Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa

Now 15-year-old girl with glycogen storage disease (GSD) type IIIa (OMIM 232400) developed severe left ventricular obstructive hypertrophy and hepatomegaly while treated with frequent cornstarch meals. Subsequently, she was introduced the ketogenic diet; continuous ketosis has been maintained for ov...

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Autores principales: Marusic, Tatiana, Zerjav Tansek, Mojca, Sirca Campa, Andreja, Mezek, Ajda, Berden, Pavel, Battelino, Tadej, Groselj, Urh
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Short Communication
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7371897/
https://www.ncbi.nlm.nih.gov/pubmed/32714838
http://dx.doi.org/10.1016/j.ymgmr.2020.100628
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author Marusic, Tatiana
Zerjav Tansek, Mojca
Sirca Campa, Andreja
Mezek, Ajda
Berden, Pavel
Battelino, Tadej
Groselj, Urh
author_facet Marusic, Tatiana
Zerjav Tansek, Mojca
Sirca Campa, Andreja
Mezek, Ajda
Berden, Pavel
Battelino, Tadej
Groselj, Urh
author_sort Marusic, Tatiana
collection PubMed
description Now 15-year-old girl with glycogen storage disease (GSD) type IIIa (OMIM 232400) developed severe left ventricular obstructive hypertrophy and hepatomegaly while treated with frequent cornstarch meals. Subsequently, she was introduced the ketogenic diet; continuous ketosis has been maintained for over the last 4 years. After the introduction of ketogenic diet, a normalization of the cardiomyopathy and improvement of hepatopathy was achieved, with enhanced overall quality of life.
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spelling pubmed-73718972020-07-23 Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa Marusic, Tatiana Zerjav Tansek, Mojca Sirca Campa, Andreja Mezek, Ajda Berden, Pavel Battelino, Tadej Groselj, Urh Mol Genet Metab Rep Short Communication Now 15-year-old girl with glycogen storage disease (GSD) type IIIa (OMIM 232400) developed severe left ventricular obstructive hypertrophy and hepatomegaly while treated with frequent cornstarch meals. Subsequently, she was introduced the ketogenic diet; continuous ketosis has been maintained for over the last 4 years. After the introduction of ketogenic diet, a normalization of the cardiomyopathy and improvement of hepatopathy was achieved, with enhanced overall quality of life. Elsevier 2020-07-16 /pmc/articles/PMC7371897/ /pubmed/32714838 http://dx.doi.org/10.1016/j.ymgmr.2020.100628 Text en © 2020 The Authors http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Short Communication
Marusic, Tatiana
Zerjav Tansek, Mojca
Sirca Campa, Andreja
Mezek, Ajda
Berden, Pavel
Battelino, Tadej
Groselj, Urh
Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa
title Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa
title_full Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa
title_fullStr Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa
title_full_unstemmed Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa
title_short Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa
title_sort normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (gsd) type iiia
topic Short Communication
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7371897/
https://www.ncbi.nlm.nih.gov/pubmed/32714838
http://dx.doi.org/10.1016/j.ymgmr.2020.100628
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