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

Year 2025, Volume: 9 Issue: 3, 494 - 503, 30.09.2025

Büşra Akyol Yılmaz , Elvan Yılmaz Akyüz , Tanyel Zübarioğlu

https://doi.org/10.30621/jbachs.1659440

Abstract

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

Abstract

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.

Keywords

Diet , Glycogen storage diseases , Metabolic control , Nutritional status , Uncooked cornstarch

Ethical Statement

Ethical approval for the study was granted by the University of Health Sciences Şişli Hamidiye Etfal Training and Research Hospital Research and Application Center Clinical Research Ethics Committee (Decision No:2120/ 1076), and the study adhered to the Declaration of Helsinki standards.

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