Abstract 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).

ABSTRACT Objective: To analyze the perception of mothers of children with Cerebral Palsy (CP) on the diagnosis moment and the child’s health. Material and Methods: Research with a qualitative approach, carried out with 19 mothers of children with CP, in a public higher education institution, in the state of Minas Gerais, Brazil. For data collection, the interview was used and for data interpretation, content analysis. Results: Mothers reported that the diagnosis of a child with CP resulted in major changes in the family’s daily life, increasing their responsibility and demands. After the diagnosis, mothers revealed oscillating feelings, with progressively replaced by her motherly ability to take care, reestablishing the psychic balance. The health associated with the absence of disease and curative practices was frequently observed. Mothers reported a great concern with oral hygiene habits and frequent visits to the dentist. Conclusion: The diagnosis of a child with CP led to changes in the family's priorities and routine. After the moment of anguish, uncertainty and fear, the mothers accepted the reality. The biomedical principle significantly influenced the mother´s perception of health, being health perceived as the absence of disease and curative practices. In relation to oral health, practices widely spread and recommended by the media and health services, such as correct tooth brushing, showed an orientation of patients to maintain oral health.

Abstract 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.

Resumo Este estudo objetivou analisar os fatores associados ao uso de serviços odontológicos por pacientes em hemodiálise. Foi realizado um estudo transversal com 467 pacientes em hemodiálise, na faixa etária de 19 a 90 anos, de Contagem e Belo Horizonte, região Sudeste do Brasil. Os dados foram coletados por meio de exame clínico bucal dos participantes e da aplicação de um questionário estruturado. A variável dependente foi o acesso odontológico, mensurado pela pergunta "Você foi ao dentista nos últimos seis meses?". A média de idade dos participantes foi de 49,9 anos. A média de dentes presentes na boca foi de 19,3. Uma média de 1,5 dentes com lesão de cárie cavitada foi diagnosticada entre os pacientes em hemodiálise. Um terço da amostra afirmou ter ido ao dentista nos últimos seis meses (27,8%). O acesso odontológico dos pacientes em hemodiálise foi associado à escolaridade (OR = 1,5 [1,1-2,4]), orientação profissional para ir ao dentista (OR = 2,1 [1,2-3,8]) e prevalência de cárie dentária (OR = 2,1 [1,3-3,2]). Os pacientes em hemodiálise com oito anos ou mais de escolaridade, que receberam orientação profissional para ir ao dentista e sem cárie dentária apresentaram maior chance de terem acesso odontológico.

Abstract The present study aimed to analyze factors associated with access of dental care services by Brazilian hemodialysis patients. A cross-sectional study was carried out with 467 hemodialysis patients aging from 19 to 90 years in two renal therapy centers located in the cities of Contagem and Belo Horizonte, Southeastern Brazil. Data were collected through an oral clinical examination of the patients and the application of a structured questionnaire. The dependent variable was the access to dental care, measured by the question "Have you consulted with a dentist in last six months?". The mean age of participants was 49.9 years. The average number of teeth present in the mouth was 19.3. An average of 1.5 teeth with dental caries cavities lesion was diagnosed among hemodialysis patients. One-third of the sample had gone to the dentist in the last six months (27.8%). The access to dental care was associated with formal education (OR = 1.5 [1.1-2.4]), professional advising to consult with a dentist (OR = 2.1 [1.2-3.8]) and prevalence of dental caries (OR = 2.1 [1.3-3.2]). Hemodialysis patients with eight or more years of formal education, who received professional advising to consult with a dentist and without dental caries cavities had higher chances obtaining access to dental care.

Resumo Este estudo objetivou analisar os fatores associados ao uso de serviços odontológicos por pacientes em hemodiálise. Foi realizado um estudo transversal com 467 pacientes em hemodiálise, na faixa etária de 19 a 90 anos, de Contagem e Belo Horizonte, região Sudeste do Brasil. Os dados foram coletados por meio de exame clínico bucal dos participantes e da aplicação de um questionário estruturado. A variável dependente foi o acesso odontológico, mensurado pela pergunta "Você foi ao dentista nos últimos seis meses?". A média de idade dos participantes foi de 49,9 anos. A média de dentes presentes na boca foi de 19,3. Uma média de 1,5 dentes com lesão de cárie cavitada foi diagnosticada entre os pacientes em hemodiálise. Um terço da amostra afirmou ter ido ao dentista nos últimos seis meses (27,8%). O acesso odontológico dos pacientes em hemodiálise foi associado à escolaridade (OR = 1,5 [1,1-2,4]), orientação profissional para ir ao dentista (OR = 2,1 [1,2-3,8]) e prevalência de cárie dentária (OR = 2,1 [1,3-3,2]). Os pacientes em hemodiálise com oito anos ou mais de escolaridade, que receberam orientação profissional para ir ao dentista e sem cárie dentária apresentaram maior chance de terem acesso odontológico.

Abstract The present study aimed to analyze factors associated with access of dental care services by Brazilian hemodialysis patients. A cross-sectional study was carried out with 467 hemodialysis patients aging from 19 to 90 years in two renal therapy centers located in the cities of Contagem and Belo Horizonte, Southeastern Brazil. Data were collected through an oral clinical examination of the patients and the application of a structured questionnaire. The dependent variable was the access to dental care, measured by the question "Have you consulted with a dentist in last six months?". The mean age of participants was 49.9 years. The average number of teeth present in the mouth was 19.3. An average of 1.5 teeth with dental caries cavities lesion was diagnosed among hemodialysis patients. One-third of the sample had gone to the dentist in the last six months (27.8%). The access to dental care was associated with formal education (OR = 1.5 [1.1-2.4]), professional advising to consult with a dentist (OR = 2.1 [1.2-3.8]) and prevalence of dental caries (OR = 2.1 [1.3-3.2]). Hemodialysis patients with eight or more years of formal education, who received professional advising to consult with a dentist and without dental caries cavities had higher chances obtaining access to dental care.

RESUMO A Osteogênese imperfeita é uma doença genética rara que ocorre devido à produção anormal de colágeno tipo I, comprometendo os tecidos ósseos e aqueles ricos em colágeno. Os indivíduos com Osteogênese imperfeita apresentam diversas anormalidades na cavidade bucal, incluindo a presença de dentinogênese imperfeita. O objetivo do relato de caso foi discutir o manejo odontológico de um menino de nove anos, diagnosticado com Osteogênese Imperfeita tipo III, com enfoque nos aspectos dentários e oclusais. O caso foi desenvolvido em uma das clínicas da Faculdade de Odontologia da Universidade Federal de Minas Gerais, na região sudeste do Brasil. O exame clínico bucal revelou presença de má oclusão de Classe III de Angle, e mordida cruzada anterior. Observou-se também, a presença de dentinogênese imperfeita na dentição decídua e permanente. A análise radiográfica revelou câmaras pulpares completamente obliteradas em ambas as dentições. O tratamento odontológico incluiu orientação de higiene bucal, fluorterapia, exodontias e restaurações. A criança foi acompanhada por um período de seis anos e, em seguida, encaminhada para a clínica de Ortodontia da mesma universidade para o tratamento da má oclusão. O atendimento odontológico precoce é importante para a prevenção ou interceptação de doenças bucais, como a dentinogênese imperfeita e má oclusão, bem como para a melhora da estética dentária nos indivíduos com Osteogênese Imperfeita.

ABSTRACT Osteogenesis imperfecta is a rare genetic disorder involving abnormal type I collagen composition that compromises bone and collagen-rich tissues. Individuals with Osteogenesis imperfecta exhibit oral and systemic abnormalities, including dentinogenesis imperfect. The purpose of the review is to report a clinical case of a nine-year-old boy diagnosed with Osteogenesis Imperfecta type III, focusing on dental and occlusal aspects. The case report was developed at the outpatient clinic at the School of Dentistry of the Federal University of Minas Gerais, in Southeastern of Brazil. The clinical oral examination revealed Angle Class III malocclusion and anterior crossbite. It was also observed the presence of dentinogenesis imperfect in both primary and permanent teeth. Radiographic analysis showed the presence of completed obliterated pulp chambers in both dentitions. Dental treatment included oral hygiene counseling, dental extraction, fluoride therapy and restorations. The child was followed up for a period of six years and then referred to the orthodontic outpatient clinic at the same university for the treatment of malocclusion. Early dental care is important to the prevention or interception of oral diseases, such as dentinogenesis imperfect and malocclusion, as well as the improvement of dental esthetics in cases of Osteogenesis Imperfecta.

Abstract 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.