Article
Recent advances
Recent advances in cystic fibrosis
Iolo J M Doull
Cystic
Fibrosis/Respiratory Unit, Department of Child Health, University
Hospital of Wales, Cardiff CF14 4XW, UK
Correspondence to: Dr Doull email: doullij{at}cf.ac.uk
Accepted 1 March 2001
The median life expectancy for cystic fibrosis is now over 30 years, and it is projected that in newborn infants it will become more
than 40 years. The identification of the cystic fibrosis gene and its
product, cystic fibrosis transmembrane conductance regulator (CFTR),
has widened the spectrum of the disease from the classical case of the
infant with cystic fibrosis to the elderly childless man with
unexplained bronchiectasis. There is increasing evidence of the
advantages of newborn screening for cystic fibrosis and subsequent
specialist care. Management concentrates on optimising nutritional
status and preventing lung infection and inflammation.
Keywords: cystic fibrosis; management
© 2001 by Archives of Disease in Childhood
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Arch. Dis. Child. 2001 85: 317.[Extract] [Full Text]
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