STUDY OF DENTAL AND ORAL CHARACTERISTICS IN PATIENTS WITH MUCOPOLYSACCHARIDOSES
Main Article Content
Abstract
Objective: To describe oral characteristics in patients with MPS through clinical examination combined with imaging.
Subjects and Methods: Thirty-two patients with confirmed MPS diagnosis at the Department of Metabolism – Genetics, National Children’s Hospital, were clinically examined, with history of oral hygiene and dental care recorded, and underwent Conebeam CT or panoramic radiographs.
Results: Characteristics in patients with MPS types I, II, VI included: macroglossia (82.6%), facial convexity (78.2%), hypodontia (34.8%), gingival hyperplasia (34.8%), diastema (34.8%), taurodontism (85.7% – 6/7 patients with radiographs), impacted teeth and dental follicular cysts (57.1% – 4/7 patients), and hyperplastic dental follicles (100% – 7/7 patients). In patients with MPS type IVA, features included: facial convexity (33.3%), microdontia (66.7%), crossbite (44.4%), amelogenesis imperfecta (33.3%), and taurodontism (25% – 1/4 patients with radiographs).
Conclusion: MPS is a rare group of disorders but presents with distinctive oral phenotypes that require early prevention, attention, and care to avoid severe dental complications later. Further studies and supportive diagnostic, monitoring, and treatment tools are needed to effectively manage oral diseases and complications in this patient population.
Keywords
Mucopolysaccharidosis, Oral manifestations, Lysosomal storage disorders, Taurodontism, Hyperplastic dental follicles, Pediatric dentistry
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References
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