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Cystic
  1. J M LITTLEWOOD, Consultant paediatrician

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    Cystic Fibrosis. Edited by Dennis J Shale. (Pp 162; £25 hardback.) BMJ Publishing Group, 1996. ISBN 0-7279-0826-X.

    This compact volume aims to present both current clinical thinking and practice with more fundamental aspects relating to the pathophysiology of cystic fibrosis. The book is written for respiratory and primary physicians, paediatricians, and all medical professionals seeking knowledge of the condition. It is a tall order to achieve this goal in only 162 pages. There are clear chapters on mouse models and on the cystic fibrosis gene but the clinical chapters, with the exception of those on respiratory infection and transplantation, are lacking in detail and under referenced to a degree that limits their value as a practical guide to treatment. The size of the book perhaps determined the relatively superficial account of the current clinical management of children and adults but in addition to the brevity there are some important omissions, for example the East Anglian controlled trial of flucloxacillin in screened cystic fibrosis infants, recent references to the use of inhaled steroids, and no mention of the flutter device. Generally referencing is more uneven with only 18 references in the chapter on the clinical management of children but no less than 172 in the chapter on respiratory infection.

    There are too few up-to-date references for an account of a condition where understanding and treatment are constantly changing. There are none to exercise later than 1982; only two of 68 on lung injury are later than 1991. The social and psychological section (22 pages) is nearly twice the length of the key chapter on management in children (13 pages) and largely deals with the importance of the role of the clinical nurse specialist. Although few would question the important role of the nurse specialist, many paediatricians and physicians would be reluctant to admit that this member of the cystic fibrosis team was alone able to consider the patient as a whole and ensure high quality care is delivered. The book is well produced with helpful tables of drug doses and key points but the photographs are of poor quality.

    Students of this challenging condition with its continually changing clinical expectations, methods of treatment, and prognosis will read this book with interest and learn much from it. However, those looking for a balanced, detailed account of cystic fibrosis in 1997 to help in clinical management will need to search elsewhere for more detailed information. Also, although all would agree that cystic fibrosis remains a very serious life threatening disorder, the clinical chapters of this book convey a degree of pessimism that is not entirely appropriate nowadays.

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