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1994-01-17
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$Unique_ID{BRK03473}
$Pretitle{}
$Title{Anodontia}
$Subject{Anodontia Anodontia Vera Partial Anodontia (Hypodontia) Complete
Anodontia}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
510:
Anodontia
** IMPORTANT **
It is possible the main title of the article (Anodontia) 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
Anodontia Vera
Includes:
Partial Anodontia (Hypodontia)
Complete Anodontia
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.
Anodontia is a genetic disorder characterized by partial or complete
absence of the primary or permanent teeth. It is associated with a group of
non-progressive skin and nerve syndromes called the Ectodermal Dysplasias.
Anodontia is usually part of a syndrome and seldom occurs as an isolated
entity.
Symptoms
Anodontia is characterized by partial or complete absence of teeth. Neither
the first nor second set of teeth erupt in children affected by this
disorder. Abnormalities of hair, nails, and sweat glands are usually present
as well. In many cases, Anodontia is a component of Ectodermal Dysplasia, a
group of hereditary disorders. (For more information, choose "Ectodermal
Dysplasia" as your search term in the Rare Disease Database.) In cases of
partial Anodontia, only some teeth are missing.
Causes
Anodontia is inherited either as an autosomal dominant or autosomal recessive
trait.
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.)
In recessive disorders, the condition does not appear unless a person
inherits the same defective gene from each parent. If one receives one
normal gene and one gene for the disease, the person will be a carrier for
the disease, but usually will show no symptoms. The risk of transmitting the
disease to the children of a couple, both of whom are carriers for a
recessive disorder, is twenty-five percent. Fifty percent of their children
will be carriers, but healthy as described above. Twenty-five percent of
their children will receive both normal genes, one from each parent and will
be genetically normal.
Affected Population
Anodontia is present at birth. The disorder affects males and females in
equal numbers. Female carriers usually have the disorder in a milder form
than males.
Related Disorders
The Ectodermal Dysplasias are a group of hereditary, non-progressive
syndromes in which the affected tissue derives primarily from the ectodermal
germ layer during development of the fetus. The skin, its derivatives, and
some other organs are involved. A predisposition to respiratory infections,
due to a somewhat depressed immune system and defective mucous glands in
parts of the respiratory tract, is the most severe characteristic of this
group of disorders. (For more information, choose "Ectodermal Dysplasia" as
your search term in the Rare Disease Database.)
Therapies: Standard
Treatment of Anodontia consists of artificial dentures. If only front teeth
are missing in Partial Anodontia, a flexible system allowing slight movement
of a bridge can be created by bonding an acrylic tooth to the supporting
structure (abutments) by means of three orthodontic wires.
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 Anodontia, 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
(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, and on the following articles:
PREVALENCE OF TOOTH AGENESIS CORRELATED WITH JAW RELATIONSHIP AND DENTAL
CROWDING: L.R. Dermaut, et al.; American Journal Orthod Dentofacial Orthop
(September 1986: issue 90(3)). Pp. 204-210.
A REVIEW OF TOOTH FORMATION IN CHILDREN WITH CLEFT LIP/PALATE: R. Ranta;
American Journal Orthod Dentofacial Orthop (July 1986: issue 90(1)). Pp. 11-
18.
A DENTAL APPROACH TO CARRIER SCREENING IN X LINKED HYPOHIDROTIC
ECTODERMAL DYSPLASIA: J.A. Spfaer; Journal Med Genet (December 1981: issue
18(6)). Pp. 459-460.
VARIABLE EXPRESSION OF FAMILIAL HYPODONTIA IN MONOZYGOTIC TRIPLETS: P.
Moller, et al.; Scandinavian Journal Dent Res (February 1981: issue 89(1)).
Pp. 16-18.
SEXUAL DIMORPHISM IN THE DEVELOPMENT, EMERGENCE, AND AGENESIS OF THE
MANDIBULAR THIRD MOLAR: G.Y. Levesque; Journal Dent Res (October 1981: issue
60(10)). Pp. 1735-1741.
NEW TECHNIQUE FOR SEMIPERMANENT REPLACEMENT OF MISSING INCISORS: J.
Artun, et al.; American Journal Orthod Dentofacial Orthop (May 1984: issue
85(5)). Pp. 367-375.