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Lipid profile status and other related factors in patients with Hyperphenylalaninaemia.

ABSTRACT: The 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.We 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.Plasma 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.Our 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.Research Ethics Committee of Santiago-Lugo 2015/393. Registered 22 September 2015, retrospectively registered.

SUBMITTER: Couce ML

PROVIDER: S-EPMC5016957 | biostudies-literature | 2016 Sep

REPOSITORIES: biostudies-literature

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Lipid profile status and other related factors in patients with Hyperphenylalaninaemia.

Couce María L ML Vitoria Isidro I Aldámiz-Echevarría Luís L Fernández-Marmiesse Ana A Roca Iria I Llarena Marta M Sánchez-Pintos Paula P Leis Rosaura R Hermida Alvaro A

Orphanet journal of rare diseases 20160909 1

<h4>Background</h4>The 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 ...[more]

PMID: 27612877

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Project description:OBJECTIVES: The main objective of this study is to assess the Breastfeeding Duration, Exclusive Breastfeeding Duration and other related factors among children aged less than 5 years old in rural areas of Northern Iran. METHODS: This is a descriptive cross-sectional conducted on 2520 children aged 6-60 months (male: 1309, female: 1211) chosen by cluster random sampling from 20 out of 118 villages. Data were collected from mothers using a questionnaire. The duration of breastfeeding was computed only for children aged over 24 months old. Breastfeeding duration and Exclusive Breastfeeding Duration were classified based on WHO definition. SPSS Version 16 was used for data analysis. RESULTS: The mean Exclusive Breastfeeding Duration was 5.59 months, while 66.4% of children had exclusive breastfeeding for at least 6 months. The lowest Exclusive Breastfeeding Duration and the highest Breastfeeding Duration were observed among the Turkman ethnic group. Exclusive Breastfeeding duration of at least 5 months was 14.6%, thus the results were significantly higher than in the Turkman ethnic group (p=0.001). Meanwhile, the results showed that exclusive breastfeeding duration significantly increased with maternal education level (p=0.004). The study found that the mean breastfeeding duration was 20.6 months, and 89.3% and 74.7% of children were breastfed for at least 18 and 24 months, respectively. A positive correlation was reported between breastfeeding duration and family size, birth order, maternal age and children nutritional status, (p<0.05). Additionally, lactation period in underweight children was significantly higher than in obese children, (p=0.023). CONCLUSION: The study found that two-thirds of children exclusively breastfed during the first six months of life and the mean breastfeeding duration was 20.6 months. While both exclusive breastfeeding duration and breastfeeding duration were influenced by socio-demographic factors in the rural areas of Northern Iran.

Project description:BackgroundThe biological pathways through which vitamin D is involved in the regulation of systemic inflammation remain largely unknown.ObjectiveThe objective of this study was to evaluate the role of vitamin D status on the relationship between lipid profile and high-sensitivity C-reactive protein (hs-CRP) in pregnant women.DesignSerum 25-hydroxyvitamin D (25(OH)D), hs-CRP, and indicators of lipid profiles (total cholesterol, TC; triglyceride, TG; high-density lipoprotein cholesterol, HDL-C; low-density lipoprotein cholesterol, LDL-C), were measured in 2479 pregnant women during the second trimester. Potential confounding including maternal sociodemographic characteristics, perinatal health status, diet, and lifestyle was prospectively collected. Multiple regression models and cubic models were used to evaluate the associations.ResultsThere was a significant non-linear relationship between lipid profile (TC, TG, HDL-C, LDL-C) and hs-CRP (P < 0.05). Increased serum 25(OH)D was significantly associated with decreasing TC, TG, HDL-C, LDL-C, and hs-CRP levels. Compared with medium levels of lipids group, pregnant women with higher levels of TC or TG have higher levels of hs-CRP, and pregnant women with lower levels of TC, HDL-C or LDL-C also have higher levels of hs-CRP in the vitamin D deficient group, and there was a significant correlation between low levels of TG and decreased hs-CRP (adjusted β for TG: -0.063, 95%CI: - 0.120,-0.007) in the non-vitamin D deficient group. Mediators that had appreciable shares of the associations between 25(OH)D and hs-CRP was TG (10.2% of the association; β = - 0.011; total indirect effect: 95% CI: - 0.019, - 0.002). The cubic model suggested that a steep increase in the adjusted regression coefficient of lipid with hs-CRP up to 50 nmol/L of 25(OH)D, and the highest adjusted regression coefficients were observed in pregnant women with 25(OH)D above 50 nmol/L.ConclusionOur findings suggest that high levels of vitamin D during pregnancy may improve lipid profile levels and inhibit elevated hs-CRP induced by high lipid metabolism.

Dataset Information

Lipid profile status and other related factors in patients with Hyperphenylalaninaemia.

Publications

Lipid profile status and other related factors in patients with Hyperphenylalaninaemia.

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