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Proactive management can improve patient survival.
Cystic fibrosis (CF) affects one in 2,500 newborns and has a UK prevalence of 8,000.
It is the most common inherited condition in white populations, and is increasingly recognised in non-white populations.
CF is an autosomal recessive disorder caused by a mutation at the CF transmembrane conductance regulator gene that affects various cellular activities. These include transport of sodium ions across the respiratory epithelium, pancreatic enzyme secretion and the surface properties of glycoproteins.
The altered sodium metabolism in patients with CF causes increased viscosity of mucus in the respiratory tract, GI tract, pancreas, sweat glands and other exocrine tissues.
CF can present at any age and the presentation varies according to age. In high risk families, the diagnosis may be suspected antenatally stage on the basis of chorionic villous sampling, amniocentesis or ultrasound.
At the neonatal stage, 10 per cent of CF patients may have meconium ileus causing …