Download What is Amelogenesis Imperfecta

What is Amelogenesis Imperfecta?
1. It is an abnormal formation of the enamel.
2. It is a disorder that affects both primary dentition (baby teeth) and permanent
dentition (permanent teeth).
3. Conditions may vary, they can have smaller teeth than normal (microdontia),
discoloring, grooved or pitted, and prone to fractures.
4. Amelogenesis Imperfecta is inherited
5. A ratio of 14,000 people to 1 is affected in the United States.
Treatment
- Since the enamel is easily fractured and damaged, it will affect the appearance of
teeth.
As hygienist we can advise the patient to seek a dentist and if they don’t have one we’ll provide
them with one. The treatment depends on the severity of the problem, the dentist will determine that
after diagnose. Full crowns will improve the appearance of the teeth and protect them from damage.
Clinical and Hereditary Characteristics of Four Main AI Types (Table 1) [8]
Type
Clinical
Appearance
Enamel
Thickness
Radiographic
Appearance
Inheritance
Hypoplastic
(Type I)
Crowns size varies
from small to normal,
small teeth may lack
proxmial contacts,
color varies from
normal to opaque
white – yellow brown
Varies from thin and
smooth to normal
thickness with
grooves, furrows
and/or pits
Enamel has normal to
slightly reduced
contrast/ thin
Autosomal
dominant,
recessive, or Xlinked
Hypomaturation
(Type II)
Varies from creamy
opaque to marked
yellow/brown, surface
of teeth soft and rough,
dental sensitivity and
open bite common
Normal thickness
with enamel that
often chips and
abrades easily
Enamel has contrast
similar to or > than
dentin, unerupted
crowns have normal
morphology
Autosomal
dominant,
recessive, or Xlinked
Hypocalcified
(Type III)
Opaque white to
yellow-brown, soft
rough enamel surface,
dental sensitivity and
open bite common,
heavy calculus
formation common
Normal thickness
with enamel that
often chips and
abrades easily
Enamel has contrast
similar to or < dentin,
unerupted crowns
have normal
morphology
Autosomal
dominant,
recessive
Hypomaturation/
Hypoplasia/
Taurodontism
(Type IV)
White/Yellow- Brown
mottled, teeth can
appear small and lack
proximal contact
Reduced,
hypomineralized
areas and pits
Enamel contrast
normal to slightly >
dentin, large pulp
chambers
Autosomal
dominant
-
http://www.dentistry.unc.edu/research/defects/pages/ai.htm
Crowns are sometimes being used to compensate for the soft enamel. Usually stainless
steel crowns are used in children who may be replaced by porcelain once they reach
adulthood. In the worst case scenario, the teeth may have to be extracted and implants
or dentures are required.
People afflicted with Amelogenesis Imperfecta have teeth with abnormal color: yellow,
brown or grey. The teeth have a higher risk for dental cavities and are hypersensitive to
temperature changes. This disorder can afflict any number of teeth.
Mutations in the AMELX, ENAM, MMP20, and KLK-4 genes have been found to cause
Amelogenesis Imperfecta (non-syndromic form). The AMELX, ENAM, KLK-4 and
MMP20 genes provide instructions for making proteins that are essential for normal
tooth development. These proteins are involved in the formation of enamel, which is a
hard, calcium-rich material that forms the protective outer layer of each tooth. Mutations
in any of these genes alter the structure of these proteins or prevent the genes from
making any protein at all.