Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 1982 Sep;16(9):751-5.
doi: 10.1203/00006450-198209000-00009.

Reduction of cerebrospinal fluid phenylalanine after oral administration of valine, isoleucine, and leucine

H K Berry, M K Bofinger, M M Hunt, P J Phillips, M B Guilfoile

Reduction of cerebrospinal fluid phenylalanine after oral administration of valine, isoleucine, and leucine

H K Berry et al. Pediatr Res. 1982 Sep.

Abstract

A supplement of the branched chain amino acids, valine, isoleucine, and leucine (VIL) was administered orally to patients with phenylketonuria, either together with unrestricted diet of natural protein or with a low phenylalanine diet. The VIL supplement brought about a significant reduction of the cerebrospinal fluid-serum ratio of phenylalanine from a mean value of 0.254 without VIL to 0.204 with VIL. The reduction varied from 15-40% (mean 21%). Concentrations of glycine, lysine, methionine, threonine, tryptophan, and tyrosine were within normal limits in serum and cerebrospinal fluid of infants with phenylketonuria. No amino acid imbalance was created by the supplement and no adverse effects from VIL were observed.

PubMed Disclaimer