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.

Low prevalence of oral protozoa colonization in Portuguese healthy and end-stage renal disease patients undergoing peritoneal dialysis / Baja prevalencia de la colonización oral de protozoos en Portugueses saludables y o pacientes renales del último estadio de enfermedad y que necesitan de diálisis del peritoneo

Infections are a major complication in end-stage renal disease (ESRD) patients undergoing peritoneal dialysis (PD). Because the oral cavity may act as a source of systemic pathogens, some authors advocated specific measures when these patients are submitted to oral interventions, such as the administration of prophylactic antibiotics. Oral protozoa colonization may vary significantly with geographic distribution and to our knowledge no studies were performed in Portugal. The aim of the present study was to evaluate protozoa colonization in the saliva of ESRD patients undergoing PD and of their family members, living in the north of Portugal. Saliva was collected from 39 PD patients with a mean time on PD therapy of 12.7 - 15.9 months, and from 18 healthy volunteers (ESRD family members) for microscopic evaluation of protozoa by Lugols’ direct smear and specific staining techniques (Giemsa, Trichrome and Kinyoun). After the analysis of 456 smears obtained from 57 participants, only one PD patient (2.6%) presented an amoeba trophozoite in saliva. In conclusion, very low oral protozoa colonization was found, both on PD patients and family controls, suggesting that the oral protozoa colonization of Portuguese population is low and not significantly modified by the presence of end-stage chronic kidney disease. Further studies are required to address this issue.

Las infecciones son la principal complicación en pacientes renales del último estadio (ESRD) y que necesitan de diálisis del peritoneo (PD). Como la cavidad oral puede funcionar como una fuente de patógenos sistémicos, algunos autores indican medidas específicas cuando esos pacientes son sometidos a intervenciones orales, como la administración de antibióticos profilácticos. La colonización oral puede variar significativamente con la distribución geográfica. Según nuestros conocimientos, no han sido realizados estudios similares en Portugal. El principal objetivo fue evaluar la colonización de protozoos en saliva de pacientes ESRD del Norte de Portugal que hacían PD y, también, de sus familiares. Muestras de saliva fueron recogidas de 39 pacientes PD, con tiempo medio de terapia de PD de 12,7-15,9 meses y, también de 18 voluntarios saludables (familiares de ESRD). Las mismas utilizadas para evaluación microscópica de protozoos en laminas con lugol y tinciones especificas (Giemsa, Trichrome and Kinyoun). Después del análisis de 456 laminas, obtenidas de los 57 participantes, solamente en un paciente PD (2.6%) se observó un trofozoíto del ameba. En conclusión, se encontró una baja prevalencia de colonización oral de protozoos en el grupo estudiado. Así, la colonización oral de la población Portuguesa por protozoos es baja y no se cambia con la evolución de la enfermedad. Para mejor analizar esta situación, futuros estudios son necesarios.

The biochemistry of saliva throughout pregnancy / O efeito da gravidez sobre a bioquímica salivar

BACKGROUND & OBJECTIVE: Sialometric and sialochemical analyses during pregnancy are not consistent, and frequently contradictory in terms of salivary flow rate, pH, and concentration of calcium, phosphorous, sodium, potassium, chloride, glucose and α-amylase. We, therefore, measured the evolution of these parameters throughout pregnancy. METHOD: A cross-sectional study compared sialometric and sialochemical analyses of 30 pregnant women vs. 30 age-matched non-pregnant women, and a longitudinal study evaluated the pregnant women in the first and third trimester of pregnancy. RESULTS: Pregnant women presented acidic non-stimulated saliva, but neutral stimulated saliva pH, and no relevant changes in salivary flow rate. Scialochemical analysis showed decreased calcium levels, increased phosphate levels, and a progressive decrease in glucose levels throughout pregnancy. CONCLUSION: Pregnancy significantly changes the oral biochemical milieu, creating a favorable environment for the development of oral pathology, in particular, dental caries.

OBJETIVO: Os achados relativos às análises sialométricas e sialoquímicas durante a gravidez não são consistentes, e por vezes são mesmo contraditórias. Assim, fizemos uma revisão da literatura e comparamos os níveis salivares de cálcio, fósforo, sódio, potássio, cloreto, glucose, α-amilase, pH e a taxa de fluxo salivar entre mulheres grávidas e não grávidas, bem como, avaliamos a evolução desses parâmetros ao longo da gravidez. MÉTODO: Realizamos um estudo transversal comparando a bioquímica salivar de um grupo inicial de 30 mulheres grávidas com um grupo inicial de 30 mulheres não gestantes da mesma idade seguido de um estudo longitudinal avaliando as mulheres grávidas no primeiro e terceiro trimestre de gravidez. RESULTADO: As mulheres grávidas apresentaram um pH da saliva não estimulada ácido, mas um pH da saliva estimulada neutro, assim como diminuição dos níveis salivares de cálcio, aumento dos níveis salivares de fosfato, e uma diminuição progressiva nos níveis de glicose na saliva ao longo da gravidez. CONCLUSÃO: A gravidez muda significativamente o ambiente bioquímico oral, criando condições favoráveis para o desenvolvimento de patologia oral, em particular da cárie dentária.

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.

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.