gingival fibromatosis 1

(redirected from Hereditary Gingival Fibromatosis)
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gingival fibromatosis 1

A rare autosomal dominant condition (OMIM:135300) characterised by a benign, slowly progressive, nonhaemorrhagic, fibrous enlargement of maxillary and mandibular keratinised gingivae.

Molecular pathology
Caused by defects of SOS1, which encodes a guanine nucleotide exchange factor for RAS proteins, a family of membrane proteins that bind guanine nucleotides and participate in signal transduction pathways.
References in periodicals archive ?
Hereditary gingival fibromatosis as an isolated trait is usually transmitted
Other genetic loci such as 8, 14q, 19p, 19q and Xq are also related to syndromes associated with hereditary gingival fibromatosis.
As mentioned earlier, hereditary gingival fibromatosis depicts the generalized gingival enlargement with no other systemic manifestations.
Oro-facial granulomatosis can be demarcated from hereditary gingival fibromatosis by observing the features of oro-facial granulomatosis in addition to gingival enlargement which are absent in hereditary gingival fibromatosis.
Such features are absent in Hereditary gingival fibromatosis.
Keeping all these facts in mind, diagnosis of hereditary gingival fibromatosis was made on the basis of positive family history, parental consanguinity, classical clinical features and absence of any syndromic association.
Gingival abnormalities of genetic origin: preliminary communication with special reference to hereditary gingival fibromatosis.

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