Cherubism: A Case Report with Surgical Intervention
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Cherubism: A Case Report with Surgical Intervention. / Friedrich, Reinhard E; Scheuer, Hanna A; Zustin, Jozef; Grob, Tobias.
in: ANTICANCER RES, Jahrgang 36, Nr. 6, 06.2016, S. 3109-15.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Cherubism: A Case Report with Surgical Intervention
AU - Friedrich, Reinhard E
AU - Scheuer, Hanna A
AU - Zustin, Jozef
AU - Grob, Tobias
N1 - Copyright© 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.
PY - 2016/6
Y1 - 2016/6
N2 - Cherubism is a rare benign, autosomal-dominant hereditary fibro-osseous condition predominantly affecting the jaws. Symmetrical cyst-like expansions of the jaws cause the characteristic facial swellings. The disease is often associated with severe malposition of teeth. The gene for cherubism is src homology 3 binding protein 2 (SH3BP2) located on chromosome 4p16.3. The repeated experience of this self-limiting disorder in affected individuals published in the medical literature has resulted in a wait-and-see strategy regarding therapeutic options. Indeed, cessation and regression of even large bone expansions can be expected in early adulthood. Nevertheless, severe facial disfiguring and functional impairment can make surgical intervention necessary. This report details the surgical procedures carried out in a patient with progressive and disfiguring jaw expansions at the end of adolescence, the mutation of SH3BP2 gene, and the limited effect of surgically assisted orthodontic tooth movement in a patient with disease-associated impaired tooth development and tooth eruption.
AB - Cherubism is a rare benign, autosomal-dominant hereditary fibro-osseous condition predominantly affecting the jaws. Symmetrical cyst-like expansions of the jaws cause the characteristic facial swellings. The disease is often associated with severe malposition of teeth. The gene for cherubism is src homology 3 binding protein 2 (SH3BP2) located on chromosome 4p16.3. The repeated experience of this self-limiting disorder in affected individuals published in the medical literature has resulted in a wait-and-see strategy regarding therapeutic options. Indeed, cessation and regression of even large bone expansions can be expected in early adulthood. Nevertheless, severe facial disfiguring and functional impairment can make surgical intervention necessary. This report details the surgical procedures carried out in a patient with progressive and disfiguring jaw expansions at the end of adolescence, the mutation of SH3BP2 gene, and the limited effect of surgically assisted orthodontic tooth movement in a patient with disease-associated impaired tooth development and tooth eruption.
M3 - SCORING: Journal article
C2 - 27272835
VL - 36
SP - 3109
EP - 3115
JO - ANTICANCER RES
JF - ANTICANCER RES
SN - 0250-7005
IS - 6
ER -