Introduction: Dentin dysplasia is a rare autosomal dominant inheriting disturbance of dentin formation characterized by normal enamel formation, but atypical dentin with abnormal pulpal morphology. There are two major patterns: type I and type II. Amelogenesis imperfecta is an autosomal dominant. X-link inherent disease that is classified by clinical manifestation into hypoplastic, hyp...

dentin dysplasia is an exceptionally rare, autosomal-dominant, hereditary condition, primarily characterized by defective dentin formation affecting both the deciduous and permanent dentitions. the etiology remains imprecise to date, in spite of the numerous hypotheses put forward and the constant updates on this condition. this case report of type i dentin dysplasia exhibits radiographic fi nd...

developmental dysplasia of the hip (ddh) or congenital hip dysplasia (cdh) is the most prevalent developmental childhood hip disorder. it includes a wide spectrum of hip abnormalities ranging from dysplasia to subluxation and complete dislocation of the hip joint. the natural history of neglected ddh in adults is highly variable. the mean age of onset of symptoms is 34.5 years for dysplastic dd...

objective: florid cemento-osseous dysplasia (fcod) is a rare bone lesion that predominantly involves the women’s jaws in middle age. this condition is usually asymptomatic and has a benign course.   case: this paper presents a rare case of fcod in a white middle aged woman, which had affected mandible bilaterally and was diagnosed after tooth extraction and treated conservatively.   we believed...

fibrous dysplasia (fd) is an osseous growth dis-order, producing immature bone and characte-rized by the replacement of normal bone with fibro-osseous connective tissue. it is a bone dys-plasia that has the potential to cause significant cosmetic and functional disturbances, particularly in the craniofacial skeleton. cra-niofacial fibrous dysplasia is one of the three types of polyostotic fibro...

Jarcho-Levin syndrome is a rare disorder characterised by defects in vertebral and costal segmentation of varying severity. Respiratory complications are the main cause of death or severe comorbidity due to a restrictive rib cage. A 3 months old infant with Spondylocostal dysostosis and associated bronchomalacia experiencing severe asynchrony during the weaning process is reported. The Neurally...

Notch signaling regulates a vast array of crucial developmental processes. It is therefore not surprising that mutations in genes encoding Notch receptors or ligands lead to a variety of congenital disorders in humans. For example, loss of function of Notch results in Adams-Oliver syndrome, Alagille syndrome, spondylocostal dysostosis and congenital heart disorders, while Notch gain of function...