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

  • Martin Biosse Duplan
  • Aurélie Hubert
  • Elvire Le Norcy
  • Alice Louzoun
  • Ariane Perry
  • Catherine Chaussain
  • Philippe Labrune
Glycogen Storage Disease
  • 90 Downloads

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.

Notes

Details of funding

The study was self-funded. The authors confirm independence from the sponsors; the content of the article has not been influenced by the sponsors.

Compliance with ethical standards

A competing interest statement

The co-authors have no competing interest to declare.

Informed consent

Written informed consent (and assent form when the child was less than 18 years) was obtained prior to enrolment in this study.

Documentation of approval from the institutional Committee for Care and use of laboratory animals (or comparable committee)

Not relevant.

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Copyright information

© SSIEM 2018

Authors and Affiliations

  1. 1.Service de Médecine Bucco-DentaireHôpital Bretonneau, HUPNVS, AP-HPParisFrance
  2. 2.UFR OdontologieUniversité Paris DescartesParisFrance
  3. 3.Centre de Référence des Maladies Héréditaires du Métabolisme HépatiqueHôpital Antoine Béclère, HUPS, AP-HPClamartFrance
  4. 4.Service de PédiatrieHôpital Antoine Béclère, HUPS, AP-HPClamartFrance
  5. 5.Université Paris SudOrsayFrance

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