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Doody's Review ServiceReviewer: Joel C Seidman, MD (William Beaumont Hospitals)
Description: This is an extensive revision of a book published seven years earlier that broadly addresses all aspects of cystic fibrosis. There are nearly 70 contributors of international origin, primarily from the United Kingdom.
Purpose: This is an attempt to adopt observations of authorities from diverse experiences in order to provide a comprehensive review of the state-of-the-art respecting cystic fibrosis.
Audience: The audience would include pediatricians, pediatric and adult pulmonologists, allergists, nurses committed to patients suffering chronic respiratory disease, and gastroenterologists, and other practitioners of nutritional sciences. It would additionally provide a real-life reference for basic science researchers in putting a face on applications carried out in the laboratory.
Features: This is the most comprehensive treatise that I have seen for the clinician covering basic molecular biology and genetics underlying the disease, diagnosis, clinical presentation, and management including the role and success of lung transplantation. In the latter respect, it offers more evidence-based medicine review and practice guidelines than offered in earlier publications, aspects often lacking in similar undertakings. There is emphasis on objective measures of monitoring disease process, which is becoming more important as survival has increased, and on multidisciplinary care as offered at a structured cystic fibrosis center, experiences not always afforded patients and/or their caregivers firsthand. Finally, detailed attempts are made to speculate upon the future direction of disease management, especially what the roles of gene and stem cell therapy might be. Illustrations and graphics are limited, but sufficient to carry out the ends of each chapter. The bibliographies at each chapter end are extensive and relevant.
Assessment: The book achieves its likely intended goals well. To an average practitioner, it comes across as very accessible. I consider it a practical resource that I would add to my personal library.