National Repository of Grey Literature 8 records found  Search took 0.01 seconds. 
Design of formula SAE front axle
Honzík, Tomáš ; Blaťák, Ondřej (referee) ; Porteš, Petr (advisor)
My thesis will be aimed at front axle design. I am going to design the assembly of the front axle in the program ADAMS by MSC. I am going to observe and record most of cinematic changes of the car such as change of position of vehicle roll centre ,wheel camber, geometry adjustment , wheel toe-in measuring and other necessary data. The final report is going to include strength calculation of particular axle parts. I am also going to solve the axle stabilizer including strength calculation.
Developmental, pathobiochemical and molecular aspects of selected inborn errors of metabolism
Kolářová, Hana ; Honzík, Tomáš (advisor) ; Drahota, Zdeněk (referee) ; Morava Kozicz, Eva (referee)
Inborn errors of metabolism represent a heterogenous group of rare conditions, most having an incidence of less than 1 in 100,000 births. Because of their low prevalence, they are on the margin of attention of general research and even more so of large pharmaceutical companies. Study of rare diseases is the only way to design therapeutic options in order to improve quality of life of affected patients. Present Thesis particularly focuses on disturbances in mitochondrial energy metabolism. The main goals were the characterization of mitochondrial biogenesis within foetal development, as well as in childhood and adulthood. Another aim was to define clinical, biochemical and molecular aspects of mitochondrial optic neuropathies in childhood and adulthood. This work supported the earlier observations that gestational week 22 is the edge of viability, which has to be taken into account in upcoming discussions about guidelines on resuscitation of preterm neonates. Secondly, over last four years, we managed to examine and describe large cohort of patients with optic neuropathies based on a mitochondrial dysfunction. We have managed to characterize the biochemical and molecular-genetic background in more than 200 patients, and both selected cases (LHON/MELAS overlap syndrome) and cohort studies (MELAS,...
Clinical aspects of selected rare diseases in children.
Mazurová, Stella ; Honzík, Tomáš (advisor) ; Hrstková, Hana (referee) ; Votava, Felix (referee)
Introduction: Diagnosing inborn metabolic diseases, as a large subgroup of rare diseases, due to their rarity and wide variety of clinical manifestations, can be demanding and often prolonged. Objective: The aim of this work is, with the regard to clinical, biochemical and genetical aspects of selected rare diseases, to contribute to their rapid detection, widen the features of the natural course of the disease and contribute to their preventability. Material: This work includes cohort studies of patiens with cardiac manifestations in mitochondrial diseases, namely a group of 48 patients with TMEM70 protein deficiency, a group of 4 patients with Barth syndrome and individual cases of rare mitochondrial cardiomyopathies, thimidine kinase 2 deficiency and alanyl tRNA synthetase 2 deficiency. By determining the frequency, severity and type of heart disease, the phenotype was expanded, and the design of a therapeutic algorithm then made a positive impact on the prognosis of these patients. The work is also focused on the role of cardiac disease in the differential diagnosis of other genetically determined rare diseases, Marfan's syndrome and especially Pompe disease, where the emphasis is on early diagnosis, mainly due to the existence of an effective therapy. Focus on a broader differential diagnosis...
Developmental, pathobiochemical and molecular aspects of selected inborn errors of metabolism
Kolářová, Hana ; Honzík, Tomáš (advisor) ; Drahota, Zdeněk (referee) ; Morava Kozicz, Eva (referee)
Inborn errors of metabolism represent a heterogenous group of rare conditions, most having an incidence of less than 1 in 100,000 births. Because of their low prevalence, they are on the margin of attention of general research and even more so of large pharmaceutical companies. Study of rare diseases is the only way to design therapeutic options in order to improve quality of life of affected patients. Present Thesis particularly focuses on disturbances in mitochondrial energy metabolism. The main goals were the characterization of mitochondrial biogenesis within foetal development, as well as in childhood and adulthood. Another aim was to define clinical, biochemical and molecular aspects of mitochondrial optic neuropathies in childhood and adulthood. This work supported the earlier observations that gestational week 22 is the edge of viability, which has to be taken into account in upcoming discussions about guidelines on resuscitation of preterm neonates. Secondly, over last four years, we managed to examine and describe large cohort of patients with optic neuropathies based on a mitochondrial dysfunction. We have managed to characterize the biochemical and molecular-genetic background in more than 200 patients, and both selected cases (LHON/MELAS overlap syndrome) and cohort studies (MELAS,...
Design of formula SAE front axle
Honzík, Tomáš ; Blaťák, Ondřej (referee) ; Porteš, Petr (advisor)
My thesis will be aimed at front axle design. I am going to design the assembly of the front axle in the program ADAMS by MSC. I am going to observe and record most of cinematic changes of the car such as change of position of vehicle roll centre ,wheel camber, geometry adjustment , wheel toe-in measuring and other necessary data. The final report is going to include strength calculation of particular axle parts. I am also going to solve the axle stabilizer including strength calculation.

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