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Atypical hemolytic uremic syndrome (aHUS) is a disease that causes abnormal blood clots in small blood vessels, particularly in the kidneys. These clots can cause serious medical problems such as hemolytic anemia, thrombocytopenia, and kidney failure. Because a number of other problems can have similar clinical features, a laboratory test to detect deficiency and inhibitors of a protein called ADAMTS13 are often used to help confirm the diagnosis. ADAMTS13 is responsible for the breakdown of von Willebrand factor (vWF), a protein that links platelets, blood clots, and the blood vessel wall in the process of blood coagulation.