Oral Manifestations in Patients with Glycogen Storage Disease: A Systematic Review of the Literature

(1) Background: Glycogen storage disease (GSD) represents a group of twenty-three types of metabolic disorders which damage the capacity of body to store glucose classified basing on the enzyme deficiency involved. Affected patients could present some oro-facial alterations: the purpose of this revi...

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Published inApplied sciences Vol. 10; no. 19; p. 6720
Main Authors Romano, Antonio, Russo, Diana, Contaldo, Maria, Lauritano, Dorina, della Vella, Fedora, Serpico, Rosario, Lucchese, Alberta, Di Stasio, Dario
Format Journal Article
LanguageEnglish
Published Basel MDPI AG 01.10.2020
Subjects
Bias
Cleft lip/palate
Criteria
Dental caries
Diagnosis
Disease
Enamel
Enzymes
Genetic aspects
Glycogen
glycogen storage disease
Glycogenosis
Glycogens
Literature reviews
Metabolic disorders
Oral cavity
oral manifestations
Oral manifestations of general diseases
oral medicine
Patient outcomes
Patients
Pediatrics
Periodontitis
Physiological aspects
review
Storage diseases
Systematic review
Tongue
Ulcers
Italy
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Summary:(1) Background: Glycogen storage disease (GSD) represents a group of twenty-three types of metabolic disorders which damage the capacity of body to store glucose classified basing on the enzyme deficiency involved. Affected patients could present some oro-facial alterations: the purpose of this review is to catalog and characterize oral manifestations in these patients. (2) Methods: a systematic review of the literature among different search engines using PICOS criteria has been performed. The studies were included with the following criteria: tissues and anatomical structures of the oral cavity in humans, published in English, and available full text. Review articles and paper published before 1990 were excluded. (3) Results: 757 articles were identified in the initial search. In the end, 45 articles that met the selection criteria has been analyzed. The information extracted from the articles was classified according to the type of GSD (Ia; Ib; II; III; V; XIV). Oral manifestations range from dental caries to severe periodontitis in paediatric patients, from diffuses and recurrent oral ulcers in the cleft lip and palate. (4) Conclusions: Although considered a rare disease, GSD can present a varied number of oral manifestations. Therefore, it is of great importance for the oral medicine specialist to know and classify them.
ISSN:2076-3417
2076-3417
DOI:10.3390/app10196720