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耳齿综合征、眼-面-心-牙(OFCD)综合征与缺叶牙:儿科放射科医生关注的牙齿疾病

Otodental syndrome, oculo-facio-cardio-dental (OFCD) syndrome, and lobodontia: dental disorders of interest to the pediatric radiologist.

作者信息

Gorlin R J

机构信息

University of Minnesota Schools of Dentistry and Medicine, 515 Delaware St S. E., 16-127, Minneapolis, MN 55455 USA.

出版信息

Pediatr Radiol. 1998 Oct;28(10):802-4. doi: 10.1007/s002470050469.

Abstract

Three unusual dental anomalies are presented which should be of interest to the pediatric radiologist: otodental syndrome - an autosomal dominant syndrome of bizarre, greatly enlarged teeth with bulbous crowns (globodontia) that spares the incisors, in combination with sensorineural hearing loss; oculo-facio-cardio-dental syndrome - an X-linked dominant syndrome that is lethal in males, characterized by congenital cataracts, unusual facies, atrial septal defect (ASD), ventricular septal defect (VSD), and canine teeth that cease to grow only when they cut off their own blood supply by growing through the orbit and lower border of mandible; and lobodontia - a dominant disorder characterized by teeth whose crowns resemble those of a wolf.

摘要

本文介绍了三种不寻常的牙齿异常情况,儿科放射科医生可能会对此感兴趣:耳牙综合征——一种常染色体显性综合征,牙齿异常且极大,牙冠呈球根状(巨牙症),切牙不受影响,同时伴有感音神经性听力损失;眼面心牙综合征——一种X连锁显性综合征,男性患者致死,其特征为先天性白内障、面容异常、房间隔缺损(ASD)、室间隔缺损(VSD),以及犬齿在生长过程中穿过眼眶和下颌骨下缘切断自身血供后才停止生长;还有叶状牙——一种显性疾病,其特征是牙齿的牙冠类似狼的牙冠。

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