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$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.