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...

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Veröffentlicht in:Molecular genetics and metabolism reports 2020-09, Vol.24, p.100628-100628, Article 100628
Hauptverfasser: Marusic, Tatiana, Zerjav Tansek, Mojca, Sirca Campa, Andreja, Mezek, Ajda, Berden, Pavel, Battelino, Tadej, Groselj, Urh
Format: Artikel
Sprache:eng
Schlagworte:
Cardiomyopathy
Case report
Glycogen storage disease
GSD type IIIa
Ketogenic diet
Short Communication
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Zusammenfassung: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.
ISSN:2214-4269
2214-4269
DOI:10.1016/j.ymgmr.2020.100628