Proceedings of an international workshop held in Sestri Levante (Genoa), Italy, April 9--11, 1990

This work is concerned with Cystic Fibrosis (CF), the most common fatal genetic disease in the Caucasian population. The decade of the 1980s was one of spectacular progress in understanding the genetic and molecu lar basis of CF. The research breakthroughs of the decade began with the first fundamental insights, published in 1981-1983, into the basic cellular pathophysiology of CF with demonstrations of altered ion transport in spe cialized exocrine epithelial tissues (1-3). Research progress shifted into a triumph of "reverse genetics," using restriction-fragment-Iength polymor phism DNA technology (4), with the localization of the CF gene to a region of chromosome 7 (5-7). Understanding, accelerated by an explOSion of in vitro methodologies for epithelial cell culture and transformation, allowed and physiological studies (8-11); these focused, controlled biochemical with increasing precision, on the molecular pathology of distal steps in the regulatory pathways for epithelial ion transport (12-19). Finally, the "end of the beginning" occurred in late 1989 with one of the great achievements of molecular genetics, the isolation and cloning of the CF gene (20). As a result, we now have a CF gene product, the cystic fibrosis transmembrane regulator (CFfR), possessing predicted amino acid sequence, suggested tertiary structure, and possible transmembrane transport function (21). These amazing developments have set the stage for the next round of advances, which surely will include: 1.

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Cystic fibrosis used to be thought of as a respiratory and digestive disease, with a uniformly and rapidly fatal outcome. The spectrum of the disease has broadened into the mild atypical case, presenting in middle age, with the potential for complications in virtually every system of the body. In the past few years there has been an explosion of knowledge of the basic science of the defect. The editors have therefore invited the leading scientists and clinicians in the field of cystic fibrosis to describe the recent advances in this disease. Although there are many 'Recent Advances' texts, previous books have been selective in their choice of topics. This book is the first to cover the entire field of this complex disease, and encompasses the rapidly moving topics of the basic molecular and cellular biology as well as the recent multi-system, multi-disciplinary advances in the clinical care of patients. The authors have been charged with writing only about new developments and not to rehash old literature. The bulk of the references is therefore less than five years old. This book addresses all professionals who treat cystic fibrosis and want to have an up-date of new findings in the field, particularly of those outside their immediate specialisation. It will also be useful for basic researchers interested in related scientific areas and the clinical context of their work.

Murray and Nadel’s Textbook of Respiratory Medicine has long been the definitive and comprehensive pulmonary disease reference. Robert J. Mason, MD now presents the fifth edition in full color with new images and highlighted clinical elements. The fully searchable text is also online at www.expertconsult.com, along with regular updates, video clips, additional images, and self-assessment questions. This new edition has been completely updated and remains the essential tool you need to care for patients with pulmonary disease. Consult this title on your favorite e-reader, conduct rapid searches, and adjust font sizes for optimal readability. Compatible with Kindle®, nook®, and other popular devices. Master the scientific principles of respiratory medicine and its clinical applications. Work through differential diagnosis using detailed explanations of each disease entity. Learn new subjects in Pulmonary Medicine including Genetics, Ultrasound, and other key topics. Grasp the Key Points in each chapter. Search the full text online at expertconsult.com, along with downloadable images, regular updates, more than 50 videos, case studies, and self-assessment questions. Consult new chapters covering Ultrasound, Innate Immunity, Adaptive Immunity, Deposition and Clearance, Ventilator-Associated Pneumonia. Find critical information easily using the new full-color design that enhances teaching points and highlights challenging concepts. Apply the expertise and fresh ideas of three new editors—Drs. Thomas R. Martin, Talmadge E. King, Jr., and Dean E. Schraufnagel. Review the latest developments in genetics with advice on how the data will affect patient care.