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A rare syndrome with unusual dental findings: Oculo-facio-cardio-dental syndrome.

作者信息

Verma Geeta, Singh Gulshan Kumar, Tandon Pradeep, Verma Sneh Late

机构信息

Department of Orthodontics and Dentofacial Orthopedics, Babu Banarasi Das College of Dental Sciences, Lucknow, Uttar Pradesh, India.

Faculty of Dental Sciences, Chhatrapati Shahuji Maharaj Medical University, Lucknow, Uttar Pradesh, India.

出版信息

J Oral Maxillofac Pathol. 2014 May;18(2):331. doi: 10.4103/0973-029X.140928.

DOI:10.4103/0973-029X.140928
PMID:25328326
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4196314/
Abstract

Radiculomegaly of a tooth is a rare condition. When it is associated with other dental abnormalities, facial dysmorphism and congenital cataracts, the condition is named as oculofaciocardiodental (OFCD) syndrome. This is an X-linked dominant trait, reported only in women, suggesting that it is lethal in males. The most consistent and pathognomic dental finding of OFCD is radiculomegaly (extremely long roots), particularly of the canines and occasionally of other teeth including premolars and incisors, which can easily be diagnosed on dental panoramic radiographs by a dentist or an orthodontist. Till date, 21 cases have been reported worldwide. The aim of this report is to present a new case of a 24 year old female affected by this syndrome and to evaluate it from a dental perspective to call the attention of dentists to this rare anomaly.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/463650153895/JOMFP-18-331b-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/638c362964d9/JOMFP-18-331b-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/95877bd2b0e1/JOMFP-18-331b-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/632228b6546f/JOMFP-18-331b-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/1b680cd9c9aa/JOMFP-18-331b-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/463650153895/JOMFP-18-331b-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/638c362964d9/JOMFP-18-331b-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/95877bd2b0e1/JOMFP-18-331b-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/632228b6546f/JOMFP-18-331b-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/1b680cd9c9aa/JOMFP-18-331b-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5544/4196314/463650153895/JOMFP-18-331b-g005.jpg

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本文引用的文献

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Oculo-facio-cardio-dental syndrome in a girl and her mother.一名女孩及其母亲患眼-面-心-牙综合征。
Indian J Hum Genet. 2010 Sep;16(3):169-71. doi: 10.4103/0971-6866.73416.
2
Oculofaciocardiodental syndrome and orthodontics.眼面心牙综合征与正畸学
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Webbed fingers.蹼状指
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Oculo-facio-cardio-dental syndrome: skewed X chromosome inactivation in mother and daughter suggest X-linked dominant Inheritance.眼-面-心-牙综合征:母亲和女儿的X染色体失活偏斜提示X连锁显性遗传。
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