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Hemolytic Uremic Syndrome
(HUS)

Medical Author: Benjamin C. Wedro, MD, FAAEM
Medical Editor: Melissa Conrad Stöppler, MD

What is a "syndrome?"

The definition of a syndrome in medicine is a collection of symptoms (patient complaints), signs (findings on physical examination), and laboratory or imaging findings that tend to group together and be associated with a specific disease or illness.

What is hemolytic uremic syndrome (HUS)?

Hemolytic uremic syndrome (HUS) is a disease of blood clotting within the capillaries, the smallest blood vessels in the body. As red blood cells pass through the clogged capillaries, they are sheared apart and broken. This is called hemolysis and accounts for the first part of the syndrome's name. The other half of the syndrome's name refers to kidney failure in which urea and other waste products build up in the bloodstream because the kidney cannot filter and dispose of them. (urea=a waste chemical + emia= in the blood).

HUS is also related to another disease caused by a similar clotting process within the capillaries called thrombotic thrombocytic purpura (TTP). Within the medical literature, these two conditions are often considered together. HUS is more widely known, however, because it has been discussed in the press because of its relationship to a special type of E. Coli (Escherichia coli) infection associated with food poisoning.

What causes hemolytic uremic syndrome?

While the cause of HUS has not been established with certainty, researchers believe that an abnormal inflammation reaction is stimulated in the blood stream causing platelets (the parts of the blood that form clots) to sludge in small blood vessels and form blood clots where they are not supposed to. This uses up the platelets and causes a shortage of them in the rest of the body. This abnormal reaction may be caused by a yet unidentified chemical that is produced in blood plasma (the fluid or non-cellular part of the bloodstream).

The triggering events for HUS are varied and include:

  • Enterohemorrhagic E. Coli is a type of bacteria that causes bloody diarrhea. E. coli is a very common type of bacteria in the body, and it is only the OH157:H7 type that causes the problem. It can be found in undercooked meat and other foods; and is one of the causes of food recalls and restaurant-related food poisoning outbreaks. This accounts for almost 90% of HUS in children.

  • Pregnancy and the early postpartum (after delivery) time period and the use of birth control pills are the more common associations with HUS

  • Pneumonia caused by the Streptococcus pneumoniae bacteria is another possible precipitating factor. HUS is an unusual complication of this common infection. Most often, this pneumonia is treated with antibiotics as an outpatient.

  • Medications may be also associated with HUS, including some chemotherapy and immunosuppression drugs, birth control pills, ticlopidine (Ticlid) and quinine [(Quinerva, Quinite) an anti–platelet drug].

  • AIDS may be associated with HUS.

Furthermore, there is an inherited type of HUS due to a gene mutation that affects the blood clotting mechanism.



Next: What are the signs and symptoms of hemolytic uremic syndrome? »


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