Abstract

Haemolytic uraemic syndrome (HUS), comprising microangiopathic haemolytic anaemia, thrombocytopaenia and acute kidney injury, remains the leading cause of paediatric intrinsic acute kidney injury, with peak incidence in children aged under 5 years. HUS most commonly occurs following infection with Shiga toxin-producing Escherichia coli (STEC-HUS). Additionally, HUS can occur as a result of inherited or acquired dysregulation of the alternative complement cascade (atypical HUS or aHUS) and in the setting of invasive pneumococcal infection. The field of HUS has been transformed by the discovery of the central role of complement in aHUS and the dawn of therapeutic complement inhibition. Herein, we address these three major forms of HUS in children, review the latest evidence for their treatment and discuss the management of STEC infection from presentation with bloody diarrhoea, through to development of fulminant HUS.