Clinical Features

Most children with CF present with malabsorption and failure to thrive accompanied by recurrent or persistent chest infections. In the lungs, viscid mucus in the smaller airways predisposes to chronic infection, particularly with Staphylococcus aureus and Haemophilis influenzae, and subsequently with Pseudomonas species. This leads to damage of the bronchial wall, bronchietasis, and abscess formation.

Approximately 90% of CF patients have pancreatic insufficiency, requiring pancreatic enzyme supplements. Untreated patients pass frequent, large, pale, offensive, greasy stools. Ten to 15% of infants present with a meconium ileus resulting from the blockage of the terminal ileum by highly proteinac-eous meconium at birth. Distal intestinal obstruction syndrome may occur later in childhood or adult life. In addition to these symptoms, a number of other complications may occur in CF that are identified in Table 2.

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