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

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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.

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Authors and Affiliations

  1. Pediatric Dentistry Department, Faculty of Dentistry, October 6 University, Giza, Egypt

    Y. S. A. Mohamed

  2. Oral and Maxillofacial Radiology Department, Faculty of Dentistry, Cairo University, Cairo, Egypt

    M. K. Zayet

  3. Department of Pediatric Dentistry, Faculty of Dentistry, Cairo University, 11 Al Saraya Str., Al Manial, Kasr Al Ainy, Cairo, Egypt

    O. M. Omar

  4. Pediatric Hematology Department, Faculty of Medicine, Cairo University, Cairo, Egypt

    A. M. El-Beshlawy

Authors
  1. Y. S. A. Mohamed
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  2. M. K. Zayet
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  3. O. M. Omar
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  4. A. M. El-Beshlawy
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Correspondence to O. M. Omar.

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All authors declare that they have no conflicts of interest.

Ethical approval

This study was approved by the Research Ethics Committee, Faculty of Medicine, Cairo University, approval number R-7-2016. The current study followed the ethical standards of Helsinki declaration.

Informed consent

The purpose of the study was explained to the guardians of children and they all signed an informed consent.

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Mohamed, Y.S.A., Zayet, M.K., Omar, O.M. et al. 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 21, 241–247 (2020). https://doi.org/10.1007/s40368-019-00471-3

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  • DOI: https://doi.org/10.1007/s40368-019-00471-3

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