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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.
-
-