Contemporary Clinical Dentistry
   
  Home | About us | Editorial board | Search
Ahead of print | Current Issue | Archives | Advertise
Instructions | Online submission| Contact us | Subscribe |

 

Login  | Users Online: 2474  Print this pageEmail this pageSmall font sizeDefault font sizeIncrease font size 

CASE REPORT
Year : 2013  |  Volume : 4  |  Issue : 3  |  Page : 363-365

Clinical spectrum of silver - Russell syndrome


1 Department of Orthodontics and Dentofacial Orthopaedics, Amrita School of Dentistry, Kochi, Kerala, India
2 Department of Pediatric Dentistry, Amrita School of Dentistry, Kochi, Kerala, India

Correspondence Address:
Sapna N.K. Varma
Department of Orthodontics and Dentofacial Orthopaedics, Amrita School of Dentistry, AIMS Campus, Ponekkara, Kochi 682 041, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0976-237X.118346

Rights and Permissions

Silver - Russell syndrome is a clinically and genetically heterogenous condition characterized by severe intrauterine and postnatal growth retardation, craniofacial disproportion and normal intelligence downward curvature of the corner of the mouth, syndactyly and webbed fingers. Diagnosis of Silver - Russell syndrome remains clinical; no definite etiology or specific tests have been established. In the recent years, it has been shown that more than 38% of patients have hypomethylation in the imprinting control region 1 of 11p15 and one-tenth of patients carry a maternal uniparental disomy of chromosome seven. The pathophysiological mechanisms resulting in the Silver - Russell phenotype remain unknown despite the recent progress in deciphering the molecular defects associated with this condition. This case report describes the clinical features of Silver - Russell syndrome in a father and daughter.


[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
    Viewed2636    
    Printed38    
    Emailed0    
    PDF Downloaded185    
    Comments [Add]    
    Cited by others 3    

Recommend this journal