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$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.