Sanfilippo D syndrome: Correction of glucosamine-6-sulphatase deficiency following fibroblast culture in chang's media

The de-O-sulphation of α-linked glucosamine-6-sulphate residues in heparan sulphate requires a specific sulphatase, glucosamine-6-sulphatase, which has been shown to be deficient in tissues of Sanfilippo D, or mucopolysaccharidosis type IIID (MPS IIID), patients. MPS IIID fibroblasts cultured in Basal Eagle's medium supplemented with either fetal calf serum or heat-inactivated fetal calf serum, MDCB or Ultraserg media had residual glucosarnine-6-sulphatase activities towards a heparin-derived trisaccharide substrate, O-(α-N-acetylglucosamine-6-sulphate)-(1→4)-L -O-(α-iduronic acid-2-sulphate)-(1→4)-D -O-2,5-anhydro [1-³H]mannitol-6-sulphate, GlcNAc6S-IdoA2S-anM6S, which were less than 1 per cent of the normal range for fibroblasts cultured in Basal Eagle's medium supplemented with fetal calf serum. However, the glucosamine-6-sulphatase activities of MPS IIID fibroblasts grown in Chang's medium were similar to the activities in normal control fibroblasts which were cultured in Basal Eagle's medium. These results indicate that caution is required for prenatal diagnosis of MPS IIID patients using chorionic villi or amniotic cells cultured in Chang's medium.     

Antenatal diagnosis of a de novo paracentric inversion of chromosome 11

A de novo paracentric inversion of chromosome 11 identified through antenatal diagnosis is described along with long-term follow-up information on the child and discussion of reported experiences with de novo inversions.     

e-Serem – A Web-Based Manual For The Estimation of Emission Reductions From JI and CDM Projects

This paper presentse-SEREM (Smart Emission ReductionEstimation Manual), a cost-free, easilyaccessed and updated, web-based manual forestimating emission reductions from Joint Implementation (JI) andClean Development Mechanism (CDM) projects. e-SEREM's main functions arethe selection of a benchmark for a specificproject type in the power or heat sectorand the calculation of the annual andcumulative emission reductions accrued bythis project for its crediting lifetime.e-SEREM was developed in order to test itsapplicability and practicality in assistingproject developers and evaluators toelaborate baselines easily and calculatethe emission credits earned by candidate JIor CDM projects hosted in several countries.     

Practical environmental monitoring of major construction projects

Independent teams undertook environmental monitoring of particular concentrations of major construction projects forming part of Hong Kong’s U.S. $20 billion airport infrastructure programme located in dense urban areas. The team combination of environmental specialists with experienced civil engineers enabled pragmatic mitigation measures to be developed and accepted by the construction personnel with the result that potentially significant adverse impacts were averted. The authors discuss the mechanism and success of this innovative approach.     

Particle monitoring techniques for water treatment applications

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Industrial ecology: a new field or only a metaphor?

In the 10 years since industrial ecology first became a topic of academic interest, it has grown as a field of inquiry and has produced a community of practice in several sectors including academia, business, and government. Even as the shape of industrial ecology becomes clearer, questions remain as to its lasting power beyond the metaphor that gave it its distinctiveness. This paper examines the development of industrial ecology and assesses its progress towards becoming a field of academic inquiry. And, in a related analysis, I look at the progress industrial ecology has made in establishing itself as an institutional (cultural) basis for action in the above sectors. Ideas like industrial ecology must become institutionalized if they are to have much effect on the reality of everyday activities.     

Hydrogen storage and distribution systems

Hydrogen storage and transportation or distribution is closely linked together. Hydrogen can be distributed continuously in pipelines or batch wise by ships, trucks, railway or airplanes. All batch transportation requires a storage system but also pipelines can be used as pressure storage system. Hydrogen exhibits the highest heating value per weight of all chemical fuels. Furthermore, hydrogen is regenerative and environment friendly. There are two reasons why hydrogen is not the major fuel of toady’s energy consumption: First of all, hydrogen is just an energy carrier. And, although it is the most abundant element in the universe, it has to be produced, since on earth it only occurs in the form of water. This implies that we have to pay for this energy, which results in a difficult economic task, because since the industrialization we are used to consuming energy for free. The second difficulty with hydrogen as an energy carrier is the low critical temperature of 33 K, i.e. hydrogen is a gas at room temperature. For mobile and in many cases also for stationary applications the volumetric and gravimetric density of hydrogen in a storage system is crucial. Hydrogen can be stored by six different methods and phenomena: high pressure gas cylinders (up to 800 bar), liquid hydrogen in cryogenic tanks (at 21 K), adsorbed hydrogen on materials with a large specific surface area (at T < 100 K), absorbed on interstitial sites in a host metal (at ambient pressure and temperature), chemically bond in covalent and ionic compounds (at ambient pressure), oxidation of reactive metals e.g. Li, Na, Mg, Al, Zn with water. These metals easily react with water to the corresponding hydroxide and liberate the hydrogen from the water. Finally, the metal hydroxides can be thermally reduced to the metals in a solar furnace.     

CLIMATE CHANGE AND NATURAL HAZARDS IN NORTHERN CANADA: INTEGRATING INDIGENOUS PERSPECTIVES WITH GOVERNMENT POLICY

A study of the relationship between natural hazards and climate change in the international context provides the background for a discussion of the expected changes. In the context of this global discussion, this paper reviews the current perspectives of those natural hazards that are likely to be influenced by climate change, using northern Canada as a regional case study. The northern implications of the United Nations Framework Convention on Climate Change are examined, including the status of climate change action by the northern territorial governments, the evolving role of indigenous people, and the responsibility for climate change impacts. The difficulties surrounding natural hazards research in remote locations, and the approaches of indigenous people to natural hazards are then presented. The paper concludes with a suggested policy approach for climate change and natural hazards in northern Canada, underscoring the need for more comprehensive adaptive strategies to complement the current tendency to focus on the mitigation of greenhouse gases produced in this region.