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$Unique_ID{BRK03652}
$Pretitle{}
$Title{Dentin Dysplasia, Coronal}
$Subject{Dentin Dysplasia, Coronal Anomalous Dysplasia of Dentin Coronal
Dentin Dysplasia Coronal Dentine Dysplasia Dentin Dysplasia, Type II Dentine
Dysplasia, Type II Pulp Stones Dentin Dysplasia, Radicular Dentinogenesis
Imperfecta (Opalescent Dentin) Pulpal Dysplasia}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
505:
Dentin Dysplasia, Coronal
** IMPORTANT **
It is possible the main title of the article (Coronal Dentin Dysplasia)
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
Anomalous Dysplasia of Dentin
Coronal Dentin Dysplasia
Coronal Dentine Dysplasia
Dentin Dysplasia, Type II
Dentine Dysplasia, Type II
Pulp Stones
Pulpal Dysplasia
Information on the following disorders may be found in the Related
Disorders section of this report:
Dentin Dysplasia, Radicular
Dentinogenesis Imperfecta (Opalescent Dentin)
General Discussion
** IMPORTANT **
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.
Coronal Dentin Dysplasia is a genetic disorder characterized by brownish-
blue opalescent baby teeth and permanent teeth that appear normal. The hard
calcified tissue beneath the enamel (dentin) is the main substance of the
teeth. The baby teeth in children affected by this disorder contain
obliterated pulp chambers and reduced root canals. Permanent teeth also have
abnormalities.
Symptoms
Coronal Dentin Dysplasia is characterized by brownish-blue opalescent baby
teeth. Later, permanent teeth appear normal. On X-rays, the baby teeth show
obliterated pulp chambers and reduced root canals. The permanent teeth
contain flame-shaped pulp chambers often with an extension reaching into the
root, and numerous pulp stones. Root formation in the permanent teeth is
usually normal. The baby teeth wear away (abrade) rapidly. Premature loss
of teeth may occur slightly more often than normal.
Causes
Coronal Dentin Dysplasia is an autosomal dominant inherited disorder. (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
Coronal Dentin Dysplasia is a rare disorder affecting males and females in
equal numbers.
Related Disorders
Symptoms of the following disorders can be similar to those of Coronal Dentin
Dysplasia. Comparisons may be useful for a differential diagnosis:
Radicular Dentin Dysplasia is a genetic disorder characterized by
atypical formation of the hard calcified tissue which forms the major part of
the tooth (dentin). The teeth lack pulp chambers or have half-moon shaped
pulp chambers in short or abnormal shaped roots. Tooth color is usually
normal. (For more information on this disorder, choose "Radicular Dentin
Dysplasia" as your search term in the Rare Disease Database.)
Dentinogenesis Imperfecta (Opalescent Dentin) is inherited as an
autosomal dominant hereditary disorder. This disorder is characterized by
bluish-brown or brown opalescent baby and permanent teeth. The tooth enamel
breaks and wears off easily. In adults, only roots may remain. X-rays
usually show the absence of dental pulp chambers and root canals. (For more
information on this disorder, choose "Dentinogenesis Imperfecta" as your
search term in the Rare Disease Database.)
Therapies: Standard
Coronal Dentin Dysplasia may be treated by scraping (curettage) around the
tips of the roots, and filling these root tips with a dental amalgam
(retrograde amalgam seal), or by more conventional root canal therapy.
However, preventive dental care provides the best available means of
maintaining the teeth.
Genetic counseling is recommended for families of children with Coronal
Dentin Dysplasia.
Therapies: Investigational
This disease entry is based upon medical information available through June
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 Coronal Dentin Dysplasia, 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) 566-2020
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 is based on outlines prepared by medical and dental students
(1984-1986) at the Medical College of Virginia for their course in human
genetics.
A SCANNING ELECTRON MICROSCOPIC STUDY OF DENTIN DYSPLASIA TYPE II IN
PRIMARY DENTITION: J.R. Jasmin, et al.; Oral Surg (July 1984: issue
58(1)). Pp. 57-63.
DENTINAL DYSPLASIA: A CLINICOPATHOLOGICAL STUDY OF EIGHT CASES AND REVIEW
OF THE LITERATURE: N.E. Steidler, et al.; British Journal Maxillofac Surg
(August 1984: issue 22(4)). Pp. 274-286.