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Lung cancer predisposition in patients with cystic fibrosis.
Kulichová, Kristýna ; Indra, Radek (advisor) ; Canová, Nikolina (referee)
Cystic fibrosis is a hereditary disease that particularly affects the respiratory and digestive systems. Although the disease is still incurable, advances in therapy have improved the quality of life of cystic fibrosis patients and also extended their life expectancy. However, as patients live longer, the risk of developing cancer also increases. It turns out that patients suffering from cystic fibrosis are likely to have an increased risk of developing digestive tract cancer. In general, the most affected organ are the lungs, which are often colonized by bacteria. In this research, I will first deal with the description of cancer and cystic fibrosis itself, the disorders that accompany cystic fibrosis, how the development of cancer could be connected with cystic fibrosis, and in the last part I will try to analyze the connection between cystic fibrosis and lung cancer.
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Study of potential anti-adherence compounds in a lung cell model as a means for the prophylaxis of bacterial infections in cystic fibrosis
Áčová, Andrea ; Hodek, Petr (advisor) ; Nosková, Libuše (referee)
5 Abstract Cystic fibrosis (CF) is an insidious genetic disease with autosomal-recessive inheritance. The impaired function of the CFTR protein, caused by a mutation in the eponymous gene, results in a wide range of symptoms, the most serious being the effect on the respiratory system. The main impact on the respiratory system is the appearance of thick mucus, which contributes to the accumulation of bacterial cells in the patient's lungs. Life-threatening pathogens include Burkholderia cenocepacia (BC) and Pseudomonas aeruginosa (PA). These bacteria produce several virulence factors such as BC2L-A or BC2L-C lectins in BC and PA-IIL in PA. The virulence factors allow bacteria to bind via protein-saccharide interactions to lung cells with altered glycosylation, which is seen in the CF patient. This diploma thesis focuses on the above-mentioned lectin interaction and the inhibitory effect of hen IgY antibodies or trivalent fucosylated glycoclusters on the adherence of BC (strain ST-32, CCM 7291) / PA (strain PAK, ST 1763) to lung epithelial cells of the CuFi-1 (from a CF patient) / NuLi-1 (from a healthy donor). The possible influence of glycoclusters on PA (PA-lux strain) cell viability was studied, but no significant effect was observed. To verify the expression of the BC2L-C lectin, it was necessary to...
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Therapeutic strategies in cystic fibrosis
Křesťan, Jan ; Bořek Dohalská, Lucie (advisor) ; Hudeček, Jiří (referee)
Cystic fibrosis is an autozomal recessive disease caused by mutations in the CFTR gene. The aim of this work is conclusion of therecent knowledgefrom theusage of CFTR modulators in the treatment of cystic fibrosis. The first part of this bachelor thesis is dedicated to the disease itself, its history and symptoms. In the form of description of the CFTR gene and its mutations which lead to the cystic fibrosis disease, the causes of the disease are described. Function of CFTR protein is also mentioned. The second part of this thesis is focused on conclusion of the current knowledge of the CFTR modulators treatment. In the last chapter of the work, possible future changes and ways of treating patients with CFTR modulators therapy are considered.
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Motility and virulence of bacterial pathogens in patients with cystic fibrosis
Benešová, Nikol ; Melter, Oto (advisor) ; Smělíková, Eva (referee)
Cystic fibrosis is an autosomal recessive disease that mainly effects the Europoid race. The disease is caused by a mutation in the cystic fibrosis transmembrane conductance regulator gene. This gene encodes the formation of chloride channel proteins. The disease leads to the formation of highly viscous secretions from the exocrine glands. The most commonly affected organ is the lung, which accumulates mucus and bacteria. Bacteria that colonize the lungs of patients with cystic fibrosis are exposed to many stress factors such as large amounts of mucus, oxidative stress, antibiotics and immune system of the host. That leads to the selection of mutants that are better adapted to the environment of the lungs. In acute infection, one of the important virulence factors for bacteria is their motility. Motility is provided by flagella and allows the bacterium to move to sites with higher nutrient content and to colonise different parts of the lung. In chronic infection, we can see changes in the genotype and phenotype of bacteria. Nonmotile mutants are selected because they activate the immune system less than motile bacteria, that helps in the persistence of bacteria in the lungs. Chronic infections lead to a higher mortality rate. The aim of this bachelor's thesis is to compare the motility of bacteria...
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Background innate reflex locomotion patterns of movement and ventilation in children patients with chronic respiratory diseases
Plešková, Jana ; Pohunek, Petr (advisor) ; Sedlák, Vratislav (referee) ; Homola, Lukáš (referee)
Reflex locomotion according to Professor Vojta (VRL) is based on the activation of complex motor patterns from certain defined positions with stimulation of the so-called trigger zones, located on the human's body. It is primarily used in patients with neurological deficits; however, it is currently also being used in internal medicine. It has been reported that VRL can indirectly affect breathing, however its use in paediatric patients with chronic respiratory disease has not yet been investigated. As part of this theses, a randomized controlled single-centre intervention study was conducted. Its aim was to evaluate the short-term effects of VRL on lung function in paediatric patients with cystic fibrosis (CF) with normal baseline spirometry. The effect of a 30minute VRL intervention was compared in a crossover design with the effect of positioning (without stimulation of trigger zones). The primary outcome was the change in global ventilation inhomogeneity, assessed by lung clearance index (LCI2.5) derived from nitrogen multiple breath washout test. Secondary outcomes included changes in regional ventilation inhomogeneity (indices of acinar [Sacin*Vt] and conductive airways [Scond*Vt] inhomogeneity) and spirometric parameters (inspiratory capacity, forced vital capacity, and forced expiratory...
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Interaction of bacterial lectins with human lung epithelium
Vyhnalová, Kateřina ; Hodek, Petr (advisor) ; Bořek Dohalská, Lucie (referee)
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene encoding the same named chloride channel, which is present on the apical membrane of epithelial cells. As a consequence of these mutations, the transepithelial transport of ions and the regulation of other ion channels are impaired. Cystic fibrosis affects primarily respiratory system, where, as a result of the dysfunction of the CFTR protein, is an accumulation of excessively viscous mucus and altered glycosylation of saccharide structures on the surface of the lung epithelium. This pathological condition predisposes CF patients to bacterial lung infections, the leading cause of death in CF patients. The most common pathogen colonizing the respiratory system of CF patients is Pseudomonas aeruginosa (PA). For adhesion to host tissue, PA uses several virulence factors, including lectin PA-IIL, which exhibits an unusually high affinity for L-fucose. In this work the influence of selected monosaccharides and multivalent fucose-based inhibitors (glycoclusters) on PA-IIL binding to human lung epithelium was investigated. To assess the effect of these agens ex vivo, paraffin-embedded formalin-fixed human lung tissues (FFPE tissues) from a healthy individual and...
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Evaluation of effect of mobile application CF Hero on compliance of inhalation treatment and respiratory physiotherapy of pediatric and juvenile patients with cystic fibrosis
Jirásek, Martin ; Plešková, Jana (advisor) ; Pokorný, Jan (referee)
This diploma thesis deals with the adherence to inhaled treatment, its measurement, telemedicine and telerehabilitation and the possible use of the CF Hero mobile app in paediatric patients with cystic fibrosis. In the theoretical part, it presents knowledge about cystic fibrosis, adherence to treatment in patients with cystic fibrosis and possibilities of its measurement, telemedicine and telerehabilitation. The practical part describes the CF Hero mobile app, which has been the target of measurements on several patients. At the same time, a questionnaire was developed focusing on satisfaction with the use of this mobile application. Methods: The practical part was conducted as a multicentre prospective cross-over study investigating the effect of the CF Hero mobile app on adherence to inhaled treatment, respiratory quality of life and chest excursion The evaluation was conducted over a 6-month period, with the primary objective being to measure the amount of exhaled solution relative to the prescribed amount. Simultaneously, each patient underwent 3 identical examinations. These examinations included spirometric testing (FEV1, FVC, MEF50), measurement of chest excursion (axillary, mesosternal, xiphoid) and quality of life assessment using the CFQ-R questionnaire. It also included measurement of...
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Social impacts of Cystic fibrosis disease on children of various age
FILIPI, Kateřina
The bachelor thesis is called Social impacts of Cystic fibrosis disease on children of various age. Cystic fibrosis is a disease which can be treated, but it cannot be completely cured yet. It is a disease that affects mostly the lungs where a thick mucus is gradually built and is hard to get rid of. However, it impacts other organs too. Cystic fibrosis is considered to be a rare disease because in the Czech Republic there are approximately 600 patients only. The aim of this bachelor thesis is to find out what aspects of life are impacted the most by the cystic fibrosis. The main focus was on the families with children who are diagnosed with the disease. Not only is the disease financially demanding, but also it impacts social life as people suffering from it should avoid contact with a large number of people because they are at higher risk of getting sick. People suffering from the disease face many restrictions, therefore they can feel disadvantaged compared to their peers. The research question was formulated as follows: What are the most common social impacts on a family with a child that is suffering from cystic fibrosis? Given the aim of the thesis, I used qualitative research, specifically interviews. In the empirical part, the snowball technique for the interviews was used. Seven families with children suffering from cystic fibrosis participated (9 - 17 years old). The results suggest that the most common social impact of the cystic fibrosis is a limited choice of a future occupation. Due to the strict restrictions which are placed on people suffering from the cystic fibrosis it is not possible to pursue just any profession that patients would want to. Another issue is that many families are not entitled to social benefits due to unjust decision making.
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Analysis of gene polymorphisms in the MBL2 gene and its diagnostic significance
PEŠKOVÁ, Jana
MBL2 gene codes a protein called mannose binding lectin, a component of our immune system. This protein takes a part in a nonspecific humoral immune response, opsonizing pathogenic microorganisms and providing activation of the lectin pathway in the complement system, leading to elimination of pathogens and simultaneously inducing the inflammation. MBL deficiencies are widely researched in cases of recurrent infections, autoimmune diseases and others. MBL-protein serum level is affected by polymorphisms in MBL2 gene, located in three codones marked as '52', '54' and '57'. A mutation of the '52' codon is referred to as an allele D, for the '54' codon as an allele B and for the '57' codon as an allele C. In many cases the alleles might be referred to as the alleles zero (0), or as an 'A' in the case of wildtype alleles. Other polymorphisms are located in non-translated locations. The first one may be found in the promotor 1, in a position -550 (variants H/L); the second one in a position -221 (variants X/Y); and the third one in a non translated 5'-end-part of a locus in a position +4 (variants P/Q). The practical part of this bachelor thesis was executed in the genetic lab in the company GENLABS s.r.o., České Budějovice. I focused on the analysis of MBL2 polymorphisms located in exon 1 (alleles B, C, D). I examined 30 patient samples, 25 of them were provided by patients with ongoing dementia and 5 of the samples were taken from patients with no signs of dementia. The protocol of the analysis consisted of an isolation of DNA, a measurement of DNA concentration, a preparation and an execution of a PCR method followed by a control of the products in a gel electrophoresis. These PCR products were then purified and sequenced. A precise description of the analysis including an overview of the obtained results are summed in the practical part of this bachelor thesis.
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The perspective of the classroom climate regarding a child suffeing from a chronic illness
DUŠKOVÁ, Barbora
The diploma thesis deals with the issue of a pupil with chronic disease and his/her impact on the class environment. In the theoretical part, the characteristics of selected chronic diseases are processed, as well as basic information about the disease in connection with the psyche. It is followed by school legislation concerning pupils with special educational needs, the topic of the school class as a social group and basic information regarding the environment of the class. In the practical part, interviews with chronically ill pupils and their class teachers are processed as well as the questionnaires which were filled out by classmates of the pupil with the disease. The research survey focuses mainly on the feelings of the pupil with chronic illness in the school environment, the attitude of the teacher towards the ill pupil and its impact on mutual relations in the classroom. Pupils with chronic diseases who participated in our research survey have no problem with integration into the classroom.
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