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Table 6 Dietary advice for illness in PKU

From: The complete European guidelines on phenylketonuria: diagnosis and treatment


Dietary advice

Phe-free L-amino acid supplement

Maintenance of Phe-free L-amino acid supplement intake to support protein synthesis.

It is better to give smaller, frequent doses throughout the day.

High carbohydrate intake

Encourage frequent high carbohydrate supplements, e.g. glucose polymer solution.

Natural protein intake

In practice, a reduced appetite leads to a lower natural protein intake.


All treatment specific medication should be continued during illness. Continue BH4 if already prescribed.

Medications should be free of aspartame in PKU.

Treat precipitating factors

e.g. anti-pyretics for pyrexia, antibiotics (aspartame-free) for bacterial infections.