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CASE REPORT
Year : 2017  |  Volume : 8  |  Issue : 3  |  Page : 469-472

Nance–Horan syndrome: A rare case report


1 Department of Pedodontics and Preventive Dentistry, Inderprastha Dental College and Hospital, Ghaziabad, Uttar Pradesh, India
2 Department of Prosthodontics, Crown and Bridge, Inderprastha Dental College and Hospital, Ghaziabad, Uttar Pradesh, India
3 Department of Oral and Maxillofacial Surgery, Inderprastha Dental College and Hospital, Ghaziabad, Uttar Pradesh, India
4 Department of Public Health Dentistry, Inderprastha Dental College and Hospital, Ghaziabad, Uttar Pradesh, India

Correspondence Address:
Shambhu Sharma
Flat No. 93, Prayag Apartments, Vasundhara Enclave, New Delhi - 110 096
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ccd.ccd_232_17

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Dentofacial anomalies may guide us to the diagnosis of many congenital and hereditary syndromes. A 9-year-old boy was diagnosed with Nance–Horan syndrome. This syndrome is an extremely rare X-linked genetic disorder which is entirely expressed in males with semi-dominant transmission which results from mutations occurring in male gametes. It is characterized by facial dysmorphism such as long face, prominent nose and mandibular prognathism, ocular abnormalities such as congenital cataract, microcornea, microphthalmia and strabismus, and dental anomalies including mulberry molars and screwdriver-shaped incisors. Heterozygous females inherit this disease and also suffer from this syndrome but in a milder form. Approximately one-third of the affected males show signs of developmental delay and intellectual abnormalities. This syndrome is very rare and the incidence of the disease has not been established so far. The present article describes the clinical and radiological features and the genetic implications of this syndrome.


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