$Unique_ID{BRK03809} $Pretitle{} $Title{Hemolytic Uremic Syndrome} $Subject{Hemolytic Uremic Syndrome HUS Gasser Syndrome Thrombotic Thrombocytopenia Purpura} $Volume{} $Log{} Copyright (C) 1989, 1991 National Organization for Rare Disorders, Inc. 745: Hemolytic Uremic Syndrome ** IMPORTANT ** It is possible that the main title of the article (Hemolytic Uremic Syndrome (HUS)) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms HUS Gasser Syndrome Information on the following diseases can be found in the Related Disorders section of this report: Thrombotic Thrombocytopenia Purpura 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. Hemolytic Uremic Syndrome (HUS) may accompany a gram-negative bacterial infection which sets up an allergic reaction resulting in massive blood clotting inside the blood vessels, thus producing anemia, a reduction in platelets, and acute kidney failure. Symptoms Hemolytic Uremic Syndrome (HUS) usually has a sudden onset. The kidneys are primarily affected, and there may be an absence or disturbance of urine excretion (anuria). Approximately 60% of children with Hemolytic Uremic Syndrome develop acute kidney failure which usually corrects itself after treatment. Chronic kidney failure occurs in approximately 10% of children. Kidney involvement is usually more severe in adults, and there may be destruction of kidney tissue. Other symptoms of Hemolytic Uremic Syndrome (HUS) may include vomiting, diarrhea with or without blood in the stool, dehydration, labored breathing, vomiting of blood (hematemesis), dark and tarry stools (melena), purplish spots in the skin (petechiae), high blood pressure and seizures. There is usually a persistent decrease in the number of blood platelets (thrombocytopenia) and a deficiency in red blood cells (anemia). Causes The exact cause of Hemolytic Uremic Syndrome (HUS) is unknown. Many scientists believe it may be associated with infection by the bacteria, Escherichia coli (E. coli). It usually appears abruptly in children three to ten days following an episode of gastroenteritis or viral upper respiratory tract infections. In adults, it most commonly affects women and is often associated with complications of pregnancy. However, the exact cause has not been definitively identified. Affected Population Hemolytic Uremic Syndrome (HUS) is a rare disorder that occurs most frequently in children under the age of four years, and in pregnant or postpartum women. It is occasionally seen in older children and non-pregnant adults. Some areas of the world such as Argentina have a much higher incidence of Hemolytic Uremic Syndrome (HUS) than North America. Related Disorders Symptoms of the following disorder can be similar to those of Hemolytic Uremic Syndrome (HUS). Comparisons may be useful for a differential diagnosis: Thrombotic Thrombocytopenic Purpura (TTP) is a rare blood disease characterized by an abnormally low blood platelet count. Major symptoms include fever, excessive bleeding into the skin and mucous membranes, jaundice, abdominal pain and varying heart rhythms. (For more information on this disorder, choose "TTP" as your search term in the Rare Disease Database). Therapies: Standard Most infants and children with Hemolytic Uremic Syndrome (HUS) will recover with supportive treatment including dialysis therapy. Recovery in adults may be more difficult. Women who develop Hemolytic Uremic Syndrome postpartum may need dialysis on a continuing basis. Dialysis and transfusion of fresh frozen plasma are other possible methods of treatment. The orphan drug erythropoetin (EPO), used in the treatment of anemia related to kidney dialysis, may be prescribed. Therapies: Investigational Plasmapheresis and plasma infusion may be of benefit in some cases of Hemolytic Uremic Syndrome (HUS). This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient's plasma is then replaced with other human plasma and the blood is retransfused into the patient. Plasma infusion is also being studied as a treatment for HUS patients. The infusion process uses substances missing from the HUS patient's blood and replaces them through an infusion of new frozen plasma into the patient. No blood is removed from the patient during this process. These therapies are still under investigation to analyze side effects and effectiveness. More research is needed before plasmapheresis and plasma infusion can be recommended for use in all but the most severe cases of Hemolytic Uremic Syndrome (HUS). This disease entry is based upon medical information available through September 1991. 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 Hemolytic Uremic Syndrome(HUS), please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Kidney and Urologic Diseases Information Clearinghouse Box NKUDIC Bethesda, MD 20892 (301) 468-6345 National Kidney Foundation 2 Park Avenue New York, NY 10016 (212) 889-2210 (800) 622-9010 American Kidney Fund 6110 Executive Blvd., Suite 1010 Rockville, MD 20852 (301) 881-3052 (800) 638-8299 (800) 492-8361 (MD) References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 860, 1007. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck Sharp & Dohme Laboratories, 1982. Pp. 1161-1162. ILLNESSES ASSOCIATED WITH ESCHERICHIA COLI 0157:H7 INFECTIONS. A BROAD CLINICAL SPECTRUM. P.M. Griffin, et al.; ANN INTERN MED (November 1, 1988; issue 109 (9)). Pp. 705-712. CYTOXIN-PRODUCING ESCHERICHIA COLI AND THE HEMOLYTIC UREMIC SYNDROME. T.G. Cleary; PEDIATR CLIN NORTH AM (June, 1988; issue 35 (5)). Pp. 485-501. HEMOLYTIC-UREMIC SYNDROME ASSOCIATED WITH AN INFECTION BY VEROTOXIN PRODUCING ESCHERICHIA COLI 0111 IN A WOMAN ON ORAL CONTRACEPTIVES. K.O. Stenger et al.; CLIN NEPHROL (March, 1989; issue 29 (3)). Pp. 153-158.