Dentinogenesis imperfecta is an autosomal dominant genetic disorder with abnormal dentin structure affecting both primary and permanent dentitions leading to discolouration and attrition of teeth. Diagnosis is usually based on family history, a detailed clinical examination and pedigree construction. Treatment involves preservation of teeth, removal of infection, restoration of function and est...

Inherited dentin defects are classified into three types of dentinogenesis imperfecta (DGI) and two types of dentin dysplasia (DD). The genetic etiology of DD-I is unknown. Defects in dentin sialophosphoprotein (DSPP) cause DD type II and DGI types II and III. DGI type I is the oral manifestation of osteogenesis imperfecta (OI), a systemic disease typically caused by defects in COL1A1 or COL1A2...

BACKGROUND Dental anomalies are not an unusual finding in routine dental examination. The effect of dental anomalies can lead to functional, esthetic and occlusal problems. The Purpose of the study was to determine the prevalence and distribution of selected developmental dental anomalies in Saudi children. MATERIAL AND METHODS The study was based on clinical examination and Panoramic radiogr...

Dentinogenesis imperfecta determines structural alterations of the collagen structure still not completely elucidated. Immunohistochemical analysis was used to assay Type I and VI collagen, various non-collagenous proteins distribution in human primary teeth from healthy patients or from patients affected by type I dentinogenesis imperfecta (DGI-I) associated with osteogenesis imperfecta (OI). ...

Teeth are specialized structural components of the craniofacial skeleton. Developmental defects occur either alone or in combination with other birth defects. In this paper, we review the dental anomalies in several multiple congenital anomaly (MCA) syndromes, in which the dental component is pivotal in the recognition of the phenotype and/or the molecular basis of the disorder is known. We wil...

Osteogenesis imperfecta (OI) is a very common, inherited autosomal dominant disease. It characterized by deformity and fragility in bone structure, blue sclera, hearing loss, scoliosis, dentinogenesis imperfecta. Molecular studies have shown that the cause of OI mutation genes named COLIA1 COLIA2 both chains collagen. Biochemical tests examine collagen structure or molecular DNA are used for ma...

We consider Smoluchowski’s equation with a homogeneous kernel of the form a(x, y) = xy + xy with −1 < α ≤ β < 1 and λ := α + β ∈ (−1, 1). We first show that self-similar solutions of this equation are infinitely differentiable and prove sharp results on the behavior of self-similar profiles at y = 0 in the case α < 0. We also give some partial uniqueness results for self-similar profiles: in th...

This article addresses treatment of patients with various types of congenital defects, including partial and total anodontia, hypohydrotic ectodermal dysplasia, dentinogenesis imperfecta, and cleft lip and palate. The psychosocial aspects of these patients and rehabilitation with removable, fixed, and implant-supported prostheses are discussed. The factors to be considered are altered anatomy, ...