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Post-Diarrheal Hemolytic Uremic Syndrome (D+HUS). Richard L. Siegler M.D. Professor Emeritus University of Utah School of Medicine. What is Post-Diarrheal Hemolytic Uremic Syndrome (D+HUS) and where did it come from ?. The syndrome includes: Acute kidney failure Hemolytic anemia

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post diarrheal hemolytic uremic syndrome d hus
Post-Diarrheal Hemolytic Uremic Syndrome (D+HUS)
  • Richard L. Siegler M.D.
  • Professor Emeritus
  • University of Utah School of Medicine
what is post diarrheal hemolytic uremic syndrome d hus and where did it come from
What is Post-Diarrheal Hemolytic Uremic Syndrome (D+HUS) and where did it come from ?
  • The syndrome includes:
    • Acute kidney failure
    • Hemolytic anemia
    • Thrombocytopenia (low platelet count)
  • Most common cause of acute renal (kidney) failure in young children; also occurs in older children and adults
how did these otherwise harmless e coli become such killers
How did these otherwise harmless E. coli become such killers?
  • DNA from a Stx producing bacterium (Shigella dysenteriae type 1) transferred by bacteriophage to E. coli
  • This provided E. coli with genes to produce Shiga toxin (Stx), one of the most potent toxins known to man  
pathogenic cascade from diarrhea to dialysis
Pathogenic Cascade;from diarrhea to dialysis
  • Chain of events:
    • ingestion of Stx producing E. coli
    • multiplication in bowel
    • absorption of Stx into circulation
pathogenic cascade cont
pathogenic cascade, cont.
  • Chain of events, cont.:
    • attachment of Stx to receptors in kidney, and occasionally other organs
    • movement of toxin into cells
    • cell injury or death
pathogenic cascade cont the end result is
pathogenic cascade, cont.the end result is:
  • hemorrhagic colitis (bloody diarrhea)
  • kidney injury, causing acute renal failure
  • damage of red blood cells causing anemia
  • trapping and destruction of platelets causing bleeding
  • occasional damage to other organs (brain, pancreas, heart, lungs, eyes)
signs and symptoms
Signs and Symptoms
  • diarrhea (usually bloody) with severe pain; can be mistaken for appendicitis
  • pallor
  • bruises
  • seizures and/or coma (occasionally)
  • little (oliguria) or no (anuria) urine
  • high blood pressure
  • pancreatic damage (diabetes)
hospital course and treatment
Hospital course and Treatment
  • meticulous attention to salt and water balance
  • dialysis for kidney failure
  • blood transfusions for anemia
  • Platelet transfusions for bleeding
  • aggressive nutritional support (e.g., total parenteral nutrition [TPN])
  • treatment of high blood pressure
  • treatment of seizures
outcomes
Outcomes
  • Death in 3-5%, due to:
    • brain damage (stroke and/or brain swelling)
    • bowel necrosis and perforation
    • heart damage
    • lung injury
    • multiorgan injury (seen in most fatal cases)
outcomes cont
outcomes, cont.
  • Those who survive may be left with:
    • permanent kidney damage
    • brain damage
    • hypertension
    • diabetes
monitoring
Monitoring
  • Chronic kidney failure may occur decades later (due to hyperfiltration injury)
    • most common in those who had prolonged kidney failure (oligoanuria) >10 days
    • proteinurea (protein in the urine) is an early sign
monitoring cont
monitoring, cont.
  • Hypertension may begin years later (in those with chronic kidney damage)
  • Diabetes can latter occur (in those with pancreatic injury)
summary
Summary
  • D+ HUS is a life threatening complication of Stx producing E. coli
  • It occurs primarily in infants and young children, but also affects adults
  • Is fatal 3-5% of victims
  • Survivors (30-50%) are left with chronic kidney damage that can be progressive later in life
  • Life long monitoring is necessary