$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 (301) 497-9888 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 (615) 322-2250 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 (203) 746-6518 NIH/National Institute of Allergy and Infectious Diseases (NIAID) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, GA 30333 (404) 639-3534 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.