Metabolic and catecholamine response to sympathetic stimulation in early-treated adult male patients with phenylketonuria.

ABSTRACT:

Purpose

Defective function of phenylalanine hydroxylase in phenylketonuria (PKU) results in the accumulation of phenylalanine (Phe) and the reduction of tyrosine (Tyr) in the blood, interfering in the normal development and function of organs and tissues in the body. Tyr is the precursor of catecholamines, secreted in response to stress by the adrenal medulla and paraganglia. The aim of this study was to evaluate plasma catecholamine and amino acid response to an escalating series of sympathetic stress tests in PKU patients.

Methods

Twelve males with classical PKU (aged 18-41 years) and ten healthy male controls were included in this study. The subjects were exposed to three different sympathetic stress stimulations: cold pressor, isometric handgrip, and peak treadmill tests

SUBMITTER: Sumanszki C 

PROVIDER: S-EPMC7426284 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature