Protein substitute for children and adults with phenylketonuria.

ABSTRACT:

Background

Phenylketonuria is an inherited metabolic disorder characterised by an absence or deficiency of the enzyme phenylalanine hydroxylase. The aim of treatment is to lower blood phenylalanine concentrations to the recommended therapeutic range to prevent developmental delay and support normal growth. Current treatment consists of a low-phenylalanine diet in combination with a protein substitute which is free from or low in phenylalanine. Guidance regarding the use, dosage, and distribution of dosage of the protein substitute over a 24-hour period is unclear, and there is variation in recommendations among treatment centres. This is an update of a Cochrane review first published in 2005, and previously updated in 2008.

Objectives

To assess the benefits and adverse effec

SUBMITTER: Yi SH 

PROVIDER: S-EPMC7212256 | biostudies-literature | 2015 Feb

REPOSITORIES: biostudies-literature