Transcript Hypohydrotic ectoderma dysplasia

PRESENTED BY:
GUADALUPE COMPEAN, DENTAL HYGIENE STUDENT
DIANA MARTINEZ, DENTAL HYGIENE STUDENT
LAMAR INSTITUTE OF TECHNOLOGY
DENTAL HYGIENE PROGRAM
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Abnormal development of
ectodermal structures
Rare condition
Occurs in an estimated one per
100,000 live births
Linked to the X-chromosome
Caused by the mutations of
different genes before birth
EDA (Ectodysplacin) Gene-Most
common
EDAR (Ectodysplacin Receptor)
Gene-Least common
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Genetic
Inherited from one or
both parents
Diagnosed at or shortly
after birth
Men more affected than
women
Considered X-linked if the
mutated gene is located
on the X chromosome
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Missing teeth
Thin hair
Dry, scaly skin
Poor sweat glands
High fevers
Inadequate
development of
fingernails
Facial Abnormalities
oProminent supra orbital ridges
oSunken cheeks and eyes
oFrontal bossing
oThick lips
oLarger chin
oSaddle nose
Malformed teeth
oPegged or conical shaped
oWidely spaced
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Full medical/dental history
Ask additional questions
Family history
Perform a thorough head & neck
exam
Oral exam
Radiographs
Refer for genetic testing
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Identifying subtle
signs from a carrier
such as slightly sparse
and thin hair, coneshaped teeth,
hypodontia, and
partial sweat
dysfunction
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Identify appropriate
treatment
Determine if dentures
are needed
Maintain the alveolar
ridge for implant
placement
Achieve a fully
functional dentition
Aesthetics
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Dentures
Dental implants
Orthodontics
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Genetic research is developing fast and making advances
in understanding the genes known for tooth
development.
In the future, tooth development and stem cell research
could indicate the possibility to create new teeth
artificially.
Clauss, F., Chassaing, N., Smahi A., Vincent, M. C.,Calvas, P., Molla, M., Lesot, H., Alembik,Y., HadjRabia, S., Bodemer, C., Maniere,M. C., & Schmittbuhl, M. (2010). X-linked and autosomal
recessive hypohidrotic ectodermal dysplasia: Genotypic-dental phenotypic
findings. Clinical Genetics, 78, 257-266.
Deshpande, S. N., & Kumar, V. (2010). Ectodermal dysplasia maxillary and mandibular alveolar
reconstruction with dental rehabilitation: A case report and review of the literature.
Indian Journal of Plastic Surgery, 43(1), 92-96.
Jayantilal, P. R., Rajesh, S., Naveen Y. G., &Nandini, P. (2010). Treatment considerations for
a patient with ectodermal dysplasia: A case report. Journal of International Oral
Health, 2(4), 73-78.
Lukes, S., & Sherry, J. S. (2010). Hypohidrotic Ectodermal Dysplasia. RDH, 31(1), 92-96.
Retrieved from http://www.rdhmag.com
Manuja, N., Passi, S., Pandit, I. K., & Singh, N. (2011). Management of a Case of Ectodermal
Dysplasia: A Multidisciplinary Approach. Journal of Dentistry of Children, 78(2), 107-110.
Shah, K. N. (2010, January 24). Ectodermal Dysplasia Treatment & Management. Medscape
Reference: Drugs, Diseases, & Procedures. Retrieved from
http://emedicine.medscape.com/article/1111595-overview
The Swedish Information Center for Rare Diseases. (2011). Hypohidrotic ectodermal dysplasia.
Retrieved from http://socialstyrelsen.se/raredisease/hypohidroticectodermal
dysplasia#anchor_14