Osteogenesis Imperfecta: A Case Report

Year 2008, Volume: 35 Issue: 2 - Volume: 35 Issue: 2, 95 - 98, 01.05.2008

Bengi Öztaş Şebnem Kurşun Şehrazat Özde Sibel Elif Gültekin

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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