Microbial Profile of Supragingival and Subgingival Plaque of Patients With Glycogen Storage Disease

Abstract

Patients with glycogen storage disease (GSD) are either orally fed (ORF) or gastronomy-tube fed (GTF) with cornstarch to maintain normal glucose levels. It is not known whether the use of cornstarch affects the microbiological oral profile of patients with GSD. Thus, the purpose of this study was to compare supragingival and subgingival plaque samples collected from 53 participants with GSD (2-56 years)-29 ORF and 24 GTF. The 16S sequence bacterial profiles of plaque DNA were obtained and a total of 768 probes were detected across the plaque groups. Orally fed patients showed higher means of cariogenic species and periodontal health-associated species, whereas GTF patients showed higher means of periopathogenic species (P < .05). Orally fed patients exhibited high levels of caries pathogens and lower levels of periodontal pathogens possibly due to the acidic environment created by their cornstarch diet, when compared to GTF patients.

Periodontal Manifestation of Type Ib Glycogen Storage Disease: A Rare Case Report

“…12 Garcia et al found that caries susceptibility in GSD patients was more in orally fed patients than in gastronomy-tube fed patients due to the acidic environment created by their cornstarch diet. 13 The appearance of dark spots and hypomineralization on the enamel of deciduous teeth represents enamel-related susceptibility to dental caries was also reported by Bartoli et al 14 Although several authors reported delayed eruption in patients with different types of GSD including Type Ib, [11][12][13] this finding was not evident in the present case. Severe periodontitis in children is often observed as a manifestation of hematologic or genetic disorders including GSDs.…”

“…5 The lack of glucose-6-phosphatase translocase prevents the transport of G6P across the endoplasmic reticulum, and as a result, glycogen cannot metabolize glucose and is deposited in gluconeogenic organs. 13 This, in turn, leads to the common metabolic phenotype that is characterized by hypoglycemia, hepatomegaly, nephromegaly, hyperlipidemia, hyperuricemia, lactic acidemia, and growth retardation. 6 GSD-Ib is associated with neutropenia and neutrophil dysfunction that cause increased susceptibility to recurrent bacterial infections, aphthous stomatitis, and inflammatory bowel disease.…”