$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.