• Users Online: 541
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Partners Subscribe Contacts Login 
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 1  |  Page : 59-63

Dental phenotype of multiple impacted supernumerary teeth in Wiedemann–Steiner syndrome


1 Department of Oral Medicine and Radiology, Maulana Azad Institute of Dental Sciences, New Delhi, India
2 Department of Paediatrics, Maulana Azad Medical College, New Delhi, India

Correspondence Address:
Dr. Sunita Gupta
Department of Oral Medicine and Radiology, Maulana Azad Institute of Dental Sciences, Bahadur Shah Zafar Marg, New Delhi - 110 002
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jclpca.jclpca_12_19

Rights and Permissions

Wiedemann–Steiner syndrome (WSS) is a rare autosomal dominant disorder characterized by short stature, intellectual disability, developmental delay, hypertrichosis cubiti, and dysmorphic facial features (thick eyebrows with a lateral flare, long eyelashes, vertically narrow and downslanting palpebral fissures, and wide nasal bridge). Dental findings reported with this syndrome are premature eruption of dentition, malocclusion, hypodontia, cleft palate, and retrognathia. We report a 12-year-old boy with a heterozygous two-base pair deletion in exon 22 of the KMT2A gene, resulting in a frameshift and premature truncation of the protein 17 amino acids downstream to codon 1974. Our patient exhibited multiple impacted supernumerary teeth which have never been reported earlier. This extends the dental phenotypic spectrum of WSS. To the best of our knowledge, this is the first reported case from India.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed266    
    Printed15    
    Emailed0    
    PDF Downloaded14    
    Comments [Add]    

Recommend this journal