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01-11-2018 | Glycogen Storage Disease

Dental and periodontal manifestations of glycogen storage diseases: a case series of 60 patients

Authors: Martin Biosse Duplan, Aurélie Hubert, Elvire Le Norcy, Alice Louzoun, Ariane Perry, Catherine Chaussain, Philippe Labrune

Published in: Journal of Inherited Metabolic Disease | Issue 6/2018

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.

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Title: Dental and periodontal manifestations of glycogen storage diseases: a case series of 60 patients
Authors: Martin Biosse Duplan
Aurélie Hubert
Elvire Le Norcy
Alice Louzoun
Ariane Perry
Catherine Chaussain
Philippe Labrune
Publication date: 01-11-2018
Publisher: Springer Netherlands
Published in: Journal of Inherited Metabolic Disease / Issue 6/2018
Print ISSN: 0141-8955
Electronic ISSN: 1573-2665
DOI: https://doi.org/10.1007/s10545-018-0182-3

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