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).

Protocol proposal for early dental care in infants with cerebral palsy

Introduction: Children with cerebral palsy (CP) are at high risk of developing oral diseases. Objective: To propose an early dental care protocol for infants with CP. Materials and Methods: A computerized systematic search was performed in the PubMed, Scopus and Embase electronic databases for relevant articles. An early dental care protocol was then proposed for infants with cerebral palsy focused on comprehensive care with a multidisciplinary approach and effective health promotion by caregivers. Results: Fifteen published papers were included in the present literature review and protocol proposal. The protocol comprised the following topics: First dental visit, aspects related to the appointment, oral hygiene recommendations, dietary recommendations and recommendations for the prevention and control of harmful oral habits. The first dental visit should occur prior to the eruption of the teeth. As a special group, it is important to determine the affective bond between the patient and caregiver who will receive the oral health care recommendations. During the clinical examination, the correct positioning and stabilization of the infant is important for the control of involuntary movements and the minimization of swallowing difficulties. Counseling with regards to adequate oral hygiene, a healthy diet and the prevention of harmful oral habits is important to the prevention of dental diseases. Children with oral-motor motility problems and feeding difficulties should be referred to therapeutic follow-up. Due to the neuropsychomotor disorders often found in cerebral palsy, affected children are more vulnerable to oral diseases. Thus, oral health care must be performed as early as possible by the parents/caregivers of these children. Conclusion: Individuals with cerebral palsy are at greater risk of developing oral problems. Thus, oral health programs starting in early childhood and targeting the specificities of these individuals is a strategy for minimizing the occurrence of such problems and the associated burden.

Introdução: Crianças com Paralisia Cerebral (PC) apresentam um alto risco para o desenvolvimento de doenças bucais. Objetivo: Propor um protocolo de atendimento odontológico precoce para lactentes com PC. Materiais e Métodos: Foi realizada uma busca computadorizada sistemática nas bases de dados eletrônicas PubMed, Scopus e Embase. Também foi desenvolvida uma proposta de protocolo de atendimento a bebês com Paralisia Cerebral com foco no cuidado integral, abordagem transdisciplinar e promoção efetiva da saúde pelos cuidadores. Resultados: Foram incluídos 15 artigos publicados na literatura científica. O protocolo desenvolvido é composto pelos seguintes tópicos: Primeira consulta odontológica, tempo ideal da consulta, recomendações de higiene bucal, recomendações sobre dieta e recomendações sobre prevenção e controle dos hábitos bucais. A primeira visita ao dentista deve ser feita antes da erupção do primeiro dente. Como um grupo especial, é importante determinar o vínculo afetivo entre o paciente e o cuidador que receberá as recomendações de cuidados com a saúde bucal. Durante o exame clínico, a correta posição e estabilização do bebê na cadeira odontológica é importante para controlar os movimentos involuntários e reduzir a dificuldade de deglutição. Recomendações quanto à higiene bucal adequada, alimentação saudável e prevenção de hábitos bucais deletérios são importantes para prevenir o desenvolvimento de doenças bucais. Crianças com problemas de motilidade oral-motora e dificuldades de alimentação devem ser encaminhadas para acompanhamento terapêutico. Devido à presença de distúrbios neuropsicomotores normalmente presentes na Paralisia Cerebral, as crianças afetadas são mais vulneráveis às doenças bucais. Assim, os cuidados com a saúde bucal devem ser realizados o quanto antes pelos pais/responsáveis. Conclusão: Indivíduos com PC apresentam maior risco de desenvolver doenças bucais e programas de saúde bucal iniciados na primeira infância e direcionados às suas especificidades podem ser uma estratégia para minimizar as consequências que possam vir a acontecer.

Factors associated with edentulism in individuals with chronic kidney disease undergoing hemodialysis: a cross-sectional study / Fatores associados ao edentulismo em indivíduos com doença renal crônica em hemodiálise: um estudo transversa

Introduction: Chronic kidney disease (CKD) is a progres-sive condition characterized by structural or functional abnormalities of the kidney. CKD may be associated with several oral alterations, such as higher prevalence rate of dental caries, periodontal disease, xerostomia, candidiasis and burning mouth. The aim of the study was to identify risk factors associated with edentulism in adults with CKD undergoing hemodialysis. Methods: A cross-sec-tional study was conducted with 650 individuals aged 18 to 90 years undergoing hemodialysis in southeastern Brazil. Oral clinical examination and administration of a questionnaire addressing demographic characteristics and dental history were performed. The study received approval from the Human Research Ethics Committee of UFMG. Findings: A total of 183 participants were eden-tulous (28.2%). Individuals with less schooling (OR = 3.99; 95% CI: 2.34-6.79), those who had not been to a dentist in the previous six months (OR = 2.49; 95% CI: 1.52-4.08), those who rated their own smile as excellent or good (OR = 2.00; 95% CI: 1.35-2.97) and those with some mucosal alteration (OR = 4.17; 95% CI: 2.83-6.13) had a greater chance of belonging to the edentulous group. Discussion: The present findings can contribute to the establishment of public health policies aimed at guiding dental care programs for individuals with chronic kidney disease that take into account the specific needs of this population. Conclusion: Edentulism was associated with low schooling, a lack of dental care in the previous six months, a positive self-perception of one's smile and alterations in the oral mucosa.

Introdução: A doença renal crônica (DRC) é uma condição caracterizada por anormalidades estruturais ou funcionais do rim. A DRC pode estar associada a diversas alterações bucais, como maior prevalência de cárie dentária, doença periodontal, xerostomia, candidíase e queimação bucal. O objetivo deste estudo foi identificar os fatores de risco associados ao edentulismo em indivíduos com DRC em tratamento com hemodiálise. Materiais e Métodos: Foi realizado um estudo transversal com 650 indivíduos de 18 a 90 anos em hemodiálise no sudeste do Brasil. Foi realizado exame clínico oral e aplicação de questionário abordando características demográficas e histórico odontológico. O estudo foi aprovado pelo Comitê de Ética em Pesquisa em Seres Humanos da UFMG. Resultados: Um total de 183 participantes eram edêntulos (28,2%). Indivíduos com menor escolaridade (OR = 3,99; IC 95%: 2,34-6,79), aqueles que não foram ao dentista nos últimos seis meses (OR = 2,49; IC 95%: 1,52-4,08), aqueles que avaliaram o seu próprio sorriso como excelente ou bom (OR = 2,00; IC 95%: 1,35-2,97) e aqueles com alguma alteração de mucosa (OR = 4,17; IC 95%: 2,83-6,13) tiveram maior chance de pertencer ao grupo de edêntulos. Discussão: Os presentes achados podem contribuir para o estabelecimento de políticas públicas de saúde voltadas a nortear programas de atenção odontológica à pessoa com doença renal crônica que atendam às necessidades específicas dessa população. Conclusão: O edentulismo esteve associado à baixa escolaridade, falta de atendimento odontológico nos últimos seis meses, sorriso autoavaliado positivo e alterações mucosas.

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

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.

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.

Integrality of care for hemodialysis patient in Brazil: an analysis of access to dental care / Integralidade do cuidado para pacientes brasileiros em hemodiálise: análise do 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.

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

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.

Características orofaciais de indivíduos com mucopolissacaridoses: um estudo transversal pareado / Orofacial characteristics of individuals with mucopolysaccharidosis: a paired cross-sectional study

As mucopolissacaridoses (MPS) caracterizam-se por um grupo de doenças genéticas metabólicas raras, com manifestações multissistêmicas progressivas. Acarretam diversas alterações físicas, motoras e intelectuais. Dentre as alterações físicas, muitas estão presentes na face e na cavidade bucal. Na área odontológica ainda são poucos os estudos dedicados à MPS, sendo insuficientes as informações sobre as características orofaciais presentes nas pessoas acometidas por essa anomalia. O presente estudo objetivou identificar as características orofaciais de indivíduos com MPS e comparar com um grupo de indivíduos sem MPS. Foi realizado um estudo observacional transversal com uma amostra pareada de 14 indivíduos com MPS e 28 sem MPS, na faixa etária de 5 a 26 anos, pareados por sexo e idade. Os participantes com MPS e sem MPS são atendidos em dois hospitais públicos de Belo Horizonte, região sudeste do Brasil. Em ambos os grupos foi realizada a análise facial e a análise cefalométrica (medidas angulares e lineares). Além disso, também foram identificadas a presença ou ausência de alterações dentárias e maxilomandibulares por meio de radiografias panorâmicas. Previamente ao estudo principal, a examinadora passou pelo processo de calibração prática. Foram obtidos valores kappa entre 0,76 e 0,98. Para as variáveis cefalométricas, não foram observados erros sistemáticos significativos (p> 0,05). Os erros aleatórios para as medidas lineares e angulares foram baixos. A análise estatística entre os grupos foi realizada por meio do teste qui-quadrado e teste t independente. O estudo foi aprovado pelo Comitê de Ética em Pesquisa da Universidade Federal de Minas Gerais. A média de idade dos 42 participantes foi de 13,9 anos (± 7,2). Em relação às alterações faciais e cefalométricas, observou-se que quando comparados com indivíduos sem MPS, a maioria dos indivíduos com MPS apresentou perfil dolicofacial, alteração nas proporções faciais, com aumento da altura facial anterior inferior (AFAI) e incompetência labial (todos p<0,05). Seis medidas angulares foram significativamente maiores entre os indivíduos com MPS: 1s.Na, 1s.NB, FMA, IMPA, AFI e Po.Or_Go.Me; todos p <0,05. Duas medidas angulares apresentaram-se significativamente reduzidas nos indivíduos com MPS: 1s.1i, Ba.N-Ptm.Gn; p <0,05. Quatro medidas lineares apresentaram-se aumentadas entre os indivíduos com MPS: 1s-NA, 1i-NB, S-UL e S-LL; todos p<0,05. E, cinco medidas lineares (PogN-Perp, Co-A, Co-Gn, Nfa-Nfp e overbite; todos p<0,05) apresentaram-se reduzidas entre os indivíduos com MPS. Em relação à presença/ausência de alterações dentárias e maxilomandibulares, observou-se que a presença de dentes supranumerários (p=0,003); dentes conóides (p = 0,009); taurodontismo (p<0,001); dentes impactados (p<0,001); dentes semi-inclusos (p=0,040); dentes inclusos (p=0,013); e dilaceração radicular (p=0,047) foi estatisticamente mais frequente nos indivíduos com MPS quando comparados aos sem MPS. Além disso, a presença de rarefação óssea/ lesão de furca óssea (p=0,032), hipoplasia condilar (p<0,001), lesões ósseas radiolúcidas (p=0,001) e cistos dentígeros (p=0,001) também foram mais frequentes em indivíduos com MPS. Conclui-se que a maioria dos indivíduos com MPS apresentou perfil dolicofacial com aumento da altura facial anteroinferior. Os indivíduos com MPS foram identificados com incisivos superiores e inferiores vestibularizados, espaço nasofaríngeo reduzido e overbite reduzido. A presença de alterações dentárias e maxilomandibulares também foram frequentes nos indivíduos com MPS, ressaltando assim a importância do conhecimento a cerca das manifestações clínicas orais e faciais que podem ser herdadas através da MPS, a fim de se realizar um atendimento odontológico preventivo e eficaz.

Mucopolysaccharidosis (MPS) are characterized as a group of rare metabolic genetic diseases, with progressive multisystemic manifestations. There are several physical, motor and intellectual alterations. Among the physical alterations, many are presented in the face and oral cavity. In the the area of dentistry, there are still few studies dedicated to MPS, with insufficient information about the orofacial characteristics presented in individuals affected by this anomaly. Therefore, the aim of the present study was to evaluate the orofacial features of individuals with Mucopolysaccharidosis (MPS) and to compare the characteristics of those individuals with a group of non-MPS individuals. A cross-sectional study was carried out with a paired sample of 14 MPS individuals and 28 non-MPS individuals between five and 26 years old matched for sex and age. Participants with and without MPS are patients attended at two public hospitals in Belo Horizonte, southeastern region of Brazil. Facial and cephalometric analysis (angular and linear measurements) was performed for both groups. Besides that, the presence/absence of dental and maxillomandibular alterations were assessed through panoramic radiographs. Prior to the main study, the examiner went through practical calibration process. Kappa scores for categorical variables obtained were 0.76 to 0.98. For the cephalometric variables, no systematic errors were observed (p>0.05). Random errors for linear and angular measurements were low. Statistical analysis between groups was performed by means of chi-square test and independent t test. The study was approved by the Research Ethics Committee of the Universidade Federal de Minas Gerais. The mean age of the 42 participants was 13.9 years (±7.2). Regarding facial and cephalometric measures, it was observed that when compared to non-MPS individuals, most MPS individuals were dolichofacial, with facial proportions alterations, increased ALFH, and no passive lip sealing (all p<0.05). Six angular measurements (1s.Na, 1s.NB, FMA, IMPA, AFI, and Po.Or_Go.Me; all p<0.05) were significantly increased among MPS individuals. Two angular measurements (1s.1i, Ba.N-Ptm.Gn; all p<0.05) were significantly decreased among MPS individuals. Four linear measurements were significantly increased among MPS individuals (1s-NA, 1i-NB, S-UL and S-LL; all p<0.05). Five linear measurements (PogN-Perp, Co-A, Co-Gn, Nfa-Nfp and overbite; all p<0.05) were significantly decreased among MPS individuals. It can be concluded that, most MPS individuals were dolichofacial with increased anterior lower facial height. MPS individuals also present pro-inclined upper and lower incisors, reduced nasopharyngeal space and reduced overbite. The presence of dental and maxillomandibular alterations were also more frequent in MPS individuals, highlighting the importance of the knowledge about the oral and facial clinical manifestations that can be inherited with the MPS disease, in order to provide preventive and effective dental care.