Oral Surgery, Oral Medicine, Oral Pathology

1977

DOI: 10.1016/0030-4220(77)90163-3

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Dentine dysplasia with sclerotic bone and skeletal anomalies inherited as an autosomal dominant trait

Merle E. Morris¹,

Russell H. Augsburger²

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Discussion2

Anomalies Of Tooth Structure1

Diagnosis and Differential Diagnosis1

Classification Of Hereditary Dentin Disorders1

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1979

1979

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Cited by 32 publications

(6 citation statements)

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“…Teeth with radiographic or histologic features of DD occur in a number of disorders such as calcinosis, Ehlers-Danlos syndrome, and the brachioskeletogenital syndrome [9]. Some association has also been reported between dentine dysplasia and osseous changes in addition to sclerotic bone formation [10] but our patient had no signs of other pathologic conditions.…”

Section: Discussionmentioning

confidence: 82%

Dentin dysplasia type I: a case report and review of the literature

¹

,

²

,

³

et al. 2010

J Med Case Reports

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IntroductionDentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. Radiographic analysis shows obliteration of all pulp chambers, short, blunted and malformed or absent roots and peri-apical radiolucencies of non carious teeth.Case presentationWe present a case of dentin dysplasia type I in a 12-year-old Iranian boy, and the clinical, radiographic and histopathologic findings of this condition and treatment are described.ConclusionsThere are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia. The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described. Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition.

“…Teeth with radiographic or histologic features of DD occur in a number of disorders such as calcinosis, Ehlers-Danlos syndrome, and the brachioskeletogenital syndrome [9]. Some association has also been reported between dentine dysplasia and osseous changes in addition to sclerotic bone formation [10] but our patient had no signs of other pathologic conditions.…”

Section: Discussionmentioning

confidence: 82%

Dentin dysplasia type I: a case report and review of the literature

¹

,

²

,

³

et al. 2010

J Med Case Reports

View full text Add to dashboard Cite

IntroductionDentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. Radiographic analysis shows obliteration of all pulp chambers, short, blunted and malformed or absent roots and peri-apical radiolucencies of non carious teeth.Case presentationWe present a case of dentin dysplasia type I in a 12-year-old Iranian boy, and the clinical, radiographic and histopathologic findings of this condition and treatment are described.ConclusionsThere are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia. The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described. Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition.

“…Dentine dysplasia type I (DDI; radicular dentine dysplasia or rootless teeth; %125400] is a rare autosomal dominant condition characterized primarily by teeth in both dentitions with incomplete root formation 223. The roots are short, blunt and apically restricted, and their morphology leads to premature exfoliation.…”

Section: Anomalies Of Tooth Structurementioning

confidence: 99%

Diseases of the tooth: the genetic and molecular basis of inherited anomalies affecting the dentition

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,

²

2012

WIREs Developmental Biology

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In humans, inherited variation in the number, size, and shape of teeth within the dentitions are relatively common, while rarer defects of hard tissue formation, including amelogenesis and dentinogenesis imperfecta, and problems associated with tooth eruption are also seen. In many cases, these anomalies occur in isolation, but they can also present as a feature of numerous well-characterized developmental syndromes. Complex reiterative signaling between the epithelium and mesenchyme is a feature of normal tooth development in the embryo, occurring from early patterning through morphogenesis, hard tissue formation and during root development. Significant events also occur during postnatal development of the dentition, including hard tissue maturation and tooth eruption. In the last decade, advances in human and mouse genetics have meant that in many cases candidate genes have been identified for these anomalies. These genes have provided a useful platform for developmental biologists, allowing them to begin elucidating how these signals interact to generate a functional dentition and understand the mechanisms underlying many of the anomalies that are seen in human populations. In this article, we review current concepts relating to the developmental biology of tooth number, size, and shape, formation of the dental hard tissues and eruption of the tooth into the oral cavity. We will focus on the molecular mechanisms underlying these processes in both health and disease.

“…But, in this patient, there was no familial history of the disease, so she was considered to be the first generation sufferer. Some association has also been reported between dentine dysplasia and osseous chances in addition to sclerotic bone formation [13], but this patient had no signs of other pathologic conditions.…”

Section: Discussionmentioning

confidence: 83%

Dentinal Dysplasia Type I: A Case Report with a 6-Year Followup

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²

,

³

et al. 2013

Case Reports in Dentistry

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Introduction. Dentin dysplasia is a rare disturbance of dentin formation characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology that is inherited as an autosomal pulpal morphology. Case Presentation. A 7-year-old female who had problems in chewing function was referred to Oral and Maxillofacial Surgery Department at the Faculty of Dentistry in Ondokuz Mayıs University. In the radiographic examination, it was determined that some of the unerupted permanent teeth of the patient had short, blunted, and malformed roots with obliterated pulp chambers, although the bone below the teeth showed well-defined margins. This unusual case of generalized short roots presents a case demonstrating both classic and atypical features of dentinal dysplasia type I (DDI) in the mixed and permanent dentitions. Conclusion. There are still many issues in the diagnosis and management of patients with dentin dysplasia. Early diagnosis, clinical and radiographic findings, as well as treatment of this condition and the initiation of effective preventive strategies may help prevent or delay loss of dentition.

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