Evaluation of oral manifestations of patients with mucopolysaccharidosis IV and VI: clinical and imaging study.
OBJECTIVE
The aim of this study is to assess oral manifestations in patients with mucopolysaccharidosis IV (MPS IVA) and mucopolysaccharidosis VI (MPS VI).


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.


CONCLUSIONS
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.
Universidade Cruzeiro do sul (Unicsul), São Paulo, SP, Brazil. renata.qa@gmail.com.|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.
Renata Quirino
RQ
de Almeida-Barros
School of Medicine, Universidade Federal de Campina Grande (UFCG), Campina Grande, Brazil.
Paula Frassinetti Vasconcelos
PFV
de Medeiros
São Leopoldo Mandic, Campinas, SP, Brazil.
Marcella Quirino
MQ
de Almeida Azevedo
Universidade Cruzeiro do sul (Unicsul), São Paulo, SP, Brazil.
Adriana
A
de Oliveira Lira Ortega
Universidade Cruzeiro do sul (Unicsul), São Paulo, SP, Brazil.
Angela Toshie Araki
ATA
Yamamoto
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.
Sheyla Kátia Lúcio
SKL
Dornelas
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.
Patrícia Meira
PM
Bento
D000293:Adolescent; D000328:Adult; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D009059:Mouth Diseases / Q000175:diagnosis / Q000209:etiology*; D009085:Mucopolysaccharidosis IV / Q000150:complications*; D009087:Mucopolysaccharidosis VI / Q000150:complications*; D011862:Radiography, Panoramic
Lysosomal; Maroteaux–Lamy syndrome; Morquio syndrome; Mucopolysaccharidosis; Storage diseases; Temporomandibular joint