BACKGROUND Amelogenesis imperfecta represents a group of developmental conditions, clinically and genetically heterogeneous, that affect the structure and clinical appearance of enamel. Amelogenesis imperfecta occurred as an isolated trait or as part of a genetic syndrome. Recently, disease-causing mutations in the FAM20A gene were identified, in families with an autosomal recessive syndrome as...

An interdisciplinary approach can be used to treat the uncommon hereditary defect known as amelogenesis imperfecta, which ischaracterised by insufficient crown length, hypersensitivity, dental caries, and decreased vertical dimension. The present case reportdescribes a successfully managed of imperfecta with full mouth rehabilitation using implant supported prosthesis. This helped restore funct...

osteogenesis imperfecta (oi) as an inherited connective tissue disorder can affect all tissues that contains type i collagen. well-known cardiac complications of this disease such as aortic root dilatation, aortic regurgitation and mitral valve prolapse have been rarely reported in the literature. coronary artery aneurysm is a rare cardiac complication in oi, as reported in a 19 year old female...

introduction: this study was carried out to investigate calcific tissue formation against mineral trioxide aggregate (mta) after pulp capping. materials and methods: the pulps of six teeth from four dogs were exposed and capped with mta. after extraction each tooth was sectioned into halves. each half was then further sectioned in the mesiodistal or buccolingual direction. calcified tissue of t...

BACKGROUND Upper limb deformity in children with osteogenesis imperfecta may substantially impair function. The aims of this retrospective work were to study the prevalence of radial head malalignment (dislocation or subluxation) in different types of osteogenesis imperfecta and to identify factors linked to it. METHODS We assessed 489 upper limbs from 254 patients (with a mean age of 9.6 yea...

Osteogenesis imperfecta is a disorder of connective tissue, existing in a severe form (congenita) and a milder form (tarda) both being inherited as an autosomal dominant (McKusick, 1966). Recently Aeschlimann, Grunt, and Crigler (1966) and Bilginturan and Ozsoylu (1966) reported three infants with osteogenesis imperfecta congenita who improved, as regards survival and frequency of spontaneous f...

We isolated dental papilla mesenchymal cells (DPMCs) from different rat incisor germs at the late bell stage and incubated them as cell pellets in polypropylene tubes. In vitro pellet culture of DPMCs presented several crucial characteristics of odontoblasts, as indicated by accelerated mineralization, positive immunostaining for dentin sialophosphoprotein and dentin matrix protein 1, and expre...

To cite: Shah KM. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2013-008958 DESCRIPTION Dentinogenesis imperfecta (DI) is a genetically determined developmental defect of dentine. It is broadly grouped into three categories: type I: the dental manifestation of osteogenesis imperfect, type II: classical hereditary opalescent dentine and type III: Brandywine isol...

``Osteogenesis imperfecta'' and ``vitreous osteoporosis'' are genetic diseases in most of their cases, that is, it is sufficient for one the parents to be a disease carrier order have children suffering from it. It main protein source bone structure leading this disorder; however, factor common accounts about 80 85% causes osteogenesis imperfecta. The congenital disorder associated with defect ...