Hemolytic-uremic syndrome history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

History and Symptoms

The classic childhood case of HUS occurs after bloody diarrhea caused by E. coli O157:H7, a strain of E. coli that expresses verotoxin (also called Shiga toxin). The toxin enters the bloodstream, attaches to renal endothelium and initiates an inflammatory reaction leading to acute renal failure (ARF) and disseminated intravascular coagulation (DIC). The fibrin mesh destroys red blood cells and captures thrombocytes, leading to a decrease of both on complete blood count. The usual age of onset is between 2 and adolescence.

HUS occurs after 2-7% of all E. coli O157:H7 infections.

Adult HUS has similar symptoms and pathology but is an uncommon outcome of the following: HIV; antiphospholipid syndrome (associated with Lupus erythematosus and generalized hypercoagulability); post partum renal failure; malignant hypertension; scleroderma; and cancer chemotherapy (mitomycin, cyclosporine, cisplatin and bleomycin).

A third category is referred to as familial HUS. It represents 5-10% of HUS cases and is largely due to mutations in the complement proteins factor H, membrane cofactor protein and factor I leading to uncontrolled complement system activation. Recurrent thromboses result in a high mortality rate.

  • HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. Persons with this condition may urinate less than normal. Urine output may almost stop.
  • Red blood cell destruction leads to symptoms of anemia.
  • Early symptoms:
  • Later symptoms:
  • Bruising
  • Decreased consciousness
  • Low urine output
  • No urine output
  • Pallor
  • Seizures -- rare
  • Skin rash that looks like fine red spots (petechiae)
  • Yellow skin (jaundice)

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