天使综合征合并癫痫、智力发育迟缓及牙龈纤维瘤病（拉蒙综合征）：一例报告
Cherubism combined with epilepsy, mental retardation and gingival fibromatosis (Ramon syndrome): a case report.
作者信息
Suhanya J, Aggarwal Chakshu, Mohideen Khadijah, Jayachandran S, Ponniah I
机构信息
Department of Oral Medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Chennai, 600 003, India.
出版信息
Head Neck Pathol. 2010 Jun;4(2):126-31. doi: 10.1007/s12105-009-0155-9. Epub 2009 Dec 11.
Abstract
Cherubism is an inherited, autosomal dominant disorder that characteristically affects the jaws of children. The disease typically manifest as a bilateral swelling with associated submandibular lymph node enlargements and usually regresses as age advances. The disease is microscopically indistinguishable from other giant cell lesions and is essentially a clinical diagnosis. The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred as Ramon syndrome. We report a case of Ramon syndrome in an 8 year old girl.
摘要
颌骨肥大症是一种遗传性常染色体显性疾病，典型地影响儿童的颌骨。该病通常表现为双侧肿胀并伴有颌下淋巴结肿大，且通常随着年龄增长而消退。在显微镜下，这种疾病与其他巨细胞病变无法区分，本质上是一种临床诊断。颌骨肥大症与牙龈纤维瘤病、癫痫、智力迟钝、生长发育迟缓及多毛症相关，被称为拉蒙综合征。我们报告一例8岁女孩患拉蒙综合征的病例。
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