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Cystic Fibrosis
Background
Cystic Fibrosis: Fibrocystic disease of the pancreas; mucoviscidosis
Cystic fibrosis (CF) was recognised as a specific entity by Dorothy Andersen of New York in 1938. The condition is characterised by early onset of severe intestinal malabsorption, failure to thrive and recurrent chest infections and pneumonia which, if untreated, leads to death from malnutrition and respiratory failure in infancy or early childhood. Due to improved treatment, particularly with antibiotics and better pancreatic enzyme replacement therapy, average survival has steadily improved to around 37 years.
