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$Unique_ID{BRK03647}
$Pretitle{}
$Title{Cytomegalovirus Infection}
$Subject{Cytomegalovirus Infection CMV Cytomegalic Inclusion Disease Salivary
Gland Disease Giant Cell Inclusion Disease CID Human Cytomegalovirus Infection
Congenital Cytomegalovirus Infection Acquired Cytomegalovirus Infection
Postperfusion Syndrome}
$Volume{}
$Log{}

Copyright (C) 1986, 1987, 1988, 1989, 1990, 1991 National Organization
for Rare Disorders, Inc.

189:
Cytomegalovirus Infection

** IMPORTANT **
It is possible the main title of the article (Cytomegalovirus Infection)
is not the name you expected.  Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     CMV
     Cytomegalic Inclusion Disease
     Salivary Gland Disease
     Giant Cell Inclusion Disease, also known as CID
     Human Cytomegalovirus Infection

DISORDER SUBDIVISIONS

     Congenital Cytomegalovirus Infection
     Acquired Cytomegalovirus Infection
     Postperfusion Syndrome

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.


Cytomegalovirus Infection (CMV) is a virus infection occuring
congenitally, postnatally or at any age.  CMV ranges in severity from a
silent infection without consequences, to a disease manifested by fever,
hepatitis, and (in newborns) severe brain damage, and stillbirth or perinatal
death.

Symptoms

Infections of Cytomegalovirus may be congenital (existing before or at birth)
or acquired after birth.

Symptoms of Congenital Infection are highly variable.  The infection may
be manifested only by cytomegaloviruria (virus in the urine) in an otherwise
normal infant.  At the other extreme, hemorrhaging, anemia, or extensive
liver or CNS (Central Nervous System) damage may occur.  Infants born with a
severe form of the disease typically have a low birth weight and develop a
fever, hepatitis with jaundice, and hemorrhagic manifestations such as
purpura.  Hepatosplenomegaly (enlargement of liver and spleen),
thrombocytopenia (decrease in number of blood platelets), chorioretinitis
(inflammation of the choroid and retina), microcephaly (abnormal smallness of
the head), and periventricular cerebral calcification may be present.
Psychomotor retardation (development of motor defects and psychic
abnormalities), spastic diplegia (spastic paralysis on both sides of body),
blindness, deafness, or seizures may develop.  Even though the
Cytomegalovirus Infection may not at times be apparent in some infants, it
may later cause hearing defects in these children.

Acquired Cytomegalovirus Infections, which occur immediately after birth
or later in life, are often asymptomatic (there are no symptoms apparent).  An
acute illness with fever, termed Cytomegalovirus Mononucleosis or
Cytomegalovirus Hepatitis, may result from contact through medical treatment
or from spontaneous contact with Cytomegalovirus.  According to a 1987 study,
acquired Cytomegalovirus infection does not appear to be a contributing risk
factor for sensorineural hearing loss, regardless of poor health or premature
birth of affected infants.

Postperfusion Syndrome develops two to four weeks after transfusion with
fresh blood containing CMV and is characterized by fever lasting two to three
weeks, hepatitis of variable degrees with or without jaundice, a
characteristic atypical lymphocytosis (excess of lymph cells in the blood or
in any effusion) resembling that of infectious mononucleosis, and
occasionally a rash.  CMV infection in patients with malignancy or receiving
immunosuppressive therapy may cause pulmonary, gastrointestinal or renal
(kidney) involvement.  This complication is of major importance in some
reported transplantation series in which immunosuppressive therapy is
utilized.

Causes

Cytomegalovirus Infection is caused by the human Cytomegaloviruses ("salivary
gland viruses"), a subgroup of agents closely related to the herpes group of
viruses, which can remain latent in man.

Affected Population

Cytomegalovirus infection has increased in the United States in recent years
probably because of the increased use of day-care facilities.  Recent studies
show that sixty percent of children in these facilities have been infected,
compared to less than twenty percent of children cared for at home.  Although
these infections have no long-term serious health consequences among
children, they can be transmitted to women of childbearing age.  If a woman
is infected during pregnancy, fetal infections known as cytomegalic inclusion
disease (CID) can occur.

According to the American Academy of Pediatrics, eighty-seven percent of
children with CID develop complications.  Thirty-one percent have serious
sensorineural hearing loss, and sixty-two percent have some degree of mental
retardation.

Perinatal CMV infection is common.  It occurs in eight to thirteen
percent of healthy newborns in the United States, and fourteen to eighteen
percent of sick or premature infants.  Healthy full-term infants rarely have
symptoms and are at lesser risk of long-term effects.  In contrast, symptoms
are common in premature and sick full-term infants and may include pneumonia,
hepatitis, hemolytic anemia, thrombocytopenia, and fever.

Related Disorders

The CMV virus is closely related to the herpes group of viruses.  (For more
information on Cytomegalovirus Infection and other herpes viruses, see
related articles in the Prevalent Health Conditions/Concerns area of NORD
Services and the AIDS Update.

Therapies:  Standard

Treatment of Cytomegalovirus Infection may include the orphan drug Cytovene
which has received FDA approval as standard treatment for CMV.  It is
manufactured by Syntex (USA), Inc., 3401 Hillview Ave., Palo Alto, CA 94304.

In April 1990 the FDA approved Cytomegalovirus Immune Globulin
Intravenous (CMV-IGIV).  This drug is given to organ transplant patients who
show no signs of CMV infection before surgery, but who will receive kidneys
from CMV positive donors.  CMV-IGIV is manufactured by the Massachusetts
Public Health Biologic laboratories.  Given to patients just before and for
some time after the transplant, it can reduce the severity of their CMV
infection by infusing antibodies to fight the CMV virus.

Therapies:  Investigational

The experimental drug, ganciclovir, is being tested on people with CMV
retinitis to determine if it can prevent blindness.  Physicians with CMV
retinitis patients who may be interested in participating in the study can
contact:

     NIH/National Institute of Allergy and Infectious Diseases (NIAID)
     Ganciclovir Study Center
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

Trials of drugs which interfere with viral DNA synthesis (floxuridine,
cytarabine, and others) have not yielded clear-cut results in the treatment
of Cytomegalovirus Infection.

The investigational orphan drug BW B759U is being tested in clinical
trials for treatment of severe human Cytomegalovirus Infections in specific
immunosuppressed patient populations (e.g., bone marrow transplant recipients
and AIDS patients.)

For more information, physicians can contact:

     Burroughs-Wellcome Co.
     3030 Cornwallis Rd.
     Research Triangle Park, NC  27709

Clinical trials of the orphan drug ganciclovir (DHPG) are underway to
determine its use as a treatment for Cytomegalovirus (CMV) infections of a
serious life- or sight-threatening nature in patients with reduced immunity.
AIDS patients with cytomegalovirus retinitis can be included in this group.
For more information, physicians can contact:

     Syntex (USA), Inc.
     3401 Hillview Ave.
     Palo Alto, CA  94304

Additionally, the Food and Drug Administration (FDA) has given a research
grant award to Stanley Plotkin, M.D., Wistar Institute, Philadelphia, PA, to
study a live attenuated cytomegalovirus vaccine.

The orphan drug designation has been given to Cytomegalovirus Immune
Globulin (Human) for use in prevention or attenuation of primary
cytomegalovirus disease in immunosuppressed recipients of organ transplants.
For more information, physicians can contact:

     Massachusetts Public Health Biologic Laboratories
     305 South Street
     Jamaica Plain, MA  02130

SOZ MSL-109 is being tested by the FDA as a preventative of CMV in solid
organ transplant patients.  The sponsor is Sandoz Pharmaceuticals Corp., 59
Route 120, East Hanover, NJ, 07936.

Clinical trials are underway to study the use of Ganciclovir for the
treatment of symptomatic Cytomegalovirus Infections.  Interested persons may
wish to contact:

     Dr. William R. Gruber
     Vanderbilt University
     Pediatric Infectious Diseases
     Rm. D-7226, MCN
     Nashville, TN  37232
     [phone]

to see if further patients are needed for this research.

For information on additional therapies that have been designated as
Orphan Drugs in the last few months, please return to the main menu of NORD
Services and access the Orphan Drug Database.

This disease entry is based upon medical information available through
January 1992.  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 Cytomegalovirus Infections, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     NIH/National Institute of Allergy and Infectious Diseases (NIAID)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

     Centers for Disease Control (CDC)
     1600 Clifton Road, NE
     Atlanta, GA  30333
     [phone]

     CMV Clinic
     Children's Hospital of St. Paul
     345 North Smith Ave.
     St. Paul, MN  55102

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  Pp. 163, 182.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 1784-6.