hemolytic-uremic syndrome
MONDO:0001549
Acute kidney injury associated with microangiopathic hemolytic anemia and thrombocytopenia.
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Hemolytic–uremic syndrome
Hemolytic–uremic syndrome (HUS) is a syndrome characterized by low red blood cells, acute kidney injury (previously called acute renal failure), and low platelets. Initial symptoms typically include bloody diarrhea, fever, vomiting, and weakness. Kidney problems and low platelets then occur as the diarrhea progresses. Children are more commonly affected, but most children recover without permanent damage to their health, although some children may have serious and sometimes life-threatening complications. Adults, especially the elderly, may show a more complicated presentation. Complications may include neurological problems and heart failure. Most cases occur after infectious diarrhea due to a specific type of E. coli called O157:H7. Other causes include S. pneumoniae, Shigella, Salmonella, and certain medications. The underlying mechanism typically involves the production of Shiga toxin by the bacteria. Atypical hemolytic uremic syndrome (aHUS) is often due to a genetic mutation and presents differently. However, both can lead to widespread inflammation and multiple blood clots in small blood vessels, a condition known as thrombotic microangiopathy. Treatment involves supportive care and may include dialysis, steroids, blood transfusions, or plasmapheresis. About 1.5 per 100,000 people are affected per year. Less than 5% of those with the condition die. Of the remainder, up to 25% have ongoing kidney problems. HUS was first defined as a syndrome in 1955.
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Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.