Haemolytic Uraemic Syndrome (HUS)

September 2, 2011 | By | Reply More

Haemolytic Uraemic Syndrome (HUS)


  • The kidney is the main organ targeted
  • Infants, young children and the elderly are mainly afflicted
  • Some cases may be associated with ADMTS-13 deficiency; most are not
  • May be caused by verotoxin producing E. Coli 0157 or Shigella dysenteriae
  • Less haemolysis than TTP, more prominent coagulation abnormalities

Pathogenesis of HUS

  • Verotoxin or Shiga toxin bind to the Gb3 receptor on the glomular capillary endothelial cells.
  • Receptors are particularly dense in infants, young children and the elderly
  • The toxin-receptor complex is endocytosed

–cytolysis, extensive endothelial swelling and desquamation

–Massive thrombus formation in the renal microvascular circulation

Clinical Features

  • Acute renal failure

– In one third of patients renal dysfunction doesn’t resolve, requiring maintenance dialysis or kidney transplantation

  • Fits
  • Diarrhoea
  • Hypertension
  • Self limiting

Laboratory Diagnosis – HUS

  • Urea and Creatinine ­­↑↑
  • Microbiological identification of E. Coli 0157 or Shigella dysenteriae
  • Fragments and features of renal failure on blood film

Treatment of HUS

  • Renal Dialysis in acute renal failure
  • Antibiotics
  • Vaccination in endemic areas
  • Plasma exchange usually not recommended
  • Anti-motility drugs not recommended

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Category: Medical, Pathology

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