Dental and Endodontic Management in a Patient with Familial X-Linked Hypophosphatemic Rickets

ABSTRACT: The term rickets refers to insufficient or retarded mineralization of the osteoide matrix. X-linked hypophosphatemic (XLH) rickets is a rare genetic disorder characterized by biochemical changes in bone mineralization due to inactivation of the phosphate regulating gene and primary defect of the osteoblasts. The aim of this article was to report a clinical case of XLH, its oral manifestations, periapical changes and dental management. A 31-year old woman female patient was referred to the school of dentistry with pain and sensitivity in the teeth. She had a childhood history of rickets, hypophosphatemia and alteration in Vitamin D. In the oral exam, enamel hypoplasia, microdontia, fistula, caries and periapical lesions and periodontal disease were diagnosed. The radiographic and tomographic exams exhibited the presence of periapical lesions involving various teeth with radiolucent images, suggestive of granuloma or periapical cysts. The treatme nt prioritized the urgency of eliminating pain and removing the foci of infection. Endodontic treatment began in the teeth that had fistula or periapical lesions and in parallel, oral hygiene guidance was provided and periodontal treatment was performed. There was an improvement in the clinical condition with reduction in inflammation and mobility of the teeth. Dentists and health professionals must evaluate the patient as a whole, considering the relations between systemic and oral health. Knowledge of systemic diseases associated with rickets and their characteristics is essential for making a correct oral diagnosis and planning the dental treatment.

RESUMEN: El término raquitismo se refiere a la mineralización insuficiente o retardada de la matriz osteoide. El raquitismo hipofosfatémico ligado al cromosoma X (XLH) es un trastorno genético caracterizado por cambios bioquímicos en la mineralización ósea debido a la inactivación del gen regulador del fosfato y al defecto primario de los osteoblastos. El objetivo de este artículo fue reportar un caso clínico de XLH, sus manifestaciones orales, cambios periapicales y manejo dental. La paciente, una mujer de 31 años, acudió a la Clínica de Semiología de la UFPR con dolor y sensibilidad en varios dientes. Tenía antecedentes infantiles de raquitismo, hipofosfatemia y alteración de la vitamina D. En el examen oral se diagnosticó hipoplasia del esmalte, microdoontia, fístula, caries y lesiones periapicales y enfermedad periodontal. Los exámenes radiográficos y tomográficos mostraron la presencia de lesiones periapicales en varios dientes con imágenes radiolúcidas, sugestivas de granuloma o quistes periapicales. El tratamiento priorizó la urgencia de eliminar el dolor y remover los focos de infección. Se inició tratamiento de endodoncia en los dientes que presentaban fístula o lesiones periapicales y paralelamente se brindó orientación de higiene oral y se realizó tratamiento periodontal. Hubo una mejoría en la condición clínica con reducción de la inflamación y movilidad de los dientes. Los odontólogos y profesionales de la salud deben evaluar al paciente como un todo, teniendo en cuenta las relaciones entre salud sistémica y oral. El conocimiento de las enfermedades sistémicas asociadas al raquitismo y sus características es fundamental para realizar un correcto diagnóstico oral y planificar el tratamiento odontológico.

Calcificaciones pulpares en pacientes con diabetes mellitus / Pulp calcifications in patients with diabetes mellitus

RESUMEN Introducción: Las células de la pulpa dental tienen la capacidad, ante estímulos locales y sistémicos, de activar a la osteopontina, formando calcificaciones pulpares, bloqueando los conductos radiculares y modificando la anatomía interna; de manera que se intensifican en personas con diabetes mellitus. Objetivo: Determinar si existe mayor presencia de calcificaciones a nivel de cámara pulpar en pacientes con diabetes mellitus versus pacientes sin diabetes mellitus. Métodos: Se realizó un estudio de casos y controles, analítico, transversal y retrospectivo. Se utilizaron los registros clínicos y las radiografías digitales periapicales de pacientes de las clínicas odontológicas de pregrado (Prótesis, Restauradora, Integral, Periodoncia) y de postgrado (Periodoncia e Implantología quirúrgica) de la Universidad Andrés Bello - Chile, entre los años 2017 y 2018. Se seleccionó una muestra de 617 dientes (45 pacientes) luego de ser aplicados los criterios de exclusión e inclusión en la base de datos clínica. La muestra completó dos grupos, grupo de casos (22 pacientes): dientes de pacientes con diabetes mellitus 228 dientes indemnes y el grupo de control (23 pacientes): dientes de pacientes sin diabetes mellitus 389 dientes indemnes. Se realizó una calibración previa entre los investigadores para la identificación y clasificación de calcificaciones pulpares durante el análisis radiográfico de estas. Se realizaron inferencias estadísticas de chi cuadrado para establecer significación de los resultados obtenidos. Resultados: Los dientes de 22 pacientes diabéticos presentaron más calcificaciones camerales que los dientes de 23 pacientes no diabéticos, para 50 por ciento de dientes calcificados en pacientes diabéticos (n=114) y 26 por ciento en pacientes no diabéticos (n=101); este resultado fue estadísticamente significativo (pvalor< 0,001). Conclusiones: Los pacientes con diabetes mellitus demostraron que son más propensos a formar calcificaciones camerales debido al aumento de glucosa presente en la sangre que activa a la osteopontina(AU)

ABSTRACT Introduction: Dental pulp cells have the capacity to respond to local and systemic stimuli by activating osteopontin, forming pulp calcifications, blocking root canals and modifying internal anatomy, in such a way that they intensify in people with diabetes mellitus. Objective: Determine whether there is a greater presence of calcifications in the pulp chamber of patients with diabetes mellitus vs. patients without diabetes mellitus. Methods: An analytical cross-sectional retrospective case-control study was conducted of medical records and digital periapical radiographs of patients attending undergraduate dental clinics (prosthesis, restoration, comprehensive, periodontics) and graduate dental clinics (periodontics and surgical implantology) of Andrés Bello University, Chile, in the period 2017-2018. A sample was selected of 617 teeth (45 patients) from the clinical database upon applying exclusion and inclusion criteria. The sample was divided into two groups: a case group, formed by 228 undamaged teeth from 22 patients with diabetes mellitus, and a control group, formed by 389 undamaged teeth from 23 patients without diabetes mellitus. Prior calibration was performed by the researchers to identify and classify the pulp calcifications during their radiographic analysis. Chi-squared statistical inferences were made to establish the significance of the results obtained. Results: The teeth from the 22 diabetic patients had more chamber calcifications than the teeth from the 23 nondiabetic patients, for 50 percent calcified teeth in diabetic patients (n= 114) and 26 percent in nondiabetic patients (n= 101). This result was statistically significant (p-value< 0.001). Conclusions: Patients with diabetes mellitus were found to be more prone to form chamber calcifications, due to the increase in glucose present in the blood which activates osteopontin(AU)

Relationship between dental calcification and skeletal maturation in a Peruvian sample

ABSTRACT Objective: the objective of the study was to determine the relationship between dental calcification stages and skeletal maturation in a Peruvian sample. Methods: panoramic, cephalometric and carpal radiographs of 78 patients (34 girls and 44 boys) between 7 and 17 years old (9.90 ± 2.5 years) were evaluated. Stages of tooth calcification of the mandibular canine, first premolar, second premolar, and second molar and the skeletal maturation with a hand-wrist and a cervical vertebrae method were assessed. The relationships between the stages were assessed using Spearman's correlation coefficient. Additionally, the associations of mandibular and pubertal growth peak stages with tooth calcification were evaluated by Fisher's exact test. Results: all teeth showed positive and statistically significant correlations, the highest correlation was between the mandibular second molar calcification stages with hand-wrist maturation stages (r = 0.758, p < 0.001) and with vertebrae cervical maturation stages (r = 0.605, p < 0.001). The pubertal growth spurt was found in the G stage of calcification of the second mandibular molar, and the mandibular growth peak was found in the F stage of calcification of the second molar. Conclusion: there was a positive relationship between dental calcification stages and skeletal maturation stages by hand-wrist and cervical vertebrae methods in the sample studied. Dental calcification stages of the second mandibular molar showed the highest positive correlation with the hand-wrist and cervical vertebrae stages.

RESUMO Objetivo: o objetivo do presente estudo foi determinar a correlação entre o estágio de calcificação dentária e a maturação esquelética, em uma amostra de indivíduos peruanos. Métodos: radiografias panorâmicas, cefalométricas e carpais de 78 pacientes (34 meninas e 44 meninos) com idades entre 7 e 17 anos (média = 9,90 ± 2,5 anos) foram avaliadas. Nelas, avaliaram-se os estágios de calcificação dentária (canino, primeiro pré-molar, segundo pré-molar e segundo molar inferiores) e de maturação esquelética, pelas avaliações radiográficas da mão e punho e das vértebras cervicais. As correlações entre esses estágios foram avaliadas usando-se o coeficiente de correlação de Spearman. Adicionalmente, a associação entre os estágios em que ocorreram os picos de crescimento mandibular e de crescimento puberal e o grau de calcificação dentária foi avaliada pelo teste exato de Fisher. Resultados: todos os dentes demonstraram correlações positivas e estatisticamente significativas. A correlação mais elevada foi verificada entre o estágio de calcificação do segundo molar inferior e o estágio de maturação esquelética da mão e do punho (r= 0,758, p < 0,001) e o estágio de maturação das vértebras cervicais (r = 0,605, p < 0,001). O surto de crescimento puberal foi identificado no estágio G de calcificação do segundo molar inferior, e o pico de crescimento mandibular foi detectado no estágio F de calcificação do segundo molar. Conclusão: na amostra estudada, houve uma correlação positiva entre os estágios de calcificação dentária e os estágios de maturação esquelética avaliada nas radiografias de mão e punho e das vértebras cervicais. Os estágios de calcificação dentária do segundo molar inferior demonstraram a mais alta correlação positiva com os estágios de maturação da mão e punho e das vértebras cervicais.

Development of dental charts according to tooth development and eruption for Turkish children and young adults

PURPOSE: In this study, we aimed to develop dental charts for Turkish children and young adults of both genders within the age group of 4.5-22.5 years according to tooth mineralization and eruption in a format similar to that proposed by AlQahtani et al. MATERIALS AND METHODS: In total, 753 digital panoramic radiographs from 350 males and 403 females were assessed. The permanent teeth were evaluated according to the classification system described by Demirjian et al. The eruption stage was assessed with Bengston's system, which was modified by AlQahtani et al at four points. RESULTS: Teeth generally developed earlier in females than in males. This was particularly notable in the age group of 5-14 years. However, this difference was usually visible in only one stage, not in all teeth. It has been determined that the mixed dentition period ended with the shedding of the second deciduous molars in both genders. CONCLUSION: The dental charts presented here included information that could be beneficial to dental clinicians in making appropriate diagnosis and planning orthodontic and surgical procedures. These charts also provided datasets for preliminary dental age estimation in Turkish children and young adults.

The chronology of second molar development in Brazilians and its application to forensic age estimation

PURPOSE: This study investigated the possible correlation between the mineralization of the second molars and the chronological age of a sample population of the state of Paraiba, Brazil. MATERIALS AND METHODS: One thousand eight hundred fifty-four digitized panoramic images using a scanner of a private dental radiology service were obtained in six months. Of these, 457 were selected from patients aged 4.6 to 16 years who met certain criteria. The images were assessed twice by a radiologist with 5 years experience. A minimum interval of 30 days between the evaluations of the same patient was established. To analyze the relationship between chronological age, calcification level proposed by Demirjian et al in 1973, gender, and tooth, a multiple linear regression model was adjusted, taking age as the response variable (p<0.05). RESULTS: The gender and calcification level were significantly correlated with the age of the patients (p<0.05). There was a significant difference between the average age of the patients' upper teeth compared to the lower ones in both genders (p<0.05). The dental development occurred earlier in female individuals than in male ones, and there was no significant difference between the right and left second permanent molars. CONCLUSION: It was observed that ethnic variables are related to certain parameters of age and sex identification in the Brazilian population, providing important information for forensic evaluations.

Association between mineralization of third molars and chronological age in a Brazilian sample / Associação entre mineralização dos terceiros molares e idade cronológica em uma amostra brasileira

Purpose: To evaluate the association between chronological age and mineralization of third molar teeth in a sample from the Northeast region of Brazil. Methods: A sample of 173 digitized panoramic radiographs were selected from a total of 2,442 panoramic radiographs obtained over a period of 6 months at a private Dental Radiology service, in the city of João Pessoa, PB, Brazil. The patients were aged from 5.5 to 21 years, and a calibrated examiner made the evaluation using the classification proposed by Demirjian. Data were analyzed by using an adjusted multiple linear regression model considering age as a response variable. The level of significance adopted was 0.05. Results: Gender and stage of calcification were significantly correlated associated with age. There was no effect of gender on mineralization of the teeth. Between the two consecutive stages of calcification of third molar teeth, the mean increase in age varied by 16 months. Conclusion: The results suggest that the development of the third molar teeth is similar in both sexes. Calcification of third molars may be an auxiliary tool in the study of chronological age.

Objetivo: O objetivo deste estudo foi avaliar a associação entre idade cronológica e mineralização dentes terceiros molares em uma amostra brasileira de João Pessoa, PB, Brazil. Metodologia: De um total de 2442 radiografias panorâmicas digitalizadas obtidas num período de seis meses, em serviço privado de Radiologia Odontológica, na cidade de João Pessoa, PB, Brazil, foram selecionadas 173. Os pacientes tinham entre 5,5 a 21 anos de idade. Um avaliador calibrado fez as avaliações utilizando a classificação proposta por Demirjian. Para a análise, ajustou-se um modelo de regressão linear múltipla, considerando a idade como variável resposta. O nível de significância adotado foi de 0,05. Resultados: Tanto o sexo quanto o estágio de calcificação estiveram significativamente associados com as idades dos pacientes. Não houve diferenças significativas na mineralização dos dentes entre os sexos. Entre dois estágios consecutivos, o aumento médio da idade variou 16 meses. Conclusão: Concluiu-se que há semelhança do desenvolvimento entre os dentes terceiros molares em ambos os sexos. A calcificação dos terceiros molares pode ser uma ferramenta auxiliar no estudo da idade cronológica.

The timing of tooth eruption and root development of permanent canine and premolars in Korean children / 대한구강악안면방사선학회지

PURPOSE: The aim of this study was to investigate the timing and sequence of eruption of permanent canine and premolars, and to evaluate tooth calcification stage on emergence in Korean children. MATERIALS AND METHODS: The sample was comprised of 1,266 children (male 720, female 546) aged from 7-13 years. Tooth eruption and calcification stages were determined through oral and panoramic radiographic examination, respectively. Probit analysis was used to calculate the timing of tooth eruption and tooth calcification stage from these cross-sectional data. RESULTS: In both males and females, eruption occurred around the time when one third of tooth root or more was formed. The sequence was as follows: first premolar, canine, and second premolar in maxilla, and canine, first premolar and second premolar in mandible. Tooth eruption occurred earlier in girls compared with boys, averaging 0.63 years. CONCLUSION: Eruption sequence is identical in males and females with a trend for females to erupt earlier than males. Tooth eruption becomes earlier over the past decades in Korean children.

Dental age in patients with Down syndrome

The aim of this research was to evaluate dental age in 102 patients with Down Syndrome, using panoramic radiographs. A software program developed by the Discipline of Radiology, School of Dentistry of São José dos Campos, São Paulo State University (UNESP), was used. A table of mineralization chronology of permanent teeth among Brazilians conceived by Nicodemo, Moraes and Medici Filho was used within the software. Statistical analysis of the results showed that 70.91 percent of the males and 61.21 percent of the females presented advanced dental age. Only 32.09 percent of the males and 38.79 percent of the females presented delayed dental age. Regarding the differences between the dental and chronological ages, two thirds of the males and females presented dental age with differences of up to 12 months, which means that they can be considered to be within normal standards, whereas only 18.87 percent of the males and 10.21 percent of the females presented dental age outside normal standards, with differences of over 24 months. In conclusion, the majority of the patients with Down Syndrome were considered to be within the normal standards of mineralization chronology.

O objetivo desta pesquisa foi avaliar a idade dentária em 102 indivíduos com síndrome de Down, por meio de radiografias panorâmicas. Foi usado um "software" desenvolvido pela disciplina de Radiologia da Faculdade de Odontologia de São José dos Campos (UNESP). Neste "software", foi utilizada a tabela de cronologia de mineralização dos dentes permanentes entre brasileiros, de Nicodemo, Moraes e Medici Filho. A análise estatística dos resultados mostrou que 70,91 por cento dos indivíduos do sexo masculino e 61,21 por cento do sexo feminino apresentaram idade dentária adiantada. Apenas 32,09 por cento dos indivíduos do sexo masculino e 38,79 por cento dos indivíduos do sexo feminino apresentaram idade dentária atrasada. Com relação às diferenças entre idade dentária e idade cronológica, dois terços dos indivíduos dos sexos masculino e feminino apresentaram idades dentárias com até 12 meses de diferença, o que significa que estão dentro do padrão de normalidade; enquanto 18,87 por cento dos indivíduos do sexo masculino e 10,21 por cento dos indivíduos do sexo feminino apresentaram idades dentárias fora do padrão de normalidade com diferenças acima de 24 meses. Concluiu-se que a maioria dos pacientes com síndrome de Down estava dentro do padrão de normalidade de desenvolvimento dentário.