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Cystic Fibrosis

2010 Edition, March 25, 2010

Complete Document

Detail Summary

Active, Most Current

Additional Comments:
ISBN: 978-0-203-09155-5
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Product Details:

  • Revision: 2010 Edition, March 25, 2010
  • Published Date: March 25, 2010
  • Status: Active, Most Current
  • Document Language: English
  • Published By: CRC Press (CRC)
  • Page Count: 546
  • ANSI Approved: No
  • DoD Adopted: No

Description / Abstract:


Improvement in the outcome of people with cystic fibrosis (CF) over the past 50 years is truly remarkable. What was once an almost universally fatal childhood genetic disorder has, through advances in research and clinical care, become a chronic illness whereby presently almost half of the people living with CF are adults. The known disease-causing mutations of the cystic fibrosis transmembrane regulator (CFTR) protein gene, which was cloned in 1989, have expanded from just over 150 in 1993 to over 1400 in 2009. Many of the physiologic processes influenced by abnormal CFTR function, from alterations in electrolyte transport to abnormalities of innate airways defense, are now recognized. Nevertheless, CF remains a disease that shortens and alters the quality of the lives of most affected individuals, and much progress remains to be made.

In addition to new insights into the basic mechanisms that cause the CF phenotype, our understanding of the pathogenesis of both pulmonary and extrapulmonary manifestations of the disease have advanced. Concurrently, new techniques have been developed to detect CF lung disease at its earliest stages, well before it is clinically apparent. The confluence of these discoveries has led to the development of new therapeutic agents and approaches for the care of people with CF.

This book attempts to detail recent insights and knowledge of CF pathogenesis, treatment, and health care systems approaches. We hope to make accessible to caregivers an increased understanding of both the molecular basis of CF and its expanding clinical features. We also hope to provide a common knowledge base that will allow the generation of testable hypotheses for future basic, clinical and translational research. We have sought to highlight new challenges for improving care and outcomes; new approaches for diagnosing, assessing, and treating CF; and the "new" clinical manifestations that have resulted from greater longevity of people with CF. We have also endeavored to relate the underlying cellular and molecular pathophysiologies to their relevant clinical phenotypes, and thereby provide the rationale for novel interventions. In this way, we hope this volume gives the reader an idea of how far we have come in the care of people living with CF, how far we still have to go, and some ideas about how we might get there.

A book such as this is not possible without the contributions of many. We would like to thank the editorial staff at Informa Healthcare, especially Joseph Stubenrauch and Aimee Laussen, for their efficiency and especially their willingness to accommodate numerous last-minute changes. And, of course, we would like to thank the authors, whose knowledge and expertise are extraordinary.