SAQ 391 – Paediatrics – haemolytic uraemic syndrome

Paediatrics – haemolytic uraemic syndrome.

a. Blood-test interpretation

• Microangiopathic haemolytic anaemia or haemolytic anaemia, supported by schistocytes, low haptoglobin, raised bilirubin, and severe anaemia.
• Thrombocytopaenia.
• Renal failure or acute kidney injury, with creatinine 250 micromol/L and eGFR 35 mL/min/1.73m2.

b. Diagnosis

• Haemolytic uraemic syndrome.

c. Pathogens

• Shiga-toxin-producing E. coli.
• Streptococcus pneumoniae.
• HIV or influenza can also be associated.

d. Differentials

• Immune thrombocytopaenia.
• Disseminated intravascular coagulation.
• Sepsis.
• Vasculitis.

e. Management

• Transfuse red cells to keep haemoglobin above 80 g/L.
• Avoid platelet transfusion unless there is significant bleeding or an invasive procedure is required.
• Manage fluids judiciously: replace deficit if depleted, but monitor for overload and use diuresis if overloaded.
• Correct electrolytes, particularly hyperkalaemia.
• Dialysis if anuric or oliguric, severe electrolyte disturbance, metabolic acidosis, or refractory fluid overload.
• Treat neurological complications such as seizures.
• Manage blood pressure, usually hypertension.
• Consider plasma exchange or eculizumab in selected severe cases, especially neurological dysfunction; antibiotics and antithrombotics are not recommended.