Oral Health Status of Individuals with Glycogen Storage Disease

Glycogen Storage Disease (GSD) is a genetic condition caused by defective glycogen metabolism resulting in hypoglycemic episodes. GSD-associated hypoglycemia is prevented by consuming cornstarch orally or via a gastrostomy tube (G-tube) every 2-4 hours throughout the day and night. Continual oral consumption of cornstarch produces a film around the teeth where caries may potentially develop.

Objective: To compare the oral health of GSD patients fed cornstarch orally with those fed via a G-tube.

Methods: Participants were aged 2 to 56 years old attending the UF GSD Program. Following informed consent and a blood sample to check for indicators of periodontal disease, participants had a dental exam and sample collection of their plaque, saliva, and gingival crevicular fluid. They completed a questionnaire about their mode and frequency of cornstarch feeding and their oral hygiene practices. Participants also received dental prophylaxis, radiographs and topical fluoride.

Results: Data collection is ongoing and so far, 13 subjects with GSD have participated. Eleven patients receive cornstarch orally and 2 are fed by G-tube. Seven of the participants had Type 1a GSD, three patients had Type 1b, two siblings had Type XI, and one patient had Type III GSD. All reported receiving cornstarch during the night and not brushing their teeth following night-time feeding. All Type 1b subjects were diagnosed with periodontal disease, while the remainder of GSD subjects had healthy teeth and gums.

Conclusion: Preliminary results suggest that despite night-time oral cornstarch feedings, patients with GSD who practice good oral hygiene habits and have regular dental care exhibit good oral health. This may not be the case for Type 1b GSD patients, who are at greater risk for periodontal disease due to neutropenia and neutrophil dysfunction.