ABSTRACT
The objective of this study was to report the clinical evaluation results and 3-dimensional (3-D) dental and craniofacial characteristics observed in 2 male patients with mucopolysaccharidosis type II. The patients were evaluated clinically (soft tissue evaluation, evaluation of occlusion, periodontal and dental examinations) and by using craniofacial computed tomography, with evaluation of 3-D images in ITK-Snap v. 2.2 (Penn Image Computing and Science Laboratory, Philadelphia, PA; http://www.itksnap.org/) and 3-D Slicer (http://www.slicer.org/) software. Mandibular 3-D volumetric label maps were built from computed tomography scans of both patients and compared through superimposition on a healthy patient's mandibular images. Clinically, the patients presented the following oral manifestations: macroglossia, total open bite and generalized diastemas, and absence of caries. Patient 1 showed dental calculus and bleeding at the gingival margin. Patient 2 showed bleeding at the gingival margin, a permanent maxillary left central incisor missing as a result of trauma, and impacted permanent mandibular left and right second molars. 3-D images showed wide arches, prominent antegonial notches, a narrow mandibular body in the region of the antegonial notches, bilateral severe condylar hypoplasia, and enlarged coronoid processes. 3-D imaging and superimpositions revealed oral and skeletal displacements, contributing to the identification of changes in the course of mucopolysaccharidosis type II in patients with a late diagnosis.
ABSTRACT
Metabolomics is an important tool for the evaluation of the human condition, in both health or disease. This study analyzed the salivary components of type I diabetic children (DM1) under six years of age, to assess oral health related to diabetes control, as well as metabolite profiling using NMR. Partial least squared discriminant analysis (PLS-DA) was used to compare healthy (HG) and uncontrolled DM1 subjects that demonstrated a separation between the groups with classificatory performance of ACC = 0.80, R(2) = 0.92, Q(2) = 0.02 and for DM1 children with glycemia >200 mg/dL of ACC = 0.74, R(2) = 0.91, Q(2) = 0.06. The metabolites that mostly contributed to the distinction between the groups in the loading factor were acetate, n-acetyl-sugar, lactate, and sugar. The univariate analysis showed a decreased salivary concentration of succinic acid and increased levels of lactate, acetate, and sucrose in uncontrolled and DM1 children with glycemia >200 mg/dL. The present study demonstrates that the salivary profile of DM1 differs from that of HG children. It appears that diabetes status control has an important effect on the salivary composition.