Cystic fibrosis (CF) is the most frequent, lethal genetic disorder among northern
Europeans. The etiology of this autosomal recessive disease is known to be a defect
in the cAMP activation of chloride (Cl—) channels in secretory cells in
many organs of the body. Although this defect usually leads to severe lung disease,
many of these patients also have nutritional deficiencies. Nutrition is one of the
key components in the management of CF. Patients are at high risk for malnutrition,
which may result in accelerated progression of the disease and increased morbidity.
This review will discuss nutrition recommendations for calories, protein, vitamins
and minerals, and enteral and parenteral nutrition support practices.
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