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25 - Cystic fibrosis

from Part IV - Abdomen

Published online by Cambridge University Press:  08 January 2010

By
Mark Davenport  and
Hilary Wyatt
Edited by
Mark D. Stringer ,
Keith T. Oldham  and
Pierre D. E. Mouriquand
Mark Davenport
Affiliation:
King's College Hospital, London, UK
Hilary Wyatt
Affiliation:
King's College Hospital, London, UK
Mark D. Stringer
Affiliation:
University of Otago, New Zealand
Keith T. Oldham
Affiliation:
Children's Hospital of Wisconsin
Pierre D. E. Mouriquand
Affiliation:
Debrousse Hospital, Lyon
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Summary

“Das Kind stirbt bald wieder, dessen Stirne beim Küssen salzig ist”

(The child will die soon, whose forehead tastes salty when kissed)

German children's song

Introduction

Cystic fibrosis (CF) is the most common lethal inherited condition in Caucasians. In the 1960s patients rarely survived their first decade, succumbing to the effects of malnutrition and lung disease. At the end of the 1980s there were approximately 5000 patients with CF within the UK with an estimated 6000 by the year 2000. In practice, in 2002, almost 7000 patients had been registered on the UK Cystic Fibrosis database (personal communication). Similar changes have been observed in North America. Improvements to treatment over the last 40 years have led to a dramatic increase in life expectancy such that the median survival is now around 32 years. Further improvements in survival are expected for babies born in the past decade. The treatment of CF is, however, complex, time-consuming and intrusive to the sufferer's daily life. With advancing age, more complications of CF develop and a number of these require surgical intervention. Discovery of the abnormal gene responsible for CF in 1989 has resulted in a much greater understanding of this multisystem disease, as well as hope of a cure for the major cause of morbidity and mortality, lung disease.

Pathophysiology

CF is an autosomal recessive condition with an incidence of about 1 in 2500 live births in the UK and Canada and 1 in 3400 live births in the USA white population.

Type
Chapter
Information
Pediatric Surgery and Urology
Long-Term Outcomes
 , pp. 315 - 328
Publisher: Cambridge University Press
Print publication year: 2006

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