Resident: Adam J. Bottrill
Date: 05MAR10
Region: Providence
Article title: Ectodermal Dysplasia
Author(s): Wright, T. et al
Journal: ?
Page #s: pp. 17211724
Year: ?
Major topic: Ectodermal Dysplasia
Minor topic(s): N/A
Type of Article: Topic Summary
Main Purpose: Describe the different clinical characteristics of Ectodermal Dysplasia (ED)
Overview of method of research: Summary
Key points in the article discussion:
A. General
1. Includes all conditions having a developmental defect that embryologically affects the ectoderm.
2. Over 120 conditions classifiable as ED’s.
3. More recent definitions of ED include any one of the following: hypotrichosis, hypodontia, onychodysplasia, anhydrosis … PLUS ectodermal involvement.
4. Most types remain a purely clinical diagnosis… not yet molecularly characterized.
5. Other non-ectoderm structures may be involved: limbs, bones, palate, neuro, organs, repro.
6. Hair 90%, skin 85%, teeth 80%, nails 75%, face 72%
7. No FH, and often hard to diagnose… VERY few are localized to specific DNA and inheritance.
a. female carriers often have tooth involvement.
8. Most treatment is only preventative or palliative… there is no cure.
9. Dental implants are often treatment of choice as early as during teenage yrs.
Assessment of article: Keep it simple stupid.
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