A life-long dietary intervention can affect the substrates’ availability for gut fermentation in metabolic diseases such as the glycogen-storage diseases (GSD). Besides drug consumption, the main treatment of types GSD-Ia and Ib to prevent metabolic complications is a specific diet with definite nutrient intakes. In order to evaluate how deeply this dietary treatment affects gut bacteria, we compared the gut microbiota of nine GSD-I subjects and 12 healthy controls (HC) through 16S rRNA gene sequencing; we assessed their dietary intake and nutrients, their microbial short chain fatty acids (SCFAs) via gas chromatography and their hematic values. Both alpha-diversity and phylogenetic analysis revealed a significant biodiversity reduction in the GSD group compared to the HC group, and highlighted profound differences of their gut microbiota. GSD subjects were characterized by an increase in the relative abundance of Enterobacteriaceae and Veillonellaceae families, while the beneficial genera Faecalibacterium and Oscillospira were significantly reduced. SCFA quantification revealed a significant increase of fecal acetate and propionate in GSD subjects, but with a beneficial role probably reduced due to unbalanced bacterial interactions; nutritional values correlated to bacterial genera were significantly different between experimental groups, with nearly opposite cohort trends.

中文翻译：

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糖原贮积病1型患者肠道菌群中的细菌过度生长和产生丁酸酯的类群消耗。

终身饮食干预可能会影响代谢疾病（例如糖原存储疾病（GSD））中肠道发酵的底物可用性。除了药物消耗外，GSD-1a和Ib型预防代谢并发症的主要治疗方法是特定的饮食，并摄入一定的营养。为了评估这种饮食疗法对肠道细菌的影响，我们通过16S rRNA基因测序比较了9名GSD-1受试者和12名健康对照（HC）的肠道菌群。我们通过气相色谱法评估了他们的饮食摄入量和营养素，微生物短链脂肪酸（SCFA）及其血液价值。α多样性和系统发育分析均显示，与HC组相比，GSD组的生物多样性显着减少，并突显了其肠道菌群的巨大差异。肠杆菌科和Veillonellaceae家族，而有益的Faecalibacterium和Oscillospira属明显减少。SCFA定量显示GSD受试者粪便中乙酸和丙酸的含量显着增加，但有益的作用可能是由于细菌相互作用的不平衡而降低。实验组之间与细菌属相关的营养价值显着不同，队列趋势几乎相反。