Paediatric Acute care Guidelines PMH Emergency Department

Haemolytic Uraemic Syndrome (HUS) is a disease characterised by:

  • Microangiopathic haemolytic anaemia (destruction of red blood cells)
  • Acute renal failure
  • Thrombocytopaenia

 

Background

General

Typical Heamolytic Uraemic Syndrome
  • Is among the commonest causes of acute renal failure in children
  • Mortality of 5-10%
  • In the majority of cases it follows gastrointestinal tract infection with verotoxin producing E. coli, but it also can be induced by Shigella, Campylobacter and different viruses.  In most of these there is preceding diarrhoea, which is frequently bloody (D+HUS)
Atypical Heamolytic Uraemic Syndrome
  • Represents in 5-10% of cases
  • May develop without a diarrhoea prodrome
  • Causes:
    • Complement defects
    • Familial cases
    • Drug toxicity (e.g. chemotherapy, Tacrolimus, Cyclosporin, oral contraceptives, Valacyclovir)
    • Immune mediated (e.g. Quinine, Clopidogrel)
    • Malignancy
    • Hereditary (e.g. inborn error of cobalamin deficiency)
    • Connective tissue disease (e.g. SLE, scleroderma, antiphospholipid antibody syndrome)
    • Other glomerunephritides (e.g. APIGN, memobranoproliferative GN)

 

Assessment

History

  • Most children with HUS present 5-10 days after the onset of a bloody diarrhoea with:
    • Oliguria
    • Haematuria
    • Anaemia
    • Oedema
    • Renal failure
    • Hypertension
  • Myocardial infarction, stroke, pancreatitis, liver necrosis, encephalopathy and seizure have also been described

Examination

Full systems examination
Assessment of cardiovascular and intravascular volume status is very important

Consider:

    • Thirst, restlessness and confusion
    • Capillary refill
    • Skin turgor
    • Olyguria
    • Fontanelle tension
    • Blood pressure
    • Heart rate
    • Evidence of oedema

 

Investigations

  • FBC, film and differential
  • U&E
  • Liver function tests
  • CRP
  • BGL
  • Coagulation profile
  • Group and hold
  • E-coli serology (clotted sample – red top tube)
  • Blood cultures if Pneumococcal cause suspected
  • Stool M, C & S
  • Urinalysis and urine M, C & S
  • Other investigations as clinically indicated (e.g. CXR, renal US, ECG, head CT or MRI, EEG)

Differential diagnoses

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

Management

Initial management

Fluid Management:

  • Establish IV access
  • Hypovolaemia should be treated with 0.9% saline boluses, or packed red blood cells, depending on the clinical status (usually 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
    • Hyponatremia 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 PICU Consultant)
Hypertension:

  • Usually secondary to volume overload
  • If unresponsive to diuretics, try vasodilator treatment (e.g. Nifedipine)
Abdominal pain and vomiting:

  • Due to colitis in post-diarrhoeal HUS
    • Treat initially with paracetamol
    • May require opiate analgesia (but this will desrease bowel motility and should be avoided if possible)
    • Do not prescribe NSAIDs such as ibuprofen
Antibiotics

  • Indicated in pneumonia related HUS (not in typical D+HUS)

 

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 need to be admitted
The Paediatric Renal Team (and General Surgical Team for PD catheter / line placement) should be consulted early

 

Nursing

Tags

We want your feedback!

Help us provide guidelines that are useful to you, the clinician.

Give feedback here