Facial and Cephalometric Features of Individuals With Mucopolysaccharidosis: A Cross-Sectional Study.

OBJECTIVE: The aim was to assess craniofacial features through facial anthropometric and lateral cephalometry measurements of individuals with mucopolysaccharidosis (MPS) and compare them with individuals without MPS. DESIGN: Cross-sectional study. PATIENTS: A total of 14 individuals with MPS and 28 non-MPS age- and sex-matched were enrolled in this study. METHODS: A clinical facial analysis to evaluate the soft tissues and cephalometric analysis that comprised linear and angular measurements were performed. The calculation of the method error suggested no systematic errors (p > .05). Random errors for linear and angular measurements were low (less than 0.5° and 1.6 mm). Chi-square test and independent t-test were performed. RESULTS: Most individuals with MPS were dolichofacial, presented altered facial proportions with an increased anterior lower facial height (ALFH) and lip incompetence (all p < .05), when compared with non-MPS individuals. Six angular measurements (1s.Na, 1s.NB, FMA, IMPA, AFI, and Po.Or_Go.Me; all p < .05) were significantly increased among individuals with MPS, and two (1s.1i and Ba.N-Ptm.Gn, all p < .05) were significantly decreased among them. Four linear measurements were significantly increased among individuals with MPS (1s-NA, 1i-NB, S-UL, and S-LL; all p < .05) and five (PogN-Perp, Co-A, Co-Gn, Nfa-Nfp, and overbite; all p < .05) were significantly decreased among them. CONCLUSION: In summary, most individuals with MPS were dolichofacial with increased ALFH. Proclined upper and lower incisors, reduced nasopharyngeal space, and reduced overbite was also noted.

Malocclusion in individuals with osteogenesis imperfecta: A systematic review and meta-analysis.

OBJECTIVE: To evaluate whether individuals with osteogenesis imperfecta (OI) are more affected by malocclusion than individuals without OI. MATERIALS AND METHODS: Searches in PubMed, Ovid, Web of Science, Scopus, Lilacs and gray literature were performed. Data extraction was conducted by two researchers. Risk of bias assessment employing the Newcastle-Ottawa Scale and meta-analysis were conducted. Results were provided with mean difference (MD), odds ratio (OR) and 95% confidence interval (CI). Strength of evidence was determined. RESULTS: Six cross-sectional studies were included. In comparison with individuals without OI, the group with OI had 19.69-fold greater chance of exhibiting Angle Class III malocclusion (OR = 19.69, CI: 9.00-43.09) and presenting anterior crossbite greater (MD = 6.08, CI: 2.40-9.77). Individuals without OI had a significantly greater ANB angle (MD = 3.88, CI: 1.15-6.61) and SNA angle (MD = 2.11, CI: 0.24-3.98) in comparison with those with OI. No difference between groups was found for SNB (MD = -0.50, CI: -2.21 to 1.21) and open bite (MD = 0.98, CI: -0.29 to 2.25). Most studies included had moderate methodological quality. Strength of evidence was low or very low. CONCLUSIONS: The occurrence of Angle Class III malocclusion and anterior crossbite was greater among individuals with OI compared to those without OI. These findings can assist stakeholders about the occlusal abnormalities affecting OI individuals.

Health promotion in Brazil: qualitative survey with primary school teachers.

School health promotion programs can reduce common health problems like obesity, diabetes and respiratory complications. This qualitative study aimed to investigate the perceptions of a group of teachers regarding issues related to the promotion of health in school. The 15 teachers sampled were from public primary schools in a city in southeastern Brazil. Interviews with teachers were conducted and data was analyzed employing content. Teacher practices regarding the promotion of health appeared to be aimed at their students and their daily habits and their practices. Teachers described approaching health promotion through their lesson content and suggested that the school should seek help from other institutions in their health promotion activities like prevention activities, monitoring of improvements in student and teacher health. Teachers mentioned a strong relationship between knowledge and the pursuit of good health. The need to identify major determinants of the disease/health process was highlighted and the connection between lifestyle, socio-economic status and the disease/health process was described. Awareness of the influence concerning both the environment and the school surroundings was demonstrated. It is important that the teachers are aware of the environment that surrounds them as well as the reality of the lives of all those involved with the school.

Factors associated with halitosis reported by parents/caregivers in individuals with Down syndrome.

AIMS: Data on halitosis among individuals with Down syndrome (DS) are scarce. The aim was to evaluate factors associated with the occurrence of halitosis reported by parents/caregivers (P/Cs) in individuals with DS. METHODS AND RESULTS: A cross-sectional study was conducted in non-governmental assistance institutions in the State of Minas Gerais-Brazil. P/Cs have answered an electronic questionnaire with sociodemographic, behavioral and oral health data. Factors associated with halitosis were evaluated by multivariate logistic regression. The sample comprised 227 P/Cs (age 48.8 ± 13.2 years; 82.9% mothers) of individuals with DS (age 20.8 ± 13.5 years). The prevalence of halitosis in the total sample was 34.4% (n = 78) and its occurrence was associated: 1) in individuals with DS ≤18 years old (26.2%; n = 27)-negative perception of oral health (OR = 3.91); 2) in individuals with DS > 18 years (41.1%; n = 51)-gingival bleeding (OR = 4.53), absence of tongue brushing (OR = 4.50), negative perception of oral health (OR = 2.72). CONCLUSIONS: The occurrence of halitosis in individuals with DS reported by P/Cs was relevant and associated with dental factors, having a negative impact on the perception of oral health. Oral hygiene practices, especially tongue brushing, should be reinforced to prevent and control halitosis.

Oral conditions of children with microcephaly associated with congenital Zika syndrome: a cross-sectional study.

The aim of the present study was to compare the oral conditions of children with congenital Zika syndrome (CZS)-associated microcephaly, non-CZS-associated microcephaly, and normotypical children, as well as to characterize their sociodemographic aspects and medical history. A paired cross-sectional study was carried out on 14 children with CZS-associated microcephaly and 24 age-matched controls, in Belo Horizonte, in southeastern Brazil. Children's oral conditions were assessed: dental caries experience (dmft/DMFT indices); developmental defects of enamel (DDE) index; dental anomalies; mucosal changes; lip sealing, and malocclusion (overjet, overbite, and/or posterior crossbite alterations). The quality of oral hygiene was analyzed by the simplified oral hygiene index. The children's mothers also answered a questionnaire about sociodemographic and medical history data. The variables were analyzed descriptively. Female participants were more prevalent (60.5%), and the mean age of the participants was 4.9 years (±1.4) (range: 2-8 years) and 92.1% of their exhibited some oral condition. All participants with CZS-associated microcephaly showed absence of lip sealing and had malocclusion (100.0%). When compared to the other groups, children with CZS had a higher percentage of dental anomalies (35.7%), mucosal changes (71.4%), and unsatisfactory oral hygiene (64.3%). In a sample composed mainly of female participants aged less than 5 years, the prevalence of oral conditions and unsatisfactory oral hygiene was higher in the group with CZS-associated microcephaly, followed by the group with non-CZS-associated microcephaly. Normotypical children had the highest percentage of dental caries experience.

Dental Profile of Brazilian Patients with Rare Skeletal Genetic Disorders: Clinical Features and Associated Factors.

The aim of this study is to compare the dental profiles of Brazilian patients with rare genetic skeletal disorders and normotypical patients. A cross-sectional study was carried out with 210 individuals aged between 2 and 54 years old [105 with rare diseases (Mucopolysaccharidosis/MPS n = 27 and Osteogenesis Imperfecta/OI n = 78) and 105 without rare diseases] and their parents/caregivers. The parents/caregivers answered a questionnaire about individual aspects of their child and the dental profile was identified from questions related to dental history and the presence/absence of dental problems. The patients' oral cavity was also examined by three examiners for dental caries, malocclusion, gingivitis, and dental anomalies. The average age of individuals with a rare disease was 14.1 years (±12.2) and the median was 9.5 years. Participants who had already used the public health system (SUS) dental care services had a 2.24 times higher chance of belonging to the group with a rare disease (OR = 2.24; 95% CI: 1.07-4.89). Patients with rare diseases are 14.86 times more likely to have difficulty receiving dental treatment (OR = 14.86; 95% CI: 5.96-27.03) and 10.38 times more likely to have one or more dental problems (OR = 10.38; 95% CI: 1.95-35.17). Individuals with rare disorders have a greater history of difficulty in accessing dental treatment, using the SUS, and were diagnosed with more dental problems compared to normotypical individuals.

Association between management, human resources, and care provided to patients with special healthcare needs in dental specialty centers in Brazil: A cross-sectional study.

AIMS: This study aimed to identify the association between management and human resource factors with the performance of dental care provided to patients with special health care needs (SHCN) in secondary care in Brazil in the second cycle of the Program for Quality Improvement and Access to Dental Specialty Centers (PMAQ-CEO). METHODS AND RESULTS: This study is a secondary data analysis from the second cycle from PMAQ-CEO, which evaluated 1097 Dental Specialty Centers (DSCs), conducted in 2018. Seventeen independent variables taken from dentists' training, and dental team management characteristics were analyzed to assess their influence on the reported "Performance of care for SHCN patients". An estimated score was generated from their performance on 23 questions related to the physical and human structure and work processes in the oral health care of SHCN patients in the DSCs. Negative binomial regression model with values p ≤ 0.05 were considered significant. Data analysis included residual deviation to the degree of freedom and the chi-square test. The patient care performance score with SHCN increases significantly when the DSC has a manager (RR = 1.019; CI = 1.011-1.026) and goal monitoring and analysis (RR = 1.012; CI = 1.007-1.005). By contrast, the DSCs in which the dentist is a statutory public servant (RR = 0.998; CI = 0.997-1.000) and performs additional unhealthy or hazardous duties (RR = 0.998; CI = 0.996-0.999) are less likely to provide a higher quality care. CONCLUSION: The heterogeneity and low-performance scores for dental care provided to SHCN was identified in Brazilian health services. It was possible to verify that management and human resource factors of the DSCs were associated with the performance of dental care provided to SHCN patients.

Oral alterations in children with microcephaly associated to congenital Zika syndrome: A systematic review and meta-analyses.

AIMS: To synthesize the oral alterations observed in children with microcephaly associated with congenital Zika virus syndrome (CZS), and to compare the oral alterations of these children to a normotypic healthy controls. METHODS AND RESULTS: A search was performed in six electronic databases. Observational studies published that reported oral alterations in children with CZS were selected. Two authors independently extracted data, assessed study quality, using the Joanna Briggs Institute Critical Appraisal Checklist tools, and the certainty of evidence, using Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. Twenty-one studies were included in this systematic review and meta-analysis. The pooled crude occurrence showed 88% of increased salivation (95%CI: 82%-94%), 83% of biofilm (95%CI: 75%-91%), and 73% of bruxism (95%CI: 52%-95%). Compared to normotypic controls, children with CZS-associated microcephaly had a higher chance to have difficulty in lip sealing (OR: 18.28; 95%CI: 1.42-235.91), inadequate lingual posture at rest (OR: 13.57; 95%CI: 4.24-43.44), and delayed eruption (OR: 12.92; 95%CI: 3.42-48.78), with very low certainty. CONCLUSION: There are several oral alterations found among children with CZS-associated microcephaly. They are more prone to present some of these alterations, such as difficulty in lip sealing, although with very low certainty of evidence.

Radiomorphometric indices and fractal dimension of the mandible in individuals with osteogenesis imperfecta: a matched cross-sectional study.

OBJECTIVE: The objective was to analyze radiomorphometric indices (RMIs) of mandibular cortical bone and fractal dimension (FD) of trabecular bone of individuals with osteogenesis imperfecta (OI) and compare the findings to those of individuals without OI. METHODS: Digital panoramic radiographs of 20 individuals with OI (case group) and 40 individuals without OI (control group) were examined. The RMIs of mandibular cortical index (MCI) and mandibular cortical thickness (MCT) were analyzed. FD of mandibular trabecular bone was calculated bilaterally in 3 regions. The chi-squared test and paired t test were used to compare the significance of differences between the groups. The effect size and minimal clinically important difference (MCID) were also determined. Significance was established at P < .05. RESULTS: Categories C2 and C3 of the MCI were more frequent in individuals with OI (P < .001). The Mean MCT was 2.08 ± 0.79 in the OI case group and 2.91 ± 0.60 in the control group (P < .001). Mean FD in the condylar region was significantly lower in the OI case group (P = .002). The effect size for these 2 measures was large, and the difference between groups was greater than the MCID. CONCLUSION: Radiographs of individuals with OI exhibited more porosity and diminished thickness of the mandibular cortical bone and lower FD values in the condylar trabecular bone.

Dental anomalies in individuals with osteogenesis imperfecta: a systematic review and meta-analysis of prevalence and comparative studies.

BACKGROUND: Osteogenesis imperfecta (OI) is a rare genetic disorder primarily caused by mutations in the genes involved in the production of type 1 collagen. OI is also known as brittle bone disease. OBJECTIVE: This study aims to describe the prevalence of dental anomalies (except dentinogenesis imperfecta) in individuals with OI, and compare the prevalence of dental anomalies between individuals with and without OI and between individuals with different types of OI. SEARCH METHODS: Searches in PubMed, Web of Science, Scopus, Ovid, and gray literature were performed in October 2022. SELECTION CRITERIA: Observational studies (with or without a comparison group) that evaluated the prevalence of dental anomalies in individuals with OI. Data collection and analysis: Data items were extracted by two authors. Quality assessment employing the Joanna Briggs Institute checklists and meta-analyses was conducted. Results were provided in prevalence values and odds ratio (OR) / 95% confidence interval (CI). Strength of evidence was determined. RESULTS: Eighteen studies were included. Most prevalent dental anomalies in individuals with OI included pulp obliteration (46.4%), dental impaction (33.5%), dental impaction of second molars (27%), and tooth agenesis (23.9%). Individuals with OI type III/IV had 20.16-fold greater chance of exhibiting tooth discoloration in comparison with individuals with OI type I (CI: 1.10-370.98). In comparison with the group without OI, the individuals with OI had 6.90-fold greater chance of exhibiting dental impaction (CI: 1.54-31.00). High methodological quality was found in 47% of the studies. Strength of evidence was low or very low. CONCLUSIONS: Pulp obliteration, dental impaction, and tooth agenesis were the most prevalent dental anomalies in the OI group. Individuals with OI were more likely to have dental impaction than individuals without OI. Individuals with OI type III/IV (severe-moderate) are more likely to have tooth discoloration than individuals with OI type I (mild).

Access to oral health care services for individuals with rare genetic diseases affecting skeletal development.

OBJECTIVE: To identify factors associated with oral health care services for individuals with and without rare genetic diseases. MATERIALS AND METHOD: A cross-sectional study was undertaken, with 140 individuals paired by sex and age (70 with rare genetic diseases and 70 without), aged between 3 and 27 years, and their parents. The sample was selected from two reference hospitals for patients with rare genetic diseases in southeastern Brazil. The parents completed a questionnaire on individual aspects and their child's medical/dental history. Participants who did and did not suffer from rare genetic diseases were examined for dental caries, malocclusion, dental anomalies, and oral hygiene. The theoretical model Directed Acyclic Graphs (DAG) was used to identify possible confounding variables in the association between rare diseases and access to dental care. Descriptive analyses and non-matched and matched logistic regression models (p < 0.05) were carried out. RESULTS: The chance of individuals without rare genetic disease having access to oral health care service was 5.32 times higher (95% CI 2.35-12.01) than those with such conditions. Individuals who had not suffered upper respiratory tract infections had a 3.16 times greater chance of being in the group with access to oral health care service (95% CI 1.45-6.90). CONCLUSION: Individuals with no rare genetic diseases and no history of upper respiratory tract infections had a greater chance of belonging to the group of individuals with access to a dental service. Individuals with rare genetic diseases have less access to oral health care.

Some Psychometric Properties of the Brazilian CDC Clear Communication Index.

The Centers for Disease Control and Prevention (CDC) Clear Communication Index (CCI) was cross-culturally adapted to Brazilian Portuguese (BR). It was necessary to analyze the reliability and validity of the BR-CDC-CCI for its use in Brazil. This study aimed to evaluate the psychometric properties of the instrument in its Brazilian version. Four specialists in health education used the BR-CDC-CCI to evaluate a population-level health education material. Primary health care professionals (n = 105) evaluated the same health material using the BRCDC-CCI, and 30 professionals performed the retest 15 to 20 days after the first assessment. Cohen Kappa and area under the receiver operating characteristic (ROC) curve analyses were developed. Inter-rater agreement ranged from moderate to almost perfect, with 90% of the items almost perfect. The percentage of agreement ranged from 8.6% to 98.1%. For the analyzed questions, the area on the ROC curve was 0.9412 (confidence interval [CI] 95%; [0.8259, 1.000]). The BR-CDC-CCI had sufficient validity and reliability for its use in the evaluation of educational/informational materials in health in the Brazilian context. In view of the good results from this psychometric assessment, we anticipated the BR-CDC-CCI could contribute to improvements in Brazilian professionals' skills in developing health communication materials, thereby improving the quality of education and, possibly health outcomes. [HLRP: Health Literacy Research and Practice. 2022;6(2):e84-e87.].

Perspectives on Social and Environmental Determinants of Oral Health.

Most oral conditions have a multifactorial etiology; that is, they are modulated by biological, social, economic, cultural, and environmental factors. A consistent body of evidence has demonstrated the great burden of dental caries and periodontal disease in individuals from low socioeconomic strata. Oral health habits and access to care are influenced by the social determinants of health. Hence, the delivery of health promotion strategies at the population level has shown a great impact on reducing the prevalence of oral diseases. More recently, a growing discussion about the relationship between the environment, climate change, and oral health has been set in place. Certainly, outlining plans to address oral health inequities is not an easy task. It will demand political will, comprehensive funding of health services, and initiatives to reduce inequalities. This paper sought to give a perspective about the role of social and physical environmental factors on oral health conditions while discussing how the manuscripts published in this Special Issue could increase our knowledge of the topic.

Mother's sense of coherence and dental characteristics in children and adolescents with osteogenesis imperfecta: A paired study.

BACKGROUND: The relevance of sense of coherence (SOC) is important to the wellbeing of parents, especially mothers of children and adolescents with osteogenesis imperfecta (OI). OBJECTIVE: Determine whether the oral health status of children/adolescents with OI is associated with mother's SOC. MATERIALS AND METHOD: A paired cross-sectional study was conducted with 37 children/adolescents with OI, 37 without OI, and their respective mothers. The children/adolescents were between two and 19 years of age, mean age 7.2 years, being 47 male and 27 female. The mothers completed Antonovsky's SOC questionnaire (SOC-13), and the oral status of the children/adolescents was investigated. The following clinical conditions were evaluated: dentinogenesis imperfecta, malocclusion, gingivitis, and dental caries experience. RESULTS: The genetic condition of the children was significantly associated with mother's SOC (P < .001). Mothers of children with OI had lower SOC scores (mean: 35.6 [± 4.9]) than mothers of children without OI (mean: 38.5 [± 4.3]). In the group with OI, a low socioeconomic status was associated with lower mother's SOC scores (P = .004). In both groups, dental caries experience was associated with lower mother's SOC scores (P = .007). Most individuals with OI presented malocclusion (78.3%) and experience of dental caries (59.4%). CONCLUSION: Having a child with OI influenced the sense of coherence of the mothers. Socioeconomic status and dental caries experience in children and adolescents with OI were associated with mother's SOC.

Dental and maxillomandibular incidental findings in panoramic radiography among individuals with mucopolysaccharidosis: a cross-sectional study.

Mucopolysaccharidosis (MPS) is a group of rare and inherited metabolic disorders caused by the accumulation of macromolecule glycosaminoglycans inside lysosomes. Affected individuals may have dental and craniofacial tissue alterations, facilitating the development of several oral diseases. OBJECTIVES: To assess, with panoramic radiographic images, the frequency of dental and maxillomandibular incidental findings among MPS individuals and compare them with non-MPS individuals. METHODOLOGY: A cross-sectional study evaluating a sample of 14 MPS individuals and 28 non-MPS individuals aged from 5 to 26 years was carried out. They were matched for sex and age on a 2:1 proportion. Panoramic radiographs were assessed for the presence/absence of the following dental and maxillomandibular alterations: dental anomalies of number (hypodontia/dental agenesis, supernumerary teeth); anomalies of form (microdontia, macrodontia, conoid teeth, taurodontism, and root dilaceration); anomalies of position (impacted tooth, inverted tooth, tooth migration, partially bony teeth, complete bony teeth); periapical alterations (furcation lesion, circumscribed bone rarefaction); other alterations (radiolucent bone lesions, radiopaque bone lesions, radiopacity in the maxillary sinus, condylar hypoplasia). Differences between groups were tested by the Fisher's exact test and chi-square test (p<0.05). RESULTS: For intrarater agreement, Kappa values were 0.76 to 0.85. The presence of supernumerary teeth (p=0.003); conoid teeth (p=0.009); taurodontism (p<0.001); impacted teeth (p<0.001); partial bony teeth (p=0.040); complete bony teeth (p=0.013); and root dilaceration (p=0.047) were statistically more frequent in MPS individuals compared to non-MPS individuals. Bone rarefaction/furcation lesions (p=0.032), condylar hypoplasia (p<0.001), radiolucent bone lesions (p=0.001), and dentigerous cysts (p=0.002) were also more frequent in MPS individuals. CONCLUSION: The presence of specific oral manifestations is more common in MPS individuals than non-MPS individuals.

Caregivers' Perception of Oral Health-Related Quality of Life of Individuals with Down Syndrome.

Purpose: To evaluate the perception of caregivers of individuals with Down syndrome (DS) regarding the impact of oral conditions on their children's oral health-related quality of life (OHRQoL) and compare with the perception of caregivers of children without DS.
Methods: This is a comparative cross-sectional study. Participants were 144 four- to 18-year-old children with DS, matched by sex and age with a group without DS, and their caregivers. The children were assessed for dental caries experience, clinical consequences of untreated dental caries, gingival bleeding, visible plaque, and malocclusion. Caregivers answered a questionnaire on sociodemographic conditions and completed the Parental-Caregiver Perceptions Questionnaire (P-CPQ). Data analysis included the Wilcoxon test, chi-square test, and Poisson regression (PR).
Results: Caregivers of individuals with DS presented more negative perceptions of their children's OHRQoL in the functional limitations domain (P<0.01) and in the total P-CPQ score (P=0.02). The determining factors were clinical consequences of untreated dental caries (PR=1.72; 95 percent confidence interval [95% CI]=1.04 to 2.84) and visible plaque (PR=1.48; 95% CI=1.08 to 2.03).
Conclusion: Caregivers of individuals with DS presented more negative perceptions of the impact of oral conditions on their children's OHRQoL. Clinical consequences of untreated dental caries and visible plaque were the determining factors with the greatest impact on the perception of their children's OHRQoL.

Cross-cultural adaptation of the Clear Communication Index to Brazilian Portuguese.

OBJECTIVE: To perform a cross-cultural adaptation of the Clear Communication Index instrument from the Centers for Disease Control and Prevention (CDC-CCI) from English to Brazilian Portuguese. METHODS: This study comprised initial discussion about the conceptual equivalence of the instrument by a committee formed by experts on health education. We performed translations, synthesis of translations, back-translations, revision by the committee, and linguistic revision. Semantic equivalence was obtained by analyzing the referential and general meaning of each item by the committee, resulting in a pre-final version of the instrument. Subsequently, thirty professionals with health sciences degrees performed a pre-test. These professionals used the pre-final version of the instrument to assess a health education material. A questionnaire was applied to evaluate the acceptability of the instrument, the understanding of each of the 20 items, as well as the individual and professional variables. We analyzed the scores attributed to the health education material, the variables related to healthcare professionals, the proportions of the acceptability of the instrument, and the comprehension of each item. RESULTS: After we obtained the conceptual equivalence of the instrument, the committee of experts, the instrument's main author, and the linguist produced the pre-final version using two translations, a synthesis of the translations, and two back-translations. A general equivalence was maintained in 15 of the 20 items (75%), four of the items were slightly altered (20%), and one item was very altered (5%). Nineteen items presented referential equivalence or near equivalence (95%). We then carried out with the pre-test, in which the professionals used the pre-final version. Two items in the domains of "risks" and "main message" were unclear and needed to be revised. CONCLUSION: The process of cross-cultural adaptation of the Clear Communication Index provided an adapted version to the Brazilian Portuguese language.

Rare genetic diseases affecting skeletal development and oral health disparities among children and adolescents: a pathway analysis.

BACKGROUND: To examine the relationships of rare genetic diseases affecting skeletal development, socio-demographic characteristics, and oral health-related behaviours with dental clinical measures in children and adolescents. METHODS: A cross-sectional study paired by age, gender and social class included 61 children and adolescents with osteogenesis imperfecta (n = 40) or mucopolysaccharidoses (n = 21) and those without genetic rare diseases (n = 60). Participants were selected at two referral hospitals for rare genetic diseases in the city of Belo Horizonte, Brazil. Caregivers completed a questionnaire to obtain age, gender, caregiver's schooling, social class, patterns of dental attendance and duration of breastfeeding. Oral hygiene, dental caries, dental anomalies and malocclusion were assessed through dental examinations. The relationships between variables were estimated through Pathway analysis using the maximum likelihood method. RESULTS: Rare genetic diseases affecting skeletal development were directly associated with dental caries (ß = 0.22), dental anomalies (ß = 0.36) and malocclusion (ß = 0.29). They were also inversely linked to a preventive pattern of dental attendance (ß = -0.25). Rare genetic diseases affecting skeletal development were associated with poor oral hygiene (ß = 0.28) and shorter breastfeeding duration (ß = -0.21). Rare genetic diseases affecting skeletal development were linked indirectly with dental caries, a reduced pattern of dental attendance and poor oral hygiene (ß = 0.43). Patterns of dental attendance mediated the link between rare genetic diseases affecting skeletal development and malocclusion (ß = -0.05). CONCLUSION: Rare genetic diseases affecting skeletal development were associated with poor oral health. Patterns of dental attendance and poor oral hygiene mediated the link between rare genetic diseases affecting skeletal development and dental clinical measures.

Oral health-related quality of life of young people with mucopolysaccharidosis: a paired cross-sectional study.

The purpose of the present study was to compare the perceptions of the parents/ caregivers of young people with and without Mucopolysaccharidosis (MPS) with regards to their oral health-related quality of life (OHRQoL). A cross-sectional study was conducted with 29 individuals with MPS and 29 normotypic individuals aged three to 21 years and their parents/caregivers. All parents/caregivers of young people with MPS in follow-up at two reference hospitals in the city of Belo Horizonte, southeastern Brazil, were invited to participate in the study. Individuals without MPS were recruited from the pediatric clinics of both hospitals. Parents/caregivers answered a structured questionnaire addressing the sociodemographic characteristics, behavioral habits and medical and dental history of the children as well as the Brazilian short-form version of the Parental-Caregiver (P-CPQ). The individuals with and without MPS were examined for malocclusion, dental caries and oral hygiene by an examiner who had undergone training and calibration exercises. Mean age of the subjects was 12.1 years (± 4.2). Comparing total P-CPQ scores and scores on the oral symptoms, functional limitations and wellbeing domains, the parents/caregivers individuos with MPS reported a statistically significant greater negative impact on OS domain than their counterparts. Regarding the clinical variables, malocclusion was also associated a greater negative impact on OHRQoL of young people with MPS when compared to those of young people without MPS. Our findings show the great negative impact caused by the malocclusion of young people with MPS.

Risk factors associated with reported bruxism among children and adolescents with Down Syndrome.

Objective: Identify factors associated with the prevalence of reported bruxism in children/adolescents with Down Syndrome (DS). Methods: The study included 112 children/adolescents with DS and their parents/caregivers. Oral habits, pacifier/finger sucking, upper respiratory infections (