Hemolytic uremic syndrome is a poorly understood condition that affects the blood and kidneys. It usually follows an infection that has caused diarrhea.
There are many causes for HUS. By far, the most common cause is an episode of diarrhea caused by infection with a special strain of the bacterium, E. coli. There have been a number of epidemics of HUS in recent years linked to eating undercooked beef containing this strain of bacterium. This bacterium produces a toxin that is very harmful to cells, particularly those of the kidneys, blood vessels, and intestinal lining. There are other less common causes of HUS in which the exact cause is not as clear.
HUS is thought to develop when the lining of small blood vessels become damaged. This most commonly occurs in the blood vessels that supply blood to the kidney. The damage causes the blood vessels to swell. Tiny blood clots can form in affected blood vessels, narrowing the vessels or blocking them completely. Because blood cannot pass through these blocked or narrowed vessels, the kidneys are less able to filter waste products from the body and may become damaged. Certain cells in the blood, such as red blood cells that carry oxygen and platelets that help clot the blood, are damaged as they try to pass the narrowed vessels. This leads to anemia, or low blood counts, and poor clotting ability.
Most cases of HUS affect infants and young children between the ages of 7 months and 4 to 6 years. Between 0.3 and 10 per 100,000 children develop HUS in the U.S. each year. The condition tends to occur in epidemics.
The most common cause of HUS is the special strain of E. coli bacteria, called E. coli 0157:H7.
Less common causes of this condition include:
In some cases, there is no obvious cause for the illness.
Several factors put a person at risk for acquiring the diarrhea-related form of HUS. These cases are due to contamination of the substance with the special strain of E. coli bacteria. Examples of substances that may be contaminated include:
The primary symptoms of HUS include:
Other symptoms are related to the underlying cause. For example, some cases are related to pregnancy, in which case diarrhea may not occur.
HUS is often suspected after the history and physical exam. A complete blood count, or CBC, can identify low platelet counts or low red blood cell counts, known as anemia. Kidney function tests can detect kidney damage.
Diarrhea-related HUS can be prevented by:
HUS sometimes causes permanent loss of kidney function, and sometimes even kidney failure. However, roughly 50 to 80% of people with this condition recover fully.
There is also a mild risk of brain damage in some cases. This may result in serious long-term problems, such as seizures, paralysis, or changes in personality or behavior.
Most of the bacteria and viruses that cause HUS can be passed to other people. E. coli, for example, remains in the feces of infected people for a week or two after the diarrhea clears up.
A person with HUS often requires admission to the hospital. Treatment may include:
All medications have possible side effects, such as allergic reactions, stomach upset, and others. Specific side effects depend on the medications used. Transfusion of blood products may cause allergic reactions or infections in some cases. Dialysis is a complicated treatment that has to be done often. There are many possible side effects, including death.
Kidney function may be slightly lower even in someone who seems to have recovered fully. The long-term effects of this are unclear. Many people can return to normal activities shortly after recovery. Those with severe kidney damage may require ongoing treatment for life.
A person who has had HUS is often monitored regularly with complete blood counts, or CBC's, and kidney function tests.
Author:John Wegmann, MD
Date Written:
Editor:Slon, Stephanie, BA
Edit Date:05/12/00
Reviewer:Adam Brochert, MD
Date Reviewed: