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

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...

Full description

Bibliographic Details
Main Authors: Tatiana Marusic, Mojca Zerjav Tansek, Andreja Sirca Campa, Ajda Mezek, Pavel Berden, Tadej Battelino, Urh Groselj
Format: Article
Language:English
Published: Elsevier 2020-09-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Glycogen storage disease
GSD type IIIa
Cardiomyopathy
Ketogenic diet
Case report
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426920300744
id doaj-d3dce2660eee48c0ad6f1e163ea7309c
record_format Article
spelling doaj-d3dce2660eee48c0ad6f1e163ea7309c2020-11-25T03:56:51ZengElsevierMolecular Genetics and Metabolism Reports2214-42692020-09-0124100628Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIaTatiana Marusic0Mojca Zerjav Tansek1Andreja Sirca Campa2Ajda Mezek3Pavel Berden4Tadej Battelino5Urh Groselj6University Children's Hospital, University Medical Center Ljubljana, Bohoričeva ulica 20, Ljubljana, SloveniaUniversity Children's Hospital, University Medical Center Ljubljana, Bohoričeva ulica 20, Ljubljana, Slovenia; Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, SloveniaUniversity Children's Hospital, University Medical Center Ljubljana, Bohoričeva ulica 20, Ljubljana, SloveniaUniversity Children's Hospital, University Medical Center Ljubljana, Bohoričeva ulica 20, Ljubljana, SloveniaClinical Institute of Radiology, University Medical Center Ljubljana, Zaloška cesta 7, 1000 Ljubljana, SloveniaUniversity Children's Hospital, University Medical Center Ljubljana, Bohoričeva ulica 20, Ljubljana, Slovenia; Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, SloveniaUniversity Children's Hospital, University Medical Center Ljubljana, Bohoričeva ulica 20, Ljubljana, Slovenia; Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, Slovenia; Corresponding author at: University Children's Hospital, University Medical Center Ljubljana, Bohoriceva ulica 20, 1000 Ljubljana, Slovenia.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.http://www.sciencedirect.com/science/article/pii/S2214426920300744Glycogen storage diseaseGSD type IIIaCardiomyopathyKetogenic dietCase report
collection DOAJ
language English
format Article
sources DOAJ
author Tatiana Marusic
Mojca Zerjav Tansek
Andreja Sirca Campa
Ajda Mezek
Pavel Berden
Tadej Battelino
Urh Groselj
spellingShingle Tatiana Marusic
Mojca Zerjav Tansek
Andreja Sirca Campa
Ajda Mezek
Pavel Berden
Tadej Battelino
Urh Groselj
Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa
Molecular Genetics and Metabolism Reports
Glycogen storage disease
GSD type IIIa
Cardiomyopathy
Ketogenic diet
Case report
author_facet Tatiana Marusic
Mojca Zerjav Tansek
Andreja Sirca Campa
Ajda Mezek
Pavel Berden
Tadej Battelino
Urh Groselj
author_sort Tatiana Marusic
title 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_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_sort normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (gsd) type iiia
publisher Elsevier
series Molecular Genetics and Metabolism Reports
issn 2214-4269
publishDate 2020-09-01
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.
topic Glycogen storage disease
GSD type IIIa
Cardiomyopathy
Ketogenic diet
Case report
url http://www.sciencedirect.com/science/article/pii/S2214426920300744
work_keys_str_mv AT tatianamarusic normalizationofobstructivecardiomyopathyandimprovementofhepatopathyonketogenicdietinpatientwithglycogenstoragediseasegsdtypeiiia
AT mojcazerjavtansek normalizationofobstructivecardiomyopathyandimprovementofhepatopathyonketogenicdietinpatientwithglycogenstoragediseasegsdtypeiiia
AT andrejasircacampa normalizationofobstructivecardiomyopathyandimprovementofhepatopathyonketogenicdietinpatientwithglycogenstoragediseasegsdtypeiiia
AT ajdamezek normalizationofobstructivecardiomyopathyandimprovementofhepatopathyonketogenicdietinpatientwithglycogenstoragediseasegsdtypeiiia
AT pavelberden normalizationofobstructivecardiomyopathyandimprovementofhepatopathyonketogenicdietinpatientwithglycogenstoragediseasegsdtypeiiia
AT tadejbattelino normalizationofobstructivecardiomyopathyandimprovementofhepatopathyonketogenicdietinpatientwithglycogenstoragediseasegsdtypeiiia
AT urhgroselj normalizationofobstructivecardiomyopathyandimprovementofhepatopathyonketogenicdietinpatientwithglycogenstoragediseasegsdtypeiiia
_version_ 1724463475463290880

Similar Items