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InicioE-BooksE-Books Médicos Life with Epidermolysis Bullosa (EB): Etiology, Diagnosis, Multidisciplinary Care and Therapy
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Monoclonal Antibodies: Methods and Protocols

Life with Epidermolysis Bullosa (EB): Etiology, Diagnosis, Multidisciplinary Care and Therapy

Product details

  • Hardcover: 338 pages
  • Publisher: Springer; 2009 edition (December 8, 2008)
  • Language: English
  • ISBN-10: 3211792708
  • ISBN-13: 978-3211792704
  • Product Dimensions: 6.7 x 0.9 x 9.7 inches
  • Shipping Weight: 2.2 pounds

Epidermolysis bullosa (EB) is a heterogenous group of genodermatoses characterized by the formation of blisters and erosions on skin and mucous membrans from birth on. The cause are mutations in the genes of structural proteins of the junction between epidermis and dermis.

This book deals with the treatment of this skin disease itself and its many extracutaneous complications. There is no previous book which has been focused on the therapy and it will be based on evidence-based data derived from the world´s largest cohort of inherited EB-patients, the American EB Registry. An important chapter will discuss gene therapy in hereditary EB which has been recently successfully performed within a localized skin site on a single EB patient as a proof-of-principle test. Given its unique collective contents, the monograph will provide the primary source for clinical informations of this oftentimes severe multiorgan disease.

Categoría: E-Books Médicos Etiquetas: Alergia e Inmunología, Cirugía Plástica, Genética
  • Descripción
  • Por qué utilizar archivos PDF
Descripción

Over the past few decades increasing numbers of health care professionals worldwide have become interested in and actively involved in the day to day care of children and adults with inherited epidermolysis bullosa (EB). Although not too many years ago the care of the EB child was provided primarily by consulting dermatologists and pediatricians, major advances in care during neonatal life and infancy have led to the survival in adulthood of most EB children, even those with the most severe forms. As a result, nearly every medical and allied health practitioner now plays a critical role in the care of these patients, and multidisciplinary care has become the norm. One very visible example of this is the creation of the eb-haus Austria (as discussed in Chapter 3.2), a freestanding, independently funded unit devoted solely to the multidisciplinary management of EB children and adults. It is hoped that similar facilities will be forthcoming elsewhere in the world, so as to best meet the many needs of these patients and their parents.

Within the same timeframe, major advances have been forthcoming which have given great insight into EB at the clinical, epidemiological, cellular, and molecular levels. As the result of nearly 20 years of epidemiological data collection and analysis by the American National EB Registry, for example, we now know for each major EB subtype its prevalence and incidence, the range of cutaneous manifestations, the risk of extracutaneous complications, and its natural history. At the same time, the molecular basis of each major EB subtype has now been elegantly elucidated, setting the stage for gene therapy. Similar advances in our detailed understanding of wound healing should also lead to the development of more effective treatment strategies for our patients, which will be greatly needed until a cure becomes a reality.

Por qué utilizar archivos PDF

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Vitra
Magisso
Louis Poulsen
KLÖBER
Joseph Joseph
Hay
Flos
Eva Solo
Alessi

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