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

Abstract

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.

Authors+Show Affiliations

Department of Pediatric Dentistry, Westmead Hospital, Sydney, Australia.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

19716495

Citation

Kumar, Harleen, et al. "KBG Syndrome: Review of the Literature and Findings of 5 Affected Patients." Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics, vol. 108, no. 3, 2009, pp. e72-9.
Kumar H, Prabhu N, Cameron A. KBG syndrome: review of the literature and findings of 5 affected patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;108(3):e72-9.
Kumar, H., Prabhu, N., & Cameron, A. (2009). KBG syndrome: review of the literature and findings of 5 affected patients. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics, 108(3), e72-9. https://doi.org/10.1016/j.tripleo.2009.04.035
Kumar H, Prabhu N, Cameron A. KBG Syndrome: Review of the Literature and Findings of 5 Affected Patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;108(3):e72-9. PubMed PMID: 19716495.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - KBG syndrome: review of the literature and findings of 5 affected patients. AU - Kumar,Harleen, AU - Prabhu,Neeta, AU - Cameron,Angus, PY - 2008/12/17/received PY - 2009/04/16/revised PY - 2009/04/29/accepted PY - 2009/9/1/entrez PY - 2009/9/1/pubmed PY - 2009/12/16/medline SP - e72 EP - 9 JF - Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics JO - Oral Surg Oral Med Oral Pathol Oral Radiol Endod VL - 108 IS - 3 N2 - 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. SN - 1528-395X UR - https://www.unboundmedicine.com/medline/citation/19716495/KBG_syndrome:_review_of_the_literature_and_findings_of_5_affected_patients_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1079-2104(09)00302-3 DB - PRIME DP - Unbound Medicine ER -