遗传性颅面骨纤维发育不良

Inherited craniofacial fibrous dysplasia.

作者信息

Pierce A M, Wilson D F, Goss A N

出版信息

Oral Surg Oral Med Oral Pathol. 1985 Oct;60(4):403-9. doi: 10.1016/0030-4220(85)90263-4.

Abstract

Craniofacial fibrous dysplasia occurring in a mother and two of her three daughters is reported. In all three patients, the condition was characterized by extensive bilateral and bimaxillary involvement. All patients exhibited radiographic evidence of abnormal maxillary and mandibular bone and tooth displacement. Morphologic examination of deciduous teeth from the daughters showed replacement of normal pulp tissue by fibro-osseous lesion. Because of the familial occurrence of the cases, it is proposed that they comprise an example of inherited craniofacial fibrous dysplasia.

摘要

据报道，一名母亲及其三个女儿中的两个患有颅面部纤维发育异常。在所有三名患者中，病情的特征是双侧和双颌广泛受累。所有患者均表现出上颌骨和下颌骨异常以及牙齿移位的影像学证据。对女儿乳牙的形态学检查显示，正常牙髓组织被纤维骨病变所取代。由于这些病例为家族性发病，因此推测它们构成了遗传性颅面部纤维发育异常的一个实例。