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  • Haagenrud, Svein, et al. (author)
  • CIB W106 Geographical Information Systems : Work Period Report
  • 2005
  • In: 10th International conference on durability of building materials and components.
  • Conference paper (peer-reviewed)abstract
    • In the building and construction sector all features are location based. The world of geographic information and application orientation is moving extremely fast, thus challenging the building sector to facilitate and implement this new technology and applications. In order to deal with these issues CIB in 1996 established the task group CIB/TG20-GIS. Based on the report CIB 256 from this group and its recommendations, CIB in 2000 established the working commission CIB W106 - Geographical Information Systems., with the overall objectives to provide an international platform for R&D of GIS applications for the built environment, and to promote and encourage the use of GIS in the building sector. The W106 has members from 14 countries/organisations and will present its final report for work period 2001-04 due for the 10DBMC conference in 2005. The work is divided into the following four Tasks: TG1- GIS-requirements and availability of geographic standards-, -data and infra -structures, TG2- GIS-based analysis and modelling of flow and distribution of materials in the built environment, TG3- Spatial dynamic modelling for Simulation of the interaction between the natural and the built environment, TG4- GIS in Education and Info sources. Objectives and work programme for each of these tasks are given and illustrated with examples, taken from state .of-the-art reports on the use of GIS elaborated by the participating countries/organisations. With the rapid development of IFC based standards for digital object oriented models of building products there is a huge need for property sets, such as environmental exposure data, reference service life, service life models, factor distributions, LCA and LCC data, which can be linked directly to the building elements. The significant drive within the AEC/IFC to provide for relevant location based data (GIS) via IFC format will be a major facilitator for access to relevant durability data on the specific building site. It is concluded that time is ripe for a broad implementation of GIS based applications in the building sector. Hence, it is recommended that the work programme of W106 for the coming working period includes a focus on support for an IFC based fully integrated design and planning process for the built environment, as well as a close link to the European based R&D frameworks for integrated life cycle management of the built environment.
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3.
  • Wraith, James E., et al. (author)
  • Recommendations on the diagnosis and management of Niemann-Pick disease type C
  • 2009
  • In: Molecular Genetics and Metabolism. - : Elsevier BV. - 1096-7192. ; 98:1-2, s. 152-165
  • Journal article (other academic/artistic)abstract
    • Niemann-Pick disease type C (NP-C) is a lysosomal storage disease in which impaired intracellular lipid trafficking leads to excess storage of cholesterol and glycosphingolipids in the brain and other tissues. it is characterized clinically by a variety of progressive, disabling neurological symptoms including clumsiness, limb and gait ataxia, dysarthria, dysphagia and cognitive deterioration (dementia). Until recently, there has been no disease-modifying therapy available for NP-C, with treatment limited to supportive measures. In most countries, NP-C is managed through specialist centers, with non-specialist support provided locally. However, effective patient Support is hampered by the absence of national or international clinical management guidelines. In this paper, we seek to address this important gap in the Current literature. An expert panel Was convened in Paris, France in January 2009 to discuss best care practices for NP-C. This commentary reviews Current literature on key aspects of the clinical management of NP-C in children, juveniles and adults, and provides recommendations based on consensus between the experts at the meeting. (C) 2009 Elsevier Inc. All rights reserved.
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