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Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence

Inborn errors of metabolism (IMDs) are a group of inherited diseases that manifest themselves through a myriad of signs and symptoms, including structural or functional cardiovascular damage. The therapy of these diseases is currently based on enzyme-replacement therapy, chaperone therapy or the adm...

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Autores principales: Luca, Alina Costina, Pădureț, Ioana-Alexandra, Țarcă, Viorel, David, Simona Georgiana, Mîndru, Dana Elena, Roșu, Solange Tamara, Roșu, Eduard Vasile, Adumitrăchioaiei, Heidrun, Bernic, Jana, Cojocaru, Elena, Țarcă, Elena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10675151/
https://www.ncbi.nlm.nih.gov/pubmed/38004189
http://dx.doi.org/10.3390/nu15224795
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author Luca, Alina Costina
Pădureț, Ioana-Alexandra
Țarcă, Viorel
David, Simona Georgiana
Mîndru, Dana Elena
Roșu, Solange Tamara
Roșu, Eduard Vasile
Adumitrăchioaiei, Heidrun
Bernic, Jana
Cojocaru, Elena
Țarcă, Elena
author_facet Luca, Alina Costina
Pădureț, Ioana-Alexandra
Țarcă, Viorel
David, Simona Georgiana
Mîndru, Dana Elena
Roșu, Solange Tamara
Roșu, Eduard Vasile
Adumitrăchioaiei, Heidrun
Bernic, Jana
Cojocaru, Elena
Țarcă, Elena
author_sort Luca, Alina Costina
collection PubMed
description Inborn errors of metabolism (IMDs) are a group of inherited diseases that manifest themselves through a myriad of signs and symptoms, including structural or functional cardiovascular damage. The therapy of these diseases is currently based on enzyme-replacement therapy, chaperone therapy or the administration of supplements and the establishment of personalized dietary plans. Starting from the major signs identified by the pediatric cardiologist that can indicate the presence of such a metabolic disease—cardiomyopathies, conduction disorders or valvular dysplasias—we tried to paint the portrait of dietary interventions that can improve the course of patients with mitochondrial diseases or lysosomal abnormalities. The choice of the two categories of inborn errors of metabolism is not accidental and reflects the experience and concern of the authors regarding the management of patients with such diagnoses. A ketogenic diet offers promising results in selected cases, although, to date, studies have failed to bring enough evidence to support generalized recommendations. Other diets have been successfully utilized in patients with IMDs, but their specific effect on the cardiac phenotype and function is not yet fully understood. Significant prospective studies are necessary in order to understand and establish which diet best suits every patient depending on the inherited metabolic disorder. The most suitable imagistic monitoring method for the impact of different diets on the cardiovascular system is still under debate, with no protocols yet available. Echocardiography is readily available in most hospital settings and brings important information regarding the impact of diets on the left ventricular parameters. Cardiac MRI (magnetic resonance imaging) could better characterize the cardiac tissue and bring forth both functional and structural information.
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spelling pubmed-106751512023-11-16 Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence Luca, Alina Costina Pădureț, Ioana-Alexandra Țarcă, Viorel David, Simona Georgiana Mîndru, Dana Elena Roșu, Solange Tamara Roșu, Eduard Vasile Adumitrăchioaiei, Heidrun Bernic, Jana Cojocaru, Elena Țarcă, Elena Nutrients Review Inborn errors of metabolism (IMDs) are a group of inherited diseases that manifest themselves through a myriad of signs and symptoms, including structural or functional cardiovascular damage. The therapy of these diseases is currently based on enzyme-replacement therapy, chaperone therapy or the administration of supplements and the establishment of personalized dietary plans. Starting from the major signs identified by the pediatric cardiologist that can indicate the presence of such a metabolic disease—cardiomyopathies, conduction disorders or valvular dysplasias—we tried to paint the portrait of dietary interventions that can improve the course of patients with mitochondrial diseases or lysosomal abnormalities. The choice of the two categories of inborn errors of metabolism is not accidental and reflects the experience and concern of the authors regarding the management of patients with such diagnoses. A ketogenic diet offers promising results in selected cases, although, to date, studies have failed to bring enough evidence to support generalized recommendations. Other diets have been successfully utilized in patients with IMDs, but their specific effect on the cardiac phenotype and function is not yet fully understood. Significant prospective studies are necessary in order to understand and establish which diet best suits every patient depending on the inherited metabolic disorder. The most suitable imagistic monitoring method for the impact of different diets on the cardiovascular system is still under debate, with no protocols yet available. Echocardiography is readily available in most hospital settings and brings important information regarding the impact of diets on the left ventricular parameters. Cardiac MRI (magnetic resonance imaging) could better characterize the cardiac tissue and bring forth both functional and structural information. MDPI 2023-11-16 /pmc/articles/PMC10675151/ /pubmed/38004189 http://dx.doi.org/10.3390/nu15224795 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Luca, Alina Costina
Pădureț, Ioana-Alexandra
Țarcă, Viorel
David, Simona Georgiana
Mîndru, Dana Elena
Roșu, Solange Tamara
Roșu, Eduard Vasile
Adumitrăchioaiei, Heidrun
Bernic, Jana
Cojocaru, Elena
Țarcă, Elena
Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence
title Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence
title_full Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence
title_fullStr Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence
title_full_unstemmed Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence
title_short Nutritional Approach in Selected Inherited Metabolic Cardiac Disorders—A Concise Summary of Available Scientific Evidence
title_sort nutritional approach in selected inherited metabolic cardiac disorders—a concise summary of available scientific evidence
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10675151/
https://www.ncbi.nlm.nih.gov/pubmed/38004189
http://dx.doi.org/10.3390/nu15224795
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