Nutritional Deficiencies in a Phe-Restricted Diet

Nutritional abnormalities associated with Phe-restricted diets can have serious health consequences.

Diet management does not adequately fulfill nutritional requirements

The Phe-restricted diet and formulas have shortcomings

• The strict diet allows very limited food choices.⁴⁴
• The synthetic nature of the protein substitutes may lead to their poor utilization by the body.⁴⁴
• Formulas, as the primary source of protein, often do not provide adequate trace minerals and other nutrients.^27,50,51
• The diet has the potential to create weight management problems.^44,50

Nutritional Abnormalities Associated With the Phe-Restricted Diet^44,50,52-54

The impact of nutritional deficiencies due to a Phe-restricted diet may be significant⁵⁵

• Decreases in bone mass due to nutritional abnormalities can lead to osteopenia and osteoporosis in diet-managed PKU patients.⁵⁴
• In a clinical study of PKU patients on a Phe-restricted diet, by a mean age of 25 years old ⁵⁴
  — 39% (11/31) of patients had osteopenia
  — 7% (2/31) of patients had osteoporosis