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

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

References

1. Chen MA, Weinstein DA. Glycogen storage diseases: diagnosis, treatment and outcomes. Transl Sci Rare Dis. 2016;1(1):45–72.
2. Walter JH, Labrune P, Laforet P. The glycogen storage diseases and related disorders. In: Saudubray JM, Baumgartner MR, Garcia-Cazorla A, Walter J, editors. Inborn Metabolic Diseases: Diagnosis and Treatment. Springer; 2022. p. 179–200.
3. Kaiser N, Gautschi M, Bosanska L, et al. Glycemic control and complications in glycogen storage disease type I: results from the Swiss registry. Mol Genet Metab. 2019;126:355–61.
4. Scott EM, Wenger OK, Robinson E, et al. Glycogen storage disease type 1a in the Ohio Amish. JIMD Rep. 2022;63(5):453–61.
5. Ross KM, Ferrecchia IA, Dahlberg KR, Dambska M, Ryan PT, Weinstein DA. Dietary management of the glycogen storage diseases: evolution of treatment and ongoing controversies. Adv Nutr. 2020;11(2):439–46.
6. Stone WL, John AT, Anastasopoulou C, Basit H, Adil A. Glycogen Storage Disease. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025.
7. Derks TGJ, Rodriguez-Buritica DF, Ahmad A, et al. Glycogen storage disease type Ia: current management options, burden and unmet needs. Nutrients. 2021;13(11):3828.
8. World Health Organization (WHO). WHO Child Growth Standards: Length/height-for-age, weight-for-age, weight-for-length, weight-for-height and body mass index-for-age: methods and development [Internet]. Geneva: WHO; 2006 [cited 2025 Jan 15]. Available from: https://www.who.int/publications/i/item/924154693X

9. Centers for Disease Control and Prevention (CDC). National Center for Health Statistics [Internet]. Atlanta (GA): CDC; 2000 [cited 2025 Jan 15]. Available from: http://www.cdc.gov/growthcharts
10. Acosta PB. Nutrition Support Protocols: The Ross Metabolic Formula System. 4th ed. Columbus (OH): Ross Products Division, Abbott Laboratories; 2001.
11. Rake JP, Visser G, Labrune P, Leonard JV, Ullrich K, Smit GPA. Guidelines for management of glycogen storage disease type I: European Study on Glycogen Storage Disease Type I (ESGSD I). Eur J Pediatr. 2002;161(1):112–9.
12. Dambska M, Labrador EB, Kuo CL, Weinstein DA. Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control. Pediatr Diabetes. 2017;18(5):327–31.
13. Santos BL, Souza CF, Schuler-Faccini L, et al. Glycogen storage disease type I: clinical and laboratory profile. J Pediatr (Rio J). 2014;90(6):572–9.
14. Santos BB, Nalin T, Grokoski KC, et al. Nutritional status and body composition in patients with hepatic glycogen storage diseases treated with uncooked cornstarch-a controlled study. J Inborn Errors Metab Screen. 2017; 5:1–7.
15. Lee PJ, Dixon MA, Leonard JV. Uncooked cornstarch—efficacy in type I glycogenosis. Arch Dis Child. 1996;74:546–7.
16. Chen YT, Bazzarre CH, Lee MM, Sidbury JB, Coleman RA. Type I glycogen storage disease: nine years of management with cornstarch. Eur J Pediatr. 1993;152(1):56–9.
17. Hanaa EK, Mona SEK, Ghada A, Mogahed E. The effect of tailoring of cornstarch intake on stature in children with glycogen storage disease type III. J Pediatr Endocrinol Metab. 2015;28(1–2):195–200.
18. Wolfsdorf JI, Plotkin RA, Laffel LM, Crigler JF. Continuous glucose for treatment of patients with type I glycogen-storage disease: comparison of the effects of dextrose and uncooked cornstarch on biochemical variables. Am J Clin Nutr. 1990;52(6):1043–50.
19. Szymańska E, Jóźwiak-Dzięcielewska DA, Gronek J, et al. Hepatic glycogen storage diseases: pathogenesis, clinical symptoms and therapeutic management. Arch Med Sci. 2021;17(2):304–13.
20. Wolfsdorf JI, Crigler JF. Cornstarch regimens for nocturnal treatment of young adults with type I glycogen storage disease. Am J Clin Nutr. 1997;65:1507–11.
21. Weinstein DA, Wolfsdorf JI. Effect of continuous glucose therapy with uncooked cornstarch on the long-term clinical course of type 1a glycogen storage disease. Eur J Pediatr. 2002;161(1):35–9.
22. Ross KM, Brown LM, Corrado MM, et al. Safety and efficacy of chronic extended-release cornstarch therapy for glycogen storage disease type I. J Inherit Metab Dis Rep. 2016;26:85–90.
23. Herbert M, Pendyal S, Rairikar M, Halaby C, Benjamin RW, Kishnani PS. Role of continuous glucose monitoring in the management of glycogen storage disorders. J Inherit Metab Dis. 2018;41:917–27.
24. Gümüş E, Özen H. Glycogen storage diseases: an update. World J Gastroenterol. 2023;29(25):3932–63.
25. Ross KM, Brown LM, Corrado MM, et al. Safety and efficacy of long-term use of extended-release cornstarch therapy for glycogen storage disease types 0, III, VI, and IX. J Nutr Ther. 2015; 4:137–42.
26. Karwowski, C., Galambos, C., Finegold, D., & Shneider, B. L. (2011). Markedly elevated serum transaminases in glycogen storage disease type III. Journal of Pediatric Gastroenterology and Nutrition, 52(5), 621–623.
27. Chehida AB, Messaoud SB, Abdelaziz RB, et al. A lower energetic, protein and uncooked cornstarch intake is associated with a more severe outcome in glycogen storage disease type III: an observational study of 50 patients. J Pediatr Endocrinol Metab. 2018;31(9):979–86.
28. Özkaya Ş, Gökmen Özel HF. Comparison of nutritional status and anthropometric measurements of seventh grade primary school students from different socioeconomic status. J of Nutr and Diet (Beslenme ve Diyet Dergisi). 2011;39(1-2):9–19.