Renatal diagnosis of mosaic tetrasomy 12p/trisomy 12p by fluorescent in situ hybridization in amniotic fluid cells: A case report of pallister–killian syndrome

A prenatally detected case of a rare mosaic tetrasomy 12p/trisomy 12p is reported, presenting as the well-known accessory isochromosome 12p and a supernumerary single 12p marker in 17/24 and 6/24 clones of cultured amniotic fluid cells, respectively. The chromosomal nature of both marker chromosomes was investigated in cultured amniotic fluid cells by fluorescent in situ hybridization with various probes: the 12-centromeric probes pa12H8 and D12Z3, a whole chromosome 12 paint, and the chromosome 12p-specific paint M28. DNA analysis revealed a maternal origin of the extra 12p material. After counselling, the parents requested termination of pregnancy. Inspection and autopsy of the fetus revealed many of the dysmorphisms and internal structural abnormalities of the Pallister–Killian syndrome.     

Prenatal diagnosis of Freeman–Sheldon syndrome (whistling face)

The diagnosis of Freeman–Sheldon syndrome was made by ultrasonographic evaluation of a 20-week fetus with a positive family history. The ultrasonographic features were abnormalities of the extremities and mouth.     

关于既有民用建筑定期安全检测与维修问题的探讨

分析了既有民用建筑的现状，论述了既有民用建筑进行定期安全检测与维修的重要性，建议国家应制定既有民用建筑定期安全检测与维修的相关法规，并加强管理，保证实现工程的设计使用年限及延长建筑物的使用寿命，最大程度地发挥投资效益。     

Immediate prenatal diagnosis of Zellweger syndrome by direct measurement of very long chain fatty acids in chorionic villus cells

We report a gas chromatographic-mass spectrometric method which allows the very long chain fatty acids content of trophoblastic tissue to be directly measured in samples collected by biopsy between 8 and 11 weeks of gestation. This method has been successfully applied to the detection of fetal Zellweger syndrome in two pregnant women who had previously delivered affected infants. In one of them, increased concentrations of C26:0 (0.254 versus 0.108±0.035 μ/mg proteins) and C24:0 (1.32 versus 0.815±0.325 μ/mg proteins) in trophoblast indicated that the fetus had Zellweger syndrome, a diagnosis confirmed by pathological findings after abortion. In the second case, the pregnancy was allowed to proceed, on the basis of normal concentrations of very long chain fatty acids in trophoblastic tissue, and its outcome was actually a healthy newborn.     

风险管理在民航事故预防中的应用

基于对航空安全极端重要性的深层次思考,将危险源辨识、风险评估和风险控制应用到民航事故预防中,对诱发事故的各种潜在因素进行分析、估计和评价,把1994-2003年的民航事故数据应用到风险水平矩阵中,得出人为因素失误和组织失调时的风险是不可接受的;进而提出风险控制措施,使人安全地与技术结合,并融入培训、管理政策或操作程序之中,有效减少人为失误;导致飞机事故的所有因素共同形成一个事故链,针对事故链中的每一个环节采取相应的措施,并协调好他们之间的关系有效地预防民航事故的发生.     

近十年中国民航事故及事故征候的统计分析

对1996—2005年我国民航发生的32起事故及1147起事故征候进行统计分析,其结果表明:我国民航事故和事故征候的万架次率及万时率均呈下降态势,机组、机械和机务原因是我国民航事故和事故征候的主要原因,事故征候的主要类型是鸟击、空中停车、偏出/冲出跑道/场外接地,事故征候发生阶段依次为巡航、起飞、着陆、爬升和进近。该研究成果有助于寻找事故和事故征候成因和规律。明确安全管理重点和难点;采取有效的预警和预控对策。     

我国民爆行业安全管理存在的主要问题及其对策

今年以来，民爆企业重、特大事故多发，安全生产日趋严峻。随着我国国民经济的快速发展，民爆行业安全问题已日益成为社会关注的热点，也成为安全监管的重点。本文从政府行业监管职责、企业主体责任两个层面存在的突出问题的剖析入手，为进一步强化民爆行业安全监督管理工作，在实施产业技术政策，落实政府监管责任，推进企业技术进步，加强企业基础管理，完善法律法规标准，深化安全专项整治，强化安全许可工作等方面提出了对策建议。     

Evaluation of thermal reaction for two azo compounds by using 20-L apparatus and calorimetry

Azo compounds are widely involved in the industrial processes of dyes, pigments, initiators, and blowing agents. Unfortunately, these compounds have a bivalent unstable –NN– composition, which can be readily broken when the ambient temperature is elevated. Self-accelerating decomposition might cause a runaway reaction and lead to a fire, explosion, or leakage when the cooling system fails or other events occur. This study investigated the explosion properties, thermal stability parameters, and thermal hazard and mechanism of 2,2′–azobisisobutyronitrile (AIBN) and 2,2′–azobis–2–methylbutyronitrile (AMBN). We used a 20-L apparatus, vent sizing package 2, synchronous thermal analysis, and differential scanning calorimetry under explosive, adiabatic, and dynamic conditions to acquire the explosive curves, thermal curves, and thermodynamic parameters of the substances. Moreover, the differential isoconversional method (Friedman method) and ASTM E698 equation were employed to obtain the apparent activation energy E_a. All the experimental results revealed that AIBN is more dangerous than AMBN. The E_a of AIBN was lower than that of AMBN. The results can be used to construct an azo compound thermal hazard database for use for searches and reference examples by industry and related research areas.     

Pyrolysis of waste animal fats in a fixed-bed reactor: Production and characterization of bio-oil and bio-char

Several animal (lamb, poultry and swine) fatty wastes were pyrolyzed under nitrogen, in a laboratory scale fixed-bed reactor and the main products (liquid bio-oil, solid bio-char and syngas) were obtained. The purpose of this study is to produce and characterize bio-oil and bio-char obtained from pyrolysis of animal fatty wastes. The maximum production of bio-oil was achieved at a pyrolysis temperature of 500 °C and a heating rate of 5 °C/min. The chemical (GC–MS analyses) and spectroscopic analyses (FTIR analyses) of bio-oil showed that it is a complex mixture consisting of different classes of organic compounds, i.e., hydrocarbons (alkanes, alkenes, cyclic compounds…etc.), carboxylic acids, aldehydes, ketones, esters,…etc. According to fuel properties, produced bio-oils showed good properties, suitable for its use as an engine fuel or as a potential source for synthetic fuels and chemical feedstock. Obtained bio-chars had low carbon content and high ash content which make them unattractive for as renewable source energy.     

Hazard property classification of waste according to the recent propositions of the EC using different methods

Hazard classification of waste is a necessity, but the hazard properties (named “H” and soon “HP”) are still not all defined in a practical and operational manner at EU level. Following discussion of subsequent draft proposals from the Commission there is still no final decision. Methods to implement the proposals have recently been proposed: tests methods for physical risks, test batteries for aquatic and terrestrial ecotoxicity, an analytical package for exhaustive determination of organic substances and mineral elements, surrogate methods for the speciation of mineral elements in mineral substances in waste, and calculation methods for human toxicity and ecotoxicity with M factors.In this paper the different proposed methods have been applied to a large assortment of solid and liquid wastes (>1 0 0).Data for 45 wastes – documented with extensive chemical analysis and flammability test – were assessed in terms of the different HP criteria and results were compared to LoW for lack of an independent classification. For most waste streams the classification matches with the designation provided in the LoW. This indicates that the criteria used by LoW are similar to the HP limit values.This data set showed HP 14 ‘Ecotoxic chronic’ is the most discriminating HP. All wastes classified as acute ecotoxic are also chronic ecotoxic and the assessment of acute ecotoxicity separately is therefore not needed. The high number of HP 14 classified wastes is due to the very low limit values when stringent M factors are applied to total concentrations (worst case method). With M factor set to 1 the classification method is not sufficiently discriminating between hazardous and non-hazardous materials. The second most frequent hazard is HP 7 ‘Carcinogenic’. The third most frequent hazard is HP 10 ‘Toxic for reproduction’ and the fourth most frequent hazard is HP 4 “Irritant – skin irritation and eye damage”. In a stepwise approach, it seems relevant to assess HP 14 first, then, if the waste is not classified as hazardous, to assess subsequently HP 7, HP 10 and HP 4, and then if still not classified as hazardous, to assess the remaining properties.The elements triggering the HP 14 classification in order of importance are Zn, Cu, Pb, Cr, Cd and Hg. Progress in the speciation of Zn and Cu is essential for HP 14. Organics were quantified by the proposed method (AFNOR XP X30-489) and need no speciation. Organics can contribute significantly to intrinsic toxicity in many waste materials, but they are only of minor importance for the assessment of HP 14 as the metal concentrations are the main HP 14 classifiers. Organic compounds are however responsible for other toxicological characteristics (hormone disturbance, genotoxicity, reprotoxicity…) and shall be taken into account when the waste is not HP 14 classified.