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Fabry disease is an X-linked inborn error of metabolism wherein deficiency of a lysosomal enzyme results in systemic deposition of glycosphingolipids. Storage deposition, and hence pathological disease, occurs preferentially in renal glomerular and tubular epithelial cells, myocardial cells, heart valve fibrocytes, neurons of dorsal root ganglia, and in endothelial smooth muscle cells of blood vessels. Thus, Fabry disease is a multi-system disorder, albeit with considerable phenotypic heterogeneity in onset and in severity; however, it is progressive, exhibits extensive morbidity, and is life-threatening. Within the past two decades, there has been a radical change in the natural course Fabry disease by virtue of the availability of specific enzyme replacement therapy. Moreover, there has been a concerted effort to better understand the underlying pathology and equally to identify patients prior to the onset of irreversible end-organ damage. It is to be hoped that the future for patients with Fabry disease can be viewed with greater, albeit guarded, optimism. This state-of-the-art textbook attempts to bridge the span of pre-clinical studies, clinical finding, and management options in a readable but comprehensive manner for the medical practitioner as well as the interested non-medical reader.

1993 American Institute of Architects International Architecture Book Award

Explores European civilisation as a concept of twentieth-century political practice and the project of a transnational network of European elites. This title is available as Open Access.

Get ready to discover the great architectural mecca that is Minneapolis and St. Paul. The first comprehensive, illustrated handbook of its kind, AIA Guide to the Twin Cities is the ultimate source to the architectural riches of the metropolitan area. Organized by neighborhood and featuring a wealth of sites--from the highest point on the Minneapolis skyline to the modest St. Paul bungalow vibrant with historical and architectural significance--this invaluable reference has it all: -Illuminating entries for more than 3,000 buildings -Behind-the-scenes details of the structures and their architects -Lively information about local history and regional styles -Highlights of important buildings nearly lost in time -Sixty easy-to-read maps that pinpoint the location of every structure -Dozens of planned walking and driving tours -Over 1,000 photos that illustrate significant buildings and features Retired Pioneer Press architecture critic Larry Millett has spent more than two decades researching and exploring the architectural heritage of the Twin Cities. Millett's AIA Guide to the Twin Cities is your ticket to the best tour in town. Sponsored in part by the American Institute of Architects Minnesota. Larry Millett has written extensively about Twin Cities architecture. His books include Lost Twin Cities, Twin Cities Then and Now, and Strange Days, Dangerous Nights (all MHS Press), as well as a series of mystery novels featuring Sherlock Holmes.

This book, drawn from the Global Trade Analysis Project (GTAP), aims to help readers conduct quantitative analysis of international trade issues in an economy-wide framework. In addition to providing a succinct introduction to the GTAP modeling framework and data base, this book contains seven of the most refined GTAP applications undertaken to date, covering topics ranging from trade policy, to the global implications of environmental policies, factor accumulation and technological change.