Definition
Hemolytic-uremic syndrome (HUS) is a disorder that usually occurs when an infection in the digestive system produces toxic substances that destroy red blood cells. It often affects the kidneys.
Causes, incidence, and risk factors
Hemolytic-uremic syndrome (HUS) often occurs after a gastrointestinal infection with E. coli bacteria (Escherichia coli O157:H7). However, the condition has also been linked to other gastrointestinal infections, including shigella and salmonella , as well as non-gastrointestinal infections.
HUS is most common in children. It was once very rare, but it is increasing in children. It is the most common cause of acute kidney failure in children. Several large outbreaks in 1992 and 1993 were linked to undercooked hamburger meat contaminated with E. coli.
Other risk factors for HUS are unknown, although some cases are due to a familial form of the disease. HUS may occur with a variety of other diseases and infections, and after taking certain medications such as mitomycin C or ticlopidine.
HUS is more complicated in adults. It is similar to another disease called thrombotic thrombocytopenic purpura (TTP).
Symptoms
HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. Persons with this condition may urinate far less than normal. Urine output may almost stop.
Red blood cell destruction leads to symptoms of anemia.
Early symptoms:
Later symptoms:
Signs and tests
The health care provider will perform a physical exam. This may show:
- Liver or spleen swelling
- Nervous system changes
Laboratory tests will show signs of hemolytic anemia and acute renal failure. Laboratory tests may include:
Other tests:
Treatment
Treatment may involve:
- Dialysis
- Medications such as corticosteroids
- Transfusions of packed red cells and platelets
Some people may have the liquid portion of their blood (plasma) removed and replaced with fresh (donated) plasma, or the plasma is filtered to remove antibodies from the blood.
Expectations (prognosis)
This is a serious illness in both children and adults, and it can cause death. With proper treatment, more than half of patients will recover. The outcome is better in children than adults.
Complications
Calling your health care provider
Call your health care provider if you develop symptoms of HUS. Emergency symptoms include:
- Blood in the stool
- No urination
- Reduced alertness (consciousness)
Call your health care provider if you have had an episode of HUS and your urine output decreases, or you develop other new symptoms.
Prevention
You can prevent the known cause, E. coli, by cooking hamburger and ground meats well and avoiding contact with unclean water.
References
McMillan R. Hemorrhagic disorders: abnormalities of platelet and vascular function. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 179.
