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Clinical and elektrophysiology longitudinal study of children with hereditary neurophathy Charlot-Marie-Tooth type 1A
Haberlová, Jana ; Seeman, Pavel (advisor) ; Syka, Josef (referee) ; Ambler, Zdeněk (referee) ; Vondráček, Petr (referee)
Hereditary peripheral neuropathy, known asCharcot Marie Tooth disease (CMT) and with an incidence of 1:2500 -1:10 000, is the most common hereditary neuromuscular disorder. Type CMT 1A is the most common form of CMT refering to the group of primary demyelinitateing motor and sensory peripheral neuropathies. CMT phenotype is clinically characterized by chronic slowly progressive distal muscle weakness and atrophy with hypo or areflexia and mild to moderate acral sensory loss. The lower limbs are predominantly affected. The aims of this study were to describe the first and most common signs of CMT1A during the first decade of life, to characterize their progression, and evaluate the sensitivity of CMTNS (Charcot-Marie- Tooth neuropath scale) for CMT1A young children. Sixteen children aged 3 to 10 years with genetically proven CMT 1A were examined. All patients were clinically examined, underwent electrophysiological examination, and were scored by CMTNS. Eight were followed for up to two years. Our data shows that CMT 1A in children under the age of 10 years causes only a mild disability. Initial signs of CMT 1A were difficulty in heel walking (15/16, 93%) and lower limb hypo or areflexia ( 13/16, 81%). The test of heel walking can be easily used as a screening test for hereditary neuropathies in pediatrics....
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Infrared measurements in medium and long wavelength on COMPASS
Vondráček, Petr ; Horáček, Jan ; Pánek, Radomír ; Gauthier, E.
The experimental study of radial profiles of parallel heat flux in the scrape-off layer (SOL) of the COMPASS tokamak shows a systematic discrepancy between medium (MWIR) and long (LWIR) wavelength infrared (IR) thermographic measurements. Significant temperature and heat flux overestimation is observed in the MWIR range, whilst the LWIR range is almost not affected. It is assumed to be caused by a presence of dust particles creating sub-pixel sized micro hot-spots. Significant inhomogeneity of the surface conditions across the limiter is observed, resulting in local disturbance of the derived heat flux profiles (decay length variation up to ~ few tens of percent). Influence of the inhomogeneously distributed micro hot-spots is suppressed using correction factors (the heat transmission coefficient α) for each pixel separately in the heat flux calculation. Procedure of the α coefficient estimation is described.
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Machinery used for drop forging
Vondráček, Petr ; Řiháček, Jan (referee) ; Podaný, Kamil (advisor)
The bachelor thesis contains distribution and overview of the machines used in the proces of die forging and their modern innovation. For individual mechanism is described principle, structural arrangements and advantages or disadvantages, or the example of used machine for which is described his charaterisctic, basic parameters and productions possibilities with example of made forged.
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Renewal of the fleet in chosen company
Čížek, Martin ; Rathouský, Bedřich (advisor) ; Vondráček, Petr (referee)
This Master´s Thesis focuses on the issue of renewal of fleet in Deblice-lesy, s.r.o. company. The aim of this thesis is selection of suitable semitrailer combination for transportation of long timbers and also selection of truck for transportation of ACTS containers for transportation of firewood, which will correspond to the selected criteria. Selection will be realized by using TOPSIS method. Theoretical part focuses on issues of business in road transport, trucks and combinations in road transport and limits of their dimensions and weights. It also describes some methods of multi-criteria decision making. Practical part focuses directly on selection of suitable semitrailer combination for transportation of long timbers and also selection of truck for transportation of ACTS containers according to the defined criteria. One of the aims is also mapping of the market and choices which exist when selecting combination for transportation of long timbers.
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Parallel Heat Flux Decay Length Study in the COMPASS Tokamak Using MWIR and LWIR Cameras
Vondráček, Petr ; Horáček, Jan ; Pánek, Radomír ; Gauthier, E.
A comprehensive study of a parallel heat flux in a tokamak scrape-off layer (SOL) has been performed in the COMPASS tokamak recently. Specially shaped high field side (HFS) limiter was used to estimate a heat flux radial decay length for small limiter radial misalignment. Long wavelength IR microbolometer and medium wavelength IR InSb camera were used for this purpose. This paper compares results obtained by the means of both cameras and demonstrates observation of very narrow heat flux decay length close to the last closed flux surface (LCFS) independantly on used camera.
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Infrared Thermography on the COMPASS Tokamak
Vondráček, Petr ; Horáček, Jan ; Cahyna, Pavel ; Pánek, Radomír ; Uličný, J.
This contribution describes a new slow infrared camera obtained by the COMPASS tokamak at IPP Prague. We focus on the camera limitations affecting the experimental data (temporal and spatial smoothing, time shift). Data time deconvolution used to correct the effect of the long response time of the camera detector is described. The second part of the contribution is devoted to the physic basis for the design of a new fast IR camera system planned for installation in COMPASS during the next year.
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