Hemolytic Uremic Syndrome (cont.)

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What is the treatment for hemolytic uremic syndrome?

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Typical HUS in children tends to be self-limiting, and supportive care is often all that is needed. This may include intravenous fluids for rehydration and rebalancing of electrolytes like sodium and potassium, which can be lost with the diarrhea.

Blood transfusions are only used for the most severe cases of anemia in which the hemoglobin falls below 6 or 7 g/dL (depending on age, the normal value is 11-16).

Kidney failure may be managed expectantly (by observation and supportive care), and dialysis is not often required.

Adults with atypical HUS tend to become more ill and need more aggressive therapy than children with the condition. In addition to the supportive care discussed above, plasmapheresis or plasma exchange may be required. Since it is thought there is an abnormal chemical in the plasma stimulating the abnormal clot formation, removing the plasma and replacing it with donor plasma is helpful in treating adult HUS.

Dialysis may be needed while awaiting recovery of the kidneys from the illness.

Eculizumab (Soliris) has been approved by the FDA for the treatment of atypical HUS. It is a monoclonal antibody that decreases the blood clotting in the capillary blood vessels, decreasing the potential destruction of cells. This type of therapy decreases the body's immune capabilities, and the risk of infection increases.

What is the prognosis of hemolytic uremic syndrome?

Typical HUS in children tends to be self-limiting, and full recovery is most likely.

Adults do less well. Without aggressive therapy like plasmapheresis and dialysis, up to 25% of those affected with atypical HUS may die in the acute phase of the disease, and 50% may have developed long-term kidney impairment.

Patients with HUS not related to a diarrheal illness have a worse prognosis than those whose illness is due to gastrointestinal infection.

In those patients with genetically caused HUS, relapsing illness is common as are kidney failure requiring dialysis and death.

Medically Reviewed by a Doctor on 11/15/2013

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