20.500.12556/RUL-138430 Data highlighting effects of ketogenic diet on cardiomyopathy and hepatopathy in glycogen storage disease type IIIa 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”. inborn genetic diseases glycogen storage disease glycogen storage disease type III hypertrophic cardiomyopathy ketogenic diet true false true Angleški jezik Ni določen Članek v reviji 2022-07-20 12:12:09 2022-07-20 12:12:11 2022-09-15 04:16:45 0000-00-00 00:00:00 2020 0 0 9 str. Vol. 32, art. 106205 2020 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 0000-00-00 616.379 2352-3409 10.1016/j.dib.2020.106205 29256195 1-s2.0-S2352340920310994-main.pdf 1-s2.0-S2352340920310994-main.pdf 1 839DE9E8A1ECCA69957DB32C18B130D7 7b38e558983de30a6b02547fdba3908926000cfbf21529fa50a824f57f377625 83ec5a7e-0814-11ed-8aca-00155dcfd717 https://repozitorij.uni-lj.si/Dokument.php?lang=slv&id=158756 https://www.sciencedirect.com/science/article/pii/S2352340920310994 0 2c082eb0-0814-11ed-8aca-00155dcfd717 https://repozitorij.uni-lj.si/Dokument.php?lang=slv&id=158755 Medicinska fakulteta 0 0 0