Hereditary Defects of Enamel

Department of Pediatric Dentistry

Lutheran Medical Center

Date: 02/05/2010

Article title: Hereditary Defects of Enamel

Author(s): J Tim Wright

Journal: Text: Dentin Enamel: Formation to Destruction

Volume (number):

Month, Year: 1995

Major topic: Enamel defects

Minor topics: Syndromes associated with enamel defects

Type of Article: Text, educational

Main Purpose: To Present the current knowledge of hereditary enamel disorders

Overview of method of research: Text based on a review of the literature

Findings:

This text has a ton of information. It is an important read, but not important for me to copy again. After board review, my feeling is that by knowing the information in Table 1, you’ll be well prepared for the exam.

I reproduced it here and added some pertinent information. I did not add any histology as it is probably not relevant to what we need for the boards.

Key points/Summary :

• Syndromic conditions
• Metabolic disorders
• Mucopolysaccharidosis type IV (mucopolysaccharides accumulate around developing teeth)
• Hypophosphatasia (abnormal cementum, early primary tooth exfoliation, pitting)
• Vitamin D-dependent rickets
• Vitamin D-resistant rickets
• Pseudohypoparathyroidism
• 
  
• Ectodermal/mesenchymal disorders
• Ectodermal dysplasias
• Epidermolysis bullosa, junctional type (skin fragility, blistering, dysplastic enamel)
• Tricho-dento-osseous syndrome
• Icontinentia pigmenti
• Tuberous sclerosis
• Oculo-dento-osseous syndrome
• 
  
• Nonsyndromic Conditions
• Amelogenesis imperfecta (usually only for defects exclusively in enamel, the 3 major groups are listed)
• Hypoplastic type
• Hypocalcified type
• Hypomaturation type

Assessment of article: This was a good read. I think that everyone should be sure to read this over as it helps us better understand teeth generally.