The effect of diet on total antioxidant status, ceruloplasmin, transferrin and ferritin serum levels in phenylketonuric children

Schulpis, Kleopatra H.; Papastamataki, Maria; Stamou, Helen; Papassotiriou, Ioannis; Margeli, Alexandra

doi:10.1111/j.1651-2227.2010.01890.x

Cited by 18 publications

(8 citation statements)

References 25 publications

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“…However, this relationship was not established in the nonobese group. Schulpis KH et al 2010 reported that, obesity has been associated with iron deficiency in overweight children and adolescents, as well as in obese adults. It was also reported that cells enriched by ferritin showed an enhanced resistance to oxidative stress, whereas cells in which ferritin was down regulated had reduced resistance to oxidative stress (Schulpis et al 2010).…”

Section: Clinical Characteristics Of the Participantsmentioning

confidence: 99%

Serum ferritin level as an early indicator of metabolic dysregulation in young obese adults — a cross-sectional study

Hitha

Gowda

Mirajkar

2018

Can. J. Physiol. Pharmacol.

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The aim of this study was to investigate the relationship between serum ferritin level and antioxidative status and metabolic dysregulation in young adult obese population. This cross-sectional study included 300 subjects of either sex, grouped as obese and non-obese subjects. The body mass index, total iron binding capacity, fasting blood glucose, superoxide dismutase activity, and levels of serum ferritin, iron, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, triglyceride, glutathione, and vitamin C were estimated. Analysis showed a significant alteration in all the parameters in obese adults. The correlation of ferritin level and body mass index showed a positive correlation (r = −0.81, p < 0.001, respectively) with levels of fasting blood glucose, superoxide dismutase, total cholesterol, low-density lipoprotein cholesterol, and triglyceride in obese individuals, whereas an insignificant correlation with vitamin C and glutathione level was observed in obese individuals. The significant positive correlation of ferritin level with the metabolic parameters and some antioxidative parameters in obese individuals signifies the development of metabolic disorders. Therefore, estimation of serum ferritin level will be an important early indicator for the risk of developing metabolic disorders in young adults.

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Section: Clinical Characteristics Of the Participantsmentioning

confidence: 99%

Serum ferritin level as an early indicator of metabolic dysregulation in young obese adults — a cross-sectional study

Hitha

Gowda

Mirajkar

2018

Can. J. Physiol. Pharmacol.

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“…Accordingly, reduced plasma β-carotene in PKU patients has been reported to negatively correlate with serum Phe levels (Sanayama et al 2011). The adherence to the PKU diet is usually related to a higher total plasma antioxidant status and a reduced lipid ingestion with increased proportion of polyunsaturated fat (Schulpis et al 2003(Schulpis et al , 2010. In other words, it is important to advise PKU patients that diet may offer other advantages besides management of Phe levels (Schulpis et al 2003, Schulpis et al 2005, Schulpis et al 2007.…”

Section: Oxidative Stress Data In Pku Patientsmentioning

confidence: 99%

Oxidative stress in Phenylketonuria: future directions

Rocha

Martins

2011

J of Inher Metab Disea

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Phenylketonuria represents the most prevalent inborn error of amino acid metabolism. In early diagnosed patients adequate and continued dietary treatment results in a good neurologic outcome. Natural protein and phenylalanine-restricted diet, even if rich in fruits and vegetables, represents a serious risk for nutritional deficiencies, albeit universally accepted. In the last few years, a growing number of reports have been describing oxidative stress as a concern in phenylketonuric patients. The diet itself includes good sources of dietary antioxidants (phytochemicals, some vitamins and minerals) but also a risk factor for some deficiencies (selenium, zinc, ubiquinone-10 and L-carnitine). Additionally, the extreme stringency of the diet may impose a reduced synthesis of endogenous antioxidants (like ubiquinone-10 and glutathione). Furthermore, increased phenylalanine levels, and its metabolites, may enhance the endogenous synthesis of reactive species and free radicals and/or interfere with the endogenous synthesis of enzymatic antioxidants (like glutathione peroxidase). Therefore, oxidative stress will probably increase, mainly in late diagnosed patients or in those with bad metabolic control. Considering the known association between oxidative stress, obesity and cardiovascular disease, it seems advisable to look further to the impact of oxidative stress on body macromolecules and structures (like lipoprotein oxidation), especially in phenylketonuric patients with late diagnosis or bad metabolic control, in order to prevent future increased risks. Recommendations for PKU patient's clinical follow-up improvement and educational goals are included.

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“…These data are not included in this table. The data for all PKU patients from these publications were pooled for the meta-analysis d For all studies by Schulpis et al [ 124 – 128 , 136 ], the PKU group was sub-grouped into well controlled PKU and poorly controlled PKU. Differences between well-controlled PKU and healthy controls only are reported in this table.…”

Section: Resultsmentioning

confidence: 99%

Status of nutrients important in brain function in phenylketonuria: a systematic review and meta-analysis

Montoya

Singh

Cetinyurek-Yavuz

et al. 2018

Orphanet J Rare Dis

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BackgroundDespite early and ongoing dietary management with a phe-restricted diet, suboptimal neuropsychological function has been observed in PKU. The restrictive nature of the PKU diet may expose patients to sub-optimal nutritional intake and deficiencies which may impact normal brain function. A systematic review of the published literature was carried out, where possible with meta-analysis, to compare the status of nutrients (Nutrients: DHA, EPA phospholipids, selenium, vitamins B6, B12, E, C, A, D, folic acid, choline, uridine, calcium, magnesium, zinc, iron, iodine and cholesterol) known to be important for brain development and functioning between individuals with PKU and healthy controls.ResultsOf 1534 publications identified, 65 studies met the entry criteria. Significantly lower levels of DHA, EPA and cholesterol were found for PKU patients compared to healthy controls. No significant differences in zinc, vitamins B12, E and D, calcium, iron and magnesium were found between PKU patients and controls. Because of considerable heterogeneity, the meta-analyses findings for folate and selenium were not reported. Due to an insufficient number of publications (< 4) no meta-analysis was undertaken for vitamins A, C and B6, choline, uridine, iodine and phospholipids.ConclusionsThe current data show that PKU patients have lower availability of DHA, EPA and cholesterol. Compliance with the phe-restricted diet including the micronutrient fortified protein substitute (PS) is essential to ensure adequate micronutrient status. Given the complexity of the diet, patients’ micronutrient and fatty acid status should be continuously monitored, with a particular focus on patients who are non-compliant or poorly compliant with their PS. Given their key role in brain function, assessment of the status of nutrients where limited data was found (e.g. choline, iodine) should be undertaken. Standardised reporting of studies in PKU would strengthen the output of meta-analysis and so better inform best practice for this rare condition.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0839-x) contains supplementary material, which is available to authorized users.

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The effect of diet on total antioxidant status, ceruloplasmin, transferrin and ferritin serum levels in phenylketonuric children

Abstract: The low ferritin levels found in 'off diet' patients with PKU may be attributed to a decreased liver production of ceruloplasmin, which evaluation may be a useful tool for the follow-up of patients with PKU.

Cited by 18 publications

References 25 publications

Serum ferritin level as an early indicator of metabolic dysregulation in young obese adults — a cross-sectional study

Serum ferritin level as an early indicator of metabolic dysregulation in young obese adults — a cross-sectional study

Oxidative stress in Phenylketonuria: future directions

Status of nutrients important in brain function in phenylketonuria: a systematic review and meta-analysis

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