Amelogenesis imperfecta is a genetic disorder that affects the development of enamel in teeth, causing them to appear abnormally small, discolored, pitted or grooved. It is caused by mutations in genes responsible for enamel protein production. Symptoms include soft, thin enamel that leads to highly sensitive teeth that are prone to cavities, damage, and early loss. Treatment may involve crowns, extractions, and dental implants or dentures in severe cases.

1. By: Janine R. Rumbaoa

2.  a disorder of tooth development
presents with abnormal formation of the enamel or external
layer of teeth.
due to the malfunction of the proteins in the enamel:
ameloblastin, enamelin, tuftelin and amelogenin.
 condition causes teeth to be unusually
small, discolored, pitted or grooved, and prone to rapid wear
and breakage.
 these defects, which vary among affected individuals, can
affect both primary (baby) teeth and permanent teeth.
 How common is amelogenesis imperfecta?
The exact incidence of amelogenesis imperfecta is uncertain.
Estimates vary widely, from 1 in 700 people in northern Sweden
to 1 in 14,000 people in the United States.

3.  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.

4.  Mutations in the AMELX, ENAM, and
MMP20 genes cause amelogenesis
imperfecta.

5.  The enamel of the tooth is soft and
thin. The teeth appear yellow and
are easily damaged. Both baby teeth
and permanent teeth are affected.

6.  X-ray
showing lack of enamel opacity and a
pathological loss of enamel in patient with
amelogenesis imperfecta
 Fullcrowns are sometimes being used to
compensate for the soft enamel.Usually
stainless steel crowns are used in children
which 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.