You are here: Home / Rare Disease Information / Rare Disease Database

Search Rare Diseases

Enter a disease name or synonym to search NORD's database of reports.

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

Amelogenesis Imperfecta

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

Synonyms of Amelogenesis Imperfecta

  • No synonyms found.

Disorder Subdivisions

  • Hypocalcified AI (Type III)
  • Hypomaturation AI (Type II)
  • Hypomaturation/Hypoplasia/Taurodontism (Type IV)
  • Hypoplastic AI (Type I)

General Discussion

Amelogenesis imperfecta (AI) refers to a group of rare, inherited disorders characterized by abnormal enamel formation. The term is restricted to those disorders of enamel development not associated with other defects of the body. In AI, the layer of enamel is thin so that the teeth appear to be discolored, showing the color of the materials under the enamel. The teeth usually appear brown or some variant of that color.

Clinical researchers usually classify AI into four main types of which 14 subtypes are recognized. The main types are based on enamel effects and the subtypes are based on clinical appearance and mode of inheritance. The main types are: hypoplastic (Type 1); hypomaturation (Type II); hypocalcified (Type III); and hypomaturation/hypoplasia/taurodontism (Type IV). Amelogenesis imperfecta may be inherited as an X-linked, autosomal dominant, or autosomal recessive genetic trait, depending on the type.

Amelogenesis Imperfecta Resources

Organizations:

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

NORD's Rare Disease Information Database is copyrighted and may not be published without the written consent of NORD.

 
Copyright ©2014 NORD - National Organization for Rare Disorders, Inc. All rights reserved.
The following trademarks/registered service marks are owned by NORD: NORD, National Organization for Rare Disorders, the NORD logo, RareConnect. .