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Predictors of eventual requirement of phenylalanine-restricted diet in young infants with phenylalanine hydroxylase deficiency initially managed with sapropterin monotherapy.
Mol Genet Metab
November 2023
Division of Pediatric Metabolism, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey. Electronic address:
Background: Phenylalanine (Phe)-restricted diet is associated with lower quality of life for patients with phenylketonuria (PKU), and a concern for caregivers of recently-diagnosed infants. Sapropterin is an oral drug used as an alternative or adjunct to dietary treatment. We have observed that some of the young infants initially managed successfully with sapropterin monotherapy have required dietary treatment in long-term follow-up.
View Article and Find Full Text PDFSuitability and Allocation of Protein-Containing Foods According to Protein Tolerance in PKU: A 2022 UK National Consensus.
Nutrients
November 2022
Birmingham Women's and Children's NHS Foundation Trust, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
Introduction: There is little practical guidance about suitable food choices for higher natural protein tolerances in patients with phenylketonuria (PKU). This is particularly important to consider with the introduction of adjunct pharmaceutical treatments that may improve protein tolerance. Aim: To develop a set of guidelines for the introduction of higher protein foods into the diets of patients with PKU who tolerate >10 g/day of protein.
View Article and Find Full Text PDFPalynziq clinic: One year and 43 patients later.
Mol Genet Metab
July 2021
Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN, United States of America.
Pegvaliase-pqpz (Palynziq) is an enzyme substitution therapy FDA approved May 2018 to treat phenylketonuria in adults with blood phenylalanine levels greater than 600 μmol/L (10 mg/dL). Pegvaliase is administered via subcutaneous injection and carries a high risk of side effects including anaphylaxis. A consensus statement on its use recommends careful education and monitoring of patients.
View Article and Find Full Text PDFAdjunctive sapropterin dihydrochloride treatment in schizophrenia: A positive proof-of-concept, rater-blind, randomized, multivitamin-controlled study.
Schizophr Res
April 2020
Department of Psychiatry, Columbia University Medical Center, 1051 Riverside Drive, New York, NY 10032, USA; New York State Psychiatric Institute, 1051 Riverside Drive, New York, NY 10032, USA.
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