A potential role of dietary lipids in the management of hepatic glycogen storage diseases (GSDs) has been proposed, but no consensus on management guidelines exists. The aim of this study was to describe current experiences with dietary lipid manipulations in hepatic GSD patients. An international study was set up to identify published and unpublished cases describing hepatic GSD patients with a dietary lipid manipulation. A literature search was performed according to the Cochrane Collaboration methodology through PubMed and EMBASE (up to December 2018). All delegates who attended the dietetics session at the IGSD2017, Groningen were invited to share unpublished cases. Due to multiple biases, only data on GSDIII were presented. A total of 28 cases with GSDIII and a dietary lipid manipulation were identified. Main indications were cardiomyopathy and/or myopathy. A high fat diet was the most common dietary lipid manipulation. A decline in creatine kinase concentrations (n = 19, P  < .001) and a decrease in cardiac hypertrophy in paediatric GSDIIIa patients (n = 7, P  < .01) were observed after the introduction with a high fat diet. This study presents an international cohort of GSDIII patients with different dietary lipid manipulations. High fat diet may be beneficial in paediatric GSDIIIa patients with cardiac hypertrophy, but careful long‐term monitoring for potential complications is warranted, such as growth restriction, liver inflammation, and hepatocellular carcinoma development.

中文翻译：

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糖原贮积病 III 型的膳食脂质：系统文献研究、案例研究和未来建议。

已提出膳食脂质在肝糖原贮积病 (GSD) 管理中的潜在作用，但尚未就管理指南达成共识。本研究的目的是描述肝脏 GSD 患者饮食脂质操作的当前经验。一项国际研究旨在确定已发表和未发表的病例，这些病例描述了通过饮食脂质操作描述肝 GSD 患者。根据 Cochrane Collaboration 方法通过 PubMed 和 EMBASE（截至 2018 年 12 月）进行文献检索。所有参加格罗宁根 IGSD2017 营养学会议的代表都受邀分享未发表的案例。由于存在多重偏见，仅提供了 GSDIII 的数据。总共确定了 28 例 GSDIII 和饮食脂质操作。主要适应症是心肌病和/或肌病。高脂肪饮食是最常见的饮食脂质操作。肌酸激酶浓度下降（n = 19, P  < .001) 并且 在引入高脂肪饮食后观察到儿科 GSDIIIa 患者的心脏肥大减少 ( n = 7, P < .01)。本研究介绍了具有不同饮食脂质操作的 GSDIII 患者的国际队列。高脂肪饮食可能对患有心脏肥大的儿科 GSDIIIa 患者有益，但需要仔细长期监测潜在并发症，如生长受限、肝脏炎症和肝细胞癌的发展。