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Book Review

Cystic Fibrosis

N Engl J Med 2008; 358:1873-1874April 24, 2008

Article

Cystic Fibrosis
Third edition. Edited by Margaret Hodson, Duncan Geddes, and Andrew Bush. 503 pp., illustrated. London, Hodder Arnold, 2007. $198.50. ISBN: 978-0-340-90758-0

Cystic fibrosis is one of the most common inherited diseases in populations of European descent. The median life expectancy of patients with cystic fibrosis is on the rise and has changed dramatically, within a period of three decades, from approximately 10 years to approximately 38 years. This favorable result has been achieved through research; through the development of adequate medications, especially enteric-coated pancreatic enzymes; through the use of prophylactic medications; through treatment by multidisciplinary cystic fibrosis teams; and through early intervention for patients who have pulmonary exacerbations. The total number of patients with cystic fibrosis worldwide is not known, but approximately 30,000 patients are listed in the cystic fibrosis registry in the United States.

The addition of editor Andrew Bush, an expert in pediatric respiratory medicine, to this edition of the book is a welcome change. The book deals with most aspects of cystic fibrosis, including basic science, diagnosis, clinical aspects, monitoring of the disease, and the multidisciplinary approach to the treatment of the disease. The emphasis on the holistic approach, which starts with the initial diagnosis and gives attention to all aspects of cystic fibrosis during the patient's growth and into his or her adult life, is a great advantage over previous editions of the book.

Some overlap is unavoidable, given that there are 66 contributors to the book. Some of the contributors focus on their own research and fail to give the reader the necessary broad view of their topic. The chapters on the multidisciplinary approach, the transition from pediatric to adult care, and nursing care are very well presented and end with informative appendixes, but dosage schedules for antibiotics and guidelines for monitoring therapy with aminoglycosides are missing.

The layout of the book would have been more interesting if color accents had been used in the tables. Higher-resolution radiographs would have been easier to interpret correctly, and better arrangements of the photomicrographs and legends would have improved readers' understanding of the pathology of cystic fibrosis. After page 259, four pages of full-color figures that appear within the chapters in black and white are inserted, but these pages would have been easier to find if the legends of the black-and-white images referred to them.

The last chapter, a discussion of the future of research in cystic fibrosis and of care for patients with the disease, is an elegant way to end the book. Overall, the book is practical and provides the reader with up-to-date information on the disease. No other textbook of this magnitude is available in the field of cystic fibrosis.

Jorrit Gerritsen, M.D., Ph.D.
University of Groningen, 9700 RB Groningen, the Netherlands