Effect of Uncooked Cornstarch Treatment on Metabolic Control in Patients With Hepatic Glycogen Storage Diseases

Background and Purpose: Glycogen storage diseases (GSD) are a group of rare inherited metabolic disorders. Hypoglycemia, the most severe clinical manifestation of hepatic GSD, can be averted particularly with the supplementation of uncooked cornstarch. This study aims to elucidate the nutritional condition of individuals with hepatic GSD and to examine the impact of dietary therapy using uncooked cornstarch on metabolic regulation. Methods: This study was a single-center cross-sectional prospective design and included 8 follow-up patients with hepatic GSD patients aged 0-18 years. Demographic characteristics, anthropometric measurements, biochemical markers, energy expenditure, and macronutrient intake of patients were assessed over a period of six months. Results: The mean age of the patients was 8.0 ± 5.9 years. 25% of the patients were female, while 75% were male. A significant difference was observed in plasma fasting glucose and alanine aminotransferase levels among patients with uncooked cornstarch intake (p=0.024; p=0.044, respectively) and extended overnight fasting durations. The patients' weight, height, and abdominal circumference exhibited significant changes during the 6-month follow-up period (p=0.038; p=0.001; p=0.008, respectively). Conclusions: An uncooked cornstarch-based diet in individuals with hepatic glycogen storage diseases effectively maintains normoglycemia and mitigates possible metabolic consequences. Its appropriate use facilitates adequate growth and development in patients and plays a vital function in metabolic regulation.