Bony and Dental Radiographic Features of Type I and Type III Gaucher Diseased Children

Abstract

Background: Gaucher disease (GD) is a lysosomal storage disease caused by an autosomal recessive inherited deficiency of the lysosomal enzyme glucocerebrosidase. The aim of this study was to describe jaw bones involvement and dental radiographic features in pediatric Gaucher disease patients (type I and type III). Subjects and Methods: The study population of this casecontrol study included 42 Gaucher patients (study group) and 84 medically free children (control group). Panoramic radiographic images of both groups were taken and analyzed for the following findings: generalized bone rarefaction, localized rarefaction, enlarged bone marrow spaces, thinning of cortex, pseudo-cystic radiolucent lesions, anodontia, and dental anomalies. Results: Generalized rarefaction showed almost similar percentages in both types of Gaucher disease cases. Localized rarefaction was noted in 30.77% and 18.75%, of Gaucher disease type III and type I respectively. Pseudo-cystic radiolucent lesions, thinning of cortex, anodontia and dental anomalies were more prevalent in type III Gaucher patients. Conclusion: Thinning of cortex, localized rarefaction and generalized rarefaction are the most common jaw bone findings in Gaucher patients.