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Congenital nephrosis is an autosomal recessive disorder requiring neonatal renal transplant for survival. The postnatal diagnosis rests upon the electron microscopic evaluation of the epithelial foot processes and basal membrane of the glomeruli. The prenatal diagnosis can be suspected in the presence of a positive family history with an amniotic fluid (AF) alpha-fetoprotein level greater than 5 standard deviations (SD) above the population mean accompanied by a negative AF acetylcholinesterase, absent haemoglobin F, and an unremarkable fetal sonographic examination. We reviewed our series of seven cases of congenital nephrosis fulfilling the above criteria; four cases had negative family histories, and in two cases the diagnosis of congenital nephrosis was further supported by the presence of elevated AF albumin concentrations. We conclude that (1) the prenatal diagnosis of congenital nephrosis is feasible in a low-risk population, and (2) an elevated AF albumin concentration may represent an additional marker for the diagnosis of congenital nephrosis, even though false-negative results have been reported.  相似文献   
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在印度洋中部查戈斯礁群的大多数珊瑚死亡之后3年,对30m水深以浅珊瑚的侵蚀和恢复情况进行了研究.北部环礁15m水深以浅、中部和南部环礁>35m处的珊瑚差不多全部死亡.由于密集珊瑚丛的损失,一些礁体"表面"下降了1 5m.珊瑚的生物侵蚀情况严重,减少了三维礁体"结构"并形成松散的碎石.幼年珊瑚数量众多,尽管大部分是在侵蚀的或不稳定的基底上,并且稳定种较少.在15m的深度,礁体间鱼类丰度和多样性仍旧较高;依赖于珊瑚生存的物种减少,而一些食草动物和食碎屑动物则增加.一个新的海面温度(SST)数据集表明,平均SST自1950年以来升高了0.65℃.造成查戈斯珊瑚礁死亡的临界SST是29.9℃.  相似文献   
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A technique is described for measuring pressure within the amniotic cavity and within fetal vessels and/or body compartments. Two saline-filled catheters were connected at one end to needles inserted during indicated invasive procedures and at the other to silicon strain gauge transducers. In 36 pregnancies with normal liquor volume, stable intra-amniotic pressure (IAP, range 1–14 mmHg) increased with gestation (r=0·48, p<0·01). In pregnancies complicated by severe oligohydramnios, IAP was ≤ 1 mm Hg and rose to normal levels with saline amnioinfusion. Raised IAP (range 17–26 mm Hg), found in pregnancies with gross polyhydramnios, fell with drainage of amniotic fluid. Subtraction manometry was used to determine supra-amniotic pressure within the intervillus space, umbilical vein, umbilical artery, abdominal and thoracic cavities, and the urinary tract in normal and/or pathological fetuses. Low intravesical and intrapelvicalyceal pressures (median 6·5, range 2–10 mmHg) were noted in fetuses with obstructive uropathies. Intrauterine subtraction manometry appears to be a useful tool in the understanding of fetal pathophysiology and may be of clinical benefit in the therapeutic drainage and infusion of amniotic fluid and in the assessment of certain fetal disease states.  相似文献   
15.
In this account, we report our findings on blends of cellulose acetate having a degree of substitution (DS) of 2.49 (CA2.5) with a cellulose acetate having a DS of 2.06 (CA2.0). This blend system was examined over the composition range of 0–100% CA2.0 employing both solvent casting of films (no plasticizer) and thermal processing (melt-compressed films and injection molding) using poly(ethylene glycol) as a common plasticizer. All thermally processed blends were optically clear and showed no loss in optical quality after storage for several months. Thermal analysis and measurement of physical properties indicate that blends in the middle composition range are partially miscible, while those at the ends of the composition range are miscible. We suggest that the miscibility of these cellulose acetate blends is influenced primarily by the monomer composition of the copolymers. Bench-scale simulated municipal composting confirmed the biodestructability of these blends and indicated that incorporation of a plasticizer accelerated the composting rates of the blends.In vitro aerobic biodegradation testing involving radiochemical labeling conclusively demonstrated that both the lower DS CA2.0 and the plasticizer significantly enhanced the biodegradation of the more highly substituted CA2.5.While this work was in progress, Robert Gardner was struck with cancer and died on June 6, 1995. This paper is dedicated to his memory and to his contributions as a friend and colleague.  相似文献   
16.
Many of the eastern states that have published best management practices (BMPs) or forest management guidelines have also developed compliance monitoring programs to assess the application of these BMPs or guidelines on public and private forest land. The approaches among these states to collecting on-site monitoring data (measuring compliance) and evaluating sites are variable. A survey of eastern states found that almost all southern states monitor application of BMPs, but proportionally fewer of the northern states have established compliance monitoring programs. The state forestry agencies provide the leadership for these programs in most of the eastern states. States that monitor tend to evaluate all public and private forest landowner categories located within their states. In general, northern states monitor a broader array of site resources (e.g., cultural resources, visual quality) as compared to southern states which focus on water quality and wetlands protection. However, northern states focus their monitoring on timber harvesting, forest road construction and maintenance as compared to southern states which tend to monitor a broader array of forest management activities (e.g., site preparation, reforestation). When selecting sites for monitoring, the most common approach is to select some sites from all landowner categories within the state. Many states do not specify any criteria to identify sites for monitoring so that all sites have an equal chance for selection. Some states do use specific criteria to filter sites for monitoring, most commonly size of forest management activity and proximity to water.  相似文献   
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The current study used Mintzberg's (1983) conceptualization of political will and political skill to evaluate the predictors and consequences of political behavior at work. As elements of political will, we hypothesized that need for achievement and intrinsic motivation would predict the use of political behavior at work. Furthermore, we argued that political skill would moderate the relationship between political behavior and emotional labor. Data gathered from employees (N = 193) representing a wide array of organizations substantiated the proposed relationships. Specifically, need for achievement and intrinsic motivation were positively associated with political behavior. In turn, those opting to employ political behavior at work experienced a higher degree of emotional labor, but this relationship was found to operate differently at low and high levels of political skill. Specifically, emotional labor was a consequence of political behavior for those low in political skill, but emotional labor reactions from political behavior were neutralized for individuals high in political skill. Implications of these results, strengths and limitations, and directions for future research are discussed. Copyright © 2005 John Wiley & Sons, Ltd.  相似文献   
19.
This article discusses creating a sustainably protective engineered and human management system in perpetuity for sites with long‐lived radiological and chemical hazards. This is essential at this time because the federal government is evaluating its property as assets and attempting to reduce its holdings, while seeking to assure that health and ecosystems are not put at risk. To assist those who have a stake in the remediation, management, and stewardship of these and analogous privately owned sites, this article discusses current end‐state planning by reviewing the federal government's accelerated efforts to reduce its footprint and how those efforts relate to sustainability. The article also provides a list of questions organized around six elements of risk management and primary, secondary, and tertiary disease and injury prevention. Throughout the article, the U.S. Department of Energy (DOE) is used as an example of an organization that seeks to reduce its footprint, manage its budget, and be a steward of the sites that it is responsible for. However, the approach and questions are appropriate for land controlled by the Department of Defense (DOD), the General Services Administration (GSA), and other public and private owners of sites with residual contamination. © 2005 Wiley Periodicals, Inc.  相似文献   
20.
An exploration of place as a process: The case of Jackson Hole, WY   总被引:1,自引:1,他引:1  
A variety of theoretical positions and frameworks have been advanced to account for how places become “places”—in other words, how places become meaningful. Most existing frameworks share the idea that a place is a complex concept, given life by people attaching meaning to a physical setting in a variety of ways. This paper explores how places evolve as ever-shifting points of meaning that mark changes in people's lives in response to a variety of influences. Data collected from in-depth personal interviews in and around Jackson, Wyoming, are used to explore the conceptualization of place as a process, rather than a static entity. Three key themes or dimensions that were frequently described by interviewees are examined: (1) life stage/course, (2) searching for a feeling, and (3) commitment to a place. Not only did some respondents describe what made places important to them (the components, such as social ties, or favorite activities), they also described how these attributes worked together to create and maintain place meanings over the course of their lives. During the interviews, it was noted that these dimensions and processes were expressed explicitly as well as implicitly.  相似文献   
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