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Cerebellofaciodental syndrome is characterized by facial dysmorphisms, intellectual disability, cerebellar hypoplasia, and dental anomalies. It is an autosomal-recessive condition described in 2015 caused by pathogenic variants in BRF1. Here, we report a Brazilian patient who faced a diagnostic challenge beginning at 11 months of age. Fortunately, whole-exome sequencing (WES) was performed, detecting the BRF1 variants NM_001519.3:c.1649delG:p.(Gly550Alafs*36) and c.421C>T:p.(Arg141Cys) in compound heterozygosity, thus finally achieving a diagnosis of cerebellofaciodental syndrome. The patient is currently 25 years old and is the oldest patient yet reported. The clinical report and a review of published cases are presented. Atlanto-occipital fusion, a reduced foramen magnum and basilar invagination leading to compression of the medulla-spinal cord transition are skeletal findings not reported in previous cases. The description of syndromes with dental findings shows that such anomalies can be an important clue to relevant differential diagnoses. The cooperation of groups from different international centers made possible the resolution of this and other cases and is one of the strategies to bring medical advances to developing countries, where many patients with rare diseases are difficult to diagnose definitively.
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Anomalías Múltiples/genética , Cerebelo/anomalías , Anomalías Craneofaciales/genética , Discapacidad Intelectual/genética , Atrofia Muscular/genética , Malformaciones del Sistema Nervioso/genética , Factores Asociados con la Proteína de Unión a TATA/genética , Anomalías Dentarias/genética , Anomalías Múltiples/diagnóstico por imagen , Anomalías Múltiples/fisiopatología , Adulto , Brasil/epidemiología , Cerebelo/diagnóstico por imagen , Cerebelo/fisiopatología , Niño , Preescolar , Anomalías Craneofaciales/diagnóstico por imagen , Anomalías Craneofaciales/fisiopatología , Discapacidades del Desarrollo/diagnóstico por imagen , Discapacidades del Desarrollo/genética , Discapacidades del Desarrollo/fisiopatología , Femenino , Predisposición Genética a la Enfermedad , Humanos , Lactante , Discapacidad Intelectual/diagnóstico por imagen , Discapacidad Intelectual/fisiopatología , Masculino , Atrofia Muscular/diagnóstico por imagen , Atrofia Muscular/fisiopatología , Malformaciones del Sistema Nervioso/diagnóstico por imagen , Malformaciones del Sistema Nervioso/fisiopatología , Anomalías Dentarias/diagnóstico por imagen , Anomalías Dentarias/fisiopatología , Secuenciación del ExomaRESUMEN
Identifying cell types and states remains a time-consuming, error-prone challenge for spatial biology. While deep learning is increasingly used, it is difficult to generalize due to variability at the level of cells, neighborhoods, and niches in health and disease. To address this, we developed TACIT, an unsupervised algorithm for cell annotation using predefined signatures that operates without training data. TACIT uses unbiased thresholding to distinguish positive cells from background, focusing on relevant markers to identify ambiguous cells in multiomic assays. Using five datasets (5,000,000-cells; 51-cell types) from three niches (brain, intestine, gland), TACIT outperformed existing unsupervised methods in accuracy and scalability. Integrating TACIT-identified cell types with a novel Shiny app revealed new phenotypes in two inflammatory gland diseases. Finally, using combined spatial transcriptomics and proteomics, we discovered under- and overrepresented immune cell types and states in regions of interest, suggesting multimodality is essential for translating spatial biology to clinical applications.
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OBJECTIVE: To identify oral characteristics found in children with liver disease in programming for liver transplantation. DATA SOURCE: The methodology was written according to PRISMA-ScR. We adopted the methodological framework and recommendations for this type of review by Arksey and O'Malley and the Joanna Briggs Institute. The protocol was registered in the Open Science Framework (https://doi.org/10.17605/OSF.IO/QCU4W). A systematic search (Medline/PubMed, Scopus, Web of Science, and ProQuest) was conducted to identify studies that met the inclusion criteria: systematic reviews; prospective clinical trials (parallel or crossover group designs); observational studies (cohort, case-control, and cross-sectional studies); clinical case series; and case reports evaluating children with liver disease in preparation for transplantation. The last search was conducted in July 2021, and no restrictions were imposed as to language or year of publication. Studies presenting mixed data with post-transplant evaluation, and studies evaluating not only liver transplantation but also other solid organs were excluded. Screening, inclusion, and data extraction were performed by two reviewers independently. A narrative synthesis was conducted to describe the findings of the study. DATA SYNTHESIS: The bibliographic search identified 830 references. A total of 21 articles were read in their entirety after the inclusion criteria assessment. Finally, after evaluating the exclusion criteria, only 3 studies were considered for the qualitative analysis. CONCLUSIONS: Children with liver disease in preparation for transplantation may present enamel defects, tooth pigmentation, caries, gingivitis, and opportunistic infections such as candidiasis.
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Hepatopatías , Trasplante de Hígado , Niño , Humanos , Estudios Transversales , Estudios ProspectivosRESUMEN
To establish the profile of patients who developed antiangiogenic agent-related osteonecrosis of the jaws, and identify the treatments currently used in dental management. We searched the PubMed®/Medline® and Scopus databases using the words "osteonecrosis AND antiangiogenic therapy", with the following inclusion criteria: articles published in English, case reports, available online, and for an unlimited period. Of the 209 articles retrieved, 18 were selected, for a total of 19 case reports, since one article included two cases that met the inclusion criteria for this study. Medication-related osteonecrosis of the jaws is characterized by exposure of necrotic bone in the oral cavity that does not heal over a period of 8 weeks in patients with no previous history of radiation therapy. Antiangiogenic drugs are indicated in the treatment of certain tumors, since they stop the formation of new blood vessels, controlling tumor growth and the chance of metastasis. Dental prevention is essential in patients who will be put on antiangiogenic agents, to minimize the risk for osteonecrosis.
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Inhibidores de la Angiogénesis/efectos adversos , Enfermedades Maxilomandibulares/inducido químicamente , Osteonecrosis/inducido químicamente , Femenino , Humanos , Masculino , Factores de RiesgoRESUMEN
This review aimed to analyze the scientific production on severity of oral mucositis as an adverse effect of chemotherapy. To this end, we performed a search at PubMed databases combining the keywords "oral mucositis" and "chemotherapy protocol". To describe the investigation, the following variables were considered: journal, year/place, study design, sample, protocol used and incidence of oral mucositis. A total of 547 articles were retrieved, of which 26 were selected. Out of these 26, only 2 reported severity of oral mucositis; the others only reported the presence of the condition. Protocols for treating different types of carcinoma were evaluated in 16 (61.53%) studies, for hematological malignancies in 6 (23.07%), and for hematopoietic stem cell transplantation in 4 (15.4%). Protocols for hematopoietic stem cell transplantation entail a high risk for oral mucositis, just as chemotherapy with cytarabine and high-dose 5-fluorouracil, alkylating agents and platinumbased compounds. To provide the best prevention and treatment for oral mucositis, it is essential to know the chemotherapy protocols used and their effects on the oral cavity.
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Antineoplásicos/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Estomatitis/inducido químicamente , HumanosRESUMEN
Abstract Objective: To identify oral characteristics found in children with liver disease in programming for liver transplantation. Data source: The methodology was written according to PRISMA-ScR. We adopted the methodological framework and recommendations for this type of review by Arksey and O'Malley and the Joanna Briggs Institute. The protocol was registered in the Open Science Framework (https://doi.org/10.17605/OSF.IO/QCU4W). A systematic search (Medline/PubMed, Scopus, Web of Science, and ProQuest) was conducted to identify studies that met the inclusion criteria: systematic reviews; prospective clinical trials (parallel or crossover group designs); observational studies (cohort, case-control, and cross-sectional studies); clinical case series; and case reports evaluating children with liver disease in preparation for transplantation. The last search was conducted in July 2021, and no restrictions were imposed as to language or year of publication. Studies presenting mixed data with post-transplant evaluation, and studies evaluating not only liver transplantation but also other solid organs were excluded. Screening, inclusion, and data extraction were performed by two reviewers independently. A narrative synthesis was conducted to describe the findings of the study. Data synthesis: The bibliographic search identified 830 references. A total of 21 articles were read in their entirety after the inclusion criteria assessment. Finally, after evaluating the exclusion criteria, only 3 studies were considered for the qualitative analysis. Conclusions: Children with liver disease in preparation for transplantation may present enamel defects, tooth pigmentation, caries, gingivitis, and opportunistic infections such as candidiasis.
RESUMO Objetivo: Identificar características bucais em crianças hepatopatas em programação para o transplante hepático. Fontes de dados: A metodologia foi descrita de acordo com o PRISMA-ScR. Adotamos a estrutura metodológica e recomendações para este tipo de revisão por Arksey e O'Malley e o Instituto Joanna Briggs. O protocolo foi registrado no Open Science Framework (https://doi.org/10.17605/OSF.IO/QCU4W). Uma pesquisa sistemática (Medline/PubMed, Scopus, Web of Science e ProQuest) foi conduzida para identificar estudos que preenchessem os critérios de inclusão: revisões sistemáticas; ensaios clínicos prospectivos (desenhos de grupos paralelos ou cruzados); estudos observacionais (coorte, caso-controle e estudos transversais); séries de casos clínicos; e relatos de casos que avaliam crianças com doenças hepáticas em preparação para o transplante. A última busca foi conduzida em julho de 2021, e não foram impostas restrições quanto ao idioma ou ano de publicação. Foram excluídos estudos que apresentavam dados mistos com avaliação pós-transplante e estudos que avaliavam não só o transplante de fígado, mas também de outros órgãos sólidos. O rastreio, inclusão e extração de dados foram realizados por dois revisores independentemente. Foi conduzida uma síntese narrativa para identificar os resultados do estudo. Síntese dos dados: A pesquisa bibliográfica identificou 830 referências. Foram lidos 21 artigos na íntegra após avaliação dos critérios de inclusão. Finalmente, após a avaliação dos critérios de exclusão, apenas três estudos foram considerados para análise. Conclusões Crianças com doença hepática em preparação para o transplante podem apresentar defeitos de esmalte, pigmentação dentária, cárie, gengivite além de infecções oportunistas como a candidíase.
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ABSTRACT During the state of immune vulnerability in hematopoietic stem cell transplantation (HSCT), the patient has an increased risk of developing a vast number of complications, including severe problems in the oral cavity. These situations require professional oral care to act in the diagnosis and treatment of these conditions, as well as to develop prevention protocols to minimize patient's complications. Oral mucositis, opportunistic infections, bleeding, specific microbiota, taste, and salivary alterations are complications that can occur during HSCT and interfere with various aspects, such as pain control, oral intake, nutrition, bacteremia and sepsis, days of hospitalization and morbidity. Several guidelines have been published to address the role of professional oral care during the HSCT, we describe a consensus regarding these recommendations.
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ABSTRACT The oral involvement in the Hematopoietic Stem Cell Transplantation is well described in the literature. The goal of the dental treatment and management of the oral lesions related to the HSCT is to reduce the harm caused by preexisting oral infection or even the worsening of oral acute/chronic GVHD and late effects. The aim of this guideline was to discuss the dental management of patients subjected to HSCT, considering three phases of the HSCT: pre-HSCT, acute phase, and late phase. The literature published from 2010 to 2020 was reviewed in order to identify dental interventions in this patient population. The selected papers were divided into three groups: pre-HSCT, acute and late, and were reviewed by the SBTMO Dental Committee's members. When necessary, an expertise opinion was considered for better translating the guideline recommendations to our population dental characteristics. This manuscript focused on the pre-HSCT dental management. The objective of the pre-HSCT dental management is to identify possible dental situations that On behalf of the Dental Committee of the Brazilian Society of Gene Therapy and Bone Marrow Transplantation (SBTMO) can worsening during the acute phase after the HSCT. Each guideline recommendations were made considering the Dentistry Specialties. The clinical consensus on dental management prior to HSCT provides professional health caregivers with clinical setting-specific information to help with the management of dental problems in patients to be subjected to HSCT.
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ABSTRACT Patients undergoing hematopoietic stem cell transplantation (HSCT) might present acute and late toxicities and the oral tissues are frequently affected. With the survival increasing, patients show late and long-term morbidities, and there is an important association between the general and the oral health. The first and second parts of this Consensus have showed the importance of the adequacy of oral health in the pre-HSCT, and the main alterations and oral care during the period of admission for HSCT. This third part aims to review specific themes of post-HSCT dental care, such as graft-versus-host disease (GVHD) and the pediatric patient. It also aims to review pertinent subjects, both during the HSCT period and post-HSCT, concerning quality of life, pain, cost-effectiveness, and remote care. Based on this review, it is evident the importance of the work of the dental surgeon (DS) in the follow-up and treatment of the HSCT patient, always collaborating with the whole multidisciplinary team.
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Cirurgiões-dentistas habilitados em práticas baseadas em evidências científicas e na aplicação de modelos de cuidados em saúde bucal bem-sucedidos trazem benefícios tanto à saúde individual quanto à coletiva. Este estudo traçou um panorama sobre cursos de Habilitação em Odontologia Hospitalar ofertados no Brasil após a regulamentação da Resolução 162/2015 pelo Conselho Federal de Odontologia (CFO), para auxiliar a escolha dos profissionais que pretendem se habilitar na área. O método de pesquisa foi a revisão integrativa da literatura, com análise quantitativa, exploratória e descritiva dos dados. O modelo de busca aplicado foi "habilitação em odontologia hospitalar", na base de dados Google, com uma única consulta em março de 2019. Dezessete (17) propostas foram encontradas. Seguiram para análise treze (13) propostas que informavam número de vagas ofertadas, periodicidade do curso, dias da semana em que ocorriam as atividades, carga horária total, conteúdo programático (teórico e prático) e local da prática. Falta de conteúdo programático (n=2) e de atualização (n=1) foram critérios de exclusão imediata. Na análise descritiva das propostas (n=10) houve exclusões por falta de definição da carga horária teórica e prática (n=4); informação sobre o conteúdo prático (n=5) e de identificação do local de desenvolvimento e carga horária mínima exigida para a prática. O estudo evidenciou que, à época da coleta de dados, apenas uma proposta atendia à legislação e à normativa exigidas pelo CFO (AU).
Dentists qualified on evidence-based practices and on the application of successful oral health care models are beneficial to individual and collective health. The current study presents an overview of Hospital Dentistry Qualification courses available in Brazil after the regulation of Resolution 162/2015 by the Conselho Federal de Odontologia (CFO - Brazil), which focuses on assisting the selection by professionals who intend to qualify in this field. Integrative literature review, with quantitative, exploratory and descriptive data analyses, was the research method adopted in the present study. The mesh "hospital dentistry qualification" was used in the single search conducted on Google database, in March 2019. Seventeen (17) proposals were found. Thirteen (13) proposals that included information about vacancies available for students, course periodicity, weekdays when the course took place, total workload, program content (theoretical and practical) and place of practice were selected for analysis. Lack of program content (n=2) and outdated information (n=1) were immediate exclusion criteria. The descriptive analysis applied to the proposals (n=10) has shown that courses were excluded due to lack of definition ofthe workload of theoretical and practical activities (n=4); as well as of information about the practical content of the course (n=5), the hospital where the practice would take place and the minimum workload required for the practice. One (01) qualification proposal was included in the research based on qualitative synthesis. The current study has shown that one proposal was in compliance with the legislation and regulations required by FCD, as well as that the presentation of the information available in the website about the proposal makes it hard for the target audience to make the option for the course that would best meet their needs (AU).
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Servicio Odontológico Hospitalario , Personal de Odontología en Hospital , Capacitación de Recursos Humanos en Salud , Necesidades y Demandas de Servicios de Salud , Hospitales , Redes de Comunicación de Computadores , Brasil/epidemiología , Salud Bucal , Epidemiología Descriptiva , Encuestas y Cuestionarios , Odontólogos , Estudios de Evaluación como AsuntoRESUMEN
ABSTRACT This review aimed to analyze the scientific production on severity of oral mucositis as an adverse effect of chemotherapy. To this end, we performed a search at PubMed databases combining the keywords "oral mucositis" and "chemotherapy protocol". To describe the investigation, the following variables were considered: journal, year/place, study design, sample, protocol used and incidence of oral mucositis. A total of 547 articles were retrieved, of which 26 were selected. Out of these 26, only 2 reported severity of oral mucositis; the others only reported the presence of the condition. Protocols for treating different types of carcinoma were evaluated in 16 (61.53%) studies, for hematological malignancies in 6 (23.07%), and for hematopoietic stem cell transplantation in 4 (15.4%). Protocols for hematopoietic stem cell transplantation entail a high risk for oral mucositis, just as chemotherapy with cytarabine and high-dose 5-fluorouracil, alkylating agents and platinumbased compounds. To provide the best prevention and treatment for oral mucositis, it is essential to know the chemotherapy protocols used and their effects on the oral cavity.
RESUMO Esta revisão teve como objetivo analisar a produção científica sobre a gravidade da mucosite oral como efeito adverso da quimioterapia. Para tal, nos bancos de dados do PubMed, foi realizada uma busca com a associação dos descritores "oral mucositis" com "chemotherapy protocol". Para descrição da investigação, foram consideradas como variáveis: periódico, ano/local, delineamento da pesquisa, amostra, protocolo utilizado e incidência de mucosite oral. Foram analisados 547 artigos e, destes, 26 foram selecionados. Destes 26, apenas 2 tinham como objetivo avaliar a gravidade de mucosite oral; nos outros, a mucosite oral foi apenas relatada. Protocolos para tratamento de diferentes tipos de carcinoma foram avaliados em 16 (61,53%) estudos, para neoplasias hematológicas, em 6 (23,07%), e para transplante de células tronco hematopoiéticas em 4 (15,4%). Protocolos para transplante de células tronco hematopoiéticas são de alto risco para o desenvolvimento de mucosite oral, da mesma forma que os quimioterápicos citarabina e 5-fluorouracil em altas doses, agentes alquilantes e compostos derivados da platina. A fim de oferecer prevenção e tratamento mais adequados para mucosite oral, é imprescindível que se conheçam os protocolos quimioterápicos utilizados e seus efeitos sobre a cavidade oral.
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Humanos , Estomatitis/inducido químicamente , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Antineoplásicos/efectos adversosRESUMEN
Objetivo: O objetivo deste artigo foi revisar os dados na literatura sobre possíveis mecanismos que estabeleçam uma relação entre o câncer oral e a doença periodontal. Material e Métodos: Foi realizada uma busca nas bases de dados Pubmed, Scopus e Web of Science por publicações até abril de 2016. Resultados: Os artigos analisados apontaram evidências que corroboram com o papel da doença periodontal na carcinogênese. Esta atuação baseia-se na liberação de mediadores inflamatórios e de periodontopatógenos das bolsas periodontais até sítios sadios através do sangue e da saliva, sendo capaz alterar o padrão epigenético do hospedeiro. Em consequência, esta alteração na expressão gênica pode inibir regiões relacionadas à supressão tumoral, crescimento celular, reparo do DNA, ligação intracelular, inibição de metástase, entre outros. Conclusão: É possível concluir que há bases biológicas para sustentar uma relação entre doença periodontal e o câncer oral, porém mais estudos são necessários para avaliar seu real impacto
Objective: The aim of this article was to review the extant literature about possible mechanisms for a relationship between periodontal disease and oral cancer. Material and Methods: A search was performed using PubMed, Scopus, and Web of Science for articles published through April 2016. The articles showed evidence supporting the role of periodontal disease in carcinogenesis. Results: The relationship between periodontal disease and oral cancer is based on the release of inflammatory mediators and periodontopathogens from periodontal pockets to healthy sites via the blood and saliva, thereby changing the host's epigenetic patterns. Consequently, these changes in gene expression can inhibit genetic regions related to tumor suppression, cell growth, DNA repair, intracellular binding, and inhibition of metastasis, among other processes. Conclusion: There is sufficient biological evidence to support the relationship between periodontal disease and oral cancer; however, more studies are needed to evaluate the impact of this relationship on patients
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Objetivo: realizar uma reflexão sobre a aplicação dos princípios bioéticos na atuação do cirurgião-dentista em âmbito hospitalar. Material e Métodos: uma revisão crítica da literatura foi realizada acessando-se as bases de dados PubMed, Scielo e Biblioteca Virtual em Saúde, utilizando-se os descritores "Bioética", "Equipe Hospitalar de Odontologia", "Ética Odontológica" e "Ética Institucional". Resultados: não foram encontrados estudos específicos sobre o tema proposto. Há escassez de trabalhos na literatura científica específicos sobre a aplicação dos princípios bioéticos ao dia a dia dos profissionais de Odontologia em âmbito hospitalar. Conclusão: a observância e o respeito aos pilares bioéticos da autonomia, justiça, beneficência e não maleficência devem estar incluídos nas atitudes do cirurgião-dentista e da equipe multiprofissional, minimizando os desconfortos ocasionados por problemas de ordem geral e bucal.
Objective: to reflect on the application of the basic principles of bioethics in the performance of the dentist in a hospital environment. Materials and Methods: a critical review of the scientific literature was performed by searching the PubMed, Scielo, and Virtual Health Library databases using the descriptors "Bioethics," "Dental Staff, Hospital," "Ethics, Dental" and "Ethics, Institutional." Results: there were no specific studies on the proposed theme. There was a lack of studies in the scientific literature on the specific application of the bioethical principles in the routine of dental professionals at hospitals. Conclusion: the observance and respect for the bioethical pillars of autonomy, justice, beneficence and non-maleficence must be included in the attitudes of the dentist and the multidisciplinary team, minimizing the discomforts caused by problems of general and oral disorders.
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INTRODUCTION: Hypophosphatemic rickets represents a group of heritable renal disorders of phosphate characterized by hypophosphatemia, normal or low serum 1,25 (OH)2 vitamin D and calcium levels. Hypophosphatemia is associated to interglobular dentine and an enlarged pulp chambers. AIM: Our goal was to verify the dental abnormalities and the oral health condition in these patients. MATERIAL AND METHODS: Prospective study of oral conditions in patients with Hypophosphatemic rickets. This report employed a simple method to be easily reproducible: oral clinical exam and radiographic evaluation. RESULTS: Fourteen patients were studied, 5 males, median age of 11 years (4 to 26). Occlusion defects (85,7%) and enamel hypoplasia (57,1%) were significant more frequently than dental abscesses (one patient). We observed enlarged pulp chambers in 43% of the patients and hypoplasia and dentin abnormalities in 14,3%. We could not detect a significant correlation between dental abnormalities and delayed treatment (p > 0,05). DMFT index for 6 to 12 years patients (n = 12) showed that the oral health is unsatisfactory (mean DMFT = 5). CONCLUSIONS: Patients with Hypophosphatemic Rickets frequently present dental alterations and these are not completely recovered with the treatment, unless dental abscess and they need a periodical oral examination.
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Hipoplasia del Esmalte Dental/diagnóstico por imagen , Oclusión Dental , Cavidad Pulpar/diagnóstico por imagen , Raquitismo Hipofosfatémico Familiar/diagnóstico por imagen , Salud Bucal/normas , Adolescente , Adulto , Distribución de Chi-Cuadrado , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios Prospectivos , Radiografía , Adulto JovenRESUMEN
Objetivo: Avaliar a efetividade das substâncias lubrificantes bucais Biotène Oral Balance® (Laclede do Brasil Produtos Médicos e Odontológicos Ltda, Cotia, Brasil) e Biotène Mouthwash® (Laclede do Brasil Produtos Médicos e Odontológicos Ltda, Cotia, Brasil) em indivíduos com epidermólise bolhosa. Métodos: Participaram deste estudo 10 indivíduos com epidermólise bolhosa, 7 do sexo feminino e 3 do masculino, com idades entre 7 a 30 anos. Foi aplicada a Escala Visual Analógica em dois momentos: pré-tratamento e um mês após o uso contínuo das substâncias descritas, e em três situações: higiene bucal, alimentação e procedimentos odontológicos. A comparação dos resultados entre os dois momentos em cada situação foi realizada pelo teste não-paramétrico de Wilcoxon, com nível de significância fixado em 5%. Resultados: Para a higiene bucal observou-se diferença significante entre os momentos pré-tratamento e uso contínuo das substâncias descritas (p=0,011), apresentando pré-tratamento valores da Escala Visual Analógica significantemente maiores com relação ao uso contínuo das substâncias descritas. Em relação à alimentação e procedimentos odontológicos não foram observados diferenças significantes nos momentos avaliados (p=0,180 e 0,672, respectivamente). Conclusão: Concluiu-se que o uso de substâncias lubrificantes orais em indivíduos com epidermólise bolhosa é efetivo na higienização oral, reduzindo a intensidade dolorosa durante o ato.
Objective: This study assessed the effectiveness of the oral lubricants Biotène Oral Balance® and Biotène Mouthwash® in individuals with epidermolysis bullosa. Methods: Ten individuals with epidermolysis bullosa participated in this study, 7 females and 3 males, aged 7 to 30 years. The Visual Analogue Scale was used on two occasions (before treatment (M1) and one month after regular use of the abovementioned substances (M2)) and in three situations (oral hygiene, diet and dental procedures). The non-parametric Wilcoxon test was used to compare the results between the two occasions with significance set at 5%. Results: Oral hygiene before and after treatment with either substance differed significantly (p=0.011). The values obtained by the Visual Analogue Scale were significantly higher before treatment. Diet and dental procedures did not differ significantly between occasions (p=0.180 and 0.672, respectively). Conclusion: Oral lubricants effectively reduced the pain experienced by individuals with epidermolysis bullosa during oral hygiene.
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Enfermedades de la Boca , Epidermólisis Ampollosa , Higiene BucalRESUMEN
INTRODUCTION: Hypophosphatemic rickets represents a group of heritable renal disorders of phosphate characterized by hypophosphatemia, normal or low serum 1,25 (OH)2 vitamin D and calcium levels. Hypophosphatemia is associated to interglobular dentine and an enlarged pulp chambers. AIM: Our goal was to verify the dental abnormalities and the oral health condition in these patients. MATERIAL AND METHODS: Prospective study of oral conditions in patients with Hypophosphatemic rickets. This report employed a simple method to be easily reproducible: oral clinical exam and radiographic evaluation. RESULTS: Fourteen patients were studied, 5 males, median age of 11years (4 to 26). Occlusion defects (85,7 percent) and enamel hypoplasia (57,1 percent) were significant more frequently than dental abscesses (one patient). We observed enlarged pulp chambers in 43 percent of the patients and hypoplasia and dentin abnormalities in 14,3 percent. We could not detect a significant correlation between dental abnormalities and delayed treatment (p>0,05). DMFT index for 6 to 12 years patients (n = 12) showed that the oral health is unsatisfactory (mean DMFT = 5). CONCLUSIONS: Patients with Hypophosphatemic Rickets frequently present dental alterations and these are not completely recovered with the treatment, unless dental abscess and they need a periodical oral examination.
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Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Masculino , Adulto Joven , Oclusión Dental , Hipoplasia del Esmalte Dental , Cavidad Pulpar , Raquitismo Hipofosfatémico Familiar , Salud Bucal/normas , Distribución de Chi-Cuadrado , Estudios ProspectivosRESUMEN
A epiderólise Bolhosa (EB) está associada a diversas manifestações orais, as quais podem envolver tecidos moles e duros intra-orais. As características e a extensão do envolvimento oral variam consideravelmente entre os tipo da doença. Nas formas brandas, a mucosa oral pode apresentar bolhas ocasionalmente e não há acometimento dos dentes, enquanto nas formas mais debilitantes, toda a mucosa oral é gravemente afetada, podendo haver alterações dentárias. O tratamento odontológico deve considerar os diversos aspectos e as limitações da doença, permitindo que esses pacientes beneficiem-se com a manutenção de uma dentição natural e saudável.
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Atención Odontológica , Epidermólisis Ampollosa , Mucosa Bucal/patología , Epidermólisis Ampollosa Distrófica , Epidermólisis Ampollosa de la Unión , Epidermólisis Ampollosa SimpleRESUMEN
O atendimento odontológico ao portador de alterações no sangue exige que o CD esteja atento aos riscos, converse com o paciente, busque a integração com outros profissionais e conheça os recursos disponíveis, como o selante de fibrina