Marusic Tatiana, Zerjav Tansek Mojca, Sirca Campa Andreja, Mezek Ajda, Berden Pavel, Battelino Tadej, Groselj Urh
University Children's Hospital, University Medical Center Ljubljana, Bohoričeva ulica 20, Ljubljana, Slovenia.
Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, Slovenia.
Data Brief. 2020 Aug 21;32:106205. doi: 10.1016/j.dib.2020.106205. eCollection 2020 Oct.
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".
本文展示了关于生酮饮食（KD）对一名IIIa型糖原贮积病患者影响的数据集，并给出了迄今为止最长的患者随访报告。一名15岁的IIIa型糖原贮积病女孩，1岁时被诊断出该病，最初按照建议采用高碳水化合物饮食进行治疗。她逐渐出现左心室梗阻性肥厚、肝肿大和骨骼肌病。11岁时，她开始采用生酮饮食，持续酮症已维持了4年多，这是迄今为止报道的最长随访时间。采用生酮饮食后，左心室参数和心室质量恢复正常，肝损伤标志物改善，肝脏大小减小。我们提供了一张生化参数表、一张详细饮食组成表、几张心脏和肝脏测量表以及描绘心脏核磁共振图像的图表；所有表格/图表均提供了采用生酮饮食前后的数据。该数据的解读可在一篇病例报告文章《IIIa型糖原贮积病（GSD）患者生酮饮食使梗阻性心肌病恢复正常并改善肝病》中找到。
