Dental and periodontal manifestations of glycogen storage diseases: a case series of 60 patients. Issue 6 (16th April 2018)

• Record Type: Journal Article
• Authors: Biosse Duplan, Martin; Hubert, Aurélie; Le Norcy, Elvire; Louzoun, Alice; Perry, Ariane; Chaussain, Catherine; Labrune, Philippe
• Is Part Of: Journal of inherited metabolic disease. Volume 41:Issue 6(2018)
• Page Start: 947
• Page End: 953
• Publication Date: 2018-04-16
• Subjects: Metabolism, Inborn errors of -- Periodicals; Metabolism -- Disorders -- Periodicals; 616.39042
• DOI: 10.1007/s10545-018-0182-3
• Languages: English
• ISSNs: 0141-8955

Abstract

Abstract: Glycogen storage diseases (GSDs) are rare genetic disorders of glycogen metabolism where the liver, kidneys, respiratory and cardiac muscles, as well as the immune and skeletal systems can be affected. Oral manifestations can also be present, but the specificity and frequency of these manifestations in the different forms of GSD are unknown. Analysis of a case series of 60 patients presenting four types of GSD (Ia, Ib, III, and IX) showed that the different types of GSDs have common and specific oral manifestations. In none of the GSD types studied, the prevalence of caries was higher than in the general population, especially in patients benefiting from current nutritional therapy, while in all GSD types the prevalence of delayed tooth eruption, agenesis, and tooth shape abnormalities was increased compared to the general population. Severe periodontitis prevalence was increased in patients with GSD Ib and neutropenia. Our results show that GSDs have oral manifestations and suggest some specificity depending on the type of GSDs.