Specific nutritional needs

Children with cystic fibrosis (CF) have several reasons to require close nutritional management:

• Increased resting energy expenditure (REE) from chronic inflammation and recurrent chest infections
  
• Anorexia
  
• Fat malabsorption secondary to pancreatic exocrine insufficiency. This is exacerbated by CF liver disease. 90% of children with CF will have pancreatic insufficiency by age 1 year
  
• Vitamin deficiency is common, and vitamin supplementation is needed from birth. Parenteral preparations may be required
  
• Pancreatic endocrine insufficiency and the development of diabetes mellitus is common and complicates the need for high calorie intake

A nutritional scoring system is now available.

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