Epidermolysis bullosa consist of a pattern of diseases which is mainly associated with genetic defects in the integrity of structures that cause the adhesion of the epidermis with the dermis,primarily called as the Ba...Epidermolysis bullosa consist of a pattern of diseases which is mainly associated with genetic defects in the integrity of structures that cause the adhesion of the epidermis with the dermis,primarily called as the Basement Membrane Zone.If the defect is associated with the lamina lucida of the basement membrane zone,it is called junctional epidermolysis bullosa(JEB).JEB is mainly inherited in an autosomal recessive manner.The characteristic feature of all the JEB subtypes is enamel hypoplasia.This article is aimed at identifying the main features of JEB in children.Fifty articles which were published between 2000 and 2022 were reviewed and the types,investigations and management of JEB are explained based on the existing literature.展开更多
Enamel hypoplasia is a surface defect of the tooth crown caused by a disturbance of enamel matrix secretion.Enamel hypoplasia may be inherited,or result from illness,malnutrition,trauma,or high concentrations of fluor...Enamel hypoplasia is a surface defect of the tooth crown caused by a disturbance of enamel matrix secretion.Enamel hypoplasia may be inherited,or result from illness,malnutrition,trauma,or high concentrations of fluorides or strontium in the drinking water or food.Different types of enamel hypoplasia have been distinguished,such as pit-type,plane-type,and linear enamel hypoplasia.Hypoplasia has been related to the intensity and duration of stress events,the number of affected ameloblasts,and their position along the forming tooth crown.Amelogenesis imperfecta(AI) is a heterogeneous group of inherited defects in dental enamel formation,most teeth are affected in both the primary and permanent dentition.The malformed enamel can be unusually thin,soft,rough and stained.The strict definition of AI includes only those cases where enamel defects occur in the absence of other symptoms.Currently,there are seven candidate genes for AI: amelogenin,enamelin,ameloblastin,tuftelin,distal-less homeobox 3,enamelysin,and kallikrein 4.Since the enamel is formed according to a strict chronological sequence,and once formed,undergoes no repair or regeneration.Then the analysis the phenotype of enamel hypoplasia can provide insights of the severity of inherited or environmental stress and the molecular mechanism during the period of enamel formation.展开更多
文摘Epidermolysis bullosa consist of a pattern of diseases which is mainly associated with genetic defects in the integrity of structures that cause the adhesion of the epidermis with the dermis,primarily called as the Basement Membrane Zone.If the defect is associated with the lamina lucida of the basement membrane zone,it is called junctional epidermolysis bullosa(JEB).JEB is mainly inherited in an autosomal recessive manner.The characteristic feature of all the JEB subtypes is enamel hypoplasia.This article is aimed at identifying the main features of JEB in children.Fifty articles which were published between 2000 and 2022 were reviewed and the types,investigations and management of JEB are explained based on the existing literature.
文摘Enamel hypoplasia is a surface defect of the tooth crown caused by a disturbance of enamel matrix secretion.Enamel hypoplasia may be inherited,or result from illness,malnutrition,trauma,or high concentrations of fluorides or strontium in the drinking water or food.Different types of enamel hypoplasia have been distinguished,such as pit-type,plane-type,and linear enamel hypoplasia.Hypoplasia has been related to the intensity and duration of stress events,the number of affected ameloblasts,and their position along the forming tooth crown.Amelogenesis imperfecta(AI) is a heterogeneous group of inherited defects in dental enamel formation,most teeth are affected in both the primary and permanent dentition.The malformed enamel can be unusually thin,soft,rough and stained.The strict definition of AI includes only those cases where enamel defects occur in the absence of other symptoms.Currently,there are seven candidate genes for AI: amelogenin,enamelin,ameloblastin,tuftelin,distal-less homeobox 3,enamelysin,and kallikrein 4.Since the enamel is formed according to a strict chronological sequence,and once formed,undergoes no repair or regeneration.Then the analysis the phenotype of enamel hypoplasia can provide insights of the severity of inherited or environmental stress and the molecular mechanism during the period of enamel formation.
