Oral Problems in Brazilian Individuals with Rare Genetic Diseases That Affect Skeletal Development.

The present study aimed to compare the prevalence of oral problems between individuals with rare genetic diseases that affect skeletal development and individuals without rare diseases. A cross-sectional study was conducted with 210 individuals between two and fifty-four years of age: 105 with rare genetic diseases (27 with mucopolysaccharidosis [MPS] and 78 with osteogenesis imperfecta [OI]) and 105 without rare diseases. The rare genetic disease group was recruited from hospital units that provide care for patients with MPS and OI in five states of Brazil, and the other group was recruited from the same location. The participants were examined with regards to malocclusion, dental anomalies, dental caries, and gingivitis. A questionnaire was administered addressing individual, sociodemographic, and behavioral characteristics as well as dental history. A descriptive analysis was performed, followed by unadjusted and adjusted binary logistic regression analyses. The mean age was 14.1 ± 12.2 years. Individuals with a rare disease were 12.9-fold more likely to have some type of oral problem (95% CI: 3.7-44.7) compared to the group without rare diseases. The prevalence of oral problems was higher among Brazilians with MPS and OI compared to normotypical individuals.

Oral health-related quality of life in Fanconi anemia: a cross-sectional study.

PURPOSE: To evaluate the oral health-related quality of life (OHRQoL) of individuals diagnosed with Fanconi anemia (FA). METHODS: A cross-sectional study was conducted with FA patients from two Brazilian referral centers. Participants underwent a complete dental, periodontal, and oral mucosa examination, as well as assessment of resting salivary flow. The short version of the Oral Health Impact Profile (OHIP-14) questionnaire was administered. Descriptive and bivariate analyses were performed, followed by multivariate analysis to examine the impact of independent variables on OHRQoL. RESULTS: The study included 20 (57.1%) males and 15 (42.9%) females, with a mean age of 18.9 years. Oral leukoplakia (OL) was found in 18 individuals. The overall OHIP-14 score was 9.9 ± 10.5. Individuals aged ≥ 16 years had higher OHIP-14 scores, indicating worse OHRQoL for physical pain (p = 0.007), psychological discomfort (p = 0.001), physical disability (p = 0.03), psychological disability (p = 0.001), handicap (p = 0.004), and overall score (p = 0.007). Females reported more negative OHRQoL than males for physical pain (p = 0.02), psychological discomfort (p = 0.03), psychological disability (p = 0.009), and overall score (p = 0.02). Individuals with OL had an overall OHIP-14 score 1.83 times higher than those without OL (95% CI: 1.02-3.28; p = 0.04). Lower salivary flow correlated with higher overall OHIP-14 scores (95% CI: 0.14-0.84; p = 0.01). CONCLUSION: This study represents the first attempt to evaluate OHRQoL in individuals with FA. The presence of OL and reduced salivary flow were identified as predictors of a negative impact on OHRQoL. It is imperative to integrate patients' quality of life in the clinical treatment protocols for the FA population.

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).

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

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).

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.

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.

Mothers of a Child with Cerebral Palsy: Perception on the Diagnosis Moment and the Child's General and Oral Health

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.

Análise orofacial de indivíduos com Osteogênese Imperfeita: índices radiomorfométricos, dimensão fractal do osso mandibular e má oclusão / Orofacial analysis of individuals with Osteogenesis Imperfecta: radiomorphometric indices, fractal dimension of the mandibular bone and malocclusion

A Osteogênese Imperfeita (OI) é uma doença genética rara, caracterizada por ossos frágeis com fraturas recorrentes. Na maioria dos casos a OI, é causada por mutações nos genes COL1A1 ou COL1A2 os quais codificam o colágeno tipo I. Mutações em novos genes envolvidos na via do metabolismo ósseo têm sido descobertas. A OI está associada a alterações dentárias e craniofaciais, sendo as mais prevalentes a dentinogênese imperfeita e a má oclusão. A literatura tem mostrado que é possível predizer o risco de fratura óssea ao analisarmos índices radiomorfométricos e dimensão fractal (DF) da mandíbula em radiografias panorâmicas. O objetivo desta pesquisa foi verificar se há diferenças no padrão de oclusão, na cortical e no trabeculado ósseo mandibular de indivíduos com OI quando comparados com indivíduos sem OI. Desse modo, a tese conta com a apresentação de dois artigos científicos. O primeiro artigo objetivou analisar dois índices radiomorfométricos, o índice cortical mandibular (ICM) e o índice mentual (IM), e a DF do trabeculado ósseo mandibular de indivíduos com OI e comparar com indivíduos sem OI. Foi realizado um estudo transversal, pareado por idade e sexo, com 20 indivíduos com OI e 40 sem OI. Os dados foram obtidos por meio de radiografias panorâmicas de pacientes com OI e sem OI atendidos na Faculdade de Odontologia da Universidade Federal de Minas Gerais. O estudo foi aprovado pelo Comitê de Ética em Pesquisa da UFMG (protocolo 02470518.3.0000.5149). O teste t pareado (p <0,05) foi usado para comparar os valores de IM e DF. O teste do qui-quadrado (p <0,05) comparou o ICM entre os grupos. A média de idade de ambos os grupos foi 13,10 anos (± 6,57). O valor médio do IM foi de 2.08 (±0.79) no grupo de indivíduos com OI e 2.91 (±0.60) para indivíduos sem OI (p<0,001). O valor médio de DF do grupo OI [0.3248 (±0.7240)] foi inferior ao do grupo sem OI [0.3814 (±0.5587)] no côndilo mandibular (p=0,002). O grau C3 do ICM foi mais frequente entre os indivíduos com OI (p <0,001). Indivíduos com OI apresentaram valores menores nos IM e DF, além de pior morfologia da cortical mandibular. O segundo artigo, uma revisão sistemática e meta-análise (já publicada), objetivou avaliar se indivíduos com OI são mais afetados por má oclusão do que indivíduos normotípicos. Foi realizada uma busca nas principais bases. A avaliação do risco de viés e a análise da força de evidência foram conduzidas. Em comparação com indivíduos sem OI, o grupo com OI teve 19,69 vezes mais chance de apresentar má oclusão de Classe III de Angle (OR = 19,69, IC: 9,00­43,09) e apresentar maior mordida cruzada anterior (MD = 6,08, CI: 2,40­9,77). Indivíduos sem OI tiveram um ângulo ANB (MD= 3,88, IC: 1,15­6,61) e ângulo SNA (MD = 2,11, IC: 0,24­3,98) significativamente maiores em comparação com indivíduos com OI. Nenhuma diferença entre os grupos foi encontrada para SNB (MD = −0,50, IC: −2,21 a 1,21) e mordida aberta (MD = 0,98, IC: −0,29 a 2,25). A maioria dos estudos incluídos teve qualidade metodológica moderada. A força da evidência foi baixa ou muito baixa. A ocorrência de má oclusão Classe III de Angle e mordida cruzada anterior foi maior entre os indivíduos com OI em comparação com aqueles sem OI.

Osteogenesis Imperfecta (OI) is a rare genetic disease characterized by fragile bones with recurrent fractures. In most cases, OI is caused by mutations in the COL1A1 or COL1A2 genes which encode type I collagen. Mutations in new genes involved in the bone metabolism pathway have been discovered. OI is associated with dental and craniofacial alterations, the most prevalent being dentinogenesis imperfecta and malocclusion. The literature has shown that it is possible to predict the risk of bone fracture when analyzing radiomorphometric indices and fractal dimension (FD) of the mandible in panoramic radiographs. The objective of this research was to verify if there are differences in the occlusion pattern, in the cortical and in the mandibular bone trabeculate of individuals with OI when compared to individuals without OI. Thus, the thesis has the presentation of two scientific articles. The first article aimed to analyze two radiomorphometric indices, the mandibular cortical index (MCI) and the mentual index (MI), and the FD of the mandibular bone trabeculate of individuals with OI and compare with individuals without OI. A cross-sectional study, matched by age and sex, was carried out with 20 individuals with OI and 40 without OI. Data were obtained through panoramic radiographs of patients with OI and without OI treated at the Faculty of Dentistry of the Federal University of Minas Gerais. The study was approved by the Research Ethics Committee at UFMG (protocol 02470518.3.0000.5149). Paired t-test (p < 0.05) was used to compare MI and DF values. The chi-square test (p < 0.05) compared the ICM between groups. The mean age of both groups was 13.10 years (± 6.57). The mean value of MI was 2.08 (±0.79) in the group of individuals with OI and 2.91 (±0.60) for individuals without OI (p<0.001). The mean FD value of the OI group [0.3248 (±0.7240)] was lower than that of the group without OI [0.3814 (±0.5587)] in the mandibular condyle (p=0.002). ICM grade C3 was more frequent among individuals with OI (p<0.001). Individuals with OI had lower MI and DF values, in addition to worse mandibular cortical morphology. The second article, a systematic review and meta-analysis (already published), aimed to assess whether individuals with OI are more affected by malocclusion than normotypic individuals. A search was carried out in the main bases. Risk of bias assessment and strength of evidence analysis were conducted. Compared with individuals without OI, the group with OI was 19.69 times more likely to have Angle Class III malocclusion (OR = 19.69, CI: 9.00­ 43.09) and to have greater anterior crossbite (MD = 6.08, CI: 2.40­9.77). Subjects without OI had a significantly greater ANB angle (MD= 3.88, CI: 1.15­6.61) and SNA angle (MD= 2.11, CI: 0.24­3.98) compared to subjects with hi. 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 of the included studies were of moderate methodological quality. The strength of the evidence was low or very low. The occurrence of Angle Class III malocclusion and anterior crossbite was higher among individuals with OI compared to those without OI.

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.

Assessing a possible vulnerability to dental caries in individuals with rare genetic diseases that affect the skeletal development.

BACKGROUND: Individuals diagnosed with a rare genetic disease that affects skeletal development often have physical limitations and orofacial problems that exert an impact on oral health. The aim of the present study was to analyze the possible vulnerability to dental caries in individuals with rare genetic diseases that affect skeletal development. METHODS: A paired cross-sectional study was carried out with a sample of 140 individuals [70 with rare genetic diseases affecting skeletal development: mucopolysaccharidosis (MPS) (n = 29) and osteogenesis imperfecta (OI) (n = 41) and 70 without rare diseases] and their parents/caregivers. The participants in the first group were recruited from two reference hospitals specialized in rare genetic diseases in the city of Belo Horizonte, Brazil. All participants were examined for the evaluation of breathing type, malocclusion, dental anomalies, oral hygiene and dental caries. The parents/caregivers answered a structured questionnaire addressing the individual/behavioral characteristics and medical/dental history of the participants. Statistical analysis involved the chi-square test and multiple logistic regression analysis for the dependent variable (dental caries) (α = 5%). This study received approval from the Human Research Ethics Committee of the Universidade Federal de Minas Gerais. RESULTS: The mean age of the individuals was 10.34 ± 6.55 years (median: 9.50 years). Individuals with inadequate oral hygiene were 4.70-fold more likely to have dental caries (95% CI: 2.13-10.40) and those with the rare genetic diseases (MPS/OI) were 2.92-fold more likely to have dental caries (95% CI: 1.38-6.17). CONCLUSION: Individuals with inadequate oral hygiene and those with MPS and OI had a greater chance of belonging to the group with dental caries. Based on the present findings, individuals with the rare genetic diseases may be considered vulnerable to caries.

Vulnerabilidade à cárie dentária em indivíduos diagnosticados com doenças genéticas raras / Vulnerabilidade à cárie dentária em indivíduos com doenças raras / Vulnerability to dental caries in individuals diagnosed with rare genetic diseases

As doenças raras são todas as doenças que afetam até 65 pessoas em cada 100.000 habitantes. Há uma grande diversidade de sinais e sintomas presentes nos indivíduos afetados, que variam conforme a etiologia da doença e também de pessoa para pessoa. Em muitas doenças raras os sinais e sintomas são percebidos ao nascimento ou ainda na infância. Comumente, indivíduos com doenças raras apresentam alterações no sistema nervoso e/ou musculoesquelético, havendo comprometimento cognitivo, distúrbios neuropsicomotores e má formações craniofaciais. Algumas manifestações orais, como má oclusões e anomalias dentárias, também são comuns nessa parcela da população. O conceito de vulnerabilidade em saúde baseia-se na compreensão das suscetibilidades dos indivíduos ao adoecimento, sendo a doença considerada o resultado de um conjunto de fatores individuais, coletivos e contextuais. Este estudo objetivou analisar a chance de cárie dentária em indivíduos com doenças raras. Foi realizado um estudo transversal com uma amostra pareada de 140 indivíduos [70 com doenças raras - Mucopolissacaridoses (n=29) / Osteogênese Imperfeita (n=41) - e 70 sem doenças raras] e os pais/responsáveis. A amostra foi selecionada em dois hospitais referência para atendimento de pacientes com doenças raras de Belo Horizonte, região sudeste do Brasil. Os indivíduos com doenças raras e sem doenças raras foram examinados quanto à respiração bucal, má oclusão, anomalia dentária, higiene bucal e cárie dentária. Os pais/responsáveis responderam um questionário estruturado sobre o filho (características individuais / comportamentais e histórico médico / odontológico). Análises univariada, bivariada (teste X 2 ) e multivariada (regressão logística múltipla) foram realizadas, sendo a cárie dentária a variável dependente. Foi considerada uma significância estatística de p <0,05. Esta pesquisa foi aprovada pelo Comitê de Ética em Pesquisa em Seres Humanos da Universidade Federal de Minas Gerais. A faixa etária dos indivíduos examinados foi de dois a 27 anos, com uma média de 10,3 anos (± 6,5) de idade. Independente de ter doença rara ou não, os indivíduos com higiene bucal inadequada apresentaram uma chance 4,70 vezes maior de pertencer ao grupo com cárie dentária (IC: 2,03-10,16, 95%). Os indivíduos com doença rara tiveram 2,92 vezes mais chances de estar no grupo com cárie dentária (IC: 1,38-6,18, 95%). Concluiu-se que os indivíduos identificados com uma higiene bucal inadequada, bem como aqueles diagnosticados com alguma doença rara apresentaram maior chance de pertencerem ao grupo com cárie dentária. Com base neste estudo, os indivíduos com doenças raras foram considerados vulneráveis à cárie dentária.(AU)

Rare diseases are all diseases that affect up to 65 people out of every 100,000 inhabitants. There is a great diversity of signs and symptoms present in the affected individuals, which vary according to the etiology of the disease and also from person to person. In many rare diseases the symptoms are noticed at birth or even in childhood. Generally, individuals with rare diseases present disturbances in the nervous and/or musculoskeletal system, with cognitive impairment, neuropsychomotor disorders and craniofacial alterations. Some oral manifestations, such as malocclusions and dental anomalies, are usually common in this population. The concept of vulnerability in health is based on the understanding of individuals' susceptibilities to illness, and the disease is considered to be a result of a set of individual, collective and contextual factors. This study aimed to analyze the chance of dental caries in individuals with rare diseases. A cross - sectional study was performed with a paired sample of 140 individuals [70 with rare diseases - Mucopolysaccharidosis (n=29) / Osteogenesis imperfecta (n=41) - and 70 without rare diseases] and their parents / guardians. The sample was selected in two reference hospitals responsible for the care of patients with rare diseases in Belo Horizonte, in the southeast of Brazil. Individuals with and without rare diseases were examined for breathing type, malocclusion, dental anomaly, oral hygiene and dental caries. Parents / guardians answered a structured questionnaire about their child (individual / behavioral characteristics and medical / dental history). Univariate, bivariate (chi-square test) and multivariate analyzes (multiple logistic regression) were performed, with dental caries being the dependent variable. A statistical significance of p <0.05 was considered. The Research Ethics Committee of the Federal University of Minas Gerais approved this research. The age group of the individuals examined was from 2 to 27 years, with an average age of 10.3 years (± 6.5). Regardless of the presence or absence of a rare disease, individuals with inadequate oral hygiene had a 4.70-fold higher chance of belonging to the group with dental caries (CI: 2.03-10.16, 95%). Individuals with rare disease were 2.92 times more likely to be in the group with dental caries (CI: 1.38-6.18, 95%). It was concluded that individuals identified with inadequate oral hygiene, as well as those diagnosed with a rare disease, were more likely to belong to the group with dental caries. Based on this study, individuals with rare diseases were considered vulnerable to dental caries.(AU)