Association of the children's dietary inflammatory index (c-DII) with thiol/disulfide homeostasis in pediatric glycogen storage disease: a cross-sectional exploratory study.

OBJECTIVE: The dietary pro-inflammatory burden observed among patients receiving medical nutritional therapies for glycogen storage disease (GSD) and its association with cellular redox balance have not been sufficiently elucidated in the literature. The aim of this cross-sectional study was to evaluate the association of the dietary inflammatory potential (Children's Dietary Inflammatory Index, c-DII) with systemic thiol/disulfide homeostasis and selected disease-specific clinical markers in pediatric patients with GSDI and GSDIII. MATERIALS AND METHODS: This cross-sectional study included 16 pediatric patients with GSD (GSDI, n = 8; GSDIII, n = 8) and 15 age-and sex-matched healthy controls. The inflammatory potential of the diet was calculated using 22 parameters obtained from 3-day retrospective dietary intake records and energy-standardized (1,000 kcal) c-DII. Serum dynamic thiol/disulfide homeostasis parameters were analyzed using a spectrophotometric method, and associations between c-DII scores and biochemical parameters were evaluated using correlation analyses. RESULTS: The mean c-DII score in the GSD cohort was positive, indicating an overall pro-inflammatory potential of the observed dietary intake. In biochemical analyses, while native and total thiol levels in the GSD group were statistically significantly lower than in healthy controls (p < 0.05), the disulfide/native thiol and disulfide/total thiol ratios, indicating a cellular pro-oxidant shift, were significantly increased (p < 0.05). Elevated c-DII scores were found to be significantly associated with high disulfide levels and disulfide-based ratios. Furthermore, significant positive correlations were found between the c-DII score and subtype-specific clinical markers of damage (uric acid in GSDI, creatine kinase in GSDIII). CONCLUSION: Medical nutritional therapies, which are of vital importance in the management of GSD, may paradoxically be associated with a pro-inflammatory dietary burden and systemic antioxidant depletion. These preliminary findings suggest that integrating the energy-adjusted c-DII into clinical monitoring may offer an innovative strategy for assessing diet-related cellular stress in pediatric GSD. However, its definitive clinical utility requires confirmation in larger prospective studies.