@article{ART002137317},
author={김미애 and 박지현 and Yonjoo Mah},
title={Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report},
journal={Journal of the Korean academy of pediatric dentistry},
issn={1226-8496},
year={2016},
volume={43},
number={3},
pages={313-319},
doi={10.5933/JKAPD.2016.43.3.313}
TY - JOUR
AU - 김미애
AU - 박지현
AU - Yonjoo Mah
TI - Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report
JO - Journal of the Korean academy of pediatric dentistry
PY - 2016
VL - 43
IS - 3
PB - The Korean Academy Of Pediatric Dentistry
SP - 313
EP - 319
SN - 1226-8496
AB - Wolf-Hirschhorn syndrome (WHS), associated with the deletion of the short arm of chromosome 4, causes multiple congenital malformations. Patients suffer from various deformities, including mental and growth disorders, epilepsy, hypotonia, congenital heart defects, and atypical craniofacial features. The “Greek warrior helmet appearance”is the most characteristic feature, with a prominent glabella, high arched eyebrow, broad nasal bridge, and hypertelorism.
Cleft lip with or without cleft palate is observed in 30% of patients. Dental structure anomalies also exist including multiple tooth agenesis and over-retained primary molars caused by MSX1 gene impairment, and coneshaped and taurodontic teeth.
This case, a 9-year-old girl with WHS, showed intellectual disability, delayed growth development, previous occurrence of seizures, otitis media, and the typical facial features of WHS. Dental findings included multiple congenital missing teeth, over-retained primary teeth, and severe caries on the primary molars. Dental treatments were performed under general anesthesia.
This report documents the characteristics of WHS, including general and oral features, and discusses the importance of oral hygiene and preventive dental management.
KW - Wolf-Hirschhorn syndrome;Dental findings;Preventive dental management
DO - 10.5933/JKAPD.2016.43.3.313
ER -
김미애, 박지현 and Yonjoo Mah. (2016). Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report. Journal of the Korean academy of pediatric dentistry, 43(3), 313-319.
김미애, 박지현 and Yonjoo Mah. 2016, "Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report", Journal of the Korean academy of pediatric dentistry, vol.43, no.3 pp.313-319. Available from: doi:10.5933/JKAPD.2016.43.3.313
김미애, 박지현, Yonjoo Mah "Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report" Journal of the Korean academy of pediatric dentistry 43.3 pp.313-319 (2016) : 313.
김미애, 박지현, Yonjoo Mah. Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report. 2016; 43(3), 313-319. Available from: doi:10.5933/JKAPD.2016.43.3.313
김미애, 박지현 and Yonjoo Mah. "Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report" Journal of the Korean academy of pediatric dentistry 43, no.3 (2016) : 313-319.doi: 10.5933/JKAPD.2016.43.3.313
김미애; 박지현; Yonjoo Mah. Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report. Journal of the Korean academy of pediatric dentistry, 43(3), 313-319. doi: 10.5933/JKAPD.2016.43.3.313
김미애; 박지현; Yonjoo Mah. Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report. Journal of the Korean academy of pediatric dentistry. 2016; 43(3) 313-319. doi: 10.5933/JKAPD.2016.43.3.313
김미애, 박지현, Yonjoo Mah. Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report. 2016; 43(3), 313-319. Available from: doi:10.5933/JKAPD.2016.43.3.313
김미애, 박지현 and Yonjoo Mah. "Dental Treatment of a Wolf-Hirschhorn Syndrome Patient: A Case Report" Journal of the Korean academy of pediatric dentistry 43, no.3 (2016) : 313-319.doi: 10.5933/JKAPD.2016.43.3.313