Cytocompatibility of filling pastes by primary teeth root simulating model.

Evaluate the cytocompatibility of Calen®/ZO, Calcicur®, Vitapex®, Endoflas®, and zinc oxide/eugenol-based (ZOE) root canal pastes (RCP) to human primary osteoblasts (HPO) through a simplified model for primary teeth. The model employed pipette tips filled with 0.037 g of paste, exposed to 185 µL of culture medium for 24 h (n = 6). Release of components was analysed by Proton Nuclear Magnetic Resonance Spectroscopy (1H-NMR). HPO were exposed to conditioned media for 24 h. Cell viability was assessed by cell density and metabolic activity, and release of interleukin 6 (IL-6), vascular endothelial growth factor (VEGF) and fibroblast growth factor (bFGF) by immunological assay. Physicochemical properties and antimicrobial efficacy were also evaluated. 1H-NMR spectra analysis showed similarity between ZOE, Endoflas®, Calcicur®, and Vitapex® compared to Calen®/ZO and positive control, which showed distinct released components. Calen®/ZO and Calcicur® exhibited high alkaline pH in all periods and showed similar solubility. Calen®/ZO, ZOE, and Vitapex® showed similar flow rate. Calen®/ZO, Calcicur®, and Vitapex® did not exhibit antimicrobial efficacy. Calen®/ZO presented cytotoxicity (p < 0.05). Pastes did not increase IL-6 release compared to control. Apart from Vitapex®, all pastes significantly induced VEGF/bFGF release. Interactive effects among released products may affect biological response to filling pastes. Calcicur®, ZOE, Endoflas® and Calen®/ZO presented good to moderate cytocompatibility, with low impact on pro-inflammatory cytokine release and induction of growth factors of interest to tissue repair. This simplified model, specific for the evaluation of the cytocompatibility of RCPs on primary teeth, suggests how these pastes might contribute to bone repair in clinical situations of apical periodontitis in children.

Prosthetic rehabilitation of a child with X-linked hypohidrotic ectodermal dysplasia: a case report and 12-month follow-up.

The hereditary condition known as ectodermal dysplasia (ED) is characterized by the absence of or a defect in 2 or more ectodermally derived structures such as skin, nails, hair, sweat glands, or teeth. Patients with this disorder usually present with reduced salivary gland function and absence of some or all teeth, which compromises orofacial function and development. In addition, children with ED usually experience difficulty in social interactions because of their appearance. This article reviews previously published case reports pertaining to ED and describes oral rehabilitation with removable partial dentures (RPDs) in a 5-year-old girl diagnosed with X-linked hypohidrotic ED, which presented as hypodontia. An orthodontic expander screw was inserted in the maxillary prosthesis to correct the patient's crossbite, and periodic recall examinations were scheduled to monitor the effects of the patient's growth on occlusion and fit of the prosthesis. The child was monitored for 12 months, during which she exhibited significant improvement in physiologic function, appearance, and social behavior. Because negative esthetic, functional, and psychological consequences are associated with this condition, dentists must be knowledgeable about its common oral manifestations.

Longitudinal Evaluation of Salivary Iga-S in Children with Early Childhood Caries Before and After Restorative Treatment.

Background: Our aim was to compare salivary levels of secretory immunoglobulin A (s-IgA) in children with early childhood caries (ECCG) and those who are caries-free (CFG) and verify these levels in a follow-up period after restorative treatment. Materials and methods: We selected 46 systemically healthy children in the complete primary dentition period, who were allocated into two groups: CFG (n = 23) and ECCG (dmf-s > 0; n = 23). Unstimulated whole saliva was obtained at baseline from both groups and during the follow-up period (7 days, 1, 2 and 3 months) in the ECCG group. The s-IgA was measured using an ELISA assay, and total protein was assessed using the Bradford method. We also evaluated the flow rate (mL/min), Streptococcus mutans and Lactobacillus spp. counting using selective media plaques. The data were submitted to statistical analysis using the software SPSS 20.0 (SPSS Inc, IL, USA) with a confidence interval set at 95%. Results: Salivary s-IgA levels were higher in baseline of ECCG than in CFG (p<0.05). No statistically significant differences were observed between s-IgA salivary levels at baseline and the evaluations after dental treatment in ECCG (p>0.05). However, we observed two different changes in s-IgA levels among participants: one group presented s-IgA reduction, and the other group demonstrated its maintenance. It was shown that patients from the ECCG group who presented a reduction in s-IgA levels during follow-up also showed a decrease in Streptococcus mutans and Lactobacillus spp. count (p<0.05), in contrast to patients who did not present this reduction. The flow rate and total protein were similar between groups (p>0.05). Conclusions: The present data support the idea that children with early childhood caries present higher levels of s-IgA in saliva than caries-free children. The restorative dental treatment does not have a significant influence on salivary levels of this immunoglobulin during the follow-up period.

Salivary Metabolite Fingerprint of Type 1 Diabetes in Young Children.

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.

Is dental caries experience increased in HIV-infected children and adolescents? A meta-analysis.

OBJECTIVE: To undertake a systematic review to assess if HIV-infected children and adolescents have an increased dental caries experience. METHODS: A search of MEDLINE, BIREME, EMBASE, GOOGLE SCHOLAR, SIGLE (Grey Literature) and reference lists of included studies was carried out. To be eligible the studies had to present HIV-infected and non-infected children/adolescents between 0-18 years old. To assess the methodological quality, the studies were categorized in scores from 'A' to 'C'. To perform a meta-analysis a random effect model was used with 95% confidence intervals and two distinct sub-group analyses were carried out in terms of caries progression: data for cavitated and non-cavitated lesions (sub-group 1) and data only for cavitated lesions (sub-group 2). RESULTS: Five studies fulfilled the selection criteria. Four studies (two ranked A and two B in the quality assessment) revealed higher caries scores in primary teeth in the HIV-infected patients with mean dmft/dmfs scores of 3.8-4.1/7.8-11.0 compared to the control group 1.5-2.4/3.4-5.1. No differences in caries index were found for permanent dentition. The meta-analysis excluded caries data of permanent teeth and showed a significant association between caries experience in primary dentition and HIV infection considering cavitated and non-cavitated lesions (OR = 2.33, 95% CI = 1.48-3.68) or only cavitated lesions (OR = 2.98, 95% CI = 1.59-5.59). CONCLUSION: Evidence exists that suggests HIV-infected children/adolescents have an increased caries experience in primary dentition.

Dental findings and oral health status in patients with mucopolysaccharidosis: a case series.

OBJECTIVE: To present a mucopolysaccharidosis (MPS) case series evaluating oral manifestations (clinical and radiographic), oral health status and discussing its implications. PATIENTS AND METHODS: All patients with MPS attending the Genetics clinic/Brazil were evaluated by means of anamnesis, clinical and radiographic examinations. RESULTS: The final sample consisted of 12 subjects (nine males and three females), with ages ranging from 3-31 years old. Concerning oral health, it was observed high levels of caries and periodontal problems. About oral manifestations, this study clinically observed more cases of delayed tooth eruption, thickness of alveolar process and thick lips. Radiographically, it was observed alterations on condyle, mandibular ramus and joint fossa. CONCLUSION: The dental changes in MPS population are high and consequently it is important to know them for differential diagnoses, early treatment intervention, prevention and education of both patients and parents/caregivers about oral health.

The oral health of HIV-infected Brazilian children.

BACKGROUND: The number of HIV-infected people has increased almost continuously. Paediatric dentists should be concerned about the oral findings in HIV-infected children and their aetiologic factors, to promote adequate treatment. AIM: To present the oral health aspects of Brazilian HIV-infected children and to verify the aetiological factors. DESIGN: A cross-sectional study was conducted with HIV-infected children. During the medical appointments, children were submitted to visual-tactile exams of oral soft tissues and teeth. All parents answered questions in a structured interview. Data were analysed using the SPSS, release 10.0 (Chicago, IL, USA). RESULTS: Of the 57 children examined, 39 (69.6%) presented one or more oral soft tissue manifestations. More than a half suffered from gingivitis and only 12.5% had no visible dental biofilm. A high prevalence of dental surfaces with active carious lesions was observed; mean DMFS-m and dmfs-m scores were 5.41 ± 5.61 and 16.77 ± 19.52, respectively. Caries activity and gingivitis were correlated with the presence of mature dental biofilm. CONCLUSIONS: Prevalence of soft tissue lesions, dental caries and gingivitis in HIV-infected children was high and correlated to lack of satisfactory oral hygiene habits, suggesting the need of therapeutic programmes that allow these children to recover their oral health.

NMR-Based Metabolomics Demonstrates a Metabolic Change during Early Developmental Stages from Healthy Infants to Young Children.

The present study aims to identify the salivary metabolic profile of healthy infants and young children, and to correlate this with age, salivary gland maturation, and dentition. Forty-eight children were selected after clinical evaluation in which all intraoral structures were examined. Total unstimulated saliva was collected, and salivary metabolites were analyzed by 1H Nuclear Magnetic Resonance (NMR) at 25 °C. Partial least squares discriminant analysis (PLS-DA), orthogonal PLS-DA (O-PLS-DA), and univariate analysis were used, adopting a 95% confidence interval. The study showed a distinct salivary metabolomic profile related to age and developmental phase. The saliva of children in the pre-eruption teeth period showed a different metabolite profile than that of children after the eruption. However, more evident changes were observed in the saliva profile of children older than 30 months. Alanine, choline, ethanol, lactate, and sugar region were found in higher levels in the saliva of patients before 30 months old. Acetate, N-acetyl sugar, butyrate, caproate, creatinine, leucine, phenylalanine, propionate, valine, succinate, and valerate were found to be more abundant in the saliva of children after 30 months old. The saliva profile is a result of changes in age and dental eruption, and these findings can be useful for monitoring the physiological changes that occur in infancy.

Use of chlorhexidine gel (0.2%) to control gingivitis and candida species colonization in human immunodeficiency virus-infected children: a pilot study.

PURPOSE: The purpose of this study was to evaluate chlorhexidine to control gingivitis and Candida species (spp.) in children infected with the human immunodeficiency virus (HIV) and their acceptance of the therapy. METHODS: Twenty-six HIV+ children were selected, and oral exam-established biofilm, gingival indexes, and stimulated saliva were collected for Candida ssp. identification. The children brushed their teeth for 21 days with chlorhexidine gel (0.2%). Salivary samples, biofilm, and gingival indexes were collected after 21-days and again 35 days after ceasing gel use. The children answered a questionnaire about the therapy. RESULTS: All children tested positive for Candida and gingivitis. After 21 days, Candida counts and gingivitis decreased in 25 and 26 children, respectively. Mean reduction was approximately 68% for Candida spp. and 74% for gingivitis. Thirty-five days after discontinuing gel use, gingivitis and Candida spp. increased in 13 and 16 patients, respectively. Considering the Candida spp., the heavy growth was lower in the first re-evaluation. Candida albicans was the most frequent species. Approximately 85% did not experience inconvenience with the gel, and approximately 48% thought it was good for tooth-brushing. CONCLUSION: Chlorhexidine therapy may be an option to treat and pre- vent gingivitis and reduce yeast counts in children infected with HIV.

Effect of antihistamine-containing syrup on salivary metabolites: an in vitro and in vivo study.

This study tested the null hypothesis that antihistamine-containing syrup does not change salivary metabolites in vitro and in vivo. For the in vitro experiments, saliva from 10 volunteers was mixed with a syrup or pill suspension of loratadine (1 mg/ml Claritin®, Schering-Plough, Rio de Janeiro, Brazil). For the in vivo experiment, 10 volunteers performed a mouth rinse with 10 mL of antihistamine syrup (Claritin®; Schering-Plough, Rio de Janeiro, Brazil) for 20 seconds and then discarded the rinse water. After 20 seconds, 5 mL of unstimulated whole saliva was spit into a plastic tube kept on ice. The protein profile of in vitro and in vivo experiments was analyzed using 12% sodium dodecyl sulfate-polyacrylamide gel electrophoresis. The samples were also analyzed by nuclear magnetic resonance (NMR) spectroscopy, followed by Principal Component Analysis and Wilcoxon test (p < 0.05). There were differences in salivary metabolites after syrup interaction. The salivary concentrations of acetate, n-caproate, arginine, glutamate, and lysine among other metabolites were reduced with the syrup in both in vivo and in vitro experiments (p < 0.05), but no differences were observed when the pill suspension was used (p > 0.05). Similar changes in metabolite profiles were observed in both in vitro and in vivo experiments. Electrophoresis revealed no difference in the salivary protein pattern. The null hypothesis was rejected because the intake of syrup medicine changes the salivary composition and influences oral homeostasis and susceptibility to oral diseases.

Oral Candida colonization and its relation with predisposing factors in HIV-infected children and their uninfected siblings in Brazil: the era of highly active antiretroviral therapy.

OBJECTIVES: To evaluate predisposing factors such as orofacial manifestations, immunosuppression status and antiretroviral therapy in relation to oral colonization by Candida spp. in Brazilian HIV-infected children and their uninfected siblings in the era of highly active antiretroviral therapy (HAART). METHODS: Whole stimulated saliva was collected from 65 HIV-infected children (HIV+) and 40 uninfected siblings (HIV-), followed by assessment of orofacial manifestation, caries indexes and the number of cavitated dentinal carious teeth (CDT). The salivary samples were cultured and the colonies were counted. After which they were identified by sugar assimilation and fermentation (API 20C). Data was analyzed using chi-square, Mann-Whitney, Spearman tests and logistic regression. RESULTS: Regarding positive growth, HIV+ presented 80% (52/65) and HIV- 57.5% (23/40) (P = 0.013). Absence of antiretroviral therapy and HAART increased the probability of Candida isolation (P < 0.05). Mean CD4%, immune-status and history of recurrent oral candidiasis (OC) had no influence on Candida isolation. Mixed Candida spp. cultures were observed in HIV+ (40%) and HIV- (52%): C. albicans was more frequently found in both groups, with a higher prevalence in HIV+ (P = 0.05); other non-albicans species were isolated in HIV+ and HIV-. Low prevalence of orofacial manifestations was observed in HIV+ (10.7% of OC). There was an association between means of CDT and Candida growth (P < 0.05) and a positive correlation between number of CDT and Candida cfu-counts in HIV+ and HIV-. Mean CD4% and immune-status had no influence on Candida isolation. Absence of antiretroviral therapy and HAART increased the probability of Candida isolation (P < 0.05). CONCLUSIONS: The HIV infected children had a significantly higher prevalence of oral Candida spp. compared to their uninfected siblings. Absence of HAART and presence of dentinal carious teeth increased significantly Candida spp. colonization in these children.

Is there a relationship between malocclusion and bullying? A systematic review.

BACKGROUND: Malocclusion is a highly prevalent public health problem, and several studies have shown its negative correlation with quality of life, self-esteem, and social perceptions. However, its association with bullying is still controversial. OBJECTIVES: To evaluate the relationship between malocclusion and bullying in children and adolescents. SEARCH METHODS: The databases used for the electronic researches were PubMed, Scopus, Lilacs/BBO, Web of Science, and Cochrane Library. Grey literature was reviewed through Open Grey literature with no language or date restrictions. Selection criteria, based on the PECO strategy, were considered eligible observational studies that included schoolchildren or adolescents (P) with malocclusion (E), compared to those with normal occlusion (C), in which the relationship between malocclusion and bullying was determined (O). DATA COLLECTION AND ANALYSIS: Risk of bias evaluation was made for the qualitative synthesis by the Fowkes and Fulton criteria. Data regarding the age of participants and types of malocclusion and of bullying were extracted among other reported data. The quality of the evidence analyzed was evaluated through the GRADE approach. RESULTS: From 2744 articles identified in databases, nine met the eligibility criteria and were included in present systematic review, of which two studies were judged with methodological soundness. The quality of the evidence was classified as very low due to very serious problems for "risk of bias" and "other considerations" and serious problems of "indirectness". The age of participants ranged from 9 to 34 years considering a cohort study, with a bullying recalling perspective. Malocclusion was both evaluated by researchers and self-reported by participants addressing dentofacial characteristics mostly related to the incisors relationship. All studies evaluated the verbal type of bullying, while 3 also considered physical type. Both types were reported as related to malocclusion, although the results showed that extreme maxillary overjet (> 4 mm, > 6 mm, > 9 mm), extreme deep overbite, and having space between anterior teeth or missing teeth were the types of malocclusion with the strongest relations to bullying. CONCLUSIONS: With very low certainty of evidence, the results of this systematic review suggest that conspicuous extreme malocclusion may be related to the occurrence of bullying among children and adolescents.

Oral manifestations in human immunodeficiency virus infected children in highly active antiretroviral therapy era.

OBJECTIVE: Conducted a literature review to identify studies that reported on the oral manifestations in human immunodeficiency virus (HIV) infected children in highly active antiretrovial therapy (HAART) era. METHODS: A search electronic data base were used and the terms used were 'oral lesions' and 'oral manifestations'. The studies of prevalence of oral manifestation in children with HIV worldwide, descriptive studies, case reports, studies on the association of oral lesions and levels of immune suppression, use of HAART and transmission of HIV were included. RESULTS: There have been substantial changes in the management of HIV disease, especially in the past decade because of the use of HAART. However, children are still being infected and present some peculiarities when compared with adults. Molecular epidemiology, transmission and therapy of the common opportunistic oral infections of HIV disease need to be better understood as a consequence of improved anti-HIV strategies. Treatment with HAART improves the immune function and decreases mortality, morbidity, and opportunistic infections in HIV-infected persons. CONCLUSION: The frequency and severity of oral disease associated with HIV infection have reduced considerably, although the use of HAART may be associated with an increased appearance of oral lesions associated with human papillomavirus and potentially increase the risk of later oral squamous cell carcinoma.

Correlation between mothers' perceptions/expectations and the behavior of children with HIV during dental treatment.

The aim of this study was to evaluate the mothers' perceptions and expectations of the behavior of their children who were HIV positive, during dental treatment. The sample was composed of 40 mother and child pairs. The mothers answered seven questions during two different appointments (M1, the child's initial appointment, and M2, the child's treatment appointment) in order to identify their perceptions and expectations about their children's behavior during dental treatment. In addition, child's behavior during the appointments was evaluated. The results of the mothers' interviews were then correlated with their children's behavior using Spearman's correlation test (p<0.05). There was no significant correlation between the mothers' perceptions and expectations and the behavior displayed by their children during dental treatment (p>0.05).

Comparison of techniques to evaluate the quantification of Candida spp. in HIV-infected children.

This study sought to compare techniques used to make a quantified evaluation of Candida spp. in children infected with HIV. Twenty-four HIV-infected children (age 3 to 13) were selected. Three sterilized swabs were used for each child: one for the dorsum of the tongue, one for the hard palate mucosa, and one for the right jugal mucosa; each swab was rubbed for 10 seconds and transferred to sterilized test tubes containing 1 mL of 0.9% saline solution. Candida spp. growth was observed in 95.8% of all samples, including 95.7% of tongue samples (Group T), 87.0% of saliva samples, 56.6% of hard palate mucosa samples (Group P), and 47.8% of right jugal mucosa samples (Group J). There was no statistical difference in Candida spp. growth between saliva samples and Group T samples, although both had higher growth compared to Groups P and J (p < 0.05; chi(2)). Regarding the sensitivity of each site for positive Candida spp. growth, Group T samples showed 69.5%, while saliva samples showed 52.2%, Group P samples showed 21.7%, and Group J samples showed 13.04%, with no significant statistical difference between Group T and saliva; however, both were more sensitive than Groups J and P (p < 0.05, chi(2)). It was concluded that whole stimulated saliva and swabbing the tongue were considered satisfactory for measuring Candida spp. in HIV-infected children.

Influence of local factors on cementoenamel junction-alveolar bone crest distance in primary dentition.

OBJECTIVE: This study illustrated the influence of local factors (dental biofilm, gingival bleeding, probing depth, proximal contact and proximal caries/inadequate restorations) on the cementoenamel junction (CEJ)-alveolar bone crest (ABC) distance in primary canines and molars of healthy children with complete primary dentition. STUDY DESIGN: Two hundred and four patients have been examined clinically with regard to biofilm's presence, gingival status, probing depth, proximal contact and dental condition (carious lesions/restorations). Two bitewings of each patient were also taken. Eight dental surfaces (second molars mesial, first molars mesial and distal, and canines distal) could be analyzed per bitewing and the CEJ-ABC distance was measured using a digimatic caliper. Statistical analyses were controlled by dental arch, tooth type and surface. Mann-Whitney test was used to verify the association between CEJ-ABC distance and local factors. RESULTS: After stratifying the data according to those influencing factors, the only local factor associated with increased CEJ-ABC distances in most of the different analyzed dental surfaces was the increased probing depth (> 2mm), although only two dental surfaces showed statistical significance (p < 0.01). CONCLUSION: Among the analyzed local factors, increased probing depth was the only factor that proved to be relevant in children's alveolar bone loss screening.

Salivary Metabolites in Patients with Mucopolysaccharidosis

ABSTRACT Objective: To identify the salivary metabolites profile of Mucopolysaccharidosis (MPS) types I, II, IV, and VI patients. Material and Methods: The participants were asked to refrain from eating and drinking for one hour before sampling, performed between 7:30 and 9:00 a.m. Samples were centrifuged at 10.000 × g for 60 min at 4°C, and the supernatants (500µl) were stored at −80°C until NMR analysis. The salivary proton nuclear magnetic resonance (1H-NMR) spectra were acquired in a 500 MHz spectrometer, and TOCSY experiments were used to confirm and assign metabolites. Data were analyzed descriptively. Results: Differences in salivary metabolites were found among MPS types and the control, such as lactate, propionate, alanine, and N-acetyl sugar. Understanding these metabolite changes may contribute to precision medicine and early detection of mucopolysaccharidosis and its monitoring. Conclusion: The composition of low molecular weight salivary metabolites of mucopolysaccharidosis subjects may present specific features compared to healthy controls.

Pediatric bitewing film holder: preschoolers' acceptance and radiographs' diagnostic quality.

PURPOSE: The purpose of this study was to evaluate a modified bitewing film holder based on preschoolers' acceptance and the diagnostic quality of radiographic images. METHODS: Sixty-six 3- to 5-year-old children were radiographed with the modified device. Acceptance was assessed according to their behavior during the radiographic examination and to their perception of the procedure. In only the 4- and 5-year-olds, the children's perception was assessed using a faces scale. Evaluation of the images' diagnostic quality for each quadrant (considering pairs of radiographs) was based on anatomic structures usually present on bitewing radiographs. RESULTS: During radiographic examination, 73% of the children were cooperative. As to the patients' perceptions (N = 43), 74% showed satisfaction with the radiographic examination. The diagnostic quality of radiographic images (N = 66) was adequate in more than 90% of the cases for all evaluated parameters, except for the visualization of alveolar bone crests (54%) and the absence of proximal overlapping (86%). CONCLUSIONS: The modified bitewing film holder produced adequate radiographs for almost all parameters and was well accepted by preschoolers.

Three-dimensional dental and craniofacial manifestations in patients with late diagnosis of mucopolysaccharidosis type II: report of 2 cases.

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.

Oral and craniofacial manifestations in a Hunter syndrome patient with hematopoietic stem cell transplantation: A case report.

We described herein the oral and craniofacial features of a 7-year-old boy, diagnosed in utero with mucopolysaccharidosis II (MPS II), who was treated with hematopoietic stem cell transplantation (HSCT) at 70 days of age. The main oral clinical findings were the following: macroglossia, posterior cross-bite, crowding, pointed cuspid teeth, delayed tooth eruption, retained primary teeth, and enamel hypoplasia. The image examination showed: retention eruption, posterior primary teeth with short roots, absence of some permanent teeth, and stretching of the stylohyoid processes bilaterally. This patient showed the importance of early diagnosis and HSCT therapy in attenuating the clinical and radiographic oral and craniofacial manifestations of the MPS II patient.