Objective: Uncooked cornstarch (UCCS) is used widely for the treatment of patients with glycogen storage disease type I (GSD-I). Previous studies suggested that glucose absorption may be impaired in GSD-I. In order to measure utilization of UCCS in young adults with GSD-Ia and healthy controls, we used a 13C-breath test based on the natural enrichment of 13C in UCCS. Design: Open, not randomized, prospective interventional study. Methods: Following 1 g/kg UCCS, we studied eight subjects with GSD-Ia (7 males, 1 female; mean age 28.3 years, range 16-42 years) and 15 healthy controls (10 males, 5 females; mean age 23.5 years, range 19-36 years). Breath samples for analysis of 13CO2 enrichment were collected at baseline and at 30-min intervals for 6 h or until hypoglycaemia occurred. Indirect calorimetry was used to measure respiratory gas exchange. Intermediate metabolites, lipids and glucose were measured in plasma. Breath H2 concentrations were measured as an indicator of malabsorption. Results: Cumulative utilization over 6 h was significantly higher in controls (18.35 ± 6.2% of total carbohydrate intake) than in subjects with GSD-Ia (11.5 ± 4.7%) (P< 0.02). However, utilization of UCCS was virtually identical up to 2.5 h. Two subjects with GSD-Ia fulfilled the criteria for malabsorption. Conclusions: Starch digestion and absorption are not impaired in GSD-Ia. However, overall utilization of UCCS appears to be lower in GSD-Ia, which is most likely secondary to perturbed intermediary metabolism. There are important implications for treatment of this disorder. Ways to improve the efficacy of UCCS in GSD-I are needed.
|Original language||English (US)|
|Number of pages||6|
|Journal||European Journal of Gastroenterology and Hepatology|
|State||Published - Nov 1 2002|
- Breath test
- Glycogen storage disease
ASJC Scopus subject areas