Cystic Fibrosis

  • R. Dinwiddie


Cystic fibrosis (CF) is the commonest recessively inherited condition in the population of the UK. It affects in 1 in 2500 live-born infants in this country (Dodge et al. 1988), approximately 85% of patients have pancreatic deficiency leading to significant malabsorption of protein, fat, carbohydrates and vitamins. The primary defect appears to be due to an abnormality of chloride secretion across secretory cell surfaces (Quinton 1983). These abnormalities cause unusually viscid intestinal secretions which result in pancreatic obstruction and distended pancreatic ducts (Grand et al. 1966). The pathophysiology of this process involves other abnormalities of pancreatic secretion, including decreased protease activity and reduced flow of pancreatic secretions which again predispose to intestinal obstruction (Durie and Forstner 1989). The cardinal features of pancreatic deficiency in cystic fibrosis consist of reduced enzyme secretion and a very low bicarbonate level (Gaskin et al. 1982; Coupleman et al. 1988).


Cystic Fibrosis Pancreatic Disease Pancreatic Insufficiency Essential Fatty Acid Deficiency Cystic Fibrosis Gene 
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© Springer-Verlag London Limited 1991

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  • R. Dinwiddie

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