Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome
Rett syndrome (RTT) is a devastating neurodevelopmental disorder with a 300-fold increased risk rate for sudden cardiac death. A subclinical myocardial biventricular dysfunction has been recently reported in RTT by our group and found to be associated with an enhanced oxidative stress (OS) status. H...
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2014-01-01
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Series: | Mediators of Inflammation |
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doaj-349b39da1d714ddaa80fab24aaefd21f2020-11-24T22:22:20ZengHindawi LimitedMediators of Inflammation0962-93511466-18612014-01-01201410.1155/2014/983178983178Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett SyndromeSilvia Maffei0Claudio De Felice1Pierpaolo Cannarile2Silvia Leoncini3Cinzia Signorini4Alessandra Pecorelli5Barbara Montomoli6Stefano Lunghetti7Lucia Ciccoli8Thierry Durand9Roberto Favilli10Joussef Hayek11Department of Cardiology, University Hospital Azienda Ospedaliera Universitaria Senese (AOUS), Viale M. Bracci 16, 53100 Siena, ItalyNeonatal Intensive Care Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Biotechnologies, University of Siena, Strada delle Scotte 4, 53100 Siena, ItalyDepartment of Molecular and Developmental Medicine, University of Siena, Via A. Moro 2, 53100 Siena, ItalyDepartment of Molecular and Developmental Medicine, University of Siena, Via A. Moro 2, 53100 Siena, ItalyDepartment of Molecular and Developmental Medicine, University of Siena, Via A. Moro 2, 53100 Siena, ItalyChild Neuropsychiatry Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Cardiology, University Hospital Azienda Ospedaliera Universitaria Senese (AOUS), Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Molecular and Developmental Medicine, University of Siena, Via A. Moro 2, 53100 Siena, ItalyInstitut des Biomolécules Max Mousseron (IBMM), UMR 5247, CNRS/UM1/UM2, BP 14491 34093, Montpellier, Cedex 5, FranceDepartment of Cardiology, University Hospital Azienda Ospedaliera Universitaria Senese (AOUS), Viale M. Bracci 16, 53100 Siena, ItalyChild Neuropsychiatry Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, ItalyRett syndrome (RTT) is a devastating neurodevelopmental disorder with a 300-fold increased risk rate for sudden cardiac death. A subclinical myocardial biventricular dysfunction has been recently reported in RTT by our group and found to be associated with an enhanced oxidative stress (OS) status. Here, we tested the effects of the naturally occurring antioxidants ω-3 polyunsaturated fatty acids (ω-3 PUFAs) on echocardiographic parameters and systemic OS markers in a population of RTT patients with the typical clinical form. A total of 66 RTT girls were evaluated, half of whom being treated for 12 months with a dietary supplementation of ω-3 PUFAs at high dosage (docosahexaenoic acid ~71.9 ± 13.9 mg/kg b.w./day plus eicosapentaenoic acid ~115.5 ± 22.4 mg/kg b.w./day) versus the remaining half untreated population. Echocardiographic systolic longitudinal parameters of both ventricles, but not biventricular diastolic measures, improved following ω-3 PUFAs supplementation, with a parallel decrease in the OS markers levels. No significant changes in the examined echocardiographic parameters nor in the OS markers were detectable in the untreated RTT population. Our data indicate that ω-3 PUFAs are able to improve the biventricular myocardial systolic function in RTT and that this functional gain is partially mediated through a regulation of the redox balance.http://dx.doi.org/10.1155/2014/983178 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Silvia Maffei Claudio De Felice Pierpaolo Cannarile Silvia Leoncini Cinzia Signorini Alessandra Pecorelli Barbara Montomoli Stefano Lunghetti Lucia Ciccoli Thierry Durand Roberto Favilli Joussef Hayek |
spellingShingle |
Silvia Maffei Claudio De Felice Pierpaolo Cannarile Silvia Leoncini Cinzia Signorini Alessandra Pecorelli Barbara Montomoli Stefano Lunghetti Lucia Ciccoli Thierry Durand Roberto Favilli Joussef Hayek Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome Mediators of Inflammation |
author_facet |
Silvia Maffei Claudio De Felice Pierpaolo Cannarile Silvia Leoncini Cinzia Signorini Alessandra Pecorelli Barbara Montomoli Stefano Lunghetti Lucia Ciccoli Thierry Durand Roberto Favilli Joussef Hayek |
author_sort |
Silvia Maffei |
title |
Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome |
title_short |
Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome |
title_full |
Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome |
title_fullStr |
Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome |
title_full_unstemmed |
Effects of ω-3 PUFAs Supplementation on Myocardial Function and Oxidative Stress Markers in Typical Rett Syndrome |
title_sort |
effects of ω-3 pufas supplementation on myocardial function and oxidative stress markers in typical rett syndrome |
publisher |
Hindawi Limited |
series |
Mediators of Inflammation |
issn |
0962-9351 1466-1861 |
publishDate |
2014-01-01 |
description |
Rett syndrome (RTT) is a devastating neurodevelopmental disorder with a 300-fold increased risk rate for sudden cardiac death. A subclinical myocardial biventricular dysfunction has been recently reported in RTT by our group and found to be associated with an enhanced oxidative stress (OS) status. Here, we tested the effects of the naturally occurring antioxidants ω-3 polyunsaturated fatty acids (ω-3 PUFAs) on echocardiographic parameters and systemic OS markers in a population of RTT patients with the typical clinical form. A total of 66 RTT girls were evaluated, half of whom being treated for 12 months with a dietary supplementation of ω-3 PUFAs at high dosage (docosahexaenoic acid ~71.9 ± 13.9 mg/kg b.w./day plus eicosapentaenoic acid ~115.5 ± 22.4 mg/kg b.w./day) versus the remaining half untreated population. Echocardiographic systolic longitudinal parameters of both ventricles, but not biventricular diastolic measures, improved following ω-3 PUFAs supplementation, with a parallel decrease in the OS markers levels. No significant changes in the examined echocardiographic parameters nor in the OS markers were detectable in the untreated RTT population. Our data indicate that ω-3 PUFAs are able to improve the biventricular myocardial systolic function in RTT and that this functional gain is partially mediated through a regulation of the redox balance. |
url |
http://dx.doi.org/10.1155/2014/983178 |
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