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PKU Talks: The expert’s voice

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Doctor Rocha: the assessment of the nutritional status of PKU patients and the impact of their diet on the metabolism.

PKU TALKS: THE EXPERT’S VOICE is the periodical column on Phenylketonuria, created by Piam in collaboration with major international specialists.
Today the interview with Doctor Julio Cesar Rocha, of the Faculdade de Ciências Médicas, Universidade Nova de Lisboa, about the assessment of the nutritional status of PKU patients and the impact of their diet on the metabolism.

“PKU patients need a specific nutritional treatment that usually is a combination of:

  • natural protein restriction
  • low-protein food
  • protein substitutes

In particular, the nutritional composition of every protein substitute is critical, not only regarding the quantity/quality of amino acids, but also regarding micronutrient intake.

If the micronutrient intake is not guaranteed, growth, body composition, neurological development and long-term outcomes could be affected.

Measuring of nutritional status in PKU patients is very important (anthropometry and body-composition markers, biochemical markers of nutritional status, clinical signs of nutritional deficiencies): all these information lead to a general overview about the nutritional status of these individuals.

Nowadays, thanks to improved treatment approaches in Inborn Errors of Metabolism, physicians are not merely focused on preventing neurological outcomes, but also on optimizing long-term outcomes/health markers.

Moreover, nowadays it is even more important to check the nutritional status and health outcomes in adulthood, because patients with inborn errors of protein metabolism usually live longer than before because they are treated in the neonatal period.

So it is also important to prevent overweight, obesity and cardiovascular diseases.”

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