Год выпуска: 2008 Издательство: Страниц: 400 ISBN: 1588299635
Описание
One premise of this book is that the study of rare lung disorders enhances our understanding of common pulmonary diseases and disease mechanisms such as fibrosis and emphysema. For example, the finding that the mutations in the alveolar type II cell specc protein, SP-C, can lead to pulmonary fibrosis is prima facie evidence that the alveolar epithelium plays a critical role in fibrogenesis. Similiarly, development of premature emphysema in patients with alpha one antitrypsin deficiency provides strong sort for the theory of protease/protease inhibitior balance in the pathogenesis of alveolar destruction. A second equally important premise is that the science in rare lung disease is inherently interesting and can often change with surprising speed. This partly related to the fact that the genetic basis of many rare lung diseases is now available, providing a unique vantage point for framing research questions.
