1/5. 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.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
2/5. 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.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
3/5. A Polish variant of isolated dentinogenesis imperfecta with a generalised connective tissue defect.A case is described of four generations of a Polish family with characteristic features of dentinogenesis imperfecta, who also presented with a variety of generalised connective tissue features, suggestive of a structural gene mutation causing abnormalities in connective tissues other than dentine.- - - - - - - - - - ranking = 6keywords = connective (Clic here for more details about this article) |
4/5. Familial dentinogenesis imperfecta, blue sclerae, and wormian bones without fractures: another type of osteogenesis imperfecta?A unique connective tissue disorder characterised by the triad of dentinogenesis imperfecta, blue sclerae, and multiple wormian bones has been identified in 20 members of three generations of a large kindred of mixed ancestry in south africa. The skeletons of affected subjects were moderately osteoporotic but, apart from minimal bowing of the femora and some vertebral flattening in late adulthood, this abnormality produced no untoward sequelae. Bone fragility was present in one young male, while a mother and her daughter had deafness of uncertain relationship with the primary disorder. Dental discolouration and a liability to caries were the only important complications. The condition is best regarded as yet another variety of osteogenesis imperfecta. It is inherited as an autosomal dominant trait with relatively consistent phenotypic expression.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
5/5. orthognathic surgery in osteogenesis imperfecta: a case report with management considerations.osteogenesis imperfecta is a disease of connective tissues with additional metabolic defects. It is associated with recognisable facial disproportion and sometimes warrants surgical intervention for aesthetic, functional and psychological reasons. A severe case of osteogenesis imperfecta type 3 is presented to illustrate the feasibility of bimaxillary surgery using a mandibular body step osteotomy and maxillary down grafting at the Le Fort I level. The medical, anaesthetic, surgical and specific maxillofacial implications of surgery in these patients are discussed.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |