Hemolytic-uremic syndrome (HUS) is a serious disorder that usually occurs in children and involves the formation of small blood clots throughout the body that block the flow of blood to vital organs such as the brain, heart, and kidneys.
Symptoms are related to where in the body blood clots form.
Diagnosis is based on the person's symptoms and blood test results.
(See also Overview of Platelet Disorders and Overview of Thrombocytopenia.)
HUS is a rare disorder in which many small blood clots (thrombi) form suddenly throughout the body. Hemolytic means that red blood cells break down, and uremic means that kidney injury causes urea (a waste product) to accumulate in the blood. HUS is related to thrombotic thrombocytopenic purpura (TTP), but it occurs more often in children and more often causes kidney failure, whereas TTP is more common among adults.
The small blood clots that form in HUS block small blood vessels throughout the body, particularly those in the brain, heart, and kidneys. The blood vessel blockage damages organs and can break apart red blood cells that pass through partially blocked vessels. The blood clots also mean that an abnormally high number of platelets are being used up, which leads to a sharp decrease in the number of platelets in the bloodstream (thrombocytopenia).
Platelets are cells that are made in the bone marrow and circulate in the bloodstream and help blood clot. Having too few platelets is termed thrombocytopenia. The blood usually contains about 140,000 to 440,000 platelets per microliter (140 to 440 × 109 per liter). When the platelet count falls below about 50,000 platelets per microliter of blood (50 × 109 per liter), bleeding can occur even after relatively minor injury. The most serious risk of bleeding, however, generally does not occur until the platelet count falls below 10,000 to 20,000 platelets per microliter of blood (10 to 20 ×109 per liter). At these very low levels, bleeding may occur without any recognized injury.
Hemolytic-uremic syndrome usually occurs following an intestinal infection that results from ingestion of food contaminated with the bacteria Escherichia coli O157:H7 or other toxin-producing bacteria.
Symptoms of Hemolytic-Uremic Syndrome
In hemolytic-uremic syndrome (HUS), symptoms develop suddenly.
Symptoms in HUS are quite distinct from symptoms of most other forms of thrombocytopenia.
In HUS, children usually first have vomiting and diarrhea. The diarrhea is often bloody. The main symptoms of complications of HUS are related to blood clots that develop in the kidneys, causing damage that is usually severe and often progresses to kidney failure, requiring dialysis. HUS does not often cause brain symptoms.
Diagnosis of Hemolytic-Uremic Syndrome
Blood tests to measure platelet count and clotting
Blood and urine tests to measure kidney function
Tests to rule out other disorders that cause a low platelet count and bleeding
Doctors suspect hemolytic-uremic syndrome (HUS) when they find a low platelet count in children who have been ill or, less commonly, in people who have taken certain medications.
Although there are no blood tests that specifically diagnose HUS, doctors do a number of blood tests that, together with people's symptoms, help make the diagnosis. These blood tests often include a complete blood count, tests demonstrating that red blood cells are being destroyed such as examination of a sample of blood under a microscope (blood smear), and tests to determine how well the kidneys are working.
Treatment of Hemolytic-Uremic Syndrome
Usually kidney dialysis
About half of children with hemolytic-uremic syndrome temporarily require kidney dialysis, in which a dialysis machine removes waste products from the blood. Most often, the kidneys recover, but some children have permanent kidney damage.
complement, a component of the immune system. They reduce the rate of kidney damage, and in some people, they may rapidly restore kidney function. People who take eculizumab,ravulizumab, or pegcetacoplan are at higher risk than normal for meningococcal meningitis, so they should receive the meningococcal vaccine to prevent meningococcal infections.
