Intrafamilial variability in Fraser syndrome

Fraser syndrome (OMIM 219000) is a rare, autosomal recessive disorder characterized by cryptophthalmos, cutanaeous syndactyly, malformations of the larynx and genitourinary tract, craniofacial dysmorphism, orofacial clefting, mental retardation and musculoskeletal anomalies. There is marked interfamilial clinical heterogeneity. However, there is strong phenotypic similarity and concordance of the degree of severity of the disease within a family. We report a family with two cases of Fraser syndrome with marked clinical heterogeneity. One case had lethal phenotype with bilateral renal agenesis, while the other had mild phenotype with normal kidneys. It has not been reported before and highlights the importance of careful screening of pregnancies in families with Fraser syndrome. Copyright © 2007 John Wiley & Sons, Ltd.     

冬季动态槽式堆肥温度的空间变异

以鲜鸡粪、蘑菇渣和污泥按照体积比3∶1∶1混合进行动态堆肥模拟试验.堆肥槽沿物料前进方向分为7个部分,对每个部分按等间距分别做5个水平方向上切分和5个垂直方向上切分,在形成的125个交叉点上进行温度监测.研究结果表明,第1天的混合物料温度在同一层中变异很小,不同层之间略有差异.随着动态过程的进行,同一层温度变异逐渐增大,从第一天相差1～3℃,增加到相差30～40℃,靠近墙体的堆料温度较低,远离墙体的温度较高.随着堆肥时间延长,差异增大.机械翻堆能起到通风的作用,同时使每一个堆方的堆料在纵向方向上上下混合,但达不到横向混匀,因此,靠近墙体两侧的堆料始终处于较低的温度,只有中部能达到较高的温度,以堆肥温度50℃作为无害化指标,自墙体向中心方向的1m为没有达到无害化厚度,无害化体积占堆肥总体积50%.整个动态堆肥过程符合二级动力学方程.     

青岛市大气中醛酮类化合物的分析及浓度变化

自1997年12月～1998年7月,在覆盖整个青岛市区布设了6个采样点,用2,4-二硝基苯肼HPLC法测定了11种醛酮类羰基化合物在不同季节、不同时间的浓度.青岛市大气中甲醛、乙醛、丙酮及醛酮总量的平均浓度冬季分别为3.64,4.18,2.50,13.9mg/m3,夏季分别为9.95, 7.00,5.75,26.9mg/m3,夏季的浓度明显高于冬季,青岛市大气中甲醛占总羰基化合物的26.2%～37%,乙醛、丙酮分别占总羰基化合物的26.0%～30.1%和18.0%～21.4%.甲醛+乙醛+丙酮之和占总羰基化合物的74.3%～84.3%,甲醛和乙醛在冬、夏两季的相关性较差(R2=0.43),而乙醛和丙酮在夏季具有较强的相关性(R2=0.7399).     

洞庭湖大气氮湿沉降的时空变异

为研究洞庭湖大气氮素湿沉降的时空变化规律,于2016年1—12月对洞庭湖不同生态区的4个监测点的湿沉降进行了为期1年的观测.结果表明:采桑湖(CSH)、断港头(DGT)、蒋家嘴(JJZ)3个监测点的全年大气氮湿沉降量分别为49.9、38.7和90.9 kg·hm~(-2),其中NO_3~--N沉降量分别占30.3%、25.8%和33.1%,NH_4~+-N沉降量分别占32.9%、41.7%和51.1%,DON沉降量分别占36.8%、32.5%和15.9%;沅江(YJ)监测点7—12月的大气氮湿沉降量为19.0 kg·hm~(-2),NO_3~--N、NH_4~+-N和DON分别占62.2%、25.1%和12.7%,除YJ监测点外,其他各监测点NH_4~+-N沉降量高于NO_3~--N沉降量.湿沉降量与降雨量呈正相关,而湿沉降浓度与降雨量呈负相关;在CSH、DGT和JJZ监测点,湿沉降量均在春季最高,分别占全年总沉降量的54.4%、60.2%和49.7%,JJZ监测点在各个季节的总氮沉降量均高于CSH和JJZ监测点.     

阜新露天煤矿排土场边坡土壤质量分异特征

以阜新露天煤矿排土场边坡为对象,分析坡向、坡位和恢复年限对土壤物理、化学和生物学性状的影响,并采用土壤质量综合指数法,研究排土场边坡植被恢复过程中土壤质量变化规律.结果表明,排土场边坡土壤含水量、有机质、碱解氮和速效磷含量、pH值以及脲酶、碱性磷酸酶和蔗糖酶活性从坡上到坡下均逐渐增加;恢复10a阴坡土壤含水量、水稳性团聚体含量、碱性磷酸酶、过氧化氢酶和蔗糖酶活性高于阳坡;随着恢复年限增加,土壤水稳性团聚体和速效磷含量及阴坡碱性磷酸酶活性提高,而土壤pH值、阳坡碱解氮含量及脲酶和过氧化氢酶活性下降.排土场边坡土壤质量表现为阴坡>阳坡、坡下>坡中>坡上的变化规律;随着恢复年限增加,阳坡坡上和坡中及阴坡土壤质量增加,而阳坡坡下下降.以上结果说明,坡位和坡向是影响排土场边坡土壤质量的重要因素,应根据具体生境特点,采取不同治理措施,才能完成生态恢复.     

Temporal variability of visibility and its parameterizations in Ningbo, China

Simultaneous and continuous measurements of visibility, meteorological parameters and air pollutants were carried out at a suburban site in Ningbo from June 1, 2013 to May 31,2015. The characteristics of visibility and their relationships with air pollutants and meteorological factors were investigated using multiple statistical methods. Daily visibility ranged from 0.6 to 34.1 km, with a mean value of 11.8 km. During the 2-year experiment,43.4% of daily visibility was found to be less than 10.0 km and only 9.2% was greater than 20.0 km. Visibility was lower in winter with a frequency of 53.4% in the range of 0.0–5.0 km.Annual visibility had an obvious diurnal variation, with the lowest and highest visibility being 7.5 km at approximately 06:00 local time and 15.6 km at approximately 14:00 local time, respectively. Multiple correspondence analysis(MCA) indicated that the different ranges of visibility were significantly affected by different levels of pollutants and meteorological conditions. Based on the analyses, visibility was found to be an exponential function of PM_(2.5) concentrations within a certain range of relative humidity. Thus, nonlinear models combining multiple linear regressions with exponential regression were subsequently developed using the data collected from June 2014 to May 2015, and the data from June 2013 to May 2014 was used to evaluate the performance of the model. It was demonstrated that the derived models can quantitatively describe the relationships between visibility, air quality and meteorological parameters in Ningbo.     

Implementing climate variability adaptation at the community level in the Amazon floodplain

The need to design measures for adapting to climate change is increasingly recognized as important and has encouraged research on the role of local ecological knowledge (LEK) in supporting adaptation. Studies of how LEK can help adapt to increasing climate variability remain limited. This article develops an approach through which the process of adaptation can be tracked at a community level. We describe how community residents in the Amazon floodplains incorporate natural hydrologic and ecological processes into their management systems to optimize ecosystem functioning.We describe two case studies where LEK is used as a resource by small-scale fisher-farmers in the Amazon floodplains to adapt to the increasing impacts on their livelihoods generated by changing climate patterns. This article draws on local histories and seeks to identify the critical factors that either facilitate or impede household ability to reduce their vulnerability. We found that the LEK of small fisher-farmers has facilitated the adaptation of a resource management system to optimize production across a broad range of floodplain habitats and conditions. There are, however, significant challenges to operationalizing these approaches, including an absence of systematically collected data on adaptation strategies and outcomes. In addition, local people must be integrated into policymaking processes so their knowledge can contribute to the design of locally appropriate policies for adapting to the impacts of climate related events.     

Vulnerability to the health effects of climate variability in rural southwestern Uganda

Vulnerability to the health impacts of climate change will be shaped by the existing burden of ill- health and is expected to be highest in poor and socio-economically marginalized populations. Sub-Saharan Africa, in particular, is considered a highly vulnerable region. This paper analyses the vulnerability and adaptive capacity of rural Bakiga communities in southwestern Uganda to climate-sensitive health risks. The objectives were threefold: i) identify key climate-sensitive, community-identified health priorities; ii) describe and characterize determinants of sensitivity to these health priorities at the individual, community and regional levels; and iii) assess the adaptive capacity of Bakiga. Data collection employed a combination of individual and key informant interviews, biographies, future storylines, and Photovoice. Three key health risks were identified by the study communities (malaria, food insecurity, and gastrointestinal illnesses) – all affected by local climatic and environmental conditions, livelihoods, land use changes, and socio-economic conditions. Adaptation within these communities is dependent on their capacity to reduce sensitivities to identified health challenges among the potential of increasing exposures. Crop diversification, reducing deforestation, expanding of livestock rearing, transfer of traditional knowledge, and access to affordable health services are among potential strategies identified. We demonstrate significant existing vulnerabilities to present day climate-related health risks and highlight the importance of non-climatic processes and local conditions in creating sensitivity to health risks. Our place-based understanding is useful to inform interventions or policies aimed to reduce exposure and sensitivity and support adaptive capacity as the conditions these communities face are consistent with many other sub-Saharan African countries.     

Interannual variability in acidic deposition on the Mt. Mitchell area forest

Recently the forest decline at high elevation mountains in the eastern U.S. has been suggested to be associated with the deposition of acidic substances on the forest canopy through dry, wet and cloud deposition pathways. To determine the relative importance of these deposition mechanisms, a field study was initiated in May 1986, at Mt. Mitchell, NC. Since Mt. Mitchell is frequently immersed in clouds (immersion time being in the range of 28–41%), our investigations were primarily focused on the collection of cloud water and the monitoring of meteorology and ambient air quality. The precipitation data and related chemistry were obtained from a nearby NADP (National Atmospheric Deposition Program) site (Clingman's Peak). To estimate the dry and cloud deposition, the deposition velocities for gases and the rates of cloud deposition for cloud droplets are calculated with the ATDD (Atmospheric Turbulence and Diffusion Division, National Oceanic and Atmospheric Administration) model and a cloud deposition model (CDM), respectively. The wet deposition is obtained from NADP annual reports. Computations show the deposition velocities for SO₂, NO₂ and O₃ to be in the range of tenths of cm s⁻¹. The mean rate of cloud deposition is about 0.13–0.21 mm h⁻¹. The rainfall ranged from 40 to 60 cm during the growing seasons (from mid-May to the end of September) of 1986–1988. Using these deposition parameters and the 3 year database, the deposition fluxes of sulfur (S) compounds are found primarily contributed through cloud capture mechanism (60%) followed by incident precipitation (25%) and dry deposition (15%). As to the deposition fluxes of nitrogen (N) compounds, cloud, wet and dry deposition contributedf about 50%, 25% and 25%, respectively. A comparison of deposition estimates at Mt. Mitchell with those at other sites shows that the sulfate deposition at sites exceeding 1200 m MSL in elevation in Bavaria, F.R.G., and the eastern U.S. is a almost identical within error limits. Reasons for large uncertainties in deposition estimates are also discussed as the mechanisms for redistribution of the deposited material on the forest canopy.     

城市表层土壤重金属与磁化率的多尺度空间变异分析

采集徐州市城区167个表层土壤的样品,并分别测定了磁化率χ的数值及其5种重金属(Cu、Zn、Mo、Cd和Pb)的含量.利用多元因子克里金分析上述研究变量在局部尺度(1 km变程)和区域尺度(5.5 km变程)的空间变异特征.研究表明,徐州城市表土中的磁化率与重金属在局部尺度上主要受到交通、工业和农业等人为因素的影响,在区域尺度上,则以土壤母岩等自然因素为主导.磁化率#与Cu、Zn、Mo、Cd、Pb在局部尺度上的结构相关性较高,在此尺度内选择适当的采样间隔,有助于提高磁化率作为上述重金属污染替代指标进行空间制图的精度.     

Adaptation strategies of climate variability impacts on alpine grassland ecosystems in Tibetan Plateau

Northern Tibet is the headstream region for the Yangtze, Salween River, Mekong River, and numerous other inflowing rivers and high mountain lakes. Sustaining the environmental conditions in the region is of vital importance for Tibet and the whole of China. The alpine grassland ecosystem in Northern Tibet is the most important ecosystem and extremely sensitive to climate change and human activity. In this study, we analyzed the characteristics of climate variability based on observed meteorological data and future climate scenarios, and reviewed the impact of climate variability and to explore adaptation strategies of alpine grassland in Northern Tibet. The result showed that the annual mean temperature has increased by 0.31 °C·10a^?1 while the annual total precipitation has increased by 14.6 mm·10a^?1 with high inter-annual and inter-seasonal fluctuations in Northern Tibet from 1961 to 2008. The rising trends of temperature and precipitation would be continued and the aridity indices showed a decreasing trend in the future, which potentially predicts that the climate in Northern Tibet becomes warmer and dryer. The climate variability results the melting of glaciers, the expansion of inland high mountain lakes and the negative impacts on alpine grassland in recent years. In order to adapt to such possible future climate changes, the alpine grassland water-saving irrigation was recommended as key adaptation measure and also rational grazing management, alpine grassland fencing and artificial grass planting were selected as adaptation measures, to lower the negative impacts of climate variability on the alpine grassland ecosystem in Northern Tibet.     

东苕溪水体氮、磷形态分析及其空间差异性

于2009~2011年在东苕溪开展了4次全流域野外调研,系统分析了东苕溪水体氮、磷形态特征及其空间差异性.东苕溪可分为上游、中-上游、中-下游3个河段,分别具有山溪性河流、城镇影响河流、平原型河道特征.结果表明,水体中氮以溶解态为主,硝态氮(NO₃^--N)是溶解态总氮(DTN)的主要形态;颗粒态磷比例略高,溶解态总磷(DTP)的相对贡献沿程降低.溶解态有机氮(DON)、磷(DOP)分别占DTN和DTP比例的22%、42%,且TN与DON、TP与DOP之间显著正相关.中-上游河段的氨氮(NH₄⁺-N),DOP和溶解性正磷酸盐(PO₄^3--P)含量最高且电导率与NH₄⁺-N、DOP之间存在显著正相关,表明城镇污、废水排放影响水体氮、磷含量及形态特征;浊度与各形态磷之间均存在极显著正相关,河段下游开矿、行船及挖沙引起的矿质颗粒输入或沉积物再悬浮是磷素的重要来源,但水体中部分溶解态磷吸附在矿质颗粒表面形成胶体物质,导致中-下游河段DTP略有降低.     

渤海夏季海水pH值年际时空变化

 收集渤海断面1978~2010年历年8月海水pH值、海水温度和大连站8月降水量观测资料,结果表明渤海夏季海水pH值年际变化范围为7.86~8.30,尚在适宜水生物生长的范围.采用EOF和最大熵谱分析,渤海夏季表底层海水pH值年际时空变化主要有两种模态,第一模态空间分量是断面南北海域同位相变化,时间分量周期为5.3a;第二模态空间分量是断面南北海域反位相变化,时间分量周期为3.2~10.7a.渤海夏季水温5a左右年际周期变化是影响夏季海水pH值年际变化的主要因素.历年8月降水量(酸雨)和月均黄河口径流量年际周期变化是影响夏季海水pH值年际变化的次要因素.渤海夏季断面海水pH值年际变化并不是规则的周期变化,其他因素也可以影响夏季海水pH值年际变化.     

水文变化条件下农田溪流营养盐滞留效应模拟

以巢湖流域某一典型农田源头溪流为对象,基于溪流水文条件的动态变化性,从水文概率密度模型与营养盐滞留率模型综合集成角度,解析较长时间尺度下营养盐滞留有效流量的动态变化特征.在对水文概率密度模型Monte Carlo随机模拟的基础上,根据先前10次野外示踪实验获得的营养盐吸收速度等数据信息,定量评估溪流营养盐滞留的总体水平,估算最有效流量和等效流量.结果表明,农田溪流渠段的NH₄⁺、PO₄^3-滞留能力总体偏低,相应的期望滞留率分别为0.0671(6.71%)和0.0541(5.41%),最有效流量分别为0.0051,0.0049m³/s,功能等效流量分别为0.044,0.043m³/s.基于溪流营养盐吸收速度明显偏低的客观现状,有必要从溪流形态和河床地貌特征的改造着手,提升溪流水体营养盐滞留能力.