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Genetic haemochromatosis (GH) is the most common, autosomal recessive disorder in Northern Europe. The studies which led to the identification of the HFE gene are described. In the UK over 90% of patients with GH are homozygous for the C282Y mutation of this gene. This mutation is confined to populations of European origin. The significance of another mutation, H63D, in causing iron overload is less certain. Preliminary studies on the localization of the protein and the effects of the mutations are described. Genetic testing and the measurement of iron status now provide the means to allow for widespread testing for the prevention of iron overload and its consequences. However, questions remain about the clinical penetrance of GH.  相似文献   
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On the basis of experimental studies which proved stimulating action on absorbing and bactericidal function of blood phagocytes and antibody genesis the methods of therapy and prophylaxis of wide spread infections in modern live-stock (transmissive gastroenteritis of pigs, parainfluenza of calves, colibacteriosis of swine and cattle) were created. It was established, that optimal one-time therapeutical dose of homologous alpha-IFN for intramuscular administration to new-born pigs was 2000-4000 IU per head, prophylactic dose was 1000-2000 IU. Therapeutical doses of alpha-IFN for calves did not exceed 4000-6000 IU, prophylactic--4000 IU. As a rule, it is necessary to introduce the preparation three times with 48 h intervals. With the account of these data the large-scale commissional trials of these preparations were carried out.  相似文献   
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Extension of the theory of a clinotron is developed by use of the scattering matrix of an oversized T-junction on the ends of a slow wave system. The matrix contains elements corresponding to the transformation of slow grating modes into fast ones and vice versa. Those fast waves with low ohmic losses provide strong resonant properties of a clinotron even in the case of strong attenuation of the surface mode. Results of the theoretical simulation are compared with experimental ones and obtained dependencies explain strong resonances in sub-THz clinotrons.  相似文献   
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The opportunity of generating ribbon-shaped polyhelical electron beams intended to excite oscillations in the new type of gyrotrons is investigated via particle-in-cell simulation. Its distinctive features are the planar geometry of an interaction space and transverse energy extraction (with respect to the direction of translational motion of a helical electron beam). The electrode configuration has been found in the gyrotron with an operating frequency 75 GHz, where the formed electron beam has a velocity spread of ~10%, the rotational energy fraction is 50%, and the beam profile is weakly distorted. When the voltage is 12 kV and the beam current is 1 A, the electron beam under study is demonstrated to excite the single-mode generation with an efficiency of up to 15% in the gyrotron’s operating space produced by the planar waveguide.  相似文献   
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Excitation of surface (slow), space (fast), and hybrid surface-space waves in BWO-clinotron with periodically non-uniform grating has been studied both theoretically and experimentally. It is shown that an interaction of electron beam with hybrid surface-space wave may be more effective than with surface wave because of higher Q-factor, and than with space wave because of higher impedance. The experimental test results of both 100-GHz CW clinotron with non-uniform three-stage grating and of 130-GHz CW clinotron with four-stage grating showed the excitation of radiation with output power more than 1 W and some specific features of electronic frequency tuning.  相似文献   
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It has been demonstrated experimentally that lectin of Bacillus subtilis sp. in comparison with generally accepted Con A, PHA and lectin of "gold rain" grass--Laburnum anagyroides M e d i k in trials on white mice of CBA line gave in 4 hours of induction maximal titers of gamma-IFN in blood serum of animals--153.6 +/- 17.0 IU/ml. Practically identical titers had been obtained after induction by lectin "gold rain", some lower--after Con A and PHA. At swine gamma-IFN synthesis optimal density of cell suspension must contain 2.5 + 10(7) immunocytes in 1 ml, owing to which it is possible to obtain the titer equal 1 : 2150. Materials with using of bacterial lectins at various degree of purification had shown that maximal titers in blood serum of mongrel white mice were registered at administration to animals of non-purified lectin, 4 times lower--at using of half-purified and purified lectins. Data of these trials in vivo were confirmed by materials of gamma-IFN induction by immunocytes of swine, cattle and even man.  相似文献   
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In the experimental study on the frequency tuning of a double-beam gyrotron, an unexpected abrupt increase of the output power was observed for some magnetic field values corresponding to the excitation of high-order axial modes (HOAM). This effect can be explained through an after-cavity interaction of the spent but bunched electron beam with the neighboring transverse mode which has the same azimuthal but the next (i.e., greater by one) radial index with respect to the operating mode. The calculations show that under certain conditions the cyclotron synchronism between the electron beam and the next radial mode occurs in the extended region of an output cone, while the bunching of the spent electron beam is responsible for the excitation of this mode. The influence of some additional factors such as reflections of both modes from the output window, electron velocity spread, and magnetic field profile was studied numerically. It was shown that reflections, mode transformation, and velocity spread can enhance the effect of an abrupt power increase and reduce the starting current of the operating mode. The discovered after-cavity interaction can be either useful for a power increase at the HOAM operation of the frequency-tunable gyrotron or harmful for high cyclotron harmonic operation due to an expansion of the zone of fundamental cyclotron resonance mode excitation.

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Problems of designing laser power transmission systems in a turbulent atmosphere using relay mirrors mounted on a stratospheric airship are analyzed. It is shown that such a system can be designed using a high-power ground-based ytterbium fiber laser, relay mirrors for transmitting highpower laser radiation, and an adaptive optical system for correcting the phase distortions of laser beams due to atmospheric turbulence. A model of a laser power transmission system was designed and tested under laboratory conditions; the tests confirmed the possibility of correcting atmospheric-turbulence distortions during guiding and high-accuracy stabilization of laser beams.  相似文献   
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