Dentinogenesis imperfecta type II or hereditary opalscent dentin is one of the most common autosomal dominant anomaly of dentin that occurs in both sex affecting approximately 1:8000 persons. Clinically this disorder is characterized by variable blue gray to yellow brown teeth, with fracture of enamel and excessive wear. The treatment strategy is focused towards protecting teeth from further wear and tear and total oral rehabilitation of patient with paramount importance to aesthetics, obtaining an appropriate vertical dimension and providing soft tissue support which will help to return the facial profile to a more normal appearance. A multidisciplinary treatment planning is required for treatment of these individuals.
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Childhood early oral ageing syndrome: prevalence and association with possible aetiological factors and consequences for the vertical dimension of occlusion: protocol for a cross-sectional study.
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Postgraduate Program in Rehabilitation Sciences, Universidade Nove de Julho, São Paulo, São Paulo, Brazil
Introduction: Childhood early oral ageing syndrome (CEOAS) is a condition involving oral abnormalities resulting from systemic diseases of different origins that are related to the current lifestyle of the paediatric population. Enamel defects associated with intrinsic and extrinsic factors promote the early loss of tooth structure at an accelerated pace, with negative impacts on function, aesthetics and quality of life. The aim of the study is to identify the prevalence of early tooth wear in childhood and its severity using the CEOAS index, which is a tool for the diagnosis of the condition and for epidemiological surveys, involving the investigation of abnormalities of the oral cavity in the paediatric population and possible factors associated with the severity of the condition.
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Hereditary dentine disorders are autosomal dominant diseases that affect the development and structure of dentine, leading to various dental abnormalities and influencing the individual's oral health. It is generally classified as dentinogenesis imperfecta (DGI) and dentine dysplasia (DD). Specifically, DGI is characterized by the abnormal formation of dentine, resulting in teeth that are discolored, translucent, and prone to fracture or wear down easily.
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Osteogenesis imperfecta, a common genetic connective tissue disorder affecting bone with multisystemic implications, is caused by genomic alterations at various levels that disrupt the biosynthesis stages of collagen Type I. This study evaluated the intraoral and clinical findings of 43 OI cases in relation to genetic variants, aiming to contribute new insights into the roles of collagen and non-collagen genes in the oral-dental pathology of OI. Significant associations were found between OI variants and dental anomalies such as dentinogenesis imperfecta, enamel hypoplasia, taurodontism, and hypodontia.
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Article Synopsis
- Osteogenesis imperfecta (OI) is a genetic connective tissue disorder leading to fragile bones, with a global occurrence of 1 in 10,000; diagnosis relies on clinical and radiological assessments.
- A study examined 91 pediatric OI patients at a national institute, revealing that most had fractures and specific physical traits like blue sclera and bowed legs; treatment typically involved bisphosphonates.
- The findings suggest the need for better OI fracture risk classification using severity scales, highlighting the importance of raising awareness and enhancing research on this rare condition.*
Dentinogenesis imperfecta in a 6-year-old male neutered Labrador retriever: Case report with atypical clinical presentation and treatment review.
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Specialty Oral Pathology for Animals, LLC., Geneseo, IL, United States.
This case report details the diagnosis and treatment of dentinogenesis imperfecta in a 6-year-old neutered male Labrador, presenting without concurrent osteogenesis imperfecta. Diagnostic modalities, including radiographs, CT imaging, and histopathological examination, are reviewed in conjunction with the latest literature on canine dentinogenesis imperfecta. This patient presented at a more advanced age than typically reported cases.
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