Cases reported "Dentinogenesis Imperfecta"

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1/49. dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case.

    This report documents a case of dentin dysplasia Type I in a 17-year-old boy and two members of his family. The clinical, radiographic, histologic, and ultrastructural findings indicate that this condition is distinct from other heritable defects of dentin. The entity is transmitted as an autosomal dominant trait and is characterized by teeth which have a normal color and exhibit pulpal obliteration, short roots, periapical radiolucencies, and spontaneous exfoliation. Our ultrastructural findings in agreement with those reported by Sauk and associates. ( info)

2/49. osteogenesis imperfecta. review of the literature with presentation of 29 cases.

    Twelve families with 29 patients showing various patterns of osteogenesis imperfecta are described, along with the genetic, pathological, and clinical features. No abnormal blochemical features were found. It has been shown that the best treatment is prophylactic (such as intramedullary nailingy or supportive. There was no remarkable improvement from any form of medical treatment. These data are compared to those of other authors. ( info)

3/49. Opalescent dentine in two affected siblings.

    This report describes the dental findings and management of siblings in a family in which three generations had been affected by osteogenesis imperfecta Type IV with opalescent dentine. Hereditary opalescent dentine, or opalescent teeth, is a pathologic dental condition characterised by a disturbance of dentine formation that occurs concurrently with osteogenesis imperfecta. osteogenesis imperfecta is a genetically heterogenous group of systemic disorders of the connective tissue. The two siblings affected with opalescent dentine were treated under general anaesthesia, and included stainless steel crowns, extractions, and strip crowns on primary teeth. These reports highlight that appropriate treatment of the dentition of young patients with opalescent dentine should be carried out early in the primary dentition, and that this initial treatment can have long-term benefits in the mixed and permanent dentitions. ( info)

4/49. dentinogenesis imperfecta: an early treatment strategy.

    dentinogenesis imperfecta (DI) type 2 is a disease inherited in a simple autosomal dominant mode. As soon as the teeth erupt the parents may notice the problem and look for a pediatric dentist's advice and treatment. early diagnosis and treatment of DI is recommended, as it may prevent or intercept deterioration of the teeth and occlusion and improve esthetics. The purpose of this article is to present the objectives, treatment options, and problems encountered in the treatment of DI in the early primary dentition. A two-stage treatment of a toddler under general anesthesia is described and discussed. This paper recommends for severe cases of DI two treatment stages performed under general anesthesia. Stage 1 is early (around age 18-20 months) and is directed to covering the incisors with composite restorations and the first primary molars with preformed crowns. Stage 2 (around age 28-30 months) seeks to protect the second primary molars with preformed crowns and cover the canines with composite restorations. ( info)

5/49. Dental management of severe dentinogenesis imperfecta in a mild form of osteogenesis imperfecta.

    dentinogenesis imperfecta (DI), in which the teeth are discolored, translucent and brittle, can occur in isolation as a familial trait and as a component of the skeletal dysplasia osteogenesis imperfecta (OI). In a Cape Town family, 20 persons in 3 generations had mild OI, with the additional manifestation of severe DI. The family was assessed at the Dental Genetic Unit of the University of the Western Cape and appropriate dental treatment was provided. In this setting, a detailed treatment plan was devised for a severely affected woman. This plan proved to be efficient and cost effective, and the final outcome was pleasing to the patient. dentinogenesis imperfecta is not uncommon and may well be encountered in conventional dental practice. The necessary clinical expertise is within the scope of the skills of the general dentist. ( info)

6/49. Diagnostic features and pedodontic-orthodontic management in dentinogenesis imperfecta type II: a case report.

    dentinogenesis imperfecta type II, also known as hereditary opalescent dentin, is an isolated inherited condition transmitted as an autosomal dominant trait affecting the primary and permanent dentition. The combined pedodontic-orthodontic management of a 4-year-old child is described. Following orthodontic analysis to encourage a favourable growth outcome, treatment comprised restoration of the primary teeth with stainless steel crowns and composite crowns. Differential diagnosis and alternative therapies, including orthodontic considerations, are discussed. ( info)

7/49. All-ceramic restorations for complete-mouth rehabilitation in dentinogenesis imperfecta: a case report.

    Prosthetic treatment of patients with dentinogenesis imperfecta is a challenge for the dental practitioner because numerous factors have to be considered. The use of all-ceramic restorations to rehabilitate the dentition of a young patient with dentinogenesis imperfecta is reported. Clinical and laboratory procedures are described. ( info)

8/49. dentinogenesis imperfecta associated with osteogenesis imperfecta: report of two cases.

    osteogenesis imperfecta (OI) is a heritable systemic disorder of the connective tissue. dentinogenesis imperfecta (DI), which is sometimes an accompanying symptom of OI, belongs to a group of genetically conditioned dentin dysplasias and is characterized clinically by an opalescent amber appearance of the dentin. Although the teeth of DI cases wear more easily and excessively compared to normal teeth, they do not appear to be more susceptible to dental caries than normal teeth. Two cases of DI associated with OI are presented in this paper, with 1 case suffering from nursing bottle caries. The purposes of this paper are to present the dental and skeletal characteristics of moderately and mildly involved DI associated with OI, and to discuss the possible methods of dental treatment. patients with OI and opalescent teeth should be evaluated as soon as the deciduous teeth erupt; immediate dental involvement and oral hygiene instruction can be of help in reducing the necessity of extensive dental care. ( info)

9/49. Restoration of a dentition affected by dentinogenesis imperfecta using In-Ceram: a case report.

    A patient suffering from dentinogenesis imperfecta and consequent noncarious tooth destruction was rehabilitated using In-Ceram crowns. The problems encountered in the diagnosis, treatment planning and treatment are discussed. ( info)

10/49. dentinogenesis imperfecta: a case report.

    dentinogenesis imperfecta is a localized form of mesodermal dysplasia of the dentin affecting both the primary and permanent dentitions. Most previous reports on dentinogenesis imperfecta describe treatment with overdentures, which have several disadvantages. The present report describes a case of dentinogenesis imperfecta in an 11-year-old girl. A combination of restorative, prosthetic, and surgical treatment was used to resolve the condition. ( info)
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