Relationship Between Occlusal Features and Enzyme Replacement Therapy in Patients With Mucopolysaccharidoses.

PURPOSE: The aim of this study was to describe the relation between occlusal features and enzyme replacement therapy in patients with mucopolysaccharidoses. MATERIALS AND METHODS: A cross-sectional study was conducted. The sample consisted of 20 patients with mucopolysaccharidoses, 10 of whom were undergoing treatment at a hospital in northeast Brazil. Occlusal features were evaluated by clinical examination and panoramic radiography. A structured questionnaire was administered to evaluate the dental care of each patient. Pearson χ2, Fisher exact, and Mann-Whitney tests were used for data analysis, with a level of significance of 5%. RESULTS: Marked overjet (75%) and anterior open bite (70%) were the most frequent occlusal alterations, and 15% had Class III disorders. Radiography visualized the presence of impacted teeth (75%) and prolonged retention of deciduous teeth (65%). Patients with enzyme replacement therapy had a lower average maximum protrusion (P = .033). A total of 75% of mothers said they had not been advised to take their children to the dentist and 10% of children had never been to the dentist. CONCLUSION: Patients with mucopolysaccharidoses exhibited notable occlusal alterations, especially marked overjet and anterior open bite. Enzyme replacement therapy seems to influence the maximum protrusion of patients.

Mucopolysaccharidosis: radiographic findings in a series of 16 cases.

OBJECTIVES: To investigate the radiographic findings of mucopolysaccharidoses (MPS) on panoramic radiographs in a series of 16 patients. STUDY DESIGN: This retrospective cross-sectional study included panoramic radiographs of 16 patients with cytogenetically diagnosed MPS. Two blinded, previously calibrated observers evaluate the presence or absence of the following features: type of dentition; stage of dental age in comparison with chronologic age; delayed exfoliation of deciduous teeth; supernumerary teeth; teeth impaction; enlarged, cyst-like dental crypt; hypercementosis; taurodontism; generalized enamel hypoplasia; enlargement of the bone marrow spaces; thinning of cortical bone; unusual morphology of condyles; and flattening of the condylar head. RESULTS: The final sample was composed of 8 MPS IV, 7 MPS VI, and 1 MPS I cases. All patients presented unusual morphology of condyles. Cyst-like dental crypt (75%), taurodontism, and teeth impaction (68.75% each) were also overall common findings. Generalized enamel hypoplasia was exclusively identified in patients with MPS IV (75% of those patients). CONCLUSIONS: Radiographic features of oral and maxillofacial manifestations in patients with MPS frequently encountered by dentists may help recognize the disorder. However, because of sample size limitations, it was not possible to infer any statistical relationship between the radiographic features and the types of MPS.