alexa Unusual Enamel Hypoplasia Associated with Teeth Mobility in a 13 Year Old Girl with Wilson Disease
ISSN: 2157-7412

Journal of Genetic Syndromes & Gene Therapy
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Case Report

Unusual Enamel Hypoplasia Associated with Teeth Mobility in a 13 Year Old Girl with Wilson Disease

Nehal F. Hassib*, Maie A. Mahmoud, Nevin M. Talaat and Tarek H. El-Badry

National Research Centre, Giza, Egypt

*Corresponding Author:
Nehal F. Hassib
National Research Centre
Giza, Egypt
E-mail: [email protected]

Received date: June 15, 2012; Accepted date:July 14, 2012; Published date: July 20, 2012

Citation: Hassib NF, Mahmoud MA, Talaat NM, El-Badry TH (2012) Unusual Enamel Hypoplasia Associated with Teeth Mobility in a 13 Year Old Girl with Wilson Disease. J Genet Syndr Gene Ther 3:118. doi:10.4172/2157-7412.1000118

Copyright: © 2012 Hassib NF, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

 

Abstract

Wilson disease is an autosomal recessive disorder caused by mutations in the ATP7B gene. It is characterized by the progressive accumulation of copper in the body leading to liver cirrhosis and neuropsychological deterioration. This case may be the first one reported Wilson disease in association with remarkable enamel hypoplasia and teeth mobility leading to severe teeth destruction and pulp exposure. The objective of this investigation was to introduce the dental management for a 13 year old female patient with Wilson disease. The patients restored her smile and she was highly satisfied of the dental work. In conclusion, the dental management of patients with Wilson disease should become the focus of research because of the difficulty in patients’ management as our patient was suffering from dystonia restricting the mouth opening and in addition of being a mouth breather which affected the time and quality of the dental work

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