Triglycerides, Cholesterol, HDL, LDL, and VLDL Cholesterol in Serum of Phenylketonuric Children Under Dietary Control

Schulpi, Kleopatra H.; Scarpalezou, Astrinia

doi:10.1177/000992288902801007

Cited by 27 publications

(20 citation statements)

References 10 publications

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“…Following the recommendation to analyze the absolute data used in the MetSyn definition, together with other biomarkers [15], we could not only substantiate the reductions in total cholesterol concentrations [41,42], but also found statistical significant lower glucose concentrations notwithstanding the sugar-rich dietary pattern without increased insulin secretion. The exact clinically significance of this finding needs to be elucidated.…”

Section: Discussionmentioning

confidence: 70%

Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome

Rocha

Spronsen

Almeida

et al. 2012

Molecular Genetics and Metabolism

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Section: Discussionmentioning

confidence: 70%

Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome

Rocha

Spronsen

Almeida

et al. 2012

Molecular Genetics and Metabolism

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“…To support this hypothesis, previously published studies have suggested a similar or even improved lipid profile in PKU children compared to healthy controls [25]. Only few studies have explored lipid profile or other coronary heart disease-related parameters in PKU patients.…”

Section: Discussionmentioning

confidence: 98%

Lipid profile status and other related factors in patients with Hyperphenylalaninaemia

Couce

Vitoria

Aldámiz‐Echevarría

et al. 2016

Orphanet J Rare Dis

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BackgroundThe mainstay of treating patients with phenylketonuria (PKU) is based on a Phe-restricted diet, restrictive in natural protein combined with Phe-free L-amino acid supplements and low protein foods. This PKU diet seems to reduce atherogenesis and confer protection against cardiovascular diseases but the results from the few published studies have been inconclusive. The aim of our study was to evaluate the relationship between the lipid profile and several treatment-related risk factors in patients with hyperphenylalaninaemia (HPA) in order to optimize their monitoring.MethodsWe conducted a cross-sectional multicentre study. A total of 141 patients with HPA were classified according to age, phenotype, type of treatment and dietary adherence. Annual median blood phenylalanine (Phe) levels, Phe tolerance, anthropometric measurements, blood pressure (BP) and biochemical parameters [(triglycerides, total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), low density lipoprotein-cholesterol (LDL-C), apolipoprotein A (ApoA), apolipoprotein B (ApoB), vitamin B12, total homocysteine (tHcy), Methionine (Met), high sensitivity C-Reactive Protein (hsCRP)] were collected for each patient.ResultsPlasma TC levels were lower in patients with PKU than in the mild-HPA group (150 ± 31 vs. 164 ± 22 mg/dL), and there was a weak inverse correlation between plasma TC and Phe levels. HDL-C, LDL-C, ApoA and ApoB levels were lower in the PKU group than in mild-HPA. Patients with PKU had higher systolic BP than the mild-HPA group and there was found a quadratic correlation between median Phe levels and systolic BP (p = 6.42e-5) and a linear correlation between median Phe levels and diastolic BP (p = 5.65e-4). In overweight or obese PKU patients (24.11 %), biochemical parameters such as TC, triglycerides, LDL-C, tHcy, hsCRP and BP were higher. By contrast, HDL-C was lower in these patients.ConclusionOur data show a direct correlation between lipid profile parameters and good adherence to the diet in PKU patients. However, lipid profile in overweight or obese patients displayed an atherogenic profile, in addition to higher hsCRP concentrations and BP. Our study contributes to a better understanding of the relationship between phenotype and treatment in patients with HPA, which could be useful in improving follow-up strategies and clinical outcome.Trial registrationResearch Ethics Committee of Santiago-Lugo 2015/393. Registered 22 September 2015, retrospectively registered.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-016-0508-x) contains supplementary material, which is available to authorized users.

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“…These findings have been explained by a high polyunsaturated=saturated fatty acid ratio (P:S), low dietary fat and cholesterol intake, high fibre intake and primarilly plant protein sources (Pan Wen Ham et al, 1993). In this respect PKU children on diet can be considered vegetarians (Schulpis & Scarpalezou, 1988). However, not all the patients adhere to the recommended diet.…”

Section: Discussionmentioning

confidence: 99%

Effect of diet on plasma total antioxidant status in phenylketonuric patients

et al. 2003

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Background: Phenylketonuria (PKU), an inborn error of phenylalanine (Phe) metabolism, is treated with a low Phe lifelong diet, which is a vegetarian and contains many antioxidants. Aim: The aim of this study was to evaluate the effect of diet on plasma total antioxidant status in our PKU patients. Methods, Results: Twenty-two PKU patients on strict diet (group A), 24 PKU patients who did not accurately follow their dietary control (group B) and 40 healthy children (controls) of comparable age took part in this study. Nutrients, as well as blood levels of lipids, vitamin C, b-carotene and a-tocopherol were evaluated in all groups. Vitamin C intake and its blood levels did not differ between the groups. However, the intake of b-carotene, a-tocopherol (2211 AE 116, 14 AE 1.0 mg=24 h) and their blood levels (0.7 AE 0.09, 34 AE 0.9 mmol=l) in group A were statistically significantly higher (P < 0.001) as compared with those of group B (1352 AE 118, 10 AE 1.0 mg=24 h and 0.49 AE 0.08, 22 AE 0.6 mmol=l) and controls (1290 AE 120, 9.0 AE 0.9 mg=24 h and 0.40 AE 0.09, 24 AE 1.6 mmol=l). Lipid intakes and their blood levels were lower in patients on the strict diet. Plasma total antioxidant status was higher in the same group of patients (group A). Additionally, positive correlations were found between the antioxidant vitamin blood levels and the plasma total antioxidant status in the groups, especially in the group A. PKU patients of group A showed significantly higher antioxidant status (1.6 AE 0.2 mmol=l) as compared with those of group B (1.0 AE 0.19 mmol=l; P < 0.001) and controls (1.01 AE 0.2 mmol=l). Conclusions: The high plasma antioxidant status in patients with PKU, especially in those with a good compliance with their diet, is possibly due to the amounts of antioxidants which are present in their special low Phe vegetarian diet.

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Triglycerides, Cholesterol, HDL, LDL, and VLDL Cholesterol in Serum of Phenylketonuric Children Under Dietary Control

Cited by 27 publications

References 10 publications

Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome

Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome

Lipid profile status and other related factors in patients with Hyperphenylalaninaemia

Effect of diet on plasma total antioxidant status in phenylketonuric patients

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