Oral manifestations in children with mucopolysaccharidosis / Manifestações orais de mucopolissacaridose em crianças

BACKGROUND: Patients with mucopolysaccharidosis have several changes of the stomatognathic complex, representing a challenge for dentists. OBJECTIVE: The study aimed to evaluate and characterize oral health in patients with mucopolysaccharidosis in a reference center of Portugal. METHOD: The sample consisted of twelve participants with mucopolysaccharidosis followed in Metabolic Diseases Unit of the S. João Hospital Centre and twelve healthy participants followed at Faculty of Dental Medicine, University of Porto. The clinical oral evaluation was performed by a complete extra-oral and intra-oral examination to assess the presence of oral pathologies, gingival index and occlusion status. RESULTS: Mucoplysaccaridosis patients and controls presented similar age ranges and sex distribution. In comparison to controls, children with mucopolysaccharidosis presented a higher prevalence of tooth decay, gingival bleeding, macroglossia, dental hypoplasia, lingual interposition, delayed tooth eruption, anterior open bite, right and left posterior cross-bite, limitation of mouth opening, alteration on the size and shape of the teeth, diastemata and maxillary compression. CONCLUSIONS: Patients with mucopolysaccharidosis have a high prevalence eruption delay, teeth morphology alterations, occlusal problems, dental caries and bleeding gums, highlighting the need of oral health care providers to improve diagnostics and preventive protocols to overcome the factors that limit the oral health of these patients and promote together with parent/caregiver efficient oral care strategies.

INFORMAÇÕES GERAIS: Os pacientes com mucopolissacaridose apresentam diversas alterações do complexo estomatognático, representando um desafio para os médicos dentistas. OBJETIVO: O estudo pretendeu avaliar e caracterizar a saúde oral em pacientes com mucopolissacaridose num centro de referência em Portugal. MÉTODO: A amostra foi constituída por doze pacientes com mucopolissacaridose (MPS) seguidos na Unidade de Doenças Metabólicas do Centro Hospitalar de São João e doze participantes saudáveis seguidos na Faculdade de Medicina Dentária da Universidade do Porto. A avaliação clínica oral consistiu num completo exame extra-oral e intra-oral para avaliação de patologias orais, índice gengival e perfil oclusal. RESULTADOS: Pacientes com MPS e controlos apresentaram médias similares de idade e de distribuição de género. Em comparação com os controlos, crianças com mucopolissacaridose apresentam maior prevalência de dentes cariados, sangramento gengival, macroglossia, hipoplasia dentária, interposição lingual, erupção dentária atrasada, mordida aberta anterior, mordida cruzada posterior direita e esquerda, limitação da abertura da boca, alteração do tamanho e forma dentária, diastemas e compressão maxilar. CONCLUSÃO: Pacientes com mucopolissacaridose apresentam maior prevalência de erupção dentária atrasada, alterações morfológicas dentárias, problemas oclusais, cáries dentárias e gengivas inflamadas, reforçando a necessidade de prestadores de saúde oral para melhorar diagnósticos e protocolos preventivos para ultrapassar os fatores que limitam a saúde oral destes pacientes e promover em conjunto com pais/cuidadores estratégias de saúde oral eficientes.

Dental Neglect, one type of child abuse - Narrative review / Negligência dentária, uma modalidade de abuso infantil - revisão narrativa

Dental neglect is defined as an intentional failure of the caretaker in the pursuit and continuity of necessary treatment to achieve an oral health that allows adequate function, free from pain and infection. We intend to clarify, according to the current scientific literature, the clinical indications for the identification of dental neglect in children. After the search in databases and search engines with the keywords "child", "dental" and "neglect", between 1996 and 2016, the results revealed that identifying this condition requires not only a detailed clinical history and clinical data that clearly show the lack of oral health care but also investigating the social and parental determinants in which the child is involved. Thus, inserting the dentist in teams of analysis in case of suspicion proves to be indispensable for the detection of this and other situations of abuse.

A negligência dentária é definida como a falta intencional do responsável pela criança na procura e manutenção do tratamento necessário para garantir um nível de saúde oral essencial para a função adequada, livre de dor e infeção. Neste sentido, pretendemos esclarecer, de acordo com a literatura científica atual, os indícios clínicos para a identificação de negligência dentária em crianças. Após pesquisa em bases de dados e motores de busca, com as palavras-chave "child", "dental", "neglect", entre 1996 e 2016, os resultados revelaram que a identificação desta condição requer, não só a obtenção de uma história clínica detalhada e de dados clínicos que evidenciem claramente a falta de cuidados na saúde oral como também a investigação dos determinantes sociais e parentais em que a criança se insere. Assim, inserir o médico dentista nas equipas de análise nos casos de suspeita revelou-se indispensável para o despiste desta e outras situações de abuso.

Hyposalivation, acidic saliva, decayed teeth and oral yeast prevalence in children with mucopolysaccharidosis / Hiposalivação, saliva acídica, dentes cariados e prevalência oral de levedura em crianças com mucopolisacaridose

OBJECTIVE: Patients with mucopolysaccharidosis present several alterations of the stomatognathic complex, however, no data is available on saliva biochemistry and yeast colonization. The aim of the study was to evaluate caries experience as well as saliva biochemistry and microbiology parameters in patients with mucopolysaccharidosis. METHOD: The sample consisted of twelve participants with mucopolysaccharidosis followed in the Metabolic Disease Unit of the Centro Hospitalar de S. João and twelve healthy participants followed at the Faculty of Dental Medicine, University of Porto. To all participants, Decayed, Missing, Filled Teeth (DMFT) index was evaluated. In addition, saliva was collected to evaluate biochemical parameters (flow rate, pH, sodium, potassium, chloride, calcium, phosphate, α-amylase and IgA) and the microbiological profile (total microorganisms, mutans streptococci and yeasts) of all participants. RESULTS: In comparison to controls, the mucoplysacharidosis patients presented a higher prevalence of decayed teeth, lower salivary flow and pH values. They also presented also lower calcium and higher phosphate ions in saliva. No differences were found between groups regarding oral microbial load for total microorganisms, mutans streptococci and yeasts as well as oral prevalence of mutans streptococci. However, MPS patients presented higher prevalence of oral Candida in comparison to controls. CONCLUSION: The higher prevalence of decayed teeth and higher oral yeast colonization in MPS patients may be related to the lower saliva calcium concentration, pH and flow.

OBJETIVO: Pacientes portadores de mucopolissacaridose apresentam várias alterações do complexo estomatognático; no entanto, não existem dados disponíveis sobre a bioquímica da saliva e ou sobre a colonização por fungos. O objetivo deste estudo foi avaliar a prevalência de cárie dentária bem como parâmetros bioquímicos e microbiológicos em pacientes com mucopolissacaridose. MÉTODOS: A amostra foi constituída por doze participantes com mucopolissacaridose, acompanhados na Unidade de Doenças Metabólicas do Centro Hospitalar de S. João do Porto e por doze participantes saudáveis acompanhados na Faculdade de Medicina Dentária da Universidade do Porto. Para todos os participantes, o índice de dentes cariados, perdidos ou obturados foi avaliado. Além disso, foram recolhidas amostras de saliva de todos os participantes para avaliar os parâmetros bioquímicos (fluxo salivar, pH, sódio, potássio, cloreto, cálcio, fosfato, α-amilase e IgA) e microorganismos tais como Streptococcus mutans e leveduras. RESULTADOS: Em comparação aos controles, os pacientes com MPS apresentam maior prevalência de dentes cariados assim como fluxo salivar e pH reduzido. Os pacientes com MPS apresentaram também taxas menores de íons de cálcio e maiores de íons de fosfato. Não foram encontradas diferenças entre os grupos quanto à carga microbiana oral por microrganismos totais, Streptococcus mutans e leveduras, bem como quanto à prevalência oral de Streptococcus mutans. No entanto, os pacientes com MPS apresentaram maior prevalência de candidíase oral em comparação com os controlos. CONCLUSÃO: A maior prevalência de dentes cariados e a maior colonização oral por leveduras em pacientes com MPS pode estar relacionada com a baixa concentração de cálcio salivar, com o pH ácido e com a hiposalivação.

Conscious sedation vs general anesthesia in pediatric dentistry: a review / Sedação consciente vs anestesia geral em pediatria odontológica: uma revisão

INTRODUCTION: Performing dental treatments on pediatric patients who present behavioral problems is usually a great clinical challenge. Depending on the patients’ emotional maturity and their physical, psychological and mental skills, the usual behavior control techniques may not offer adequate efficacy and safety when dental procedures are being carried out. In these circumstances, alternative and more invasive methods such as conscious sedation and general anesthesia may become necessary. OBJECTIVE: To compare the indications and contraindications of conscious sedation and general anesthesia in pediatric dentistry. METHOD: Literature research undertaken through the MEDLINE/Pubmed, Science Direct and B-on search engines between April and June 2013 using a 5 year time frame and the following keywords: conscious sedation, general anesthesia, pediatric dentistry and guidelines. RESULTS: Conscious sedation is recommended to anxious patients who have dental and needle phobia, to patients that present an increased vomiting reflex and also to patients with special needs but capable of communicating. General anesthesia is indicated for non-cooperating patients under the age of four, in mouth breathers, in children ASA III or higher and in pediatric patients who do not communicate. CONCLUSIONS: Each technique must be selected according to the characteristics showed by patient. Although conscious sedation and general anesthesia introduce benefits to pediatric dentistry, they should not be regarded as priority intervention techniques for behavior control. The pediatric dentist’s previous knowledge of these techniques provides a better level of dental care quality as well as more safety and less discomfort for their patients.

RESUMO INTRODUÇÃO: A execução de tratamentos odontológicos em pacientes pediátricos que apresentam alterações comportamentais constitui frequentemente um grande desafio clínico para o odontopediatra. Dependendo da maturidade emocional dos pacientes e de suas habilidades físicas, psicológicas e mentais, as técnicas de controle de comportamento usuais podem não oferecer a eficácia e a segurança adequadas à realização dos procedimentos dentários. Nestas circunstâncias, podem ser necessários métodos alternativos e mais invasivos, como a sedação consciente e a anestesia geral. Neste trabalho comparam-se as indicações e contra-indicações de sedação consciente e anestesia geral em odontopediatria. MÉTODO: A revisão da literatura foi realizada através dos motores de pesquisa MEDLINE/PubMed, Science Direct e B-ON, entre abril e junho de 2013, com uma limitação temporal de cinco anos e as seguintes palavras-chave: sedação consciente, a anestesia geral, odontopediatria e diretrizes. RESULTADOS: A sedação consciente é recomendada para pacientes ansiosos, com "medo de dentista e de agulhas", para pacientes que apresentam um maior reflexo do vómito e também para pacientes com necessidades especiais, mas capazes de comunicação. Por outro lado, a anestesia geral é recomendada para pacientes com idade inferior a quatro anos que não colaboram, para respiradores orais, para crianças ASA III ou superior e para crianças que não se comunicam. CONCLUSÕES: Cada técnica deve ser escolhida de acordo com as características do paciente. Apesar da anestesia geral e da sedação consciente introduzirem benefícios no atendimento dentário do paciente infantil, estas não devem ser consideradas como técnicas de intervenção prioritária no controle comportamental. O conhecimento prévio das indicações e contraindicações destas técnicas permitem ao odontopediatra proporcionar um maior nível de qualidade no atendimento dentário, assegurando a maior segurança e o menor desconforto para os seus pacientes jovens.

Comparative Study of the Prevalence of Occlusal Anomalies in Down Syndrome Children and Their Siblings.

Aims: The aim of this study was to characterize occlusal anomalies in a Portuguese population with Down Syndrome (DS) and to compare its distribution with that of their siblings. Study Design: A sibling-matched, population-based and cross-sectional survey was performed. Methodology: This study involved 132 children with DS and 84 of their siblings, aged 2 to 26 years. Data were gathered through the use of a complete questionnaire and clinical observation. Data analysis was performed by using SPSS® v.20.0 and any p-value <.05 was considered significant. Results: Seventy six percent of the DS children and 72% of their siblings showed class I of Angle. There were no statistically significant differences between both groups regarding class I (P=.60). Only 4% of the DS children presented class II versus 22% of the siblings (P=.001). Twenty percent of the DS children and 6% of the siblings presented class III (P=.008). The DS group presented occlusal anomalies more frequently, especially posterior crossbite (P<.001), anterior open bite (P=.005) and anterior crossbite (P=.001). Deep overbite was only found in the siblings group (P=.001). Conclusions: The results of this study suggest that children with Down syndrome have more occlusal anomalies than general population.

Prevalence and distribution of tooth agenesis in a pediatric population:a radiographic study / Prevalência e distribuição de agenesias dentárias em pacientes pediátricos: um estudo radiográfico

Objective: The purpose of this study was to assess the prevalence of hypodontia and its distribution by sex, arch and quadrant, through the analysis of panoramic radiographs from the pediatric population of the Faculty of Dentistry of the University of Oporto, in Portugal. Methods: Panoramic radiographs of 1,438 patients of the Faculty of Dentistry of the University of Oporto (714 boys and 724 girls between the ages of 6 and 15) were evaluated to determine the occurrence and distribution of tooth agenesis. Chi-square and Fisher?s Exact Tests were performed. The level of significance was set at 5%. Results: The prevalence of hypodontia was 8% (excluding the third molar). Hypodontia was found in the mandible of 74 patients (64.3%). The most commonly missing teeth were the mandibular second premolars (28.6%). The maxilla had 52.3% of the dental agenesis detected. Similarity in the distribution of missing teeth between left and right sides of the dental arches was also detected. The prevalence of oligodontia was 0.42% and the average number of missing teeth per affected child was 2.31. There were no statistically significant differences between sexes. Conclusion: In a pediatric population of 1,438 patients, the prevalence of hypodontia was 8% (excluding the third molar). The results are consistent with those previously described in different populations.

Objetivo: Realizar um estudo epidemiológico sobre as agenesias dentárias, através da análise radiográfica de uma população pediátrica da Faculdade de Medicina Dentária da Universidade do Porto, definindo a sua prevalência e conhecendo a sua distribuição por sexo, arcada e quadrante. Métodos: As radiografias panorâmicas de 1.438 pacientes da Faculdade de Medicina Dentária da Universidade do Porto (714 do sexo masculino e 724 do sexo feminino, de idades compreendidas entre os 6 e os 15 anos) foram analisadas para determinar a ocorrência e distribuição de agenesias dentárias. A análise estatística foi realizada através da aplicação do Teste do Qui-quadrado de independência e do Teste Exato de Fisher, utilizando o nível de significância de 5%. Resultados: A prevalência de hipodontia foi de 8,0% (excluindo o 3º molar). Foram identificados 74 pacientes (64,3%) com agenesias na mandíbula. Ospré-molares inferiores foram os dentes com maior frequência de agenesia (28,6%). A maxila continha 52,3% das agenesias detetadas. Não foi detetada diferença estatisticamente significativa entre o lado esquerdo e direito das arcadas. A prevalência de oligodontia foi de 0,42% e obteve-se uma média de 2,31 dentes ausentes por paciente afetado. Não se encontraram diferenças estatisticamente significativas na variávelsexo. Conclusão: Numa população de 1438 pacientes pediátricos, 8,0% apresentaram agenesias dentárias (excluindo o 3º molar). Os resultados obtidos enquadram-se nos anteriormente descritos em diferentes populações.

Reduced salivary flow and colonization by mutans streptococci in children with Down syndrome

OBJECTIVES: Although individuals with Down syndrome have considerable oral disease, the prevalence of dental caries in this group is low. The present study aimed to compare known risk factors for dental caries development in children with Down syndrome and a matched population (siblings). In both populations, the number of acidogenic microorganisms, such as mutans streptococci, lactobacilli and Candida species, and the paraffin-stimulated pH, flow rate and IgA concentration in whole saliva were evaluated and compared. METHOD: Saliva was collected, and the caries index was evaluated in 45 sibling pairs aged between 6 and 18 years old. The salivary IgA concentration was determined by immunoturbidimetry. Salivary mutans streptococci, lactobacilli and Candida species were quantified on mitis salivarius agar containing bacitracin and 20% sucrose, rogosa agar supplemented with glacial acetic acid and sabouraud agar supplemented with chloramphenicol, respectively. RESULTS: Down syndrome children had a higher caries-free rate (p<0.05) and lower salivary mutans streptococci counts (p<0.03) compared to their siblings. Similar numbers of lactobacilli and Candida species were found in both groups. Salivary flow rates were 36% lower in Down syndrome children compared to their siblings (p<0.05). The salivary pH did not differ between Down syndrome children and controls. The Down syndrome children had an IgA secretion rate 29% lower than that of their siblings, but this difference was not statistically significant. CONCLUSIONS: In conclusion, the lower number of mutans streptococci in the saliva may be one of the factors contributing to the lower caries rate observed in Down syndrome children, despite evidence of hyposalivation.

Caries in Portuguese children with Down syndrome

OBJECTIVES: Oral health in Down syndrome children has some peculiar aspects that must be considered in the follow-up of these patients. This study focuses on characterizing the environmental and host factors associated with dental caries in Portuguese children with and without Down syndrome. METHODS: A sibling-matched, population-based, cross-sectional survey was performed. RESULTS: Down syndrome children presented a significantly greater percentage of children without caries, 78 percent vs. 58 percent of non-Down syndrome siblings. This difference in the DMFT index (number of decayed, missing and filled teeth) essentially reflects data obtained from treated teeth, for which 91 percent of children with Down syndrome had never had a tooth treated vs. 67 percent of siblings. This result was statistically significant, whereas results for decayed and lost teeth did not differ between Down syndrome children and their unaffected siblings. Additionally, in Down syndrome children, a delayed eruption of the second molar occurs. Down syndrome children and their siblings have similar oral hygiene habits, but a higher percentage of Down syndrome children visit a dentist before the age of three years, in comparison to their siblings. Bruxism was also more common in Down syndrome children compared to their siblings. CONCLUSIONS: Our results show that Portuguese children with Down syndrome have lower caries rates than children without Down syndrome. This reduced prevalence may be associated with the parents' greater concern about oral health care in Down syndrome children, resulting in their taking them sooner to visit a dentist, as well as to a higher bruxism prevalence and delayed tooth eruption.