A Possible Case of Cherubism in a 17th-Century Korean Mummy
Identifieur interne : 000276 ( Main/Merge ); précédent : 000275; suivant : 000277A Possible Case of Cherubism in a 17th-Century Korean Mummy
Auteurs : Israel Hershkovitz [Israël] ; Mark Spigelman [Israël] ; Rachel Sarig [Israël] ; Do-Sun Lim [Corée du Sud] ; In Sun Lee [Corée du Sud] ; Chang Seok Oh [Corée du Sud] ; Hila May [Israël] ; Elisabetta Boaretto [Israël] ; Yi-Suk Kim [Corée du Sud] ; Soong Deok Lee [Corée du Sud] ; Nathan Peled [Israël] ; Myeung Ju Kim [Corée du Sud] ; Talya Toledano [États-Unis] ; Gila Kahila Bar-Gal [Israël] ; Dong Hoon Shin [Corée du Sud]Source :
- PLoS ONE [ 1932-6203 ] ; 2014.
Abstract
Cherubism is a benign fibro-osseous disease of childhood limited specifically to the maxilla and mandible. The progressive replacement of the jaw bones with expansile multilocular cystic lesions causes eventual prominence of the lower face, and hence the classic “cherubic” phenotype reflecting variable extents of jaw hypertrophy. Histologically, this condition has been characterized as replacement of the normal bone matrix with multicystic pockets of fibrous stroma and osteoclastic giant cells. Because of radiographic features common to both, primarily the presence of multiloculated lucencies with heterogeneous “ground-glass” sclerosis on CT imaging, cherubism was long mistaken for a craniofacial subtype of fibrous dysplasia. In 1999, however, the distinct genetic basis for cherubism was mapped to chromosome 4p16.3 and the SH-3 binding protein SH3BP2. But while there are already three suspected cases of fibrous dysplasia amongst archaeological populations, no definitive cases of cherubism have yet been reported in historical populations. In the current study we describe micro- and macro-structural changes in the face of a 17th century Joseon Dynasty Korean mummy which may coincide with the clinic-pathologic and radiologic features of cherubism.
Url:
DOI: 10.1371/journal.pone.0102441
PubMed: 25093864
PubMed Central: 4122385
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-Century Korean Mummy</title>
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-Century Korean Mummy</title>
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<author><name sortKey="Oh, Chang Seok" sort="Oh, Chang Seok" uniqKey="Oh C" first="Chang Seok" last="Oh">Chang Seok Oh</name>
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<author><name sortKey="Boaretto, Elisabetta" sort="Boaretto, Elisabetta" uniqKey="Boaretto E" first="Elisabetta" last="Boaretto">Elisabetta Boaretto</name>
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<author><name sortKey="Lee, Soong Deok" sort="Lee, Soong Deok" uniqKey="Lee S" first="Soong Deok" last="Lee">Soong Deok Lee</name>
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<author><name sortKey="Peled, Nathan" sort="Peled, Nathan" uniqKey="Peled N" first="Nathan" last="Peled">Nathan Peled</name>
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<author><name sortKey="Kim, Myeung Ju" sort="Kim, Myeung Ju" uniqKey="Kim M" first="Myeung Ju" last="Kim">Myeung Ju Kim</name>
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<author><name sortKey="Shin, Dong Hoon" sort="Shin, Dong Hoon" uniqKey="Shin D" first="Dong Hoon" last="Shin">Dong Hoon Shin</name>
<affiliation wicri:level="3"><nlm:aff id="aff4"><addr-line>Department of Anatomy, Seoul National University College of Medicine, Seoul, Korea</addr-line>
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<front><div type="abstract" xml:lang="en"><p>Cherubism is a benign fibro-osseous disease of childhood limited specifically to the maxilla and mandible. The progressive replacement of the jaw bones with expansile multilocular cystic lesions causes eventual prominence of the lower face, and hence the classic “cherubic” phenotype reflecting variable extents of jaw hypertrophy. Histologically, this condition has been characterized as replacement of the normal bone matrix with multicystic pockets of fibrous stroma and osteoclastic giant cells. Because of radiographic features common to both, primarily the presence of multiloculated lucencies with heterogeneous “ground-glass” sclerosis on CT imaging, cherubism was long mistaken for a craniofacial subtype of fibrous dysplasia. In 1999, however, the distinct genetic basis for cherubism was mapped to chromosome 4p16.3 and the SH-3 binding protein SH3BP2. But while there are already three suspected cases of fibrous dysplasia amongst archaeological populations, no definitive cases of cherubism have yet been reported in historical populations. In the current study we describe micro- and macro-structural changes in the face of a 17<sup>th</sup>
century Joseon Dynasty Korean mummy which may coincide with the clinic-pathologic and radiologic features of cherubism.</p>
</div>
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