Osteogenesis Imperfecta: A Case Report

Abstract

Osteogenesis imperfecta OI , also known as"brittle bone disease" is a relatively common hereditary connective tissue disorder characterized bybone deformation, fragility and fractures. Molecular studies have demonstrated that most casesresult from mutations affecting the genes responsible for the formation of type 1 collogen. Otherfrequently affected tissues include tendon, ligament, skin, sclera, teeth, middle and inner ear.Commonly observed dental abnormalities includedentinogenesis imperfecta and malocclusion

Keywords

• Osteogenesis imperfecta, brittlebone disease, dentinogenesis imperfecta

Osteogenezis imperfekta: bir olgu sunumu

Abstract

Osteogenezis İmperfekta Oİ , diğer adıylakırılgan kemik hastalığı, oldukça yaygın görülenkalıtımsal geçiş gösteren kemiklerde deformite,fraktür ve frajilite ile seyreden konnektif dokuhastalığıdır. Moleküler çalışmalar, bu bozukluğunsebebinin, çoğu vakada tip 1 kollajenin yapımındansorumlu olan genin mutasyonu olduğunu göstermiştir. Kemiklerin dışında kollajeni yapısındabulunduran tendon, ligament, deri, sklera, dişler,orta ve iç kulak etkilenebilir. Hastalarda sıkgörülen dental anomaliler, dentinogenezis imperfekta Dİ ve maloklüzyondur

Keywords

• Osteogenezis imperfekta, k›r›lgan kemik hastalıı, dentinogenezis imperfekta.

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