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Kabuki syndrome: description of dental findings in 8 patients.
Clin Genet. 1999 Aug; 56(2):154-7.CG

Abstract

The cardinal features of Kabuki (Niikawa-Kuroki) syndrome (KS) include characteristic facial dysmorphic features, mild to moderate mental deficiency, skeletal abnormalities, dermatoglyphic abnormalities, and postnatal growth retardation. We identified 8 patients with KS in a genetics clinic over the past 5 years. All were Caucasians, except for 2 who were of mixed Aboriginal and Caucasian descent. All had the facial gestalt, the dermatoglyphic abnormalities characteristic of the syndrome, and developmental delay. Dental abnormalities of permanent teeth were seen in all 8 cases; 6 had missing lower incisors. Five patients had uniquely abnormal upper incisor teeth shape; the upper incisors had a 'flat head' screwdriver-shaped appearance. Other dental abnormalities included missing lower lateral incisors, missing second premolars, and ectopic upper 6-year molars. We believe the presence of the unique dental findings will prove useful in the diagnostic assessment of individuals with KS.

Authors+Show Affiliations

Department of Human Genetics, University of Manitoba and Children's Hospital, Winnipeg, Canada.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

10517254

Citation

Mhanni, A A., et al. "Kabuki Syndrome: Description of Dental Findings in 8 Patients." Clinical Genetics, vol. 56, no. 2, 1999, pp. 154-7.
Mhanni AA, Cross HG, Chudley AE. Kabuki syndrome: description of dental findings in 8 patients. Clin Genet. 1999;56(2):154-7.
Mhanni, A. A., Cross, H. G., & Chudley, A. E. (1999). Kabuki syndrome: description of dental findings in 8 patients. Clinical Genetics, 56(2), 154-7.
Mhanni AA, Cross HG, Chudley AE. Kabuki Syndrome: Description of Dental Findings in 8 Patients. Clin Genet. 1999;56(2):154-7. PubMed PMID: 10517254.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Kabuki syndrome: description of dental findings in 8 patients. AU - Mhanni,A A, AU - Cross,H G, AU - Chudley,A E, PY - 1999/10/12/pubmed PY - 1999/10/12/medline PY - 1999/10/12/entrez SP - 154 EP - 7 JF - Clinical genetics JO - Clin. Genet. VL - 56 IS - 2 N2 - The cardinal features of Kabuki (Niikawa-Kuroki) syndrome (KS) include characteristic facial dysmorphic features, mild to moderate mental deficiency, skeletal abnormalities, dermatoglyphic abnormalities, and postnatal growth retardation. We identified 8 patients with KS in a genetics clinic over the past 5 years. All were Caucasians, except for 2 who were of mixed Aboriginal and Caucasian descent. All had the facial gestalt, the dermatoglyphic abnormalities characteristic of the syndrome, and developmental delay. Dental abnormalities of permanent teeth were seen in all 8 cases; 6 had missing lower incisors. Five patients had uniquely abnormal upper incisor teeth shape; the upper incisors had a 'flat head' screwdriver-shaped appearance. Other dental abnormalities included missing lower lateral incisors, missing second premolars, and ectopic upper 6-year molars. We believe the presence of the unique dental findings will prove useful in the diagnostic assessment of individuals with KS. SN - 0009-9163 UR - https://www.unboundmedicine.com/medline/citation/10517254/Kabuki_syndrome:_description_of_dental_findings_in_8_patients_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0009-9163&date=1999&volume=56&issue=2&spage=154 DB - PRIME DP - Unbound Medicine ER -