ABSTRACT
Cystic fibrosis • Cystic fibrosis affects 1 in 2500 live births in Caucasian populations. • The main characteristics are pancreatic exocrine insufficiency and maldigestion,
pulmonary disease, intestinal obstruction, liver disease, diabetes and infertility. • Promoting better growth and nutrition is thought to improve survival. • Dietary energy intake should be raised by around 20-30% through increasing
dietary fat and sugar intake. • Dietary supplements are frequently needed and overnight tube feeding common. • 90% of patients have pancreatic exocrine insufficiency and require pancreatic
enzyme replacement therapy. • Diabetes is common with 2% of patients affected by 15 years of age, 12% by
25 years of age and 17% over 25 years of age.
