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In the past 5 years we have discovered 8 boys and 3 girls who suffer from fructose-1,6-bisphosphatase deficiency. Although they all showed the typical symptoms of the deficiency such as frequent vomiting, hypoglycemia, lactic acidosis, and hepatomegaly, the age at diagnosis varied from 2 months to 4 years. All the boys revealed the deficient enzyme activity in leukocytes but none of the girls. The liver biopsy was investigated in six patients to confirm the diagnosis. These results suggest the existence of tissue-specific isoenzymes for fructose-1,6-bisphosphatase possibly with a different gene origin.  相似文献   
3.
If you don't know the current productivity rates, usage volumes, and other quantitative baseline data for your software operations, then software might not be a key component of the company's core business. The evaluation criteria in this article can help the IS executive determine the outsourcing options for software operations.  相似文献   
4.
Current cementing techniques of distal plugging, pulsatile water cleaning of the canal, and retrograde filling with PMMA in a low-viscous state prepared using porosity reduction techniques and then pressurized have been shown to give excellent results at 10 or more years after operation. However, physical and aging characteristics of PMMA do not guarantee that those results will hold up in the 20- to 30-year time frame. Fifteen-year experience with bony ingrowth systems indicate the development of a durable interface without PMMA, although the interface does appear more vulnerable to attack by the biologic reaction to HMWPE debris. Bone remodeling appears more favorable around proximally fixed bone ingrowth prostheses than around distally fixed prostheses. Softening of the physical characteristics of the stem tip may reduce the incidence of thigh pain. Patients with high activity potential and a life expectancy of greater than 25 years should be strongly considered for a proximally fixed bone ingrowth or ongrowth prosthesis.  相似文献   
5.
Elements of the lactose operon were used to study parameters affecting gene expression in cultured cells and transgenic animals. A Lac repressor protein containing a nuclear transport signal was shown to inhibit expression of a reporter gene by interacting with lac operator sequences. In cultured cells, operator sequence, operator placement and induction parameters were all shown to be important for obtaining tight repression of a reporter gene followed by high level expression upon induction. Induction levels were also dependent on the reporter gene, with the luciferase gene yielding higher induction levels than the chloramphenicol acetyltransferase gene. In transgenic animals, the lacI mRNA was not detected in the C57BL/6 mouse strain until the animal was exposed to a demethylating agent. After 5-azacytidine treatment, expression of lacI mRNA was detected in the brain, heart, kidney, lung and ovary. In the FVB transgenic mouse strain, expression of lacI mRNA was detected without 5-azacytidine treatment in the kidney, liver, lung, and testes. Preliminary experiments with double transgenic animals containing both lacI and operator/luciferase transgenes showed a decrease in luciferase expression compared to the luciferase-only animals in both tissue extracts and transgenic fetal primary cultures, although IPTG induction was not achieved in these animals or primary cultures. The applicability and challenges of the system for regulation of gene expression are discussed.  相似文献   
6.
Complexation of chitosan in aqueous solutions by low molecular weight electrolytes is one of the simplest methods for the preparation of aqueous chitosan dispersions. In this work, the influence of storage time, sulfate concentration, method of preparation and surfactant content on some properties of the resultant chitosan dispersions (turbidity, viscosity and zeta potential) was analyzed. Turbidimetry was adequate to monitor the formation of particles, while viscometry was suitable to monitor changes in the dispersing phase. An analysis of the properties of these systems, mainly in terms of particle–particle and macromolecule–macromolecule interactions was carried out. Copyright © 2004 Society of Chemical Industry  相似文献   
7.
This paper derives the governing equations for the thermomechanical behaviour of composites. When the basic equations for the thermoelastic behaviour of solids were first derived in the nineteenth century several approximations were made. The effect of these assumptions are discussed and illustrated by the results of a simple laboratory test. The implications of this work on the analysis of impact damaged laminates are then discussed.  相似文献   
8.
Discovering unknown adverse drug reactions (ADRs) in postmarketing surveillance as early as possible is highly desirable. Nevertheless, current postmarketing surveillance methods largely rely on spontaneous reports that suffer from serious underreporting, latency, and inconsistent reporting. Thus these methods are not ideal for rapidly identifying rare ADRs. The multiagent systems paradigm is an emerging and effective approach to tackling distributed problems, especially when data sources and knowledge are geographically located in different places and coordination and collaboration are necessary for decision making. In this article, we propose an active, multiagent framework for early detection of ADRs by utilizing electronic patient data distributed across many different sources and locations. In this framework, intelligent agents assist a team of experts based on the well‐known human decision‐making model called Recognition‐Primed Decision (RPD). We generalize the RPD model to a fuzzy RPD model and utilize fuzzy logic technology to not only represent, interpret, and compute imprecise and subjective cues that are commonly encountered in the ADR problem but also to retrieve prior experiences by evaluating the extent of matching between the current situation and a past experience. We describe our preliminary multiagent system design and illustrate its potential benefits for assisting expert teams in early detection of previously unknown ADRs. © 2007 Wiley Periodicals, Inc. Int J Int Syst 22: 827–845, 2007.  相似文献   
9.
Boron is observed to diffuse very slowly in germanium, as opposed to its behaviour in silicon where it exhibits Transient Enhanced Diffusion effects in implanted samples. As a result of this slow diffusion, boron is a very stable dopant, allowing devices to be created with very well-defined doping regions. To understand this superior performance, calculations were performed on a variety of boron diffusion paths, within vacancy and interstitial mediated methods. It was found that the vacancy mediated diffusion which is associated with the fast diffusion of many other species in germanium exhibits a total barrier of 5.8 eV. Interstitial-mediated diffusion had a total barrier for migration of 3.4 eV in the neutral and singly negative charge states, 3.2 eV for the positive charge state, but a formation barrier of 4.1 eV. Thus the barrier for interstitial-mediated diffusion is dominated by the formation energy of the self-interstitial.  相似文献   
10.
A study of the trace elements emission (As, Se, Cd, Co, Cr, Cu, Zn, Hg, Tl, Pb, Ni, Sn, Sb, V, Mn and Fe) from pulverized coal combustion has been made at six heating and power stations situated in the Czech Republic. The amount of chlorine in coal has considerable influence on volatilization of some elements such as Zn, Cu, Pb, Hg and Tl, which is explained by the formation of thermodynamically stable compounds of these elements with chlorine. Generally, the affinities for Cl follows the order Tl > Cu > Zn > Pb > Co > Mn > Sn > Hg. The experimental data indicates enrichment of some of the trace toxic elements in the emissions (Cu, Zn, As, Se, Cd, Sn, Sb, Hg and Pb) and good agreement was obtained by thermodynamic equilibrium calculations with a few exceptions. In the case of Fe, Mn, Co, Cr and Sn calculated values are overestimated in the bottom ash and there are zero predicted amounts of these elements in the fly ash. In comparison, the results from experiments show up to 80% of these elements retained in fly ash. This implies that there exist additional steps leading to the enrichment by Fe, Mn, Co, Cr and Sn of small particles. Such mechanisms could include the ejection during devolatilization of small inorganic particles from the coal of bottom ash particles, or disintegration of the char containing these metals to small particles of fly ash. On the other hand, there are slightly overestimated or similar values of relative enrichment factors for As, V, Cu, Cd, Sb, Tl and Pb in the fly ashes and zero predicted values for bottom ashes. Our experimental results show about 5% or less of these elements are retained in bottom ashes, so they probably remain in the bottom ash inside unburned parts of coal. Copyright © 2003 John Wiley & Sons, Ltd.  相似文献   
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