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Haemolytic uraemic syndrome 

Haemolytic uraemic syndrome

Chapter:
Haemolytic uraemic syndrome
Author(s):

Paul Warwicker

and Timothy H J Goodship

DOI:
10.1093/med/9780199204854.003.211005_update_001

Update: Pregnancy related HUS—frequently associated with mutations in complement components.

Updated on 25 May 2011. The previous version of this content can be found here.
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date: 30 March 2017

The haemolytic uraemic syndrome (HUS) is characterized by the triad of: (1) microangiopathic haemolytic anaemia, (2) thrombocytopenia, and (3) acute renal failure. Endothelial cell activation leads to a change in phenotype from an anticoagulant to a procoagulant state.

Diarrhoeal associated (D+) HUS—this accounts for over 90% of cases and is the commonest cause of acute renal failure in children. The usual pathogen is enterohaemorrhagic Escherichia coli, notably E. coli O157:H7, and the commonest vehicle for transmission is ground (minced) beef. Release of a microbial toxin (verocytotoxin) inhibits ribosomal function and causes severe illness (mortality of 3–5%, renal replacement therapy is required in 50%). Treatment is supportive as no specific treatment has yet been proved to be beneficial, although plasma exchange is often used.

Nondiarrhoeal (D−) HUS—this can be familial (with 60% of cases having mutations in various complement components), or related to drugs (ciclosporin, tacrolimus, various cytotoxic drugs, etc.), pregnancy (also known as postpartum renal failure), infections (e.g. HIV), systemic disorders (e.g. systemic lupus erythematosus), malignancies, or transplantation (e.g. renal), or is of unknown cause (idiopathic). Treatment is supportive, with plasma exchange also used in most cases. There is a 50% risk of recurrence after renal transplantation.

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