Hemolytic Uremic Syndrome: NIDDK
Article title: Hemolytic Uremic Syndrome: NIDDK
Main condition: Hemolytic Uremic Syndrome
Conditions: Hemolytic Uremic Syndrome
Hemolytic (HEE-mo-LIT-ik) uremic (yoo-REE-mik) syndrome is a rare condition affecting mostly children under the age of 10. Hemolytic uremic syndrome (HUS) is characterized by destruction of red blood cells, damage to the lining of blood vessel walls, and, in severe cases, kidney failure.
Most cases of HUS occur after an infection in the digestive system caused by the Escherichia colibacterium found on contaminated food like meat, dairy products, and juice. The first stages of HUS frequently present with gastrointestinal symptoms such as abdominal pain, vomiting, and bloody diarrhea. This stage lasts from 1 to 15 days. Recovery from this acute colitic phase is the rule. However, more severe problems in the bowel and colon may develop in some cases. (The most vulnerable and severely stricken of children with HUS can die during the first acute stage of the disease--between 5 percent and 10 percent.)
For most children with HUS, the vomiting and diarrhea stop after 2 or 3 days. Even when the gastroenteritis has stopped, the child does not recover full health, remaining pale, tired, and irritable. The bacteria originally lodged in the digestive system make toxins that enter the bloodstream, and these toxins destroy red blood cells. Small, unexplained bruises or small clot-sized hemorrhages visible in the mucosal lining of the mouth may occur because the toxins also destroy the platelets, cells that normally contribute to the clotting system.
The damaged red blood cells and other factors may clog the tiny blood vessels in the kidneys or cause lesions in the kidneys, making the kidneys work harder to remove wastes and extra fluid from the blood. The child may produce little urine as a result. The body's inability to rid itself of excess fluid and waste may in turn cause hypertension or swelling of the hands and feet or may cause generalized fluid accumulation (edema).
Once HUS develops, there is no known treatment that can stop the progress of the syndrome. Unfortunately, it must run its course. Most treatments are supportive in nature and aimed at easing the immediate symptoms and signs of this disease and at preventing further complications. Supportive therapy consists of maintaining specific levels of fluids and salts, which are replaced only as needed. Blood transfusions (packed red blood cells) are sometimes needed. Treatment of high blood pressure is often necessary. Dialysis is also needed at times to help the kidneys perform their function. Other complications, such as neurologic complications, necessitate additional medications.
Ninety percent of children with HUS who receive careful supportive care survive past the initial harrowing stages of the condition, and most of those will have no long-term effects. But between 10 percent and 30 percent of the survivors will have kidney damage that leads to permanent kidney failure either immediately or over the course of several years. Most children with kidney failure will require peritoneal dialysis or hemodialysis to wash the wastes and extra fluid from their blood or may require a kidney transplant.
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Additional Information on Hemolytic Uremic Syndrome
The National Kidney and Urologic Diseases Information Clearinghouse collects resource information on kidney and urologic diseases for the Combined Health Information Database (CHID). CHID is a database produced by health-related agencies of the Federal Government. This database provides titles, abstracts, and availability information for health information and health education resources.
To provide you with the most up-to-date resources, information specialists at the clearinghouse created an automatic CHID search. To obtain this information you may view the results of the automatic search on Hemolytic Uremic Syndrome.
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The National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). NIDDK is part of the National Institutes of Health under the U.S. Department of Health and Human Services. Established in 1987, the clearinghouse provides information about diseases of the kidneys and urologic system to people with kidney and urologic disorders and to their families, health care professionals, and the public. NKUDIC answers inquiries; develops, reviews, and distributes publications; and works closely with professional and patient organizations and Government agencies to coordinate resources about kidney and urologic diseases.
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NIH Publication No. 99-4570
e-text posted: 2 October 1998
last updated: March 2000
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