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1.
Anticancer Res ; 41(4): 2083-2092, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33813418

RESUMO

BACKGROUND/AIM: Numerical aberrations of permanent dentition and dystopic tooth eruption are part of the phenotype of the tumor predisposition syndrome neurofibromatosis type 1 (NF1). In these cases, surplus tooth germs usually develop in the alveolar processes of the jaw. This report attests to the dystopic development of a dysplastic supernumerary tooth in NF1 arising outside the jaw. CASE REPORT: The 8-year-old male patient developed a microdont outside the bone and above the occlusal plane of the retained maxillary right second molar. The supernumerary tooth was completely embedded in oral soft tissue. Hyperplastic oral soft tissue in the molar region and microdont were excised. Specimen of the mucosa surrounding the teeth was interspersed with diffuse and plexiform neurofibroma. The retained upper right first molar emerged spontaneously within a few months after surgery. The upper right second molar did not change position. CONCLUSION: Odontogenesis can take place within tumorous oral mucosa in NF1. Surgical removal of the tumorous mucous membrane facilitates tooth eruption in some cases.


Assuntos
Processo Alveolar/patologia , Neoplasias Bucais/diagnóstico , Neurofibroma Plexiforme/diagnóstico , Neurofibromatose 1/diagnóstico , Erupção Ectópica de Dente/diagnóstico , Processo Alveolar/anormalidades , Processo Alveolar/diagnóstico por imagem , Processo Alveolar/cirurgia , Criança , Humanos , Masculino , Neoplasias Bucais/complicações , Neoplasias Bucais/patologia , Neoplasias Bucais/cirurgia , Neurofibroma Plexiforme/complicações , Neurofibroma Plexiforme/patologia , Neurofibroma Plexiforme/cirurgia , Neurofibromatose 1/complicações , Neurofibromatose 1/patologia , Neurofibromatose 1/cirurgia , Anormalidades Dentárias/diagnóstico , Anormalidades Dentárias/etiologia , Anormalidades Dentárias/patologia , Anormalidades Dentárias/cirurgia , Erupção Ectópica de Dente/etiologia , Erupção Ectópica de Dente/cirurgia , Dente Decíduo/anormalidades , Dente Decíduo/diagnóstico por imagem , Dente Decíduo/patologia , Dente Decíduo/cirurgia , Dente Supranumerário/diagnóstico , Dente Supranumerário/etiologia , Dente Supranumerário/patologia , Dente Supranumerário/cirurgia
2.
Gen Dent ; 69(2): 64-68, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33661118

RESUMO

Taurodontism, usually associated with genetic disorders, is an anomaly in dental development in which the tooth presents with a vertically elongated pulp chamber due to an invagination failure of the Hertwig epithelial sheath. This article presents 2 case reports describing the endodontic treatment of permanent taurodontic molars in systemically healthy patients. In case 1, a maxillary left first molar presented with hypertaurodontism and 5 root canals. In case 2, a mandibular left second molar presented with hypertaurodontism and a C-shaped canal. Treatment of both teeth was based on careful radiographic evaluation with magnification, which allowed the root canal orifices to be identified and the endodontic treatment to be performed. Chemical-mechanical preparation was performed with mechanized instruments and a 2.5% sodium hypochlorite solution. As an auxiliary protocol to finish cleaning, passive ultrasonic irrigation was performed, and a thermoplastic filling technique was used. When complex internal anatomy and accessory root canals are present, endodontic treatment of a tooth presenting with hypertaurodontism can be challenging. Given the complexities of the present cases, the positive outcomes can be attributed to the use of magnification during endodontic procedures and meticulous attention to tooth preparation and obturation techniques.


Assuntos
Cavidade Pulpar , Anormalidades Dentárias , Cavidade Pulpar/diagnóstico por imagem , Humanos , Dente Molar/diagnóstico por imagem , Preparo de Canal Radicular , Tratamento do Canal Radicular , Anormalidades Dentárias/diagnóstico por imagem , Anormalidades Dentárias/terapia
3.
Am J Orthod Dentofacial Orthop ; 159(3): 360-372, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33485720

RESUMO

Tooth transposition is an uncommon disorder related to ectopic eruption; it can be classified as complete or incomplete on the basis of the position of the crowns and roots of transposed teeth. Aligning the transposed teeth to a normal sequence is always complex and challenging, especially in patients with complete transposition. The segmented archwire technique with cantilever or loops has been used in many transposition patients; however, it requires considerable laboratory work and is sometimes uncomfortable for the patient. In this case report, we present a novel orthodontic treatment for an 8-year-old boy with unilateral complete transposition of the maxillary central incisor and lateral incisor (Mx.I2.I1). During the alignment stage, the lateral incisor was moved palatally to bypass the central incisor, using a 0.012-in nickel-titanium wire continuously. Active orthodontic treatment was conducted for 44 months, and the final outcome was esthetically and functionally effective. Stable and satisfactory results were achieved within 4 years of follow-up.


Assuntos
Anormalidades Dentárias , Erupção Ectópica de Dente , Criança , Dente Canino , Humanos , Incisivo , Masculino , Maxila/diagnóstico por imagem , Maxila/cirurgia
4.
BMJ Case Rep ; 14(1)2021 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-33431460

RESUMO

Oculodentodigital dysplasia (ODDD) is a rare congenital disorder characterised by developmental abnormalities of the eye, dentition and digits of the hands and feet, with neurological symptoms reported in 30% of individuals. Dental anomalies associated with ODDD include enamel hypoplasia and subsequent caries, microdontia, missing teeth, amelogenesis imperfecta, pulp stones and delayed tooth development. Here, we describe the comprehensive dental management of a 3-year-old girl who presented with rapid deterioration of the primary dentition due to generalised enamel hypomineralisation. Conservative, comprehensive restorative management was performed under general anaesthesia. Within 6 months, further breakdown of the remaining unrestored enamel was noted. This case documents the challenges of conservative management in dental anomalies that are not well documented due to the extreme rarity of the disorder.


Assuntos
Anormalidades Craniofaciais/complicações , Assistência Odontológica para Crianças/métodos , Hipoplasia do Esmalte Dentário/terapia , Anormalidades do Olho/complicações , Deformidades Congênitas do Pé/complicações , Sindactilia/complicações , Anormalidades Dentárias/complicações , Anestesia Geral , Pré-Escolar , Anormalidades Craniofaciais/diagnóstico , Anormalidades Craniofaciais/genética , Anormalidades Craniofaciais/terapia , Coroas , Assistência Odontológica para Crianças/efeitos adversos , Assistência Odontológica para Crianças/instrumentação , Esmalte Dentário/diagnóstico por imagem , Hipoplasia do Esmalte Dentário/diagnóstico , Hipoplasia do Esmalte Dentário/genética , Anormalidades do Olho/diagnóstico , Anormalidades do Olho/genética , Anormalidades do Olho/terapia , Feminino , Deformidades Congênitas do Pé/diagnóstico , Deformidades Congênitas do Pé/genética , Deformidades Congênitas do Pé/terapia , Humanos , Dor Processual/etiologia , Dor Processual/prevenção & controle , Linhagem , Selantes de Fossas e Fissuras , Radiografia Dentária , Sindactilia/diagnóstico , Sindactilia/genética , Sindactilia/terapia , Anormalidades Dentárias/diagnóstico , Anormalidades Dentárias/genética , Anormalidades Dentárias/terapia , Dente Decíduo/diagnóstico por imagem
5.
J Am Dent Assoc ; 152(4): 318-328, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33323284

RESUMO

BACKGROUND AND OVERVIEW: Tooth agenesis is the most common congenital condition affecting the oral cavity. This condition can involve single or multiple missing teeth. Severe forms of tooth agenesis are best managed by a team of specialists, as they can be daunting for a single provider. CASE DESCRIPTION: As the goal of most patients is to replace missing teeth to improve cosmesis and function, a prosthodontist or restorative dentist plays a major role in the team's effort. Although diagnostic skills and careful planning are still the mainstay of this treatment, digital technology and modern materials can greatly facilitate implementation of the treatment plan. The authors present a case of a patient with nonsyndromic oligodontia who had a deficient occlusal vertical dimension that was treated by means of an interdisciplinary approach. The management included a combination of orthodontics, fixed and removable prosthodontics, and oral surgery. CONCLUSIONS AND PRACTICAL IMPLICATIONS: When treating patients with complex congenital tooth anomalies, the prosthodontist or restoring dentist is well positioned to take the lead and orchestrate treatment of the patient.


Assuntos
Anodontia , Anormalidades Dentárias , Dente , Anodontia/terapia , Humanos , Planejamento de Assistência ao Paciente
6.
Rev. Odontol. Araçatuba (Impr.) ; 41(3): 48-54, set./dez. 2020. ilus
Artigo em Português | LILACS, BBO - Odontologia | ID: biblio-1121747

RESUMO

A Disostose Cleidocraniana (DC), também conhecida como Displasia Cleidocraniana (DCC), é uma doença autossômica de caráter dominante, relatada pela primeira vez no século XVIII, sendo descrita em 1897 por Pierre Marie e Paul Sainton, relacionando-a a fatores genéticos. Suas manifestações clínicas apresentam-se através do desenvolvimento tardio de estruturas e defeitos ósseos, anormalidades estomatognáticas e craniofaciais. O diagnóstico baseia-se nas características clínicas e imaginológicas e o tratamento odontológico geralmente é cirúrgico, envolvendo a exodontia de unidades supranumerárias e decíduas, em associação com a abordagem ortodôntica. Este trabalho possui como objetivo, relatar o caso clínico sobre as principais manifestações bucais evidenciadas em uma paciente jovem diagnosticado com Displasia Cleidocraniana, bem como demonstrar a importância do diagnóstico preciso para auxílio no sucesso do tratamento e qualidade de vida do paciente(AU)


Cleidocranial Dysostosis (CD), also known as Cleidocranial Dysplasia (DCC), is an autosomal dominant disease, first reported in the 18th century, being described in 1897 by Pierre Marie and Paul Sainton, relating it to genetic factors. Its clinical manifestations appear through the late development of bone structures and defects, stomatognathic and craniofacial abnormalities. The diagnosis is based on clinical and imaging characteristics and dental treatment is usually surgical, involving the extraction of supernumerary and deciduous units, in association with the orthodontic approach. This work aims to report the clinical case about the main oral manifestations evidenced in a young patient diagnosed with Cleidocranial Dysplasia, as well as to demonstrate the importance of accurate diagnosis to aid in the success of the treatment and quality of life of the patient(AU)


Assuntos
Assistência Odontológica , Displasia Cleidocraniana , Anormalidades Craniofaciais , Procedimentos Cirúrgicos Bucais
7.
Rev. cuba. estomatol ; 57(4): e3192, Oct.-Dec. 2020. graf
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1144455

RESUMO

RESUMEN Introducción: La presencia de estructuras dentofaciales atípicas puede ser el primer indicador de otros defectos congénitos relacionados con síndromes también de origen genético. El síndrome Nance-Horan es un trastorno monogénico ligado al cromosoma X, caracterizado fenotípicamente por alteraciones dismorfológicas dentales y craneofaciales distintivas, cataratas congénitas y déficit cognitivo. Objetivo: Describir un caso inusual de anomalías dentarias en el curso del síndrome Nance-Horan. Presentación de caso: Paciente de 13 años de edad, masculino, de piel blanca. Al examen clínico se constató un patrón dismórfico dado por facies alargada y estrecha, orejas prominentes con anteversión de la aurícula, nariz grande con puente nasal alto, diastema generalizado en ambas arcadas, todos los dientes con anomalías de forma y microdónticos. En el estudio radiológico periapical se constataron raíces cortas y cámaras pulpares amplias. Los antecedentes patológicos personales de nuestro paciente, el patrón dismórfico cráneo facial y las radiografías coinciden con características de otros casos de síndrome Nance-Horan reportados en la literatura. La mutación del gen síndrome Nance-Horan se expresa completamente solo en los varones. Como los varones son hemicigóticos para los genes ligados al cromosoma X, basta con una copia del alelo mutado para que aparezca una enfermedad de herencia recesiva ligada al sexo. Conclusiones: Se evidenció que es de crucial importancia realizar un cuidadoso examen, tanto clínico como radiográfico, de los pacientes con anomalías dentales. Se insiste en el trabajo mancomunado entre diferentes disciplinas y especialidades, tanto médicas como estomatológicas(AU)


ABSTRACT Introduction: The presence of atypical dentofacial structures may be the first indicator of other congenital defects related to syndromes of likewise genetic origin. Nance-Horan syndrome is a monogenic X linked disorder phenotypically characterized by distinctive dysmorphic dental and craniofacial alterations, congenital cataracts and cognitive deficit. Objective: Describe an unusual case of dental anomalies in the course of Nance-Horan syndrome. Case presentation: A case is presented of a white male 13-year-old patient. Clinical examination revealed a dysmorphic pattern characterized by long narrow facies, prominent ears with auricular anteversion, a big nose with a high nasal bridge, generalized diastema in both arches, and all the teeth microdontic and abnormally shaped. Periapical radiological examination found short roots and broad pulp chambers. The personal pathological antecedents of the patient, the dysmorphic craniofacial pattern and the radiographs correspond to characteristics of other cases of Nance-Horan syndrome reported in the literature. Mutation of the Nance-Horan syndrome gene is completely expressed only in males. Since males are hemizygous for X linked genes, one copy of the mutated allele is sufficient for the appearance of a sex-linked recessive inheritance disease. Conclusions: Evidence was found of the crucial importance of conducting careful examination, both clinical and radiographic, of patients with dental anomalies. Emphasis is placed on the joint work of various disciplines and specialties, both medical and dental(AU)


Assuntos
Humanos , Masculino , Adolescente , Anormalidades Dentárias/diagnóstico por imagem , Anormalidades Congênitas/etiologia , Catarata/diagnóstico , Literatura de Revisão como Assunto
8.
Medicentro (Villa Clara) ; 24(4): 833-841, oct.-dic. 2020. graf
Artigo em Espanhol | LILACS | ID: biblio-1143250

RESUMO

RESUMEN Los dientes retenidos pueden asociarse a enfermedades de diversa índole con complicaciones: mecánicas, nerviosas, infecciosas, quísticas y tumorales. El objetivo de esta investigación es informar un caso de quiste dentígero y odontoma concurrentes, asociados a un canino superior retenido. Se presentó un paciente de 15 años que acudió al Servicio de Cirugía Maxilofacial de la Facultad de Estomatología, con evidente aumento de volumen en la superficie vestibular de reborde alveolar superior en la zona del 13, y desplazamiento progresivo coronal distal del 12. Se realizaron: interrogatorio, examen clínico y estudio radiográfico; se observó retención del 13, con asociación de área radiopaca redondeada definida y con área radiolúcida circunscrita. Se realizó una biopsia de los tejidos descritos y se obtuvo un diagnóstico de quiste dentígero y odontoma complejo. El fenómeno de retención dentaria aparece de forma concomitante con otras lesiones que pueden producir destrucción ósea y desorden de posición y número.


ABSTRACT Retained teeth can be associated with a variety of diseases with mechanical, nervous, infectious, cystic and tumoral complications. The aim of this research is to report a case of concurrent dentigerous cyst and odontoma, associated with a retained upper canine. We present a 15-year-old patient who comes to the Maxillofacial Surgery Service from the Dental Faculty due to an evident volume increase in the vestibular surface of the maxillary alveolar ridge in 13 area, and progressive distal coronal displacement of 12. An interview, a clinical examination and a radiographic study were performed; tooth retention of 13 was observed, with association of defined rounded radiopaque area and circumscribed radiolucent area. A biopsy of the described tissues was performed and a diagnosis of dentigerous cyst and complex odontoma was obtained. The phenomenon of tooth retention appears concomitantly with other lesions that can produce bone destruction and disorder of position and number.


Assuntos
Anormalidades Dentárias , Cisto Dentígero , Anodontia
9.
Eur J Paediatr Dent ; 21(4): 292-298, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33337905

RESUMO

AIM: To evaluate the frequency and distribution of dental anomalies (DA) in a paediatric population. MATERIALS AND METHODS: Panoramic digital radiographs of children between 6 and 12 years old performed at a reference centre for radiographic exams were accessed. Two calibrated examiners evaluated the radiographs. The association between variables and outcomes was assessed using non-parametric tests. The significance level was set at 5%. RESULTS: Five hundred and twelve individuals were included and 61.3% presented at least one DA. The mean age of patients with DA (9.35±1.60) was significantly higher compared to patients with no anomalies (7.90±1.58). Patients with supernumerary tooth, however, were significantly younger. The most frequent DA were permanent tooth radicular dilacerations (38.1%), permanent tooth agenesis (29.3%), supernumerary tooth (6.4%) and impacted tooth (6.4%). Anterior teeth and female patients were more affected by radicular dilacerations. Dental agenesis was more frequent in the third molars followed by the upper lateral incisor. CONCLUSION: DA are quite frequent in the paediatric population and the most common DA observed herein could only be identified through imaging exams. The most appropriate timing to perform an investigation for the detection of dental anomalies appears to be between 9 and 10 years old.


Assuntos
Anodontia , Anormalidades Dentárias , Dente Supranumerário , Anodontia/diagnóstico por imagem , Anodontia/epidemiologia , Criança , Feminino , Humanos , Prevalência , Radiografia Panorâmica , Anormalidades Dentárias/diagnóstico por imagem , Anormalidades Dentárias/epidemiologia , Dente Supranumerário/diagnóstico por imagem , Dente Supranumerário/epidemiologia
10.
Rev. Cient. CRO-RJ (Online) ; 5(1): 35-41, Jan.-Apr. 2020.
Artigo em Inglês | LILACS, BBO - Odontologia | ID: biblio-1130168

RESUMO

Dimension, number and shape alterations are frequently observed dental anomalies. Objective: The aim of this study was to map the frequency, the preferred location and the most prevalent types of supernumerary teeth, in the studied population. In addition, to assess the interference that supernumerary teeth may cause in the ectopic eruption of the normal dental series teeth. Methods: This is an observational retrospective study. Data collection was performed by evaluation of dental records of patients treated in an orthodontic clinic. Patients' radiographs, photographs and gypsum dental models were analyzed and data recorded in protocol files, by previously trained undergraduate and postgraduate students. Records from 1984 to 2019 were evaluated, and evaluation lasted two months. Data were stored in Microsoft Office Excel (version 2016) for descriptive analysis. Results: From the 920 patients' records analyzed, 2.7% presented at least one supernumerary tooth, with preferred location on the anterior maxillary area and mandibular parapremolar area. The most prevalent type was the supplemental tooth, with normal eruption orientation and normal alveolar location. Conclusion: The supernumerary teeth of the studied population showed little interference in the ectopic eruption of the normal dental series. Most of the supernumerary teeth were impacted, and, approximately one third of the supernumerary teeth showed deviated eruptions.


Introdução: Alterações de dimensão, número e forma são anomalias dentárias comumente observadas. Objetivo: O objetivo foi mapear a frequência de dentes supranumerários na população estudada, sua localização preferida e os tipos mais prevalentes. Além disso, avaliar a interferência que os dentes supranumerários podem causar na erupção ectópica da série dental normal. Métodos: A pesquisa possui desenho retrospectivo observacional. A coleta de dados foi realizada pela avaliação de prontuários odontológicos dos pacientes atendidos em uma clínica ortodôntica. Foram analisados radiografias, fotografias e modelos de gesso dental dos pacientes e as informações foram registradas em arquivos de protocolo do estudo. A coleta de dados foi realizada por uma estudante de graduação e uma da pós-graduação, previamente calibradas. A coleta durou dois meses e foram avaliados registros de 1984 a 2019. Os dados foram tratados no programa Microsoft Office Excel (version 2016) com análise descritiva dos dados. Resultados: Dos 920 prontuários analisados, 2,7% apresentavam pelo menos um dente supranumerário, com localização preferida na área superior anterior e na área parapremolar mandibular. O tipo mais prevalente foi o dente suplementar, com orientação de erupção normal e localização alveolar normal. Conclusão: Os dentes supranumerários da população estudada neste estudo mostraram pouca interferência na erupção ectópica da série dental normal. A maioria dos dentes supranumerários estava em posição de impactação. E, aproximadamente um terço dos dentes supranumerários apresentaram desvios de erupção.


Assuntos
Anormalidades do Sistema Estomatognático , Anormalidades Dentárias , Erupção Ectópica de Dente , Dente Supranumerário
11.
Rev. cuba. estomatol ; 57(3): e3060, jul.-set. 2020. graf
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1126525

RESUMO

RESUMEN Introducción: Durante la odontogénesis se pueden producir malformaciones congénitas que afectan la forma, el número, el tamaño, la estructura, la posición, el color y la erupción de los dientes. En las personas con discapacidades como parálisis cerebral, trastorno del desarrollo intelectual, síndrome de Down y trastorno del espectro autista, pueden presentarse variedad de anomalías dentales. Objetivo: Describir las anomalías dentales en las condiciones de discapacidad de parálisis cerebral, trastorno del desarrollo intelectual, síndrome de Down y trastorno del espectro autista. Métodos: Se realizó una búsqueda bibliográfica en las bases de datos Clinical Key, Medline, Dialnet y SciELO. Se aplicó la lista de comprobación PRISMA. Análisis e integración de la información: Posterior al proceso de lectura y análisis de la información, se recuperaron 800 artículos de las bases de datos, se eliminaron 590 por encontrarse repetidos. Luego de la discriminación, quedaron para revisar 210, a estos restantes se hizo la revisión de texto completo. Se eliminaron 193 no hacían referencia a anomalías dentales y/o a los trastornos o síndromes. De los 17 restantes, solo 15 cumplieron con los criterios de inclusión. Conclusiones: No se encontraron diferencias para afirmar que algunas de las anomalías y alteraciones presentadas correspondan de manera individual a cada tipo de discapacidad. Sin embargo, el síndrome de Down presenta anomalías dentales relacionadas al estado del paciente. La parálisis cerebral reporta otros hallazgos como bruxismo, debido al deficiente desarrollo muscular, lo que afecta la cavidad bucal y sus estructuras(AU)


ABSTRACT Introduction: During odontogenesis, congenital malformations can occur that affect teeth shape, number, size, structure, position, color and eruption. In people with disabilities such as cerebral palsy, intellectual development disorder, Down syndrome, and autism spectrum disorder, a variety of dental abnormalities can occur. Objective: To describe dental anomalies in such disability conditions as cerebral palsy, intellectual development disorder, Down syndrome and autism spectrum disorder. Methods: A bibliographic search was performed in the databases Clinical Key, Medline, Dialnet and SciELO. The PRISMA checklist was applied. Information analysis and integration: After reading and analyzing the information, 800 articles were retrieved from the databases, of which 590 were deleted because they were repeated. After the discrimination, 210 were pending to review; the remaining ones were reviewed full-text. 193 were deleted because they did not do any reference to dental anomalies and/or disorders or syndromes. Of the remaining 17, only 15 met the inclusion criteria. Conclusions: No differences were found to affirm that some of the anomalies and alterations presented correspond individually to each type of disability. However, Down syndrome has dental abnormalities related to patient condition. Cerebral palsy coincides with other findings such as bruxism, due to poor muscle development, which affects the oral cavity and its structures(AU)


Assuntos
Humanos , Anormalidades Dentárias/terapia , Anormalidades Congênitas/diagnóstico , Pessoas com Deficiência , Odontogênese/fisiologia , Literatura de Revisão como Assunto , Bases de Dados Bibliográficas , Síndrome de Down/diagnóstico , Transtorno do Espectro Autista/diagnóstico
12.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi ; 37(9): 1029-1031, 2020 Sep 10.
Artigo em Chinês | MEDLINE | ID: mdl-32820523

RESUMO

OBJECTIVE: To explore the genetic basis for a pedigree affected with KBG syndrome. METHODS: Clinical data of three patients from the pedigree (the proband, his mother and sister) was collected. Genomic DNA was extracted from peripheral blood samples and subjected to whole exome sequencing (WES). Suspected variant was verified by Sanger sequencing. RESULTS: The proband was found to harbor a heterozygous c.4398_4401del (p.Glu1467AsnfsTer63) frameshift variant of the ANKRD11 gene by WES. Sanger sequencing confirmed that the same variant was also present in his mother and sister, but not in his father. CONCLUSION: The c.4398_4401de (p.Glu1467AsnfsTer63) variation of the ANKRD11 gene probably underlies the KBG syndrome in this pedigree.


Assuntos
Anormalidades Múltiplas/genética , Doenças do Desenvolvimento Ósseo/genética , Deficiência Intelectual/genética , Proteínas Repressoras/genética , Anormalidades Dentárias/genética , Facies , Feminino , Humanos , Masculino , Linhagem , Fenótipo
13.
Rev. ADM ; 77(4): 222-226, jul.-ago. 2020. ilus
Artigo em Espanhol | LILACS | ID: biblio-1130183

RESUMO

Introducción: La disostosis cleidocraneal es un trastorno genético raro con patrón hereditario. Sus rasgos patognomónicos son la aplasia clavicular, fontanelas y suturas abiertas, múltiples anomalías dentales. Su origen se relaciona con alteraciones en el gen RUNX2, importante para la síntesis de CBFA1, que a su vez funciona como un conformador óseo y un diferenciador de osteoblastos. Caso clínico: Paciente de 11 años con características clínicas de CCD, se corroboran los antecedentes genéticos hereditarios y alteraciones dentales relacionados con disostosis cleidocraneal. Resultado: A tres años de tratamiento se observa mejor definición facial, la erupción de piezas permanentes retenidas y mejor función masticatoria. Conclusión: Mejorar la calidad de vida del paciente con tratamientos interceptivos y el conocimiento de las alteraciones causadas por el síndrome, así como el trabajo interdisciplinario (AU)


Introduction: Cleidocranial dysostosis is a rare genetic disorder with a hereditary pattern. Its pathognomonic features are clavicular aplasia, fontanelles and open sutures, multiple dental anomalies. Its origin is related to alterations in the RUNX2 gene, important for the synthesis of CBFA1, which in turn functions as a bone conformer and an osteoblast differentiator. Clinical case: Patient with eleven years old with clinical characteristics of CCD, hereditary genetic background, and dental alterations related to cleidocranial dysostosis are corroborated. Result: After three years of treatment, the facial definition is better, the eruption of permanent pieces retained and better chewing function. Conclusion: Improve the quality of life of the patient with interceptive treatments and the knowledge of the alterations caused by the syndrome, as well as the interdisciplinary work (AU)


Assuntos
Humanos , Feminino , Criança , Ortodontia Interceptora , Anormalidades Dentárias/terapia , Displasia Cleidocraniana/terapia , Doenças Genéticas Inatas , Equipe de Assistência ao Paciente , Qualidade de Vida , Faculdades de Odontologia , Erupção Dentária , México
14.
Cient. dent. (Ed. impr.) ; 17(2): 107-114, mayo-ago. 2020. tab, ilus, graf
Artigo em Espanhol | IBECS | ID: ibc-195098

RESUMO

La mucopolisacaridosis tipo IV (MPS-IV) también conocida como enfermedad de Morquio en recuerdo del pediatra uruguayo Luis Morquio que la describió por primera vez, es una enfermedad congénita causada por la deficiencia de la enzima N-acetilgalactosamina 6 sulfatasa o de la enzima B-Galactosidasa. Estas anomalías enzimáticas tienen como consecuencia que se acumulen en diferentes tejidos del organismo cantidades elevadas de mucopolisacaridos. En la bibliografía se describe con detalle los defectos del esmalte que presentan los pacientes diagnosticados del síndrome de Morquio. Estos defectos son una característica aparentemente constante en la enfermedad y, por lo tanto, hace necesaria las visitas al odontólogo para su control evitándose problemas mayores. Dichos defectos consisten en un esmalte anormalmente delgado, que es áspero debido a los numerosos hoyos diminutos y a una superficie irregular. La delgadez del esmalte da como resultado una forma alterada y decoloración de los dientes que, añadido a los diastemas interdentales, provocan alteraciones en la oclusión. Aparte de estos defectos, el esmalte es histológicamente normal y tiene una du-reza y radiodensidad normales. El trata-miento odontológico de los pacientes con MPS-IV requiere colaboración multidisciplinar, debido a que las manifestaciones orales de la enfermedad pueden aparecer a cualquier edad, resultando en ocasiones tedioso para el paciente y complicado para el profesional. Especial mención merecen las terapias utilizadas como trata-miento sintomático de la enfermedad, así como el manejo de la vía aérea en el caso de intervenciones bajo anestesia general o sedación para tratar ciertas patologías del territorio bucomaxilodental


Mucopolysaccharidosis type IV (MPS-IV) also known as Morquio’s disease in memory of the Uruguayan pediatrician Luis Morquio who described it for the first time, is a congenital disease caused by the deficiency of the enzyme N-acetylgalactosamine 6 sulfatase or enzyme B -Galactosidase. These enzymatic anomalies result in high amounts of mucopolysaccharides accumulating in different tissues of the organism. The enamel defects presented by patients diagnosed with Morquio syndrome are described in detail in the bibliography. These defects are an apparently constant feature in the disease and, therefore, make visits to the dentist necessary for their control, avoiding major problems. These defects consist of an abnormally thin enamel that is rough due to numerous tiny holes and an irregular surface. The thinness of the enamel results in an altered form and discoloration of the teeth, which added to the interdental diastemas, cause alterations in the occlusion. Apart from these defects, the enamel is histologically normal and has a normal hardness and radiodensity.Dental treatment of patients with MPS-IV requires multidisciplinary collaboration, because the oral manifestations of the disease can appear at any age, being sometimes tedious for the patient and complicated for the professional. Special mention should be made of the therapies used as a symptomatic treatment of the disease, as well as the management of the airway in the case of interventions under general anesthesia or sedation to treat certain pathologies of the bucomaxillodental territory


Assuntos
Humanos , Anormalidades Dentárias/etiologia , Anormalidades Dentárias/patologia , Mucopolissacaridoses/genética , Mucopolissacaridoses/fisiopatologia , Anormalidades Dentárias/terapia , Mucopolissacaridoses/classificação , Mucopolissacaridoses/etiologia , Acuidade Visual , Caixa Torácica/diagnóstico por imagem , Caixa Torácica/fisiopatologia , Esqueleto/anormalidades , Esqueleto/diagnóstico por imagem , Esmalte Dentário/anormalidades
15.
Niger J Clin Pract ; 23(6): 805-810, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32525115

RESUMO

Background: Hypodontia and hyperdontia may occur with other dental anomalies such as microdontia, taurodontism, talon cusp, macrodontia and germination. Aims: The aim of this study to evaluate the relationship between hypodontia and hyperdontia with taurodontism, macrodontia and microdontia. Subjects and Methods: In this retrospective study, 2,348 Turkish patients aged 7 to 12 years and treated between 01.01.2017 and 01.01.2018 in Bahcelievler Oral and Dental Health Hospital were evaluated. Data were collected and differences in the distribution of hypodontia and hyperdontia including other dental anomalies were analysed. Results: Of the total sample of 2,348 patients, 1,126 (48%) were girls, 1,222 (52%) were boys. Hypodontia was found in 177 children (93 girls (53%), 84 boys (47%)). The prevalence of hypodontia and hyperdontia were 7.5% and 0.9%. Taurodontism is the most common dental anomalies in hypodontia patients (39%) followed by microdontia (10%). Taurodontism was more prevalent in girls (42%) than in boys (36.5%). Microdontia was found in 10 patients and macrodontia was observed in 9 hypodontia patients. Hyperdontia was found in 21 children [8 girls (38%), 13 boys (62%)]. The most common supernumerary tooth found was mesiodens (85%) and it's more prevalent in boys (67%) than in girls (33%). Taurodontism is the most common dental anomaly (48%) following macrodontia (19%) and were found to be much more prevalent in boys (53%) (23%) than in girls (37.5%) (12.5%). Microdontia was found in only 1 boy (%7.7) in hyperdontia patients. Conclusion: Hypodontia and hyperdontia with taurodontism, microdontia, and macrodontia need much more complex treatment plan. All cases should be evaluated using interdisciplinary approach for appropriate treatment choice. This helps in longterm and effective treatment planning according to a child's individual requirements.


Assuntos
Anodontia/epidemiologia , Cavidade Pulpar/anormalidades , Anormalidades Dentárias/epidemiologia , Dente Supranumerário/epidemiologia , Adolescente , Anodontia/diagnóstico por imagem , Criança , Pré-Escolar , Cavidade Pulpar/diagnóstico por imagem , Feminino , Humanos , Masculino , Saúde Bucal , Prevalência , Radiografia Panorâmica , Estudos Retrospectivos , Anormalidades Dentárias/diagnóstico por imagem , Dente Supranumerário/diagnóstico por imagem , Turquia/epidemiologia
16.
Artigo em Inglês | MEDLINE | ID: mdl-32560490

RESUMO

BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare, ciliopathic disorder. In many ciliopathies, dental anomalies are observed alongside other symptoms of the disease. To date, there are no published reports concerning the dental developmental problems that are associated with ciliary defects in PCD patients. METHODS: Patients suffering from PCD underwent dental clinical examination, which included the assessment of developmental disorders regarding the number and morphological structure of the teeth (size and shape) as well as developmental disorders of mineralised dental tissues. Then, three-dimensional radiographic examination was performed utilising Cone Beam Computed Tomography (CBCT). RESULTS: Four PCD patients, aged 31-54, agreed to enter the study. Dental examinations showed the presence of dental developmental disorders in three of them. Additionally, CBCT showed abnormalities in those patients. CONCLUSIONS: 1. The dental phenotype in PCD patients seems to be heterogeneous. Tooth developmental disorders resulting from abnormal odontogenesis may be a symptom of PCD that is concomitant with other developmental abnormalities resulting from malfunctioning primary cilia. 2. Patients with ciliopathies are likely to develop dental developmental defects. Therefore, beginning in early childhood, they should be included in a targeted specialised dental programme to enable early diagnosis and to ensure dedicated preventive and therapeutic measures.


Assuntos
Transtornos da Motilidade Ciliar , Anormalidades Dentárias , Adulto , Criança , Pré-Escolar , Transtornos da Motilidade Ciliar/complicações , Humanos , Pessoa de Meia-Idade , Dente/crescimento & desenvolvimento
17.
Int J Esthet Dent ; 15 Suppl 1: S46-S53, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32467934

RESUMO

New digital technologies have significantly improved patient treatment modalities, especially in interdisciplinary cases. Tooth morphology can be modified at different stages of orthodontic treatment. Defining and achieving the final tooth form at the beginning or during the treatment can help the orthodontist to move teeth into the correct position quickly and more easily. The reshaping of anterior teeth can be obtained using digital technologies and CAD/CAM procedures. Composite resins are preferred to ceramics because they can be modified and adjusted whenever necessary. The parameters to assess the timing of restorative intervention are related to the age of the patient, degree of tooth eruption, and space availability in the interproximal area, especially if an additive approach is indicated to enlarge teeth.


Assuntos
Anormalidades Dentárias , Dente , Resinas Compostas , Desenho Assistido por Computador , Humanos , Planejamento de Assistência ao Paciente
18.
Br Dent J ; 228(7): 515-518, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32277206

RESUMO

Vitamin D is key to the musculoskeletal system. Its deficiency can arise from lack of exposure to sunlight and through dietary insufficiency. This can have an impact upon the oral health of an individual, including resulting in chronological hypoplasia enamel defects. Enamel hypoplasia is a quantitative defect in the enamel, presenting as pits, grooves, missing enamel or smaller teeth. The management of these defects can present a challenge to the dentist. This paper outlines the oral manifestations of vitamin D deficiency in the permanent dentition and the treatment modalities used in their management.


Assuntos
Hipoplasia do Esmalte Dentário , Anormalidades Dentárias , Deficiência de Vitamina D , Criança , Esmalte Dentário , Hipoplasia do Esmalte Dentário/etiologia , Dentição Permanente , Humanos , Anormalidades Dentárias/etiologia , Dente Decíduo , Deficiência de Vitamina D/complicações
19.
Eur J Paediatr Dent ; 21(1): 53-54, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32183529

RESUMO

BACKGROUND: Macrodontia is a rare dental anomaly, and isolated macrodontia is even more infrequent. The aim of this article is to report on a young male patient with macrodontia of the mandibular premolars. CASE REPORT: We herein present a case report of a young male patient receiving pharmacological growth hormone therapy for 10 years, who was diagnosed with macrodontia of the mandibular premolars. The patient underwent surgical treatment at the School of Dentistry of the University of Buenos Aires and was followed-up for more than 3 years. CONCLUSION: Macrodontia is a rare condition. Early diagnosis and treatment of this anomaly favors adequate formation of the dental arches. In the light of this case report, a review of paediatric patients who received growth hormone therapy during the tooth formation stage would seem relevant.


Assuntos
Hormônio do Crescimento , Anormalidades Dentárias , Dente Pré-Molar , Criança , Humanos , Masculino
20.
J Am Dent Assoc ; 151(5): 358-367, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32209245

RESUMO

BACKGROUND: Teeth with dens evaginatus (DE) are more commonly observed in Western countries than previously. This is due to the increase in populations of patients of Asian origin, in whom DE is more common than in people of European origin. The interest in DE has also increased with the introduction of a procedure called regenerative endodontics. CASE DESCRIPTION: A narrative review of treatment options for teeth with DE is presented, based on pulpal conditions and maturity of the teeth. PRACTICAL IMPLICATIONS: Early recognition of teeth with DE allows for treatment choices that generally lead to good outcomes and can aid in preserving developing teeth in young patients.


Assuntos
Doenças da Polpa Dentária , Anormalidades Dentárias , Dente Pré-Molar , Humanos , Incisivo , Coroa do Dente
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