Data highlighting effects of ketogenic diet on cardiomyopathy and hepatopathy in glycogen storage disease type IIIa

Marusic, Tatiana (author), Žerjav-Tanšek, Mojca (author), Širca-Čampa, Andreja (author), Mezek, Ajda (author), Berden, Pavel (author), Battelino, Tadej (author), Grošelj, Urh (author)

Datasets highlighting effects of ketogenic diet (KD) in a glycogen storage disease type IIIa patient is presented with the longest patient follow up report to date. Now a 15-year old girl with GSD type IIIa, diagnosed at 1 year of age, had initially introduced treatment with diet high carbohydrates, according to the recommendations. Progressively she developed left ventricular obstructive hypertrophy, hepatomegaly and skeletal myopathy. At the age of 11 years, she was introduced KD and continuous ketosis has been maintained for over 4 years providing longest reported follow up to date. KD introduction lead to a normalization of left ventricular parameters and ventricular mass and to an improvement in hepatic injury markers and decrease in liver size. We provided a table with biochemical parameters, a table providing detailed diet composition, tables with cardiac and hepatic measures and figures depicting cardiac NMR images; all the tables/figures are provided referring to the KD introduction (values prior/after). Interpretation of this data can be found in a case report article titled “Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa”.

• Language: English
• Keywords: inborn genetic diseases, glycogen storage disease, glycogen storage disease type III, hypertrophic cardiomyopathy, ketogenic diet
• Work type: Article
• Typology: 1.01 - Original Scientific Article
• Organization: MF - Faculty of Medicine
• Publication status: Published
• Publication version: Version of Record
• Year: 2020
• Number of pages: 9 str.
• Numbering: Vol. 32, art. 106205
• PID: 20.500.12556/RUL-138430
• UDC: 616.379
• ISSN on article: 2352-3409
• DOI: 10.1016/j.dib.2020.106205
• COBISS.SI-ID: 29256195
• Publication date in RUL: 20.07.2022

Record is a part of a journal

• Title: Data in brief
• Publisher: Elsevier
• ISSN: 2352-3409
• COBISS.SI-ID: 32117977

Licences

• License: CC BY-NC-ND 4.0, Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
• Link: http://creativecommons.org/licenses/by-nc-nd/4.0/
• Description: The most restrictive Creative Commons license. This only allows people to download and share the work for no commercial gain and for no other purposes.

Projects

• Funder: ARRS - Slovenian Research Agency
• Project number: V3-1505
• Name: Analiza in razvoj področja redkih bolezni v Sloveniji

• Funder: ARRS - Slovenian Research Agency
• Project number: P3-0343
• Name: Etiologija, zgodnje odkrivanje in zdravljenje bolezni pri otrocih in mladostnikih