$Unique_ID{BRK04200} $Pretitle{} $Title{Rubella, Congenital} $Subject{Rubella Congenital Expanded Rubella Syndrome Congenital Rubella Syndrome Congenital German Measles } $Volume{} $Log{} Copyright (C) 1986, 1989 National Organization for Rare Disorders, Inc. 276: Rubella, Congenital ** IMPORTANT ** It is possible the main title of the article (Congenital Rubella) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Expanded Rubella Syndrome Congenital Rubella Syndrome Congenital German Measles 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. Congenital Rubella is a syndrome which occurs when a fetus has been infected with the Rubella virus while in the uterus. It is primarily characterized by abnormalities of the cardiovascular system, the eyes and the hearing. Women who contract Rubella during pregnancy have a high risk of having a baby with Congenital Rubella. Symptoms As many as two thirds of infants with Congenital Rubella will be free of any abnormality at birth. The classic Congenital Rubella syndrome has been characterized by the combination of heart, eye and hearing defects, although infection and damage can occur in almost every organ system. Of the abnormalities most likely to be present at birth, cardiovascular defects are most common, such as underdevelopment (hypoplasia) of the pulmonary artery and the failure of closure of a duct connecting the pulmonary artery and aorta (patent ductus arteriosus). Low birth weight, inflammation of the bones (osteitis), enlarged liver and spleen (hepatosplenomegaly), disease of the retina (retinopathy), and cataracts of the crystalline lens of the eye also occur frequently. Brain infection (encephalitis), an abnormally small head (microcephaly), swollen lymph glands (adenopathy), inflammation of the lungs (pneumonitis), jaundice, reduced number of blood platelets (thrombocytopenia), pinpoint purplish red spots due to bleeding in the skin (petechiae) or purpura, and anemia may also occur in babies with Congenital Rubella. Congenital Rubella can be viewed as a chronic infection capable of producing progressive damage. Central nervous system abnormalities such as hearing loss, mental retardation, behavior problems and slowness in muscular development, are the frequent and significant clinical problems. Most patients who are symptomatic, and many of those who lack signs of infection at birth, will develop some degree of hearing loss or psychomotor damage during early childhood. (For more information on Congenital Rubella, see "Rubella" in the Prevalent Health Conditions/Concerns section of NORD Services.) Causes Congenital Rubella can affect a fetus when a pregnant woman who is not immune to the virus contracts Rubella (German Measles). The baby may also be affected if the mother contracts Rubella immediately before conception. Affected Population Congenital Rubella is found in newborns and infants of mothers who were infected with Rubella immediately before or during the early months of pregnancy. The frequency of Congenital Rubella thus depends upon the number of women of childbearing age who are susceptible to the virus, and the frequency of Rubella infection in the community. Before the development of Rubella virus vaccine, epidemics of Rubella and Congenital Rubella occurred about every 6 to 9 years. During epidemic years Congenital Rubella infection was found (using serologic testing to identify nonsymptomatic cases) in as many as 2% of newborns; the rate of its presence at other times (the endemic rate) is 0.1%. Widespread use of Rubella vaccine in the United States has eliminated epidemics, but the endemic rate of congenital infection appears to be about the same. Both the chance of transmission of Rubella to the fetus during pregnancy and the consequences of the infection to the unborn baby are related to the stage of development of the fetus at the time of maternal infection. Maternal infection during the first 8 weeks of pregnancy results in an infection rate in the fetus of about 50%. Subsequently the rate of transmission drops sharply to less than 10% by the 16th week of pregnancy. The proportion of infected fetuses with damage due to Rubella follows a similar pattern. With maternal Rubella at 8, 12, 13 to 20, and over 20 weeks of pregnancy, 85%, 50%, 15%, and 0% (respectively) of infected live-born infants will have Rubella-caused defects at birth or during early childhood. Therapies: Standard There is no treatment for maternal or Congenital Rubella infection. Therefore, prevention assumes paramount importance. It is most important to immunize all children with the goal of preventing epidemics. Children should receive Rubella immunization at 15 months of age, along with mumps and measles in a combined vaccine. Many authorities now recommend that a repeat Rubella immunization be given to 10-year-olds, because vaccine-induced immunity may not persist as long as naturally acquired immunity. Women of childbearing age who are susceptible to Rubella (a serum test can establish the presence of the Rubella-antibody in their blood) should also be vaccinated. Care should be taken that they should not conceive for at least three months following the vaccination. Therapies: Investigational This disease entry is based upon medical information available through March 1987. 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 Congenital Rubella, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Child Health and Human Development 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 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 RUBELLA; Public Health Education Information Sheet: March of Dimes Birth Defects Foundation, 1984.