Osteogenesis imperfecta in spremembe zobovja = Osteogenesis imperfecta and dental aberrations
Bratanič, Nevenka ...
Osteogenesis imperfecta (OI) is a hereditary disorder characterised by increased bone fragility and low bone mass. Recurrent fractures result in bonedeformities, loss in mobility and chronic pain. ...Basilar invagination is anuncommon but devastating complication of this disease. Additional clinical manifestations such as blue sclerae, dentinogenesis imperfecta, joint laxity and maturity onset deafness are described in the literature. OI has been classified into four major types according to clinical severity and radiographic findings. These main types of 0I are further divided into subtypes according to the presence or absence of dentinogenesis imperfecta. Recently, several new types of OI have been described, namely type V, type VI and the rhizomelic form. OI results from various mutations in genes COL1A1 andCOL1A2 leading to defects in type I collagen, which is the major component of both bone and dentin. The medical care of OI patients follows a team approach. Recently, several studies have shown that treatment with bisphosphonate pamidronate significantly reduced the morbidity associated withmoderate to severe forms of OI. The fracture rate decreases, chronic bone pain disappears and the quality of life improves. The high prevalence of dental aberrations in OI for careful clinical and radiographic examination of the oral cavity. Dentists should be involved in the care of OI patients. Through early diagnosis and appropriate dental procedures several consequencedof oral pathology can be prevented.
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