Renata Quirino de Almeida-Barros1,2, Paula Frassinetti Vasconcelos de Medeiros3, Marcella Quirino de Almeida Azevedo4, Adriana de Oliveira Lira Ortega5, Angela Toshie Araki Yamamoto5, Sheyla Kátia Lúcio Dornelas6, Patrícia Meira Bento6. 1. Universidade Cruzeiro do sul (Unicsul), São Paulo, SP, Brazil. renata.qa@gmail.com. 2. Department of Dentistry, Universidade Estadual da Paraíba (UEPB), 599 Str. Manuel Elias de Araújo, Apt 1302, Jardim Tavares, Campina Grande, PB, 58.402-022, Brazil. renata.qa@gmail.com. 3. School of Medicine, Universidade Federal de Campina Grande (UFCG), Campina Grande, Brazil. 4. São Leopoldo Mandic, Campinas, SP, Brazil. 5. Universidade Cruzeiro do sul (Unicsul), São Paulo, SP, Brazil. 6. Department of Dentistry, Universidade Estadual da Paraíba (UEPB), 599 Str. Manuel Elias de Araújo, Apt 1302, Jardim Tavares, Campina Grande, PB, 58.402-022, Brazil.
Abstract
OBJECTIVE: The aim of this study is to assess oral manifestations in patients with mucopolysaccharidosis IV (MPS IVA) and mucopolysaccharidosis VI (MPS VI). MATERIALS AND METHODS: Seventeen 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. RESULTS: Among 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). CONCLUSIONS: Enamel 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 RELEVANCE: The 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.
OBJECTIVE: The aim of this study is to assess oral manifestations in patients with mucopolysaccharidosis IV (MPS IVA) and mucopolysaccharidosis VI (MPS VI). MATERIALS AND METHODS: Seventeen 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. RESULTS: Among 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 VIpatients 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). CONCLUSIONS:Enamel 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 RELEVANCE: The 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.
Authors: Yasuo Yamakoshi; Jan C C Hu; Shengxi Liu; Xiaoling Sun; Chuhua Zhang; Shinichiro Oida; Makato Fukae; James P Simmer Journal: Connect Tissue Res Date: 2002 Impact factor: 3.417
Authors: Klane K White; Andrea Jester; C Edward Bache; Paul R Harmatz; Renée Shediac; Mihir M Thacker; William G Mackenzie Journal: J Child Orthop Date: 2014-07-08 Impact factor: 1.548
Authors: Andrea Gómez-González; Miguel Ángel Rosales-Berber; Paola De Ávila-Rojas; Amaury Pozos-Guillén; Arturo Garrocho-Rangel Journal: Case Rep Dent Date: 2020-01-22