AI Article Synopsis
- Glycogen storage diseases (GSDs) are rare genetic conditions that impact glycogen metabolism and can affect multiple body systems, including the liver and muscles, with possible oral implications.
- A study of 60 patients with four types of GSD (Ia, Ib, III, and IX) identified both common and specific oral health issues across the different GSD types, including delayed tooth eruption and tooth shape abnormalities.
- The research found that while caries rates were similar to the general population, GSD Ib patients showed a higher prevalence of severe periodontitis, particularly due to associated neutropenia.
Article 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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| http://dx.doi.org/10.1007/s10545-018-0182-3 | DOI Listing |
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