Protein Substitute Requirements of Patients with Phenylketonuria on BH4 Treatment: A Systematic Review and Meta-Analysis

Protein Substitute Requirements of Patients with Phenylketonuria on BH4 Treatment: A Systematic Review and Meta-Analysis

The traditional treatment for phenylketonuria (PKU) is a phenylalanine (Phe)-restricted diet, supplemented with a Phe-free/low-Phe protein substitute. Pharmaceutical treatment with synthetic tetrahydrobiopterin (BH4), an enzyme cofactor, allows a patient subgroup to relax their diet. However, dietar...

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Bibliographic Details
Main Authors: Fatma Ilgaz, Cyril Marsaux, Alex Pinto, Rani Singh, Carmen Rohde, Erdem Karabulut, Hülya Gökmen-Özel, Mirjam Kuhn, Anita MacDonald
Format: Article
Language:English
Published: MDPI AG 2021-03-01
Series:Nutrients
Subjects:
phenylalanine hydroxylase deficiency
hyperphenylalaninemia
PKU
protein substitute
medical formula
amino acid mixture
Online Access:https://www.mdpi.com/2072-6643/13/3/1040

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