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Phenylketonuria (PKU) | Protein metabolism | Inborn Errors of Metabolism

Phenylketonuria (PKU)

What is Phenylketonuria (PKU)?

Amino acids - phenylalanine

The ‘building blocks’ of protein are called amino acids. In PKU, due to the lack of an enzyme, the body cannot properly deal with one of the amino acids, phenylalanine. People with PKU therefore need to severely restrict the amount of protein they eat.


Protein substitutes for PKU

A key element in the management of PKU is the use of protein substitutes. Protein substitutes for PKU provide all the amino acids found in protein but no phenylalanine. In combination with the small allowance of regular dietary protein, protein substitutes ensure that an individual with PKU has an adequate intake of protein. 


Vitaflo produces a range of age-specific, lifestyle-friendly and palatable protein substitutes for PKU.

Our product range for PKU

Our support product range 

Our healthcare professional resources for PKU

PKU case studies

Starting Solids and Introducing a Second Stage Protein Substitute (PKU gel™)

Prof. Anita MacDonald, Consultant Dietitian in Inherited Metabolic Disorders, Birmingham Children’s Hospital

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Use of PKU sphere as a protein substitute during preconception for a patient with Hyperphenylalaninemia

Melanie Hill, Specialist Dietitian for Adults with Inherited Metabolic Diseases, Sheffield Teaching Hospitals NHS Foundation Trust

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Online resource for PKU patients

Inborn Errors of Metabolism