Impact of Lipophilic Antioxidants and Level of Antibodies Against Oxidized Low-Density Lipoprotein in Polish Children with Phenylketonuria

Abstract

The treatment of phenylketonuria (PKU) patients constitutes a phenylalanine (Phe) intake restriction in their diet, which is achieved by adding a special Phe-free amino acid mixture to the diet. It has been reported that this diet could have some micronutrient deficiency. Several authors have also reported an increased oxidative stress or impaired antioxidant status in human and experimental PKU. Our project assessed the concentrations of retinol, alpha-tocopherol, coenzyme Q10, and anti-oxidized low-density lipoprotein (ox-LDL) antibodies in PKU children's plasma. It was found that retinol concentration in PKU children remains within the norm despite a low intake. The lower plasma alpha-tocopherol concentration in PKU children compared with normal children was associated with the lower level of antibodies against ox-LDL. This raises the question whether higher than observed circulatory alpha-tocopherol is indeed beneficial to lower plasma ox-LDL levels. Further studies are needed to explain the genetic factor in PKU patients (e.g., CD36/FAT polymorphism gene). The open clinical question is whether daily supplementation of alpha-tocopherol changes the PKU patients' level of antibodies against ox-LDL.

Fenilcetonúria: Dieta Restritiva E Carência Nutricional

“…Artuch et al 9 e Gassió et al 24 , obtiveram resultados semelhantes. Mikoluc et al 6 divergiu dos demais estudos, evidenciando em seu resultado uma média de CoQ 10 superior em portadores da doença (Figura 3). Sete estudos constituíram a amostra com comparação de GPx entre fenilcetonúricos e indivíduos sadios.…”

Vitamin Status in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis

“…The included studies were published from 1994 [38] to 2023 [39], and their characteristics are detailed in Table 1. Four studies were conducted in Greece [25,27,40,41], three in Turkey [31,42,43], three in Spain [32,44,45], two in Austria [26,46], two in Chile [39,47], two in Japan [29,30], two in the United Kingdom [48,49], two in the USA [38,50], and one each in Germany [51], France [52], Poland [28], and Switzerland [53]. All studies, except for one case-control study [26], were cross-sectional and compared individuals with PKU to healthy controls.…”

“…All studies, except for one case-control study [26], were cross-sectional and compared individuals with PKU to healthy controls. The PKU groups varied from 10 [39,52] to 107 [28], while control groups ranged from 6 [38] to 1676 [49]. Five studies assessed children [25,27,28,31,40], one studied adolescents [43], children and adolescents were included in nine studies [26,32,38,41,42,45,46,50,51,53], six studies examined adults [29,30,39,48,49,52], and the remaining two studies had a mixed population [44,47].…”

“…Five studies assessed children [25,27,28,31,40], one studied adolescents [43], children and adolescents were included in nine studies [26,32,38,41,42,45,46,50,51,53], six studies examined adults [29,30,39,48,49,52], and the remaining two studies had a mixed population [44,47]. Most studies included males and females [24,[26][27][28][29][30]37,38,[40][41][42]45,46,[49][50][51][52], and while it was not explicitly stated in other studies [25,27,40,44,45,48,49], they likely involved both male and female participants. Table 2<...…”

“…However, studies examining the vitamin status of PKU patients have reported conflicting findings. Some studies found elevated vitamin levels in PKU patients [17,25,26], while others have reported lower levels [27][28][29]. Additionally, certain studies found no significant differences in vitamin levels between PKU patients and healthy controls [30][31][32].…”