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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.
-
-