Dietary Fiber Supplementation in Type I Glycogen Storage Disease; Could it Contribute to a Better Metabolic Control?

Abstract

Introduction: Glycogen storage disease type I (GSD I) is one of rare inborn error of metabolic disorder inherited with autosomal recessively. It has a key role both in glycogenolysis and gluconeogenesis. Infants exhibit hypoglycemic episodes, developmental delay, growth retardation, hepatomegaly accompanying with increased blood concentrations of lactic acid, triglycerides, cholesterol, and uric acid. The major treatment is frequent feeding containing slow release carbohydrate supplementation. In this study we aimed to investigate the effects of adequate fiber supplementation to metabolic controls of patients with GSD I. Materials and Methods: Patients followed up in our clinic with GSD I were enrolled to the study. Fiber amounts to be supplemented for each induvidual were calculated according to 3 day diet dairy and daily recommended amounts. Patients were subjected to 6 day continuous glucose monitorization and their biochemical metabolic parameters were reqruited before and at the end of 6 week fiber supplementation. Results: Mean, lowest and highest glucose values insignificantly decreased, however HbA1c levels significantly increased. Lactate, cholesterol, LDL, AST, and uric acid mean values also decreased, however the differences were not statistically significant. In addition, decreased mean levels of triglyceride, GGT were statistically significant. Conclusion: The results of this study suggest the improvment of glucose homeostasis and biochemical parameters which encourage for the use of adequate fiber supplementation in terms of contributing metabolic control in GSD I patients. However, randomized, double blinded controlled prospective long-term trials with expanded number of patients are required to investigate the effects of fiber supplementation perspectively.