Acute exercise in treated phenylketonuria patients: Physical activity and biochemical response

Mazzola, Priscila Nicolao; Schirmbeck, Gabriel Henrique; Oliveira, Álvaro Reischak de; Derks, Terry G J; Spronsen, Francjan J. van; Dutra-Filho, Carlos Severo; Schwartz, Ida Vanessa Döederlein

doi:10.1016/j.ymgmr.2015.10.003

Cited by 13 publications

(21 citation statements)

References 40 publications

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“…Another study similarly showed no significant increase in Phe levels after submaximal exercise [10]. The discrepancy between the previous studies and our findings could be due to the different intervals of blood sample collection and exercise intensity.…”

Section: Discussioncontrasting

confidence: 99%

“…Resting catecholamine levels were comparable between the two groups. Similarly, Mazzola et al also found no significant difference in basal catecholamine levels between PKU subjects and healthy controls [ 10 ]. Therefore, it is questionable whether plasma catecholamine levels are only affected in children with PKU [ 18 , 19 ].…”

Section: Discussionmentioning

confidence: 99%

“…A recent study comparing blood Phe and Tyr levels before and after 1 h of ergometric endurance exercise of PKU subjects found no difference in Phe levels and a slight decrease in Tyr levels [ 23 ]. Another study similarly showed no significant increase in Phe levels after submaximal exercise [ 10 ]. The discrepancy between the previous studies and our findings could be due to the different intervals of blood sample collection and exercise intensity.…”

Section: Discussionmentioning

confidence: 99%

“…A study conducted in a pediatric population found significantly lower plasma catecholamine levels in non-adherent PKU patients compared with compliant PKU patients and controls [ 9 ]. However, a dynamic study conducted in a mixed age and gender population found no significant difference in plasma catecholamine levels of PKU patients compared with controls [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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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. Conclusion PKU itself might not influence stress-induced catecholamine changes. Only strenuous exercise increased blood Phe levels in PKU subjects.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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“…There is evidence to suggest that short acute exercise does not affect blood phenylalanine levels but the impact of endurance exercise has not been examined [32].…”

Section: Sport and Nutritionmentioning

confidence: 99%

PKU dietary handbook to accompany PKU guidelines

MacDonald

Wegberg

Ahring

et al. 2020

Orphanet J Rare Dis

141

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Background: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine. Main body: In 2017 the first European PKU Guidelines were published. These guidelines contained evidence based and/or expert opinion recommendations regarding diagnosis, treatment and care for patients with PKU of all ages. This manuscript is a supplement containing the practical application of the dietary treatment. Conclusion: This handbook can support dietitians, nutritionists and physicians in starting, adjusting and maintaining dietary treatment.

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Competitive, multi‐objective, and compartmented Flux Balance Analysis for addressing tissue‐specific inborn errors of metabolism

Liu

Westerhoff

2023

J of Inher Metab Disea

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The inborn error of metabolism phenylketonuria (PKU, OMIM 261600) is most often due to inactivation of phenylalanine hydroxylase (PAH), which converts phenylalanine (Phe) into tyrosine (Tyr). The reduced PAH activity increases blood concentration of phenylalanine and urine levels of phenylpyruvate. Flux balance analysis (FBA) of a single‐compartment model of PKU predicts that maximum growth rate should be reduced unless Tyr is supplemented. However, the PKU phenotype is lack of development of brain function specifically, and Phe reduction rather than Tyr supplementation cures the disease. Phe and Tyr cross the blood–brain barrier (BBB) through the aromatic amino acid transporter implying that the two transport reactions interact. However, FBA does not accommodate such competitive interactions. We here report on an extension to FBA that enables it to deal with such interactions. We built a three‐compartment model, made the common transport across the BBB explicit, and included dopamine and serotonin synthesis as parts of the brain function to be delivered by FBA. With these ramifications, FBA of the genome‐scale metabolic model extended to three compartments does explain that (i) the disease is brain specific, (ii) phenylpyruvate in urine is a biomarker, (iii) excess of blood‐phenylalanine rather than shortage of blood‐tyrosine causes brain pathology, and (iv) Phe deprivation is the better therapy. The new approach also suggests (v) explanations for differences in pathology between individuals with the same PAH inactivation, and (vi) interference of disease and therapy with the functioning of other neurotransmitters.

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Acute exercise in treated phenylketonuria patients: Physical activity and biochemical response

Cited by 13 publications

References 40 publications

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

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

PKU dietary handbook to accompany PKU guidelines

Competitive, multi‐objective, and compartmented Flux Balance Analysis for addressing tissue‐specific inborn errors of metabolism

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