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Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009 Sep;108(3):e72-9. doi: 10.1016/j.tripleo.2009.04.035.

KBG syndrome: review of the literature and findings of 5 affected patients.

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Department of Pediatric Dentistry, Westmead Hospital, Sydney, Australia.


KBG syndrome is a rare, multiple congenital anomaly/mental retardation (MCA/MR) syndrome characterized by neurological disturbance, short stature, a distinct craniofacial appearance, and skeletal anomalies. It is likely to be autosomal dominant in nature with a wide range of expressivity in its clinical features. Dentally, macrodontia, particularly of the maxillary permanent central incisors is a common finding. The aim of this article was to review the familiar clinical presentations of this syndrome and to highlight previously unreported findings of generalized macrodontia and shovel-shaped incisors. Dental and clinical findings of 2 affected brothers with a 3-year follow-up of their dental progress following orthodontic treatment are outlined. Additionally, dental and clinical findings of an affected mother and her daughter, and another sporadic case are also presented.

[Indexed for MEDLINE]

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