$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.