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

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Metabolic and catecholamine response to sympathetic stimulation in early-treated adult male patients with phenylketonuria.
Hormones ( IF 3.2 ) Pub Date : 2020-01-28 , DOI: 10.1007/s42000-020-00176-z
Csaba Sumanszki ₁ , Krisztian Kovacs ₂ , Gellert Balazs Karvaly ₂ , Erika Kiss ₃ , Erika Simon ₃ , Attila Patocs _{4,

5} , Miklos Toth ₆ , Zsolt Komka ₆ , Peter Reismann ₁

Affiliation

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 to exhaustion. Physiological, metabolic, and hormonal changes were determined.

Results

Aerobic capacity (VO_2max) was significantly lower in the PKU group (p = 0.018); however, relative VO_2max was similar in the two groups during the spiroergometric test. No significant differences in norepinephrine or in epinephrine response were found between the two groups during the different stimulation tests. Blood Phe increased significantly in the PKU group compared with controls (p = 0.027) during the spiroergometric test, while Tyr levels remained stable in both groups.