Assessment of Alternative Energy/Environment Futures for Austria, 1977-2015: An Executive Summary

This report was prepared to complement a presentation made October 25, 1977 at IIASA. The presentation, titled "An Executive Briefing Session", was designed to present the final results of a thirteen-month study of the Austrian Energy/Environment System to leaders in Austrian government, industry, and science. This written documentation of the results (of which a German translation is also available) presents in a brief form the final conclusions of this study. The study results provide a comprehensive spatial and sectoral description of Austrian energy consumption, and examine alternative energy and environmental policy strategies. This report, however, is only a summary and a more complete description will appear in Research Report form in 1978

Assessment of Alternative Energy/Environment Futures for Austria: 1977-2015

The Austrian Regional Energy/Environment Study is the fourth in a series of IIASA studies on regional energy and environmental systems. The regions studied previously were the German Democratic Republic, the Rhone-Alpes Region in France and the state of Wisconsin in the U.S.A. The Austrian case study, regional in scope, complements the work of the IIASA Energy Systems Program which focuses primarily on global aspects of energy. This report presents the major results of the l5-month, Austrian case study, which examines alternative energy futures and strategies for Austria and some of their environmental implications. A secondary objective is the development of appropriate concepts and methods for energy/environment management and policy design in Austria

Assessment of Alternative Energy/Environment Futures for Austria 1977-2015: Final Summary Report

This study had two primary objectives: (1) To examine alternative energy futures and strategies for Austria and to consider some of their environmental implications. (2) To investigate and apply appropriate concepts and . methodologies for energy/environment management and policy design in Austria. The establishment of these objectives was based upon the conviction that in Austria, as in most regions and nations of the world, there is an urgent need for the development and application of methods for studying regional energy systems and for testing the impact of alternative policies. In view of the major role which energy plays in the determination of environmental quality, this study was designed to aid in the integration of energy and environmental management from a systems perspective. "Regional," in the context of our previous studies, is not strictly defined as subnational or as a specific class of geographic units; rather, it refers to a region, appropriately bounded so that it is possible to speak of energy and environmental systems from a physical, socioeconomic, or administrative perspective, or from all three. At the beginning of this study, we intended to limit its scope to a selected few Austrian Lander (states); we quickly realized that Austria's size and vigorous interregional links precluded anything less than a national study

The Wisconsin-IIASA Set of Energy/Environment (WISE) Models for Regional Planning and Management: An Overview

This report presents an overview of the analytical framework and quantitative methods used in the IIASA case studies on Regional Energy/Environment Management and Planning. Its purpose is to summarize the structure of the models, to provide a complete listing of the sources of more detailed model and data descriptions, and to indicate how the models are integrated to provide a foundation for regional energy/environment policy maker analysis. The audience for the report includes managers, planners, technical advisors, and modelers. The set of models used in the research project encompasses socioeconomic links to the energy system; energy demand in the residential, industrial, commercial/service, agricultural and transportation sectors; the energy supply sector, environmental impacts associated with the energy system; and policy makers' preferences. The report gives a brief description of the purpose and general structure of each model, data requirements, examples of input and output, and model limitations. As a whole, the models integrate information about energy flows in a region to simulate the energy system and its relationship to other regional variables, e.g., demographic and economic trends and the environment

Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterised by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However, approximately half of children with sJIA develop destructive, long-standing arthritis that appears similar to other forms of JIA. Using genomic approaches, we sought to gain novel insights into the pathophysiology of sJIA and its relationship with other forms of JIA. METHODS: We performed a genome-wide association study of 770 children with sJIA collected in nine countries by the International Childhood Arthritis Genetics Consortium. Single nucleotide polymorphisms were tested for association with sJIA. Weighted genetic risk scores were used to compare the genetic architecture of sJIA with other JIA subtypes. RESULTS: The major histocompatibility complex locus and a locus on chromosome 1 each showed association with sJIA exceeding the threshold for genome-wide significance, while 23 other novel loci were suggestive of association with sJIA. Using a combination of genetic and statistical approaches, we found no evidence of shared genetic architecture between sJIA and other common JIA subtypes. CONCLUSIONS: The lack of shared genetic risk factors between sJIA and other JIA subtypes supports the hypothesis that sJIA is a unique disease process and argues for a different classification framework. Research to improve sJIA therapy should target its unique genetics and specific pathophysiological pathways

S100A12 Serum Levels and PMN Counts Are Elevated in Childhood Systemic Vasculitides Especially Involving Proteinase 3 Specific Anti-neutrophil Cytoplasmic Antibodies

Objectives: Chronic primary systemic vasculitidies (CPV) are a collection of rare diseases involving inflammation in blood vessels, often in multiple organs. CPV can affect adults and children and may be life- or organ-threatening. Treatments for adult CPV, although effective, have known severe potential toxicities; safety and efficacy of these drugs in pediatric patients is not fully understood. There is an unmet need for biologic measures to assess the level of disease activity and, in turn, inform treatment choices for stopping, starting, or modifying therapy. This observational study determines if S100 calcium-binding protein A12 (S100A12) and common inflammatory indicators are sensitive markers of disease activity in children and adolescents with CPV that could be used to inform a minimal effective dose of therapy.Methods: Clinical data and sera were collected from 56 participants with CPV at study visits from diagnosis to remission. Serum concentrations of S100A12, C-reactive protein (CRP) and hemoglobin (Hb) as well as whole blood cell counts and erythrocyte sedimentation rate (ESR) were measured. Disease activity was inferred by physician's global assessment (PGA) and the pediatric vasculitis activity score (PVAS).Results: Serum concentrations of standard markers of inflammation (ESR, CRP, Hb, absolute blood neutrophil count), and S100A12 track with clinically assessed disease activity. These measures—particularly neutrophil counts and sera concentrations of S100A12–had the most significant correlation with clinical scores of disease activity in those children with vasculitis that is associated with anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3.Conclusions: S100A12 and neutrophil counts should be considered in the assessment of disease activity in children with CPV particularly the most common forms of the disease that involve proteinase 3 ANCA.Key messages:- In children with chronic primary systemic vasculitis (CPV), classical measures of inflammation are not formally considered in scoring of disease activity.- Inflammatory markers—specifically S100A12 and neutrophil count—track preferentially with the most common forms of childhood CPV which affect small to medium sized vessels and involve anti neutrophil cytoplasmic antibodies (ANCA) against proteinase-3

Novel Sulfated Polysaccharides Disrupt Cathelicidins, Inhibit RAGE and Reduce Cutaneous Inflammation in a Mouse Model of Rosacea

Rosacea is a common disfiguring skin disease of primarily Caucasians characterized by central erythema of the face, with telangiectatic blood vessels, papules and pustules, and can produce skin thickening, especially on the nose of men, creating rhinophyma. Rosacea can also produce dry, itchy eyes with irritation of the lids, keratitis and corneal scarring. The cause of rosacea has been proposed as over-production of the cationic cathelicidin peptide LL-37.We tested a new class of non-anticoagulant sulfated anionic polysaccharides, semi-synthetic glycosaminoglycan ethers (SAGEs) on key elements of the pathogenic pathway leading to rosacea. SAGEs were anti-inflammatory at ng/ml, including inhibition of polymorphonuclear leukocyte (PMN) proteases, P-selectin, and interaction of the receptor for advanced glycation end-products (RAGE) with four representative ligands. SAGEs bound LL-37 and inhibited interleukin-8 production induced by LL-37 in cultured human keratinocytes. When mixed with LL-37 before injection, SAGEs prevented the erythema and PMN infiltration produced by direct intradermal injection of LL-37 into mouse skin. Topical application of a 1% (w/w) SAGE emollient to overlying injected skin also reduced erythema and PMN infiltration from intradermal LL-37.Anionic polysaccharides, exemplified by SAGEs, offer potential as novel mechanism-based therapies for rosacea and by extension other LL-37-mediated and RAGE-ligand driven skin diseases

Gut mucosal DAMPs in IBD: From mechanisms to therapeutic implications

Endogenous damage-associated molecular patterns (DAMPs) are released during tissue damage and have increasingly recognized roles in the etiology of many human diseases. The inflammatory bowel diseases (IBD), ulcerative colitis (UC) and Crohn’s disease (CD), are immune-mediated conditions where high levels of DAMPs are observed. DAMPs such as calprotectin (S100A8/9) have an established clinical role as a biomarker in IBD. In this review, we use IBD as an archetypal common chronic inflammatory disease to focus on the conceptual and evidential importance of DAMPs in pathogenesis and why DAMPs represent an entirely new class of targets for clinical translation. </p