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1.
Ambiguous abdominal situs, asplenia/polysplenia and severe cardiac malformations characterize heterotaxy in humans. These anomalies result from the inability of the developing embryo to establish normal left-right asymmetry. We have studied an interesting family in which the heterotaxy phenotype segregates as an X-linked recessive trait. In order to map the heterotaxy locus (HTX), we have analysed 39 family members using highly-polymorphic microsatellite markers from the X chromosome. One of these markers, DXS994, shows no recombination with the disease locus in 20 informative meioses. Linkage analysis results in a maximum lod score of 6.37. Current genetic and physical mapping data assign the order of loci in Xq24-q27.1 as cen-DXS1001-(DXS994, HTX)-DXS984-tel. These results establish the first mapping assignment of situs abnormalities in humans.  相似文献   
2.
Bone marrow examination revealed a lipid-laden histiocytosis in seven patients undergoing long-term total parenteral nutrition necessitated by extensive short-bowel surgical resection. Clinical abnormalities occurred during this treatment which required bone marrow examination. These included hepatosplenomegaly and peripheral blood cytopenia; the median time to the detection of these abnormalities was 64 months. The most striking change within the bone marrow was the presence of many pigment-laden histiocytes which had the typical morphology of sea-blue histiocytes seen in the so-called idiopathic sea-blue histiocyte syndrome. The occurrence of sea-blue histiocytosis in the bone marrow in association with long-term parenteral nutrition for short-bowel syndrome has not, to our knowledge, been reported previously and should now be considered in the differential diagnosis of bone marrow sea-blue histiocytosis.  相似文献   
3.
A decoupling circuit using an operational amplifier is proposed to suppress substrate crosstalk in mixed-signal system-on-chip (SoC) devices. It overcomes the parasitic inductance problem of on-chip capacitor decoupling. The effect of the proposed decoupling circuit is not limited by parasitic fine impedance. A 0.13-/spl mu/m CMOS test chip showed that substrate noise at frequencies from 40 MHz to 1 GHz was incrementally suppressed by sequentially activating three of the proposed circuits in parallel. The power dissipation of each circuit was 3.3 mW at a 1.0-V power supply. The test chip measurement showed that the proposed decoupling reduced crosstalk by 31% at 200 MHz, whereas it was reduced by 4.4% with capacitor decoupling. This 7:1 ratio, or 17 dB, corresponds to the gain of the opamp. Design of the opamp and its feedback loop for active decoupling is simple, making the opamp useful for SoC applications.  相似文献   
4.
Inertinite concentrates from three Australian bituminous coals were hydrogenated at various temperatures ranging from 350 to 475 °C in the presence of tetralin without any added catalyst. Both conversion yields and microscopic observations of the benzene-extracted hydrogenation residues have revealed that the hydrogenation of the inertinite macerals becomes significant only > 400 °C, whereas the dissolution-hydrogenation of the accompanying vitrinite macerals occurs mainly between 350 and 400 °C. The major reaction pathway for the inertinite particles in the hydrogenation process appears to be one of initial mild carbonization followed by hydrogenation. A simplified reaction scheme is proposed which describes the reaction pathway involved in the hydrogenation of inertinite.  相似文献   
5.
The morphologic changes and signal intensity of the spinal cord on preoperative magnetic resonance images were correlated with postoperative outcomes in 74 patients undergoing decompressive cervical surgery for compressive myelopathy. The transverse area of the spinal cord on T1-weighted images at the level of maximum compression was closely correlated with the severity of myelopathy, duration of disease, and recovery rate as determined by the Japanese Orthopaedic Association score. In patients with ossification of the posterior longitudinal ligament or cervical spondylotic myelopathy, the increased intramedullary T2-weighted magnetic resonance imaging signal at the site of maximal cord compression and duration of disease significantly influenced the rate of recovery. A multiple regression equation was then developed with these three variables to predict surgical outcomes.  相似文献   
6.
Three phase partitioning (TPP) uses t-butanol and ammonium sulfate to precipitate enzymes and proteins from aqueous solutions. The method is useful both upstream with crude samples and downstream where a scaleable simple step is needed. About 25 enzymes and proteins have been isolated by various laboratories using TPP-t-butanol. The relation of t-butanol used in TPP, with n-butanol used as an extraction agent from Morton's work, is reviewed. Some t-butanol appears bound to TPP-precipitated proteins which are actually protein-t-butanol coprecipitates. They float above denser aqueous salts because bound t-butanol increases their buoyancy, similar to the behavior of many lipoproteins. On redissolving TPP-precipitated enzymes, total and specific activities usually are regained and sometimes increased. Sulfate ion-in large concentrations-likely exerts itself through its kosmotropic action as in conventional salting out. t-Butanol likewise appears to be a kosmotrope and crowding agent at room temperature or above, whereas C1 and C2 cosolvents (e.g., ethanol) do not so behave except at near or below zero temperatures. However, kosmotropy is not the entire origin of TPP, nor probably of conventional salting out. Electrostatic forces, capacity to force protein conformation tightening and protein hydration shifts, also contribute. Electrostatic forces, and the tendency for salt ions to bind and tighten protein molecule conformation, are indicated by the sharp pH dependency of both conventional salting out and TPP, around pH regions where proteins undergo conformation changes. Sulfate anion is densely-perhaps extraordinarily-hydrated, adding much to its effective size, and therefore it has a tendency to crowd or exclude proteins, when sulfate concentrations are in the 0.5 to 3 M range.  相似文献   
7.
This paper describes a prototype video coding platform meant for the conception and testing of multimedia products such as next-generation videophones. The platform is largely based on ITU-T Recommendation H.263, with a number of additional object-oriented quality enhancement features which make it especially well suited for very low bit-rate coding of “head-and-shoulders” video material typical of real-time multimedia applications, video teleconferencing, and video telephony. These features consist of: (1) segmentation into objects of interest, (2) segmentation-based prefiltering, (3) model-assisted rate control, (4) adaptive vector quantization, and finally (5) segmentation-based postfiltering. In the spirit of Recommendation H.263, these enhancements are modular and can be selectively turned on or off, thereby enabling a wide variety of coding modes  相似文献   
8.
A new finishing process for metal molds by large-area electron beam (EB) irradiation is proposed in this study. In the large-area EB irradiation equipment used here, an EB with high-energy density is irradiated without focusing the beam, and so the EB with a maximum diameter of 60 mm can be used for melting or evaporating metal surface instantly. Experimental results show that the surface roughness decreases from 6 μmRz to less than 1 μmRz in just a few minutes under proper machining conditions. The corrosion resistance of metal mold surface also could be greatly improved by large-area EB irradiation. Furthermore, the surface roughness of tilting surface close to 90° could be well improved. Therefore, large-area EB irradiation method has a possibility to become a high-efficiency finishing process for metal molds.  相似文献   
9.
目前在日本,存在大量工业垃圾,包括各种塑料制品,ASR(汽车破碎垃圾),含有毒PCB(多氯联苯)的110变压器和PVC(聚氯乙稀)制品等.而来自城市垃圾焚烧炉的废气含有大量能量.因此,在充分考虑环境、避免二垩英生成的同时,安全回收来自工业垃圾、城市和/或工业垃圾焚烧炉的资源和能量是个非常重要的课题,这也将对抑制CO2排放作出贡献.正确地阐明焚烧过程中二垩英的生成机理及详细研究含少量二垩英的焚烧废气在冷却过程中大量形成二垩英的临界温度是项很重要的工作.解决这个问题可有效实现资源和能量的回收.本报告介绍日本在二垩英排放方面目前采取的对策和存在的问题以及大阪大学为阻止二垩英生成所做的基础研究工作.  相似文献   
10.
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