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$Unique_ID{BRK03437}
$Pretitle{}
$Title{AIDS Dysmorphic Syndrome}
$Subject{AIDS Dysmorphic Syndrome Dysmorphic Acquired Immune Deficiency
Syndrome Dysmorphic AIDS}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
519:
AIDS Dysmorphic Syndrome
** IMPORTANT **
It is possible the main title of the article (AIDS Dysmorphic Syndrome)
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
Dysmorphic Acquired Immune Deficiency Syndrome
Dysmorphic AIDS
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.
AIDS Dysmorphic Syndrome is an inborn infectious disorder affecting
children. The AIDS virus (HIV virus) is transmitted to a fetus during
pregnancy by an infected woman who may or may not have any symptoms of AIDS.
AIDS Dysmorphic Syndrome is characterized by the development of abnormal
facial and skull features. In addition, children with this disorder usually
experience delays in physical and mental development. The virus is thought
to act directly on the central nervous system. The basic characteristic of
AIDS (Acquired Immune Deficiency Syndrome) is a severely impaired immune
system. Children with AIDS Dysmorphic Syndrome are extremely susceptible to
infections by bacteria and viruses, especially a form of pneumonia caused by
the Pneumocystis carinii bacterium. However, treatment with intravenous
gamma globulin (IGIV) and the drug azidothymidine (AZT) may be beneficial to
children with Dysmorphic AIDS.
Symptoms
Symptoms of AIDS Dysmorphic Syndrome become apparent before one year of age.
AIDS Dysmorphic Syndrome is characterized by abnormalities of the skull and
face. These include some or all of the following: an abnormally small head
(microcephaly), wide-set eyes, a prominent box-like forehead, a flat nasal
bridge, mild upward or downward slant of the eyes, long eyelid fissures, a
blue tinge to the white of the eyes, a shortened nose, a triangular groove in
the upper lip, and distended lips. These features are usually the first sign
of AIDS Dysmorphic Syndrome in a child. They vary in severity and may not be
noticeable until the child is a few months old.
Children with this disorder usually also experience various degrees of
delay in their physical and mental development. Without treatment, mental
retardation can occur in some cases.
Infections resulting from the depressed immune system usually develop
later during infancy. Affected infants are highly susceptible to infections
such as pneumocystis carinii pneumonia, meningitis, urinary infections or
soft tissue infections.
Causes
Dysmorphic AIDS Syndrome is caused by transmission of the HIV virus from an
AIDS-infected mother (who may have no symptoms) to an unborn baby.
Affected Population
Dysmorphic AIDS usually becomes apparent among affected children between 3
weeks and 23 months of age. The disorder affects males and females in equal
numbers.
Therapies: Standard
AIDS Dysmorphic Syndrome can be diagnosed prenatally by testing for the
presence of the HIV virus in the fetus after the 14th week of pregnancy.
Blood from the umbilical cord may also be tested for the virus. After an
infant is born a blood test for AIDS will reveal the presence of the HIV
antibody.
In children with Dysmorphic AIDS, treatment with intravenous
immunoglobulin (IGIV) helps restore the immune system thus decreasing the
chance of infections. Treatment with the drug azidothymidine (AZT) may
prevent the facial abnormalities and the slowed mental and physical
development. The long-term outcome of these treatments is unknown at this
time.
Therapies: Investigational
This disease entry is based upon medical information available through
October 1989. 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 AIDS Dysmorphic Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
American Foundation for AIDS Research
40 W. 57th Street, Suite 406
New York, NY 10019
(212) 333-3118
Computerized AIDS Information Network (CAIN)
1213 North Highland Avenue
P.O. Box 38777
Hollywood, CA 90038
(213) 464-7400
NIH/National Institute of Allergy and Infections Diseases (NIAID)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5717
Centers for Disease Control
1600 Clifton Road, NE
Atlanta, GA 30333
(404) 639-3534
The AIDS Information Clearinghouse has been set up by the ICOA Health
Information Network to continuously provide updated information on AIDS to
the public. This electronic news and information service is available via
AT&T's ACCUNET packet or AT&T Mail and provides information on research,
screening and prevention programs, health care costs, confidentiality and
discrimination issues.
For more information on AIDS, see the AIDS Update section listed on the
NORD Services menu.
References
AIDS IN CHILDREN: Barbara J. Proujan; Research Resources Reporter (January
1988: issue 12(1). Pp. 1-5.
FETAL AIDS SYNDROME SCORE: R.W. Marion, et al.; American Journal
Diseases Children (1987: issue 141). Pp. 429-431.
PEDIATRIC AIDS: A. Rubinstein; Current Problems in Pediatrics (1986:
issue 16). Pp. 364-409.
HUMAN T-CELL LYMPHOTROPIC VIRUS TYPE III (HTLV-III) EMBRYOPATHY: R.W.
Marion, et al.; American Journal Diseases Children (July 1986: issue
140(7)). Pp. 638-640.
INTRAVENOUS GAMMA-GLOBULIN IN INFANT ACQUIRED IMMUNODEFICIENCY SYNDROME:
T.A. Calvelli, et al.; Pediatric Infectious Diseases (1986: issue 5). Pp.
S207-S210.