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PKU Talks: The expert’s voice

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“PKU TALKS: THE EXPERT’S VOICE” is the periodical column on Phenylketonuria, created by Piam in collaboration with some of the major international specialists.

Today an interview with Dr. Sabrina Paci, pediatrician medical director of San Paolo and Carlo Hospital in Milan, on the importance of a diet for life in the PKU patients and the advantages of slow release amino acid mixtures compared to conventional ones.

What is the importance of life-long diet therapy in the overall management of PKU?

Dietary treatment for PKU patients is the gold standard for PKU treatment. The primary target is to maintain plasmatic Phenylalanine concentration in a safe range, different for age, to avoid neuropsychological and neurocognitive negative outcomes and to guarantee a normal growth, normal parameters and normal body composition, so, generally speaking, good health for the patients.

Dietary treatment is recommended life long for all PKU patients, from infancy to adulthood,  but is well known how difficult it is to maintain strict adherence.

What is the rational for using slow- versus normal- release formulas in PKU patients?

Free amino acids realize, in conventional amino acid mixture, higher plasmatic concentration with an early peak and rapid reduction while the protein substitute with slow release could improve the protein status and avoid plasmatic Phenylalanine fluctuations for better management of PKU dietary treatment. 

 

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