P1026 - IS UNCOOKED CORNSTARCH CONSUMPTION ASSOCIATED WITH ALTERED ANTHROPOMETRIC STATUS IN PEDIATRIC HEPATIC GLYCOGEN STORAGE DISEASE?

P1026

IS UNCOOKED CORNSTARCH CONSUMPTION ASSOCIATED WITH ALTERED ANTHROPOMETRIC STATUS IN PEDIATRIC HEPATIC GLYCOGEN STORAGE DISEASE?

O. Aydin^1,*, H. Ozen², S. Dagdelen³, H. Gokmen-Ozel¹

¹Faculty of Health Sciences, Department of Nutrition and Dietetics , ²Faculty of Medicine, Department of Internal Medicine, Division of Pediatrics, , ³Faculty of Medicine, Department of Internal Medicine, Division of Endocrinology and Metabolism, Hacettepe University, Ankara, Türkiye

Rationale: Hepatic glycogen storage diseases (GSDs) are primarily managed through dietary interventions, including frequent meals and uncooked cornstarch (UCCS) supplementation to maintain euglycemia. These strategies may impact anthropometric parameters. This study aimed to assess anthropometric measures and UCCS consumption in Turkish children with hepatic GSD.

Methods: Sixteen pediatric patients diagnosed with hepatic GSD (types I/III/VI/IX; n=5/6/1/4, respectively) with a mean age of 12.25±3.55 years, were included. Age, sex, anthropometric measurements, and UCCS intake habits were recorded. Body composition was evaluated using bioelectrical impedance analysis. Patients were grouped as non-ketotic (GSD I) and ketotic GSDs (III/VI/IX) for comparison.

Results: The median BMI z-score was 0.26 (IQR: -0.70 – 1.05). Patients with GSD I had higher median BMI z-scores (1.99 vs. 0.24) and lower height-for-age z-scores (–2.20 vs. –1.18) compared to those with ketotic GSDs, although these differences were not statistically significant (p>0.05). Patients with GSD I had also higher median body fat percentage (45.40 vs. 24.60; p<0.05) compared to ketotic GSDs. All patients were receiving UCCS. Median daily energy intake (2355 vs. 2246 kcal/day; p>0.05) and UCCS consumption (160 vs. 36 g/day and 3.61 vs. 1.11 g/kg/day, p<0.05) were also higher in GSD I patients. A significant positive correlation was observed between body fat percentage and both total daily UCCS intake (r=0.713, p=0.002) and intake per kilogram of body weight (r=0.708, p=0.002).

Conclusion: Children with hepatic GSD, particularly type I, show increased adiposity and impaired linear growth, possibly due to excessive caloric and UCCS intake. These findings highlight the importance of individualized and carefully monitored nutritional management in this population.

Disclosure of Interest: None declared