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

Autor: Urh Groselj, Tadej Battelino, Pavel Berden, Ajda Mezek, Andreja Sirca Campa, Tatiana Marusic, Mojca Zerjav Tansek
Rok vydání: 2020
Předmět:
Zdroj: Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports, Vol 24, Iss, Pp 100628-(2020)
ISSN: 2214-4269
Popis: 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.
Databáze: OpenAIRE