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We present the first confirmed case by molecular analysis of a metaphyseal chondrodysplasia, McKusick type, in a 22-week fetus. Two novel compound heterozygous mutations, 64T> A and 79G > T, were found in the highly conserved regions of the RMRP gene. Twenty-two heterozygous g.1018 T> C mutations, two homozygous g.1018 T> C mutations, two heterozygous insertion mutations g.799_g.800insC and one heterozygous insertion mutation g.849_g.850insT were found among 100 normal controls. Careful radiological examination of the fetus for skeletal dysplasia allowed definitive diagnosis, proper genetic counselling and future prenatal diagnosis. Copyright © 2006 John Wiley & Sons, Ltd.  相似文献   
134.
Enteric duplication cyst is a congenital abnormality that is believed to arise from abnormal recanalization of the bowel during embryogenesis. Previous reports suggest that the condition may be suspected prenatally by sonographic demonstration of an intra-abdominal cystic mass in the second and third trimesters. We present the sonographic features of a fetus with ileal duplication cyst at 12 weeks of gestation, which show that the condition may present in the first trimester of pregnancy. Copyright © 2002 John Wiley & Sons, Ltd.  相似文献   
135.
模糊层次综合评判法在火电厂选址决策中的应用   总被引:1,自引:0,他引:1  
根据影响火电厂选址的因素较多、相互之间具有不确定性和模糊性这一特点,结合火电厂选址的实际情况,建立了火电厂选址综合效益评价指标体系;运用模糊层次综合评判法确定了各指标的权重分配;从可持续发展的角度对火电厂选址综合效益进行了评价;经过专家投票,将各因素层次化,得出模糊评价结果,即找到综合效益最好的厂址;并通过实例验证了该方法的可行性。可为火电厂决策者投资建设项目提供依据。  相似文献   
136.
热镀锌板表面硅烷处理耐蚀性能的研究   总被引:4,自引:0,他引:4  
将镀锌钢板经表面处理后涂覆硅烷,研究成膜后的耐蚀性能。在3.5%NaCl溶液中的电化学极化曲线测试和交流阻抗表明,涂覆硅烷膜后,硅烷膜的存在能够明显地抑制腐蚀过程中的阴极和阳极反应,使电荷转移阻力大增,显著降低了锌的腐蚀速率,耐蚀效果较好。初步讨论了硅烷膜的成膜及耐蚀机理。  相似文献   
137.
氟碳涂层体系在我国水环境中防腐性能研究   总被引:1,自引:0,他引:1  
通过现场曝露试验,获得氟碳涂层体系在我国淡水、海水和盐湖水环境中4年腐蚀结果,总结了它们的腐蚀行为。无论是在自然环境下的曝露试验,还是实验室条件下的盐雾试验和紫外光老化试验结果,都表明氟碳涂层具有优异的耐候性和耐水性;水气渗透性试验也表明氟碳涂层具有较低的渗水率;氟碳涂层虽具有低的表面能,但在淡水和海水环境中,其抗水生物附着性能仍不理想。  相似文献   
138.
This is the first detailed characterization of the airborne bacterial profiles in indoor environments. Two restaurants were selected for this study. Fifteen genera of bacteria were isolated from each restaurant and identified by three different bacterial identification systems including MIDI, Biolog and Riboprinter®. The dominant bacteria of both restaurants were Gram-positive bacteria in which Micrococcus and Bacillus species were the most abundant. Most bacteria identified were representative species of skin and respiratory tract of human, and soil. Although the bacterial levels in these two restaurants were below the limit of the Hong Kong Indoor Air Quality Objective (HKIAQO) Level 1 standard (i.e., < 500 cfu/m3), the majority of these bacteria were opportunistic pathogens. These results suggested that the identity of airborne bacteria should also be included in the IAQ to ensure there is a safety guideline for the public.  相似文献   
139.
Environmental Modeling & Assessment - The São Francisco River Basin plays a critical role in the hydroelectrical operational planning of Brazil. Understanding the hydroclimatic dynamic...  相似文献   
140.
Soman is one of the most toxic nerve agents of the known chemical warfare agents. There is actually few knowledge on the behavior of soman in the event of an environmental contamination. It is in particular unknown whether soman remains on a given surface, evaporates into air, or degrades. Here, we studied the evaporation of soman deposited on silica sand using a laboratory-sized wind tunnel, thermal desorption, and gas chromatography. We also investigated the degradation of soman on silica sand by 31phosphorus solid state magic angle spinning nuclear magnetic resonance and gas chromatography–mass spectrometry. Results show that a drop of soman on silica sand spreads laterally while evaporating. The maximum vapor concentration was found when the spreading of the soman drop was maximum on the surface. Simultaneously, soman was absorbed gradually into the pore of sand and degraded to O-pinacolyl-methylphosphonic acid following a pseudo-first-order rate reaction over weeks. To our best knowledge, this is the first report on the fate of soman in a sandy environment.  相似文献   
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