• Renata Quirino de Almeida-Barros, de MedeirosPaula Frassinetti VasconcelosPFVSchool of Medicine, Universidade Federal de Campina Grande (UFCG), Campina Grande, Brazil., Marcella Quirino de Almeida Azevedo, Adriana de Oliveira Lira Ortega, Angela Toshie Araki Yamamoto, Sheyla Kátia Lúcio Dornelas, and Patrícia Meira Bento.
• Universidade Cruzeiro do sul (Unicsul), São Paulo, SP, Brazil. renata.qa@gmail.com.
• Clin Oral Investig. 2018 Jan 1; 22 (1): 201-208.

ObjectiveThe aim of this study is to assess oral manifestations in patients with mucopolysaccharidosis IV (MPS IVA) and mucopolysaccharidosis VI (MPS VI).Materials And MethodsSeventeen patients were assessed, nine with MPS IVA and eight with MPS VI, treated at the Medical Genetics Outpatient Clinic of Hospital Universitário Alcides Carneiro (HUAC) in Campina Grande, Paraíba State, Brazil. Assessments included clinical and intraoral examinations, analysis of occlusal function, and panoramic X-rays.ResultsAmong all, 64.7% of the patients had unerupted teeth and 52.9% of them had enamel hypoplasia. Regarding the temporomandibular joint, 88.2% of the patients showed flattening of the mandibular condyle, 52.9% showed condylar hypoplasia, and all of them showed decreased mobility. Enamel hypoplasia was observed only in patients with MPS IVA (p < 0.05). On the other hand, only MPS VI patients presented with anterior open bite, restricted mouth opening (p < 0.05), and a higher rate of unerupted teeth, hyperplastic tooth follicle, and condylar defects (p < 0.05).ConclusionsEnamel hypoplasia was observed only in patients with MPS IVA, whereas anterior open bite was observed only in patients with MPS VI. Abnormal findings in the maxillomandibular complex were more frequent in patients with MPS VI.Clinical RelevanceThe relevant frequency of MPS VI and IVA in the sample allows us to compare the changes occurring in both groups of patients, therefore enabling us to further comprehend the oral manifestations in specific types of MPS.

19 keywords...

hide…