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Fabry Disease
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 Fabr...
Joe Learns about Fabry Disease
Joe's story was developed to help explain Fabry disease and its treatment from the perspective of a ten year old.
The Fabry-Perot Interferometer
The Fabry-Perot Interferometer: History, Theory, Practice and Applications presents an invaluable introduction to the Fabry-Perot interferometer, including a brief overview of its history, a look at its applications, and plenty of practical advice on how to use the instrument.
The Metabolic & Molecular Bases of Inherited Disease
Presents clinical, biochemical, and genetic information concerning those metabolic anomalies grouped under inborn errors of metabolism.
The Political Economy of Hungary
This book explores the political economy of Hungary from the mid-1970s to the present. Widely considered a ‘poster boy’ of neoliberal transformation in post-communist Eastern Europe until the mid-2000s, Hungary has in recent years developed into a model ‘illiberal’ regime. Constitutional checks-and-balances are non-functioning; the independent media, trade unions, and civil society groups are constantly attacked by the authorities; there is widespread intolerance against minorities and refugees; and the governing FIDESZ party, led by Prime Minister Viktor Orbán, controls all public institutions and increasingly large parts of the country’s economy. To make sense of the politico-ec...
Fabry-Perot Interferometers
This book describes the Fabry-Perot interferometer and its variants as well as its use, optimisation and applications. The author begins with an historical perspective on the development of the instrument. Because of the quantitative uses of the device, the text tends to be mostly mathematical in its treatment. However, there is also much practical detail on the use and optimization of the Fabry-Perot interferometer and discussion of its classical uses (such as in metrology) and its contemporary applications (such as in lasers). In addition the book contains a comprehensive bibliography summarizing the extensive literature on the subject. This book will appeal both to high-resolution practitioners, such as spectroscopists, and to the laser community, since the Fabrv-Perot is not only an integral part of the laser but is also usea to characterize its optical and spectroscopic behaviour.
Progressive Brain Disorders in Childhood
A review of childhood neurodegenerative and other progressive but non-degenerative disorders to guide their diagnosis and management.
Muscles in Motion
Comic book superheroes abound in bulging muscles. Glenn Fabry focuses on the musculature of bodybuilders & athletes to offer what most books on figure drawing cannot - guidance specifically for artists who wish to create a convincing superhuman character.
Borders of the Heart
A Christy Award finalist from the best-selling author of War Room! Desperate to escape haunting memories, J. D. Jessup travels from Nashville to Tucson and volunteers on an organic farm. The hardened landowner has one prevailing rule: If J. D. sees an “illegal,” call the border patrol. But when an early morning ride along the fence line leads him to a beautiful young woman named Maria, near death in the desert, his heart pulls him in another direction. Longing to atone for the choices that drove him to Tucson, J. D. hides her and unleashes a chain of deadly events he could never have imagined. Soon they are running from a killer and fighting for their lives. As secrets of their pasts emerge, J. D. realizes that saving Maria may be the only way to save himself.
Almost Heaven
Billy, a gifted mandolin player in Dogwood, West Virginia, lives his life as an offering to his divine creator, and when Malachi, an angel sent to observe Billy, begins to see the bigger picture of how each painful step Billy takes is a note added to a beautiful symphony, it will forever change the lives of those who hear it.
