CHERUBISM : A CASE REPORT WITH REVIEW OF LITERATURE

Rohilla M, Bodh M

Abstract


Cherubism – first described by Jones – is a benign fibroosseous disorder of childhood involving the lower two-thirds of the face1. The true incidence is unknown but the age if onset is between 2 to 10 years2,3,4. It is a rare disease of autosomal dominant inheritance characterised by painless, frequently symmetrical, enlargement of the jaws as a result of the replacement of bone with fibrous tissue5. It has been associated
with mutations in SH3BP2 gene, which has been mapped to locus 4p16.3. The SH3BP2 mutation is thought to lead to parathyroid harmone receptor signalling and Msx-1 activation6.


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References


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