Haemolytic Uraemic Syndrome


These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline. 

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To guide Emergency Department (ED) staff with the assessment and management of haemolytic uraemic syndrome.


Haemolytic Uraemic Syndrome (HUS) is a disease characterised by:
  • Microangiopathic haemolytic anaemia (destruction of red blood cells).
  • Acute renal failure.
  • Thrombocytopaenia.
  • HUS is among the commonest causes of acute renal failure in children.
  • Mortality of 5-10%. 



  • Post diarrhoeal illness (usually bloody):
    • Shiga toxin producing Escherichia coli (STEC), accounts for 90% of cases.
    • Shigella, Campylobacter and other viruses.
  • Non-diarrhoeal
    • Streptococcus pneumoniae, Approximately 10% of all paediatric HUS.
    • Usually present with pneumonia with effusion.


  • Complement defects.
  • Familial cases.
  • Medications (e.g. chemotherapy, tacrolimus, ciclosporin, oral contraceptives, valaciclovir, quinine).
  • Immune mediated.
  • Malignancy.
  • Hereditary (e.g. inborn error of cobalamin deficiency).
  • Connective tissue disease (e.g., systemic lupus erythematosus (SLE), scleroderma, antiphospholipid antibody syndrome).
  • Other glomerulonephritides (e.g., acute post-infectious glomerulonephritis (APIGN), membranoproliferative glomerulonephritis (GN)).



Most children with HUS present 5-10 days after the onset of a bloody diarrhoea with:
  • Oliguria
  • Haematuria
  • Anaemia
  • Oedema
  • Renal failure
  • Hypertension.

Pneumococcal associated HUS usually presents with pneumonia.

Myocardial infarction, stroke, pancreatitis, liver necrosis, encephalopathy and seizure have also been described.


  • Full system examination
  • Assessment of cardiovascular and intravascular volume status is very important.
  • Thirst, restlessness and confusion
  • Capillary refill
  • Skin turgor
  • Oliguria
  • Fontanelle tension
  • Blood pressure
  • Heart rate
  • Evidence of oedema.


  • Full blood picture
  • Electrolytes, Urea and Creatinine (EUC)
  • Liver function tests (LFT)
  • C-Reactive Protein (CRP)
  • Blood glucose level (BGL)
  • Coagulation profile
  • Group and hold
  • Blood cultures if pneumococcal cause suspected
  • Stool microscopy, culture and sensitivity (MC&S)
  • Stool – Shigella-toxin producing E-Coli PCR
  • Urinalysis and urine MC&S.
  • Other investigations as clinically indicated (e.g. Chest X-ray, renal ultrasound, electrocardiogram (ECG), head computerised tomography (CT) or magnetic resonance imaging (MRI), electroencephalogram (EEG)).

Differential diagnoses

  • Sepsis
  • Acute post-Streptococcal glomerulonephritis
  • Disseminated intravascular coagulation
  • Immune thrombocytopaenic purpura (ITP)
  • Thrombotic thrombocytopaenia purpura
  • Systemic lupus erythematosis (SLE)
  • Vasculitis.


Fluid management
  • Establish IV access
  • Hypovolaemia should be treated with sodium chloride 0.9% saline boluses, or packed red blood cells, depending on the clinical status (usually sodium chloride 0.9% saline in the ED setting)
  • In the presence of oligo-anuria and fluid overload, fluid should be administered cautiously and should not exceed insensible fluid losses plus urine output (or less)
    • Insensible fluids:
      • 0-10kg weight - give 25mL/kg/day
      • 10-20kg weight - give 12.5mL/kg/day
      • Then 5mL/kg/day for each additional kg over 20kg weight
  • Hyponatraemia is treated with fluid restriction
  • Electrolyte abnormalities: please discuss with paediatric renal team
Altered consciousness/focal neurological signs
  • If these develop, immediate discussion should be undertaken with a senior colleague (e.g. ED consultant, renal consultant or Paediatric Critical Care (PCC) consultant)
  • Usually secondary to volume overload
  • If unresponsive to diuretics, try vasodilator treatment (e.g. Isradipine, clonidine).
Abdominal pain and vomiting
  • Due to colitis in post-diarrhoeal HUS
    • Treat initially with paracetamol (refer to Paracetamol Monograph – Medication Management Manual (internal WA health only)
    • May require opiate analgesia (but this will decrease bowel motility and should be avoided if possible)
    • Do not prescribe non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen.

Further management

Indications for dialysis

  • Fluid overload resistant to diuretic therapy
  • Hyperkalaemia
  • Intractable acidosis
  • Symptoms of uraemia
  • Likely progression of one of the above

Patients needing dialysis (most) will usually be placed on peritoneal dialysis (PD). Exceptions are those with severe colitis, cerebral HUS or profound metabolic abnormalities (where haemodialysis or haemofiltration techniques may be considered).

All patients diagnosed with HUS must be admitted.

The paediatric renal team (and general surgical team for PD catheter / line placement) should be consulted early.


  • Complete and record a full set of observations on the Observation and Response Tool and record additional information on the Clinical Comments chart.
  • Complete a full set of neurological observations if clinically indicated.
  • Maintain an accurate record of fluid balance
  • Obtain an accurate patient weight.


  1. Niaudet P & Boyer OG, UpToDate: Overview of hemolytic uremic syndrome in children. 2021 Overview of hemolytic uremic syndrome in children - UpToDate (health.wa.gov.au)
  2. Textbook of Paediatric Emergency Medicine 3rd Edition Cameron P, Browne GJ, Mitra B, et al (2018) Publisher: Elsevier Edition updated
  3. Nelson Textbook of Pediatrics: 21st Edition Robert M. Kliegman, St Geme JW, Blum MJ et al. 2020 Publisher: Elsevier
  4. Fleisher and Ludwig’sTextbook of Pediatric Emergency Medicine, 8th Edition. Shaw K and Bachur RG (2020) Publisher: Wolters Kluwer

Endorsed by:  Nurse, Co-director, Surgical Services  Date: Apr 2024

 Review date:   Apr 2022

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