Recent advances in cystic fibrosis.
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.
Doull
I J
IJ
Cystic Fibrosis/Respiratory Unit, Department of Child Health, University Hospital of Wales, Cardiff CF14 4XW, UK. doullij@cf.ac.uk
D000293:Adolescent; D000328:Adult; D000818:Animals; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000235:genetics* / Q000503:physiopathology / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000502:physiology; D003695:Delivery of Health Care; D005820:Genetic Testing / Q000379:methods; D015316:Genetic Therapy / Q000379:methods; D005838:Genotype; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008017:Life Expectancy; D016040:Lung Transplantation / Q000379:methods; D018942:Macrolides; D051379:Mice; D009894:Opportunistic Infections / Q000150:complications; D010641:Phenotype; D011379:Prognosis; D014031:Tobramycin / Q000627:therapeutic use
D000900:Anti-Bacterial Agents; C505032:CFTR protein, human; D018942:Macrolides; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D014031:Tobramycin