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Description du livre Etat : New. N° de réf. du vendeur 9638942-n
Description du livre Hardcover. Etat : new. N° de réf. du vendeur 9789048190324
Description du livre Etat : New. N° de réf. du vendeur 9638942-n
Description du livre Etat : New. Dieser Artikel ist ein Print on Demand Artikel und wird nach Ihrer Bestellung fuer Sie gedruckt. * first full-length textbook about Fabry disease * includes chapters on the therapeutic management options * complete and stat-of-the-art range of pre-clinical studies * comprehensive chapters on clinical findings * all authors are experts with many years o. N° de réf. du vendeur 5822486
Description du livre Etat : New. N° de réf. du vendeur ABLIING23Apr0316110339682
Description du livre Hardcover. Etat : Brand New. 1st edition. 512 pages. 9.25x6.25x1.25 inches. This item is printed on demand. N° de réf. du vendeur zk9048190320
Description du livre Etat : New. PRINT ON DEMAND Book; New; Fast Shipping from the UK. No. book. N° de réf. du vendeur ria9789048190324_lsuk
Description du livre Buch. Etat : Neu. This item is printed on demand - it takes 3-4 days longer - Neuware -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. 552 pp. Englisch. N° de réf. du vendeur 9789048190324
Description du livre Buch. Etat : Neu. Druck auf Anfrage Neuware - Printed after ordering - 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. N° de réf. du vendeur 9789048190324