Wrodzona łamliwość kości w aspekcie stomatologicznym – ocena kliniczna w oparciu o piśmiennictwo

© Borgis - Nowa Stomatologia 3/2011, s. 134-138

*Iwona Sobiech¹, Anna Grzybowska¹, Elżbieta Jelonek³, Julian Komarnitki², Dorota Olczak-Kowalczyk¹

Summary
Osteogenesis imperfecta is genetically determined disorder of connective tissue inherited in an autosomal dominant, less often recessive. Belongs to the same group of disease entities as team Ehlers’a-Dalnos’a, Marfan’a team, the team Hurler’a, pseudoxantoma elasticum. Pathology is related to mutation in genes COL1 and COL2, which encode chains alfa1 and collagen type alfa2 and, the main whites of proteins of ectodermal structural tissues. General symptoms of bone structures and irregularities of mineralized tooth tissues were discussed based on the literature. Literature reports of 150 to 250 different mutations responsible for the formation of collagen OI. Among the different classifications currently the most widely used is the division of the 4 types of OI by Silence’a, taking into account clinical and genetic aspects. The complex treatment is indispensable in patient with diagnosed inherent bone brittleness disease. Patients with known congenital fragility need a comprehensive and multi-general and dental treatments. Currently in OI from infancy for the prevention of fractures of the number and severity of drug therapy is implemented which promotes better bone remodeling. Also take into account the method of rehabilitation. Patients regardless of the classification for any of the types of OI have to be individually diagnosed. Many doctors of different specialities cooperate to try to assure the patients about the optimal development and the progress in the orthopedic and pharmacology scope and they give a chance on independent functioning in mature years by new forms of rehabilitations.

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