Y S A Mohamed1, M K Zayet2, O M Omar3, A M El-Beshlawy4. 1. Pediatric Dentistry Department, Faculty of Dentistry, October 6 University, Giza, Egypt. 2. Oral and Maxillofacial Radiology Department, Faculty of Dentistry, Cairo University, Cairo, Egypt. 3. Department of Pediatric Dentistry, Faculty of Dentistry, Cairo University, 11 Al Saraya Str., Al Manial, Kasr Al Ainy, Cairo, Egypt. ola.omar@dentistry.cu.edu.eg. 4. Pediatric Hematology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
Abstract
PURPOSE: 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 is to describe jaw bones' involvement and dental radiographic features in paediatric Gaucher disease patients (type I and type III). METHODS: The study population of this case-control study included: 42 Gaucher patients (study group) and 84 medically free children (control group). The radiographic images of both groups were analysed for the following findings: generalised bone rarefaction, localised rarefaction and enlarged bone marrow spaces, thinning of cortex, pseudocystic radiolucent lesions, anodontia and dental anomalies. Dental age assessment of Gaucher patients using the Demirjian's method was also performed. RESULTS: Generalised rarefaction showed almost similar percentages in both types of Gaucher disease cases. Localised rarefaction was noted in 30.77% and 18.75% of Gaucher disease type III and type I, respectively. Pseudocystic radiolucent lesions, thinning of cortex, anodontia and dental anomalies were more prevalent in type III Gaucher patients. The mean chronological and mean dental ages in both sexes of Gaucher patients were not statistically significant. CONCLUSION: Thinning of cortex, localised rarefaction and generalised rarefaction are the most common jaw bone findings in Gaucher patients.
PURPOSE: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 is to describe jaw bones' involvement and dental radiographic features in paediatric Gaucher diseasepatients (type I and type III). METHODS: The study population of this case-control study included: 42 Gaucher patients (study group) and 84 medically free children (control group). The radiographic images of both groups were analysed for the following findings: generalised bone rarefaction, localised rarefaction and enlarged bone marrow spaces, thinning of cortex, pseudocystic radiolucent lesions, anodontia and dental anomalies. Dental age assessment of Gaucher patients using the Demirjian's method was also performed. RESULTS: Generalised rarefaction showed almost similar percentages in both types of Gaucher disease cases. Localised rarefaction was noted in 30.77% and 18.75% of Gaucher disease type III and type I, respectively. Pseudocystic radiolucent lesions, thinning of cortex, anodontia and dental anomalies were more prevalent in type III Gaucherpatients. The mean chronological and mean dental ages in both sexes of Gaucher patients were not statistically significant. CONCLUSION: Thinning of cortex, localised rarefaction and generalised rarefaction are the most common jaw bone findings in Gaucher patients.
Authors: Elena Lukina; Nora Watman; Elsa Avila Arreguin; Marta Dragosky; Marcelo Iastrebner; Hanna Rosenbaum; Mici Phillips; Gregory M Pastores; Ravi S Kamath; Daniel I Rosenthal; Mathilde Kaper; Tejdip Singh; Ana Cristina Puga; M Judith Peterschmitt Journal: Blood Date: 2010-08-16 Impact factor: 22.113