KBG syndrome: review of the literature and findings of 5 affected patients
| Autor: | Harleen Kumar, Angus Cameron, N T Prabhu |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
Adult
Male Pediatrics medicine.medical_specialty Cephalometry media_common.quotation_subject Dentistry Dwarfism Malocclusion Angle Class I Malocclusion Angle Class II Short stature Bone and Bones Craniofacial Abnormalities Young Adult stomatognathic system Intellectual Disability medicine Humans Maxillary central incisor Abnormalities Multiple Expressivity (genetics) Craniofacial Child General Dentistry media_common Daughter business.industry Tooth Abnormalities Syndrome Craniometry medicine.disease Incisor stomatognathic diseases Otorhinolaryngology Macrodontia (tooth) Child Preschool Surgery Female Oral Surgery medicine.symptom Malocclusion business Follow-Up Studies |
| Zdroj: | Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics. 108(3) |
| ISSN: | 1528-395X |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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