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$Unique_ID{BRK03654}
$Pretitle{}
$Title{Dentinogenesis Imperfecta, Type III}
$Subject{Dentinogenesis Imperfecta, Type III Brandywine Type Dentinogenesis
Imperfecta Dentinogenesis Imperfecta, Shields Type III Dentin Dysplasia,
Coronal Dentin Dysplasia, Radicular Dentinogenesis Imperfecta, Type I
(Hereditary Opalescent Dentin; Dentinogenesis Imperfecta, Type II; Capdepont
Teeth; Hereditary Brown Teeth)}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
551:
Dentinogenesis Imperfecta, Type III
** IMPORTANT **
It is possible the main title of this article (CHARGE Association) is not
the name you expected. Please check the SYNONYMS listing on the next page to
find alternate names and disorder subdivisions covered by this article.
Synonyms
Brandywine Type Dentinogenesis Imperfecta
Dentinogenesis Imperfecta, Shields Type III
Information on the following disorders may be found in the Related
Disorders section of this report:
Dentin Dysplasia, Coronal
Dentin Dysplasia, Radicular
Dentinogenesis Imperfecta, Type I (Hereditary Opalescent Dentin;
Dentinogenesis Imperfecta, Type II; Capdepont Teeth; Hereditary Brown
Teeth)
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Dentinogenesis Imperfecta, Type III is a genetic dental disorder
characterized by rapid erosion of crowns of the baby and permanent teeth soon
after they erupt. The inside of the teeth (dental pulp) which is filled with
blood vessels and nerve endings is usually exposed. Other genetic traits may
accompany these tooth abnormalities.
Symptoms
Dentinogenesis Imperfecta, Type III is characterized by rapid erosion of the
crowns in baby and permanent teeth. Dental pulp inside several teeth may be
exposed. This pulp may be opalescent, smooth, and amber colored. Pulp
chambers and root canals may appear very large on X-ray photos of baby teeth.
Permanent teeth may have a reduction or even complete loss of the pulp
chambers and root canals. Carriers of the gene for this disorder may have
teeth that appear normal. However, upon examination their teeth have only an
extremely thin ivory layer and an enlarged pulp chamber (shell teeth).
Pitting of the tooth enamel may occur in the permanent teeth of patients.
Causes
Dentinogenesis Imperfecta, Type III is a disorder inherited through an
autosomal dominant gene; this gene is thought to be Gc on the chromosome 4q.
(Human traits including the classic genetic diseases, are the product of the
interaction of two genes for that condition, one received from the father and
one from the mother. In dominant disorders, a single copy of the disease
gene (received from either the mother or father) will be expressed
"dominating" the normal gene and resulting in appearance of the disease. The
risk of transmitting the disorder from affected parent to offspring is 50%
for each pregnancy regardless of the sex of the resulting child.)
Affected Population
Patients with Dentinogenesis Imperfecta, Type III are affected in a ratio of
approximately 55 males to 45 females. Symptoms begin as soon as baby teeth
erupt. It tends to run in families. The disorder was first found to occur
in people who lived in Brandywine, MD, but it can also affect persons of
Ashkenazi Jewish heritage.
Related Disorders
Symptoms of the following disorders can be similar to those of Dentinogenesis
Imperfecta, Type III. Comparisons may be useful for a differential
diagnosis:
Radicular Dentin Dysplasia is a genetic disorder characterized by
abnormal formation of tooth ivory (dentin). The teeth lack pulp chambers, or
have half-moon shaped pulp chambers in short or abnormally shaped roots. The
color of the teeth is usually normal. (For more information, choose "Dentin
Dysplasia" as your search term in the Rare Disease Database.)
Coronal Dentin Dysplasia is a genetic disorder characterized by brownish-
blue opalescent baby teeth and permanent teeth that appear normal. The baby
teeth in children affected by this disorder contain obliterated pulp chambers
and reduced root canals. Permanent teeth also have abnormalities. (For more
information, choose "Dentin Dysplasia" as your search term in the Rare
Disease Database.)
Osteogenesis Imperfecta, or Brittle Bone Disease, is a group of
hereditary connective tissue disorders characterized by unusual bone
fragility and tendency to fracture. Dentinogenesis Imperfecta is one of the
features of Osteogenesis Imperfecta. (For more information, choose
"Osteogenesis Imperfecta" as your search term in the Rare Disease Database.)
Dentinogenesis Imperfecta Type I (DGI 1; Opalescent Dentin; Opalescent
Teeth without Osteogenesis Imperfecta; Dentinogenesis Imperfecta, Shields
Type II; Capdepont Teeth; Hereditary Brown Teeth) is an inherited disorder
characterized by blue-gray or amber brown, opalescent teeth, without
brittleness of the bones. Dental X-rays indicate the teeth have bulbous
crowns, roots are narrower than normal, and pulp chambers and root canals are
smaller than normal or completely absent. The tooth enamel splits readily
from the ivory when the upper teeth close forcefully against the lower teeth,
as in chewing or biting.
Therapies: Standard
Treatment for children with Dentinogenesis Imperfecta, Type III consists of
placement of a full set of dental crowns over the teeth. In adults, all
teeth may be extracted carefully by elevation and replaced with a full set of
dentures. It is recommended that treatment is started early to improve the
facial appearance of young patients.
Genetic counseling is recommended for families of children with
Dentinogenesis Imperfecta.
Therapies: Investigational
This disease entry is based upon medical information available through
November 1988. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section for
the most current information about this disorder.
Resources
For more information on Dentinogenesis Imperfecta, Type III, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
National Foundation for Ectodermal Dysplasia
P.O. Box 114
Mascoutah, IL 62258
(618) 556-2020
NIH/National Institute of Dental Research
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4261
For genetic information and genetic counseling referrals, please contact:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD 20815
(800) 336-GENE
(301) 652-5553
References
This report in the Rare Disease Database is based on outlines prepared by
medical and dental students (1984-1986) at the Medical College of Virginia
for their course in human genetics.
MENDELIAN INHERITANCE IN MAN, 7th ed.: Victor A. McKusick; Johns Hopkins
University Press, 1986. P. 193.
A NEW CLASSIFICATION OF HERITABLE HUMAN ENAMEL DEFECTS AND A DISCUSSION
OF DENTIN DEFECTS: E.D. Shields; Birth Defects (1983: issue 19(1)). Pp.
107-127.
DENTINOGENESIS IMPERFECTA IN THE BRANDYWINE ISOLATE (DI TYPE III):
CLINICAL, RADIOLOGIC, AND SCANNING ELECTRON MICROSCOPIC STUDIES OF THE
DENTITION: L.S. Levin, et al.; Oral Surg (September 1983: issue 56(3)). Pp.
267-274.
AN AUTOSOMAL-DOMINANT FORM OF JUVENILE PERIODONTITIS: ITS LOCALIZATION TO
CHROMOSOME 4 AND LINKAGE TO DENTINOGENESIS IMPERFECTA AND Gc Journal Craniofac
Genet Dev Biol GENESIS (1986: issue 6(4)). Pp. 341-350.
AN UNUSUAL PRESENTATION OF OPALESCENT DENTIN AND BRANDYWINE ISOLATE
HEREDITARY OPALESCENT DENTIN IN AN ASHKENAZIC JEWISH FAMILY: A. Heimler, et
al.; Oral Surg al.; Oral Surg Oral Med Oral Pathol (June 1985: issue 59(6)).
Pp. 608-615.