$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.