Jaw bones' involvement and dental features of type I and type III Gaucher disease: a radiographic study of 42 paediatric patients

Eur Arch Paediatr Dent. 2020 Apr;21(2):241-247. doi: 10.1007/s40368-019-00471-3. Epub 2019 Sep 17.

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.

Keywords: Child; Dental radiography; Gaucher disease; Jaw abnormalities.

MeSH terms

  • Case-Control Studies
  • Child
  • Female
  • Gaucher Disease*
  • Humans
  • Male