microorganisme ja està establert de manera crònica al pulmó, la densitat bacteriana augmenta i P.aeruginosa canvia de morfologia no mucosa a mucosa afavorint la formació de biofilm en el qual la susceptibilitat als antibiòtics es veu enormement disminuïda. L’elevada resistència de P.aeruginosa a múltiples antimicrobians ens condueix a un escenari on gairebé no hi ha opcions de tractament disponibles. En aquest sentit, la recerca en la introducció d’antimicrobians menys tòxics així com l’ús de noves formes farmacèutiques amb la capacitat de reduir la dosi, allargar els intervals d’administració així com reduir la toxicitat adquireix molta rellevància. Per tant, l’objectiu d’aquesta tesi va ser desenvolupar nanopartícules lipídiques (Solid Lipid Nanoparticles: SLN y Nanostructured Lipid Carriers: NLC) carregades amb colistina I també les partícules amb tobramicina, explorar la seva activitat antimicrobiana comparant-la amb la seva forma lliure contra soques clíniques de P.aeruginosa aïllades de pacients amb FQ, i investigar l’eficàcia d’aquestes noves nanoformulacions en l’eradicació dels biofilms ja que és un dels mecanismes més rellevants associat a les infeccions cròniques.
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Nelson, Andrew. "An investigtion of the polymicrobial nature of lower respiratory tract infections in cystic fibrosis patients." Thesis, Northumbria University, 2011. http://nrl.northumbria.ac.uk/5842/.
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Cystic fibrosis (CF) is a genetically inherited condition most prevalent amongst Caucasians. In previous studies, it has been demonstrated that bacterial, fungal and viral pathogens cause lung function decline and ultimately result in death due to respiratory failure. Patients with CF produce sputum daily, which makes it an ideal infection to study in terms of access to samples. However, it is unknown how transport of the samples from the patient to the laboratory will affect the results of molecular microbiological analysis. We found that the bacterial community profiles were significantly different in samples stored at room temperature from those which were refrigerated. Furthermore, a significant increase in bacterial load and numbers of Pseudomonas spp. and a significant decrease in number of H. influenzae were seen in the samples stored at room temperature. In this study we also aimed to characterise the factors which have an effect on the bacterial and fungal communities present in the CF lung in patients who possessed the F508del CFTR allele. We found that gender was a significant factor in the assembly of bacterial communities, due to a reduction in bacterial diversity and community evenness. Furthermore, we identified that P. aeruginosa colonisation affected bacterial community composition. We have also identified that bacterial community assembly in the CF lung appears to be stochastic. However, our data also shows that gender and P. aeruginosa colonisation affect assembly suggesting that, in some respects, a deterministic community assembly is also being observed. Our data also suggests that fungal communities are more diverse than is currently recognised. Additionally, we have found that patients who are homozygous for the F508del CFTR mutation harbour more rich fungal communities than patients who are heterozygous. A further objective was to follow these patients longitudinally to determine the stability of the CF lung microbiota, to determine the effects of antibiotic therapy, and to assess if any changes occurred in the CF lung during times of pulmonary exacerbation which could be identified as the causative agent. We did not find a significant relationship between exacerbations and the bacterial communities present in CF. However, in one patient we found that a particular bacterial taxa was present when the patient presented with an exacerbation but was absent when the patient was stable, suggesting that acquisition of a new bacterial taxa can potentially cause an exacerbation. We also found that an increase in bacterial load was not the cause of exacerbations in our cohort. Furthermore, the presence and abundance of fungal species was found not to be the cause of exacerbations.
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Hedqvist, Camilla. "Lyophilization of specific IgY antibodies against Pseudomonas Aeruginosa used as therapy for Cystic fibrosis patients." Thesis, Uppsala universitet, Institutionen för kvinnors och barns hälsa, 2013. http://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-227236.
Full textAbstract:
Pseudomonas Aeruginosa is a common gram-negative bacterium present in the environment. It causes severe infections in immunosuppressed patients. Cystic fibrosis patients are especially at risk of being infected with Pseudomonas Aeruginosa. Ongoing studies are preformed to find alternative therapies to antibiotics, due to increased resistance. One new treatment is intake of specific IgY antibodies against Pseudomonas Aeruginosa as an oral therapy. The problem today is that IgY solutions must be kept frozen until consumed. In this study we examined the possibility to freeze-dry specific IgY antibodies without losing any activity or specificity of the antibodies. This would be more convenient of patients, as well as it makes transportation and storage easier. The methods used were ELISA for control of activity, western blot analysis and SDS-PAGE gel for control of specificity. Three different batches of the IgY anti-Pseudomonas Aeruginosa solution were tested. The results showed that no loss in activity occurred that would affect clinical outcome or change of specificity in the antibodies after freeze-drying appears. This indicates that it is possible to replace the liquid antibody to a freeze-dried powder.
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Laiho, Kirsi Marjut. "Digestion, absorption and metabolic disposal of dietary lipid in cystic fibrosis patients and control subjects." Thesis, University of Southampton, 2000. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.323770.
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Bolt, Isabel Bettina. "High morbidity and mortality in cystic fibrosis patients compound heterozygous for 3905insT and [delta]F508 /." [S.l : s.n.], 1998. http://www.ub.unibe.ch/content/bibliotheken_sammlungen/sondersammlungen/dissen_bestellformular/index_ger.html.
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Hendon-Dunn, Charlotte. "Bacteriophages as a potential treatment for Pseudomonas aeruginosa mediated chest infections in cystic fibrosis patients." Thesis, University of Brighton, 2011. https://research.brighton.ac.uk/en/studentTheses/f92ff33d-5bf8-48c3-aba3-76d5fa5936f8.
Full textAbstract:
Cystic fibrosis (CF) affects between 1in 2000 and 1 in 4500 births in caucasians of European descent. It is caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) resulting in abnormal membrane osmolarity. CF is a multi-organ disease, however the cause of death is most often due to respiratory failure caused by infection of the airway epithelia with the bacterium Pseudomonas aeruginosa. Ps. aeruginosa grows in the airways as a biofilm and is recalcitrant to treatment with antibiotics. Therefore alternative therapies are urgently required. Bacteriophages have been and still are used in ex-Soviet bloc countries as a treatment for many infections. However, to date very few comprehensive studies have been conducted into phage therapy in humans. The aim of this study was to characterise several Ps. aeruginosa phage and to develop an in vitro co-culture model simulating a Ps. aeruginosa lung infection on which bacteriophage could be assessed for their efficacy as a therapeutic agent.
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Yung, Rossitta Pui Ki. "Polymorphisms of CF modifier genes : their relationship to Pseudomonas aeruginosa infection and severity of disease in CF patients." Thesis, University of British Columbia, 2008. http://hdl.handle.net/2429/2396.
Full textAbstract:
Cystic Fibrosis is one of the most common genetic recessive diseases among Caucasians and is caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene on chromosome 7. There are different classes of CFTR mutation, leading to differences in disease severity among patients. In addition to the CFTR genotype, secondary genetic factors, modifier genes, also influence CF phenotypes. Due to the dysfunction of CFTR protein and production of thickened mucus, bacterial infection in the lungs is favored and can lead to further clinical complications in CF patients. Pseudomonas aeruginosa is one of the most common bacteria detected among patients. The aim of this project was to investigate four candidate modifier genes, Factor B, Complement Factor 3, Toll-like Receptor 4 and Heme oxygenase-1, which might affect the status of Pseudomonas aeruginosa infection. A total of 22 single nucleotide polymorphisms (SNPs) were selected in these four genes and they were tested against five phenotypic traits, including age of diagnosis, FEV1% predicted andstandard deviation value, age of first Pseudomonas aeruginosa infection and Pseudomonas aeruginosa infection status. Among the selected SNPs, both case-control studies and family-based analysis were performed in order to establish any correlation between the genotypes and the phenotypes. In addition, haplotype analysis was performed to determine whether there was interaction between SNPs or whether there were unidentified SNPs in the vicinity of the selected ones that might contribute to the observed phenotypic traits. Among the 22 chosen SNPs, 13 of them were found to be significantly linked to one or more of the tested phenotypes. The three most significant associations were BF_2557 with lung function, HMOX1_9531 with lung function and BF_7202 with age of diagnosis. Several haplotypes were significantly associated with one of the five phenotypes. There was no evidence for the presence of unidentified SNPs or interaction between SNPs. Most of haplotype associations were likely due to the presence of a single SNP which was found to be significantly linked to the phenotype. Conclusively, both SNPs and haplotype analyses suggest that the four candidate genes are modifiers of disease severity in CF.
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Ankrum, Andrea L. "Comparison of health measurements between Cystic Fibrosis Patients Colonized with Methicillin-Resistant Staphylococcus aureus Harboring either the Sccmec II or Sccmec IV Cassette." University of Cincinnati / OhioLINK, 2016. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1470672575.
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