Article
Neonatal phenylalanine wash-out in phenylketonuria.
Metabolic brain disease - 1 Oct 2020
Porta Francesco, Ponzone Alberto, Spada Marco
Abstract excerpt
Phenylketonuria (PKU) is the most common inborn error of amino acids metabolism. PKU management aims to keep as soon as possible blood phenylalanine (Phe), a non-acutely neurotoxic metabolite, within safe ranges through a dietary Phe restriction tailored to individual dietary Phe tolerance. Information on initial neonatal management of PKU, when Phe tolerance is still unknown, is scanty. We reviewed the metabolic...
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