Abstract

Glycogen storage diseases (GSDs) are complex hereditary metabolic diseases regarding glycogen metabolism involving different organs and systems. Patients with GSDs require a multidisciplinary approach to therapy, from diagnosis and throughout life. Improving the life course prognosis of patients has shifted the primary objective of management from exclusively avoiding hypoglycemia and guaranteeing survival to the prevention of long-term complications through personalized dietary approaches based on the individual's needs. Nevertheless, the special diet followed by patients may promote gut dysbiosis. The alteration of gut-microbiota composition may probably lead to clinical manifestations linked to an oxy-inflammatory status due to the bidirectional communication through the gut-liver axis. These considerations lay the foundations for a more comprehensive clinical study to define possible therapeutic interventions that can modulate the composition of gut microbiota and reduce oxy-inflammation.