Craniofacial and Dental Features in Six Children With Cherubism
Sign Up to like & getrecommendations! Published in 2017 at "Journal of Craniofacial Surgery"
DOI: 10.1097/scs.0000000000003819
Abstract: Abstract Cherubism is an autosomal-dominant benign bone disorder, characterized by fibro-osseous lesions in the mandible and maxilla commonly caused by mutations in the SH3-binding protein 2-gene. The purpose of the authors’ study was to analyze… read more here.
Keywords: craniofacial dental; age; cherubism; six children ... See more keywords
Imatinib has minimal effects on inflammatory and osteopenic phenotypes in a murine cherubism model.
Sign Up to like & getrecommendations! Published in 2021 at "Oral diseases"
DOI: 10.1111/odi.14073
Abstract: OBJECTIVE Cherubism is a genetic disorder characterised by bilateral jawbone deformation. The associated jawbone lesions regress after puberty, whereas severe cases require surgical treatment. Although several drugs have been tested, fundamental treatment strategies for cherubism… read more here.
Keywords: imatinib minimal; cherubism mutant; cherubism; model ... See more keywords
Neutrophil developmental defects cause hyperinflammation in cherubism
Sign Up to like & getrecommendations! Published in 2023 at "Physiology"
DOI: 10.1152/physiol.2023.38.s1.5723270
Abstract: Cherubism is a rare autosomal dominant disease caused mainly by a P416R point mutation of Src homology 3 Domain Binding Protein 2 (SH3BP2). Though macrophages and osteoblasts were thought to be the major contributors to… read more here.
Keywords: hyperinflammation; defects cause; neutrophil developmental; physiology ... See more keywords
Efficacy and safety of denosumab treatment in a prepubertal patient with cherubism
Sign Up to like & getrecommendations! Published in 2020 at "Journal of Pediatric Endocrinology and Metabolism"
DOI: 10.1515/jpem-2019-0581
Abstract: Abstract Background Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand, which strongly suppresses osteoclasts. Cherubism is a rare autosomal dominant disorder characterized by symmetrical swelling of the jaws, in which the… read more here.
Keywords: efficacy safety; cherubism; treatment; denosumab ... See more keywords