home
***
CD-ROM
|
disk
|
FTP
|
other
***
search
/
CD-ROM Today 1996 January
/
CD-ROM Today 1996 January.iso
/
dp
/
0365
/
03653.txt
< prev
next >
Wrap
Text File
|
1994-01-17
|
6KB
|
161 lines
$Unique_ID{BRK03653}
$Pretitle{}
$Title{Dentin Dysplasia, Radicular}
$Subject{Dentin Dysplasia, Radicular Dentin Dysplasia, Type I Nonopalescent
Opalescent Dentine Radicular Dentin Dysplasia Rootless Teeth Coronal Dentin
Dysplasia (Dentin Dysplasia, Type II; Pulpal Dysplasia) Dentinogenesis
Imperfecta}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
521:
Dentin Dysplasia, Radicular
** IMPORTANT **
It is possible the main title of the article (Radicular 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
Dentin Dysplasia, Type I
Nonopalescent Opalescent Dentine
Radicular Dentin Dysplasia
Rootless Teeth
Information on the following diseases can be found in the Related
Disorders section of this report:
Coronal Dentin Dysplasia (Dentin Dysplasia, Type II; Pulpal Dysplasia)
Dentinogenesis Imperfecta
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.
Radicular Dentin Dysplasia is a genetic disorder characterized by
atypical formation of the calcified tissue between the enamel pulp of the
teeth (dentin). The teeth lack pulp chambers or have half-moon shaped pulp
chambers in short or abnormal shaped roots. The color of the teeth is
usually normal.
Symptoms
Radicular Dentin Dysplasia is characterized by teeth with a bluish-brown
shine. However, in some cases the teeth may appear to have normal enamel.
X-ray photos indicate the tooth pulp chambers in the roots are half-moon
shaped or obliterated, and the roots are very short. Areas around these
short roots sometimes appear dark (radiolucent) on X-rays.
Both the baby teeth and the permanent teeth are affected. The teeth are
often poorly aligned and can be chipped easily. Without treatment, persons
with Radicular Dentin Dysplasia may lose their teeth by age 30-40.
Causes
Radicular Dentin Dysplasia is inherited as an autosomal dominant disorder,
possibly caused by degenerated blood vessels in the dental papilla. (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
Radicular Dentin Dysplasia affects about 1 in 100,000 persons. Males and
females are affected in equal numbers.
Related Disorders
Symptoms of the following disorders can be similar to Radicular Dentin
Dysplasia. Comparisons may be useful for a differential diagnosis:
Coronal Dentin Dysplasia (Dentin Dysplasia, Type II; Pulpal Dysplasia)
is an autosomal dominant hereditary disorder. It is characterized by
brownish-blue shiny baby teeth with reduced root canals and normal appearing
permanent teeth. (For more information on this disorder, choose "Coronal
Dentin Dysplasia" as your search term in the Rare Disease Database).
Dentinogenesis Imperfecta is an autosomal dominant hereditary disorder.
It is characterized by bluish-brown or brown opalescent baby and permanent
teeth. The tooth enamel breaks and wears off easily. In affected adults,
only roots may remain. X-rays usually show the absence of dental pulp
chambers and root canals. (For more information on thia disorder, choose
"Dentinogenesis Imperfecta" as your search term in the Rare Disease
Database.)
Therapies: Standard
The affected teeth in persons with Radicular Dentin Dysplasia can be treated
by a specialist who treats the roots and pulp of the teeth (endodontist).
Filling the tips of the root canals can allow these abnormal teeth to remain
in their natural position. Sometimes the affected teeth must be extracted
and replaced with dentures.
Genetic counseling is recommended for families of children with Radicular
Dentin Dysplasia.
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 Radicular 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 Dysplasias
219 E. Main St.
Mascoutah, IL 62258
(618) 566-2020
NIH/National Institute of Dental Research
9000 Rockville Pike
Bethesda, MD 20892
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.
DENTIN DYSPLASIA TYPE I: A CLINICAL REPORT: J.A. Petrone, et al.;
Journal Am Dent Assoc (December 1981: issue 103(6)). Pp. 891-893.
DENTIN DYSPLASIA TYPE I: A SCANNING ELECTRON MICROSCOPIC ANALYSIS OF THE
PRIMARY DENTITION: M. Melnick, et al.; Oral Surg (October 1980: issue
50(4)). Pp. 335-340.